Alford Otology PDF

Grand Rounds Archive - Otology/Neurotology
Grand Rounds Archive
Otology/Neurotology
Acute Otitis Media:A Public Health Perspective [3/18/99]
Autoimmune Inner Ear Disease [4/8/93]
Barotrauma of the Middle and Inner Ear [3/23/95]
Bell's Palsy [7/20/95]
Unusual Cerebellopontine Angle Neoplasms [11/4/93]
Pediatric Cholesteatoma [10/7/93]
Chordomas of the Skull Base [10/1/92]
Congenital Aural Atresia [11/2/95]
Congenital Inner Ear Malformations [11/11/93]
Diving Medicine [01/13/00]
Eustachian Tube Function and Dysfunction [7/11/96]
Traumatic Injuries to the Facial Nerve [10/12/91]
Facial Reanimation of the Chronically Paralyzed Face [9/29/94]
Therapeutic Options for Treatment of Glomus Tumors [10/26/91]
http://www.bcm.edu/oto/grand/otology.html (1 of 2) [5.11.2005 10:38:17]
Grand Rounds Archive - Otology/Neurotology
Considerations in the Evaluation of the Hearing Impaired Child [3/11/93]

Update on Hearing Aids [6/3/93]
Herpes Zoster Oticus [8/21/97]
Neurotologic Manifestations Of HIV Infection [3/24/94]
Infectious Intracranial Complications of Suppurative Ear Disease [3/18/93]
Lateral Sinus Thrombosis [5/7/92]
Acute Mastoiditis [2/3/94]
Surgical Management Of Meniere's Syndrome [4/14/94]
Myringoplasty and Tympanoplasty [2/16/95]
Osteodystrophies of the Middle Ear and Temporal Bone [2/8/95]
Ototoxicity [8/20/92]
Ototoxicity [4/25/96]
Otitis Externa [10/12/95]
Pathology And Pathogenesis Of Otitis Media [4/21/94]
The Diagnosis and Management of Perilymphatic Fistulas [10/21/93]
Lesions of the Petrous Apex [8/4/94]
Complications of Stapedectomy [11/11/99]
Issues in Stapedectomy [2/6/92]
Sudden Sensorineural Hearing Loss [3/5/92]
Sudden Sensorineural Hearing Loss [11/18/93]
Temporal Bone Fractures [4/2/92]
Temporal Bone Gunshot Wounds: Evaluation and Management [8/5/93]
Subjective Tinnitus [03/12/98]
Tuberculous Otitis Media [11/22/92]
Tympanostomy Tubes [2/23/95]
Vestibular Adaptation and Rehabilitation [5/21/92]
Vestibular Neuritis [7/14/94]
Laryngology | Neoplasms | Pediatric | Plastics | Rhinology | Others | Main | Home
2001, The Bobby R. Alford Department of Otorhinolaryngology and Communicative Sciences,

Baylor College of Medicine
One Baylor Plaza, NA102, Houston, TX 77030 oto@bcm.tmc.edu
URL: http://www.bcm.tmc.edu/oto (Modified: 12/12/01)
http://www.bcm.edu/oto/grand/otology.html (2 of 2) [5.11.2005 10:38:17]
From the Grand Rounds Archive at Baylor
Grand Rounds Archives
**** DISCLAIMER ****

The information contained within the Grand Rounds Archive is intended for use by
doctors and other health care professionals. These documents were prepared by
resident physicians for presentation and discussion at a conference held at The
Baylor College of Medicine in Houston, Texas. No guarantees are made with
respect to accuracy or timeliness of this material. This material should not be used
as a basis for treatment decisions, and is not a substitute for professional
consultation and/or peer-reviewed medical literature.
Acute Otitis Media: A Public Health Perspective

Derek Drummond, M.D.
March 18, 1999
Case Presentation
J.D., a previously healthy girl, presented to her pediatrician at 6
http://www.bcm.edu/oto/grand/031899.html (1 of 6) [5.11.2005 10:38:33]
months of age with a 3-day history of increasing irritability and

fevers. History revealed that she had upper respiratory tract
symptoms for 3 days that included nasal congestion, rhinorrhea,
dry cough, decreased appetite and low-grade fevers. The parents
became concerned the night before presentation when she
became very irritable, appeared to be in pain, and vomited once
after bottle-feeding. The girl was seen pulling on her ears and
was inconsolable prior to arrival at the pediatrician's office. She
had no previous medical problems and was not taking any
medication other than acetaminophen.
Physical exam demonstrated a 6-month old girl who was crying
in her mother's arms. She was alert but not cooperative. She had
a temperature of 101.5 F. Examination of the ears revealed a
right tympanic membrane that was bulging, erythematous, and
didn't move with pneumotoscopy. The nasal mucosa was
congested and the patient was mouth breathing. The remainder
of the physical exam was unremarkable. She was diagnosed with
acute otitis media and prescribed a 10-day course of amoxicillin
at 40mg/kg/day. Within 24 hours, her symptoms were improving
and she began to feed normally. Follow-up at 2 weeks revealed
bilateral middle-ear effusions in a happy, asymptomatic child.
The MEE would resolve spontaneously over the next 3 months.
Bibliography
Berman S. Otitis media in developing countries. Pediatrics 1995; 96:126-131.
Berman S, Byrns PJ, Bondy J, Smith PJ, Lezotte D. Otitis media-related antibiotic prescribing
patterns, outcomes, and expenditures in a pediatric medicaid population. Pediatrics 1996;100:585592.
Bluestone CD. Otitis media. In: Behrman RE, Vaughan RE, Nelson WE, editors. Nelson
Textbook of Pediatrics, 13th edition. Philadelphia: W.B. Saunders; 1987. pp. 880.
Bluestone CD, Klein JO. Otitis media, atelectasis, and eustachian tube dysfunction. In: Bluestone
CD, Stool SE, editors. Pediatric Otolaryngology, 3rd edition. Philadelphia: W.B. Saunders;1996.
pp. 388-582.
Bluestone CD, Stephenson JS, Martin LM. Ten-year review of otitis media pathogens. Pediatr
Infect Dis J 1992;11:S7-S11.
Blumer JL. Pharmacokinetics and pharmacodynamics of new and old antimicrobial agents for
acute otitis media. Pediatr Infect Dis J 1998;17:1070-1075.
Bottenfield GW, Burch DJ, Hedrick JA, Schaten R, Rowinski CA, Davies JT. Safety and
tolerability of a new formulation (90 mg/kg/day divided every 12 h) of amoxicillin/clavulanate
(Augmentin) in the empiric treatment of pediatric acute otitis media caused by drug-resistant
Streptococcus pneumoniae. Pediatr Infect Dis J 1998;17:963-968.
Boulesteix J, Dubreuil C, de La Roque F, Trinh A, Scheimberg A. Effects of cefixime or coamoxiclav treatment on nasopharyngeal carriage of Streptococcus pneumoniae and Haemophilus
influenzae in children with acute otitis media: J Antimicrob Chemother 1998;41:253-258.
Branthaver B, Greiner DL, Eichelberger R Determination of cost-effective treatment of acute
otitis media from HMO records. Am J Health Syst Pharm 1997;54:2736-2740.
Brown DH. Education of medical practitioners in otitis media management. J Otolaryngol 1998;
2749-52.
Canafax DM, Yuan Z, Chonmaitree T, Deka K, Russlie HQ, Giebink GS. Amoxicillin middle ear
fluid penetration and pharmacokinetics in children with acute otitis media. Pediatr Infect Dis J
1998;17:149-156.
Casselbrant ML, Mandel EM, Kurs-Lasky M, Rockette HE, Bluestone CE. Otitis media in a
population of black American and white American infants, 0-2 years of age. Int J Pediatr
Otorhinolaryngol 1995;33:1-16.
Clements DA, Langdon L, Bland C, Walter E. Influenza A vaccine decreases the incidence of
otitis media in 6- to 30-month-old children in day care. Arch Pediatr Adolesc Med
1995;149:1113-1117.
Craig WA. Antimicrobial resistance issues of the future. Diagn Microbiol Infect Dis 1996;25:213217.
Culpepper L, From J. Routine antimicrobial treatment of otitis media: Is it necessary? JAMA
1997;278:1643-1645.
David CB, Hamrick HJ, Schwartz RJ. Follow-up after otitis media. N Engl J Med 1982;307:252.
DeMaria TF, Prior RB, Briggs BR, Lim DJ, Birck HG. Endotoxin in middle-ear effusions from
patients with chronic otitis media with effusion. J Clin Microbiol 1984;20:15-17.
Dowell SF, Schwartz B. Resistant pneumococci: protecting patients through judicious use of
antibiotics. Am Fam Phys 1997;55:1647-1654, 1657-1658.
Gates G.A. Acute otitis media and otitis media with effusion. In: Cummings CE, Harker LE,
editors. Otolaryngology, Head and Neck Surgery, 2nd edition. St. Louis: Mosby;1993. pp. 28082822.
Gates G.A. Workshop on effects of otitis media on the child: socioeconomic impact of otitis
media. Pediatrics 1983;71:639-652.
Gehanno P, N'Guyen L, Derriennic M, Pichon F, Goehrs JM, Berche P. Pathogens isolated
during treatment failures in otitis. Pediatr Infect Dis J 1998;17:885-890.
Giebink GS. Vaccination against middle-ear bacterial and viral pathogens. Ann N Y Acad Sci
1997; 830:330-352.
Heikkinen T, Ruuskanen O. New prospects in the prevention of otitis media. Ann Med
1996;28:23-30.
Hoekelman RA. Infectious illness during the first year of life. Pediatrics 1977;59:119-121.
Kaleida PH., Casselbrant ML, Rocket HE, Paradise JL, Bluestone CD, Blatter MM, Reisinger
KS, Wald ER, Supance JS. Amoxicillin or myringotomy or both for acute otitis media. Results of
a randomized clinical trial. Pediatrics 1991;87:466-474.
Klein JO. Clinical implications of antibiotic resistance for management of acute otitis media.
Pediatr Infect Dis J 1998;17:1084-1089.
Kozyrskyj AL, Hildes-Ripstein GE, Longstaffe SE, Wincott JL, Sitar DS, Klassen TP, et al.
Treatment of acute otitis media with a shortened course of antibiotics: a meta-analysis. JAMA
1998;279:1736-1742.
Klein JO, Teele DW, Masson R, Menyuk P, Rosner BA. Otitis media with effusion during the
first three years of life and the development of speech and language. In: Lim DJ, Bluestone CD,
Klein JO, Nelson DJ, editors. Recent Advances in Otitis Media With Effusion. Philapelphia, PA:
BC Decker;1983. pp. 332-333.
Ling D, McCoy RH, Levinson ED. The incidence of middle ear disease and its educational
implications among Baffin Island Eskimo children. Can J Public Health 1969;60:385-390.
Lister PD, Pong A, Chartrand SA, Sanders CC. Rationale behind high-dose amoxicillin therapy
for acute otitis media due to penicillin-nonsusceptible pneumococci: support from in vitro
pharmacodynamic studies. Antimicrob Agents Chemother 1997;41:1926-1932.

Lynn GE, Benitez JT. Temporal bone preservation in a 2600-year-old Egyptian mummy. Science
1974;183:200-202.
Makela PH, Karma P, Leinonen MK. Pneumococcal vaccine and otitis media in infancy.
Bull Eur Physiopathol Respir 1983;19:235-238.
Mandel EM, Casselbrant ML, Rockette HE, Bluestone CD, Kurs-Lasky M. Efficacy of 20versus 10-day antimicrobial treatment for acute otitis media. Pediatrics 1995;96:5-13.
Nelson WL, Kennedy DL, Lao CS, Kuritsky JN. Outpatient systemic anti-infective use by
children in the United States, 1977 to 1986. Pediatr Infect Dis J. 1988;7:505-509.
Paradise JL: Controversies: Treatment of acute otitis media. JAMA 1998;279:1784-1785.
Paradise JL. Managing otitis media: a time for change. Pediatrics 1995;96:712-715.
Pean Y, Goldstein FW, Guerrier ML. Highlights of the French antimicrobial resistance
surveillance project. French Study Group. Diagn Microbiol Infect Dis 1996;25:191-194.
Population Estimates Program, Population Division, U.S. Bureau of the Census, Washington, DC
Contact: Statistical Information Staff, Population Division, U.S. Bureau of the Census, 301-4572422 Internet Release date: March 17, 1998. www.census.gov/population/estimates/county/co-971/97C1_48.txt
Rathbun TA, Mallin R. Middle ear disease in a prehistoric Iranian population. Bull N Y Acad
Med 1977;53:901-905.
Rosenfeld RM. An evidence-based approach to treating otitis media. Pediatr Clin North Am
1996;43:1165-1181.
Rosenfeld RM, Vertrees JE, Carr J, Cipolle RJ, Uden DL, et al. Clinical efficacy of antimicrobial
drugs for acute otitis media: meta-analysis of 5400 children from thirty-three randomized trials. J
Pediatr 1994;124:355-367.
Saainen UM. Prolonged breast feeding as prophylaxis for recurrent otitis media. Acta Paediatr
Scand 1982;71:567-571.
Schaefer O. Otitis media and bottle-feeding. An epidemiological study of infant feeding habits
and incidence of recurrent and chronic middle ear disease in Canadian Eskimos. Can J Public
Health 1971;62:478-489.
Scheaffer RL, Mendelhall W, Ott L. Simple Random Sampling. In: Scheaffer RL, Mendelhall W,
Ott L, editors. Elementary Survey Sampling, 5th edition. Wadsworth Publishing: Belmont,
California, 1996. pp.79-124.
Schappert, SM. Office visits for otitis media, United States 1975-1990. Vital and Health Statistics
of The Centers for Disease Control / National Center for Health Statistics. 1992;214:1.
Schwartz RH, Rodriguez WJ, Hayden GF, Grundfast KM. The reevaluation visit for acute otitis
media. J Fam Pract 1987;24:145-148.
Teele DW, Kleine JO, Rosner B. et al. Middle ear disease and the practice of pediatrics. Burden
during the first five years of life. JAMA 1983; 249:1026-1029.
Teele DW, Kleine JO, Rosner B. Epidemiology of otitis media during the first seven years of life
in children in greater Boston: a prospective cohort study. J Infect Dis 1989;160:83-94.
Timmermans FJ, Gerson S. Chronic granulomatous otitis media in bottle-fed Inuit children. Can
Med Assoc J 1980;122:545-547.
White LL, Holimon TD, Tepedino JT, Portner TS, Wan JY, Thompson JW. Antimicrobials
prescribed for otitis media in a pediatric Medicaid population. Am J Health Syst Pharm
1996;53:2963-2969.
Click here to view the slides from this presentation
Return to the Grand Rounds Archive Index

Return to BCM Otolaryngology Home Page
&REG; Copyright, 1995-9. All Rights Reserved.

Baylor College of Medicine. The Bobby R. Alford Department of Otorhinolaryngology and Communicative Sciences.
Acute Otitis Media
Slide 1 of 62
http://www.bcm.edu/oto/grand/03-18-99/sld001.htm [5.11.2005 10:39:23]
http://www.bcm.edu/oto/grand/4893.html

The information contained within the Grand Rounds Archive is intended for use by doctors and other
health care professionals. These documents were prepared by resident physicians for presentation and
discussion at a conference held at The Baylor College of Medicine in Houston, Texas. No guarantees are
made with respect to accuracy or timeliness of this material. This material should not be used as a basis
for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed
medical literature.
AUTOIMMUNE INNER EAR DISEASE

Douglas D. Backous,, MD
April 8, 1993
The role of immunity in sensorineural hearing loss was first suggested in 1958 by Lenhart. Kikuchi, in
1959, wrote of "sympathetic otitis" whereby surgery on one ear affected hearing in the other. He
proposed an autoimmune phenomena as the etiology. In 1961 Beickert, and two years later, Terrayama
presented data supporting autoimmunity in experimental guinea pig cochleas. McCabe described 18
patients with bilateral asymmetric hearing loss progressing over weeks to months which responded to
steroid therapy. His 1979 paper asserted the importance of a high index of suspicion in these patients
since, if diagnosed early, they could be treated and their hearing preserved.
Humoral and cell mediated immunity, the lymphocyte-macrophage system, and the complement cascade
work in homeostatic harmony to provide immune protection to the host. B cells are produced in the bone
marrow and, through antigen stimulation and differentiation into plasma cells, produce specific
antibodies. T cells are derived in the thymus and provide regulatory function for B cells, cytotoxic
activity, and generate lymphokines. B and T cells also form immunologic memory. Cells of the
lymphocyte-macrophage system phagocytose foreign cellular components, process antigen, and produce
interferons. The complement cascade amplifies antigen antibody reactions. Chemotactic, anaphylotoxic,
opsoninization, and immune adherence functions arise from the complement system. Kinin-like
substances are also complement generated.
The inner ear is immunologically active. The endolymphatic sac acts as the afferent limb of inner ear
immunity since it can concentrate and primarily synthesize antibody. IgG is the most common antibody
produced with IgM, IgA, and secretory component being present in lower concentrations. The distal
endolymphatic sac is the site of immunologic activity due to extensive perisaccular lymphatics. Antibody
production is independent of serum or cerebrospinal fluid levels. Secondary exposure to antigen in the
inner ear induces a more intense response than primary exposure to antigen.
Autoimmunity occurs with loss of homeostatic control in the immune system. Host tissues become
recognized as foreign and induce damaging vasculitis and fibrosis. Veldman described a continuum of
autoimmunity. On one end, organ specific responses with organ specific autoantibodies and T cells
produce tissue alteration (i.e. Hashimoto's thyroiditis). On the opposite end of the spectrum is non-organ
specific diseases with circulating non-specific autoantibodies (i.e. systemic lupus erythematosus). In
between is organ specific disease with non-specific autoantibodies (ie primary biliary cirrhosis).
Patients with idiopathic autoimmune sensorineural hearing loss present most commonly with bilateral
progressive hearing loss. Fifty percent have vestibular signs, and symptomatically progress over weeks to
months. Females between the ages of 17 to 42 years represent 65% of the cases reported by Hughes.
Twenty percent of Hughes' study later manifested signs of systemic autoimmune disease.
McCabe proposed using ESR, ANA, RF, complement levels, and quantitative immunoglobulin levels as
a screening panel for autoimmune inner ear disease in high risk patients. Positive values in any of the
screening tests would warrant leukocyte inhibition testing. Hughes classified patients as high risk if they
had bilateral and progressive sensorineural hearing loss, no response to conventional therapy,
concomitant immune disorders, abnormal screening tests or improvement of hearing with steroid therapy.
Treatment goals in autoimmune inner ear disease include improving speech thresholds to levels treatable
with hearing aids in severely affected patients and recovery of hearing to near normal levels in those with
mild to moderate losses. Steroids, cytoxan, and plasmapheresis compose the available therapeutic
modalities. Hughes advocates high dose (prednisone 20 mg four times daily for 10 days then 10 mg
every other day for 3-6 months) steroids as initial treatment. Patients are tapered slowly and restarted if
symptoms recur. As initial therapy, McCabe recommends cytoxan (2mg/kg twice daily) combined with
steroids (prednisone 30 mg every other day) for 3 weeks. If speech discrimination scores increase by
20% or pure tone average improves by 15 dB, therapy is continued for 3 months. Cyclophosphamide is
tapered first followed by steroids. If symptoms recur both drugs are restarted. Three month cycles are
continued until patients can be weaned. No patient required more than 24 months of treatment in
McCabe's study. Hughes advises plasmapheresis for those patients unresponsive to steroids and cytoxan
after 6 to 8 weeks at the above stated doses. Plasmapheresis theoretically removes unwanted humoral and
cellular elements. Treatments are given three times weekly for 2 weeks followed by once weekly for 4
additional weeks.
In summary, otolaryngologists need a high index of suspicion for autoimmune etiologies in patients with
sensorineural hearing loss. Ophthalmologic, neurologic and rheumatologic consultations are useful in
ruling out systemic vasculitic diseases. Steroids and cyclophosphamide remain the cornerstones of
treatment in autoimmune inner ear disease, with reservation of plasmapheresis for refractory cases. If
caught early, and with aggressive medical management, hearing stabilization and possible improvement
are feasible.
Case Presentation
A 53-year-old white woman was first seen in November 1991 by a private MD, for sore throat, otalgia,
scleritis, and temporal headaches. ANA, RF, RPR, and VDRL were all negative at that time. Her ESR
was mildly elevated to 56. Magnetic resonance imaging of the head and neck was read as normal. She
was treated unsuccessfully for occipital nerve impingement with local steroid injection. Systemic steroids
relieved all symptoms until attempted taper when the headaches returned. In December 1991 she was
admitted to the Neurology service at The Methodist Hospital with a diagnosis of temporal artery
headaches. Further past medical history revealed an episode of temporal headache and pleuritic chest
pain 6 months prior to her workup in Beaumont. Neurological and ophthalmologic evaluations revealed
no specific anomalies. Lumbar puncture and temporal artery biopsy were without pathological change.
VDRL was nonreactive. RF, ANA, and HIV testing were all negative. She had improvement of her
headaches with systemic steroids. She was readmitted to Hospital in January 1992 with new onset
nausea, vomiting, and sudden hearing loss in her left ear. ESR on admission was 37, urinalysis clear, and
blood hematologic assessment showed a very mild iron deficiency anemia. C3, C4, RPR, FTA:ABS,
SSA, SSB, RF, ANA, and antineutrophil cytoplasmic antibody were all within normal values. ACE
inhibitor level was 8.4 (1.8 to 6.2). Serum protein electrophoresis was consistent with an acute phase
response to inflammation. Chest x-ray was normal. Repeat MRI was remarkable only for left middle ear
inflammation. Gallium scan showed increased uptake in the auricular region. Electronystagmography
was consistent with a left peripheral (nerve or end organ) deficit. Lip biopsy was negative for
inflammatory changes. Mastoid and middle ear biopsies obtained after complete mastoidectomy showed
no pathologic abnormality. She has been managed with long-term steroid therapy with good control of
her vestibular symptoms.
Bibliography
Arnold W, Pfaltz R, Altermatt H-J. Evidence of serum antibodies against inner ear tissues in the blood of patients with
certain sensorineural hearing disorders. Acta Otolaryngol 1985;99:437-444.
Bauwens LJJM, Veldman JE, Huizing EG. Progress in temporal bone histopathology. III. An improved technique for
immunohistochemical investigation of the adult human inner ear. Acta Otolaryngol Suppl 1990;470:34-39.
Berlinger NT. Immunobiology. In: Paparella MM, Shumrick DA, Gluckman JL, Meyerhoff WL, editors. Otolaryngology.
Volume I. Basic sciences and related principles. 3rd edition. Philadelphia: WB Saunders, 1991:725-737.
Harris JP. Autoimmunity of the inner ear. Am J Otol 1989;10:193-195.
Harris JP. Immunology of the inner ear: evidence of local antibody production. Ann Otol Rhinol Laryngol 1984;93:157162.
Harris JP. Immunology of the inner ear: response of the inner ear to antigen challenge. Otolaryngol Head Neck Surg
1983;91:18-23.
Hughes GB, Barna BP, Kinney SE, Calabrese LH, Nalepa NJ. Clinical diagnosis of immune inner-ear disease.
Laryngoscope 1988;98:251-253.
Hughes GB, Barna BP, Kinney SE, Calabrese LH, Nalepa NL. Predictive value of laboratory tests in "autoimmune" inner
ear disease: preliminary report. Laryngoscope 1986;96:502-505.
Hughes GB, Kinney SE, Barna BP, Calabrese LH. Practical versus theoretical management of autoimmune inner ear
disease. Laryngoscope 1984;94:758-767.
Kanzaki J, O-Uchi T. Circulating immune complexes in steroid-responsive sensorineural hearing loss and the long-term
observation. Acta Otolaryngol Suppl 1983;393:77-84.
McCabe BF. Autoimmune inner ear disease: therapy. Am J Otol 1989;10:196-197.
]McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol 1979;88:585-589.
McCabe BF, McCormick KJ. Tests for autoimmune disease in otology. Am J Otol 1984;5:447-449.
Mogi G, Lim DJ, Watanabe N. Immunologic study on the inner ear. Arch Otolaryngol 1982;108:270-275.
Mogi G, Maeda S, Watanabe N. The development of mucosal immunity in guinea pig middle ears. Internatl J Pediatr
Musiek FE, Morgan GJ. The use of central auditory tests in a case of vasculitis. Ear Hear 1981;2:100-102.
Plester D, Soliman AM. Autoimmune hearing loss. Am J Otol 1989;10:188-192.
Quick CA. Antigenic causes of hearing loss. Otolaryngol Clin North Am 1975;8:385-394.
Rask-Andersen H, Stahle J. Immunodefence of the inner ear? Lymphocyte-macrophage interaction in the endolymphatic
sac. Acta Otolaryngol 1980;89:283-294.
Ryan AF, Cleveland PH, Hartman MT, Catanzaro A. Humoral and cell-mediated immunity in peripheral blood following
introduction of antigen into the middle ear. Ann Otol 1982;91:70-75.
Stephens SDG, Luxon L, Hinchcliffe R. immunological disorders and auditory lesions. Audiology 1982;21:128-148.
Veldman JE, Cochlear and retrocochlear immune-mediated inner ear disorders. Pathogenetic mechanisms and diagnostic
tools. Ann Otol Rhinol Laryngol 1986;95:535-540.
Veldman JE. The immune system in hearing disorders. Acta Otolaryngol Supple 1988;458:67-75.
Veldman JE. Immunology of hearing: experiments of nature. Am J Otol 1989;10:183-187.
Veldman JE, Roord JJ, O'Connor AF, Shea JJ. Autoimmunity and inner ear disorders: an immune-complex mediated
sensorineural hearing loss. Laryngoscope 1984;94:501-507.
Yoo TJ, Stuart JM, Kang AH, Townes AS, Tomoda K, Dixit S. Type Ii collagen autoimmunity in otosclerosis and
Meniere's disease. Science 1982;217:1153-1155.
Yoo TJ, Tomoda K, Stuart JM, Cremer MA, Townes AS, Kang AH. Type II collagen-induced autoimmune sensorineural
hearing loss and vestibular dysfunction in rats. Ann Otol Rhinol Laryngol 1983;92:267-271.
Yoo TJ, Tomoda K, Stuart JM, Kang AH, Townes AS. Type II collagen-induced autoimmune otospongiosis. A
preliminary report. Ann Otol Rhinol Laryngol 1983;92:103-108.
Return Grand Rounds Archive Index

Return to BCOM Otolaryngology Home Page

resident physicians for presentation and discussion at a conference held at The Baylor
College of Medicine in Houston, Texas. No guarantees are made with respect to
accuracy or timeliness of this material. This material should not be used as a basis for
treatment decisions, and is not a substitute for professional consultation and/or peerreviewed medical literature.
BAROTRAUMA OF THE MIDDLE AND INNER EAR

March 23, 1995
Willard C. Harrill, M.D.
The most common causes of barotrauma today are from the use of the Self-Contained
Underwater Breathing Apparatus (i.e. SCUBA gear), commercial air travel, and from
hyperbaric oxygen chambers. In fact, hyperbaric oxygen therapy has been found to
produce over a 50% incidence of barotrauma. Well over 50% of the medical problems
that are related to barotrauma are referred to an Otolaryngologist. Over 90% of these
complaints involve the ear.
The noncompressible middle ear cavity makes the ear susceptible to damage from these
ambient pressure changes. Middle ear pressure is governed by a law of physics known
as Boyle's Law, which states that at a constant temperature, the volume of a body of gas
is inversely related to the pressure to which it is subjected. Applying this law to diving,
demonstrates that if a diver descends 33 feet (or the equivalent of 1 atmosphere of
pressure), the ambient pressure will double from 1 atmosphere to 2 atmospheres. This
will cause the volume of gas to be cut in half in the middle ear, resulting in a 50%
increase in negative middle ear pressure if the eustachian tube is closed. The first 33 feet
of descent represents the largest change in the volume of the middle ear while diving or
during hyperbaric decompression. A diver must dive to greater than 150 feet in depth to
equal the total volume change produced during the first 33 feet. This explains why the
majority of otologic diving and hyperbaric injuries occur during shallow dives and not
during deep water dives as one might expect. In fact, MEBT and IEBT have been
reported to occur in as little as 8 ft of water.
The pressure experienced during air travel is closely regulated by artificial means
through the use of pressurized cabins. Commercial air lines maintain a constant pressure
differential in the cabin of 8.5 psi above the changing ambient pressure outside the
plane. At an altitude of 18,000 feet above sea level, a person in an unpressurized cabin
would equilibrate the middle ear to an ambient pressure of 1/2 an atmosphere.
Regulating cabin pressure at 8.5 psi above the changing ambient pressures, a passenger
will experience a cabin pressure equal to that at sea level (i.e., 1 Atmosphere) while
flying at an altitude of 16,000 feet and will require minimal middle ear equilibration. At
40,000 feet, the passenger will have a cabin pressure equal to 7000 feet above sea level.
This pressurization is beneficial when a plane descends from a cruising altitude of
40,000 feet to land at sea level. The ambient pressure change experienced by the
passenger is decreased by 2/3 since the passenger will be actually experiencing a
descent equivalent to a 7000 foot descent. Although pressurized air travel has reduced
the potential risks of barotrauma, it is important to keep in mind that only a pressure
differential of 80mm Hg is required to close the eustachian tube in normal individuals.
A descent from 37,000 ft to 27,000 feet cruising altitude will result in a cabin gauge
pressure change equal to 80mm hg, resulting in difficulty with clearing the middle ear.
In order to fully understand the effects that Boyle's Law has on Eustachian tube
function, we will briefly review the normal anatomy and function of the eustachian tube.
In the resting state, the eustachian tube is closed. A positive pressure within the
nasopharynx, or a contraction of the tensor veli palatini, levator palatini, or the
salpingopharyngeus is required to open the eustachian tube when middle ear pressure
equalization is attempted. There are three common maneuvers which can be used to aid
in middle ear pressure equalization. These are the Valsalva maneuver, the Frenzel
maneuver, and the Toynbee maneuver. The Valsalva maneuver opens the eustachian
tube by increasing the nasopharyngeal pressure above the middle ear pressure as a result
of closing the naries and glottis while increasing intrathoracic and intra-abdominal
pressure. This maneuver is the most common cause of barotrauma to the ear if
performed too forcefully. The Frenzel maneuver is performed with a low pressure
valsalva while contracting the muscles of the pharynx. This forces air into the
eustachian tube without increasing the total intrathoracic pressure. Finally the Toynbee
maneuver involves swallowing while pinching the nose, thus creating a negative
nasopharyngeal pressure which can force the eustachian tube open. By far, the Frenzel
and Toynbee maneuvers are the safest to perform during eustachian tube dysfunction as
neither have been associated with barotrauma. .
To date, there is no readily available test to identify patients with eustachian tube
dysfunction other than a history of difficulty with clearing the middle ear. Factors which
would lead to a decrease in eustachian tube function include recent upper respiratory
infections, uncontrolled nasal allergy, nasal polyposis, and deviated nasal septum.
The differential diagnosis of the disorders related to barotrauma are damage to the
middle and inner ear, inner ear decompression sickness, and alternobaric vertigo.
Middle ear barotrauma, also known as aerotitis media, is due to an inability to
equilibrate to ambient pressure changes. The etiology can be located either in the middle
ear or external ear. This process also occurs most commonly during the descent as a
forced valsalva is attempted, increasing the middle ear pressure and allowing for
damage of the tympanic membrane to occur. This process can also occur during ascent
if eustachian tube dysfunction causes air trapping, otherwise known as "reverse
Squeeze."
The external ear canal can also be a source of middle ear barotrauma if a closed space is
created between the outer rim of the concha bowl and the tympanic membrane. The
closed space may be due to either a cerumen impaction, ear plugs, or external otitis. As
a diver descends, ambient pressure will increase, causing a net negative pressure
gradient between the external ear canal obstruction and the tympanic membrane. The
obstructing plug is then forced deeper into the external ear, resulting in a tympanic
hemorrhage or perforation. The patient typically experiences extreme pain as the
descent phase of the dive begins, despite an ability to clear the middle ear.
Edmonds et al from the Australian Diving Medical Center, devised a grading system for
middle ear barotrauma. The grading scale is from zero to five. Grade 0 is when a patient
experiences symptoms of middle ear barotrauma and no physical findings are present.
Grade I is when the presence of tympanic membrane injection can be seen. Grade II has
injection as well as hemorrhage within the tympanic membrane. Grade III includes
gross hemorrhage and Grade IV includes gross hemotympanum. Grade V includes the
presence of a tympanic perforation.
There are three common forms of IEBT: Cochlear Damage resulting in intracochlear
and intralabyrinthine hemorrhage, perilymphatic fistula formation, and IEDS secondary
to the formation of gas bubbles beneath the round window.
IEBT usually occurs with MEBT, although the absence of MEBT cannot exclude the
presence of IEBT. The symptoms of sensory neural hearing loss, vertigo, and or tinnitus
should indicate the presence of possible IEBT. The etiology of IEBT has been proposed
by Goodhill et al to be secondary to implosive or explosive forces within the cochlea.
Goodhill proposed that forces exerted on the cochlea during a forced Valsalva have
different effects depending on the patency of the eustachian tube. When the eustachian
tube is forced open suddenly, an acute rise in middle ear pressure will result causing an
inward bulge of the round window and an outward bulge of the stapes foot plate. If the
force is strong enough, implosion of the round window and a secondary outward pull on
the stapes footplate may occur.
If the eustachian tube is blocked, a valsalva maneuver will cause an elevation of CSF
pressure which will be transmitted through a patent cochlear aqueduct or internal
auditory canal causing a rise in the intracochlear pressure. If the difference between the
perilymphatic space is sufficiently greater than the middle ear pressure, an explosive
rupture of the round or oval window ligament will occur.
Both the implosive and explosive forces generated by a force valsalva are theorized to
cause a perilymphatic fistulae or dislocation and rupture of Reissner's membrane, as
well as the basilar membrane, the saccule, the utricle, or the semicircular canals.
Antonelli and Paparella have studied the temporal bone pathology in scuba diving
deaths and confirmed the presence of these pathologic findings. Simmons et al have
demonstrated through experimental models that pressure differentials of less than 2 cm
of water can cause labyrinthine ruptures.
IEBT secondary to cochlear damage will present with nonfluctuating high frequency
sensorineural or mixed hearing loss with or without tinnitus or vertigo. There is
typically no progression of symptoms. The treatment of IEBT secondary to damage to
the membranous labyrinth and cochlea includes bed rest with head elevation, the use of
vasodilators, steroids, histamine, and carbogen, in an effort to decrease inflammatory
changes and increase the delivery of oxygen. Parell et al have shown that if proper
precautions are taken to maintain proper eustachian tube function, no further
deterioration takes place in hearing if a patient returns to diving after experiencing
cochlear IEBT.
IEBT secondary to a perilymphatic fistulae typically presents with fluctuating
sensorineural or mixed hearing loss, as well as vertigo exacerbated with positional
changes, and a sense of constant disequilibrium. On physical exam, a positive
Hennebert's sign (the presence of nystagmus when positive and negative pressure is
applied to the EAC in the presence of an intact tympanic membrane) has been cited by
Thompson and Kohut as a strong positive indicator of a perilymphatic fistula in patients
with or without the presence of hearing loss. Healy et al have indicated that a positive
Romberg sign and the presence of positional nystagmus are consistent with the presence
of a perilymphatic fistula.
When present, the most common location of the perilymphatic fistula has been
demonstrated by Goodhill et al to be at the anterior rim of the oval window in the area
of the fistula ante fenestram, which is one of the weakest areas of the otic capsule. The
management of patients suspected of having a perilymphatic fistula includes bed rest
with head elevation and avoidance of increased intracranial pressure for a variable
period of days, with intermittent regular audiograms.
The length of time a patient should be observed before an exploratory tympanotomy is
performed is very controversial. In a study by Paparella et al, it was shown that in
chinchillas suffering a traumatic round window perforation, all perforations had
partially healed after 3 days, and all were completely healed after 9 days. The results of
this study form the basis for conservative management of perilymphatic fistulas.
Simmons et al recommend that if hearing loss or vestibular symptoms are progressive,
or if after 10 days any vestibular symptoms remain, an exploratory tympanotomy should
be performed. Parell and Becker also advocate this 10 day observation period. Singleton
and Kohut are proponents of a 5 day observation period. Goodhill et al suggest a 48
hour observation period. Pullen et al suggest that the shape of the audiogram should
indicate if an exploratory tympanotomy should be performed. He advocates immediate
surgery if an audiogram demonstrates a flat shaped total or near total SNHL in the
presence of a history of diving or air travel within the past 72 hours. However, he states
that if the hearing loss is limited to only the high frequencies, a down-sloping
audiogram, a closure of a perilymphatic fistula has not been shown to improve hearing
and, thus, surgery is contraindicated unless vertigo is present.
Inner ear decompression sickness (IEDS) is a form of nontraumatic cochlear damage
and is the result of gas bubble formations within the inner ear. It is commonly seen after
dives to extreme depths using a Helium oxygen mixture as a substitute for nitrogen
oxygen mixture, in order to minimize the narcotic effects of nitrogen. IEDS occurs as a
diver returns to the surface and, in an attempt to accelerate helium elimination from the
tissues, changes from an oxygen-helium mixture to an air mixture. McCormick et al
demonstrated that if rapid decompression occurs, inert gas bubbles of helium will form
within the microvessels and otic fluids, causing a blockage of the microcirculation and
resulting ischemia of the stria vascularis, spiral ligament and semicircular canals. A
hypercoagulable state is produced secondary to the activation of factor XII, resulting in
further vascular occlusion. As previously stated, the treatment of this disorder is
immediate recompression to approximately three atmospheres deeper than the depth at
which the symptoms began to occur. Farmer et al have demonstrated a near total return
to baseline hearing if recompression is initiated immediately. Proper diagnosis is
crucial, as the hyperbaric chamber recompression will aggravate inner ear damage due
to perilymphatic fistulas and cochlear barotrauma.
The differentiation between IEDS and IEBT can be made based on the following
criteria.
Dive profile:
1. IEDS is rare in shallow water (only 3 cases reported in less than 100 feet
of water),
2. diving to a depth that is near the limits of the nodecompression zone and
not undergoing decompression,
3. using a HE-02 gas mixture (IEDS),
4. h/o rapid ascent. (IEBT associated with rapid descent);
Time of symptom onset:
1. During descent (IEBT),
2. During ascent (IEDS),
3. Shortly after decompression (IEDS);
Associated symptoms:
1. the presence of decompression sickness (only 6 cases in literature of IEDS
without systemic decompression sickness),
2. h/o difficulties in clearing the ear (IEBT),
3. h/o nasal or sinus problems (IEBT);
Physical findings:
1. MEB (IEBT),
2. Central neurologic signs (IEDS).
The last disorder related to barotrauma is the phenomenon known as alternobaric

vertigo. This is a syndrome first described by Lundgren in 1965 as vertigo occurring
during ascent due to unequal pressure in the right and left middle ear. The duration of
the vertigo is usually from a few seconds to minutes and is not associated with hearing
loss. In his review of 2053 Swedish divers, Lundgren found a 16.7% incidence of
alternobaric vertigo. A review of 526 Australian naval divers by Bayliss cited a 0.4%
incidence. Although this process is self-limiting, a diver experiencing alternobaric
vertigo while attempting a valsalva maneuver at the surface, should not undergo a dive
or air travel.
As people become more active in both air travel and recreational water sports, education
regarding the hazards of extreme middle ear pressure changes should be expanded.
Patients who are more susceptible to aural barotrauma, either due to lifestyle, upper
respiratory infections, or after ear surgery, should take extra precautions to guard against
eustachian tube function during air travel or underwater sports or simply avoid these
activities when the risks of barotrauma are the greatest.
Case Presentation
A 36-year-old man experienced difficulty clearing his left ear while snorkeling to a
depth of 35 feet in Cozumel, Mexico. After attempting a forced valsalva maneuver, the
patient reported sudden hearing loss and tinnitus in his left ear that continued to persist.
He denied any sensation of dysequilibrium or vertigo. A history of a previous high
frequency SNHL was noted before the diving incident. No other past history of middle
or inner ear disorders was noted. No history of a recent URI, nasal obstruction, or recent
eustachian tube dysfunction was reported. Physical exam demonstrated a Grade III
tympanic membrane hemorrhage with an intact drum. The right ear canal, tympanic
membrane, and middle ear appeared normal. The Weber exam lateralized to the right
ear and ear conduction was greater than bone conduction in the left ear. No evidence of
spontaneous nystagmus was noted. Hennebert's sign and Rhomberg sign were negative.
Fistula test was negative. An audiogram was obtained demonstrating an 80 dB SNHL on
the left and no change in the high frequency loss on the right. An MRI scan
demonstrated no abnormalities. The patient was placed on steroids and antibiotics.
Carbogen therapy was initiated and two stellate ganglion blocks were performed. A
repeat audiogram was obtained one week later that demonstrated improvement in the
left SNHL. The patient continued to report no dizziness. The patient was advised to
refrain from exertion for 1 month and schedule a follow-up audiogram in six months.
Bibliography
Anson B, Caldwell DW, Bast TH. The fissula ante fenestram of the human otic capsule. Ann Otol
Rhinol Laryngol 1948;57:103-128.
Antonelli PJ, Parell GJ, Becker GD, Paparella MM. Temporal bone pathology in scuba diving deaths.
Otolaryngol Head Neck Surg 1993;109:514-521.
Bayliss GJA. Aural barotrauma in naval divers. Arch Otolaryngol 1968;88:141-147.
Becker GD, Parell GJ. Otolaryngologic aspects of scuba diving. Otolaryngol Head Neck Surg
1979;87:569-571.
Caparosa RJ, Shamblin JD, Junter CW. Stapedectomy-fistula repair. Laryngoscope 1977;87:13731377.
Caruso VG, Winkelmann PE, Correia MJ, et al. Otologic and otoneurologic injures in divers: clinical
studies on nine commercial and two sport divers. Laryngoscope 1977;87:508-512.
Eichel BS, Landes BS. Sensorineural hearing loss caused by skin diving. Arch Otolaryngol
1970;92:315-319.
Farmer JC, Thomas WG, Youngblood DB, et al. Inner ear decompression sickness. Laryngoscope
1976;86:1315-1327.
Farmer JC. Diving injuries to the inner ear. Ann Otol Rhinol Laryngol 1977;86:1-20.
Freeman P, Edmonds C. Inner ear barotrauma. Arch Otolaryngol 1972;95:556-563.
Goodhill V. Sudden deafness and round window rupture. Laryngoscope 1971;81:1462-1474.
Goodhill V. Leaking labyrinth lesions, deafness, tinnitus and dizziness. Ann Otol Rhinol Laryngol
1981;90:99-106.
Goodhill V. The conductive loss phenomenon in post stapedectomy perilymphatic fistulas.
Laryngoscope 1967;77:1179-1190.
Green SM, Rothrock SG, Green EA. Tympanometric evaluation of middle ear barotrauma during
recreational scuba diving. Int J Sports Med 1993;14:411-415.
Head PW. Decompression injuries in the temporal bone. J Laryngol Otol 1980;94:111-116.
Healy GB, Friedman J, Strong MS. Vestibular and auditory findings of perilymphatic fistulas: a review
of 40 cases. Trans Am Acad Ophthalmol Otolaryngol 1976;82:44-49.
House HP. The fistula problem in otosclerosis surgery. Laryngoscope 1967;77:1410-1426.
Igarashi Y, Watanabe Y, Mizukoshi K. Middle ear barotrauma associated with hyperbaric oxygen
treatment. Acta Otolaryngol Suppl 1993;504:143-145.
Love JT Jr, Caruso VG. Civilian air travel and the otolaryngologist. Laryngoscope 1978;88:1732-1742.
McCormick JG, Philbrick T, Holland W, Harrill JA. Diving induced sensorineural deafness:
prophylactic use of heparin and preliminary histopathology results. Laryngoscope 1973;63:1483-1501.
McNicoll WD. Eustachian tube dysfunction in submariners and divers. Arch Otolaryngol
1982;108:279-283.
Molvaer OI, Natrud E. Ear damage due to diving. Acta Otolaryngol Suppl 1979;360:187-189.
Money KE, Buckingham IP, Calder IM, Johnson WH, King KD, Landolf JP, et al. Damage to the
middle ear and the inner ear in underwater divers. Undersea Biomed Res 1985;12:77-84.
Neblett LM. Otolaryngology and sport scuba diving: update and guidelines. Ann Otol Rhinol Laryngol
1985;94:1-12.
Novotny GM. Cochlear bends. J Otolaryngol 1980;9:395-398.
Parell GJ, Becher GD. Conservative management of inner ear barotrauma resulting from scuba diving.
Parell GJ, Becker GD. Inner ear barotrauma in scuba divers. A long-term follow-up after continued
diving. Arch Otolaryngol Head Neck Surg 1993;119:455-457.
Pullen FW 2d. Perilymphatic fistula induced by barotrauma. Am J Otol 1992;13:270-272.
Reuter SH. Underwater medicine: otolaryngologic considerations of the skin and scuba diver. In:
Paparella MM, Shumrick DA, editors. Otolaryngology. Volume IV: Plastic and Reconstructive
Surgery and Interrelated Disciplines, 3rd ed. Philadelphia: Saunders, 1991:3231-3257.
Schuchman G, Joachims HZ. Tympanometric assessment of eustachian tube function of divers. Ear
Hear 1985;6:325-328.
Seltzer S, McCabe BF. Perilymph fistula: the Iowa experience. Laryngoscope 1986;94:37-46.
Shupak A, Sharoni Z, Ostfeld E, Doweck I. Pressure chamber tympanometry in diving candidates.
Simmons FB. The double membrane break syndrome in sudden hearing loss. Laryngoscope
1979;89:59-66.
Simmons FB. Theory of membrane breaks in the sudden hearing loss. Arch Otolaryngol 1968;88:4148.
Singleton GT, Karlan MS, Posh KN, Bock DG. Perilymph fistulas: diagnostic criteria and therapy.
Ann Otol Rhinol Laryngol 1978;87:797-803.
Talmi YP, Finkelstein Y, Zohar Y. Barotrauma-induced hearing loss. Scand Audiol 1991;20:1-9.
Talmi YP, Finkelstein Y, Zohar Y. Decompression sickness induced hearing loss. A review. audiol
1991;20:25-28.
Thompson JN, Kohut RI. Perilymph fistulae: variability of symptoms and results of surgery.


Copyright, 1996. All Rights Reserved.
Baylor College of Medicine. Bobby R. Alford Department of Otorhinolaryngology and Communicative Sciences.

BELL'S PALSY
July 20, 1995
S. Mark Overholt, M.D.
Bell's palsy is synonymous with idiopathic facial paralysis. It is the most common
cause of an acute facial paralysis, and accounts for 75 to 80 percent of all cases.
The annual incidence is 20 to 30 per 100,000. It's name is derived from Sir Charles
Bell, a surgeon and accomplished artist from Edinburgh, Scotland who lived from
1774 to 1842. In 1821, he published his anatomic diagrams of the course of the
facial nerve and its innervation of the facial musculature.
Early treatment strategies for Bell's palsy were primarily surgical. In the 1930's
Balance and Duel described decompression of the distal one centimeter of the
facial nerve at the stylomastoid foramen. Tumarkin felt that decompression of the
stylomastoid artery was necessary to adequately treat Bell's palsy. By the 1940's the
main treatment modality was facial nerve decompression in the mastoid segments.
In the 1960's electrical testing allowed refinement of the indications for surgery.
Jongkees proposed decompression of the nerve when a difference of greater than
3.5 mAmps was noted by nerve excitability testing when comparing the unaffected
to the affected side. Alford further delineated indications and discussed the
importance of associated symptoms, the cause of the paralysis, and the experience
of the surgeon in deciding to proceed with decompression. Important surgical
advances were also developed in the 1960's. These include the middle cranial fossa
approach which was initially described by House in 1961, and the total facial nerve
decompression described by Pulec in 1966. The 1970's was a transition period for
the theoretical treatment approach to a patient with Bell's palsy. With the
refinement of electrical testing and the use of steroids, a trend had begun away
from surgery. Adour was one of the first neurotologists to state that surgery might
not be beneficial. In 1985 May discussed the failure of transmastoid decompression
to improve outcome. However, Fisch and Esslen found good results after combined
middle cranial fossa and mastoid decompression of the facial nerve in 11 out of 12
patients with Bell's Palsy. The role of surgery remains controversial and indication
will be discussed later. By the 1980's and 1990's the treatment had changed to
being primarily medical. Coker additionally demonstrated that the electrical
evidence of a degenerating facial nerve on electroneuronography was reliable and
correlated with histologic evidence of neuronal degeneration.
Several theories exist regarding the cause of Bell's palsy. Most favor a viral inciting
event that triggers edema and inflammation in the nerve leading to infarction and
nerve damage. Many viruses have been implicated but there is mounting evidence
supporting the herpes simplex virus serotype 1 as the etiologic culprit.
Mechanical entrapment in the fallopian canal is the cause of the ischemic injury to
the facial nerve. In 1981 Fisch described the "physiologic bottleneck" at the meatal
foramen. In his comparative study of adult temporal bone specimens, the average
diameter of the fallopian canal was found to be 1.02 to 1.53 mm, except at the
meatal foramen where it was 0.68 mm. Histologic examinations have demonstrated
hemorrhage and edema in the nerve at this site. Additionally, intraoperative
stimulation blockage has been documented to occur in this narrow segment with
intact stimulation distally.
Nerve injury is classified according to the schema of Sunderland. This system
describes progressively worsening injury to the nerve starting with a neuropraxic
injury and ending in neuronotmesis or nerve transection. Five grades of injury are
described. The grade of the injury correlates to the final functional recovery of the
facial nerve. Clinically facial nerve injury is classified using the House-Brackmann
system. Grade I is normal function. Grade II shows slight weakness but no
synkinesis. Grade III shows obvious weakness with some mass movement. Grade
IV has inability to elevate the brow, significant synkinesis, and obvious weakness.
Grade V has barely perceptible motion, and grade VI has no movement.
The differential diagnosis for facial paralysis is broad and must include
inflammatory causes such as herpes zoster oticus or sarcoidosis, traumatic injuries
such as temporal bone fractures, mastoiditis, cholesteatomas, the MelkerssonRosenthal syndrome, and primary or metastatic neoplasms of the temporal bone.
By far though, idiopathic facial paralysis predominates. It accounts for almost 80 %
of all cases of facial palsy.
The epidemiology of Bell's palsy is best described in Peitersen's monograph from
the Copenhagen facial nerve study. In over 1000 patients he found there to be no
sex predilection. A broad age range was noted, typically from 15 to 60 years old.
Rarely did he note bell's palsy in patients less than 15 years of age. Patients
presented with a classic constellation of symptoms. There is usually a prodrome of
periauricular pain that heralds the onset of facial palsy within 24 hours. The palsy
is unilateral and involves all muscle groups. In order to make the diagnosis of Bell's
palsy the evaluating clinician must exclude the following: concurrent CNS disease,
otologic infection, auricular blebs suggesting the Ramsey-Hunt syndrome, parotid
masses, and an occult neoplasm such as a facial neuroma in a patients who has a
recurrent palsy.
There is no consensus regarding the work up of a patient with Bell's palsy. Most
would include a routine basic audiogram in order to rule out an asymmetric hearing
loss, which might suggest another process. Topognostic tests, which include the
Schirmer's test of tearing, the salivary flow test popularized by May, and the
acoustic reflex test are of historic interest only. While once felt to assist in
localizing the nerve injury, we know now that the injury occurs at the meatal
foramen. Additionally, they do not offer prognostically valuable information.
Radiographic imaging is reserved for those who have no improvement in there
palsy within 6 months and for those who a have recurrent palsy. The MRI is the
best study to image the facial nerve. In patients with Bell's palsy gadolinium MRI
enhancement of the perigeniculate region is prominent. However, as Schwaber
pointed out in 1990, this enhancement does not correlate with outcome.
Furthermore, Gribarski showed that 76% of normal subjects will show
enhancement of the perigeniculate region with gadolinium.
Electrodiagnostic test are reserved for patients with complete facial paralysis.
Available tests include the nerve excitability test(NET), maximal stimulation
test(MST), electroneuronography (ENog), and electromyography. The latter is
appropriate when the paralysis has been present for several weeks. In the acute
period the first three are most appropriate. NET and the MST are both performed
using the Hilger stimulator. The first is a threshold test and the second a
supramaximal stimulation test. A criticism of these two tests is that the grading of
the response to the stimulus is subjective, and thus may not reliably predict
denervation. However, Coker and Fordice demonstrated that a greater than 3.5
mAmp difference side-to-side on NET correlated closely with objective ENog data
showing greater than 90% degeneration of the nerve. May showed that in patients
who did not have a response to the MST for 10 days, over 85% did not fully
recover. Those who did have a response to MST within the first 10 days had
excellent recovery. Enog is the most widely used test to evaluate an acute complete
facial paralysis. It is an objective reliable test that compares the difference in the
amplitude of the compound action potential of the unaffected to affected side of the
face. Fisch and Esslen found that when the amplitude of the affected side is less
than 10% of the unaffected side the prognosis is poor. Coker correlated the
evidence of electrical degeneration with histologic evidence of nerve degeneration.
Treatment of an acute facial paralysis is initially medical. The regimen currently
used by most otologists is prednisone 1mg/kg/day tapered over a 10 day period.
With the mounting evidence implicating the herpes virus, many additionally
recommend acyclovir 800 mg three times a day for 10 days.
Indications for surgery are controversial, but most neurotologists feel that electrical
evidence of greater than 90% degeneration or a difference of 3.5mAmp from sideto-side is sufficient to proceed with a decompression. Surgery must be performed
as early as possible. After 21 days there is probably no benefit. The decompressive
surgery must focus on the meatal foramen. May found that transmastoid
decompression alone did not improve outcome, whereas when combining
transmastoid and middle cranial fossa decompression Fisch found good outcome in
11 of 12 patients .
Good prognostic variables include presentation with an incomplete palsy, onset o f
return of facial function within three weeks, and age below 60 years. Peitersen
found that 94% of patients who presented with an incomplete palsy progressed to
full recovery. If return of facial function was within one week almost 90% fully
recovered Return of function in the second and third weeks yielded full recovery in
83% and 60% respectively. Overall, 85% of patients with Bell's palsy will recover
to normal function.
In conclusion, Bell's palsy is the most common cause of an acute facial palsy. It is
unilateral and sudden in onset, often with a prodrome of auricular pain. After
excluding other potential causes of a facial paralysis, treatment is with prednisone
and acyclovir. Surgery is reserved for those who meet electrodiagnostic criteria or
are worsening on medical treatment. Decompression must include the site of

pathologic compression of the facial nerve, the meatal foramen. Finally, fortunately
85% of patients with Bell's palsy have full return of facial function.
Case Presentation
A white female initially presented at the age of 29, with a 16-day history of a
complete right-sided facial paralysis. Complicating this problem was the fact that
she was also 34 weeks pregnant. No complaints of associated dizziness or changes
in her hearing were reported, but she did note some facial dysesthesias in the
distribution of the mandibular branch of the trigeminal nerve. There was no history
of recurrent facial edema or a fissured tongue. Physical exam did not show any
auricular blebs or parotid masses. An audiogram was normal with the exception of
absent right-sided acoustic reflexes. Nerve excitability testing showed a good
response at 3.0 milliamps on the unaffected side and no response at 7.0 milliamps
on the right. Because of her pregnancy and late presentation she was not offered
further treatment. Her recovery was incomplete. In May, she began to notice some
unusual movements and spasms in her face. Final recovery left her with good
symmetry at rest and excellent eye closure, but she had mild brow ptosis,
synkinesis, and gustatory tearing. She was graded according to the HouseBrackmann scale as a III out of VI. She subsequently did well with some
intermittent dysesthesias of the right face until age 36 when she suffered an attack
of Bell's Palsy on the left side of her face. She was treated with prednisone and
acyclovir and eventually fully recovered. Approximately two years later she
experienced two recurrences of her right-sided facial paralysis. Both episodes were
treated with prednisone and acyclovir with recovery to her baseline grade III
deficit. She is currently being followed by the neurotology service at The
Methodist Hospital to monitor any further recurrence.
Bibliography
Abraham-Inpijn L, Oosting J, Hart AAM. Bell's palsy: factors affecting the prognosis in 200
patients with reference to hypertension and diabetes mellitus. Clin Otolaryngol 1987;12:349-355.
Adour KK. Medical management of idiopathic (Bell's) palsy. Proc R Soc Med 1973;66:554-556.
Adour KK, Bell DN, Hilsinger RL Jr. Herpes simplex virus in idiopathic facial paralysis (Bell's
palsy). JAMA 1975;233:527-530.
Adour KK, Byl FM, Hilsinger RL Jr, Kahn ZM, Sheldon MI. The true nature of Bell's palsy:
analysis of 1000 consecutive patients. Laryngoscope 1978;88:787-801.
Adour KK, Hissinger RL Jr., Callan EJ. Facial paralysis and Bell's palsy: a protocol for
differential diagnosis. Am J Otol Suppl 1985:68-73.
Adour KK, Sheldon MI, Kahn ZM. Maximal nerve excitability testing versus neuromyography:
prognostic value in patients with facial paralysis. Laryngoscope 1980;90:1540-1547.
Adour KK, Wingerd J. Idiopathic facial paralysis (Bell's palsy): factors affecting severity and
outcome in 446 patients. Neurology 1974;24:1112-1116.
Adour KK, Wingerd J, Bell DN, Manning JJ, Hurley JP. Prednisone treatment for idiopathic
facial paralysis (Bell's palsy). N Engl J Med 1972;287:1268-1272.
Alford BR. Electrodiagnostic studies in facial paralysis. Arch Otolaryngol 1967;85:259-264.
Alford BR. Facial nerve grafting. Laryngoscope 1976;86:257-258.
Alford BR, Ickey JR, Pratt FE. Management of facial paralysis. Tex Med 1966;62:56-60.
Alford BR, Jerger JF, Coats AC, Peterson CR, Weber SC. Neurophysiology of facial nerve
testing. Arch Otolaryngol 1973;97:214-219.
Alford BR, Jerger JF, Coats AC, Peterson CR, Weber SC. Diagnostic tests of facial nerve
function. Otolaryngol Clin North Am 1974;7:331-342.
Alford BR, Sessions RB, Weber SC. Indications for surgical decompression of the facial nerve.
Laryngoscope 1971;81;620-635.
Alford BR, Weber SC, Sessions RB. Neurodiagnostic studies in facial paralysis. Ann Otol Rhinol
Laryngol 1970;79:227-233.
Austin, JR., Peskind SP, Austin SG, Rice DH. Idiopathic facial nerve paralysis: a randomized
double blind controlled study of placebo versus prednisone. Laryngoscope 1993;103:1326-1333.
Balkany T, Fradis M, Jafek BW, Rucker NC. Intrinsic vasculature of the labyrinthine segment of
the facial nerve--implications for site of lesion in Bell's palsy. Otolaryngol Head Neck Surg
1991;104:20-23.
Brackmann DE. Bell's palsy: incidence, etiology, and results of medical treatment. Otolaryngol
Clin North Am 1974;7:357-368.
Brandt TW, Jenkins HA, Coker NJ. Facial paralysis as the initial presentation of an internal
carotid artery aneurysm. Arch Otolaryngol Head Neck Surg 1986;112:198-202.

Burgess RC, Michaels L, Bale JF Jr, Smith RJ. Polymerse chain reaction amplification of herpes
simplex viral DNA from the geniculate ganglion of a patient with Bell's palsy. Ann Otol Rhinol
Laryngol 1994;103:775-779.
Cawthorne T, Haynes DR. Facial palsy. Br Med J 1956;2:1197-2000.
Coker NJ. Facial electroneurography: analysis of techniques and correlation with degenerating
motorneurons. Laryngoscope 1992;102:747-759.
Coker NJ. Facial nerve disorders. Otolaryngol Head Neck Surg 1992;106:8-9.
Coker NJ. Minutes of the annual meeting of the Facial Nerve Study Group September 1992. Am
J Otol 1994;15:114-116.
Coker NJ, Fordice JO, Moore S. Correlation of the nerve excitability test and electroneurography
in acute facial paralysis. Am J Otol 1992;13:127-33.
Coker NJ, Kendall KA, Jenkins HA, Alford BR. Traumatic intratemporal facial nerve injury:
management rationale for preservation of function. Otolaryngol Head Neck Surg 1987;97:262269.
Coker NJ, Salzer TA. The use of masseter electromyography with electro-neurography in the
evaluation of facial paralysis. Otolaryngol Head Neck Surg 1990;103:391-395.
Danielides V, Skevas A, Kastanioudakis I. Assimakopoulos D. Comparative study of evoked
electromyography and facial nerve latency test in the prognosis of idiopathic facial nerve palsy in
childhood. Childs Nerv Syst 1994;10:122-125.
Danielides VG, Skevas AT, Panagopoulos K, Kastanioudakis I. Value of the facial nerve latency
test in the prognosis of childhood Bell's palsy. Childs Nerv Syst 1992;8:126-128.
Devriese PP. Prednisone in idiopathic facial paralysis (Bell's palsy). ORL J Otorhinolaryngol
Relat Spec 1977;39:257-271.
Eicher SA, Coker NJ, Alford BR, Igarashi M, Smith RJ. A comparative study of the fallopian
canal at the meatal foramen and labyrinth segment in young children and adults. Arch
Engstrm M, Thuomas KA, Naeser P, Stlberg E, Jonsson L. Facial nerve enhancement in Bell's
palsy demonstrated by different gadolinium-enhanced magnetic resonance imaging techniques.
Arch Otolaryngol Head Neck Surg 1993;119:221-225.
Fisch U. Surgery for Bell's palsy. Arch Otolaryngol 1981;107:1-11.
Fisch U, Esslen E. Total intratemporal exposure of the facial nerve. Arch Otolaryngol
1972;95:335-341.
Fisch U, Felix H. On the pathogenesis of Bell's palsy. Acta Otolaryngol 1983;95:532-538.
Fitzgerald DC. Role of electrical stimulation therapy for Bell's palsy. Am J Otol 1993;14:413414.
Frach JP, Osterbauer PJ, Fuhr AW. Treatment of Bell's palsy by mechanical force, manually
assisted chiropractic adjusting and high-voltage electrotherapy. J Manipulative Physio Ther
1992;15:596-598.
Gantz BJ, Gmr A, Fisch U. Intraoperative evoked electromyography in Bell's palsy. Am J
Otolaryngol 1982;3:273-278.
Glasscock ME, 3d, House WF, Alford BR. Middle fossa facial nerve decompression. Ann Otol
Gontier J, Fisch U. Shirmer's test: its normal values and clinical significance. ORL J
Otorhinolaryngol Relat Spec 1976;38:1-10.
Gorodezky C, Carranza JM, Bustamante A, Yescas P, Martinez A, Alonso Vilatela ME. The
HLA system and T-cell subsets in Bell's palsy. Acta Otolaryngol 1991;1111:1070-1074.
Halvorson DJ, Coker NJ, Wang-Bennett LT. Histologic correlation of the degenerating facial
nerve with electroneurography. Laryngoscope 1993;103:178-184.
Honda H, Takahashi A. Virus-associated demyelination in the pathogenesis of Bell's palsy. Intern
Med 1992;31:1250-1256.
Hyden D, Roberg M, Forsberg P, Fridell E, Fryden A, Linde A, Odkvist L. Acute "idiopathic"
peripheral facial palsy: clinical, serological, and cerebrospinal fluid finding and effects of
corticosteriods. Am J Otolaryngol 1993;114:179-186.
Ivarsson S, Andreasson L, Ahlfors K. Acyclovir treatment in a case of facial paralysis caused by
herpes zoster. Pediatr Infect Dis 1987;6:84.
Jenkins HA, Herzog JA, Coker NJ. Bell's palsy in children. Cases of progressive facial nerve
degeneration. Ann Otol Rhinol Laryngol 1985;94:331-336.
Jongkees LBW. On the histology of Bell's palsy. Acta Otolaryngol 1954;44:336-343.
Jonsson L, Alm G, Thomander L. Elevated serum interferon levels in patients with Bell's palsy.
Jonsson L, Larsson A, Thomander L. Immune complexes and complement components in Bell's

palsy. ORL J Otorhinolaryngol Relat Spec 1987;49:294-301.
Katusic SK, Beard CM, Wiederholt WC, Bergstralh EH, Kurland LT. Incidence, clinical features,
and prognosis in Bell's palsy. Rochester, Minnesota, 1968-1982. Ann Neurol 1986;20:622-627.
Kohsyu H, Aoyagi M, Tojima H, Tada Y, Inamura H, Ikarashi T, Koike Y. Facial nerve
enhancement in Gd-MRI in patients with Bell's palsy. Acta Otolaryngol Suppl 1994;511:165169.
Lipkin AF, Coker NJ, Jenkins HA, Alford BR. Intracranial and intratemporal facial neuroma.
Magielski JE, Blatt IM. Submaxillary salivary flow: a test of chorda tympani nerve function as an
aid in diagnosis and prognosis of facial nerve paralysis. Laryngoscope 1958;68:1770-1789.
Marsh MA, Coker NJ. Surgical decompression of idiopathic facial palsy. Otolaryngol Clin North
Am 1991;24:675-689.
May M, Harvey JE, Marovitz WF, Stroud M. The prognostic accuracy of the maximal
stimulation test compared with that of the nerve excitability test in Bell's palsy. Laryngoscope
1971;81:931-938.
May M, Klein SR. Differential diagnosis of facial nerve palsy. Otolaryngol Clin North Am
1991;24:613-645.
May M, Klein SR, Taylor FH. Indications for surgery for Bell's palsy. Am J Otol 1984;5:503512.
McCormick DP. Herpes-simplex virus as a cause of Bell's palsy. Lancet 1972;1:937-939.
McGovern FH, Estevez J, Jackson R. Immunological concept for Bell's palsy. Ann Otol Rhinol
Laryngol 1977;86:300-305.
Michaels L. Histopathological changes in the temporal bone in Bell's palsy. Acta Otolaryngol
Suppl 1990;470:114-117
Murphy TP, Teller DC. Magnetic resonance imaging of the facial nerve during Bell's palsy.
Murr AH, Benecke JE Jr. Association of facial paralysis with HIV positivity. Am J Otol
1991;12:450-451.
Neely JG, Alford BR. Facial nerve neuromas. Arch Otolaryngol 1974;100:298-301.
Njoo FL, Wertheim-van Dillen P, Devriese PP. Serology in facial paralysis caused be clinically
presumed herpes zoster infection. Arch Otorhinolaryngol 1988;245:230-233.

Palva T, Hortling L, Ylikoski J, Collan Y. Viral culture and electron microscopy of ganglion cells
in Meniere's disease and Bell's palsy. Acta Otolaryngol 1978;86:269-275.
Peitersen E. The natural history of Bell's palsy. Am J Otol 1982;4:107-111.
Peitersen E. Natural history of Bell's palsy. Acta Otolaryngol Suppl 1992;492:122-124.
Rosenberg JJ, Alford BR. Experimentally controlled facial nerve injuries. Arch Otolarryngol
1966;84:337-342.
Ruboyianes JM, Adour KK, Santos DQ, Van Doersten PG. The maximal stimulation and facial
nerve conduction latency tests: predicting the outcome of Bell's Palsy. Laryngoscope 1994;104:16.
Saito O, Aoyagi M, Tojima H, Koike Y. Diagnosis and treatment for Bell's palsy associated with
diabetes mellitus. Acta Otolaryngol Suppl 1994;511:153-155.
Salzer TA, Coker NJ, Wang-Bennett LT. Stimulation variables in electroneurography of the
facial nerve. Arch Otolaryngol Head Neck Surg 1990;116:1036-1040.
Schwaber MK, Larson TC 3d, Zealer DL, Creasy J. Gadolinium-enhanced magnetic resonance
imaging in Bell's palsy. Laryngoscope 1990;100:1264-1269.
Shafshak TS, Essa AY, Bakey FA. The possible contributing factors for the success of steroid
therapy in Bell's palsy: a clinical and electrophysiological study. J Laryngol Otol 1994;108:940943.
Sillman JS, Niparko JK, Lee SS, Kileny PR. Prognostic value of evoked and standard
electromyography in acute facial paralysis. Otolaryngol Head Neck Surg 1992;107:377-381.
Smith IM, Cull RE. Bell's palsy--which factors determine final recovery? Clin Otolaryngol
1994;19:465-466.
Smith IM, Maynard C, Mountain RE, Barr-Hamilton R, Armstrong M, Murray JA. The
prognostic value of facial electroneurography in Bell's palsy. Clin Otolaryngol 1994;19:201-203.
Stankiewicz JA. A review of the published data on steroids and idiopathic facial paralysis.
Tojima H, Aoyagi M, Inamura H, Koike Y. Clinical advantages of electroneurography in patients
with Bell's palsy within two weeks after onset. Acta Otolaryngol Suppl 1994;511:147-149.
Vahlne A, Edstrom S, Arstila P, Beran M, Ejnell H, Nylen O, et al. Bell's palsy and herpes
simplex virus. Arch Otolaryngol 1981;107:79-81.

Welkoborsky HJ, Amedee RG, Elkhatieb A, Mann WJ. Auditory-evoked brain-stem responses
and auditory disorders in patients with Bell's palsy. Eur Arch Otorhinolaryngol 1991;248:417419.

Copyright, 1995-7. All Rights Reserved.

medical literature.
UNUSUAL CEREBELLOPONTINE ANGLE NEOPLASMS

Glenn W. Knox, MD
November 4, 1993
Cerebellopontine angle lesions are the predominant skull base neoplasms that affect the posterior fossa.
Although vestibular schwannomas account for the majority of primary neoplasms, a wide array of other
lesions must also be considered in the differential diagnosis. In Brackmann and Bartels' series of CPA
neoplasms, vestibular schwannoma accounted for over 90%. The remaining primary tumors were
meningiomas (3.1%), primary cholesteatomas (2.4%), facial nerve schwannomas (1.2%), other
schwannomas (0.2%), and other tumors (1.9%).
Meningiomas represent up to 18% of all intracranial tumors and approximately 3% of CPA tumors. The
cells lining the arachnoid villae are the cells of origin. These cells are distributed throughout the
intracranial space predominantly in relation to veins and dural sinuses. Meningiomas are benign but
locally aggressive tumors which occur at different anatomic sites in the following order of frequency:
parasaggital, falx, convexity, olfactory groove, tuberculum sellae, sphenoid ridge, CPA, tentorium,
lateral ventricle, clivus. The gross appearance is typically a globular mass that is firmly adherent to the
dura mater, with characteristic speckles scattered throughout the tumor that correspond to the
microscopic psammoma bodies. The tumor displaces but does not invade adjacent neural tissue.
Case Presentation
A 51-year-old white man noted the onset of vertigo in 1980. This resolved, but returned in the late 1980s,
reaching a peak in 1988. His vertigo symptoms had improved since then. He also complained of a multiyear history of mild right-sided tinnitus, which had been variable but more noticeable this year. He
complained of a two-year history of mild right-sided hearing loss which had also been more noticeable
this year. His major complaint was a sense of fullness in the right ear which he had noticed in January of
1993, which did not improve with medical treatment. Past medical history was noncontributory. Physical
examination was unremarkable. A recent audiogram showed a right-sided 40 dB high frequency
sensorineural hearing loss with a PB max of 96%. Tympanograms were type A. MRI revealed an
intracanalicular mass.
The patient was admitted to TMH and underwent a right excision of cerebellopontine angle mass via the
midfossa approach. During surgery, the tumor appeared to be associated with the superior vestibular
nerve. The tumor was noted to be tightly adherent to the surrounding structures, was rubbery in
consistency, and contained several apparent venous sinuses. The patient recovered uneventfully with
useful hearing preserved. Pathology revealed benign fibrous tissue with abundant vessels, consistent with
a benign vascular tumor.
Bibliography
Arriaga MA, Brackmann DE, Hitselberger WE. Extended middle fossa resection of petroclival and cavernous sinus
neoplasms. Laryngoscope 1993;103:693-698.
Balkany T, Fradis M, Jafek BW, Rucker NC. Hemangioma of the facial nerve: role of the geniculate capillary plexus. Skull
Base Surg 1991;1:59-63.
Batsakis JG. Tumors of the Head and Neck, 2nd ed. Baltimore: Williams & Wilkins, 1979.
Beatty CW, Ebersold MJ, Harner SG. Residual and recurrent acoustic neuromas. Laryngoscope 1987;97:1168-1171.
Bellet PS, Benton C Jr, Matt BH, Myer CM 3d. The evaluation of ear canal, middle ear, temporal bone, and
cerebellopontine angle masses in infants, children, and adolescents. Adv Pediatr 1992;39:167-205.
Bourgouin PM, Tampiere D, Robitaille Y, Robert F, Bergeron D, del Carpio R, et al. Low-grade myxoid chondrosarcoma
of the base of the skull: CT, MR, and histopathology. J Comput Assist Tomogr 1992;16:268-273.
Brackmann DE, Anderson RG. Cholesteatomas of the cerebellopontine angle. In: Silverstein H, Norrell H, editors.
Neurological Surgery of the Ear. Birmingham: Aesculapius, 1979.
Brackmann DE, Bartels LJ. Rare tumors of the cerebellopontine angle. Otolaryngol Head Neck Surg 1980;88:555-559.
Brackman DE, Gherini SE. Differential diagnosis of skull base neoplasms involving the posterior fossa. In: Cummings
CW, Harker MD, editors. Otolaryngology - Head and Neck Surgery IV. St. Louis: Mosby, 1986:3421-3436.
Brackmann DE, Kwartler JA. A review of acoustic tumors: 1983-1988. Am J Otol 1990;11:216-232.
Bushe KA, Naumann M, Warmuth-Metz M, Meizensberger J, Muller J. Maffucci's syndrome with bilateral cartilaginous
tumors of the cerebellopontine angle. Neurosurgery 1990;27:625-628.
O'Keefe LJ, Ramsden RT, Birzgalis AR. Cerebellopontine angle lipoma. J Laryngol Otol 1993;107:553-555.
Cosnard G, Galidie G, Cordoliani YS, Le Gall R. Rare tumors of the cerebellopontine angle. Ann Radiol (Paris)
1992;35:36-43.
Dort JC, Fisch U. Facial nerve schwannomas. Skull Base Surg 1991;1:51-56.
Elster AD, Challa VR, Gilbert TH, Richardson DM. Meningiomas: MR and histopathologic features. Radiology
1989;179:857-862.
Gao PY, Osborn AG, Smirniotopoulos JG, Harris CP. Radiologic-pathologic correlation: epidermoid tumor of the
cerebellopontine angle. AJNR Am J Neuroradiol 1992;13:863-872.
Garcia CA, McGarry PA, Rodriguez F. Primary intracranial squamous cell carcinoma of the right cerebellopontine angle. J
Neurosurg 1981;54:824-828.
Giddings NA, Brackmann DE, Kwartler JA. Transcanal infracochlear approach to the petrous apex. Otolaryngol Head
Neck Surg 1991;104:29-36.
Haberkamp TJ, Monsell EM, House WF, Levine SC, Piazza L. Diagnosis and treatment of arachnoid cysts of the posterior
fossa. Otolaryngol Head Neck Surg 1990;103:610-614.
Hamann G, Bier B, Schimrigk K. Basilar aneurysm in the cerebello-pontine angle. Case report with review of the
literature. Nervenarzt 1992;63:113-115.
Iplikcioglu AC, Ozer F, Benli K, Bertan V, Ruacan S. Malignant teratoma of the cerebellopontine angle: case report.
Neurosurgery 1990;27:137-139.
Jackler RK, Shapiro MS, Dillon WP, Pitts L, Lanser MJ. Gadolinium-DTPA enhanced magnetic resonance imaging in
acoustic neuroma. Otolaryngol Head Neck Surg 1990;102:670-677.
Jamjoom A, Jamjoom ZA, Griffiths D, Moss T, Coakham H. Cerebellopontine angle epithelial cyst. A case report.
Neurosurg Rev 1993;16:67-70.
Janecka IP, Sekhar LN, Horton JA, Yonas H. General blood flow evaluation. In: Cummings CW, et al. Otolaryngology Head and Neck Surgery Update II. St. Louis: Mosby, 1990:54-63.
Jenkins HA. Hearing preservation in acoustic neuroma surgery. Laryngoscope 1992;102:125-128.
Jerger J, Oliver TA, Jenkins H. Suprathreshold abnormalities of the stapedius reflex in acoustic tumors: a series of case
reports. Ear Hear 1987;8:131-139.
Johnson EW. Results of auditory tests in acoustic tumor patients. In: House WF, Luetje CM, editors. Acoustic Tumors.
Baltimore: University Park Press, 1979.
Kartush JM, Niparko JK, Graham MD, Kemink JL. Electroneurography: preoperative facial nerve assessment for tumors
of the temporal bone. Otolaryngol Head Neck Surg 1987;97:257-261.
Kasantikul V, Netsky MG, Glasscock ME 3d. Acoustic neurilemmoma: clinicoanatomical study of 103 patients. J
Neurosurg 1980;52:28-35.
Kitamura K, Futaki T, Miyoshi S. Fluctuating hearing loss in lipoma of the cerebellopontine angle. ORL J
Knorr JR, Ragland RL, Smith TW, Davidson RI, Keller JD. Squamous carcinoma arising in a cerebellopontine angle
epidermoid: CT and MR findings. AJNR Am J Neuroradiol 1991;12:1182-1184.
Koci TM, Chiang F, Mehringer CM, Yuh WT, Mayr NA, Itabashi H, et al. Adult cerebellar medulloblastoma: imaging
features with emphasis on MR findings. AJNR Am J Neuroradiol 1993;14:929-939.
Lalwani AK. Meningiomas, epidermoids, and other nonacoustic tumors of the cerebellopontine angle. Otolaryngol Clin
North Am 1992;25:707-728.
Lalwani AK, Jackler RK. Preoperative differentiation between meningioma of the cerebellopontine angle and acoustic
neuroma using MRI. Otolaryngol Head Neck Surg 1993;109:88-95.
Langman AW, Jackler RK, Althaus SR. Meningioma of the internal auditory canal. Am J Otol 1990;11:201-204.
Le-Marc Hadour F, Mouret P, Bost F, Pasquier D, Pasquier B, Charachon R. Lipoma of the internal auditory canal: an
anatomo-clinical case study and review of the literature about cranial nerve lipomas. Arch Anat Cytol Pathol 1991;39:147150.
Linthicum FH Jr , Waldorf R, Luxford WM, Caltogirone S. Infrared/video ENG recording of eye movements to evaluate
the inferior vestibular nerve using the minimal caloric test. Otolaryngol Head Neck Surg 1988;98:207-210.
Lipper MH, Cail WS. Chordoma of the petrous bone. South Med J 1991;84:629-631.
Lo WW. Tumors of the temporal bone and cerebellopontine angle. In: Som PM, Bergeron RT, editors. Head and Neck
Imaging, 2nd ed. St. Louis: Mosby, 1991:1046-1108.
Lunardi P, Missori P, Salvati M, Licastro G. Cholesterol granuloma of the cerebello-pontine angle. Clin Neurol Neurosurg
1992;94:265-268.
Martuza RL, Eldridge R. Neurofibromatosis 2 (bilateral acoustic neurofibromatosis). N Engl J Med 1988;318:684-688.
McCormick PC, Bello JA, Post KD. Trigeminal schwannoma: surgical series of 14 cases with review of the literature. J
Neurosurg 1988;69:850-860.
Mohsenipour I, Deusch E, Ortler M, Pallua A. Meningiomas of the cerebellopontine angle. Neurochirugia 1993;36:90-92.
Pappas DG, Brackmann DE. Arachnoid cysts of the posterior fossa. Otolaryngol Head Neck Surg 1981;89:328-332.
Pappas DG, Schneiderman TS, Brackmann DE, Simpson LC, Chandra Sekar B, Sofferman RA. Cavernous hemangiomas
of the internal auditory canal. Otolaryngol Head Neck Surg 1989;101:27-32.
Parnes LS, Lee DH, Peerless SJ. Magnetic resonance imaging of facial nerve neuromas. Laryngoscope 1991;101:31-35.
Press GA, Hesselink JR. MR imaging of cerebellopontine angle and internal auditory canal lesions at 1.5T. AJR Am J
Roentgenol 1988;150:1371-1381.
Rubenstein LI. Tumors of the central nervous system. In: Firminger HI, editor. Atlas of Tumor Pathology, Second series,
fascicle 6. Washington, D.C.: Armed Forces Institute of Pathology, 1972.
Russell DS, Rubenstein LJ. Pathology of Tumors of the Nervous System, 4th ed. Edinburgh: T & A Constable, 1977.
Sataloff RT, Davies B, Myers DL. Acoustic neuromas presenting as sudden deafness. Am J Otol 1985;6:349-352.
Saunders JE, Kwartler JA, Wolf HK, Brackmann DE, McElveen JT Jr. Lipomas of the internal auditory canal.
Laryngoscope 1991;101:1031-1037.
Segall HD, Zee CS, Naidich TP, Ahmadi J, Becker TS. Computed tomography in neoplasms of the posterior fossa in
children. Radiol Clin North Am 1982;20:237-253.
Sekhar LN, Jannetta PJ. Cerebellopontine angle meningiomas: microsurgical excision and follow-up results. J Neurosurg
1984;60:500-505.
Selters WA, Brackmann DE. Acoustic tumor detection with brainstem electric response audiometry. Arch Otolaryngol
1977;103:181-187.
Shinozaki N, Sekiya T, Suzuki S, Iwabuchi T, Suzuki M. Five cases of intracranial lipoma; CT and magnetic resonance
images. No Shinkei Geka 1992;20:289-293.
Spector GJ, Wobol S, Thawley SE, Maisel RH, Ogura JH. Glomus jugulare tumors of the temporal bone: patterns of
invasion of the temporal bone. Laryngoscope 1979;89:1628-1639.
Tekkok IH, Suzer T, Erbengi A. Non-acoustic tumors of the cerebellopontine angle. Neurosurg Rev 1992;117-123.
Valvanis A, et al. Clinical Imaging of the Cerebellopontine Angle. Berlin: Springer-Verlag, 1986.
Weber AL. Magnetic resonance imaging and computed tomography of the internal auditory canal and cerebellopontine
angle. Isr J Med Sci 1992;28:173-182.
Westra I, Drummond GT. Occult pontine glioma in a patient with hemifacial spasm. Can J Ophthalmol 1991;26:148-151.
Wilms G, Plets C, Boossens L, Goffin J, Vanwambeke K. The radiological differentiation of acoustic neuronoma and
meningioma occurring together in the cerebellopontine angle. Neurosurgery 1992;30:443-5, 445-6.
Yokota J, Imai H, Okuda O, Sato K. Inverted Bruns' nystagmus in arachnoid cysts of the cerebellopontine angle. Eur
Neurol 1993;33:62-64.


medical literature.
PEDIATRIC CHOLESTEATOMA
A. Paul Vastola, MD
October 7, 1993
The term cholesteatoma is actually a misnomer coined by Johannes Muller in 1838. He described "a
layered pearly tumor of fat which was distinguished from other fat tumors by the presence of biliary fat or
cholestrin that is interspersed among sheets of polyhedral cells." Aural cholesteatomas are best thought of
as skin growing in the middle ear space. Cholesteatomas actually do not contain fat but are composed of
an outer matrix which surrounds layers of desquamated epithelium. The matrix is comprised of fully
differentiated keratinizing squamous epithelium resting on a collagenous perimatrix. Various theories
have been advanced to account for the destructive bone resorption seen commonly with these lesions.
These include activation of osteoclastic bone destruction, mechanical pressure necrosis and bone
degradation by enzymes such as collagenases and lysozymes.
Cholesteatomas may be classified as either congenital or acquired. Congenital cholesteatomas develop

behind an intact tympanic membrane; according to the classic teachings of Derlacki and Clemis there
must be no antecedent history of infection or breach of the tympanic membrane. This definition has
recently been challenged by Friedman et al who note that approximately 70% of children will have had at
least one episode of otitis media. There is little doubt that patients with epidermal rests of cells in the
protympanum may also have episodes of AOM. The acquired form of cholesteatoma is much more
common. Primary acquired disease arises from a skin lined retraction pocket within which retained
keratin debris accumulates. Primary acquired cholesteatoma occurs most commonly in the posteriorsuperior quadrant of the pars tensa and in the pars flaccida. Secondary acquired cholesteatoma develops
from an ingrowth of skin through a tympanic membrane perforation that is then retained within the
middle ear, mastoid or both.
The pathogenesis of cholesteatoma growth is still poorly understood as evidenced by the multiple theories
currently found in the literature. However, a common denominator appears to be eustachian tubal
dysfunction. Bluestone proposes that patients with acquired cholesteatoma have a functional obstruction
of the eustachian tube and are thus predisposed to high negative middle ear pressures. The areas of the
tympanic membrane most susceptible to these forces are the pars flaccida and posterior-superior regions.
The expansion or growth of cholesteatomas are channeled along well defined pathways determined by
ligaments and folds. Review of embryologic development is helpful in understanding these pathways.
Between the 3rd and 7th months of development the gelatinous tissue of the middle ear space is absorbed.
A primitive tympanic cavity develops by growth of an endothelium lined pouch extending from the
eustachian tube to the middle ear cleft. Four primary sacs then bud into the cleft. The remnants of these
sacs direct the growth of cholesteatoma along predictable pathways in the middle ear. Prussaks space is
commonly thought of as the most common point of invasion for primary acquired cholesteatomas. From
this space cholesteatomas expand in one of three directions: the posterior route is most commonly seen,
and follows the superior incudal space above the incus into the epitympanum. The inferior route follows
the inferior incudal space into the mesotympanum. The anterior route from Prussaks space is the route
least frequently travelled. The anterior pouch of von Trolsch serves as the avenue for spread into the
protympanum.
The presence of cholesteatoma requires surgical intervention unless underlying medical problems
contraindicate exposure to general anesthesia. Most cholesteatomas are asymptomatic in their early
development; children may come to medical attention with otorrhea but will rarely complain of decreased
hearing. Microscopic examination of the ear is imperative in identifying and delineating cholesteatoma.
Audiometric evaluation is part of the standard preoperative workup. Computerized tomography may be
used to delineate the extent of disease, check the aeration of the mastoid and to help rule out
intratemporal and /or intracranial complications.
The primary surgical goal is to achieve a safe, dry ear by removing disease with the preservation of
normal anatomy. Improving hearing is a secondary goal. The principles of surgical management are based
on the extent of the disease and the presence of complications arising from cholesteatoma. Much debate
has emerged in the literature concerning canal wall up procedures vs canal wall down approaches. It is
clear that the consensus among otologists over the last several years has been that canal wall up
procedures are indicated in patients with well pneumatized mastoids and adequate middle ear clefts with
cholesteatoma limited to the middle ear space or mastoid. Relative contraindications to canal wall up
procedures include a sclerotic mastoid, a fistula, an only hearing ear and poor eustachian tubal function.
There is no objective measurement of eustachian tubal function though several clinical observations can
help predict the ability of the middle ear to ventilate itself. According to Parisier et al the appearance of
the pars tensa, the amount of mastoid cellular development and the appearance of middle ear mucosa will
reflect the eustachian tubal function and guide the surgical approach. The success of any given approach
may be measured in terms of the rate of recidivism. Originally a term applied to criminal behavior,
recidivism is the measurement of residual disease plus recurrent cholesteatoma.
Cholesteatoma in children is widely considered to be a more aggressive disease than in the adult
population for two major reasons. First, very extensive disease is found more frequently in children
compared to adults and second, higher rates of residual and recurrent disease have been documented in
the pediatric population. Of note however is the observation that the incidence of complications arising
from cholesteatoma is directly related to the duration of the disease and, as such, adults tended to have
higher complication rates.
Glasscock in 1981 published a retrospective review of charts comparing rates of recidivism in patients
less than 16 years old to those older than 16. Almost 90% of both groups had a canal wall up approach
regardless of initial findings. Rates of recidivism were compared and it was found that patients less than
16 had an almost two-fold higher rate and a much shorter interval to recurrence. Based on these findings
Glasscock concluded that pediatric cholesteatoma is more aggressive than that seen in adults. There was
no statistical analysis presented and no criteria were set forth regarding canal wall up vs canal wall down
approaches.
In 1977 Palva published a retrospective evaluation of 65 pediatric patients (<16 years old) matched with
65 adults with cholesteatoma. All patients underwent canal wall down procedures. The duration of
disease in the pediatric population was shorter and the number of complications was higher in adults than
in children. Operative findings revealed that 65% of pediatric ossicular mechanisms were disrupted
compared to 84% in the adult group despite the finding that cholesteatoma was more extensive in the
pediatric group. The authors calculated a 5% rate of recidivism in the pediatric group but did not calculate
rates in the adult group. Based on the finding that cholesteatoma tended to be more extensive in children
and have an overall shorter duration of disease these workers conclude that pediatric cholesteatoma is
more aggressive. There is no mention of recurrence rates in adults however.
A study published in 1988 by Parisier illustrates the results of a single surgeon over 15 years. Operative
approach was dictated by intraoperative findings with 53% of patients undergoing canal wall down
procedures and 30 % of patients undergoing canal wall up approaches. The remainder of patients had a
tympanotomy for localized disease. Average follow-up was 4 years and rates of recidivism did not differ
significantly between the pediatric population and an adult population. Based on these findings, the
authors conclude that pediatric cholesteatoma is not a different disease than that seen in adults but that
surgical approaches must be individualized to the patient's disease.
A preliminary evaluation of the Baylor experience with pediatric cholesteatoma was performed. There
were 26 patients (19 males and 7 females) for a total of 27 operated ears. The age at presentation
averaged 8 years (range 2-15 years) with an average follow-up of 4 years (range 3 months to 15 years).
The most common presenting symptoms were otorrhea (11/26), hearing loss (4/26) and tympanic
membrane perforation (3/26). Five patients had congenital cholesteatoma. Pre-operative and postoperative audiograms were performed in all patients and there was no significant difference in pure tone
averages (30dB pre-op compared with 27dB post-op). Fifty-four percent of patients had canal wall up
procedures while 27% had canal wall down procedures. Nineteen percent of patients had a tympanotomy
approach. Planned second look surgery was performed in 16 of 27 ears. Two patients underwent primary
ossicular reconstruction while 9 patients had a secondary ossicular reconstruction. The rate of residual
disease was 4/27 (15%) while the rate of recurrent disease was 3/27 (11%) for a recidivism rate of 26%.
In conclusion there remain many questions concerning the pathophysiology of cholesteatoma; whether or
not the disease differs in children and adults is still a matter of debate. The next area of inquiry will be on
a cellular level. It is clear however, from the above data, that a reasoned individualized approach to
cholesteatoma is the best way to achieve low rates of recidivism.
Case Presentations
An eight-year-old white female child presented with a history of chronic eustachian tube dysfunction,
numerous episodes of acute otitis media and recent bloody otorrhea from the left ear. Her otologic history
dates to the age of three when she had an episode of acute otitis media which was treated with antibiotics
and decongestants. The patient subsequently failed a school audiogram and continued to require
intermittent courses of oral antibiotics. Subsequent school audiogram failures were attributed to "fluid in
the ears." There was no history of facial nerve palsy, vertigo or otologic surgery.
Physical exam was significant for a white mass behind the posterior half of the right tympanic membrane
and a central posterior, superior perforation of the left tympanic membrane. There was no evidence of
keratin debris although the drum was severely retracted over the malleus. Audiometric evaluation of the
right ear demonstrated a slight loss over the lower frequencies and a type C tympanogram. CTT of the
temporal bones revealed a soft tissue mass involving the tympanic membrane extending toward the
epitympanum without erosion of the scutum.
The child was brought to the operating room where a right tympanomastoidectomy (canal wall up) was
performed. Cholesteatoma was found involving the posterior half of the eardrum, extending towards the
epitympanum and antrum. The ossicular mechanism was noted to be intact although there was some
attenuation of the incudostapedial joint. The cholesteatoma was excised completely, leaving the ossicles
intact. A medial graft was incorporated. The patient has done well postoperatively.
Bibliography
Abramson M, Lachenbruch PA, Press BA, McCabe BF. Results of conservative surgery for middle ear cholesteatoma.
Laryngoscope 1977;87:1281-1286.
Derlacki EL, Clemis JD. Congenital cholesteatoma of the middle ear and mastoid. Ann Otol Rhinol Laryngol 1965;74:706727.
Edelstein DR, Parisier SC. Surgical techniques and recidivism in cholesteatoma. Otolaryngol Clin North Am 1989;22:10291040.
Edelstein DR, Parisier SC, Ahuja GS, Jurabe C, Clute P, Wenig S, et al. Cholesteatoma in the pediatric age group. Ann
Otol Rhinol Laryngol 1988;97:23-29.
Edelstein DR, Parisier SC, Han JC. Acquired cholesteatoma in the pediatric age group. Otolaryngol Clin North Am
1989;22:955-965.
Glasscock ME III, Dickins JRE, Wiet R. Cholesteatoma in children. Laryngoscope 1981;91:1743-1753.
Jackson DG, Glasscock ME III, Nissen AJ, Schwaber MK, Bojrab DI. Open mastoid procedures: contemporary indications
and surgical technique. Laryngoscope 1985;95:1037-1043.
Jahn AF. Cholesteatoma: What is it, how did it get there, and how do we get rid of it? Otolaryngol Clin North Am
1989;22:847-857.
Jansen C. Cholesteatoma in children. Clin Otolaryngol 1978;3:349-352.
McGill TJ, Merchant S, Healy GB, Friedman EM. Congenital cholesteatoma of the middle ear in children: a clinical and
histopathological report. Laryngoscope 1991;101:606-613.
Nadol JB Jr. Causes of failure of mastoidectomy for chronic otitis media. Laryngoscope 1985;95:410-413.
Palva A, Karma P, Krj J. Cholesteatoma in children. Arch Otolaryngol 1977;103:74-77.
Schmid H, Dort JC, Fisch U. Long-term results of treatment for children's cholesteatoma. Am J Otol 1991;12:83-87.
Schulerati N, Bluestone CD. Pathogenesis of cholesteatoma. Otolaryngol Clin North Am 1989;22:859-881.
Sheehy JL. Cholesteatoma surgery in children. Am J Otol 1985;6:170-172.
Sheehy JL. Cholesteatoma surgery: canal wall down procedures. Ann Otol Rhinol Laryngol 1988;97:30-35.
Sheehy JL, Patterson ME. Intact canal wall tympanoplasty with mastoidectomy: a review of eight years' experience.
Laryngoscope 1967;77:1502-1542.
Sismanis A, Hutchinson L, Abedi E. Chronic otitis media: surgical failures and management. Am J Otol 1989;10:460-465.
Stern SJ, Fazekas-May M. Cholesteatoma in the pediatric population: prognostic indicators for surgical decision making.
Laryngoscope 1992;102:1349-1352.
Toner JG, Smyth GDL. Surgical treatment of cholesteatoma: a comparison of three techniques. Am J Otol 1990;11:247249.
Tos M. Treatment of cholesteatoma in children: a long-term study of results. Am J Otol 1983;4:189-197.
Vartiainen E, Nuutinen J. Long-term results of surgical treatment in different cholesteatoma types. Am J Otol 1993;14:507511.
Weiss MG, Parisier SC, Han JC, Edelstein DR. Surgery for recurrent and residual cholesteatoma. Laryngoscope
1992;102:145-151.
Wullstein SR. Cholesteatoma in children: is the disease different in childhood? Clin Otolaryngol 1978;3:353-362.


medical literature.
CHORDOMAS OF THE SKULL BASE

Carla M. Giannoni, MD
October 1, 1992
Chordomas are relatively rare neoplasm arising from embryonic notochordal remnants and comprise less
than 1% of intracranial neoplasms. They typically occur along the neuraxis, especially at the
developmentally more active cranial and caudal ends, notably in the spheno-occipital, sacrococcygeal,
and vertebral locations. Twenty-five percent to 40% of chordomas occur in the speno-occipital or skull
base region. These tumors occur predominately in the 30 to 50 year old age range and show a slight
predominance in men.
These tumors can occur extra-axially, in unusual locations, and are then termed primary. They probably
arise from ectopic notochordal elements. Chordomas have been described in the nasopharynx, mandible,
maxillary sinus, frontal sinus, and, as in our case report, in the petrous apex of the temporal bone.
In 1856 Luschke, working in Virchow's lab, described jelly-like tumors in the region of the clivus of
Blumenbach. In 1857 Virchow also examined these tumors and named them "ecchondrosis
physaliphora." He believed they arose from the spheno-occipital synchondrosis and were of cartilaginous
origin. Muller, in 1858, examined embryonic tissues and, after noting their histologic similarities,
postulated these tumors derived from embryonic notochord. In 1895 Ribbert demonstrated the origin of
these tumors when he pierced the nucleus pulposus of the vertebral column and found that similar tumors
developed. He applied the term "chordoma" to these tumors.
The embryonic development of the notochord is important in understanding the anatomic distribution of
these tumors. The primitive streak forms in the third week of fetal development and forms the notochord.
During the fifth week of life, it extends from the coccyx cephalad to the skull base, where it courses
through the odontoid process, the posterior sphenooccipital plate and ends at Rathke's pouch. The
notochord becomes surrounded by mesodermal tissue which then forms the vertebral column. Ultimately
the notochord disappears, but remnants persist in the nucleus pulposus of the intervertebral discs.
Binkhorst et al described seven points of origin of craniocervical chordomas: the dorsum sellae, the
Blumenbach's clivus, retropharyngeal region, squama occipalis, nuclei pulposi of cervical vertebrae, and
the ligament of the dens.
SYMPTOMS
The clinical presentation depends on the origin and extension of each particular tumor. Delay in
diagnosis is common, secondary to the occult nature of the disease and poorly localizing signs and
symptoms. A headache arising in the vertex, ipsi-parietal, orbital, or frontal regions is a typical early
symptom. This is due to stretching of middle fossa dura and is often severe. Patients may also present
with paresthesias or anesthesia of the jaw region, serous otitis media, hearing loss, nasal
obstruction/anosmia, syncope and, rarely, vertigo. Recurrent meningitis has also been noted in these
patients.
The late symptoms generally depend on the direction of tumor extension. Anterior extension leads to
diplopia and ophthalmoplegia, or both. Posterior extension presents as facial or other lower cranial nerve
neuropathies.
RADIOGRAPHIC FEATURES
CT findings of an expansile, destructive, lytic lesion with associated soft tissue mass are characteristic of
chordomas. However, they are also seen with chondrosarcomas and other similar lesions. Foci of
calcification may also be seen. The CT scan is useful in defining the anatomy of bone destruction.
MRI is better than CT for defining the limits of a lesion and any vascular relationships, especially on the
T2-weighted images. MRI of chordomas show hyperintensity in T2 and hypointensity in the T1-weighted
images. The T1 images are especially useful in defining any tumor-SCF interfaces.
On arteriogram, an avascular may be seen displacing the basilar artery. Radiographic studies cannot
reliably distinguish chordomas from chondrosarcomas and other similar lesions, but can help eliminate
other possible diagnoses.
DIFFERENTIAL DIAGNOSIS
Benign lesions most frequently occurring at the skull base include: meningiomas,
neuromas/schwannomas, glomus tumors, vascular anomalies (e.g., internal carotid aneurysms),
congenital cholesteatoma, mucocele, osteomyelitis, eosinophilic granuloma, and cholesterol cysts.
Malignant lesions of this location include primary carcinoma (squamous-, adeno-, acinic, adenoid cystic),
metastatic carcinoma (breast, prostatic, renal cell, bronchogenic), rhabdomyosarcoma, nasopharyngeal
cancer, lymphoma and chordoma, and other mesenchymal tumors (chondroma, chondrosarcoma, and
osteoclastoma).
HISTOPATHOLOGY
Four criteria have been used in the histologic diagnosis of chordoma: 1) a lobular arrangement of cells;
2) a tendency of the cells to grow in cords, irregular bands, or pseudoacinar forms; 3) production of
abundant intercellular mucinous matrix; and 4) the presence of large physaliphorous cells. A
microscopically mixed population of cell types exist: stellate or primordial cells, intermediate cells and
the physaliphorous cells. The stellate cell is the only actively proliferating cell. Chordoma cells then
proceed through a stage of vacuolization until they reach the characteristic physaliphorous appearance.
The cells then progress to destruction and rupture, completing their life cycle.
Immunohistochemical tests have been developed in an effort to aid in the differentiation of these lesions.
Chordomas are frequently positive for epithelial antigens - cytokeratin (CK) and epithelial membrane
antigen (EMA), and negative for vimentin.
A subtype of chordoma, chondroid chordoma, deserves special mention. Histologically these tumors are
a mixture of chordoma, chondroma, and chondrosarcoma.
PROGNOSIS AND TREATMENT
The reported average survival for chordomas of the skull base is 4.1 years. There appears to be a better
prognosis for chordomas of the nasopharynx and paranasal sinuses. Notably, chondroid chordomas have
a reported 15.8 years average survival. The best treatment for these lesions is total surgical excision.
Recurrence is the rule and metastases are extremely uncommon; patients usually succumb to local
disease.
Radiation therapy has been used in the postoperative care of these patients because of the tumor's usual
relentless course to local recurrence and death with current conventional treatments. Fractionated proton
radiation therapy is currently advocated for the treatment of chordomas. Because of its higher biological
effectiveness, patients can be treated with a higher total equivalent radiation dose.
Case Presentation
A 57-year-old white female presented with a six- to nine-month history of intermittent left temporal
headaches. They occurred monthly and usually lasted three days. She had no complaints of visual
difficulties, hearing loss, vertigo, hoarseness, or dysphagia. Her past medical, surgical, and family
histories were unremarkable. On physical exam she was found to be healthy. All cranial nerves were
functionally intact.
An MRI of the brain was done and revealed a signal abnormality of the left petrous apex and adjacent
basiocciput with associated enhancement. A CT scan of the head and cervical spine showed a
destructive, lytic lesion of the left basiocciput just anterior to the jugular foramen and anterolateral to the
foramen magnum with probable erosion of the carotid canal.
A full metastatic workup ensued including routine laboratory work, chest x-ray, mammogram, CT of the
abdomen and pelvis, thyroid ultrasound, and serum protein electrophoresis. No significant abnormalities
were found. A radionucleotide bone scan showed no additional bony lesions. Four vessel cerebral
angiogram was negative for vascular abnormality. Three months later a follow-up CT scan of the head
with thin cuts of the temporal bone showed a 1.5 X 2 X 1 cm lytic lesion corresponding with the
previously identified lesion, but slightly increased in size. The bony margins of the lesions were irregular
and suggestive of an aggressive process. She was then referred for surgical evaluation.
She underwent a joint neurotologic and neurosurgical procedure comprised of a combination left type B
infratemporal fossa and temporal fossa approach with biopsy and removal of the tumor. Frozen section
revealed the tumor to be a chordoma. The patient tolerated the procedure well and had an uneventful
postoperative course. The patient has been referred to Boston for fractionated proton radiation therapy.
Bibliography
Austin-Seymour M, Munzenrider J, Boitein M, Verhey L, Urie M, Gentry R, et al. Fractionated proton radiation therapy of
chordoma and low-grade chondrosarcoma of the base of the skull. J Neurosurg 1989;70:13-17.
Batsakis JG. The pathology of head and neck tumors: neoplasms of cartilage, bone and the notochord. Head Neck Surg
1980;3:43-57.
Batsakis JG. Tumors of the head and neck. Baltimore: Williams and Wilkins, 1979.
Batsakis JG, Kittleson AC. Chordomas. Otorhinolaryngologic presentation and diagnosis. Arch Otolaryngol 1963;78:168175.
Brooks JJ, LiVolsi VA, Trojanowski JQ. Does chondroid chordoma exist? Acta Neuropathol 1987;72:229-235.
Carlson BM. Patten's foundations of embryology, 5th edition. New York: McGraw-Hill, 1988.
Crown RV, Sage MR, Brophy BP. CT and MR findings in patients with chordomas of the petrous apex. AJNR Am J
Neuroradiol 1990;11:121-124.
Crumley RL, Butin PH. Surgical access for clivus chordoma. The University of California, San Francisco, experience.
Dahlin DC, MacCarty CS. Chordoma. A study of fifty-nine cases. Cancer 1952;5:1170-1178.
Fisch U. Infratemporal fossa approach to tumors of the temporal bone and base of skull. J Laryngol Otol 1978;92:969-977.
Fisch U, Pillsbury HC. Intratemporal approach to lesions in the temporal bone and base of skull. Arch Otolaryngol
1979;105:99-107.
Flood LM, Kemink JL. Surgery in lesions of the petrous apex. Otolaryngol Clin North Am 1984;17:565-75.
Gacek RR. Diagnosis and management of primary tumors of the petrous apex. Ann Otol Rhinol Laryngol 1975;84(Suppl
18):1-20.
Glasscock ME III, Gulya AJ, Pensak ML. Surgery of the posterior fossa. Otolaryngol Clin North Am 1984;17:483-497.
Glasscock, ME III, Miller GW, Drake FD, Kanok MM. Surgery of the skull base. Laryngoscope 1978;88:905-923.
Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW. Chordomas and cartilaginous tumors at the skull base. Cancer
1973;32:410-420.
House WF. Middle cranial fossa approach to the petrous pyramid. Arch Otolaryngol 1963;78:460-469.
House WF, DeLaCruz A, Hitselberger WE. Surgery of the skull base; transcochlear approach to the petrous apex and
clivus. Otolaryngology 1978;86:770-779.
Jenkins HA, Franklin DJ. Infratemporal approaches to the skull base. In: Jackson CG, editor. Surgery of skull base tumors.
New York: Churchill Livingstone, 1991:121-139.
Kaneko Y, Sato Y, Iwaki T, Shin RW, Tateishi J, Fukui M. Chordoma in early childhood: a clinicopathological study.
Kendal BE, Lee BCP. Cranial chordomas. Br J Radiol 1977;50:687-698.
Kondziolka D, Lunsford LD, Flickinger JC. The role of radiosurgery in the management of chordoma and chondrosarcoma
of the cranial base. Neurosurgery 1991;29:38-46.
Krespi YP, Levine TM, Oppenheimer R. Skull base chordomas. Otolaryngol Clin North Am 1986;19:797-804.
Kumar PP, Good RR, Skultety FM, Leibrock LG. Local control of recurrent clival and sacral chordoma after interstitial
irradiation with iodine 125: new techniques for treatment of recurrent or unresectable chordomas. Neurosurgery
1988;22:478-483.
Lipper MH, Cail WS. Chordoma of the petrous bone. South Med J 1991;84:629-631.
Lushka H. Cited by: Windeyer BW. Chordoma. Proc R Soc Med 1959;52:1088-1100.
Meis JM, Giraldo AA. Chordoma. An immunohistochemical study of 20 cases. Arch Pathol Lab Med 1988;112:553-556.
Miller RH, Woodson GE, Neely JG, Murphy EC. A surgical approach to chordomas at the base of the skull. Otolaryngol
Head Neck Surg 1982;90:251-255.
Muller H. Cited by: Windeyer BW. Chordoma. Proc R Soc Med 1959;52:1088-1100.
Pile RF, Melville GE, New PF, Austin-Seymour M, Munzenrider J, Pile-Spellman J, et al. The role of MR and CT in
evaluating clival chordomas and chondrosarcoms. AJR AM J Roentgenol 1988;151:567-575.
Ribbert H. Cited by: Windeyer BW. Chordoma. Proc R Soc Med 1959;52:1088-1100.
Rupa V, Rajshekhar V, Bhanu TS, Chandi SM. Primary chondroid chordoma of the base of the petrous temporal bone. J
Laryngol Otol 1989;103:771-773.
Salisbury JR. Demonstration of cytokeratins and an epithelial membrane antigen in chondroid chordoma. J Pathol
1987;153:37-40.
Sen CN, Sekhar LN, Schramm VL, Janecka IP. Chordoma and chondrosarcoma of the cranial base: an 8-year experience.
Shugar JMA, Som PM, Krepsi UP, Arnold LM, Som ML. Primary chordoma of the maxillary sinus. Laryngoscope
1980;90:1825-1830.
Spoden JE, Bumsted RM, Warner ED. Chondroid chordoma, case report and literature review. Ann Otol Rhinol Laryngol
1980;89:279-285.
Virchow R. Cited by: Windeyer BW. Chordoma. Proc R Soc Med 1959;52:1088-1100.
Wright D. Nasopharyngeal and cervical chordoma. Some aspects of their development and treatment. J Laryngol Otol
1967;81:1337-1355.


medical literature.
CONGENITAL AURAL ATRESIA

January 12, 1995
Alberto D. Fernandez, M.D.
The first mention of attempts to surgically correct congenital aural atresia was made by Thomson in 1843
in England. Although 2 out of the three cases were aborted when a thick atresia plate was encountered, a
third record documented what was presumably a tympanic membrane, although it appears that stenosis
became a problem postoperatively. In 1883, Kiesselbach attempted an atresia repair on a child that
resulted in facial paralysis. It was not until 1947 and the work of Pattee and Ombredonne that a
significant improvement in hearing could be obtained with surgery. Pattee reported that fixation of the
stapes via the lateral chain to the atresia plate was responsible for these patients' deficits. Ombredonne
devised an approach through the mastoid in order to fenestrate the lateral semicircular canal, with
reportedly good hearing results.
Using Wullstein and Zollner's tympanoplasty techniques, Bellucci reported a 30 decibel gain in hearing
level in 50% of his cases. Shambaugh, in 1967, recommended surgery in unilateral cases only if the
cochlear reserve allowed hearing improvement to approach that of the normal ear by 25dB. Several
studies by Gill, Naumann and Schuknecht reported their individual experiences adding minor variations
to the basic transmastoid approach. With the advent of polytomography and subsequently, computed
tomography, finer detail of middle and inner ear structures could be obtained and used to select patients
for surgery. Jahrsdoerfer, in 1978, reported the first large series using the anterior approach for
congenital aural atresia repair, whereby the middle ear cavity was entered directly via a newly-created
external canal without performing a mastoidectomy.
An appreciation of the events that occur during embryogenesis with regard to the inner, middle and
external ear is requisite to understanding the trends that occur in atresia.
The inner ear, including all of the components of the membranous labyrinth, develops from an
invagination of ectoderm, the otic placode, at approximately 3 week's gestation, and achieves adult
dimensions at midterm. Further elaboration on inner ear development and abnormality is the subject of
Congenital Sensorineural Hearing Loss.
The middle ear space, including the mastoid air cell system, the Eustachian tube and the inner surface of
the tympanic membrane derive from endoderm of the first pharyngeal pouch. Most of these elements
complete their development by the 30th week, excepting the mastoid system in which pneumatization
can progress well into adulthood. Due to the late development of the mastoid system, several authors
have pointed out that patients with atresia who maintain a well-developed mastoid generally manifest
greater development of their middle ear space and ossicles, thus making them better surgical candidates.
Ossicular development, which progresses from the 8th through the 26th week, derives from the
mesoderm of the first and second branchial arches. The malleus head and body and the short process of
the incus arise from the first branchial arch (Meckel's) cartilages. Second branchial (Hyoid) arch
derivatives include the manubrium of the malleus, the long process of the incus and the stapes
superstructure. The footplate derives from both the second arch and the lamina stapedialis of the otic
capsule. Ossification occurs through endochondral bone growth. Release of the ossicles except at their
ligamentous attachments occurs by endodermal resorption as it gives rise to the tympanic cavity.
Ectoderm of the first branchial cleft gives rise to the external auditory canal, which develops as an
invagination at the site of the future auricle at the fourth gestational week. By the eighth week, a solid
core of epithelium arises and extends to the area of the middle ear space, separated from it by a thin layer
of mesoderm. At approximately 28 weeks, this core begins to recanalize from medial to lateral until the
surface ectoderm is reached giving rise to the external auditory canal. At its medial extent, ectoderm
persists as the outermost layer of the TM, with the mesodermal layer reduced to a fibrous sheet
interposed between outer ectoderm and inner cuboidal endoderm.
Mesoderm from the tympanic ring serves as the primary impetus for the formation of the tympanic bone
and the osseous portion of the external auditory canal, starting at about the 12th week. Finally, the auricle
is the result of the fusion of six hillocks (His) from the first and second branchial arches, which is usually
complete by the 12th week as well. Malformations are graded I through III, with Grade I manifesting as a
small, but well-formed auricle; Grade II representing a recognizable auricle with varying degrees of
anomaly; and Grade III, which describes a rudimentary appendage or none at all. Auricular anomalies are
the subject of Microtia.
Congenital aural atresia has a reported incidence that varies between 1 in 10 to 1 in 20,000. Congenital
aural atresia is usually found in isolation and in these instances patterns of inheritance have not been
seen. Its association with several known craniofacial syndromes is well known. Aural atresia has been
found in association with 22 of the 71 known craniofacial syndromes. The most common of these are
Treacher-Collins (mandibulofacial dysostosis), Nager (acrofacial dysostosis), Crouzon's (craniofacial
dysostosis), hemifacial microsomia, and the first and second branchial arch syndromes including
Goldenhar's (oculoauriculovertebral dysplasia). In unilateral cases, Jafek et al and others have cited an
increased incidence of atresia on the right. Bilateral atresia has been reported to occur in approximately
30% of cases.
Establishing a classification system of the congenital ear malformations has been almost as difficult as
the operative procedures themselves. Several schemes have been proposed over time, one the first being
that proposed by Marx in 1926 for auricular anomalies, which were graded I - III for mild to severe
deformities. Altmann in 1955 proposed the first schema based on the temporal bone itself, looking at the
status of the canal, tympanic bone, drum and ossicles. Lapchenko in 1967 and Gill in 1969 both set up a
four tier scale, examining the degree of middle ear and external canal development, and the presence of
ossicular abnormality. Additionally, Gill incorporated into his system, the degree of pneumatization of
the mastoid, as this seemed to predict the relative success of operative interventions. Ombredonne and
Nager and Colman each tried to establish simplified and clinically practical systems based on Altmann's
original scheme, but these were inconsistent at predicting outcome in those cases of major aplasia or
group II anomalies. Jahrsdoerfer in 1992 established not so much a classification system, but rather a
grading scale as a means selecting patients that would most likely benefit from attempts at repair of their
atresias.
This system of grading allows for a quantitative analysis of the temporal bone and those structures that
are considered vital to the success of an operation. Whereas prior systems were excellent descriptions of
surgical and anatomical findings, they lacked predictive power and were subject to interobserver bias.
This scale, which is based on temporal bone CT findings, assesses nine different parameters that are used
in making the determination of candidacy for surgery. Of note, the stapes and oval window complex
account for 3 out of the 10 points possible in the scale. Other parameters include the middle ear cleft,
facial nerve position, status of the ossicles and round window, and pneumatization of the mastoid. Scores
of 6 through 10 range from marginal to excellent candidates for surgery while a score of 5 or less usually
anticipates a poor outcome.
In general, patients with congenital aural atresia are seen in referral from neonatologists in those cases
where gross craniofacial anomalies are present. However, many cases of isolated atresia or even those
with very mild head and neck syndromes may be delayed until later in development and may be referred
by pediatricians for either atresia or for problems relating to a stenotic canal. As is usual, a thorough
history is important in the initial evaluation of these patients, including questions relating to the gestation
of the infant. Specifically, issues of drug utilization, toxic exposures, prior family history of hearing
impairment and developmental craniofacial disorders and maternal infections should be probed as
possible etiologies.
A thorough physical exam of the head and neck as well as of other organ systems should be performed,
especially the spine, extremities and genitourinary systems as these develop concurrently with those of
the head and neck. Associated anomalies of the midface and mandible should be noted. Position of the
auricle and the degree of external canal development should be checked. The degree of mastoid
prominence should be noted as this may give an early insight to the operative potential of the patient.
Cranial nerve function, especially that of the facial nerve should be assessed.
An audiologic evaluation is essential in the initial assessment of these patients. A screening ABR should
be obtained for several reasons. The first is to establish the presence of a functional inner ear. In very
young patients this is accomplished with multichannel air and bone conduction ABR. In cases of bilateral
atresia, this form of ABR will also allow one to establish with greater certainty the ear with greater
cochlear function by examining the response obtained in wave I, which is indicative of ipsilateral
cochlear status only. The second reason for early ABR is that if an intact inner ear is present, the child
can be fitted early on with a bone-conducting device. The usual finding is that of moderate to maximal
conductive loss, although mild conductive losses are sometimes seen in cases with membranous atresia.
A third reason for early ABR is to assess the functional status of the contralateral ear in unilateral
atresias. Several cases of sensorineural or mixed losses in the contralateral, normal-appearing ear have
been reported and should be ruled out. Having ascertained the functional status of the inner ear,
diagnostic imaging is used to assess the degree and nature of the temporal bone deformity.
With CT scanning, an assessment of the ossicular mass, the nature of the atretic plate (whether bony or
soft), the position and course of the facial nerve, the degree of external canal development and mastoid
pneumatization is possible. The presence of a cholesteatoma, which has a slight predilection for stenotic
ears, can also be ruled out. Current recommendations for the timing of CT scan is at approximately 4
years when mastoid pneumatization is most complete. A review of the literature by Cressman et al has
shown that approximately 50% of patients seen for this condition are ultimately found to be candidates
for reconstruction based on CT scan findings.
The current consensus among those who undertake these procedures is that surgery on cases of either
bilateral or unilateral atresia should be deferred until the patient is at least 4 to 6 years of age, with an
additional delay to consenting age espoused by others for those cases of unilateral atresia. The need to
foster continued mental development and the evolution of speech skills requires that interim bone
conducting appliances be provided for these patients with bilateral atresias that have documented
cochlear function on ABR. Unilateral atresia patients usually do not require any intervention as long as
normal hearing is present in the contralateral ear.
Once the decision to proceed with surgery has been made, a consideration of both the functional and
cosmetic aspects of the operation needs to be taken together. Close coordination with the plastic surgeon
reconstructing the auricle is needed and usually takes place as a multi-staged procedure. Because of
problems relating to healing in a fibrotic, previously-operated area, current practice is to allow the plastic
surgeon to operate first with the implantation of an auricular framework of autogenous rib graft in a
subcutaneous pocket. Stage II consists of lobule transposition and remnant excision. Correction of the
atresia with the creation of a new external meatus, canal, drum and conducting mechanism in continuity
with the stapes and inner ear is completed in Stage III along with alignment of the new auricle to the
meatus. Stages IV and V usually consist of the creation of a new tragus and the elevation of the auricle
off the post-auricular skin.
Contrary to the standard practice of operating on the poorer hearing ear first, as is the case with chronic
ear infection, cholesteatoma and otosclerosis, the better hearing ear is operated on first in CAA as this
will afford the best chance for an optimal outcome. Two general approaches to the repair of atresia are
currently described, both of which commence with postauricular incisions. The first of these is the
transmastoid approach that makes use of the familiar landmarks of the tegmen and sinodural angle to
approach the mastoid antrum and the bony atresia plate. This dissection is conducted with the aid of a
facial nerve monitor, as the course of this nerve is commonly aberrant owing to the anomalous
development of the tympanic bone and mastoid. Some surgeons opt to perform incudostapedial
dislocation if this is feasible in order to prevent transmitted acoustic trauma to the inner ear with resultant
sensorineural loss. Once the plate is removed, a canal wall down mastoidectomy is completed leaving an
open cavity.
A second, newer, approach described by Jahrsdoerfer and known as the Anterior Approach involves
exposure of the tympanum and ossicular mass by directly removing bone from lateral to medial between
the glenoid fossa and the middle cranial fossa dura. Drilling begins just posterior to the condyle, and
proceeds medially using the middle fossa dura as a superior guide. An anterior and superior approach
presents the least risk in injuring an anomalous facial nerve. This usually leads to an ossicular mass
consisting of a fused incus and malleus attached to the atretic plate. Once identified, the atretic plate can
be taken down with diamond burrs and curettes, taking care to avoid contact with the ossicular mass.
Mobility of the stapes and the status of the oval and round windows is checked. Middle ear
reconstruction with a prosthesis is performed if necessary. Otherwise, temporalis fascia and splitthickness skin grafting is performed to create a new eardrum and line the newly-created canal. A
meatoplasty completes the procedure and places the canal in continuity with the auricular meatus.
Modification of this approach has been described including a canal wall-up approach that allows the
surgeon to identify the nerve and atresia plate prior to opening a canal.
The goal of attaining hearing improvement in an ear with a previously moderate to maximal conductive
loss varies from persistent maximal conductive loss to cases of near-total closure of the air-bone gap.
Although different parameters for successful improvement in hearing have been reported, according to
Cressman et al, if one chooses a speech reception threshold of 25 decibels or less, then anywhere from 20
to 80% of patients will have a successful operation. Much of this range hinges on patient selection,
experience and technique. Crabtree, de la Cruz and Glasscock have each reported varying degrees of
success with this operation. However, this may reflect the utilization of different parameters and
definitions by each of these authors. Jahrsdoerfer reported in 1992 on his results based on patients
selected using a 10-point grading system. He reported achieving speech reception thresholds of 25
decibels or less in 75% of 86 cases operated on. Several authors have noted that cases requiring a
prosthesis for ossicular reconstruction tend to have greater air-bone gaps postoperatively than those
receiving grafts over a mobile ossicular mass.
Complications relating to CAA repair can be divided into intraoperative and postoperative categories.
Intraoperative complications include facial nerve injury, sensorineural deafness, and perilymph fistula.
Although cited as a potential complication, permanent facial paralysis is exceedingly rare despite its
aberrant course through atretic temporal bones. High frequency sensorineural loss secondary to
transmitted vibrations has been reported with both approaches. Postoperative or delayed complications
for the most part include stenosis and chronic drainage, more common with the transmastoid approach,
and graft lateralization that plagues both approaches as well. This can be prevented by the creation of a
bony ledge medial to the ossicular mass wherein the graft can be placed, or through the use of a silastic
button just lateral to the graft.
Bilateral atresia patients that are either unable or unwilling to undergo surgery have the option of being
fitted with a bone-conducting hearing appliance. Both percutaneous and transcutaneous devices have
been developed; however, only the transcutaneous version is FDA approved. Older devices that are held
to the head by a tight-fitting headband are unsightly and uncomfortable.
Despite the advances that have been made in technique and imaging over the past twenty years, several
issues regarding the timing of surgery in general, when to operate on cases of unilateral atresia, and the
operability of cases with severe craniofacial malformations remain difficult questions. The issue of when
to operate on these patients is a difficult one because many surgeons with considerable experience in this
area maintain such varied opinions. Generally speaking, most would agree that the earliest age at which
to proceed is at 4 to 5 years of age, thereby allowing time for adequate mastoid and middle ear
pneumatization and increasing patient compliance with the postoperative care that is required. Also, most
authors state that in cases of unilateral atresia with evidence of cholesteatoma, infection or with very thin
atresia plates, surgery should be undertaken earlier. However, there are differing opinions regarding
cases of grade II and III unilateral atresia in patients with normal hearing in the other ear. Jahrsdoerfer
finds that the benefit of binaural hearing far exceeds the risk of facial nerve injury and other
complications. De la Cruz also favors operating earlier on unilateral atresias if a good outcome can be
expected based on CT findings. Others, including Crabtree, Bellucci, Glasscock and Fisch, favor waiting
until the patient is of consenting age at which point an individually informed decision can be made. All
points considered, the decision to proceed with early intervention ultimately depends on the experience
of the surgeon and the relative degree of malformation that the individual patient has and with which the
surgeon is comfortable.
In summary, congenital aural atresia is a congenital developmental anomaly of the middle ear that
manifests with varying degrees of external auditory canal stenosis or atresia, ossicular derangements,
poorly developed mastoid and tympanic cavities and is characterized by a conductive hearing loss that is
of a moderate to maximal degree. Heritable forms of CAA are found in association with many of the
craniofacial syndromes, but nonheritable, isolated CAA is more frequent. History and physical exam
findings are important in the early identification of the problem complemented by audiometric/ABR
evaluation in order to allow speech development. CT scanning to assess operative potential is then
performed at about 4 years of age, with an approximate yield of 50%. Grading of the patient based on CT
findings has been shown to be predictive of outcome. Success rates of as high as 75% have been reported
with speech reception thresholds of 25dB or less. Historically, a transmastoid approach has been used,
but the problems of stenosis, drainage and long-term care of a mastoid cavity have favored the anterior
approach. Issues regarding the timing of unilateral cases are complex and the decision to operate early on
in these cases ultimately depends on the experience of the surgeon and the degree of malformation
present.
Case Presentation
A 2-year and 9-months-old male, former 36-week premature infant that at birth was noted to have
midfacial and mandibular hypoplasia consistent with the diagnosis of Treacher-Collins Syndrome. The
family history was negative for relatives with craniofacial or auricular malformations. His past medical
history was notable for uncomplicated neonatal jaundice. Medical work-up for congenital cardiac and
renal anomalies was negative. On exam, the patient is noted to have malar hypoplasia, absent zygomatic
arches, hypoplasia of the mandible with Class III occlusion, and bilateral Grade III microtia with aural
atresia. The mastoids, however, are well developed bilaterally. A tracheotomy is present in the neck. The
remainder of the physical exam was unremarkable. Upper airway obstruction secondary to severe
mandibular hypoplasia necessitated elective tracheotomy in the first week of life. A gastrostomy tube
was placed secondary to poor oral intake. At two months of age, he underwent audiometric evaluation
consisting of air and bone conduction multichannel ABR. The patient was fitted with a bone-conducting
type hearing aid at age four months.
Subsequently, the patient underwent repair of his palatal defect at age two, and most recently underwent
bilateral Ilizarov mandibular distraction procedures. After more than two years with his bone-conducting
aids, he has acquired comprehensible speech and a vocabulary comparable to that of children with
normal hearing.
Bibliography
Andrews JC, Anzai Y, Mankovich NJ, Favilli M, Lufkin RB, Jabour B. Three-dimensional CT scan reconstruction for the
assessment of congenital aural atresia. Am J Otol 1992;13:236-240.
Chang SO, Min YG, Kim CS, Koh TY. Surgical management of congenital aural atresia. Laryngoscope 1994;104:606611.
Cole, RR. The "buttock sign" in congenital aural atresia. Otolaryngol Head Neck Surg 1993;109:140-141.
Cole RR, Jahrsdoerfer RA. The risk of cholesteatoma in congenital aural stenosis. Laryngoscope 1990;100:576-578.
Cole RR, Jahrsdoerfer RA. Congenital aural atresia. Clin Plast Surg 1990;17:367-371.
Cressman WR, Pensak ML. Surgical aspects of congenital aural atresia. Otolaryngol Clin North Am 1994;27:621-633.
Fuente del Campo A, Elizondo MM, Arnaud E. Treacher Collins syndrome (mandibulofacial dysostosis). Clin Plast Surg
1994;21:613-623.
Glasscock MR, Jackson CG, Nissen AJ, Schwaber MK. Management of congenital ear malformations. Ann Otol Rhinol
Laryngol 1983;92:504-509.
Granstrom G, Bergstrom K, Tjellstrom A. The bone-anchored hearing aid and bone-anchored epithesis for congenital ear
malformations. Otolaryngol Head Neck Surg 1993;109:46-53.
Hall JW, Morgan SH, Aguilar EA, Mackey J, Jahrsdoerfer RA. Neuro-otologic applications of simultaneous multi-channel
auditory evoked response recordings. Laryngoscope 1994;94:883-889.
Hayes D. Hearing loss in infants with craniofacial anomalies. Otolaryngol Head Neck Surg 1994; 110:39-45.
Jahrsdoefer RA, Hall JW. Congenital malformations of the ear. Am J Otol 1986;7:267-269.
Jahrsdoerfer RA, Garcia ET, Yeakley JW, Jacobson JT. Surface contour three-dimensional imaging in congenital aural
atresia. Arch Otolaryngol Head Neck Surg 1993;119:95-99.
Jahrsdoerfer RA, Worth JW, Aguilar EA, Cole RR, Gray LC. Grading system for the selection of patients with congenital
aural atresia. Am J Otol 1992;13:6-12.
Jahrsdoerfer RA. Congenital atresia of the ear. Laryngoscope 1978;88:1-49.
Jones RL. Smith's Recognizable Patterns of Human Malformation, 4th ed. Philadelphia: Saunders, 1988:709.
Lambert PR. Congenital Aural Atresia. In: Bailey BJ., et al. Head and Neck Surgery - Otolaryngology. Philadelphia, .
Lippincott, 1993:1579-1591.
Mattox DE. Fisch U. Surgical correction of congenital aural atresia of the ear. Otolaryngol Head Neck Surg 1986;94:574577.
Molony TB, De la Cruz A. Surgical approaches to congenital atresia of the external auditory canal. Otolaryngol Head
Neck Surg 1990;103:991-1001.

Pattee GL. An operation to improve hearing in cases of congenital atresia of the external auditory meatus. Arch
Otolaryngol 1947;46:568-580.
Patten BM. Human Embryology. New York: McGraw-Hill, 1968:335-344.
Schuknecht HF. Congenital aural atresia. Laryngoscope 1989;99:908-917.
Shambaugh GE. Surgery of the Ear. Philadelphia: Saunders, 1959:493-523.
Shih L, Crabtree JA. Long-term surgical results for congenital aural atresia. Laryngoscope 1993; 103:1097-1102.
Swartz JD, Harnsberger HR. Imaging of the Temporal Bone. New York: Thieme Medical Publishers, 1992:20.
Portmann M, Guillen G, Richards AE. The Ear and Temporal Bone. New York: Masson, 1979:382-384.


medical literature.
CONGENITAL INNER EAR MALFORMATIONS

H. Jeff Kim, MD
November 11, 1993
The reported incidence of significant sensorineural hearing loss in young children varies from 1:1000 to
1:2000 depending on the population studies. Approximately 20% of patients with congenital
sensorineural hearing loss have radiographic anomalies of the inner ear.
The structure of the inner ear consists of a membranous labyrinth surrounded by a bony labyrinth within
the petrous temporal bone. The primordium of the membranous labyrinth, which is referred to as the otic
placodes, appears in the third week of gestation as thickened planes of surface ectoderm on either side of
the developing hindbrain. It differentiates into the otic pit and then fuses to become the otic vesicles, or
otocyst, by the fifth week. The otocyst subdivides into two pouches. The ventral portion develops into the
cochlear duct and saccule. The dorsal segment is transformed into the endolymphatic sac and duct,
utricle, and semicircular canals. By the 10th week, the adult membranous labyrinth is recognized, and
completely developed by week 26. The bony semicircular canals began at the sixth week and are
complete by week 22. The superior semicircular canals are completed first, followed by the posterior and
finally the lateral. The bony labyrinthine begins in the fourth week as a condensation of mesenchyme and
forms a cartilage capsule around the developing membranous labyrinth. Its ossification begins at week 14
and is completed by week 23. Congenital ear malformation results from a defect in the development of
the membranous labyrinthine, the osseous labyrinthine, or both.
The malformations limiting the membranous labyrinthine cannot be detected radiographically. Complete
labyrinthine dysplasia is very rare, and was first described by Bing and Siebenmann in 1907. It has been
associated with Jervell-Lange Nielson and Usher's syndromes. Cochleosaccular dysplasia was first
described by Schiebe in 1892. It is characterized by a collapse of the cochlear duct and saccule. It is
probably the most common form of inner ear pathology in patients with congenital deafness. The
Alexander's ear deformity, or the cochlear base turn dysplasia, is related to familial high frequency
sensorineural hearing loss.
Jackler has classified cochlear anomalies based on inner ear embryogenesis. The complete labyrinthine
aplasia, known as Michel's deformity, represents an early failure in development correlating to the third
week of gestation. It is extremely rare. The common cavity deformity represents developmental arrest at
the fourth week, and a common cavity of the cochlea and vestibule is formed with internal architecture.
Cochlear aplasia results from the arrested development of the cochlea during the fifth week. The cochlea
fails to form and appears as a single cavity. The vestibule and semicircular canals may be normal or
malformed. Cochlear hypoplasia displays a small, rudimentary cochlea but is associated with a normal or
malformed vestibule and semicircular canals. This lesion is due to an arrest at the sixth week of gestation.
Incomplete partition deformity, also well known as Mondini's deformity, represents a small cochlea with
incomplete or no intrascalar septa. The cochlea is usually flat and has one and one-half turns instead of
the normal two and one-half turns. Arrest of maturation at the gestational seventh week may result in the
Mondini deformity.
The internal auditory canal (IAC) may be enlarged or stenosed. The narrow IAC can be associated with a
failure of the 8th nerve development. Patients with the wide IAC may be predisposed to cerebral spinal
fluid (CSF) leaks, resulting in recurrent meningitis.
Patients with the Mondini deformity and other congenital inner ear malformations are at an increased risk
for developing recurrent meningitis or perilymphatic fistula. They are predisposed to develop a CSF leak
due to the enlarged cochlear aqueduct or an abnormal connection between the internal auditory canal and
the membranous labyrinth.
A thorough clinical, audiological and radiological evaluation should be made of all patients suspected of
having these deformities. Clinical history should include possible exposure to teratogen during
pregnancy, family history of hearing impairment, progression and/fluctuation of the hearing loss, and
associated vestibular symptom. A routine audiologic evaluation is required. Work-ups can be helpful in
determining possible etiology. These include TORCH titers, FTA-ABS, urinalysis, and thyroid function
tests. High resolution, computed tomography provides excellent visualization of the bony labyrinthine of
the inner ear.
Patients are advised to avoid contact sports because of the increased risk of CSF leak following minor
head injury. Middle ear infections are treated aggressively because of the increased risk for meningitis.
Genetic counseling is provided after a careful analysis of the family history. Hearing rehabilitation,
including amplification and especially educational efforts, are also indicated.
Currently, cochlear implantation is indicated for bilaterally profound sensorineural hearing loss without
speech discrimination using hearing aids. It is contraindicated in the patient with complete cochlear
aplasia and narrow IAC since neural elements for stimulation would be absent. Further study is required
to evaluate the long-term benefits of cochlear implantation in the congenital inner malformation.
Case Presentation
A patient was referred to Texas Children's Hospital at eight months of age with a history of
developmental delay and no perception to auditory stimuli. She was a term infant, weighing 7 lbs 11 oz
when delivered by Caesarean section due to breech presentation. Her postnatal care was uncomplicated.
The pregnancy was only complicated by a urinary tract infection and hypertension during the third
trimester. She was recently diagnosed with congenital hypotonia, but was otherwise healthy without any
previous ear problems. An ABR demonstrated no response to air or bone conduction click stimuli.
Her family history was only significant for a maternal grandmother with a hearing loss. Physical
examination was unremarkable. Titers were negative for cytomegalovirus, rubella, toxoplasma, and
herpes simples virus. Thyroid function tests were within normal limits. A high resolution temporal bone
CT scan revealed cochlear common cavity deformity.
She was fitted with bilateral behind-the-ear (BTE) hearing aids. Repeat audiogram demonstrated her
aided speech detection threshold to be 60 dB, and a 1000 Hz warble tone at 70 dB. She has been involved
in a total communication program. Her parents noted significant differences in her sound perception with
hearing aids. Currently she has been using a five-word vocabulary.
Bibliography
Brookhouser PE. Sensorineural hearing loss in children. In: Cummings CW, Harker LA, editors. Otolaryngology - Head
and Neck Surgery, 2nd ed. St. Louis: Mosby, 1993:3080-3102.
Clars P, Guirado C, Clars C, Clars A Jr, Clavera T, Wienberg P. Association of spontaneous anterior fossa CSF
rhinorrhea and congenital perilymphatic fistula in a patient with recurrent meningitis. Internatl J Pediatr Otorhinolaryngol
1993;27:65-71.
Emmett JR. The large vestibular aqueduct syndrome. Am J Otol 1985;6:387-415.
Gussen R. Middle and inner ear changes in congenital rubella. Am J Otolaryngol 1961;2:314-320
Ilium P. The Mondini type of cochlear malformation: a survey of the literature. Arch Otolaryngol 1972;96:305-311.
Jackler RK, Luxford WM, Brackmann DE, Monsell EM. Endolymphatic sac surgery in congenital malformations of the
inner ear. Laryngoscope 1988;98:698-704.
Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis.
Laryngoscope 1987;97(Suppl 40):2-14.
Jackler RK, Luxford WM, House WF. Sound detection with the cochlear implant in five ears of four children with
congenital malformations of the cochlea. Laryngoscope 1987;97(Suppl):15-17.
Johnsson L-G, Hawkins JE Jr, Rouse RC, Kingsley TC. Four variations of the Mondini inner ear malformations as seen in
microdissections. Am J Otolaryngol 1984;5:242-257.
Jorgensen MB, Kristensen HK, Buch NH. Thalidomide-induced aplasia of the inner ear. J Laryngol Otol 1964;78:10951101.
Lindsay JR. Profound childhood deafness: inner ear pathology. Ann Otol Rhinol Laryngol 1973;82(Suppl 5):1-73.
Makishima K, Snow JB Jr. Pathologic features of the inner ear in congenital deafness. Arch Otolaryngol 1975;101;600604.
Mangabeira-Alberrnaz PL. The Mondini dysplasia - from early diagnosis to cochlear implant. Acta Otolaryngol
1983;95:627-631.
Mangabeira-Albernaz PL, Fukuka Y, Chammas F, Ganana MM. The Mondini dysplasia - a clinical study. ORL J
Mitchell DP, Rubin AM. Mondini dysplasia - late complications. J Otolaryngol 1985;265-267.
Miyamoto RT, McConkey Robbins AJ, Myres WA, Pope ML. Cochlear implantation in the Mondini inner ear
malformation. Am J Otol 1986;7:258-261.
Molter DW, Pate BR Jr, McElvenn JT Jr. Cochlear implantation in the congenitally malformed ear. Otolaryngol Head Neck
Surg 1993;108:174-177.
Murakami Y, Schuknecht HF. Unusual congenital anomalies of the inner ear. Arch Otolaryngol 1968;87:335-349.
Nadol JB Jr. Histological considerations in implant patients. Arch Otolaryngol 1984;110:160-163.

Nadol JB Jr, Young Y-S, Glynn RJ. Survival of spiral ganglion cells in profound sensorineural hearing loss: implications
for cochlear implantation. Ann Otol Rhinol Laryngol 1989;98:411-416.
Ohlms LA, Edwards MS, Mason EO, Igarashi M, Alford BR, Smith RJH. Recurrent meningitis and Mondini dysplasia.
Otte J, Schuknecht HF, Kerr AG. Ganglion cell populations in normal and pathological human cochleae: implications for
cochlear implantation. Laryngoscope 1978;88:1231-1246.
Paparella MM. Mondini's deafness: a review of histopathology. Ann Otol Rhinol Laryngol 1980;89(Suppl 65):1-23.
Phelps PD. Cochlear implants for congenital deformities. J Laryngol Otol 1992;106:967-970.
Phelps PD. Congenital lesions of the inner ear, demonstrated by tomography: a retrospective study of 34 cases with special
reference to the lateral semicircular canal. Arch Otolaryngol 1974;100:11-18.
Reilly JS. Congenital perilymphatic fistula: a prospective study in infants and children. Laryngoscope 1989;99:393-397.
Sando I, Takahara T, Ogawa A. Congenital anomalies of the inner ear. Ann Otol Rhinol Laryngol 1984;93:(Suppl 112):110118.
Schmidt JM. Cochlear neuronal populations in developmental defects of the inner ear. Acta Otolaryngol 1985;99:14-20.
Schuknecht HF. Mondini dysplasia: a clinical and pathological study. Ann Otol Rhinol Laryngol 1980;89:3-23.
Shelton C, Luxford WM, Tonokawa LL, Lo WW, House WF. The narrow internal auditory canal in children: a
contraindication to cochlear implants. Otolaryngol Head Neck Surg 1989;100:227.
Suehiro S, Sando I. Congenital anomalies of the inner ear: introducing a new classification of labyrinthine anomalies. Ann
Supance JS, Bluestone CD. Perilymph fistulas in infants and children. Otolaryngol Head Neck Surg 1983;91:663-671.
Swartz JD, Harnsberger HR. Imaging of the Temporal Bone, 2nd ed. NY: Thieme, 1992;192-246.
Tom LW, Bilaniuk L, Roa RA, Potsic WP. Recurrent meningitis and a congenital perilymph fistula. Ear Nose Throat J
1992;71:287-290.
Tsuzuki T, Kaga K. The relation between motor function development and vestibular function tests in four children with
inner ear anomaly. Acta Otolaryngol 1991;481:443-446.
Wetmore SJ, Herrmann P, Fisch U. Spontaneous cerebrospinal fluid otorrhea. Am J Otol 1987;8:96-102.
Zalzal GH, Shott SR, Towbin R, Cotton RT. Value of CT scan in the diagnosis of temporal diseases in children.

From the Grand Rounds Archive at Baylor: Allergic Rhinitis

The information contained within the Grand Rounds Archive is intended for
use by doctors and other health care professionals. These documents were
prepared by resident physicians for presentation and discussion at a
conference held at The Baylor College of Medicine in Houston, Texas. No
guarantees are made with respect to accuracy or timeliness of this material.
This material should not be used as a basis for treatment decisions,
and is not a substitute for professional consultation and/or peerreviewed medical literature.
Diving Medicine
Brian H. Weeks, M.D.
January 13, 2000
There are about 3.4 million certified scuba divers in the United States. And
about 300,000 become certified per year. About one-third of all divers
experience some diving related medical problem in their careers. The
important thing to remember is that even in healthy divers with safe diving
profiles, there is a 1% to 5% incidence of decompression sickness, which
can be an extremely serious problem.
The most common diving related injury involves middle ear barotrauma.
The incidence of vestibular symptoms reaches about 20% among divers in
some studies. Most importantly for this talk, as an otolaryngologist we are
considered the diving experts. So it is quite probable that sometime in our
career we will all see a patient who is a scuba diver.
In literature there are many referrals to breath-hold divers used by kings to
recover treasures for their wealth and fame. In the 17th century an open
diving bell was documented, which was an air-holding chamber that
prolonged possible diving times. In 1810 a copper helmet was designed,
which was sealed to a watertight canvas. This allowed air to be pumped
down from the surface. Today we have a modification of this original design
for very deep hard-hat diving.
In early 1940 Jacques Cousteu designed what was then referred to as the
aqua-lung. Today it is known as the scuba device. Scuba is an acronym for
self-contained underwater breathing apparatus. These were actually initially
used in World War II, but because of their relatively low cost and their
reliability, the apparatus was made available to the everyday diver and for
recreational purposes.
For those unfamiliar with scuba diving, the basic set up involves a tank,
which the diver wears on his back, that delivers pressurized air via a hose
and a mouthpiece, which is known as a regulator. The pressure is
comparable to the aquatic depth that the diver obtains. A demand regulator
either at the tank or at the mouthpiece controls the differential on the
pressure from the tank air to the ambient pressure. The duration of the dive
is limited to the amount of air in the tank as well as to the physiology of the
diver.
The weight of the air at sea level is one atmosphere, so atmospheric
pressure is sea level, which is described as one square inch of air for as far
as the atmosphere extends. Atmospheric pressure is also recorded as 14.7
lb. per square inch or 760 tore. One atmosphere under water is 33 feet of
seawater, which is the equivalent of 34 feet of fresh water. So, there is onefoot difference in the amount of an atmospheric pressure. Probably the
most important concept to understand is that when a body is submerged in
water, it not only experiences the pressure from the water, but it also
experiences the pressure from the air, which is called absolute pressure atmospheric plus water pressure.
As divers, we refer to the gauge pressure, which is really the pressure that
is important to us and is the pressure that is exerted by the water alone.

Just for comparison, 18,000 feet into the atmosphere in an airplane is the
equivalent of a half of atmosphere. This gives a perspective as to what we
are looking at from a diving standpoint. As you descend to deeper depths,
33 feet, 66 feet, your absolute pressure is the air pressure plus the water
pressure.
Basic laws govern scuba diving. Probably the most important law to us as
otolaryngologists and divers is Boyle's Law. This law says that at constant
temperature, volume and pressure are inversely related. Since we deal with
air-filled cavities, this is clearly the most common law relating to us as
otolaryngologists. Remember that soft tissue is mostly made of water and is
relatively incompressible compared to air-filled spaces. However the
sinuses and the middle ears are all affected by pressure changes.
Again, a scuba diver breathes air through his tank, through his glottis, which
is delivered to him at one atmosphere pressure. As the diver descends
deeper into the ocean, the pressure increases but the lung volume remains
constant. However, even at deeper depths, inspiration becomes more
difficult because as the density of the molecules changes, it becomes more
difficult to breathe the air. As the diver descends, the pressure increases in
the lungs because depth pressure increases. However, the volume stays
the same. The volume is constant, the density changes and the pressure
changes. The fact that the lung volume stays the same is what protects the
lungs while we are diving.
Breath-hold diving or free diving is a very common sport especially in
Hawaii and along the West Coast. Even snorkeling involves diving using
only mask, fins, and a snorkel. Most of these divers are limited to 1-2
atmospheres of gauge pressure in their depth. Again the thing to remember
with these divers is that their lung volumes will be changing because they
are not using a scuba apparatus to equalize their pressures. At one
atmosphere, the lung volume goes down by and at two atmospheres
their lung volume is reduced by 1/3 again. Those are important concepts to
remember.
The second law is Dalton's Law, which basically says that in a gas mixture,
pressure exerted by each gas is the same as it would exert if it alone
occupied the same volume. So, as you increase the total pressure, the
relative contributions of each gas in the mixture remain the same. If oxygen
is one-third of the mixture at sea level, even if you decrease or descend to
three atmospheres pressure, it still would have an equal contribution. The
contribution of oxygen triples to the total mixture. This is extremely
important with the biological effects of gas. The partial pressures of each
individual gas in the tank determine the tissue diffusion and the amount of
gas that is ultimately dissolved in the body tissues and in the blood stream.
Remember that oxygen delivery depends on PO2 and not on total gas
pressure. A good example to illustrate this is that at sea level the PO2 is 16
tore, which is 21% of 760 tore. At 132 feet of seawater, which is four
atmospheres, the PO2 is equivalent to breathing 100% air at sea level. This
shows that even though it has the same amount of concentration at that
depth and the relative contribution is the same, because the pressure is so
much greater, the effects are quite different. This can lead to oxygen
toxicity, because breathing the equivalent of 100% oxygen is very difficult
and very damaging to the body over long periods of time.
The last law of Physics to consider is Henry's Law, which basically says
that given temperature, the mass of the gases dissolved, and the given
volume of solvent is proportional to the pressure of the gas with which it is
at equilibrium. Basically, this law dictates how much of a gas is absorbed at
a given pressure. This is important when we discuss things like nitrogen
narcosis and decompression sickness. Again, this is most important when
applied with nitrogen solubility in the body tissues during descent and then
the relative insolubility when ascent is performed.
Nitrogen narcosis is often called The Rapture of the Deep." And was first
described by Captain Albert Binky, a seaman. He learned that nitrogen
under pressure acts as an anesthetic. At greater depths, there is a greater
force driving nitrogen into the tissues. This is an application of Henry's Law.
Divers themselves refer to another law: Martini's Law. This law states for
every 50 feet of descent, a diver breathing tank air experiences the
equivalent of drinking one martini on an empty stomach. At dives of 100
feet of seawater, or even down to close to 150 feet of seawater, the effect
can be like drinking three mixed drinks with no food in your stomach. This
can become extremely dangerous especially when the diver needs to make
quick decisions and needs to determine exactly what his dive bottom times
should be. Commercial divers who dive much deeper than recreational
divers, often substitute helium in their gas mixtures for nitrogen in order to
limit the anesthetic effect and to allow them to dive deeper without
intoxicating effects.
Ears, sinuses, and the neighboring structures are all susceptible to injury
during diving. The ear and the Eustachian tube are considered to be the
weak links in our body's ability to tolerate diving situations. The
nasopharyngeal opening of the Eustachian tube is usually closed. As we all
know, it opens with positive nasopharyngeal pressure or by contracture of a
number of muscles: tensor vallee palantini, lavatar palantini, and the palato
pharyngeus muscles contract and open the nasopharyngeal orifice of the
Eustachian tubes.
In 1937 Armstrong and his colleagues did a very interesting study of
something that is probably obvious to us now. Upon descent with divers he
found that the Eustachian tube acts as a flutter valve and actually is closed
and only opens if the diver equalizes. The Eustachian tube will not open on
its own under these pressures to equalize middle ear pressure. It has to be
reflexive or voluntary, an act made by the diver himself to ensure that his
middle ear pressure is equalized throughout his dive. If pressure is not
equalized, the diver experiences what is commonly called a middle ear
squeeze, which refers to barotrauma of the middle ear. A Valsalva, which
involves holding the nose, closing the mouth and exhaling against a closed
glottis, is one of the techniques used to re-inflate or auto-inflate the middle
ear and equalize the pressures. If divers have upper respiratory infections,
allergies, nasal polyposis or septal deviation, they increase their risk of
middle ear squeeze because of their inability to equalize their pressures.
The most common or simple problem that can occur is occlusion of the
EAC, which can be related to cerumen, ear plugs, or a hood used in cold
water diving. The diver experiences pain during descent because of the
pressure build-up due to the occlusion. On examination of these patients,
we see congestion of the external auditory canal skin and often of the TM
as well, with edema in the canal. There may be rupture of the skin and even
a tympanic membrane perforation from this problem, which will cause
hemorrhage and severe, severe pain. Treatment is conservative: stay out of
the water and do not scuba dive until the area is completely healed.
Probably the most common problem involving the EAC with scuba divers is
otitis externa. This is often due to the water exposure or divers drying their
ears excessively with cotton after a dive. It is a fairly simple problem, and
for a mild case, the patient should use acidified alcohol solution after dives,
often called Swim Ear. There are also silicone oil sprays that divers can
apply to the EAC skin before dives, providing a protective coat.
The most common problem in the middle ear is barotrauma, also known as
aerotitis media or middle ear squeeze. When scuba divers make their
descent, the most critical time is within the first atmosphere. It is most
important that pressure is equalized properly. If pressures are not
equalized, the diver will experience severe otalgia. This can lead to
subsequent tympanic membrane rupture. This can actually occur at even
just a few feet of water and in pressure differences as low as 5 pounds per
square inch. With a tympanic membrane rupture, there is often otorrhea

and the diver frequently experiences dizziness and some form of a mild
hearing loss. At surface pressure all pressures are equalized. As a diver
descends, the ambient pressure increases and the pressure in the middle
ear should be equalized through the Eustachian tubes. However, if there is
a block in the Eustachian tubes, the diver can no longer equalize his
pressures. If he continues to submerge or to descend, this pressure will
increase and eventually the weak link in the system will give, which means
that the tympanic membrane will likely rupture. If the diver chooses to return
to the surface he can do so. However, he will continue to experience the
problem and the situation is often even worsened because small amounts
of oxygen are actually absorbed through the middle ear, further increasing
the negative pressure differential in the middle ear and causing further
pressure. Mucosa edema of the middle ear occurs and there is hemorrhage
into the small capillaries. Treatment involves the use of systemic
decongestants and mucolytic agents plus or minus an antihistamine for
patients experiencing allergy. The otolaryngologist should also instruct the
diver to perform periodic auto-inflation if he is not having pain without
movement. If continued pain occurs, it usually indicates a middle ear
infection. At that time, a decision needs to be made whether the patient
would benefit from a myringotomy or PE tube and certainly antibiotics. With
a tympanic membrane perforation, the original treatment is the same as
always: with oral hygiene and ototopical agents, these usually close nonsurgically. However, tympanoplasty is indicated for persistent perforation.
Divers should be encouraged to concentrate on prevention of these injuries.
A nasal decongestant before a dive can help prevent this sort of problem.
When these medicines are applied to the nose, the position of the head is
very important. The diver should actually have his head down so that the
medicine can reach the Eeustachian tube ostium more easily. Some divers
use systemic decongestants, such as Sudafed, and often add an
antihistamine for allergy. It is very important to remember that these should
be non-sedating medicines and that no new medicines should be given
before a dive unless that have already been tried on the surface.
And, as we have all seen before, Afrin can even have rebound hypercongestion, which can lead to nasal obstruction. Because this is a delayed
phenomenon, it can happen at depths of maybe 60 or 70 feet below the
surface in the middle of the dive. This can lead to what is known as a
reverse squeeze. That is expansion of the middle ear air, which can lead to
TM distention and perforation, but through the opposite direction - an
increase in the middle ear pressure.
Some divers begin Valsalva and auto-inflation in the middle ear before they
even begin their dive. With a very forceful Valsalva, a diver can experience
barotrauma. This is related to an increased intrathoracic and abdominal
pressure, which ultimately increases the CSF pressure via engorged spinal
veins, which can be transmitted to the endolymph, the cochlea, perilymph,
and eventually to the round window and oval window membranes. This
leads to tinnitus and usually a unilateral high frequency hearing loss.
Another preventative measure is the Frenzel maneuver, which involves
holding the nose, closing the glottis and contracting the pharyngeal
muscles. This also forces air into the Eustachian tubes. Some people are
able to perform this to equalize pressure and don't have to use a Valsalva
technique. Other preventive measures include using a feet-first descent.
Multiple studies have shown that it is easier to inflate the middle ear with
the head up, as this causes less hyperemia of the Eustachian tubes and
mucosa. The diver is also less distracted with his head up, and it may help
him to remember to continuously auto-inflate his middle ear every 2-3 feet
as he makes his descent. An anchor line on the boat should also be used to
verify exactly how deep the diver is. If a diver experiences middle ear
squeeze, he should ascend a few feet and attempt to auto-inflate, and then
begin the redescent. It is also important to have a form-fitting mask, so that
nasal compression is easier to perform. Jaw and head movements from
side to side are often used to facilitate middle ear inflation.
One of the more uncommon problems seen in divers is what is known as
ultinebaric facial paralysis. This is extremely uncommon. This is due to
dehiscent portions of the facial nerve, which are vulnerable to barometric
trauma. It is usually seen in normal people who descend during a dive, but
when they ascend and get to the surface, they have a facial paralysis. This
is almost always a transient paralysis and is related to barotrauma. These
divers almost always resume normal function of the facial nerve over time.
Inner ear problems in divers are usually related to middle ear barotrauma.
As we have discussed, middle ear negative pressure causes a depression
of the tympanic membrane. This can be transmitted to the ossicle of the
stapes and cause significant and rapid foot plate depression, which can
tear the sensitive inner ear membranes and lead to the types of hearing
losses we have discussed. The second thing that can occur in the inner ear
is sudden pressure equalization, which can cause a rush of air up the
Eustachian tube, putting an outward force on the tympanic membranes and
the ossicles and pulling the stapes footplate up. This can cause a pressure
wave in the inner ear and distort the inner ear membranes. All these
membranes are extremely fragile. There are multiple theories as to how the
trauma occurs. But it is thought that either Ricener's membrane or the
basilar membranes are involved or the vestibule and semi-circular canals

are involved. The shearing force of this rapid fluid movement is transmitted
to the labyrinth and cochlea and can cause tears in the membranes and
hemorrhage from the small torn vessels.
Round window implosion is a well-described problem in the literature from
inner ear trauma during diving. This involves a pull on the stapes footplate
and a shock wave through the scala vestibuli to the scala tympani. It
causes a bulging inward from a rapid pull on the stapes footplate and it
causes an inward pull on the round window membrane, which causes an
implosion injury and the leakage of perilymph into the middle ear. A sudden
pull on the stapes footplate can also lead to a tear of the annular ligament
of the oval window, leading to a crack in the footplate and also leakage.
Frequently when divers are having a difficult time auto-inflating, their first
inclination is to try harder to equalize the pressure. This can lead to the
elevation of the CSF pressure that can be transmitted directly through a
patent cochlear aqueduct into the internal meatus, raising intracochlear
pressure to dangerous levels. The difference between the middle ear and
the perilymph space can cause an explosive outward rupture of the round
window membranes. It is the opposite of the implosion injury, an outward
injury, which can cause perilymph leakage and a perilymph fistula.
Dr. Healy and his colleagues did a study that looked at 40 cases of
perilymph fistula in divers. The patients' chief complaints with this problem
were episodic positional vertigo. They did not have as many complaints of
hearing loss or tinnitus. These patients all underwent surgical exploration.
At exploration, 31 out of 40 of these patients (75% of them) had oval
window leaks, 5 of them had round window leaks, and 4 of them had both
oval window and round window leaks. None of these patients had a hearing
loss for longer than 3 weeks. They regained any significant hearing in those
ears. If the suspicion is high for a perilymph fistula, early exploration leads
to the best hearing restoration. Healy felt that the most important factors
during these procedures were proper magnification, and patience. He
commented that after blotting in the middle ear, it often took 5 to 10 minutes
for perilymph to build back up or for a leak to be revisualized. In two
patients he had to perform bilateral jugular vein compression to enhance
the perilymph leakage.
To summarize, hearing loss and dizziness are indicative of inner ear
barotrauma and require immediate treatment. Depending on the etiology, or
presumed etiology, treatment involves strict bedrest and convalescence, no
coughing, no sneezing, auto inflating or straining. These patients all
deserve an audiogram to determine whether this is a middle ear or an inner
ear problem. With inner ear problems with a cochlear etiology, you will see
a non-progressive centrally neural hearing loss. And, as I said, with a
perilymph fistula one way to distinguish it is that there is often a progressive
and fluctuating hearing loss. In the study by Healy, he also found that
posturography with EAC pressure changes have a 97% sensitivity for
diagnosing a perilymph fistula.
Antonelli and his colleagues did a study looking at 18 temporal bones
examined from 11 divers who died from complications during diving, either
from rapid ascent or drowning. He found that bleeding into the middle ear
and mastoid air cells was nearly universal in all these divers. The most
common damage seen was bleeding around Ricener's membrane and the
round window membrane. He also found that a number of these divers,
usually the ones who had rapid ascent injuries, had rupture of both the
utricle and saccule and that tympanic membrane rupture from rapid ascent
was far more common in people who dive than had been presumed. The
other interesting point was that most of the inner ear damage was not
surgically treatable. Other than for the perilymph fistula, most of these
injuries do not have surgical treatments.
Lastly, Zannini and his colleagues followed 160 professional divers with
serial audiograms. He found that these 160 divers had significantly worse
hearing than control divers. The greatest loss was in the group with the
longest diving times. He found that their hearing losses were in the highest
frequencies, usually in the 8 kilohertz. Divers who reported difficulty auto
inflating their middle ears had even worse hearing or the most central
hearing loss. Zannini also commented that perilymph fistula demands early
surgical exploration and that inner ear barotrauma is managed medically
with vasodilators, steroids, histamines, and Carbogen.
There is a different set of problems that divers can experience during
descent which involve the sinuses. This is called aerosinusitis or sinus
squeeze and involves osteomuculuson and pressure equalization. It is
really infrequently an issue in the sinuses but if you have a demitasse
mucosa with an upper respiratory infection, an allergy, a polyp occluding
the ostium or a septal deviation, this can cause a problem and mimic what
happens in the middle ear. You get a negative pressure in the sinuses
because of the inability for the pressures to equalize. This causes a
vacuum, which can lead to mucosal edema, hemorrhage and severe pain.
Management includes sinus irrigation and either endoscopic sinus surgery,
if it is a polyp, or septoplasty to straighten the septum.
Tooth squeeze or aerodontalgia is a very uncommon problem. The most
common cause of tooth pain in the diver is from aerosinusitis. But

aerodontalgia can be caused by an air pocket underneath the tooth cap or
filling. It causes severe pain when the diver descends and the pressure
changes. To treat this condition, dental work should be done and the filling
or cap should be replaced.
Facemask squeeze is failure to equalize pressure changes in the facemask.
It can lead to tissue damage from the pressure differential in the mask
along the soft tissues. The most common areas of injury are around the
eyeball and the lining of the eyelids where the mucosa is quite sensitive.
Exhaling through the nose and into the facemask and equalizing the
pressure that way easily prevents facemask squeeze.
Epistaxis is bleeding from the nose into the mask underwater. It is a very
common problem. The bleeding is due to pressure changes on
Keisselbach's plexus. Oftentimes the diver will not even recognize that he
has had a nosebleed. As you move underwater, the selective absorption of
color by the blue green seawater makes blood appear blue green. So
oftentimes the diver will ascend to the surface and then realize that his
mask is full of blood. There is no treatment required. Almost all of these
invariably resolve within minutes of reaching the surface just through
external compression on the nose.
Alternobaric vertigo is another problem seen in divers. This is transient
vestibular dyfunction due to dysequilibrium of pressures in the two middle
ears. It can lead to vertiginous feeling and has been seen in up to 15% to
16% of divers in some series. When you are underwater, any sort of
incoordination or change in equilibrium can be extremely dangerous. These
patients usually have poor auto-inflation and don't usually have any hearing
loss or tinnitus. To correct this problem, the diver should reverse the
direction of movement, allowing the vestibular system to equilibrate.
Another way to correct this involves auto-inflating the middle ear to equalize
the pressures.
Finally, unequal caloric stimulation vertigo is a problem seen when cold
water enters only one ear (due to either an external canal obstruction or a
hood). It is like a caloric stimulation test. The best way to treat this is by
removing the obstruction so that both of the ears are sensing the cold water
at the same time.
Anxiety is the most common cause of dizziness in the diver. It is actually not
due to the vestibular system. There are multiple studies that say that
greater than 50% of all scuba deaths are due to panic or anxiety in the
diver. Treatment would include educating yourself properly before you
scuba dive. Go through proper certification and never dive alone.
TMJ syndrome is caused by forcefully biting down on the regulator. This is
frequently seen in novice divers who become anxious and excited and then
bite down. It is treated just like any other treatment of TMJ, with
reassurance, heat, NSAIDS and a soft diet
There are other problems that divers may encounter that are associated
with ascent. These are pulmonary rather than pressure accidents and these
are very serious problems. These are all caused by holding of the breath
during ascent and can be predisposed in patients with obstructive lung
disease. At the surface, the pressures are all equal. As we descend, the
ambient pressure increases and the lung pressure increases as well. As we
are breathing, the pressures all stay equal because of our scuba apparatus.
However, if we don't breath during the ascent, the pressure in the lungs
stays high and the pressure on the outside stays low. The lung pressures
are higher than ambient pressure and this pressure has to go somewhere.
The first one is mediastinal emphysema, which is caused by local air

trapping with escaping alveolar air dissecting along the bronchi into the
mediastinum. This can lead to neck and chest discomfort, and shortness of
breath. We have all seen what subcutaneous emphysema looks like in the
soft tissues of the neck. The treatment for this is 100% oxygen, expectant
management and, if the airway becomes an issue, that needs to be treated
emergently.
The second thing is pneumothorax, which is caused by air from alveoli
rupturing into the pleural cavity. It is sudden and rapid and causes
shortness of breath and labored breathing and can actually be lifethreatening underwater. The treatment is a chest tube. On a boat, or out in
the water when a chest tube is not available, a large bore needle with a
condom or a piece of plastic can substitute as a one way air valve to
ventilate the air pressures. These patients need to be put on 100% oxygen
immediately and, for this type of pneumothorax, patients should undergo
chamber recompression.
The most serious problem that occurs in diving is an air embolism, a
devastating problem that can lead to neurologic complications. This occurs
when alveoli and pulmonary blood vessels rupture and air bubbles get to
the blood stream and then track to the body. If these end up in the cerebral
circulation they can coalesce to form larger bubbles and actually entirely
block the cerebral circulation. These divers usually experience loss of
consciousness after surfacing. Frequently, while still in the water, they have
frothy, bloody sputum and complain of chest pain, confusion, and blurry
vision. These patients need to be transferred immediately to recompression
chambers and frequently need treatment in these chambers intermittently
for up to 2 weeks. If you are ever with a diver and suspect air embolism, the
diver needs to be transported in the left lateral decubitus position. Air rises,
so we want them in the left lateral decubitus to prevent a bubble from
blocking the left ventricular outflow track. They also need 100% oxygen.
Another issue is air evacuation, since flying in an airplane or at elevation
can cause an increased bubble formation in the body. However, if the issue
is getting the patient to a recompression chamber as quickly as possible,
then the risk is usually warranted, as opposed to taking them by ground.
Not holding the breath, and just breathing normally as the ascent is made
will prevent air embolism. Also, make sure that patients with obstructive
lung disease do not scuba dive.
Case Presentation
The patient is a 25-year-old male with extensive SCUBA diving experience
who was on a dive with friends. The patient was nearing the end of his dive
and noted his gauge air pressure to be at 500 psi. He began to surface
when, at 50 fsw depth, he begun to breathe 'heavy air', indicating a lack of
adequate remaining air in his scuba tank. He emergently descended to his
dive buddy at 80 fsw depth and began to share his companion's air. During
his emergent re-descent, he noted severe otalgia while failing to equalize
his middle ear pressure. Upon reaching the surface, the patient noted leftsided bloody otorrhea and rhinorrhea. He also experienced mild dizziness.
On subsequent otolaryngologic exam, the patient had a 25% left tympanic
membrane perforation. His dizziness had completely resolved. He was
treated with ototopic agents and aural hygiene, and had complete closure
of his perforation on 1-month follow-up.
Bibliography
Antonelli PJ, Adamczyk M, Appleton CM, Parell GJ. Inner ear barotrauma after
stapedectomy in the guinea pig. Laryngoscope 1999;109:1991-1995.
Antonelli PJ, Parell GJ, Becker GD, Paparella MM. Temporal bone pathology in scuba
diving deaths. Otolaryngol Head Neck Surg 1993;109:514-521.
Arthur DC, Margulies RA. A short course in diving medicine. Ann Emerg Med
1987;16:689-701.
Becker GD, Parell GJ. Medical examination of the sport scuba diver. Otolaryngol Head
Neck Surg 1983;91:246-250.
Becker GD, Parell GJ. Otolaryngologic aspects of scuba diving. Otolaryngol Head Neck
Surg 1979;87:569-571.
Camporesi EM. Diving and pregnancy. Semin Perinatol 1996;20:292-302.
Crockett AT, Pauley SM, Zehl DN, Pilmanis AA, Cockett WS. Pathology of the bends
and decompression sickness. Arch Surg 1979;114:296-301.
Edge CJ, Grieve AP, Gibbons N, O'Sullivan F, Bryson P. Control of blood glucose in a
group of diabetic scuba divers. Undersea Hyperb Med 1997;24:201-207.
Eichel BS, Landes BS. Sensorineural hearing loss caused by skin diving. Arch
Otolaryngol 1970;92:128-131.
Fields JA. Skin diving: its physiological and otolaryngological aspects. Arch Otolaryngol
1958;86:531-541.
Harrill WC, Jenkins HA, Coker NC. Barotrauma after stapes surgery: a survey of
recommended restrictions and clinical experiences. Am J Otol 1996;17:835-845.
Miller JM, Axelsson A, Potter W. Chronic effects of phasic middle ear pressure changes.
Parell GJ, Becker GD. Conservative management of inner ear barotrauma resulting from
scuba diving. Otolaryngol Head Neck Surg 1985;93:393-397.
Reuter SH. "No decompression" dive tables explained. Undersea J 1971;5:6-9.
Reuter SH. Dive table rearrangement and simplification. Third International Conference
on Underwater Education. Nation Association of Underwater Instructors, Dallas, TX,
September 1971.
Reuter SH. Medical management of near-drowning victims. Physician's Newsletter, St.
Joseph Hospital. August 1982.
Reuter SH. Ninety-five percent of divers' ear problems start in the Eustachian tube. Clin
Trends Ophthalmol Allergy. 1971;10:8.
Reuter SH. Scuba diving and respiratory problems. McGovern Allery Clinic Newsletter.
Summer 1988.
Reuter SH. Three means of preventing barotrauma of the ear in scuba divers. Med
Tribune Med New 1971;12:23.
Reuter SH. Underwater medicine: otolaryngologic considerations of the skin and scuba
diver. In: Paparella MM, editor. Otolaryngology, 3rd ed. Philadelphia: Saunders; 1991.
Pp. 3231-3257.
Simmons FB. Theory of membrane breaks in the sudden hearing loss. Arch Otolaryngol
1968;99:41-48.
Strauss RH, ed. Diving Medicine. New York: Grune & Stratton; 1976.
Taylor GD. The otolaryngologic aspects of skin and scuba diving. Laryngoscope
1959;69:829-858.
Tirpitz D, Schipke JD. Delayed recompression after SCUBA diving-induced barotrauma:
a case report. Aviat Space Environ Med 1996;67:266-267.
Wilson HL. Otolaryngologic complications of skin and scuba diving. Their causes and
treatment. Trans Pac Coast Otoophthalmol Soc Annu Meet 1974;55:61-76.
Wolf SL, Twarog F, Weiler JM, Barron RJ, Lang DM, Wells JH, et al. Discussion of risk
of scuba diving in individuals with allergic and respiratory diseases: SCUBA
Subcommittee. J Allergy Clin Immunol 1995;96:871-873.
Zannini D, Odalglia G, Sperati G. Audiographic changes in professional divers.
Proceedings of the Fifth Symposium on Underwater Physiology. Freeport, British
Bahamas;August 1972.




Eustachian Tube Function and Dysfunction

July 11, 1996
Ronald B. Kuppersmith, M.D.
The eustachian tube is an 3-4 cm tubular structure which links two of the major
areas of interest in our specialty, the nose and the ear. Dysfunction of the
eustachian tube causes many common symptoms that present to our clinics, and
may have important management implications. This presentation will consists of a
review of the history, anatomy and physiology of the eustachian tube, and the role
of the eustachian tube in clinical situations.
History
The first description of the eustachian tube is attributed to Alcmaceon of Sparta in
400 BC. It was his belief that the eustachian tube allowed goats to breath through
their ears as well as their noses.
In 1562, Bartolomeus Eustachius, the Chair of Anatomy in Rome, published the
first detailed description in his thesis Epistola de Auditus organis, accurately
describing the structure, course, and relations eustachian tube.
Antonio Valsalva, Professor of Anatomy at Bologna, applied the name "Eustachian
Tube" to the pharyngotympanic tube, which was described by Eustachius.
In 1724, around the time of Valsalva, Edme-Gilles Guyot, a postmaster at
Versailles, described the technique of eustachian tube catheterization. He
succeeded in relieving his own deafness by passing a curved pewter tube through
his mouth into the opening.
Since that time many noted otolaryngologists, including Drs. Politzer, Bezold, and
Bluestone, have contributed significantly to our understanding of the intricacies of
eustachian tube anatomy and function, and management of the various disorders
that it is embroiled in.
Development and Anatomy
Understanding the development and anatomy of the eustachian tube provides
insight into its role in several pathologic processes.The eustachian tube develops as
a persistence of the first pharyngeal pouch. At 10 weeks post conception only the
epithelial lining of the lumen has differentiated. Between the 10th and 12th weeks
post conception the levator veli palatini and tensor veli palatini muscles develop.
At 14 weeks the tensor tympani muscle becomes apparent, cartilage differentiation
begins and rugae begin to develop within the tube.
The tube increases in length from 1 mm at 10 weeks post conception to 13 mm at
birth. Also, the angle between the eustachian tube and the skull base is 10 degrees
at birth.
This is in contrast to the adult length of 35 mm and angle of 45 degrees in adults.
Vertical development of the skull, and increases in the angle of the skull base,
allow the eustachian tube to reach its adult length and angle by age 7.
Basic Anatomy
In the adult, the eustachian tube can be visualized as two truncated cones attached
at their narrowed ends. It runs from the middle ear to the nasopharynx and is
approximately 31-38 mm in length. Its lumen is roughly triangular and has average
diameter of 2-3 mm. The lumen is lined by ciliated psuedostratified, columnar
epithelium, which sweeps material from the middle ear to the nasopharynx.
Mucous glands predominate near the pharyngeal orifice, and there is a gradual
change to a mixture of goblet, columnar, and ciliated cells as the middle ear is
approached.
The eustachian tube is composed of an osseous and a cartilaginous portion. The
osseous eustachian tube or protympanum measures 11 to 14 mm and extends from
the anterior and medial portion of the petrous temporal bone. Its orifice is oval
shaped, measures 5 x 2 mm and is located above the floor of the middle ear space.
When healthly, the osseous portion of the eustachian tube is always patent. The
cartilaginous portion measures 20-25 mm and opens into the nasopharynx
approximately 10 mm above the plane of the hard palate. The cartilage protrudes
into the nasopharynx, and the protruding portion is known as the torus tubarius.
The fossa of Rosenmuller is this area in the nasopharynx superior to the torus
tubaris.
The cartilaginous portion is composed of one main piece of cartilage and can be
accompanied by several accessory cartilages. Its composition and elasticity is
similar to that found in the pinna and nose. It is attached at the sphenoid sulcus on
the base of skull superiorly and its anteriomedial end is attached to a small tubercle
on the posterior edge of the medial pterygoid plate.
Blood Supply and Innervation
The blood supply to the eustachian tube is from branches of the internal maxillary
artery, ascending pharyngeal artery, and the ascending palatine artery. Sensory and
motor innervation of the eustachian tube is provided by a branch from the otic
ganglion, sphenopalatine nerve, and the pharyngeal plexus, predominately through
branches of the glossopharyngeal nerve. Sympathetic branches reach the eustachian
tube from the sphenopalatine ganglion, otic ganglion, glossopharyngeal nerve,
petrosal nerves, and the carticotympanic nerve. Parasympathetic innervation is
from the tympanic branch of the glossopharyngeal nerve. The multiple nerves
innervating the eustachian tube, may be a source for referred pain to other anatomic
regions of the head and neck.
Muscles of the Eustachian Tube
There are four muscles associated with the eustachian tube. These include the
tensor veli palatini, levator veli palatini, salpingopharyngeus, and the tensor
tympani.
The tensor veli palatini is composed of two distinct bundles of muscle fibers
mediolateral to the tube. The lateral bundle takes its origin from the scaphoid fossa,
and the lateral osseous ridge of the sulcus tubae for the course of the eustachian
tube. It descends anteriorly and lateral and inferiorly, to converge in a tendon
which passes around the hamulus and inserts on the posterior border of the
horizontal process of the palatine bone and into the palatine aponeurosis of the
velum.
The medial most portion of the tensor veli palatini originates on the lateral
membranous wall of the eustachian tube and blends with the lateral bundle of the
tensor veli palatini. This medial portion of the tensor veli palatini, referred to as the
dilator tubae muscle, is probably responsible for active dilation of the eustachian
tube by inferolateral displacement of the membranous wall.
The levator veli palatini arises from the inferior aspect of the petrous apex, passes
inferomedially, paralleling the tubal cartilage, and attaches to the dorsal surface of
the soft palate. It is thought to assist in active dilation and provide support.
The salpingopharyngeus arises from the medial and inferior portion of the
eustachian tube and descends posterior and inferior to blend with the
palatopharyngeus muscle. Its physiologic function is undefined.
The tensor tympani muscle arises from fibers common to the tensor veli palatini.
The tendon of the tensor tympani rounds the cochleaform process and inserts into
the manubrium of the malleus. It is not thought to play a role in eustachian tube
function.
Normal Function
The normal eustachian tube is functionally collapsed at rest, with slight negative
pressure present in the middle ear. It opens during swallowing, sneezing, and
yawning. The eustachian tube is thought to close through passive reapproximation
of the tubal walls by extrinsic forces and recoil of the elastic fibers.
The eustachian tube has three functions: ventilation, drainage, and protection.
When the eustachian tube is patent it allows ventilation of the middle ear and
equalization of middle ear and atmospheric pressure. It also allows the middle ear
to clear unwanted secretions. By staying physiologically obstructed, it protects the
middle ear from nasopharyngeal secretions and sound.
Conditions interfering with normal eustachian tube function cover the pathologic
spectrum from benign to malignant. Resultant middle ear complications can be the
primary condition that the clinician needs to address, may be a sign of something
more serious, or may have implications that will affect the outcome of surgical
interventions.
Eustachian Tube Dysfunction
Bluestone has classified eustachian tube disorders into obstructive disorders, and
disorders of abnormal patency.
Obstructive disorders can be mechanical or functional. Mechanical obstruction can
be intrinsic due to intraluminal factors such as mucosal inflammation due to allergy
or infection, or extrinsic obstruction resulting in compromise of the lumen.
Extrinsic obstruction can be physiologic such as when the patient is supine, or may
be caused by a mass lesion such as a neoplasm or an adenoidal mass.
Functional obstruction results from persistent collapse of the eustachian tube due to
increased tubal compliance, an abnormal opening mechanism, or both. Functional
obstruction is more common in infants and young children, and in many cases can
be related to normal or abnormal developmental factors.
Evaluating Eustachian Tube Function
There are many methods for evaluating the condition of the eustachian tube, which
reflect its deep location and complex physiology.
IN THE CLINIC
During the physical examination, otoscopy, pneumatic otoscopy, indirect
nasopharyngoscopy, and endoscopy of the nasopharynx can provide clues to the
condition of the eustachian tube.
Several maneuvers can be easily performed in clinic that may indicate patency of
the eustachian tube. These include the Valsalva test, the Toynbee test, the Politzer
test, and eustachian tube catheterization.
The Valsalva test is performed by visual inspection of the tympanic membrane
while the eustachian tube and middle ear are inflated by a forced expiration with
the mouth closed and the nose occluded by the thumb and forefinger. The test is
positive when an intact tympanic membrane is observed moving, or by air heard
through a perforated TM. A positive valsalva test only indicates an anatomically
patent and probably distensible eustachian tube.
The Politzer test is performed by visual inspection of the tympanic membrane

while compressing one naris into which the end of a rubber tube attached to an air
bag has been inserted while the opposite naris is compressed with digital pressure.
The patient is asked to repeat the letter K or to swallow while air is injected into the
nasal cavity. When positive, the overpressure that develops in the nasopharynx is
transmitted to the middle ear, and only indicates an anatomically patent ET tube.
Both the Politzer and Valsalva test may be beneficial as a temporary treatment of
effusion or high negative middle ear pressure.
The Toynbee Test is performed by visual inspection of the tympanic membrane
while the patient swallows with their nose manually occluded. This generates a
positive pressure within the nasopharynx, followed by a negative pressure phase
and is considered positive when there is an alteration in middle-ear pressure as
assessed by pneumatic otoscopy before and after the maneuver. Negative middleear pressure or temporary negative middle ear pressure followed by return to
ambient pressure after the Toynbee test usually is indicative of normal eustachian
tube function. This is in contrast to the Politzer and Valsalva tests which only test
patency. The results of this maneuver can often be equivocal, since several studies
have shown that a significant portion of normal adults and children can not open
their eustachian tubes with this maneuver, and patients with patulous eustachian
tube often can not maintain a negative pressure within their middle ears.
Eustachian tube catheterization can be performed, and also can indicate eustachian
tube patency.
Radiographic evaluation includes computed tomography, and magnetic resonance
imaging. The use of contrast materials to evaluate patency has been described in
the past, but is infrequently used today.
IN THE LAB
There are several more complex methods of evaluating eustachian tube function
that have been described and most involve the use of manometry, sonometry, of
tympanometry. Besides tympanometry most of these tests require complex
equipment, and are mainly used in a research setting.
Non-intact Tympanic Membrane Tests
The Inflation-Deflation test

Forced Response test
clearance test
Intact Tympanic membrane tests
pressure chamber technique

sonometry
tympanometry
Clinical Examples Of Eustachian Tube Dysfunction

Here are a small sample of the clinical scenarios where eustachian tube dysfunction
is important:
OTITIS MEDIA WITH EFFUSION
Obstruction may result in persistent high negative middle-ear pressure. If pressure
equalization does not occur, atelectasis of the tympanic membrane-middle ear,
sterile otitis media with effusion, or both can occur. If the negative pressure is
overcome, it can aspirate secretions from the nasopharynx resulting in an acute
otitis media.
Serous otitis media with effusion can result from either inadequate ventilation of
the middle ear or from reflux of unwanted nasopharyngeal secretions into the
middle ear. Both types of eustachian tube dysfunction can result in otitis media,
abnormal patency and obstruction.
This is common in children and infants probably due to the configuration of their
eustachian tube, shorter length, and lower efficiency of their tensor veli palantini.
While serous otitis media is something that many of us treat on a daily basis, Dr.
Gacek of Syracuse reminds us, in an article entitled "A Differential Diagnosis of
Unilateral Serous Otitis Media", of the potentially serious nature of this condition.
Clinicians need to maintain a high index of suspicion, particularly in adults, in
unilateral cases, and in persistent or recurrent cases. From Dr. Gacek's article, it is
important to remember:
1. The eustachian tube lumen can be obstructed and this is usually from
inflammatory, allergic or functional disorders.
2. The nasopharynx is usually obstructed by adenoid hypertrophy, but
extensive nasal polyposis, benign neoplasms, and malignant neoplasms also
may present in this location.
3. Obstruction may occur from laterally in the infratemporal fossa, by
parapharyngeal space masses and neoplasms, or skull base lesions.
4. Medial obstruction from the petrous apex may be caused by solid or cystic
lesions, including congenital epidermoids, cholesterol granulomas,
neurofibromas, internal carotid artery aneurysms and other rare petrous apex
lesions.
5. Effusion of CSF from the middle ear and mastoid caused by temporal bone
trauma, surgery, or congenital defects may mimic otitis media with effusion
and must be remembered as part of the differential diagnosis.
Dr. Gacek reinforces the importance of a thorough head and neck examination
including the nasopharynx, CT scan of the head including the neck, and
myringotomy as the minimal workup in any pediatric or adult patient with
unilateral recurrent or persistent serous otitis media without an obvious explanation
for eustachian tube obstruction.
He also emphasizes that in pediatric patients, the eustachian tube lumen and
nasopharynx are the anatomic locations most frequently responsible, but congenital
CSF leaks should be suspected in patients with a history of meningitis, or if the
fluid after myringotomy resembles CSF.
In adults, all levels should be suspected.
NASOPHARYNGEAL CARCINOMA
Patients with nasopharyngeal carcinoma frequently have complications that relate
to their eustachian tube. The frequently present with serous otitis media. Also, highdose radiation therapy, the treatment for nasopharyngeal carcinoma, causes edema,
vasodilation, mucosal damage, and fibrosis of the eustachian tube and middle ear
resulting in damage to the middle ear contents and poor middle ear ventilation.
While it seems intuitive that serous otitis media with effusion in patients with
nasopharyngeal carcinoma would be caused by mechanical obstruction of the
pharyngeal orifice of the eustachian tube, several studies question whether
nasopharyngeal tumors, actually obstruct the lumen of the eustachian tube, and
instead propose that eustachian tube dysfunction and resulting otitis media with
effusion is caused by infiltration of the tensor veli palatini muscle.
With regard to patients after radiation, a study by Hsu, et al, in 1995, showed that
95% of 38 eustachian tubes were patent prior to radiotherapy, 34% where patent at
6 months after radiotherapy, and 60% were patent at 5 years after radiotherapy
using the passive opening test. They also showed decreased dynamic function and
clearance at six months after radiotherapy and improved at 5 years. They attributed
these findings to inflammation caused by radiation rather than tumor obstruction.
Electromyographic evaluation of the tensor veli palatini in patients with

nasopharyngeal carcinoma status post radiation indicated neurogenic paralysis.
These authors have also found that in patients treated with ventilation tube insertion
for post-irradiation OME tend to develop a chronic draining ear, and deterioration
of hearing. They suggest myringotomy, avoidance of ventilating tubes, and
frequent local treatment of infections of the nose, sinuses, and nasopharynx to
avoid this outcome.
PATULOUS EUSTACHIAN TUBE
Patulous eustachian tubes often present a frustrating problem for patients and
clinicians.The incidence is reported to be between 0.3-6.6% of the general
population.
Patients with patulous eustachian tubes complain of aural fullness, humming
tinnitus, and autophony. They also may hear their own breath sounds, which is
known as tympanophonia. The sound is synchronous with nasal respiration and
resolves when the patient is supine or when upper respiratory tract inflammation
occurs. The sounds may be aggravated by mastication.
Symptoms are usually absent when the patient is supine or relieved when the
patient bends forward with the head between the knees. For this reason, patients
should not be examined in a supine position. Physical examination may reveal a
tympanic membrane that moves during forced breathing through one nostril, and an
amorphic sound may be heard using a diagnostic tube in the patient's ear.
The eustachian tube is usually closed, and closure is maintained by the elasticity of
its cartilage, mucosal lining, surrounding muscles and fat. Alteration of any of
these anatomic components may cause patulous eustachian tubes.
Conditions associated with patulous eustachian tubes include: radiation therapy,
hormonal therapy, pregnancy, nasal decongestants, fatigue, stress, and weight loss.
Patulous eustachian tubes in the most severe form may be patent at all times,
whereas a less severe form has been reported, where the tube is anatomically closed
at rest, but may open easily during exercises or in association with a decrease in
peritubal extracellular fluid.
Many patients can be treated with simple reassurance after a thorough history and
physical examination. Treatment or removal of underlying factors may reverse the
problem. Such as weight gain by patients who have lost weight.
Many medical regimens have been described including agents which produce
intraluminal and extraluminal swelling, including: insufflation of boric acid and
salicylate powder as described by Bezold, application of nitric acid and phenol, oral
administration of saturated solution of potassium iodide (10 drops in juice TID),
premarin nasal spray (25 mg in 30 cc NS).
New medications are currently under investigation including a herbal combination
being evaluated in Japan, and a medication reported Dr. DiBartolomeo of Santa
Barbara, California that is composed of chlorobutanol, benzyl alcohol, diluted
hydrochloric acid, and propylene glycol. In the initial report, complete elimination
of symptoms was reported by 8 of 10 patients. This formulation was derived from
chlorinated pool water based on the observation that several patients had eustachian
tube congestion proportional to the frequency of time they spent in a public pool. In
letter to the editor in American Journal of Otology, Dr. DiBartolomeo indicated
that the medication was held up with the FDA.
In patients who do not improve with medical therapy and who want further
treatment, several surgical interventions have been used including
electrocauterization of the eustachian tube orifice, peritubal injection with gelfoam,
paraffin, avitene, or teflon paste, transposition of the tensor veli palatini muscle
medial to the pterygoid hamulus, myringotomy with ventilation tube placement,
and insertion of an indwelling catheter and subsequent ventilation tube placement.
Catheter placement is through either an anterior tympanomeatal flap or through a
myringotomy.
The close anatomic relationship of the eustachian tube and the carotid artery should
be noted by clinicians who plan inject materials into the eustachian tube orifice, as
injection of telfon paste into the carotid artery has been reported.
HYPERBARIC OXYGEN THERAPY
Another clinical situation where proper eustachian tube function is important is in
the use of hyperbaric oxygen therapy, particularly in patients who require multiple
sessions.
Hyperbaric oxygen therapy involves intermittent inhalation of 100% oxygen under
greater than 1 atmosphere of pressure and is being used increasingly in patients
with decompression sickness, osteomyelitis, carbon monoxide poisoning, crush
injuries, radiation necrosis, and poorly healing wounds. Many of these patients
develop otalgia and aural fullness that may be long-standing. Reports in the
literature indicate that the incidence of middle ear barotrauma ranges from 5% to
28% of all patients.

Fernau, et al suggest patients should be taught clearing techniques such as the
Valsalva or Politzer maneuver, supplemented with topical and/or systemic
decongestants, subjected to slower compression rates, or possibly have ventilation
tubes placed.
In a study of 33 patients undergoing hyperbaric oxygen therapy by Fernau, et al. in
1992, 82% of patients developed fullness in their ears, 52% developed serous otitis
media, and 21% developed otalgia requiring ventilation tubes. Of 11 patients
managed with decongestants,10 patients resolved their effusion and pain and did
not require further therapy. 45% of 33 patients had evidence of pre-existing
eustachian tube dysfunction using the inflation-deflation test. Of these patients
100% developed aural fullness, 87% developed serous otitis media, and 47%
required tympanostomy tubes. Fernau, et al, identified a history of eustachian tube
dysfunction as a risk factor for serous otitis media in patients undergoing
hyperbaric oxygen therapy.
An article by Presswood, et al, points out that the middle ear complication rate in
intubated patients receiving hyperbaric oxygen therapy is 94% compared to 46% of
non-intubated patients. (Hemotympanum and otalgia) They state that the use of
nasal decongestants in this population is controversial, and probably of no value in
patients who are intubated. They recommend ventilation tubes should be placed in
their ears prophylactically.
OTHER CLINICAL SITUATIONS
Obviously, there are many more clinical situations that the role of eustachian tube
dysfunction is important. Disorders of the eustachian tube present important issues
that in diagnosis and management that are faced in daily clinical practice. Despite
the large volume of literature on eustachian tube dysfunction, the lack of welldesigned prospective studies make the literature difficult to decipher, and these
disorders continue to represent some of the most challenging management
problems we face as otolaryngologists.
Summary
The eustachian tube is an important anatomic structure that ventilates, protects, and
drains the middle ear.
During development the eustachian tube lengthens and the angle between it and the
skull base increases from 10 degrees in infancy to 45 degrees in adulthood.
Eustachian tube dysfunction can be caused by mechanical obstruction, which may

be intrinsic or extrinsic, by functional obstruction, or by the presence of patulous
eustachian tubes.
Otitis media with effusion is a common sequalae of eustachian tube dysfunction,
but a high index of suspicion must be maintained in adults, in unilateral cases, and
in patients with recurrent or persistent disease without an obvious explanation.
Case Presentation
A 54-year-old male was referred to the Bobby R. Alford Department of
Otorhinolaryngology and Communicative Sciences for evaluation and management
of a right patulous eustachian tube. The patient had previously undergone a right
myringotomy and ventilation tube placement without resolution of his symptoms.
He complained of right aural pressure and autophony for over one year. The
autophony was particularly bothersome while singing. The patient had a history of
high pitched noise exposure while in the military, and wears ear protection while at
work. He denied headaches, nausea, vomiting, vertigo, otorrhea, otalgia, ear
trauma, or ear surgery. He had no history of recent weight loss, and the remainder
of his medical history was unremarkable.
Physical examination revealed his left tympanic membrane to be clear, intact, and
mobile. The right tympanic membrane had a scar in the anterior inferior quadrant,
but was otherwise unremarkable. The right tympanic membrane did not move with
swallowing or Valsalva maneuver. There were no masses noted in the
nasopharynx. The rest of physical examination was unremarkable.
The patient had an audiogram which was remarkable for symmetric severe
sensorineural hearing loss above 2000 hertz bilaterally. He had Type A
tympanograms bilaterally.
The patient was reassured of his condition and Premarin nose drops were started,
but the patient failed to respond. The patient was given the option of surgical
intervention, and has noted an improvement in symptoms 2 weeks status post
eustachian tube obliteration and ventilating tube placement.
Bibliography
Bluestone CD. Current concepts in eustachian tube function as related to otitis media. Auris
Nasus Larynx 1985, 12 (Suppl 1): S1-S4.
Bluestone CD. Eustachian tube obstruction in the infant cleft palate. Ann Otol Rhinol Laryngol
1971, 80: 1-30.
Deron P, Clement PA, Derde MP. Septal surgery and tubal function. Rhinology 1995, 33: 7-9.
DiBartolomeo JR, Henry DF. A new medication to control patulous eustachian tube disorders.
Am J Otol 1992, 13: 323-327.
Dyer RK, McElveen JT. The patulous eustachian tube: management option. Otolaryngology
Head Neck Surg 1991, 105: 832-835.
Fernau JL, Hirsch BE, Derkay C, Ramasastry S, Schaefer SE. Hyperbaric oxygen therapy: effect
on middle ear and eustachian tube function. Laryngoscope 1992, 102: 48-52.
Gacek RR. A differential diagnosis of unilateral serous otitis media. Laryngoscope 1992, 102:
461-468.
Halsted TH. Pathology and surgery of the eustachian tube. Arch Otolaryngol 1926, 4: 189-195.
Henry DF, DiBartolomeo JR. Patulous eustachian tube identification using tympanometry. J Am
Acad Audiol 1993, 4: 53-57.
Holborow C. Eustachian tubal function: changes throughout childhood and neuromuscular
control. J Laryngol Otol 1975, 89:47.
Hsu MM, Young YH, Lin KL. Eustachian tube function of patients with nasopharyngeal
carcinoma. Ann Otol Rhinol Laryngol 1995, 104: 453-455.
Iwano T, Kinoshita T, Hamada E, Doi T, Ushiro K, Kumazawa T. Otitis media with effustion and
eustachian tube dysfunction in adults and children. Acta Otolaryngol 1993, Suppl 500, 66-69.
Presswood G, Zamboni WA, Stephenson LL, Santos P. Effect of artificial airway on ear
complications from hyperbaric oxygen. Laryngoscope 1994, 104: 1383-1384.
Proctor B. Embryology and anatomy of the eustachian tube. Arch Otolaryngol 1967, 86: 503514.
Sade J. The nasopharynx, eustachian tube and otitis media. J Laryngol Otol 1994, 108: 105-110.
Sakakihara J, Honjo I, Fujita A, Kurata K, Takahashi. Eustachian tube compliance in sniffinduced otitis media with effusion. Acta Otolaryngol 1993, 113: 187-190.
Sakikawa Y, Kobayashi H, Normura Y. Changes in middle ear pressure in daily life.
Laryngoscope 1995, 105:1353-1357.
Shambaugh GE. Continuously open eustachian tube. Arch Otolaryngol 1938, 27: 420-425.
Young Y, Lin KL, Ko JY. Otitis media with effusion in patients with nasopharyngeal carcinoma,
postirradiation. Arch Otolaryngol 1995, 121: 765-768.


medical literature.
TRAUMATIC INJURIES TO THE FACIAL NERVE

October 12, 1991
Randall S. Zane, M.D.
Traumatic injuries to the facial nerve can be classified by site (extracranial, intratemporal, intracranial)
and by the type of trauma (penetrating, non-penetrating, or iatrogenic). The general principles of
management remain the same regardless of the location of the injury, but the details vary and will be
presented by location.
Blunt trauma to the face rarely causes significant extracranial facial nerve injury. An example is birth
trauma, most often the result of a forceps delivery where the exposed facial nerve is compressed against
the rigid cervical spine. Penetrating lesions of the facial nerve distal to the parotid gland and medial to
the lateral canthus of the eye rarely result in severe disfigurement and most often do not require repair.
Repair of the cervical or platysmal division is also usually unnecessary because of the minimal functional
loss that results from transection. Lesions of the main trunk or the temporozygomatic or cervicofacial
divisions should be repaired or a major deficit in facial movement will persist. Clean facial lacerations
with immediate onset of facial paralysis should be promptly explored. After three days, the severed
branch loses its excitability, and that can make its identification tedious and uncertain. If there is gross
contamination of the wound requiring debridement, other life threatening injuries where it is not
advisable to prolong anesthesia, or if there is a lack of equipment or expertise, an attempt should be made
to identify the severed nerve endings and to tag them for later identification. Whenever possible, primary
end-to-end anastomosis is ideal and allows the greatest functional return. Interpositional grafting is
second best, but a certain percentage of regenerating axons are lost across each anastomosis. For
interposition grafting, the greater auricular and sural nerves are popular largely because of the ease in
which these sensory nerves are harvested.
Iatrogenic injuries occur most often with extirpation of parotid malignancy where the facial nerve passes
through the tumor and must be removed. In this instance, interpositional grafting is the usual procedure.
Even when there is a question as to the adequacy of resection, potential long-term survival or the need
for postoperative radiotherapy, grafting should be undertaken since it adds only a short time to the
operation. The psychological
advantages of even incomplete return of function as soon as possible after surgery should not be
underestimated. In more routine parotid surgery, the facial nerve should be stimulated near the
stylomastoid foramen before wound closure. If brisk facial movement occurs, subsequent facial motion
will be satisfactory, even if there is paresis immediately postop. If movement does not occur in all major
muscle groups, the nerve should be inspected carefully under magnification for evidence of injury, for
example, ligature on the nerve, crush injury, and the like. If a crush injury is suspected it may be
necessary to split the sheath to ascertain whether the fascicles are in continuity. If they are not, excision
of the damaged segment and repair may be the best course of action.
Temporal bone fractures are a unique form of non-penetrating injury that can cause significant damage to
the intratemporal facial nerve. They may be classified as longitudinal or transverse according to the
orientation of the fracture planes on high resolution CT scanning, and they may occur in a mixed form.
Facial nerve injuries are more common with transverse fractures, but because the incidence of
longitudinal fractures is higher, they are seen more frequently as a result of longitudinal fractures.
With longitudinal fractures where the associated hearing loss is conductive in nature, exploration of the
geniculate ganglion should be carried out via a middle cranial fossa approach, followed by transmastoid
exploration of the tympanic and vertical segments for the occasional injury caused by fractures through
the osseous canal. Transverse fractures, by virtue of the associated severe sensorineural hearing loss are
explored through transmastoid and translabyrinthine approaches. Injuries in the tympanic and
labyrinthine segments are easily accessible for repair via this approach. Lesions including edema,
hemorrhage, contusion, and impacted bony spicules are best managed by decompression of the nerve in
the fallopian canal. Transection of more than 50% of the nerve requires an interpositional graft to ensure
regeneration of an adequate number of motoneurons.
Penetrating injuries of the temporal bone are most often caused by gunshot wounds but can be the result
of some stab wounds. If there is any suspicion of a vascular injury of the sigmoid or lateral sinus, jugular
vein or carotid system, carotid arteriography is indicated. When surgery is indicated, most can be
explored through standard transmastoid approaches. Primary reanastomosis of a severed nerve in the
fallopian canal is rarely encountered as an option to restore continuity - Interpositional grafts are
frequently necessary using the nerve's natural self-adhesiveness without the use of suture, and this may
be enhanced by the application of autologous fibrin glue. The fallopian canal can be used as a sort of
stent to hold the graft in place.
The most common sites of inadvertent injury to the nerve in tympanomastoid surgery are in the middle
ear where the tympanic segment of the nerve above the oval window is occasionally dehiscent, and in the
mastoid at the second genu where it is susceptible to injury during antrotomy if the usual landmarks
including the lateral semicircular canal are distorted or not recognized. If a postoperative paralysis
occurs, sufficient time must elapse
to ensure that the effects of any local anesthesia have worn off. If the surgeon did not identify the nerve
during the operation, exploration is mandatory. If the nerve was identified and the surgeon knows it to be
intact, the patient should be followed with electrodiagnostic testing. If only postoperative paresis is
evident, the patient is followed closely by examination, and if the palsy becomes complete the patient
should undergo electrodiagnostic testing.
Injury to the intracranial segment of the facial nerve is usually iatrogenic, secondary to tumor removal,
but infrequently can result from penetrating injuries or medial transverse temporal bone fracture.
Collagen splints have been used for grafting in this location, but results are poor.
As the quality of radiographic studies, primarily high resolution CT scan, has improved, the importance
of topognostic testing has fallen off. However, although it is sometimes inaccurate and has not been
shown to be of prognostic significance, they can be helpful in determining the proximal extent of injury
and should not be forgotten.
In the past, decisions as to surgery traditionally were based on the time of onset of complete paralysis,
such that patients with immediate onset following trauma were explored and those with delayed onset
were managed conservatively. Electrophysiologic testing has changed the scene quite a bit, since cases of
paralysis with an immediate onset have been monitored and have had good spontaneous return of
function, while delayed onset has been monitored to progress and lead to suboptimal results. In the first
three days, electrical testing takes a back seat to clinical evaluation including the physical examination.
Between three days and three weeks, if the patient has visible facial motion, electrical testing is not
needed and will be normal if obtained. If there is paralysis, early screening with a Hilger nerve stimulator
is used. If the threshold on the involved side stays less than 3.5 mAmps above the normal side, no further
testing is necessary. If the threshold difference goes above 3.5 mAmps, some authors recommend
exploration. However, if ENoG is available to you, it is recommended that serial examinations be
performed every one or two days to follow the course of degeneration. Fisch has set up guidelines such
that should degeneration, to greater than 90% of nerve fibers as calculated by ENoG, take place in the
first three weeks, the prognosis is poor for spontaneous recovery and that patient should be explored.
Anytime after three weeks, there is some controversy regarding the decision to explore, primarily
because some nonpenetrating injuries can show spontaneous recovery. Dr. Coker and Dr. Jenkins feel
that all penetrating and iatrogenic injuries with evidence of complete degeneration need exploration to
document location and cause of the problem. The intact nerve can then be left alone and observed, while
the severed nerve is appropriately managed. All blunt injuries outside the stylomastoid foramen can be
observed for six to twelve months before intervention.
The most controversy is generated over late exploration for temporal bone fractures with complete
paralysis. This is because the natural course of recovery has not been established.
Except by perceptible facial movement or EMG activity, there is no way of knowing whether or not
regeneration is occurring across the injury site and that with time facial function
could be acceptable. Some argue that bony fragments and fibrosis can impede the spontaneous
regeneration at the injury site and that exploration can enhance the opportunity for regeneration. This is a
difficult problem to reconcile and should be approached on a case-by-case basis with careful counseling
of the patient. Should there be no return of function by twelve to eighteen months post temporal bone
fracture, there is little argument that the nerve should be explored with interpositional grafting at the site
of injury.
The following management rationale for injuries to the facial nerve is reproduced (by permission) from
the award-winning scientific exhibit presented by Dr. Newton Coker and Dr. Herman Jenkins at the 1990
American Academy of Otolaryngology - Head and Neck Surgery meeting in San Diego.
Case Presentation
A 54 year old black male was assaulted and stabbed multiple times in the back, shoulders and right face.
The single facial wound was one centimeter in length and was vertically oriented 1.5 centimeters anterior
to the tragus. Examination revealed complete right peripheral facial paralysis without significant edema
or hematoma. Clear saliva was easily expressed from Stenson's duct. On the third day post injury, after
stabilization and complete evaluation of his other injuries, he was taken to the operating room for right
facial nerve exploration. The main trunk of the facial nerve was identified and found to be transected just
proximal to the pes anserinus. All branches of the nerve were distally intact as demonstrated by electrical
stimulation of the distal cut edge. A primary neurorrhaphy was carried out under no tension with 10-0
Prolene suture using a perineural technique.
Bibliography
Adkins WY, Osguthorpe JD: Management of trauma of the facial nerve. Otolaryngol Clin North Am 24:587-611, 1991.
Adour KK, Bayajian JA, Kahn ZM, Schneider GS: Surgical and nonsurgical management of facial paralysis following
closed head injury. Laryngoscope 87:380-90, 1977.
Aguilar EA, Yeakley JW, Ghorayeb BY, Hauser M, Cabrera J, Jahrsdoerfer RA: High resolution CT scan of temporal
bone fractures: Association of facial nerve paralysis with temporal bone fractures. Head Neck Surg 9:162-66, 1987.
Alford BR: Electordiagnostic studies in facial paralysis. Arch Otolaryngol 85:259-64, 1967.
Alford BR, Jerger JF, Coats AC, Peterson CR, Weber SC: Diagnostic tests of facial nerve function. Otolaryngol Clin
North Am 7:331-42, 1974.
Alford BR, Sessions RB, Weber SC: Indications for surgical decompression of the facial nerve. Laryngoscope 81:620-35,
1971.
Alford BR, Weber SC, Sessions RB: Neurodiagnostic studies in facial paralysis. Ann Otol Rhinol Laryngol, 79:227-33,
1970.
Babin RW: Topognostic and prognostic evaluation of traumatic facial nerve injuries. Otolaryngol Head Neck Surg 90:61011, 1982.
Baker DC, Conley J; Facial nerve grafting: A thirty year retrospective review. Clin Plast Surg 6:343-60, 1979.
Cannon CR, Jahrsdoerfer RA: Temporal bone fractures. Arch Otolaryngol 109:285-88, 1983.
Coker NJ, Jenkins HA, Psifidis A: Electrophysiologic prognostication of acute facial nerve trauma, in Fisch U, Valavanis
A, and Yasargil MG (eds): Neurological Surgery of the Ear and the Skull Base: Proceedings of the Sixth international
Symposium. Amsterdam, Kugler and Ghedini, 1989.
Coker, NJ, Kendall KA, Jenkins HA, Alford BR: Traumatic intratemporal facial nerve injury: Management rationale for
preservation of function. Otolaryngol Head Neck Surg 97:262-69, 1987.
Dott NM: Facial nerve reconstruction by graft bypassing the petrous bone. Arch Otolaryngol 78:426-28, 1983.
Duncan NO, Coker NJ, Jenkins HA: Gunshot injuries of the temporal bone. Otolaryngol Head Neck Surg 94:47-55, 1986.
Fisch U: Facial nerve grafting. Otolaryngol Clin North Am 24:691-708, 1991.
Fisch U: Facial paralysis in fractures of the petrous bone. Laryngoscope 84:2141-54, 1974.
Fisch U: Prognostic value of electrical tests in acute facial paralysis. Am J Otol 5:494-98, 1984.
Glasscock ME, House WF, Alford BR: Middle fossa facial nerve decompression. Laryngoscope 79:234-40, 1969.
Grobman LR, Pollak A, Fisch U: Entrapment injury of the facial nerve resulting from fracture of the temporal bone.
Otolaryngol Head Neck Surg 101:404-08, 1989.
Guilford FR: Surgical consideration in disorders of the horizontal and vertical portions of the facial nerve. Ann Otol Rhinol
Laryngol 79:241-51, 1970.
Harker LA, McCabe BF: Temporal bone fractures and facial nerve injury. Otolaryngol Clin North Am 7:425-31, 1974.
Hasso AN, Ledington JA: Traumatic injuries of the temporal bone. Otolaryngol Clin North Am 21:295-316, 1988.
Johns ME, Crumley RL: Facial nerve injury, repair and rehabilitation (SIPac). Alexandria, VA, American Academy of
Otolaryngology, 1979.
Kamerer DB: Intratemporal facial nerve injuries. Otolaryngol Head Neck Surg 90:612-15, 1982.
Lambert PR, Brackmann DE: Facial paralysis in longitudinal temporal bone fractures: A review of 26 cases. Laryngoscope
94:1022-26, 1984.
May M: Total facial nerve exploration: Transmastoid, extralabyrinthine, and subtemporal indications and results.
Laryngoscope 89:906-17, 1979.
May M, Klein SR: Differential diagnosis of facial nerve palsy. Otolaryngol Clin North Am 24:613-45, 1991.
Miehlke A: Extratemporal injury and repair of the facial nerve. Otolaryngol Clin North Am 7:467-92, 1974.
Millen SJ, Daniels D, Meyer G: Gadolinium-enhanced magnetic resonance imaging in facial nerve lesions. Otolaryngol
Head Neck Surg 102:26-33, 1990.
Pillsbury HC, Fisch U: Extratemporal facial nerve grafting and radiotherapy. Arch Otolaryngol 105:441-46, 1979.
Pulec JL: Total decompression of the facial nerve. Laryngoscope 75:1015-28, 1966.
Schwaber MK, Zealear D, Netterville JL, Seshul M, Ossoff RH: The use of magnetic resonance imaging with highresolution CT scan in the evaluation of facial paralysis. Otolaryngol Head Neck Surg 101:449-58, 1989.
Sunderland S: Some anatomical and pathophysiological data relevant to facial nerve injury and repair, in: Facial Nerve
Surgery. New York, Aesculapius Publishing, 1977.
Tucker HM: The management of facial paralysis due to extracranial injuries. Laryngoscope 88:348-54, 1978.
Wiet RJ: Iatrogenic facial paralysis. Otolaryngol Clin North Am 15:773-80, 1982.
Yanagihara N: Transmastoid decompression of the facial nerve in temporal bone fracture. Otolaryngol Head Neck Surg
90:616-21, 1982.


medical literature.
FACIAL REANIMATION OF THE CHRONICALLY PARALYZED FACE

September 28, 1994
Amy Y. Chen, M.D.
Facial nerve paralysis is a devastating injury that can result in blindness, oral incontinence and social
handicaps. The muscles of the face are exquisitely designed for both voluntary and involuntary
expression.
The facial nerve emerges from the brainstem via the internal auditory canal and exits the brain through the
stylomastoid foremen. At this point, the nerve travels a short distance to the pes where it divides into five
motor branches--the temporal, zygomatic, buccal, mandibular and cervical branches.
The nerve is responsible for orchestrating an intimately designed facial musculature system. The facial
musculature is capable of maintaining tone as well as expressing both voluntary and involuntary
emotions.
First, we will discuss the importance of eye protection and methods used to achieve a healthy eye.
Paralysis of orbicularis oculi results not only in drying of the cornea but also in poor distribution of tears.
The lower lid is not able to contract and disperse the tears across the eye. Furthermore, loss of
parasympathetic innervation can result in decreased tear production. This can result in keratitis and rarely,
corneal ulceration and cataract formation. Therefore, the primary objective of the surgeon is to protect the
eye via surgical and/or nonsurgical methods.
Surgical methods may involve both upper lid procedures such as tarsorraphy, gold weights, and palpebral
springs as well as lower lid procedures.
Muscle transposition can be utilized to reanimate the face. Two muscles primarily used are the masseter
and the temporalis. The patient must have an intact V for these procedures. Movement of the face can be
achieved by instructing the patient to clench the teeth.
Masseter muscle transposition involves harvesting two anterior slips of masseter muscle and transposing
it to the upper and lower lip. Advantages include supplementing other reanimation techniques such as XIIVII or temporalis sling and providing static tone for the face. However, a downwards vector may cause
the lip to droop.
Temporalis muscle transposition is also commonly used. This muscle is used primarily to reanimate the
mouth and lower aspect of the face. The temporalis muscle is harvested and sewn to upper lip,
commissure and lower lip. The key to success is to ensure overcorrection and exaggeration of the lip.
Goretex strips can be used to lengthen the muscle so that it can reach the mouth. The defect in the
temporalis can be corrected with synthetic insert or a temporoparietal fascial flap. May & Drucker in 1993
reported that the success of temporalis muscle transfer to restore a smile was 80% and to improve mouth
function was 96%.
Various neural methods have also been employed to attempt facial reanimation. Primary anastomosis can
be utilized effectively in the acute injury. But in the chronic palsy, primarily three techniques are
currently used--XII-VII traditional, XII-VII jump, and VII-VII cross face graft. 4-6 months usually pass
before the facial muscles begin to show signs of recovery.
The XII-VII traditional anastomosis was first performed in 1903 by Korte. Indications for this procedure
include an intact peripheral facial nerve and a nonatrophic facial musculature. However, if the patient has
injured the peripheral facial nerve or has atrophied muscles, or if other cranial nerve palsies exist, this
procedure is to be avoided. The patient must learn to push his tongue against the teeth when he wishes to
smile. Advantages of the procedure are that majority of patients has good movement. However, some
patients can develop synkinesis and mass motion as well as atrophy of the tongue.
Another method of XII-VII anastomosis is that of placing an interposition graft between the two, thus
"jumping" from XII-VII. This procedure involves partially severing the XII nerve. One usually uses either
the greater auricular nerve or the sural nerve as grafts. The former is preferred since it does not involve
another operative exposure. However, the sural nerve can be advantageous for it provides a longer graft as
well as larger diameter graft.
For best results, the anastomoses should be performed within one year of surgery. This jump graft
procedure can diminish the incidence of tongue atrophy, the problems with synkinesis and mass motion
and those with swallowing, mastication and speech. However, patients can have weak facial contractures.
Another disadvantage is that the procedure involves two anastomoses rather than one as in the traditional
XII-VII.
The incidences of swallowing deficiencies, mastication problems and speech difficulty are greater for XIIVII traditional grafts as compared to XII-VII jump. However, the results of achieving symmetry and
excellent facial movement are similar between the two. One is able to reduce the incidence of mass
movement with a jump graft.
VII -VII cross face grafts have also been used. Scaramella first described the procedure in 1970. It
involves grafting VII from the functional side of the face to the stump on the paralyzed side. Obvious
disadvantages include long operative time involving two anastomoses and surgical intrusion of the
functional side of the face. Synkinesis and low grade neural input can result in less forceful contracture of
the face. This procedure is often used in conjunction with free muscle transfers.
With the advent of microsurgery, it is also possible to transpose free flaps. Harii et al in 1975 were the
first to transfer free muscle tissue for facial reanimation. Free muscle transfer provides soft tissue
coverage of a defect as well as the opportunity for one to develop voluntary control of the face. This is
usually a two stage procedure. The first stage involves anastomosing the nerve graft, usually a cross face
graft. 6-12 months later, the free flap is harvested and anastamosed to the site. Commonly used donor
sites include the gracilis, serratus anterior, latissimi dorsi, and rectus abdominis. The recipient vessels are
typically facial artery and common facial vein.
In summary, the management of the permanently paralyzed face is threefold. First, one must provide
protection of the eye. Second, muscle transposition is performed to restore tone to the patient's face.
Third, efforts are made to restore facial movement that automatically reflects the breadth and depth of
human expression. It is this last goal that remains elusive. However, with these methods, a patient can use
the restored movement to express human emotion by practice and adaptation.
Case Presentation
A 65-year-old black woman presented with a long history of right-sided hearing loss. Over the past few
months, she has had dizziness and unsteadiness in her gait. Past medical history was significant for
diabetes and hypertension. A hysterectomy was her only surgical history.

Imaging studies revealed a large 5 cm tumor in the cerebellopontine angle consistent with a vestibular
schwannoma. Audiogram revealed a profound right sensorineural hearing loss.
Excision of the tumor was performed via a transotic and translabyrinthine approach. The VII nerve was
not preserved and therefore the patient developed a right facial paralysis. She did not have other cranial
nerve deficits.
Approximately 6 weeks after the initial surgery, the patient was evaluated for facial reanimation. At this
time, gold weights were implanted in the right eyelid and a XII-VII hypoglossal-facial nerve
interpositional-jump graft procedure was performed. The patient had an uneventful postoperative course
and was discharged home two days after surgery.
Bibliography
Anderl H. Cross-face nerve transplant. Clin Plast Surg 1989;6:433-449.
Aviv JE, Urken ML. Management of the paralyzed face with microneurovascular free muscle transfer. Arch Otolaryngol
Head Neck Surg 1992;118:909-912.
Baker DC, Conley J. Facial nerve grafting: a thirty year retrospective review. Clin Plast Surg 1979;6:343-360.
Baker DC, Conley J. Regional muscle transposition for rehabilitation of the paralyzed face. Clin Plast Surg 1979;6:317331.
Conley J. Myths and misconceptions in the rehabilitation of facial paralysis. Plast Reconstr Surg 1983;71:538-539.
Harii K, Ohmori K, Torii S. Free gracilis muscle transplantation, with microneurovascular anastomoses for the treatment of
facial paralysis. Plast Reconstr Surg 1976;57:133-143.
Hata Y, Yano K, Matsuka K, Ito O, Matsuda H, Hosokawa K. Treatment of chronic facial palsy by transplantation of the
neurovascularized free rectus abdominis muscle. Plast Reconstr Surg 1990;86:1178-1179.
Kartush JM, Linstrom CJ, McCann PM, Graham MD. Early gold weight eyelid implantation for facial paralysis.
Levine RE. Reanimation of paralyzed eyelids. Facial Plast Surg 1992;8:121-126.
May M, Sobol SM, Mester SJ. Hypoglossal-facial nerve interpositional-jump graft for facial reanimation without tongue
atrophy. Otolaryngol Head Neck Surg 1991;104:818-825.
May M. Gold weight and wire spring implants as alternatives to tarsorraphy. Arch Otolaryngol Head Neck Surg
1987;113:656-660.
May M. Paralyzed eyelids reanimated with a closed-eyelid spring. Laryngoscope 1988;98:382-385.
May M, Hoffmann DF. Buerger GF, Soll DB. Management of the paralyzed lower eyelid by implanting auricular cartilage.
May M, Drucker C. Temporalis muscle for facial reanimation: a 13-year experience with 224 procedures. Arch Otolaryngol
Head Neck Surg 1993;119:378-382.
May M. Muscle transposition for facial reanimation: indications and results. Arch Otolaryngol 1984;110:184-189.
May M. Muscle transposition for facial reanimation. Facial Plast Surg 1992;8:115-120.
May M, Sobol SM, Brackmann DE. Facial reanimation: the temporalis muscle and middle fossa surgery. Laryngoscope
1991;101:430-432.
O'Brien BM, Franklin JD, Morrison WA. Cross-facial nerve grafts and microneurovascular free muscle transfer for long
established facial palsy. Br J Plast Surg 1980;33:202-215.
O'Brien BM, Pederson WC, Khazanchi RK, Morrison WA, MacLeod AM, Kumar V. Results of management of facial
palsy with microvascular free-muscle transfer. Plast Reconstr Surg 1990;86:12-22.
Mackinnon SE, Dellon AL. Technical considerations of the latissimus dorsi muscle flap: a segmentally innervated muscle
transfer for facial reanimation. Microsurg 1988;(;36-45.
Neuman AR, Weinberg A, Sela M, Peled IJ, Wexler MR. The correction of seventh nerve palsy lagophthalmos with gold
lid load (16 years experience). Ann Plast Surg 1989;22:142-145.
O'Connell JE, Robin PE. Eyelid gold weights in the management of facial palsy. J Laryngol Otol 1991;105:471-474.
Rosenwasser RH, Liebman EM, Jimenez DF, Buchheit WA, Andrews DW. Neurosurg 1991;29:568-574.
Scaramella LF. Anastomosis between the two facial nerves. Laryngoscope 1975;85:1359-1366.
Seiff SR, Chang J. Management of ophthalmic complications of facial nerve palsy. Otolaryngol Clin North Am
1992;25:669-690.
Wells MD, Manktelow RT. Surgical management of facial palsy. Otolaryngol Clin North Am 1990;17:645-653.
Whitney TM, Buncke JH, Alpert BS, Buncke GM, Lineaweaver WC. The serratus anterior free-muscle flap: experience
with 100 consecutive cases. Plast Reconstr Surg 1990;86:481-490.


medical literature.
THERAPEUTIC OPTIONS FOR TREATMENT

OF GLOMUS TUMORS OF THE TEMPORAL BONE
October 26, 1991
Judith J. Owens, M.D.
Glomus tumors arise from paraganglionic chemoreceptor cells and are part of the APUD system of cells
of neural crest origin. Paragangliomas of the jugular-tympanic area are the most common tumors
involving the middle ear. In 1941, Guild described the distribution of normal glomus bodies within the
temporal bone. Then in 1945, Rosenwasser correctly recognized and reported the first case of a tumor
arising from these cells. After this report, many new cases were reported and many older cases were
reclassified as true glomus tumors.
These neoplasms can occur at any age but the peak incidence is in the fifth decade. Glomus tumors occur
four to five times as often in women as in men and there is a predilection for them in the Caucasian race.
The majority of these tumors arise from along the jugular bulb or Jacobson's nerve (85%), while 12%
arise from glomus bodies overlying the promontory and three percent have their origin attributed to
Arnold's nerve. The most common presenting symptoms include conductive hearing loss and pulsatile
tinnitus. Other symptoms may include aural hemorrhage or otorrhea, otalgia and facial palsy. When the
tumors enlarge within the jugular foramen neuropathies of IX, X, XI and XII occur. Anterior expansion
toward the clivus may produce abducens and trigeminal palsies. Aquino's sign is blanching of the
tympanic mass with gentle pressure on the carotid artery. The sign of Brown is the pulsation sign elicited
by pneumatic compression and abolished with further compression. These tumors may be multicentric.
Association of these tumors with other tumors arising from cells of neural crest origin (MEN I and
pheochromocytoma) is not uncommon. This must be considered to avoid the devastating consequences of
inducing general anesthesia in a patient with an unrecognized pheochromocytoma. Malignancy occurs in
one to three percent of these tumors.
Evaluation includes a careful history and physical examination. High resolution computed tomography
provides accurate information about bony destruction and the extent of the disease. Arteriography
remains the most useful radiologic modality available for the assess-ment of a glomus tumor. Prior to
angiography, serum catacholamines and urinary vanillylmandelic acid (VMA) levels should be obtained
to screen for tumors which secrete these vasoactive compounds.
Therapeutic options for glomus tumors of the temporal bone include surgical excision, radiotherapy,
combined therapy and in selected cases observation. Surgical excision offers the only chance for total
tumor eradication. Gary Jackson writes that "...surgical management is curative and the treatment of
choice." Proponents of radiotherapy argue that radiation provides for the amelioration of symptoms and
arrests the progression of the glomus tumors providing a safe and efficacious modality of therapy.
Many surgical approaches have been proposed for the resection of these tumors (see bibliography). No
single approach is optimal for all tumors. Jackson and Glasscock proposed guidelines for a planned
excision of these tumors. They suggest approaching type I glomus tympanicum tumors via a transcanal
approach, and an extended facial recess approach for type II-IV glomus tympanicum tumors. Type I and
II glomus jugulare lesions are treated by standard skull base techniques. Type III and IV tumors require
an infratemporal fossa approach. The infratemporal fossa as described by Fisch provides for direct
surgical access to the entire length of the intratemporal carotid artery and for control of the great venous
sinuses. These infratemporal fossa approaches provide access as follows: Type A provides access to the
temporal bone in its infralabyrinthine and apical compartments and its inferior surface. Type B provides
access to the clivus and type C provides access to the parasellar region and nasopharynx. Utilizing these
skull base approaches it is now possible to perform total extirpation of tumors that were previously
considered unresectable. Skull base surgery is not undertaken without certain risks. Mortality rates range
from four to seven percent. Reported complications include CSF leaks, new cranial nerve palsies, wound
infections, and deafness. The risks of surgery are balanced out by the chance for total tumor eradication.
The reported rate of local control for glomus tumors of the temporal bone with surgical treatment from
several studies is shown in Table 4.
Although radiotherapy is unable to provide complete tumor eradication, the success of radiotherapy in
controlling the progression of symptoms is quite high. The reported rate of local control with radiation
therapy for glomus tumors of the temporal bone from several studies is shown in Table 5. Complications
of radiotherapy include osteoradionecrosis, brain necrosis, abscess formation, and radiation induced
malignancies.
Case Presentation
A 69-year-old black female had initially sought assistance from a hearing aid dispenser after
experiencing several years of slow, progressive left-sided hearing loss. However, a medical referral was
made when the presence of a unilateral conductive hearing loss and pulsatile mass on the tympanic
membrane were discovered. CT examination of the temporal bones demonstrated an opacification of the
left mastoid air cells and middle ear cavity.
She was then referred to Baylor College of Medicine for further evaluation and treatment. Upon
questioning, she reported that she could appreciate a rhythmic sound in her left ear that was synchronous
with her heart rate. Examination revealed a pulsating tympanic membrane with dilated vasculature that
extended onto the skin of the external canal. The sign of Aquino was negative and the Weber lateralized
to the left. There were no cranial nerve deficits. Arteriography was obtained. This demonstrated a
vascular mass of the left temporal bone supplied by the penetrating vessels of the external carotid artery.
These findings were consistent with a glomus tympanicum. The options of surgical resection,
radiotherapy and observation were explained to the patient. It was elected to observe the tumor for the
time being and to offer her radiotherapy should the tumor enlarge or become symptomatic.
Bibliography
Alford BR, Guilford FR: A comprehensive study of tumors of the glomus jugulare. Laryngoscope 72:765-787, 1962.
Aquino J: Glomus jugulare tumors. Arch Otolaryngol 65:263-268, 1957.
Bartels LJ, Gurucharri M: Pediatric glomus tumors. Otolaryngol Head Neck Surg 98:392-395, 1988.
Bataini J, Pontvert D, Jaulerry C, et al: Tumeurs du glomus jugulaire. Neurochirurgie 31:377-380, 1985.
Bojrab DI, Glasscock ME, Roland PS, et al: Glomus tumors of the temporal bone. Washington DC: AAO-HNS, 1988.
Boyle, JO, Shimm DS, Coulthard SW: Radiation therapy for paragangliomas of the temporal bone. Laryngoscope 100:896901, 1990.
Brackmann DE, Hitseleberger WE: Retrolabyrinthine approach technique and newer indications. Laryngoscope 88:286297, 1978.
Brandt TW, Jenkins HA, Coker NA: Facial paralysis as the initial presentation of an internal carotid artery aneurysm. Arch
Brismar J, Cronqvist: Therapeutic embolization in the external carotid artery region. Radiologica Diagnosis 19:713-731,
1978.
Brown, LA: Glomus jugulare tumor of the middle ear clinical aspects. Laryngoscope 53:281292, 1953.
Bundgaard T, Tandrup O, Elbrond O, et al: Treatment of glomus tumours. A retrospective survey. Clin Otolaryngol
14:155-160, 1989.
Cece JA, Lawson W, Biller HF: Complications in the management of large glomus jugulare tumors. Laryngoscope 97:152157, 1987.
Cole JM: Glomus jugulare tumor. Laryngoscope 87:1244-55, 1977.
Conley JJ: Multiple paragangliomas in the head and neck. Ann Otol Rhinol Laryngol 65:356-360, 1956.
Cummings BJ, Beale FA, Garrett PG: The treatment of glomus tumors in the temporal bone by megavoltage radiation.
Cancer 53:2635-2640, 1984.
Dawes PJ, Filippou M, Welch AR, et al: The management of glomus jugulare tumours. Clin Otolaryngol 12:15-24, 1987.
Dayal VS, Hinojosa R, Amenta CA: Surgical inferences from study of temporal bones with glomus jugulare tumor.
Dickens JW, Million RR, Singleton GT, et al: Chemodectomas arising in temporal bone structures. Laryngoscope 92:188191, 1982.
Ferrara P, Cimino A, Tortorici M: Role of radiation therapy in glomus tumor. Am J Otol 8:390-395, 1987.
Fisch U: Infratemporal fossa approach for extensive tumors of the temporal bone and base of the skull, in Silverstein H,
Norrell H (eds): Neurological Surgery of the Ear. Birmingham, Ala., Aesculapius, 1977.
Fisch U: Infratemporal fossa approach for glomas tumors of the temporal bone. Ann Otol Rhinol Laryngol 92:474-479,
1982.
Fisch U: La voie d'abord infratemporale pour les tumeurs du glomus jugulaire. Neurochirurgie 31:367-376, 1985.
Fisch U, Fagan P, Valavanis A: The infratemporal fossa approach for the lateral skull base. Otolaryngol Clin North Am
17:513-552, 1984.
Franklin DJ, Jenkins HA, Horowitz, BL: Management of petrous apex lesions. Arch Otolaryngol 115:1121-1125, 1989.
Fuller AM, Brown HA, Harrison, EG: Chemodectomas of the glomus jugulare tumors. Laryngoscope 77:218-238, 1967.
Gardner G, Cocke EW, Robertson, JT et al: Glomus jugulare tumours - combined treatment. J Laryngol Otol 95:437-580,
1981.
Glasscock ME, Harris PF, Newsome G: Glomus tumors: Diagnosis and treatment. Laryngoscope 84:2006-2032, 1974.
Glassock ME, Smith PG, Bond AG: Management of aneurysms of the petrous portion of the internal carotid artery by
resection and primary anastomosis. Laryngoscope 93:1445-1453, 1983.
Guild SR: The glomus jugulare, a nonchromaffin paraganglion, in man. Ann Otol Rhinol Laryngol 62:1045-1071, 1953.
Gulya AJ, Glasscock ME, Jackson CG, et al: Skull-base surgery: Operative refinements. Otolaryngol Head Neck Surg
94:315-320, 1986.
Hatfield PM, James AE, Schulz MD: Chemodectomas of the glomus jugulare. Cancer 30:1164-1168, 1972.
House, WF: Surgical exposure of the internal auditory canal and its contents through the middle cranial fossa.
Laryngoscope 71:1363-1385, 1961.
House WF, Glasscock ME: Glomus tympanicum tumors. Arch Otolaryngol 87:124-128, 1968.
House WF, Hitselberger WE: The transcochlear approach to the skull base. Arch Otolaryngol 102:334-342, 1976.
Hudgins PT: Radiotherapy for extensive glomus. Radiology 103:427-429, 1972.
Hurst WB: Glomus jugulare tumours. J Laryngol Otol 95:581-588, 1981.
Iaccarino V, Sodano A, Belfiore G, et al: Embolization of glomus tumors of the carotid: Temporary or definitive?
Cardiovasc Intervent Radiol 8:206-210, 1985.
Jackson CG: Skull base surgery. Am J Otol 3:161-171, 1981.
Jackson CG, Glasscock ME, Harris PF: Glomus tumors: Diagnosis, classification, and management of large lesions.
Otolaryngol 108:401-406, 1982.
Jackson CG, Glasscock ME, Nissen AJ: Glomus tumor surgery: The approach, results, and problems. Otolaryngologic
Clin North Am 15:897-902, 1982.
Jackson CG, Glasscock ME, McKennan KX: The surgical treatment of skull-base tumors with intracranial extension.
Jackson CG, Welling DB, Chironis P: Glomus tympanicum tumors: Contemporary concepts in conservation surgery.
Laryngoscope 99:875-916, 1989.

Jenkins HA, Fisch U: Glomus tumors of the temporal region. Otolaryngol 107:209-214, 1981.
Kennedy DW, Nager GT: Glomus tumor and multiple endocrine neoplasia. Otolaryngol Head Neck Surg 94:644-648,
1986.
Kim J, Elkon D, Lim M, et al: Optimum dose of radiotherapy for chemodectomas of the middle ear. Int J Radiation
Oncology Biol Phys 6:815-819, 1980.
Makek M, Franklin DJ, Zhao J, et al: Neural infiltration of glomus temporale tumors. Am J Otol 11:1-5, 1990.
Marsh MA, Jenkins HA, Coker NJ: Extended lateral cranial base surgery, in Cummings DW, et al: Otolaryngology - Head
and Neck Surgery. St. Louis: Mosby, in press.
McCabe B, Flether M: Selection of therapy of glomus jugulare tumors. Arch Otolaryngol 89:182-185, 1969.
Moffat DA, Hardy DG: Surgical management of large glomus jugulare tumours: Infra-and trans-temporal approach. J
Laryngol Otol 103:1167-1180, 1989.
Murphy TP, Brackmann DE: Effects of preoperative embolization on glomus jugulare tumors. Laryngoscope 99:12441247, 1989.
Ossama A, Kersh JE, Routh A, et al: The long-term side effects of radiation therapy for benign brain tumors in adults. J
Neurosurg 73:502-512, 1990.
Pellet W, Cannoni M, Pech A: The widened transcochlear approach to jugular foramen tumors. J Neurosurg 69:887-894,
1988.
Pilepich MV, Konefal JB, Perez CA, et al: Radiation therapy in the treatment of chemodectomas. Laryngoscope 97:13311338, 1987.
Pryzant RM, Chou JL, Easley JD: Twenty year experience with radiation therapy for temporal bone chemodectomas. Int J
Radiation Oncology Biol Phys 17:1303-1307, 1989.
Rosenwasser H: Carotid body tumor of the middle ear and mastoid. Arch Otolaryngol 41:64-7, 1945.
Rosenwasser H: Current management glomus jugulare tumors. Ann Otol Rhinol Laryngol 76:603-10, 1967.
Rosenwasser H: Glomus jugulare tumors. Arch Otolaryngol 88:37-77, 1968.
Rosenwasser H: Glomus jugulare tumors. Arch Otolaryngol 89:186-192, 1969.
Rosenwasser H: Metastasis from glomus jugulare tumors. Arch Otolaryngol 67:197-203, 1957.
Schwaber MK, Glasscock ME, Nissen AJ, et al: Diagnosis and management of catecholamine secreting glomus tumors.
Laryngoscope 94:1008-1015, 1984.
Shambraugh GE: Surgical approach for so-called glomus jugulare tumors of the middle ear. Laryngoscope 65:185-198,
1955.
Shapiro MJ, Neues DK: Technique for removal of glomus jugulare tumors. Arch Otolaryngol 79:219-224, 1961.
Sharma D, Johnson AP, Whitton AC: Radiotherapy for jugulo-tympanic paragangliomas. J Laryngol Otol 98:621-629,
1984.
Simpson GT, Konrad HR, Takahashi M, et al: Immediate postembolization excision of glomus jugulare tumors.
Otolaryngol 106:1979.
Spector GJ, Maisel RH, Ogura JH: Glomus tumors in the middle ear. I. An analysis of 46 patients. Laryngoscope 83:16521672, 1973.
Spector GJ, Maisel RH, Ogura JH: Glomus jugulare tumors II. a clinicopathologic analysis of the effects of radiotherapy.
Ann Otol 83:26-36, 1974.
Spector GJ, Compagno J, Perez CA: Glomus jugulare tumors: Effects of radiotherapy. Cancer 35:1316-1321, 1975.
Spector GJ, Sobol S: Surgery for glomus tumors at the skull base. Otolaryngol Head Neck Surg 88:524-530, 1980.
Springate SC, Weichselbaum RR: Radiation or surgery for chemodectoma of the temporal bone: A review of local control
and complications. Head Neck Surg 303-307, 1990.
Taylor DM, Alford BR, Greenberg SD: Metastases of glomus jugulare tumors. Arch Otolaryngol 82:5-13, 1965.
Tidwell TJ, Montague ED: Chemodectomas involving the temporal bone. Radiology 118:147-149, 1975.
Wang M, Hussey DH, Doornbos JF: Chemodectoma of the temporal bone: A comparison of surgical and radiotherapeutic
results. Int J Radiation Oncology Biol Phys 14:643-648, 1988.
Zinreich ES, Lee D: Radiotherapy for the treatment of paragangiomas in the temporal bone. Ear Nose Throat J 65:181-4,
1986.


medical literature.
CONSIDERATIONS IN THE EVALUATION OF THE HEARING

IMPAIRED CHILD
Judith J. Owens, MD
March 11, 1993
Approximately 5,000 children are born every year in the United States who will be found to have a
significant hearing impairment. The hearing loss may be secondary to congenital or postnatally acquired
conditions. Congenital hearing loss is attributed to a defect that the child is born with, either an inherited
genetic defect or the result of a prenatally acquired condition. The important non-hereditary causes of
congenital hearing loss include drug exposure, prenatal infections (TORCH) and erythroblastosis fetalis.
Three-quarters of childhood hearing impairment is postnatally acquired. Infections, drugs,
hyperbilirubinemia, noise exposure, and trauma have all been implicated in contributing to postnatally
acquired hearing loss. In addition to meningitis and sepsis, the important infectious diseases associated
with postnatally acquired hearing loss include adenovirus, mumps, chicken pox, hepatitis, EBV, and
influenza. Hyperbilirubinemia when greater than 20 mg % will result in damage to the cochlear nucleus.
Although most hereditary disorders appear to follow the rules of Mendelian inheritance, marked
variability of gene expression and incomplete penetrance may confound the diagnosis and clinical
recognition of many of these disorders. A complete family history is essential to uncover these types of
hearing loss. This is especially true for nonsyndromic inherited hearing loss. The hearing loss may be
present at birth or may develop later in life. All forms of inheritance, autosomal dominant, autosomal
recessive, and X-linked, have been documented in families with nonsyndromal hereditary hearing
impairment (HHI).
Hearing loss is a component of over 200 inherited syndromes. Many syndromes in which hearing loss is
a significant component were reviewed for this presentation. The major features of some of these
syndromes will be reviewed in this abstract.
For convenience these syndromes can be categorized by the association with other morphologic
abnormalities. Important syndromes where deafness is associated with craniofacial abnormalities include
Treacher-Collins, Goldenhar's, Crouzon's, and Apert's syndromes. All except Goldenhar's (AR) are
inherited in an autosomal dominant fashion. Hearing loss in these syndromes is mixed or conductive and
may be surgically correctable.
Hearing loss in association with visual loss is a particularly devastating combination. There are four
important syndromes with this combination: Usher's, Alstrom's, Cockayne's and Refsum's disease. All
are inherited in an autosomal recessive fashion. Retinitis pigmentosa is associated with all except for
Cockayne's syndrome. There are four types of Usher's syndrome. Type I is the most severe and is
associated with vestibular dysfunction
Apert's syndrome consists of progressive renal insufficiency, progressive sensorineural hearing loss and
various ocular abnormalities. This syndrome demonstrates X-linked dominant inheritance: males are
more severely affected and females may have a variable course to their disease because of random X
chromosome inactivation.
Jervell and Lange-Nielsen syndrome is the most common syndrome with cardiac dysfunction and
hearing loss. There is a conduction defect resulting in a prolonged QT interval on electrocardiogram, and
clinically with fainting spells or sudden death. Jervell and Lange-Nielsen is inherited in an autosomal
recessive manner.
Pendred's syndrome is the most frequently occurring example of associated deafness and endocrine
dysfunction. This disorder results from an error in thyroxin metabolism. A goiter may be clinically
palpable and profound sensorineural hearing loss is usually present.
Dermal abnormalities and deafness characterize Waardenburg's syndrome and the LEOPARD syndrome.
Clinical features of Waardenburg's include dystopia canthorum, deafness, a high broad nasal root,
synophrys, heterochromia irides, and early graying. There are two types of this syndrome characterized
by the presence or absence of dystopia canthorum. When dystopia canthorum is absent, but the other
features of the syndrome are present, there is a higher incidence of deafness.
Metabolic syndromes, especially the mucopolysaccharidoses, are frequently associated with conductive
or mixed hearing loss. Chromosomal abnormalities, specifically trisomy 13 and 18 are often associated
with deafness. Because these conditions are lethal in early infancy the deafness is of relatively minor
significance. Down's syndrome is often associated with a mixed hearing loss.
Early identification of hearing loss is desirable to optimize rehabilitation. Identification of hearing loss in
young children is facilitated by a clinician who looks carefully for a family history of early hearing loss,
and who is attuned to the many syndromes associated with hearing impairment. Complete otologic and
auditory evaluation of these children is essential.
Case Presentation
An eight-month-old child presented to Texas Children's Hospital for an otologic evaluation. The child
was born full term via an uncomplicated vaginal delivery. There was no history of infections, drug or
alcohol use during the pregnancy. Both parents are hearing- impaired. The mother reported the etiology
of her deafness as "tonsillitis" and the etiology of her husband's hearing loss as "high fever." On physical
examination the child demonstrated multiple stigmata of Waardenburg syndrome including dystopia
cantorum, heterochromia irides, a broad nasal root, and synophrys or confluence of the eyebrows. The
mother has similar features and in addition has a white forelock that is also associated with this
syndrome.
Audiologic evaluation revealed no responses to speech, warble tones, or narrow band noise in the sound
room. Auditory brainstem response audiometry showed no response to air or bone conducted clicks at
equipment levels in either ear. In light of her significant sensitivity loss she was referred for hearing aid
evaluation.
Despite the fact that both parents are hearing-impaired and communicate through signing, they were
anxious to have the child fitted with hearing aids. The parents were referred for genetic counselling to
provide them with a better understanding of the full implications of this disease.
Bibliography
Considerations in the Evaluation of the Hearing-Impaired Child

Alford BR. Rubella - la bte noire de la mdecine. Laryngoscope 1968;78:1623-1659.
Alport AC. Hereditary familial congenital haemorrhagic nephritis. Br Med J 1927;1:504-506.
Anagnostakis D, Petmezakis J, Papazissis G, Messaritakis J, Matsaniotis N. Hearing loss in low-birth-weight infants. Am J
Dis Child 1982;136:602-607.
Andreoli SP, Deaton M. Alport's syndrome. Ear Nose Throat J 1992;71:508-511.
Batsakis JG, Nishiyama RH. Deafness with sporadic goiter: Penred's syndrome. Arch Otolaryngol 1962;76:401-406.
Cockayne EA. Dwarfism with retinal atrophy and deafness. Arch Dis Child 1936;11:1-8.
Crysdale WE. Otorhinolaryngologic problems in patients with craniofacial anomalies. Otolaryngol Clin North Am
1981;14:145-155.
d'Avignon M, Barr B. Ear abnormalities and cranial nerve palsies in thalidomide children. Arch Otolaryngol 1964;80:136140.
Dimitrius A, Petmezakes J, Papazesses G, et al. Hearing loss in low birth weight infants. Am J Dis Child 1982;136:602604.
Dodge PR, Hallowell D, Feigin RD, Holmes SJ, Kaplan SL, Jubelirer DP, et al. Prospective evaluation of hearing
impairment as a sequela of acute bacterial meningitis. N Engl J Med 1984;311:869-874.
Everberg G. Deafness following mumps. Acta Otolaryngol 1957;48:397-403.
Feinmesser M, Landau J. Deafness in toxoplasmosis. J Laryngol 1961;75:171-174.
Fisch L. Deafness as part of an hereditary syndrome. J Laryngol Otol 1959;73:355=82.
Fraser GR. The causes of profound deafness in childhood. Baltimore: Johns Hopkins University Press, 1976:11-48.
Fraser GR. Profound childhood deafness. J Med Genet 1964;1:118-151.
Friedmann I, Fraser GR, Froggatt P. Pathology of the ear in the cardio-auditory syndrome of Jervell and Lange-Nielsen
(recessive deafness with electrocardiographic abnormalities). J Laryngol Otol 1966;80:451-470.
Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head and neck, 3rd edition. NY: OUP, 1990.
Grundfast KM, Lalwani AK. Practical approach to diagnosis and management of hereditary hearing impairment (HHI).
Ear Nose Throat J 1992;71:479-493.

Hageman MJ, Delleman JW. Heterogeneity in Waardenburg syndrome. Am J Hum Genet 1977;29:468-485.
Hanshaw JB. Congenital cytomegalovirus infection: a fifteen year perspective. J Infect Dis 1971;123:555-561.
Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis.
Laryngoscope 1987;97(Suppl 40):2-14.
Jervell A, Lange-Nielsen F. Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval, and
sudden death. Am Heart J 1957;54:59-68.
Johnsen T, Larsen C, Friis J, Hougaard-Hensen F. Pendred's syndrome: acoustic, vestibular and radiological findings in 17
unrelated patients. J Laryngol Otol 1987;101:1187-1192.
Kelemen G. Hurler's syndrome and the hearing organ. J Laryngol Otol 1966;80:791-803.
Kloepfer HW, Laguaite JK. The hereditary syndrome of congenital deafness and retinitis pigmentosa (Usher's syndrome).
Konigsmark BW. Hereditary congenital severe deafness syndromes. Ann Otol Rhinol Laryngol 1981;80:269-288.
Konigsmark BW. Hereditary deafness in man. N Engl J Med 1969;281:713-720,774-778,827-832.
Konigsmark BW, Gorlin RJ. Genetic and metabolic deafness. New York: WB Saunders, 1976.
Kos AO, Schuknecht HF, Singer JD. Temporal bone studies in 13-15 and 18 trisomy syndromes. Arch Otolaryngol
1966;83:439-445.
MacDonald WB, Firch KD, Lewis IC. Cockayne's syndrome. Pediatr 1960;25:997-999.
Matthews NL. Lentigo and electrocardiographic changes. N Engl J Med 1968;278:780-781.
McLay K, Maran AGD. Deafness and the Klippel-Feil syndrome. J Laryngol Otol 1969;83:175-184.
Nance WE, Setleff RC, McLeod A, et al. X-linked mixed deafness with congenital fixation of the stapedial footplate and
perilymphatic gusher. Birth Defects 1971;7:64-69.
Paparella MM, Fox RY, Schachern PA. Diagnosis and treatment of sensorineural hearing loss in children. Otolaryngol
Clin North Am 1989;22:51-74.
Pass RF, Stagno S, Myers GJ, Alford CA. Outcome of symptomatic congenital cytomegalovirus infection: results of longterm longitudinal follow-up. Pediatr 1980;66:758-762.
Peckham CS, Stark O, Dudgeon JA, et al. Congenital cytomegalovirus infection: a cause of sensorineural hearing loss.
Arch Dis Child 1987;62:1233-1237.

Pendred V. Deaf-mutism and goitre. Lancet 1896;2:532.
Preus M, Fraser FC. Genetics of hereditary nephropathy with deafness (Alport's disease). Clin Genet 1971;2:331-337.
Quisling RW, et al. Osteogenesis imperfecta. A study of 160 family members. Arch Otolaryngol 1979;105:207-211.
Refsum S. Heredopathia atactica polyneuritis formis. Acta Psychiatr Scand 1946;38:1-303.
Reynolds DW, Stagno S, Stubbs G, Dahle AJ, Livingston MM, Saxon SS, et al. Inapparent congenital cytomegalovirus
infection with elevated cord IgM levels. N Engl J Med 1974;290:291-296.
Richards BW, Runderl AT. A familial hormonal disorder associated with mental deficiency, deaf-mutism, and ataxia. J
Ment Defic Res 1959;3:33-55.
Robinson GC, Cambon KG. Hearing loss in infants of tuberculous mothers treated with streptomycin during pregnancy. N
Engl J Med 1964;271:949-953.
Scheibe A. A case of deaf-mutism, with auditory atrophy and anomalies of development in the membranous labyrinth of
both ears. Arch Otolaryngol 1892;21:120-122.
Shiller JG. Craniofacial dysostosis of Crouzon. Pediatr 1959;23:107-112.
Schuknecht HF. Pathology of the ear. Cambridge, MA: Harvard University Press, 1974;165-209.
Smith RJH. Medical diagnosis and treatment of hearing loss in children. In: Cummings CW, Harker LA, editors.
Otolaryngology - Head and Neck Surgery. St. Louis: CV Mosby, 1986;3225-3246.
Smith RJH. A DNA linkage study of Usher's syndrome excluding much of chromosome 4. Laryngoscope 1989;99:940949.
Waardenburg PJ. A new syndrome combining developmental anomalies of the eyelids, eyebrows and nose root with
pigmentary defects of the iris and head hair and with congenital deafness. Am J Hum Genet 1951;3:195-253.
Wahl RA. Dick M. Congenital deafness with cardiac arrhythmias: the Jervell and Lange-Nielsen syndrome. Am Ann Deaf
1980;125:34-37.


medical literature.
UPDATE ON HEARING AIDS

Michael G. Stewart, MD
June 3, 1993
Approximately 22 million Americans have some degree of hearing loss.
Currently, patients with sensorineural hearing loss represent approximately 90%
of hearing aid users. The sales of hearing aids in the United States have grown
steadily, from 830,000 units in 1981 to 1,780,000 units in 1992. Behind-the-ear
(BTE) aids represent 18.5% of this total, with body and eyeglass aids accounting
for only 0.4%; the remaining 81.1% sold are in-the-ear (ITE) aids. This is a
distinct change from only ten years ago, when ITE and BTE aids each represented
about 50% of the market.

Electronic hearing aids consist of a microphone, an amplifier, and a receiver. The
microphone transduces sound into an electrical signal and sends it to the
amplifier. The amplifier increases the amplitude of the electrical signal, and the
signal may be further modified by filters and volume or tone controls. The
amplified signal is transmitted to the receiver, where it is transduced into sound.
When the amount of amplification is expressed in decibels, it is called the gain of
the aid.
One complaint of most patients with cochlear loss is a decrease in dynamic range,
also called cochlear recruitment. This is because the loudest sounds are still
perceived with the same intensity, whereas patients with cochlear loss are unable
to hear quiet sounds. Therefore the range between comfortable listening and
uncomfortable loudness is much smaller than in normal subjects. Aids must then
be able to supply adequate gain to the user without creating sounds which are too
loud. Most aids sold today use linear amplifiers, which increase the signal
amplitude by a constant amount, regardless of input level. These aids then require
some kind of output limiting - such as peak clipping or compression amplification
- to differentially amplify soft sounds more than loud sounds.
Earmold design is an important factor in aid design. With BTE type aids, a
silicone earmold is used to deliver sound to the ear. With ITE type aids, the aid
itself fits into the EAC. In both cases, the aid potentially acts as an earplug and
creates the so-called "occlusion effect." Completely blocking the canal results in
an increased amplification and perception of low frequencies, especially in the
sound of the wearer's own voice. One solution to this problem is venting, or
making an opening in the earmold or aid, which attenuates (or decreases) the
amplification of low frequencies. However, venting may create a problem of its
own: feedback. Feedback occurs when a microphone is placed very near a
speaker. The microphone picks up the speaker output, which is amplified and sent
to the speaker, which is picked up by the microphone and amplified again, etc.
The capacity of the amplifier is quickly overloaded, and the high-pitched sound
called feedback results. Feedback is an inherent problem with ITE aids because
the microphone and speaker are on the same piece of equipment. Venting may
further decrease the amount of gain possible before the microphone picks up the
speaker output and feedback results. Feedback is a less significant problem in
BTE aids because the microphone and speaker are physically separated.
There are several types of aids available. Body aids are seldom used today, but
have very high gain possible because of the separation of microphone and
speaker. Body aids are still used for some patients with profound hearing gloss.
Eyeglass aids allow the fitting of CROS and BICROS hearing aids. CROS aids
are helpful for patients with unaidable loss in one ear only, and BICROS aids are
used for a combination of unaidable loss in one ear and aidable loss in the other.
In BTE aids, the controls, microphone and amplifier are in the body, and sound is
transduced through an earmold. There are three varieties of ITE aids: the fullconcha aid, the half-concha aid, and the canal aid. Typically ITE and BTE aids
have 65 to 75 dB of gain, and body aids may have 80 dB or higher gain.
The latest technology in hearing aids is the digital aid. In the past, electronic aids
have all used analog circuitry, where the sound is transformed into an electrical
signal and then further modified. There are natural limitations in the amplifier's
ability to amplify the signal without exceeding power limitations or introducing
distortion. In digital technology, the electrical waveform is converted into a
digital code of a series of ones and zeros. This digital signal is then modified by
computer, and transformed back into an electrical signal and sent to the speaker.
The advantage of digital processing is that flexibility is much greater. The digital
waveform can be transformed in complex ways without loss of precision, and the
natural limitations of electronic equipment are not imposed. Specifically, the
frequency response can be adjusted more accurately, the output can be limited at
any frequency to prevent uncomfortable sound levels, filtering is more accurate,
noise is not introduced, and acoustic feedback can be controlled. These
modifications result in the more "natural" sound of digital aids, according to
many aid users.
Although hearing aids are tested and have published specifications, these are
obtained by placing the aid in a standardized device called a 2 cm coupler, and
measuring performance. Real ear testing involves the placement of a tiny probe in
the patient's ear canal. The frequency response of the unaided ear canal is
measured, then the aid is placed, and the aided frequency response is measured
using different sound inputs.
ASSISTIVE LISTENING DEVICES
Many patients have a mild to moderate hearing loss or a narrow band of
frequency loss (especially high-frequency loss) that makes hearing aid use
difficult. In addition, some patients do not have the manual or mental dexterity to
use hearing aids. Assistive listening devices are designed to improve hearing
ability in special listening situations - especially if background noise, multiple
talkers or large distances are a problem. There are several types of assistive
devices.
A hard-wired device uses a microphone close to the source which is directly
connected to an amplifier and earphone that the patient wears. The AM radio
signal has been used as a wireless system to transmit sound from the source to
individual receivers. However, AM signal is limited in power and range, and is
very sensitive to electrical disturbances and electronic equipment. Wireless FM
systems are much more popular because FM provides a clear signal that is almost
immune to outside interference. Finally, infrared light has been used as a signal
carrier. Infrared light is invisible, but requires an unobstructed view to the
receiver, and is interfered with by some other sources of light.
These assistive listening devices (ALDs) are all based on the principle of
improving signal to noise ratio (SN ratio), which means that the desired sound is
amplified with respect to surrounding noise. This is very useful for hearingimpaired persons in lecture halls, school, or church, or for such activities as TV
viewing with a large group of people. ALDs may also be used in conjunction with
a hearing aid.
Case Presentation
A 48-year-old male veteran suffered noise exposure while in the service. He was
fitted with a right-side monaural behind-the-ear aid in 1965 while in the military.
He has been followed at the Houston Veterans Affairs Medical Center Audiology
Clinic since 1972, preferring right ear only hearing aid use. His most recent
audiogram shows a stable bilateral moderate to severe sensorineural hearing loss
from low to high frequencies. His discrimination was 80% in the right ear, and
70% in the left. Acoustic reflex thresholds were elevated, but consistent with the
extent of the sensitivity loss, and tympanometry revealed Type A tympanograms.
The patient was recently fitted with bilateral in-the-ear hearing aids, which the
patient uses regularly with good results. His real ear testing reveals excellent
tuning to the computer-simulated parameters.
Bibliography
Berger KW. History and development of hearing aids. In: Pollack MC, editor. Amplification for the Hearing Impaired, 3rd
ed. Orlando: Grune & Stratton, 1988:1-20.
Byrne D. Key issues in hearing aid selection and evaluation. J Am Acad Audiol 1992;3:67-80.
Carver WF. Development of the hearing aid and the hearing aid industry. In: Hodgson WR, Skinner PH, editors. Hearing
Aid Assessment and Use in Audiologic Habilitation. Baltimore: Williams and Wilkins, 1977:5-16.
Cheple M. Hearing aids. In: Paparella MM, et al. Otolaryngology, 3rd ed. Philadelphia: W.B. Saunders, 1991:1017-1024.
Cole WA. Current design options and criteria for hearing aids. JSLPA Monograph 1993;Suppl 1:7-14.
Cranmer-Briskey KS. 1992 Hearing Instruments dispenser survey results. Hear Instrum 1992;(12)43:8-15.
Elfenbein JL, Bentler RA, Davis JM, Niebuhr DP. Status of school children's hearing aids relative to monitoring practices.
Ear Hear 1988;9:212-217.
Gelfand SA, Silman S. Usage of CROS and IROS hearing aids by patients with bilateral high-frequency hearing loss. Ear
Hear 1981;3:24-29.
Harris RW, Brey RH, Chang Y-S, Soria BD, Hilton LM. The effects of digital quantization error on speech intelligibility
and perceived speech quality. J Speech Hear Res 1991;34:189-196.
Hecox KE. Digital hearing aid technology: medical perspective. Otolaryngol Clin North Am 1989;22:129-142.
Hecox KE, Punch JL. The impact of digital technology on the selection and fitting of hearing aids. Am J Otol 1988;9:7785.
Jerger J, Speaks C, Malmquist C. Hearing-aid performance and hearing-aid selection. J Speech Hear Res 1966;9:136-149.
Jerger J, Malmquist C, Speaks C. Comparison of some speech intelligibility tests in the evaluation of hearing aid
performance. J Speech Hear Res 1966;9:253-258.
Jerger J, Silman S, Lew HL, Chmiel R. Case studies in binaural interference: converging evidence from behavioral and
electrophysiologic measures. J Am Acad Audiol 1993;4:122-131.
Kirkwood DH. 1992 U.S. hearing aid sales: latest HIA reports show lull in market growth. Hear J 1992;45(12):7-15.
Kuk FK. Amplification devices for the hearing-impaired individual. In: Cummings CW, et al. Otolaryngology--Head and
Neck Surgery, 2nd ed. St. Louis: C.V. Mosby, 1993:3127-3141.
Letowski TR, Richards WD, Burchfield SB. Transmission of sound, vibration through earmold materials. Hear Instrum
1992;43(12):11-15.
Levitt H. Future directions in signal processing hearing aids. Ear Hear 1991;12(6 suppl):125S-130S.
Murray DJ, Hanson JV. Application of digital signal processing to hearing aids: a critical survey. J Am Acad Audiol
1992;3:145-152.
Pehringer JL: Assistive devices: technology to improve communication. Otolaryngol Clin North Am 1989;22:143-174.
Pehringer JL, Schirber CM: Assistive devices: new opportunities for rehabilitating hearing impairment. In: Paparella MM,
et al. Otolaryngology, 3rd ed. Philadelphia: W.B. Saunders, 1991:1025-1035.
Podoshin L, Kremer M, Fradis M, Heiglin H. Effect of hearing aids on hearing. Laryngoscope 1984;94:113-117.
Revit LJ. Two techniques for dealing with the occlusion effect. Hear Instrum 1992;43(12):16-18.
Sammeth CA, Ochs MT. A review of current "noise reduction" hearing aids: rationale, assumptions, and efficacy. Ear
Hear 1991; 12(6 suppl):116S-124S.
Skinner MW. Recent advances in hearing aid selection and adjustment. Ann Otol Rhinol Laryngol 1984;93:569-575.
Smriga DJ, Huber TP, Paparella MM. Developments in hearing aid fitting and delivery: a decade of revolution.
Otolaryngol Clin North Am 1989;22:105-127.
Stach BA, Loiselle LH, Jerger JF. Special hearing aid considerations in elderly patients with auditory processing disorders.
Ear Hear 1991;12(6 suppl):131S-138S.

Surr RK, Montgomery AA, Mueller HG. Effect of amplification on tinnitus among new hearing aid users. Ear Hear
1985;6:71-75.
Tecca JE. Further investigation of ITE vent effects. Hear Instrum 1992;43(12):8-10.
Tyler RS. What can we learn about hearing aids from cochlear implants? Ear Hear 1991;12(6 suppl):177S-186S.
Vernon J, Griest S, Press L. Attributes of tinnitus and the acceptance of masking. Am J Otolaryngol 1990;11:44-50.

&REG; Copyright, 1995-7. All Rights Reserved. Last modified: July 28, 2000 (ASM)
Baylor College of Medicine. The Bobby R. Alford Department of Otorhinolaryngology and Communicative Sciences.

Herpes Zoster Oticus

August 21, 1997
F. Christopher Holsinger, M.D.
History
Herpes zoster has been known since antiquity. Herpes is derived from the
Greek meaning to creep and zoster sword belt or girdle. The Greeks
called it zona and for centuries thereafter the disease was thought of as a
cutaneous process. By the nineteenth century, physicians had begun to
question this understanding.
Bright (1831) and Henley (1840) first suggested that herpes zoster
represented a cutaneous manifestation of a nerve disease involving the
sensory portion of the spinal nerves. Dr. von Barensprung (1861)
demonstrated definitely at autopsy inflammatory lesions at the posterior root
ganglion in a patient with herpes zoster.
Numerous observers described cases of herpetic eruption at the cephalic
extremity due to herpetic infections involving the various cranial nerve
ganglia or the upper cervical spinal root ganglia. Tryde gave the first
description of cases of herpes zoster associated with facial palsy in 1872.
Head and Campbell published their landmark study in 1900. By correlating
autopsy findings and careful, detailed clinical descriptions, these
investigators mapped the sensory dermatomes defined by the dorsal root
ganglia. Their work laid the foundation for future work and focused the
energy and interest of others in this area.
Koerner, writing in the 1904, coined the term herpes oticus designating a
syndrome that consists of the triad: blisters at the auricle, facial paralysis,
and inner ear disturbances.
In 1907, Dr. Hunt, chief of Neurological Sciences at the Cornell University
Medical Center, published his hypothesis regarding the etiology of herpes
zoster oticus. Rather than presenting a new theoretic construct for disease,
Dr. Hunts theory advanced the work begun by Drs. Head and Campbell
and accurately classified herpes zoster of the head and neck. His paper
represented the capstone of nearly a century of medical research and
debate in this area. Hunt felt that herpetic eruption and associated
symptoms (a viral prodrome, severe otalgia, facial nerve dysfunction,
vesicular eruption involving the pinna, and occasionally vestibulocochlear
symptoms), which he was the first to describe together as part of a clinical
syndrome, were actually the result of, in his words, geniculate ganglionitis.
Hunt analyzed the sensory innervation of the ear and described the zoster
zone for the geniculate ganglion as comprising a portion of the tympanic
membrane, the external auditory canal, the tragus, antitragus, concha, a
part of the helix, and a strip of the lobule. Here the typical herpetic lesions
of geniculate zoster are most commonly found. The geniculate zone of the
auricle is bounded anteriorly by the gasserian zone and posteriorly by the
cervical zone, with some overlapping. Hunt classified herpes zoster at the
cephalic extremity according to the sensory ganglion involved and the site
of the rash into the following groups: (1) geniculate herpes zoster (herpes
oticus), (2) gasserian herpes zoster (herpes facialis), and (3) cervical
herpes zoster (herpes occipito-collaris)
Hunt then further classified the herpes zoster oticus syndrome according to
severity of symptoms and the degree of extension: (1) herpes zoster
auricularis without neurological signs, (2) herpes zoster oticus with facial
palsy, (3) herpes zoster oticus with facial palsy and mild acoustic symptoms
(diminished hearing and tinnitus), and (4) herpes zoster oticus with facial
palsy and Mnires syndrome complex (deafness, tinnitus, vertigo,
nystagmus, nausea, and vomiting). Ramsay Hunt described not so much a
discrete clinical syndrome but rather a spectrum of herpetic disease,
involving the ear and cranial nerves VII and VIII.
To support this classification scheme, he postulated that the major site of
the pathologic lesion in herpes zoster oticus was the geniculate ganglion of
the facial nerve. Increased pressure of the swollen ganglion on the facial
nerve resulted in facial palsy. He further postulated that the etiology of the
Mnires syndrome complex was either the simultaneous infection of the
adjacent ganglions of the eighth nerve or the extension of the inflammatory
process from the facial to the auditory nerve by means of nerve fibers that
communicate between the two. He believed that this clinical syndrome
represented geniculate ganglionitis. Unfortunately, he was not able to
provide evidence for his theory. With histopathologic studies of autopsy
speciments, subsequent investigators demonstrated little, if any, ganglion
involvement.
Denny-Brown and his colleagues in 1944 were the first to challenge Hunts
theory. They examined histopathologic specimens from a patient with
auricular and occipital herpes zoster and facial palsy. At autopsy, they
found no changes in the geniculate ganglion, but were able to demonstrate
other significant findings: a necrotizing ganglionitis of the second cervical
ganglion and a patchy motor neuritis of the facial nerve. They concluded
the evidence for geniculate ganglionitis in the Ramsay Hunt syndrome is
invalid.
In time, virology research further clarified these questions. Tissue culture
studies published by Drs. Weller and Coons in 1954 confirmed the theory
that the virus that causes varicella and herpes zoster is one in the same. In
1965, Hope-Simpsons novel hypothesis unified thinking on herpes zoster
and has laid the foundation for our current understanding. He suggested
that the varicella virus lies dormant in the sensory ganglion after the initial
infection. In a patient with low circulating antibodies, the virus would
reactivate and infection would then manifest itself as herpes zoster,
anywhere along the distribution supplied by that particular ganglion. Indeed,

the facial nerve may be affected at any site from the brainstem to the
periphery by the herpes virus. What is still not known is what activates the
virus after it has lain dormant for so long.
In 1967, Blackley presented histologic evidence to support this emerging
notion. He found extensive lymphocytic infiltration along the course of the
facial nerve, as well as divisions of the VIII cranial nerve including vestibular
and cochlear ganglia. Massive lymphocytic infiltration of the right facial
nerve was seen. In addition, there was collapse and disruption of
Reissners membrane, an increase of round cells in the stria vascularis, and
destruction of the organ of Corti near the apical turn. Broad perivascular
cuffing of several vessels is seen and marked atrophy of spiral neurones.
Epidemiology
A Mayo clinic study estimated the annual incidence of herpes zoster,
regardless of site, as 130 cases per 100,000. The attack rate increased
dramatically over the age of 60, and 10% this population had identifiable
risk factors for decreased cell-mediated immunity including carcinoma,
trauma, radiation therapy, or chemotherapy. The increased incidence in the
elderly population is explained by a decrease in cellular immune response
to varicella-zoster virus with age.
Adour reported the incidence as 5 per 100,000 a year, or one case every
52 minutes. These age-adjusted incidence figures for VZV cranial neuritis
with facial paralysis parallel those Hope-Simpson estimated for VZV in the
general population in 1965. These figures compare with those for Bells
palsy: 20 per 100,000 per year. Few studies outside J.R. Hunts own
assess the incidence of herpes zoster oticus in the population.
Microbiology: The Herpesvirus family
Members of the family herpesviridae are found in a wide range of host
systems. To date, at least seven different species are known to infect man,
including herpes simplex virus (HSV); cytomegalovirus (CMV), Epstein Barr
virus (EBV), and varicella zoster (VZV).
Nomenclature is critical. It is important to distinguish VZV as a member of
the greater family of herpes viruses. But it is distinct from herpes simplex.
This is a common point of confusion.
The varicella-zoster virus (VZV) belongs to the herpes family. It is a doublestranded DNA virus that causes chicken pox (varicella) and zoster
infections. Again what distinguishes Varicella from Zoster is the time of
presentation. Reactivation of virus stored in sensory ganglia from previous
varicella infection results in zoster.
Herpes viruses have an envelope surrounding an icosahedral capsid,
approximately 100nm in diameter, which contains the dsDNA genome.
When the envelope breaks and collapses away from the capsid, negatively
stained virions have a typical "fried-egg" appearance.
Work Up and Evaluation
A thorough history and physical examination, a fundamental axiom in
medicine, is the most crucial element of the work-up. Diagnosis still hinges
on the clinical findings described in Hunt's classification scheme.
Clinical Presentation
Frequently, the first symptom is a deep, burning pain in the region of the
ear. This is shortly followed in 1 to 4 days by a vesicular eruption of the
EAC and concha, or, less frequently, of the face, neck, trunk, palate or
fauces. The distribution of the vesicles depends on which sensory afferent
fibers are involved by the viral eruption, but all the fibers may be involved,
including cranial nerves V, IX, X and the cervical plexus arising from
cervical roots II, III, IV. Cranial nerves VII and VIII are almost always both
involved. During the acute illness, a varicelliform rash often accompanies
the painful vesicular eruption. Facial nerve paralysis, vertigo, and hearing
loss are commonly seen.
Natural History
The natural history of herpes zoster oticus differs from that of Bells palsy in
several ways, perhaps reflecting the difference in the behavior of herpes
simplex type I and VZV. Bells palsy recurs in 10% -12% of cases, but
herpes zoster oticus rarely recurs. In addition, the acute phase of the
infection, as measured by electrical response and progression, peaks in 5
to 10 days with Bells palsy, whereas herpes zoster oticus peaks in days 10
to 14. Finally, 84% of individuals with Bells palsy have a satisfactory
recovery of function, but only 60% of patients with herpes zoster oticus
recover function.
Crabtree (1968) was among the first to suggest that complete facial nerve
recovery is less likely following herpes zoster oticus than in other cases of
idiopathic facial palsydespite treatment with high-dose steroids. Ten
percent of patients with total facial nerve paralysis and 66 percent of those
with partial paralysis recover completely. Recovery is better in those cases
in which vesicles appear prior to nerve paralysis. Two percent of patients
over age 50 will have severe, while nine percent will have moderate, post
herpetic neuralgia.
The timing of the appearance of the vesicular eruption may have prognostic
significance. In most cases, eruption and paralysis occur simultaneously. In
approximately 25% of cases, the eruption precedes the paralysis, and the
likelihood of recovery is higher in this group (Devriese and Moesker, 1988).
Diagnosis
The Tzanck prep, which is useful with herpes zoster, located on the truck in
more peripheral nerve distribution areas, required toluidine blue staining a
scraping from the blister. Occasionally, these blisters are located medially in
the ear canal, andl are quite small. So, often it is difficult to obtain adequate
specimens for the prep. A positive prep demonstrates multinucleated giant
cells. Vesicular fluid, when present, can be cultured with human diploid
fibroblasts and after 3-5 days multinucleated giant cells within the fibroblast
population can confirm clinical diagnosis. These studies, however, require
five days or more to produce results. Laboratory confirmation of the
diagnosis is based on increasing antibody titers in repeated complement
fixation tests. Immunofluorescence of varicella antigen obtained from
exfoliated cells from lesions can provide a more expedient verification of
clinical suspicion. Often, diagnosis rests alone on the clinical criteria,
defined by Dr. Hunt
Diagnosis: Immunological Evaluation
Clinical diagnosis can be confirmed by either viral culture or fluorescence
antibody testing using VZV identification reagent (Fluorescein
isothiocyanate-conjugated monoclonal anti-VZV; Ortho Diagnostic
Systems).
Hadar and colleagues from the Tel-Aviv University Medical Centers
designed and tested VZV-specific IgG and IgA antibody titers in serial
serum samples of 23 patients with Ramsay Hunt using immunoperoxidase
assay. They demonstrated that all patients had VZV-specific IgG
antibodies, but IgA can be a useful marker in confirming early diagnosis of

the disease.
The mechanism of reactivation of VZV in herpes zoster oticus has not been
clarified. Although the mechanism involved in general herpes zoster is also
unclear, deterioration of cell-mediated immunity is thought to play a specific
role as the trigger in reactivating the virus. The term "cellular immunity"
colloquially refers to the T cell system. In fact, cell-mediated immunity is
initiated by lymphokines produced by activated CD4+ T cells, activate
macrophages and the precursors of CD8+ cytotoxic T cells. These effector
cells then cause cell-mediated immune responses such as delayed- types
hypersensitivity and cell-mediated cytotoxicity.
Nucleic acid hybridization and more recently polymerase chain reaction
technology have confirmed VZV latency in human sensory dorsal root
ganglia.
Diagnosis: Audiological Evaluation
In 1976, Byl and Adour were the first to thoroughly review the auditory
symptoms and audiological data associated with herpes zoster oticus. They
compared auditory symptoms in patients with Bells palsy and herpes
zoster oticus. of 1080 patients with idiopathic facial paralysis, 29% of
patients had auditory symptoms, while 37% of 172 patients with HZO had
these symptoms. In their series of 1252 patients, 377 patients with facial
paralysis had auditory symptoms. However, in only 11 of these 377 patients
were abnormal cause-related sensorineural hearing loss documented with
audiological testing. All of these patients had been diagnosed with HZO.
These authors were the first to recommend a diagnosis in patients with
idiopathic facial nerve paralysis and hearing losseven when the
characteristic vesicular eruption was absent. In their series, when recovery
of auditory function occurs, a high-frequency sensorineural loss may
persist, except in younger patients. Factors that appeared favorable for the
recovery of hearing include not being older than 64 years, a mild initial
hearing loss, a cochlear pattern of hearing loss, and absence of vertigo.
Wayman et al in 1990 retrospectively reviewed the audiological
manifestations of herpes zoster oticus in 186 patients. In their study, active
herpes zoster infection was confirmed by a four-fold increase between
acute and convalescent complement fixation serum titres of VZV.
Audiograms were performed on 152 patients, 82%, not all of whom had
auditory symptoms at the time. Seven of these underwent more extensive
evaluation to determine a cochlear or retrocochlear pattern of hearing loss.

Patients with a demonstrated cause-related hearing loss were treated with
oral prednisone, 60mg/day for six days, then gradually tapered. Of the 152
initial audiograms, 93 or 61% were normal, 29 or 19% were cause-related
abnormal, and 30 or 20% were unrelated abnormal examinations. This final
group was excluded from their analysis. Vertigo was documented in 8 of 29
of the cause-related hearing loss patients and in only 5 of 93 (5%) of
normal hearing patients [p=0.002]. Vertigo was more likely with increased
severity of hearing loss in the isolated high-frequency hearing loss group.
Of the seven patients who underwent more extensive audiological work-up,
six had findings consistent with cochlear pathology: elevated SISI, ABLB
recruitment pattern, type II Bekesy tracing, with no evidence of tone delay.
The seventh patient had severe speech-frequency hearing loss with a
speech discrimination score of 18%, type IV Bekesy tracing, evidence of
tone delay, and grossly abnormal brainstem evoked response with marked
delay or wave V latency, all suggestive of retrocochlear hearing loss. No
correlation was demonstrated between severity of facial paralysis and
presence of any hearing loss. The incidence of incomplete paralysis was
68% in the normal hearing and 62% in the cause-related abnormal hearing
group. Extrapolating their clinical data to the previous histopathologic
findings, especially of Blackley et al., these investigators suggested that a
cochlear pattern represents inflammation confined to the cochlea while the
retrocochlear pattern represents a more profound change involving the
entire nerve.
Diagnosis: Radiological Evaluation
Magnetic resonance imaging has added a new dimension to the research
of herpes zoster oticus and confirmed the histopathologic findings of early
investigators. Images are taken with and without the paramagnetic agent
Gadolinium as a contrast medium. Gadolinium does not normally cross the
blood-brain barrier but this barrier is broken down in the presence of
inflammation or edema. This results in increased signal density and
enhancement in these areas. Another reason suggested for abnormal
enhancement in these situations is venous congestion in the epineurium
and perineurium. There is little dispute that the facial nerve enhances on
MRI in the majority of patients with acute facial palsy, but the role of MRI in
differential diagnosis and prognostic determination is not entirely clear. The
majority of published studies refer to MRI of the facial nerve in idiopathic
Bells facial palsy. It has been shown that even the normal facial nerve
shows some mild to moderate enhancement of the geniculate ganglion and
the tympanic-mastoid segment. Nearly twenty studies of the use of MRI in
evaluation have been published. All the papers reported enhancement of
the facial nerve on MRI scanning in the majority of patients with facial palsy
but only three papers suggested that the degree of enhancement or the
anatomical level of the facial nerve enhancement had any prognostic
significance. Brugel et al (1993) concluded that moderate enhancement in
the geniculate ganglion as well as in the labyrinthine segment correlated
with a good prognosis with respect to restoration of facial movement while
an increased enhancement correlated with poor prognosis. Yanagida et al
in 1993 noted that in subjects with Ramsay Hunt syndrome who experience
internal auditory symptoms such as vertigo and tinnitus, enhancement was
not only in the facial nerve but also in the vestibular and cochlear nerves.
Treatment: Medical Management
Introduced in 1977, the antiviral agent acyclovir has dramatically improved
the treatment of herpesviridae infections. In the treatment of herpes zoster
oticus, acyclovir therapy can be expected to produce House-Brackmann
grade I-III recovery in most cases. Consequently, the success of antiviral
therapy has greatly diminished enthusiasm for surgical decompression in
cases of herpes zoster oticus with facial nerve paralysis.
Acyclovir remains the most widely prescribed and clinically effective
antiviral drug available. It is 9-(2-hydroxy-methyl) guanine and a selective
inhibitor of the replication of varicella-zoster and both herpes simplex types
1 and 2. It is converted by virus-encoded thymidine kinase to its
monophosphate derivative, an event that does not occur to any substantial
extent in uninfected cells. Subsequent disphosphorylation and
triphosphorylation are then catalyzed by cellular enzymes resulting in
acyclovir triphosphate concentrations that are 40 to 100 times higher in VZVinfected cells than uninfected cells. Acyclovir triphosphate inhibits viral DNA
synthesis by competing with deoxyguanosine triphosphate as a substrate
for viral DNA polymerase. Because acyclovir triphosphate lacks the 3hydroxyl group required to elongate the DNA chain, the synthesis of viral
DNA is terminated. Furthermore, the viral DNA polymerase is tightly
associated with the terminated DNA chain and is functionally inactivated.
Viral polymerase has greater affinity for acyclovir triphosphate than does
cellular DNA polymerase, resulting in little incorporation of acyclovir into
cellular DNA. In vitro, acyclovir is most active against HSV-1, HSV-2, and
VZV (average median effective concentrations 0.04, 0.10, and 0.50 g per
mL respectively.) Higher concentrations are required to inhibit replication of
the Epstein Barr virus. Cytomegalovirus, which lacks a virus-specific
thymidine kinase, is resistant.
Absorption from the GI tract is only 15% - 25% of the ingested dose. In
addition, the blood-brain barrier results in a 50% reduction of circulating

acyclovir into cerebrospinal fluid. Thymidine kinase found in VZV has
diminished affinity for acyclovir than TK found in HSV. For these reasons,
the oral dose is more substantial than the recommended dose for herpes
simplex.
A recent randomized prospective clinical study in the NEJM compared 7day treatment of acyclovir to two other treatment arms: 21-day course of
acyclovir with and without prednisone. Neither additional treatment reduced
the frequency of post-herpetic neuralgia.
HSV can develop resistance to acyclovir through mutations in the viral gene
encoding thymidine kinase, through the generation of thymidine-kinase
deficient mutants or through the selection of mutants possessing a
thymidine kinase that is unable to phosphorylate acyclovir.
Acyclovir-resistant isolates of VZV have been identified much less
frequently than acyclovir-resistant HSV, but they have recently been
recovered from bone-marrow transplant patients and AIDS patients. The
acyclovir-resistant isolates all had altered or absent thymidine kinase
function but remained susceptible to vidarabine and foscarnet. Acyclovir
therapy has been associated with very few adverse effects. Renal
dysfunction has been reported, especially in patients given large doses of
acyclovir by rapid intravenous infusion. This appears to be an uncommon
finding and usually reversible. Administering the drug in a slow infusion and
ensuring adequate hydration can minimize the risk of administering
acyclovir. On the other hand, oral acyclovir even at doses of 800mg five
times daily has not been associated with renal dysfunction.
Several limited retrospective studies have been done to examine
specifically the role of acyclovir in herpes zoster oticus.
Hall and Kerr from the Royal Victoria Hospital in Belfast reported in a 1985
edition of the Lancet the first use of acyclovir for herpes zoster oticus. They
treated seven patients with HZO with a parenteral dose of 5 mg/kg three
times per day. Within 3 days these investigators noted a striking
improvement in the toxemia associated with this condition. Of the six
patients who had total facial paralysis, four recovered completely, one
partially recovered, and one patient had no recovery.
Stafford and Welch reported a year later from Newcastle upon Tyne
another small series of only 5 patients. Each patient received intravenous
administration of acyclovir (5mg/kg) TID for a minimum of three days,

followed by a two-week course of oral acyclovir. High-dose oral steroids
(20mg PO QID in four cases; 10mg IVSS dexamethasone QID x 3 days)
were prescribed for 5 days in full dosage, tapered gradually over another
week. Each patient made a satisfactory recovery (4 patients with complete
recovery by six months; one with partial recovery).
Dickens et al published their results in 1988. All patients received
intravenous acyclovir 10mg/kg every 8 hours over a 7-day hospitalization
period. Five of seven patients showed some return of facial function at the
time of discharge. Their study suggests that prognosis depends on
immediate initiation of therapy, and that multiple cranial nerve involvement
did not appear to be a negative prognostic indicator.
Newer antiviral agents are available now for the treatment of herpes zoster.
Valaciclovir and Famciclovir are both available in oral preparations and in
more convenient thrice-daily dosing regimen.
Valaciclovir is the l-valine ester of acyclovir and essentially an acyclovir
prodrug. It is rapidly and almost completely converted to acyclovir in vivo.
Valaciclovir requires less frequent dosing than acyclovir due to its superior
bioavailability over acyclovir. Acyclovir bioavailability is 35 times greater
when administered as valaciclovir compared to 800 mg oral doses of
acyclovir. Major adverse reactions include nausea (16%-19%), headache
(11%-14%), vomiting (4%-9%), diarrhea (4%-6%).
Famciclovir is an oral prodrug for penciclovir and acts through a mechanism
that is similar to that of acyclovir. Recommended dose for herpes zoster is
500mg PO TID for 7 days. It also is reported to diminish post-herpetic
neuralgias.
Oral steroids have been used in zoster infections since the early 1950's.
Some studies advocate the use of steroids along with acyclovir to reduce
the incidence of post herpetic neuralgia and to enhance facial nerve
recovery. Recovery of labyrinthine dysfunction after pharmacological
therapy is not delineated in the current literature.
Case Presentation
A 62-year-old white male presented for consultation to the VAMC

OtolaryngologyHead and Neck Surgery service. The patient reported a 3day history of left facial weakness, left otalgia, and left hearing loss. There
was no vertigo, nausea or vomiting, no recent otologic infection, and no
recent trauma to the head and neck. On physical examination, a vesicular
eruption was noted over the left concha and extending into the left external
auditory canal. Grade IV House-Brackmann facial nerve weakness was
noted. There was numbness and vesicular eruption along the V2 trigeminal
nerve distribution. No nystagmus was elicited. Cranial nerves I-VI, IX, X, XI,
XII were grossly intact. A diagnosis of herpes zoster oticus was made.
Treatment was begun with oral regimen of acyclovir 800mg five doses per
day. Oral prednisone (20mg PO TID) for 5 days was begun and gradually
tapered over the next week. The patient was instructed in the use of
artificial tears, lacrilube, and nightly application of a left eyepatch. At 3month follow-up, the vesicular eruption had completely resolved and facial
nerve function had returned to normal (House-Brackmann Grade I). There
was no post-herpetic neuralgia.
Bibliography
Aleksic SN, Budzilovich GN, Lieberman AN. Herpes zoster oticus and facial paralysis
(Ramsay Hunt Syndrome). J Neurol Sci 1973;20:149-159.
Anderson RE, Laskoff JM. Ramsay Hunt syndrome mimicking intracanalicular acoustic
neuroma on contrast-enhanced MR. Am J Neuroradiol 1990;11:409.
Aono T, Murakami S, Yanagihara N, Yamanishi K. Detection of human alphaherpesvirus DNA using consensus primers and specific probes. Acta Otolaryngol Suppl
1994;514:132-134.
Berg R, Forsgren M, Schiratzki H. Acute facial palsy. Acta Otolaryngol 1976;81:462-467.
Brandle P, Satoretti-Schefer S, Bohmer A, Wichmann W, Fisch U. Correlation of MRI,
clinical, and electroneuronographic findings in acute facial nerve palsy. Am J Otol
1996;17:154-161.
Beutner KR, Friedman DJ, Forszpaniak C, Andersen PL, Wood MJ. Valaciclovir
compared with acyclovir for improved therapy for herpes zoster in immunocompetent
adults. Antimicrob Agents Chemother 1995;39:1546-1553.
Blackley B, Friedmann I, Wright I. Herpes zoster auris associated with facial nerve palsy
and auditory nerve symptoms: a case report with histopathological findings. Acta
Otolaryngol 1967;63:533-550.
Burgoon CF, Burgoon JS, Baldridge GD. The natural history of herpes zoster. JAMA
1957;164:265-268.
Byl FM, Adour KK. Auditory symptoms associated with herpes zoster or idiopathic facial
paralysis. Laryngoscope 1977;87:372-379.
Carroll SM, Mastaglia FL. Optic neuropathy and ophthalmoplegia in herpes zoster
oticus. Neurol 1979;29:726-729. Crabtree JA. Herpes zoster oticus. Laryngoscope
1968;78:1853-1878.
Crabtree JA. Herpes zoster and facial paralysis. Otolaryngol Clin North Am 1974;7:369373.
Crumpacker C . The pharmacological profile of famciclovir. Sem Dermatol
1996;15(Suppl 1):14-26, 1996. Daniels DL, Czervionke LF, Millen SJ. MR findings in the
Ramsay Hunt syndrome. Am J Neuroradiol 1988;9:609.
Denny-Brown D, Adams RD, Fitzgerald PJ. Pathologic features of herpes zoster. Arch
Neurol Psychiatr 1944;51:216.
Devriese PP, Moesker WH. The natural history of facial paralysis in herpes zoster. Clin
Otolaryngol 1988;13:289-298.
Dickins JRE, Smith JT, Graham SS. Herpes zoster oticus: treatment with intravenous
acyclovir. Laryngoscope 1988;98:776-779.
Downie AC, Howlett DC, Koefman RJ, Banerjee AK, Tonge KA . Case report: prolonged
contrast enhancement of the inner ear on magnetic resonance imaging in Ramsay Hunt
syndrome. Br J Radiol 1994;67:819-821.
Djupesland G, Degre M, Stien R, Skrede S. Acute peripheral facial palsy. Arch
Otolaryngol 1977;103:641-644.
Fisch U. Surgery for Bell's palsy. Arch Otolaryngol 1981;107:1-11.
Gelfand ML. Treatment of herpes zoster with cortisone. JAMA 1954;154:911-912.
Goldsmith P, Zammit-Maempel I, Meikle D. Ramsay Hunt syndrome mimicking acoustic
neuroma on MRI. J Laryngol Otol 1995;109:1013-1015.
Hall SJ, Kerr AG. Acyclovir in herpes zoster oticus. Lancet 1985;1(8437):1103.
Harner SG, Heiny BA, Newell RC. Herpes zoster. Arch Otolaryngol 1970;92:632-635.
Head H, Campbell AW. The pathology of herpes zoster: a long-term study and a new
hypothesis. Brain 1900;23:353.
Hope-Simpson RE. The nature of herpes zoster: a long-term study and a new
hypothesis. Proc Royal Soc Med 1965;58:9-20.
House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg
1985;93:146-147.
Huff JC, Bean B, Balfour HH Jr, Laskin OL, Connor JD, Corey L, et al. Therapy of
herpes zoster with oral acyclovir. Am J Med 1988;85:84-89.
Hunt JR. Herpetic inflammations of the geniculate ganglion. A new syndrome and its
complications. J Nerv Ment Dis 1907;34:73-96.
Ikeda M, Hiroshige K, Abiko Y, Onoda K. Impaired specific cellular immunity to the
varicella-zoster virus in patients with herpes zoster oticus. J Laryngol Otol 1996;110:918921.
Inamura H, Aoyagi M, Tojima H, Koike Y. Viral infection in acute peripheral facial palsy:
investigation in Yamagata prefecture. Acta Otolaryngol Suppl 1988;446:23-26.
Johnson L, Zonderman B. Herpes zoster oticus (Ramsay Hunt Syndrome): a report of
a case. Arch Otolaryngol 1948;48:1-8.
Jonsson L, Tien R, Engstrom M, Thuomas KA. Gd-DPTA enhanced MRI in Bell's palsy
and herpes zoster oticus: an overview and implications for future studies. Acta
Otolaryngol 1995;115:577-584.
Kanzaki J. Electrodiagnostic findings in the early stages of Bell's palsy and RamsayHunt's syndrome. Acta Otolaryngol Suppl 1985;446:42-46.
Keane JR. Delayed trochlear nerve palsy in a case of zoster oticus. Arch Ophthalmol
1975;93:382-383.
Korzec K, Sobol SM, Kubal W, Mester SJ, Winzelberg G, May M. Gadolinium-enhanced
magnetic resonance imaging of the facial nerve in herpes zoster oticus and Bell's palsy:
clinical implications. Am J Otol 1991;12:163-168.
Kukimoto N, Ikeda M, Yamada K, Tanaka M, Tsurumachi M. Tomita H. Viral infections in
acute peripheral facial paralysis: nationwide analysis centering on CF. Acta Otolaryngol
Suppl 1988;446:17-22.
Kuo MJ, Drago PC, Proops DW, Chavda SV. Early diagnosis and treatment of Ramsay
Hunt syndrome: the role of magnetic resonance imaging. J Laryngol Otol 1995;109:777780.
LaBagnara J, Jahn AF, Habif DV, Solomon EM. MRI findings in two cases of acute facial
paralysis. Otolaryngol Head Neck Surg 1989;101:562-565.
Lee D, Belmont M, Lucente FE. Pathologic quiz case 2. Bilateral herpes zoster oticus.
Arch Otolaryngol Head Neck Surg 1996;122:195,197-198.
May M, Schlaepfer WW. Bell's palsy and the chorda tympani nerve: a clinical and
electron microscopic study. Laryngoscope 1975;85:1957-1975.
May M, Shambaugh GE Jr. Facial nerve paralysis. In: Paparella MM, Shumrick DA,
Gluckman JL, Meyerhoff WL, editors. Otolaryngology, 3rd ed. Philadelphia: Saunders;
1991. pp.1123-1124.
Neely JG, Joaquin AH, Kohn LA, Cheung JY. Quantitative assessment of the variation
within grades of facial paralysis. Laryngoscope 1996;106:438-442.
Obituary: JR Hunt. J Nerv Disorders Ment Dis 1938;87:394-402.
Osumi A, Tien RD. MR findings in a patient with Ramsay-Hunt syndrome. J CompAssist
Tomogr 1990;14:991-993
Peterslund NA, Seyer-Hansen K, Ipsen J, Esmann V, Schonheyder H, Juhl H. Acyclovir
in herpes zoster. Lancet 1981;2(8251):827-830.
Proctor L, Lindsay J, Perlman H, Matz G. Acute vestibular paralysis in herpes zoster
oticus. Ann Otol Rhinol Laryngol 1979;88:303-309.
Ragozzino MW, Melton LJ 3d, Kurland LT, Chu CP, Perry HO. Population-based study
of herpes zoster and its sequelae. Medicine 1982;61:310-316.
Rathore MH, Friedman AD, Barton LL, Dunkle LM. Herpes zoster oticus. Am J Dis Child
1991;45:722-723.
Robillard RB, Hilsinger RL, Adour KK. Ramsay Hunt facial paralysis: clinical analyses of
185 patients. Otolaryngol Head Neck Surg 1986;95:292-297.
Sartoretti-Schefer S, Wichmann W, Valavanis A. Idiopathic, herpetic, and HIVassociated facial nerve palsies: abnormal MR enhancement patterns. Am J Neuroradiol
1994;15:479-485.
Schuknecht HF. Pathology of the Ear, 2nd ed. Boston: Harvard University Press; 1993.
pp. 236-237.
Stafford FW, Welch AR. The use of acyclovir in Ramsay Hunt syndrome. J Laryngol Otol
1986;100:337-340.
Tien R, Dillon WP, Jackler RK. Contrast-enhanced MR imaging of the facial nerve in 11
patients with Bell's palsy. Am J Neuroradiol 1990;11:735-741.
Tschiassny K. The site of facial nerve lesion in cases of Ramsay Hunts syndrome. Ann
Uri N, Meyer W, Greenberg E, Kitzes-Cohen R. Herpes zoster oticus: treatment with
Acyclovir. Ann Otol Rhinol Laryngol 1992;101:161-162.
Wayman DM, Pham HN, Byl FM, Adour KK. Audiological manifestations of Ramsay
Hunt syndrome. J Laryngol Otol 1990;104:104-108.
Weller S, Blum MR, Doucette M, Burnette T, Cederberg DM, de Miranda P, et al.
Pharmacokinetics of the acyclovir pro-drug valaciclovir after escalating single- and
multiple-dose administration to normal volunteers. Clin Pharmacol Therap 1993;54:595605.
Weller TH.,Coons AH. Fluorescent antibody studies with agents of varicella and herpes
zoster propagated in vitro. Proc Soc Exper Biol 1954;86:789.
Wilson WR. The relationship of herpesvirus family to sudden hearing loss: a prospective
clinical study and literature review. Laryngoscope 1986;96:870-877.
Whitley RJ, Gnann JW Jr. Acyclovir: a decade later. N Engl J Med 1992;327:782-789.
Wood MJ, Johnson RW, McKendrick MW, Taylor J, Mandal BK, Crooks J. A randomized
trial of acyclovir for 7 days or 21 days with and without prednisone for treatment of acute
herpes zoster. N Engl J Med 1994;330:896-900.
Yanagida M, Ushiro K, Yamashita T, Kumazaqa T, Katoh T. Enhanced MRI in patients
with Ramsay Hunts Syndrome. Acta Otolaryngol Suppl 1993;500:58-61.
Zajtchuk JT, Matz GJ, Lindsay JR. Temporal bone pathology in herpes oticus. Ann Otol
Zammit-Maempel I, Campbell RSD. Prolonged contrast enhancement of the inner ear
on MRI in Ramsay Hunt syndrome. Br J Radiol 1995;68:334-335


medical literature.
NEUROTOLOGIC MANIFESTATIONS OF HIV INFECTION

March 24, 1994
Thomas A. Salzer, M.D.
As health care providers we are faced with the prospect of caring for an increasing number of AIDS
patients. The disease is no longer limited to specific populations and nearly everyone is at risk.
Familiarity with the protean manifestations of AIDS is important for early diagnosis and treatment. AIDS
is of particular interest to the Otolaryngologist-Head and Neck surgeon since 40-84% of patients have a
symptom or physical finding in the head and neck region at initial presentation. Early diagnosis is
essential because prompt initiation of treatment significantly diminishes morbidity and improves both
quality and length of life in affected individuals. We will briefly review current statistics relevant to the
AIDS epidemic, discuss the classification of HIV associated diseases and review in detail the
neurotologic manifestations of HIV infection.
The causative agent for AIDS, HIV, is a retrovirus of the subfamily Lentovirinae. The term retrovirus is
used because the virus contains the enzyme reverse trancriptase, with transcribes viral RNA to DNA- the
reverse of other viral genetic transcription. HIV appears to be neurotropic and lymphotrophic and
preferencially attacks T-helper cells, which are central to the function of the human cell-mediated
immune system. Impairment of this system renders the host susceptible to numerous opportunistic
infections from viruses, fungi, and protozoas, many of which are native to the oral cavity, pharynx and
larynx. Aside from infectious consequences, HIV is also associated with malignancy, degenerative
diseases and autoimmune sequelae.
OTOLOGIC-NEUROTOLOGIC MANIFESTATIONS
Otologic complaints are common in AIDS patients and include: hearing loss (62%), otalgia (50%),
otorrhea (31%), vertigo (15%) and tinnitus (15%). It is not known if the neurotologic effects seen in
AIDS is the effect of HIV alone or a combination of the effects of HIV infection coupled with
opportunistic infections and/or possible toxic effects of certain therapeutic agents. The brain may be the
site of tumors such as lymphoma or metastatic Kaposi's sarcoma. The HIV itself may cause aseptic
meningitis, subacute encephalitis and isolated cranial nerve neuropathy. The following case reports
associate HIV infection with otologic diagnoses:
RAMSAY HUNT SYNDROME
Mishell and Applebaum 1990, reported a case of a 29 y/o homosexual male who was HIV+ with Ramsay
Hunt syndrome associated with facial nerve palsy. The patient was treated with intravenous nafcillin and
acyclovir.
HERPES ZOSTER MYRINGITIS
Kohan et al 1988 reported two cases of Herpes zoster myringitis which were successfully treated with
acyclovir.
KAPOSI'S SARCOMA
KS is a slowly progressive, malignant mesenchymal tumor characterized clinically by red-purple plaques
and nodules that may be seen externally or internally. Proliferation of atypical spindle cells within
vascular channels is seen on histopathology. KS has been reported to involve the mastoid (Linstrom
1993) and external ear (Morris 1990).
GRADENIGO'S SYNDROME
Linstrom et al 1993 reported a 28 year old male IVDA with otalgia, purulent otorrhea, and sixth and
seventh cranial nerve palsy secondary to petrous apicitis due to Aspergillus infection
PROGRESSIVE MULTIFOCAL LEUKENCEPHALOPATHY
Langford and Kuntz 1988 presented two adult patients with AIDS who had multiple cranial neuropathies
resulting from progressive multifocal leukencephalopathy. Clinical symptoms included facial paralysis,
trigeminal hypaesthesia, hemianopsia, and deafness. Cranial MRI in both patients showed progressive
PML, an unusual demyelinating disease seen in AIDS patients and thought to result from a direct effect
of HIV in the cerebral white matter.

PNEUMOCYSTIS CARINII OTITIS / MASTOIDITIS
Pseudomonas carinii is the most common opportunistic pathogen infecting persons with HIV infection,
affecting nearly 85% patients at some point of the illness. Otic Pneumocystis typically presents as a
unilateral polypoid mass associated with otalgia, hearing loss and occasionally otorrhea. Pneumocystis
carinii otitis media and mastoiditis have been. Pneumocystis carinii recovered from aural polyps is
occasionally the initial manifestation of HIV infection in patients presenting to otologists for evaluation
of otorrhea, otalgia and hearing loss. Pneumocystis carinii of the external canal has been effectively
treated with local debridement and a 3- week course of Bactrim.
OPPORTUNISTIC INFECTIONS
Toxoplasmosis is the most common opportunistic infection to effect the CNS (Real, 1988). Kohan
reported two such patients which presented with bilateral SNHL. Cryptococcal meningitis is known to be
associated with SNHL in up to 25% cases (Real, 1987). Tuberculous meningitis is not infrequent in
AIDS patients and is known to be associated with hearing loss.
OTOSYPHILIS
Otosyphilis must always be considered and investigated in a patient with fluctuating, asymmetric or
sudden hearing loss. The diagnosis and treatment of syphilitic cochleovestibular dysfunction remains
controversial, however the presumptive diagnosis is made in patients with cochleovestibular dysfunction,
positive serology for syphilis and no other known etiology for the inner ear disturbance.. Smith and
Canalis, 1989 found otosyphilis to develop at an accelerated rate from primary syphilis in AIDS patients.
All cases arose in patients previously treated for primary syphilis (latent syphilis). They propose that the
HIV somehow hastens the development of otosyphilis effectively activating the disease.. Furthermore
they propose that all patients with otosyphilis should have an HIV test.
The index of suspicion should be high in the homosexual population because of the high incidence of
syphilis in this group. 35-55% patients with AIDS have a past history of other sexually transmitted
diseases including syphilis. Otosyphilis is well recognized as a cause of otologic disease in patients with
AIDS (Morris and Prasad, 1990).
Gleich et al identified three factors associated with hearing improvement in patients with otosyphilis
treated with intravenous penicillin and corticosteroids. In there study 31% patients with otosyphilis
experienced hearing improvement, tinnitus decreased in 85% and vertigo improved in 86% . Specific
factors associated with hearing improvement include: hearing loss present less than 5 years, fluctuating
hearing, and age less than 60 years. Improvement was unrelated to the severity of the loss or previous
treatment.
CENTRAL NERVOUS SYSTEM
HIV is frequently accompanied by neurological complications which are observed in 40% to 75% of
patients during the course of the disease. De la Monte discovered CNS involvement in more than 90% of
HIV infected patients at autopsy. Neurological findings are detectable in approximately one third of
AIDS patients. At autopsy however, up to of 75% subjects reveal neuropathological changes related to
HIV infection. The most common neurologic syndrome seen in AIDS patients is subacute encephalitis.
Early stages are characterized by subtle cognitive changes that may progress to dementia in several
weeks or months. The HIV appears to be directly involved in both clinical and pathological changes in
the CNS. Neurotologic workup may reveal central vestibular and auditory dysfunction. ENG findings
show ataxic pursuit and optikinetic nystagmus with total loss of caloric excitability. ABR indicate
absolute and interpeak latencies and synthetic sentence identification test yield reduced scores. At
autopsy the AIDS virus was found in mononuclear and multinuclear giant cells in the cortical and
subcortical gray matter, cerebral and cerebellar white matter and through out the brain stem.
Hausler compared the incidence of peripheral and central auditory and vestibular disorders occurring in
different stages of HIV infection. The results of audiological, vestibular and electrophysiologic tests
performed on symptomatic and asymptomatic HIV-positive homosexual males with age matched
seronegative homosexual males were compared. 57% of symptomatic and 45% off asymptomatic HIV
positive patients had substantial abnormalities in comparison to minor abnormalities detected in 12%
seronegative patients. The results suggested a high incidence of neurotologic disorders in HIV positive
patients and that subclinical involvement of the auditory and vestibular system is common.
The frequency of electrophysiologic abnormalities in asymptomatic patients indicates subclinical
involvement of the Central nervous system.
SENSORINEURAL HEARING LOSS
The incidence of SNHL in patients with HIV ranges from 23 - 49% . Kohan reported seven patients with
persistent SNHL. In three instances the hearing loss could be traced to the use of well-known ototoxic
drugs used in the treatment of AIDS. The remaining four patients were divided among CNS
toxoplasmosis and cryptococcal meningitis, CNS toxoplasmosis alone, tuberculous meningitis and leutic
otitis. For example, acyclovir may cause vertigo, trimethoprim-sulfamethoxazole may cause vertigo and
tinnitus, and azidothymidine may lead to vertigo and hearing loss.
The characteristics of the SNHL in patients with HIV are not uniformly defined. The degree of hearing
loss, range of loss and even incidence appear variable. Hearing loss in the high frequencies is more
commonly reported than in the low frequencies. Delayed latencies of brainstem auditory-evoked
responses have been observed in patients with HIV, suggesting neuropathies of the central auditory and
vestibular region.
Boccellari found an association between immune suppression in HIV patients and neurophysiologic
measures. Asymptomatic subjects without evidence of immune suppression do not appear to be at greater
risk for neurophysiological impairment than HIV-negative subjects. The HIV-positive individuals with
evidence of immunosuppression, however, appear to have an increased likelihood of central conduction
time slowing. In contrast, studies by Pagano suggest that subclinical involvement of the upper brain stem
occurs in HIV infection and that brainstem auditory evoked potential abnormalities could be the direct
result of the HIV effect on central nervous system structures.
Case Presentation
A 35-year-old man with human immunodeficiency virus infection diagnosed seven years ago was
hospitalized for disseminated histoplasmosis which served as his AIDS-defining illness. During that
admission he was also found to have Mycobacterium avium-intracellulare septicemia and bilateral
external otitis secondary to Pseudomonas aeruginosa. CT scan of the temporal bones and gallium scan
failed to reveal bony involvement. The otitis externa was treated with local debridement, culture specific
intravenous antibiotics and otic drops. The patient experienced near total resolution of his symptoms with
decreased otalgia, erythema and edema. Post treatment cultures were negative. Two months later he
presented to the Otolaryngology service for evaluation and treatment of recurrent otitis externa which
had progressed despite oral ciprofloxacin and Cortisporin otic drops. Examination revealed severe
external otitis with erythema, edema and ulceration limited to the concha cavum and a right paranasal
ulcer. The possibility of drug reaction was entertained and initial improvement was seen with withdrawal
of the otic drops, however symptoms worsened thereafter prompting tissue biopsy of the ear and
paranasal region for histology and culture. Histologic evaluation revealed multiple rrport inclusion
bodies consistent with Herpes simplex infection. Audiometric evaluation was normal. The patient's
condition improved dramatically on intravenous acyclovir.
Bibliography
Barzan L, Carbone A, Tirelli U, Crosato IM, Vaccher E, Volpe R, et al. Nasopharyngeal lymphatic tissue in patients
infected with human immunodeficiency virus. A prospective clinicopathologic study. Arch Otolaryngol Head Neck Surg
1990;116:928-931.
Barzan L, Tavio M, Tirelli U, Comoretto R. Head and neck manifestations during HIV infection. J Laryngol Otol
1993;107:133-136.
Birdsall HH, Ozluoglu LN, Lew HL, Trial J, Brown DP, Wofford MJ, et al. Auditory P300 abnormalities and leukocyte
activation in HIV infection. Otolaryngol Head Neck Surg 1994;110:53-59.
Boccellari AA, Dilley JW, Yingling CD, Chambers DB, Tauber MA, Moss AR, et al. Relationship of CD4 counts to
neurophysiological function in HIV-1--infected homosexual men. Arch Neurol 1993;50:517-521.
Breda SD, Hammerschlag PE, Gigliotti F, Schinella R. Pneumocystis carinii in the temporal bone as a primary
manifestation of the acquired immunodeficiency syndrome. Ann Otol Rhinol Laryngol 1988;97:427-431.
Chandrasekhar SS, Siverls V, Sekhar HK. Histopathologic and ultrastructural changes in the temporal bones of HIVinfected human adults. Am J Otol 1992;13:207-214.
Chilla R, Booken G, Rasche H. Bell's palsy as the initial symptom of HIV infection. Laryngol Rhinol Otol 1987;66:629630.
Chow JH, Stern JC, Kaul A, Pincus RL, Gromisch DS. Head and neck manifestations of the acquired immunodeficiency
syndrome in children. Ear Nose Throat J 1990;69:416-9, 422-3.
Gleich LL, Linstrom CJ, Kimmelman CP. Otosyphilis: a diagnostic and therapeutic dilemma. Laryngoscope
1992;102:1255-1259.
Hart CW, Cokely CG, Schupbach J, Dal Canto MC, Coppleson LW. Neurotologic findings of a patient with acquired
immune deficiency syndrome. Ear Hear 1989;10:68-76.
Hausler R, Vibert D, Koralnik IJ, Hirschel B. Neuro-otological manifestations in different stages of HIV infection. Acta
Otolaryngol Suppl 1991;481:515-521.
Kohan D, Hammerschlag PE, Holliday RA. Otologic disease in AIDS patients: CT correlation. Laryngoscope
1990;100:1326-1330.
Kohan D, Rothstein SG, Cohen NL. Otologic disease in patients with acquired immunodeficiency syndrome. Ann Otol
Lannigan FJ, Jones NS, von Schoenberg MV. An avoidable occupational hazard during mastoid surgery. J Laryngol Otol
1989;103:566.
Linstrom CJ, Pincus RL, Leavitt EB, Urbina MC. Otologic neurotologic manifestations of HIV-related disease.
Lucente FE. Impact of the acquired immunodeficiency syndrome epidemic on the practice of laryngology. Ann Otol
Rhinol Laryngol Suppl 1993;161:1-24.
Mishell JH, Applebaum EL. Ramsay-Hunt syndrome in a patient with HIV infection. Otolaryngol Head Neck Surg
1990;102:177-179.
Morrison GA, Butler P, Booth JB. AIDS to the post nasal space. J Laryngol Otol 1989;103:1091-1092.
Murr AH, Benecke JE,Jr. Association of facial paralysis with HIV positivity. Am J Otol 1991;12:450-451.
Northfelt DW, Clement MJ, Safrin S. Extrapulmonary pneumocystosis: clinical features in human immunodeficiency virus
infection. Medicine 1990;69:392-398.
Pagano MA, Cahn PE, Garau ML, Mangone CA, Figini HA, Yorio AA, et al. Brain-stem auditory evoked potentials in
human immunodeficiency virus-seropositive patients with and without acquired immunodeficiency syndrome. Arch
Neurol 1992;49:166-169.
Paller AS, Sahn EE, Garen PD, Dobson RL, Chadwick EG. Pyoderma gangrenosum in pediatric acquired
immunodeficiency syndrome. J Pediatr 1990;117:63-66.
Park S, Wunderlich H, Goldenberg RA, Marshall M. Pneumocystis carinii infection in the middle ear. Arch Otolaryngol
Head Neck Surg 1992;118:269-270.
Rarey KE. Otologic pathophysiology in patients with human immunodeficiency virus. Am J Otolaryngol 1990;11:366369.
Real R, Thomas M, Gerwin JM. Sudden hearing loss and acquired immunodeficiency syndrome. Otolaryngol Head Neck
Surg 1987;97:409-412.
Rowe Jones JM, Pringle MB. Prevention of occupational transmission of HIV in the ENT clinic. Ann R Coll Surg Engl
1992;74:5-8.
Sandler ED, Sandler JM, LeBoit PE, Wenig BM, Mortensen N. Pneumocystis carinii otitis media in AIDS: a case report
and review of the literature regarding extrapulmonary pneumocystosis. Otolaryngol Head Neck Surg 1990;103:817-821.
Shapiro AL, Shechtman FG, Guida RA, Kimmelman CP. Head and neck lymphoma in patients with the acquired immune
deficiency syndrome. Otolaryngol Head Neck Surg 1992;106:258-260.
Strauss M, Fine E. Aspergillus otomastoiditis in acquired immunodeficiency syndrome. Am J Otol 1991;12:49-53.
Taylor DN, Wallace JG, Masdeu JC. Perception of different frequencies of cranial transcutaneous electrical nerve
stimulation in normal and HIV-positive individuals. Percept Mot Skills 1992;74:259-264.
Timon CI, Walsh MA. Sudden sensorineural hearing loss as a presentation of HIV infection. J Laryngol Otol
1989;103:1071-1072.
Tosti A, Gaddoni G, Peluso AM, Misciali C, Piraccini BM, Menni B. Acquired hairy pinnae in a patient infected with the
human immunodeficiency virus. J Am Acad Dermatol 1993;28:513.
Wasserman L, Haghighi P. Otic and ophthalmic pneumocystosis in acquired immunodeficiency syndrome. Report of a
case and review of the literature. Arch Pathol Lab Med 1992;116:500-503.
Welkoborsky HJ, Lowitzsch K. Auditory brain stem responses in patients with human immunotropic virus infection of
different stages. Ear Hear 1992;13:55-57.



medical literature.
INFECTIOUS INTRACRANIAL COMPLICATIONS OF SUPPURATIVE

EAR DISEASE
Andrew L. de Jong, MD
March 18, 1993
The existence and treatment of the intracranial complications of suppurative ear disease can be traced to
the European Neolithic period. It is well-known that prehistoric man performed trephining, or the
surgical opening of the skull with primitive instruments, for such infections. Until the 1940s the outlook
for these patients remained grim, with a mortality of 75%. However, the introduction of penicillin by
Fleming drastically changed this, decreasing mortality to 10%.
There are four well-known infectious otitic intracranial complications:
1. Epidural abscess
2. Subdural abscess
3. Meningitis
4. Brain abscess
The signs and symptoms of these complications can often be difficult to detect initially. Impending
complications should be suspected when: 1) otologic infection recurs two to three weeks after initial
treatment; 2) fetid aural discharge is present; 3) headache or subtle mental status changes in the presence
of otologic disease; and 4) otalgia in the setting of chronic ear disease. These warning signs should
prompt further investigation.
The most useful initial study is computerized tomography (CT) with contrast. If the CD findings are
equivocal and the index of suspicion remians high, this should be followed by magnetic resonance
imaging (MRI) with and without contrast. Therapy is tailored to each specific complication but should
include intravenous antibiotics, medical management of elevated cerebrospinal fluid pressure, and
neurosurgical consultation. Once the patient is neurologically stable, attention should be focused on
definitive otologic intervention.
The pathogenesis, microbiology, and treatment of each otitic intracranial complication were discussed in
detail.
Case Presentation
A 20-year-old white woman with an extensive otologic history including left mastoidectomy in 1984
with revision in 1991, presented to another hospital with a five-day history of right-sided fetid aural
discharge, otalgia, headache, and fever. She had been placed on oral antibiotics three days earlier by a
private physician. On admission she was noted to be lethargic with neck rigidity, vomiting, and
photophobia. Her aural exam was significant for the discharge and a posterior, superior deep retraction
pocket. CT scanning revealed right mastoiditis and probable posterior fossa bony erosion. A lumbar
puncture was consistent with meningitis, with the CSF fluid growing staphylococcus, proteus, and
pseudomonas species.
The patient was transferred to BTGH on broad spectrum antibiotics. A right modified radical
mastoidectomy with removal of a cholesteatoma was performed. A bony defect was found over the
lateral sinus, but the sinus was patent. Postoperatively, DM had persistent headaches and neck pain. She
developed somnolence, ataxia, and dysdiadochokinesia. CT and MRI imaging showed an infratentorial
subdural empyema. A suboccipital craniotomy was performed by the neurosurgical team with removal of
a sterile empyema. DM then made steady progress with only mild residual ataxia.
Bibliography
Alford BR, Cohn AM. Complications of suppurative otitis media and mastoiditis. In: Paparella MM, Shumrick DA,
editors. Otolaryngology, Volume II. Philadelphia: WB Saunders, 1980;1490-1509.
Alford BR, Pratt FE. Intracranial complications from otitis media. Tex Med 1966;62:66-70.
Chole RA. Osteoclasts in chronic otitis media, cholesteatoma, and otosclerosis. Ann Otol Rhinol Laryngol 1988;97:661666.
Friedman EM, McGill TJ, Healy GB. Central nervous system complications associated with acute otitis media in children.
Froeschner EH. Two examples of ancient skull surgery. J Neurosurg 1992;76:559-562.
Glasscock ME, Shambaugh GE. Surgery of the Ear, 4th edition. Philadelphia: WB Saunders, 1990:249-275.
Gower D, McGuirt WF. Intracranial complications of acute and chronic infectious ear disease: a problem still with us.
Laryngoscope 1983;93:1028-1033.
Grossman RG, editor. Principles of Neurosurgery. NY: Raven Press, 1991:179-190.
Holt GR, Gates GA. Masked mastoiditis. Laryngoscope 1983;93:1034-1037.
Hoyt DJ, Fisher SR. Otolaryngolgic management of patients with subdural empyema. Laryngoscope 1991;101:20-24.
Jahn AF. Chronic otitis media; diagnosis and treatment. Med Clin North Am 1991;75:1277-1291.
Keet PC. Cranial intradural abscess management of 641 patients during the 35 years from 1952 to 1986. Br J Neurosurg
1990;4:273-278.
Koltai PJ, Eames FA, Parnes SM, Wood GW, Bie B. Comparison of computed tomography and magnetic resonance
imaging in chronic otitis media with cholesteatoma. Arch Otolaryngol Head Neck Surg 1989;115:1231-1233.
Kurihara A, Toshima M, Yuasa R, Takasaka T. Bone destruction mechanisms in chronic otitis media with cholesteatoma:
specific production by cholesteatoma tissue in culture of bone-resorbing activity attributable to interleukin-1 alpha. Ann
Martin-Hirsch DP, Habashi S, Page R, Hinton AE. Latent mastoiditis: no room for complacency. J Laryngol Otol
1991;105:767-768.
Mathews TJ, Marus G. Otogenic intradural complications: a review of 37 patients. J Laryngol Otol 1988;102:121-124.
McLaurin RL, editor. Pediatric Neurosurgery, 2nd edition. Philadelphia: WB Saunders, 1989:479-489.
Munz M, Farmer JP, Auger L, O'Gorman AM, Schloss MD. Otitis media and CNS complications. J Otolaryngol
1992;21:224-246.
Murthy PS, Sukumar R, Hazarika P, Rao AD, Raja M, Raja A. Otogenic brain abscess in childhood. Int J Pediatr
Nalbone VP, Kuruvilla A, Gacek RR. Otogenic brain abscess; the Syracuse experience. Ear Nose Throat J 1992;71:238242.
Neely JG. Complications of Suppurative Otitis Media. Part 2: Intracranial Complications.
Washington D.C.: AAO-HNS, 1979.
Neely JG. Complications of temporal bone infection. In: Cummings CW, Harker LA, editors. Otolaryngology - Head and
Neck Surgery, Volume 4, 2nd edition. St. Louis: Mosby, 1992:2840-2864.
Nunez DA, Browning GG. Risks of developing an otogenic intracranial abscess. J Laryngol Otol 1990; 104:468-472.
Ponka A, Ojala K, Teppo AM, Weber TH. The differential diagnosis of bacterial and aseptic meningitis using
cerebrospinal fluid laboratory tests. Infection 1983;11:129-131.
Rupa V, Raman R. Chronic suppurative otitis media: complicated versus uncomplicated disease. Acta Otolaryngol
1991;111:530-535.
Schmidek HH, editor. Operative Neurosurgical Techniques: Indications, Methods, and Results, 2nd edition. Philadelphia:
WB Saunders, 1988:490-495.
Schwaber MK, Pensak ML, Bartels LJ. The early signs and symptoms of neurotologic complications of chronic
suppurative otitis media. Laryngoscope 1989;99:373-375.
Stevensen RS, Guthrie D. A History of Oto-Laryngology. Edinburgh: Livingstone, 1949;112-118.
Wackym PA, Canalis RF, Feuerman T. Subdural empyema of otorhinological origin. J Laryngol Otol 1990;104:118-122.
Weingarten K, Zimmerman RD, Becker RD, Heier LA, Haines AB, Beck MD. Subdural and epidural empyemas: MR
imaging. AJR Am J Roentgen 1989;152:615-621.
Yaniv E, Pocock R. Complications of ear disease. Clin Otolaryngol 1988;13:357-361.

medical literature.
LATERAL SINUS THROMBOSIS

Judith J. Owens, MD
May 7, 1992
Although seldom encountered in the practice of modern otology, lateral sinus thrombosis (LST) and
other intracranial complications of otitis media still occur. The classic picture of this disease is often
modified by prior antibiotic treatment, making the diagnosis and management difficult.
Infective thrombosis of the lateral sinus was first described in 1826 by Hooper. This disease was
universally fatal until surgical intervention was established in 1888 by Lane. Despite later advances in
surgical techniques mortality remained at nearly 50% until the introduction of antibiotics. Prior to the
advent of antibiotic therapy, the mortality of all intracranial complications was extraordinarily high. In a
study of autopsy statistics at LA County Hospital, it was found that before the introduction of antibiotics
approximately 25:1,000 deaths were due to an intracranial complication of otitis media. The death rate
from these complications dropped 90% after the introduction of antibiotics.

The intracranial complications of otitis media include purulent meningitis, extradural or peridural
abscess, LST, brain abscess and otitic hydrocephalus. Respiratory mucosa, intact boney walls and
protective granulations provide natural defense barriers within the middle ear; complications occur when
these are overcome. The spread of infection through the natural defenses can occur by osteothrombosis,
bone erosion and when present along preformed pathways.
Classic symptoms of LST include a "picket fence" fever pattern; chills; progressive anemia (especially
with beta-hemolytic strep); and, symptoms of septic emboli, headache and papilledema may indicate
extension to involve the cavernous sinus. The Toby-Ayer test is measured by monitoring the CSF
pressure during a lumbar puncture. No increase in CSF pressure during external compression of the
internal jugular vein on the affected side, and an exaggerated response on the patent side, is suggestive of
LST.
Since the introduction of antibiotics, some authors have noted that a high percentage of cases are due to
chronic rather than acute cases of otitis media; however this finding has not been consistent in all reports.
Teenagers and young adults are more commonly affected in modern reports whereas younger children
were reported in higher numbers in earlier series. In the pre-antibiotic era streptococcus and
staphylococcus were reported as causing the majority of cases of LST, recent reports have included
anaerobic and gram negative organisms as well.
The diagnostic procedure of choice is MRI with MR angiography. The thrombus can be identified by its
signal intensity on MRI and the flow void in the affected sinus is clearly documented on MR
angiography.
Treatment is always surgical removal of the infected thrombosis in addition to broad spectrum antibiotic
coverage. Once a highly controversial issue, ligation of the internal jugular vein is seldom necessary. In
the majority of recent cases, anticoagulation has not been found to be necessary. However, it has been
advocated by Shambaugh and may be indicated in selected cases.
Case Presentation
A 6-year-old Hispanic male presentedwith no prior history of systemic illness or otologic disease. He
was seen at Ben Taub General Hospital with a five day history of upper respiratory infection, headache,
and bilateral otorrhea. Two days earlier swelling and tenderness had developed along the left
sternocleidomastoid muscle. On arrival, his temperature was 101F and white blood cell count was
18,300; he was alert and appropriate, but irritable and uncooperative. A CT scan was obtained that
showed bilateral opacification of the mastoid air cells, and an abscess in the left upper neck. That night
he was taken to the OR for incision and drainage of the neck abscess and complete otologic examination.
There was marked swelling of the left external auditory canal which precluded visualization of the left
tympanic membrane. The right external auditory canal was also swollen, but the tympanic membrane
could be visualized. The tympanic membrane was found to be thickened, a myringotomy was performed,
purulent fluid was aspirated from the middle ear cleft and a ventilating tube was left in place. He was
initially treated with Ceftriaxone (Rocephin) and Cortisporin otic drops. Cultures obtained from the right
tympanic aspiration and the left neck abscess grew Streptococcus pneumonia and a Pseudomonas grew
from the left external canal specimen. Antibiotic therapy was changed to ticarcillin and clavulanate
(Timentin). However, despite this, he continued to spike fevers to 101 and 102 F. On April 2, 1992 a
second CT scan with thin cuts through the temporal bones was then obtained. This study suggested
thrombosis of the left lateral sinus. While arrangements were being made for surgical decompression, an
MR angiogram was performed. This confirmed vascular flow through the right sigmoid sinus and jugular
vein, but no flow through the left lateral sinus or jugular vein. A few hours later, he underwent a left
complete mastoidectomy with incision of the lateral sinus and removal of an obstructing thrombus.
Postoperatively, his antibiotics were changed to Ceftazidime (Fortaz), Amikacin and Penicillin G.
Anticoagulation therapy was instituted for two weeks following surgical decompression. Subsequent
MRA studies showed the development of collateral flow around the left sigmoid sinus and internal
jugular vein. Additionally, reduced flow in the right system persisted. On April 21, 1992, he underwent
right mastoidectomy. An MRA obtained on April 30, 1992 demonstrated unobstructed flow through the
right venous system and no evidence of recanalization through the left sigmoid sinus or jugular vein.
Bibliography
Albert DM, Williams SR. Clinical and anatomical considerations of the TobeyAyer test in lateral sinus thrombosis. J
Laryngol Otol 1986;100:13111313.
Alford BR, Pratt FE. Intracranial complications for otitis media. Texas Med 1966;62:66-70.
Amirmajdi NM. Sigmoid sinus involvement in middleear infection. Laryngoscope 1988;98:310-312.
Coates GM, Ersner MS, Persky AH. Lateral sinus thrombosis with a review of the literature. Ann Otol Rhinol Laryngol
1934;43:419-440.
Cody CC. Thrombosis of the lateral sinus. Arch Otolaryngol 1939;29:674-680.
Courville CB. Intracranial complications of otitis media and mastoiditis in the antibiotic ear. I. Modification of the
pathology of otitic intracranial lesions by antibiotic preparations. Laryngoscope 1955;65:31-46.
Einhaupl KM, Villringer A, Meister W, Mehraein S, Garner C, Pellkofer M, et al. Heparin treatment in sinus venous
thrombosis. Lancet 1991;338:597-600.
Erbguth F, Brenner P, Schuierer G, Druschky KF, Neundorfer B. Diagnosis and treatment of deep cerebral vein
thrombosis. Neurosurg Rev 1991;14:145-148.
Goldenberg RA. Lateral sinus thrombosis: medical or surgical treatment? Arch Otolaryngol 1985;111:5658.
Gower D, McGuirt WF. Intracranial complications of acute and chronic infectious ear disease: a problem still with us.
Laryngoscope 1983;93:1028-1033.
Habib RG, Girgis NI, Abu El Ella AH, Farid Z, Woody J. The treatment of outcome of intracranial infections of otogenic
origin. J Trop Med Hyg 1988;91:83-86.
Hawkins DB. Lateral sinus thrombosis: a sometimes unexpected diagnosis. Laryngoscope 1985;95:674-677.
Holmes FA, Obbens EAMT, Griffin E, Lee YY. Cerebral venous sinus thrombosis in a patient receiving adjuvant
chemotherapy for stage II breast cancer through an implanted central venous catheter. Am J Clin Oncol 1987;10:362-366.
Irving RM, Jones NS, HallCraggs MA, Kendall B. View from within: radiology in focus. CT and MR imaging in lateral
sinus thrombosis. J Laryngol Otol 1991;105:693-695.
Jackson CG, Dickens JRE. Lateral sinus thrombosis. Am J Otolaryngol 1979;1:4951.
Jahrsdoerfer RA, FitzHugh GS. Lateral sinus thrombosis. South Med J 1968;61:1271-1275.
Jensen AM. Sinus thrombosis and otogene sepsis. Acta Otolaryngol 1962;55:237-244.
Kelly KE, Jackler RK. Diagnosis of septic sigmoid sinus thrombosis with magnetic resonance imaging. Otolaryngol Head
Neck Surg 1991;106:617-624.
Kopetzky SJ. Acute and chronic otitis media, sinus thrombosis and suppuration of the petrous pyramid. Arch Otolaryngol
1936;24:505-526.
Kraus M, Tovi F. CNS complications of ear, nose and throat infections: an analysis of 50 consecutive cases. J Otolaryngol
1991;20:329-335.
Lane WA. Five cases of complicated disease of the middle ear. Tr Clin Soc London 1889;22:255-265.
Lund WS. A review of 50 cases of intracranial complications from otogenic infection between 1961 and 1977. Clin
Mas JL, Meder JF, Meary E, Bousser MG. Magnetic resonance imaging in lateral sinus hypoplasia and thrombosis. Stroke
1990;21:1350-1356.
Mathews TJ. Lateral sinus pathology (22 cases managed at Groote Schuur Hospital). J Laryngol Otol 1988;102:118-120.
Meltzer PE. Treatment of thrombosis of the lateral sinus: a summary of the results obtained during twelve years at the
Massachusetts Eye and Ear Infirmary. Arch Otolaryngol 1935;22:131-142.
O'Connell JE. Lateral sinus thrombosis: a problem still with us. J Laryngol Otol 1990;104:949-951.
Pennybacker J, Dixon SW, Fulton CJ. Discussion on intracranial complications of otogenic origin. Proc Roy Soc Med
1961;54:309-320.
Persson L, Lilja A. Extensive dural sinus thrombosis treatment by surgical removal and local streptokinase infusion.
Proctor CA. Intracranial complications of otitic origin. Laryngoscope 1966;76:288-308.
Purvin V, Dunn DW, Edwards M. MRI and cerebral venous thrombosis. Comput Radiol 1987;11:7579.
Samuel J, Fernandes CMC. Lateral sinus thrombosis (A review of 45 cases). J Laryngol Otol 1987;101:1227-1229.
Scott JA, Pascuzzi RM, Hall PV, Becker GJ. Treatment of dural sinus thrombosis with local urokinase infusion : case
report. J Neurosurg 1988;68:284-287.
Seid AB, Sellars SL. Management of otogenic lateral sinus disease at Groote Schuur Hospital. Laryngoscope 1973;83:397403.
Shambaugh GE, Glasscock ME. Surgery of the ear. 3rd ed. Philadelphia:W.B. Saunders, 1980:288-315.
Sneed WF. Lateral sinus thrombosis. Am J Otol 1983;4:258-262.
Teichgraeber JF, PerLee JH, Turner JS. Lateral sinus thrombosis: a modern perspective. Laryngoscope 1982;92:744-751.
Tovi F, Hirsch M, Gatot A. Superior vena cava syndrome: presenting symptom of silent otitis media. J Laryngol Otol
1988;102:623-625.
Tveteras K, Dristensen S, Dommerby H. Septic cavernous and lateral sinus thrombosis: modern diagnostic and therapeutic
principles. J Laryngol Otol 1988;102:877-882.
Vidalihet M, Piette JC, Wechsler B, Bousser MG, Brunet P. Cerebral venous thrombosis in systemic lupus erythematosus.
Stroke 1990;21:1226-1231.
Whitaker CW. Intracranial complications of ear, nose, and throat infections. Laryngoscope 1971;81:1375-1380.
Woflowitz BL. Otogenic intracranial complications. Arch Otolaryngol 1972;96:220-222.
Wright JLW, Grimaldi PMGB. Otogenic intracranial complications. J Laryngol Otol 1973;87:1085-1096.


medical literature.
ACUTE MASTOIDITIS
February 3, 1994
Jennifer L. Parker, M.D.
Acute mastoiditis is rarely seen today. Prior to the discovery of antibiotics, acute mastoiditis was the
most common complication of acute otitis media and often resulted in death. The incidence has dropped
significantly with the advent of antibiotics. Likewise, the frequency of mastoidectomy for this condition
has decreased ten fold, down from 20% in 1938 to 2.8% in 1948 with a 90% decrease in mortality rate.
Acute mastoiditis is a natural extension of acute otitis media. At the onset of infection, acute
inflammation of the middle ear also involves inflammation of the mastoid air cells, which is not
associated with bony resorption and must be differentiated from clinically significant mastoiditis.
Temporal bone development is integral in the ability to develop this disease. The degree of
pneumatization varies greatly in temporal bones. Infection, heredity, ventilation, environment, and
nutrition all play a role in the pneumatization process. Inflammation that develops is easily passed
through these contiguous pneumatized regions. Not only can inflammation spread to these pneumatized
regions, but it can spread to adjacent areas.
The evolution of acute mastoiditis begins when the mucosal lining of the pneumatized cells become
inflamed and produce an exudate. Serosanguinous fluid eventually becomes mucopurulent. Spontaneous
perforation of the tympanic membrane or myringotomy would halt the process at this point. However, 15% of these cases go on to the next phase. The cellular walls of the pneumatized cells then become
demineralized due to: increased osteoclastic activity, pressure of the purulent exudate on the thin bony
septae, and ischemia of the septae secondary to reduced blood flow. As the bony septae breakdown small
abscess cavities form leading to coalescence. Finally the coalescent cells form an empyema, or pus under
pressure, which then escapes to surrounding areas. Treatment of acute mastoiditis depends on the
pathologic stage at which it is encountered.
The bacteriology of acute mastoiditis surprisingly differs from that of acute otitis media. The causative
organism in acute otitis media are usually S Pneumo or H Flu. However, acute mastoiditis is more
commonly due to Group A Beta-Hemolytic Strep and S Pneumo with rare involvement by H. Flu.
Subacute and chronic disease is usually attributed to S. Aureus and gram negative rods such as E. Coli,
Proteus and Pseudomonas.
The signs and symptoms of acute mastoiditis mimic severe acute suppurative otitis media; the disease
entities are distinguished by the duration of symptoms. When the symptoms persist or recur after several
weeks of acute otitis media, they point toward development of a coalescent process within the mastoid.
The most common symptoms are otorrhea and otalgia. Subperiosteal abscess is noted by a fluctuant mass
with overlying edema and erythema. This process produces displacement of the ear downward, outward,
and forward. Sagging of the posterosuperior meatal wall occurs secondary to thickening of the
periosteum overlying the bone in the area of the antrum. The tympanic membrane can simply appear
normal, thickened or can demonstrate a small central perforation. Neurologic changes may be seen with
intracranial complications. Perforation of the mastoid tip along the medial aspect of the SCM through the
incisura mastoidea produces a deep abscess in the neck known as a Bezold's abscess.
Acute mastoiditis is one of the 10 basic complications of acute otitis media. Some worth noting are listed
here. Petrositis is indicated by Gradenigo triad -- acute or subacute otitis media, retro-orbital pain and
abducens palsy. Labyrinthitis due to suppurative ear disease is potentially fatal with spread to the
cerebrospinal fluid producing meningitis. Presence of an extradural abscess is best evaluated by CT scan
but must be excluded intraoperatively by visualizing normal dura through thin intact bone. Sigmoid sinus
thrombosis may be asymptomatic or associated with toxemia or septic emboli. Griesinger's sign may be
encountered and is noted by the presence of edema erythema of the posterior aspect of the mastoid
process associated with mastoid emissary vein thrombosis. The Tobey - Ayer or Queckenstedt test is
usually of historic significance and shows a rise in CSF pressure with occlusion of the normal IJ and NO
change in CSF pressure with occlusion of the thrombosed vein. Brain abscess formation begins with
cerebritis and should be closely monitored if suspected with CT scan every 1-2 weeks.
Evaluation of the patient with acute mastoiditis begins with the history and physical examination. The
majority of the diagnosis is based on clinical judgment. Laboratory evaluation reveals a leukocytosis and
elevated erythrocyte sedimentation rate. Mastoid radiographs are characteristic and will show cloudiness
of the mastoid air cells associated with fuzziness of the bony partitions. Although helpful in the
diagnosis, most agree that mastoid x-rays are not helpful in determining whether mastoid surgery is
necessary. CT scan of the temporal bones is helpful in evaluation of concomitant intracranial
complications as well as discerning any anatomical variations preoperatively. Chest x-ray is useful to rule
out infiltrates in patients with septicemia and possible embolic phenomena secondary to lateral sinus
thrombosis. Tympanocentesis is performed for guidance of antibiotic therapy.
Acute mastoiditis may be associated with periostitis, osteitis, or may be masked. This differentiation is
key in the management of the patient. Acute mastoiditis with periostitis can be described as infection in
the mastoid air cells which spreads to cause inflammation of the overlying periosteum. The route of
infection may be through direct spread through a defect in the mastoid cortex or more commonly through
venous channels, a.k.a. mastoid emissary veins. Periostitis should not be confused with subperiosteal
abscess which requires surgical intervention.
Acute mastoiditis with osteitis is also known as coalescent mastoiditis. Less than 15% of patients with
periostitis will progress to coalescence. This coalescence may spread in several directions. The spread of
the pus anteriorly to the middle ear via the aditus ad antrum producing spontaneous resolution. If the pus
spreads to the soft tissues lying anteromedially a Bezold's abscess may form. The infection may also
spread lateral to produce a subperiosteal abscess. The spread of pus medially to the petrous air cells
causes petrositis. Posterior spread to the occipital bone may result in osteomyelitis of the calvarium a.k.a.
Citelli abscess.
Masked mastoiditis has an insidious course, owing to the use of broad spectrum antibiotics to treat
middle ear disease. The apparent resolution of symptoms of acute otitis media "masks" the development
of mastoiditis and leads to the presentation with intracranial complications. The presence of pathology
may only be apparent on careful neurologic examination. Diagnosis is usually made on CT of the
temporal bones which demonstrates temporal bone pathology in conjunction with the accompanying
intracranial complication.
Acute mastoiditis can also occur in conjunction with chronic ear disease. It has been the teaching in the
past that acute mastoiditis only occurred in well pneumatized mastoids in which the thin bony trabeculae
are easily broken down. Chronic ear disease has long been associated with a sclerotic "cue - ball" like
mastoid which is less susceptible to demineralization. However, several studies note the presence of
cholesteatoma in patients with acute mastoiditis.
Antibiotic therapy is guided by the patients history. If the history is otherwise uncomplicated by ear
disease or protracted episode of otitis media, the infecting organism is probably S. pneumo or S.
pyogenes which is best treated by ampicillin. If the patient has a protracted course of otitis media then
coverage for S. Aureus and gram negative organisms is necessary with a penicillinase resistant penicillin
and an aminoglycoside or single antibiotic coverage with a cephalosporin. Anaerobic coverage should be
added to the above therapy when suspected.
There exists controversy over the management of this disease. All studies on acute mastoiditis are
retrospective and conclusions drawn regarding therapy are limited. There have been no prospective or
controlled studies on the surgical and medical management of the disease. This is limited by the relative
rarity of the disease in the current day. Options for treatment are antibiotics alone, antibiotics plus
myringotomy, Incision and drainage of abscess, and mastoidectomy. If periostitis is present
tympanocentesis followed by myringotomy should be performed with placement on intravenous
antibiotics directed toward gram stain results. It is preferable to insert pressure equalization tubes for
drainage over a longer period of time. This periosteal involvement usually resolves within 24 to 48 hours
after initiation of therapy. If clinical deterioration occurs, manifest by persistent fever and otalgia, or
development of subperiosteal abscess, then further surgical intervention is indicated. Analysis of the
literature reveals approximately 57% of cases are able to be treated in this manner.
Indications for surgery include acute mastoiditis with subperiosteal abscess, acute mastoiditis not
responsive after 24 to 48 hours of intravenous antibiotics and myringotomy, and intracranial
complications with evidence of mastoid coalescence. These indications were derived from the current
literature.
Some have managed the disease with simple I & D of the abscess which does not afford decompression
and drainage of the underlying mastoid. There have been no studies to evaluate the efficacy of this mode
of therapy versus mastoidectomy. Most reported cases of simple I & D have been anecdotal. 60% of the
cases reported in the literature have come to mastoidectomy after I & D.
The procedure of choice is a complete simple mastoidectomy. Simple mastoidectomy is an emergency
procedure and should be performed on a clinically stable patient with control of sepsis before undergoing
anesthesia. Mastoidectomy may be performed at a later date if the patient is unstable after drainage of
intracranial process. The goal of surgery is to effect drainage. Establishment of good communication
between the middle ear and mastoid is achieved by removing the edema and granulation in the aditus ad
antrum. External drainage is necessary with a drain in the mastoid cavity to drain the antrum, and a
pressure equalization tube inserted to ventilate the middle ear space.
In conclusion, acute mastoiditis is a diagnosis based on clinical acumen. When acute mastoiditis is
suspected a careful history and physical examination should be ascertained to evaluate for intratemporal
or intracranial complications. The presence of acute mastoiditis does not exclude the presence of
underlying chronic ear disease, and should be taken into account in the evaluation and treatment of the
patient. The presence of subperiosteal abscess or progression of symptoms on the above therapy warrants
surgical intervention. Simple mastoidectomy is the surgery of choice. Prospective controlled studies are
necessary to firmly identify indications for medical versus surgical therapy.
Case Presentation
An 11-year-old boy with a history of chronic otitis media with effusion presented with a 10-day history
of fever, right otalgia, and a right, dull occipital headache. The headache was not associated with
photophobia. A CT scan of the head revealed a right parieto-occipital epidural abscess.. Physical
examination revealed a young, alert boy with a temperature of 102F. Examination of his right ear
revealed a thickened, but intact tympanic membrane and middle ear effusion. Postauricular edema,
erythema, tenderness, and fluctuance was evident. The remainder of the physical examination, including
a neurological exam, was normal. White blood cell count was 18.7 cells/mm3 with a left shift. CT scan
of the temporal bones revealed soft tissue changes within the middle ear and mastoid and an overlying
subperiosteal abscess and possible lateral sinus thrombosis.
The patient was taken to the operating room and underwent bur hole drainage and irrigation of a right
parieto-occipital epidural abscess by the neurosurgical team followed by a right simple mastoidectomy,
exploration of the lateral sinus, insertion of a pressure equalization tube, and Penrose drainage of the
mastoid cavity. Findings included coalescent mastoiditis and no evidence of lateral sinus thrombus.
Intraoperative cultures grew microaerophilic streptococcus; the antibiotic regimen was changed to
Cefotaxime and Penicillin G. The right temporoparietal cerebritis showed no progression to brain abscess
and no neurologic sequelae. Drains were discontinued when drainage decreased. The patient was
discharged on the thirteenth postoperative day in good condition to continue intravenous antibiotics for a
total course of 6 weeks. Weekly CT scan of the head demonstrated slow resolve of the cerebritis. The
patient has had no further episodes of otitis media and is currently doing well in the sixth grade.
Bibliography
Alford BR, Pratt FE. Intracranial complications from otitis media. Texas Med 1966;62:66-70.
Allam AF. Pneumatization of the temporal bone. Ann Otol Rhinol Laryngol 1969;78:49-64.
Allam AF, Schuknecht HF. Pathology of petrositis. Laryngoscope 1968;78:1813-1832.
Arcand P, Cerat J, Spenard JR. Acute otomastoiditis in the leukemic child. J Otolaryngol 1989;18:380-383.
Bluestone CD, Klein JO. Otitis Media in Infants and Children. Philadelphia: WB Saunders, 1988.
Davison FW. Otitis media - then and now. Laryngoscope 1955;65:142-151.
Dolowitz DA, Wakefield RH. A case of mastoiditis without apparent otitis media. Ann Otol Rhinol Laryngol 1949;58:512516.
Eby TL, Nadol BJ Jr. Postnatal growth of the human temporal bone: implications for cochlear implants in children. Ann
Faye-Lund H. Acute and latent mastoiditis. J Laryngol Otol 1989;103:1158-1160.
Ginsburg CM, Rudoy R, Nelson JD. Acute mastoiditis in infants and children. Clin Pediatr 1980;19:549-553.
Glasscock ME III, Shambaugh GE Jr. Surgery of the Ear, 4th ed. Philadelphia: WB Saunders, 1990.
Goodwin MR. Acute mastoiditis and acute labyrinthitis without mastoidectomy. Laryngoscope 1968;78:227-235.
Goodwin MR. Complicated acute mastoiditis treated conservatively. N Y State J Med 1969;69:594-598.
Hawkins DB, Dru D. Mastoid subperiosteal abscess. Arch Otolaryngol 1983;109:369-371.
Hawkins DB, Dru D, House JW, Clark RW. Acute mastoiditis in children: a review of 54 cases. Laryngoscope
1983;93:568-572.
Holt GR, Young WC. Acute coalescent mastoiditis. Otolaryngol Head Neck Surg 1981;89:317-321.
Jazrawy H, Wortzman G, Kassel EE, Noyek AM. Computed tomography of the temporal bone. J Otolaryngol 1983;12:3744.
Juselius H, Kaltiokallio K. Complications of acute and chronic otitis media in the antibiotic era. Acta Otolaryngol
1972;74:445-450.
Lim DJ, DeMaria TF. Pathogenesis of otitis media. Bacteriology and immunology. Laryngoscope 1982;92:278-287.
Maharaj D, Jadwat A, Fernandes CM, Williams B. Bacteriology in acute mastoiditis. Arch Otolaryngol Head Neck Surg
1987;113:514-515.
Mathews TJ. Acute and acute-on-chronic mastoiditis (a five-year experience at Groote Schuur Hospital). J Laryngol Otol
1988;102:115-117.
McKenzie W. The Schwartze mastoidectomy, its value at the present time. Arch Otolaryngol 1958;67:417-423.
Meyerhoff WL, Giebink GS, Shea DA, Le CT. Effect of tympanostomy tubes on the pathogenesis of acute otitis media.
Am J Otolaryngol 1982;3:189-195.
Meyerhoff WL, Gates GA, Montalbo PJ. Pseudomonas mastoiditis. Laryngoscope 1977;87:483-492.
Milstein S. The history of mastoid surgery. Am J Otol 1980;1:174-178.
Neely JG: Complications of suppurative otitis media, Part 1: Aural complications. American Academy of Otolaryngology
Self-Instruction Package 78300. Rochester, 1978.
Nielsen JM. Intracranial complications of otitis media and mastoiditis in the antibiotic era. II. Intracranial complications of
otitis media as modified by sulfa drugs and antibiotics - clinical aspects. Laryngoscope 1955;65:47-52.
Ogle JW, Lauer BA. Acute mastoiditis: diagnosis and complications. Am J Dis Child 1986;1178-1182.
Palva T, Virtanen H, Makinen J. Acute and latent mastoiditis in children. J Laryngol Otol 1985;99:127-136.
Palva T, Pulkkinen K. Mastoiditis. J Laryngol Otol 1959;78:573-588.
Palva T. Mastoiditis in children. Laryngoscope 1962;72:353-360.
Paparella MM, Meyerhoff WL, Oliviera CA. Mastoiditis and brain hernia (mastoiditis cerebri). Laryngoscope
1978;88:1097-1106.
Pfaltz CR, Griesemer C. Complications of acute middle ear infections. Ann Otol Rhinol Laryngol Suppl 1984;112:133137.
Ronis BJ, Ronis ML, Liebman EP. Acute mastoiditis as seen today. Eye Ear Nose Throat 1968;47:502-507.
Rosen A, Ophir D, Marshak G. Acute mastoiditis: a review of 69 cases. Ann Otol Rhinol Laryngol 1986;95:222-224.
Rubin JS, Wei WI. Acute mastoiditis: a review of 34 patients. Laryngoscope 1985;95:963-965.
Samuel J, Fernandes CM. Otogenic complications with an intact tympanic membrane. Laryngoscope 1985;95:1387-1390.
Scheibel WR, Urtes MA. Mastoiditis. Am Fam Physician 1987;35:123-127.
Scott TA, Jackler RK. Acute mastoiditis in infancy: a sequela of unrecognized acute otitis media. Otolaryngol Head Neck
Surg 1989;101:683-687.
Shaffer HL, Gates GA, Meyerhoff WL. Acute mastoiditis and cholesteatoma. Otolaryngol 1978;86:ORL394-399.
Smouha EE, Levenson MJ, Anand VK, Parisier SC. Modern presentations of Bezold's abscess. Arch Otolaryngol Head
Neck Surg 1989;115:1126-1129.
Sorensen H. Antibiotics in suppurative otitis media. Otolaryngol Clin North Am 1977;10:45-50.
Venezio FR, Naidich TP, Shulman ST. Complications of mastoiditis with special emphasis on venous sinus thrombosis. J
Pediatr 1982;101:509-513.


medical literature.
SURGICAL MANAGEMENT OF MENIERE'S SYNDROME

April 14, 1994
Glenn Knox, M.D.
Although the underlying etiology of Meniere's syndrome is unknown, a consistent histopathologic finding
is hydrops (dilatation) of the endolymphatic spaces. The hydrops presumably results from a malfunction
of the resorptive function of the endolymphatic sac. The classic constellation of symptoms includes:
fluctuating hearing loss, episodic vertigo, tinnitus, and a sensation of fullness in the ear. These symptoms,
however, do not necessarily develop simultaneously and many patients do not develop them at all.
Subcategories of Meniere's syndrome describe these other conditions; for instance, cochlear hydrops
(fluctuating hearing loss alone) or vestibular hydrops (vestibular symptoms without hearing loss).
In most patients, Meniere's syndrome is ultimately self-limited; over time the patient suffers deterioration
of hearing and a gradual subsiding of the episodic dizzy spells. This evolution, however, may require 1 to
2 decades. In the interim, the patient's lifestyle may be severely impaired.

A few patients cannot be adequately managed by medical means alone and surgical intervention must be
considered. The surgical procedures for Meniere's disease may be categorized as those designed to alter
the function of the vestibular system and those that ablate the vestibular system either with or without
preservation of hearing.
Case Presentation
A 58-year-old white female noted the onset of fluctuating right hearing loss, right tinnitus and vertigo
eight years prior to presentation. For the first 5 years her symptoms were controlled with sodium
restriction and diuretics. Three years prior to presentation she began to experience intense episodic
vertigo associated with nausea and vomiting. Two years prior to presentation she underwent a right
endolymphatic shunt procedure with some relief of her symptoms. After a 6-month period the vertigo and
dizziness returned. She underwent a revision of the endolymphatic shunt 1 year prior to presentation,
again followed by initial relief and then recurrence of symptoms. She was referred to The Methodist
Hospital for further evaluation. Preoperative evaluation showed predominantly low-frequency
sensorineural hearing loss in the right ear, with a PTA of 45 and a PBmax of 40%. Left ear showed a PTA
of 10 and PBmax of 100%. ENG showed right unilateral weakness. ABR was normal bilaterally. The
patient underwent a right middle cranial fossa vestibular nerve section. Postoperatively the patient did
well and has been free of vertiginous episodes
Bibliography
Amedee RG, Norris CH, Risey JA. Selective chemical vestibulectomy: preliminary results with human application.
Andrews JC, Ator GA, Honrubia V. The exacerbation of symptoms in Meniere's disease during the premenstrual period.
Austin DF. Class A results in Meniere's disease. Am J Otol 1991;12:317-322.
Barun V, Richter HP. Results of 100 consecutively operated patients with acoustic neuroma. Nervenarzt 1993;64:238-243.
Bohmer A, Fisch U. Bilateral vestibular neurectomy for treatment of vertigo. Otolaryngol Head Neck Surg1993;109:101107.
Boles R, Rice DH, Hybels R, Work WP. Conservative management of Meniere's disease: Furstenberg regimen revisited.

Brackmann DE. Surgical treatment of vertigo. J Laryngol Otol 1990;104:849-859.
Cass SP, Kartush JM, Graham MD. Clinical assessment of postural stability following vestibular nerve section.
Laryngoscope 1991;101:1056-1059.
Cass SP, Kartush JM, Graham MD. Patterns of vestibular function following vestibular nerve section. Laryngoscope
1992;102:388-394.
Cody DT. The tack operation for endolymphatic hydrops. Laryngoscope 1969;79:1737-1744.
Colebatch JG, Halmagyi GM. Vestibular evoked potentials in human neck muscles before and after unilateral vestibular
deafferentation. Neurology 1992;42:1635-1636.
Curthoys IS, Dai MJ, Halmagyi GM. Human ocular torsional position before and after unilateral vestibular neurectomy.
Exp Brain Res 1991;85:218-225.
Debruyne F. Clinical observations on the on-off effect of the acoustic stapedius reflex. Clin Otolaryngol 1992;17:10-12.
Derebery MJ, Valenzuela S. Meniere's syndrome and allergy. Otolaryngol Clin North Am 1992;25:213-224.
Fick IA. Decompression of the labyrinth: a new surgical procedure for Meniere's disease. Arch Otolaryngol 1964;79:447458.
Furman JM, Schor RH, Kamerer DB. Off-vertical axis rotational responses in patients with unilateral peripheral vestibular
lesions. Ann Otol Rhinol Laryngol 1993;102:137-143.
Gibson WP. The use of intraoperative electrocochleography in Meniere's surgery. Acta Otolaryngol Suppl 1991;485:65-73.
Giddings NA, Shelton C, O'Leary MJ, Brackmann DE. Ocohleosacculotomy revisited: long-term results poorer than
expected. Arch Otolaryngol Head Neck Surg 1991;117:1150- 1152.
Goin DW, Mischke RE, Esses BA, Young D, Priest EA, Whitmoyer-Goin V. Hearing results from endolymphatic sac
surgery. Am J Otol 1992;13:393-397.
Graham MD. Transmastoid labyrinthectomy: further experience with the indications, complications and early postoperative
results. J Laryngol Otol 1981;95:1205-1211.
Graham MD, Colton JJ. Transmastoid labyrinthectomy: indications, technique and early postoperative results.
Laryngoscope 1980;90:1253-1263.
Green JD Jr, Blum DJ, Harner SG. Longitudinal follow-up of patients with Meniere's disease. Otolaryngol Head Neck Surg
1991;104:783-788.
Halmagyi GM, Curthoys IS, Todd MJ, D'Cruz DM, Cremer PD, Henderson CJ, Staples MJ. Unilateral vestibular
neurectomy in man causes a severe permanent horizontal vestibulo- ocular reflex deficit in response to high-acceleration
ampullofugal stimulation. Acta Otolaryngol Suppl 1991;481:411-414.
Hausler R, Kasper A, Demierre B. Intraoperative electrically evoked vestibular potentials in humans. Acta Otolaryngol
1992;112:180-185.
Heermann J. Predominance of left ear in Meniere's disease, sudden deafness, inner ear damage, tinnitus and abnormally
patent Eustachian tube. Ear Nose Throat J 1993;72:205- 208.
Hickey SA, O'Commor AF. Measurement of drill-generated noise levels during ear surgery. J Laryngol Otol 1991;105:732735.
Huang TS, Lin CC. Endolymphatic sac surgery for refractory luetic vertigo. Am J Otol 1991;12:184-187.
Huang TS, Lin CC. Revision endolymphatic sac surgery for recurrent Meniere's disease. Acta Otolaryngol Suppl
199;485:131-144.
Huang TS, Linn C. Surgical treatment of chronic otitis media and Meniere's syndrome. Laryngoscope 1991;101:900-904.
Huang TS, Lin CC, Chang YL. Endolymphatic sac surgery for Meniere's disease: a cumulative study of twelve years'
experience. Acta Otolaryngol Suppl 1991;485:145-154.
Hughes GB, et al. Autoimmune reactivity in Meniere's disease: a preliminary report. Laryngoscope 1983;93:410-417.
Itoh A, Sakata E. Treatment of vestibular disorders. Acta Otolaryngol Suppl 1991;481:617- 623.
Jackson CG, personal communication.
Karmody CS, Schuknecht HF. Deafness in congenital syphilis. Arch Otolaryngol 1966;83:18- 27.
Kemink JL, Telian SA, Graham MD, et al. Transmastoid labyrinthectomy: reliable surgical management of vertigo.
Li CW, Cousins V, Hooper R. Vestibulo-ocular compensation following unilateral vestibular deafferentation. Ann Otol
Linstrom CJ, Stockwell CW. Clinical evaluation of the monothermal caloric test. Otolaryngol Head Neck Surg
1993;108:27-35.
Love JT. Basilar artery migraine presenting as fluctuating hearing loss and vertigo. Otolaryngol Head Neck Surg
1978;86:450-458.
Luetje CM. Hearing results following posterior semicircular canal injury during antesigmoid retrolabyrinthine selective
vestibular nerve section. Am J Otol 1992;13:600-602.
McCabe B. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 1979;88:585- 589.
Maddox D, personal communication.
Magliulo G, Vingolo GM, Petti R, de Vincentiis M. Vestibular neurectomy by the infralabyrinthine approach. J Laryngol
Otol 1992;106:780-782.
Magnan J, Bremond G, Chays A, Gignac D, Florence A. Vestibular neurotomy by retrosigmoid approach: technique,
indications, and results. Am J Otol 1991;12:101-104.
Moffat DA, Baguley DM, Harries ML, Atlas M, Lynch CA. Bilateral electrocochleographic findings in unilateral Meniere's
disease. Otolaryngol Head Neck Surg 1992;107:370-373.
Moffat DA, Toner JG, Baguley DM, Hardy DG. Posterior fossa vestibular neurectomy. J Laryngol Otol 1991;105:10021003.
Monsell EM, Shelton C. Labyrinthotomy with streptomycin infusion: early results of a multicenter study. The LSI
Multicenter Study Group. Am J Otol 1992;13:416-425.
Nedzelski JM, Schessel DA, Bryce GE, Pfleiderer AG. Chemical labyrinthectomy: local application of gentamicin for the
treatment of unilateral Meniere's disease. Am J Otol 1992;13:18-22.
Nguyen CD, Brackmann DE, Crane RT, Linthicum FH Jr, Hitselberger WE. Retrolabyrinthine vestibular nerve section:
evaluation of technical modification in 143 cases. Am J Otol 1992;13:328-332.
Nomura Y, Okuno T, Hara M, Young YH. "Floating" labyrinth: pathophysiology and treatment of perilymph fistula. Acta
Otolaryngol 1992;112:186-191.
Ohlms LA, Lonsbury-Martin BL, Martin GK. The clinical applications of acoustic distortion products. Otolaryngol Head
Neck Surg 1990;103:52-59.
Paparella MM, DaCosta SS, Fox R, Yoon TH. Meniere's disease and other labyrinthine diseases. In: Paparella MM,
Shumrick DA, Gluckman JL, Meyerhoff WL, eds. Otolaryngology. Philadelphia: WB Saunders, 1991:1689-1714.
Parnes LS, Campbell KC. Chronic perilymph fistula in the guinea pig with implications in the human. Ann Otol Rhinol
Laryngol 1992;101:176-182.
Pulec JL. Meniere's disease: results of two and a half years' study of etiology, natural history and results of treatment.
Laryngoscope 1972;82:1703-1715.
Pulec JL. The otic-periotic shunt. Otolaryngol Clin North Am 1968;1:643-648.
Pulec JL. The surgical treatment of vertigo. Laryngoscope 1979;79:1783-1822.
Ramsden RT, Timms MS. Promontory stimulation following labyrinthectomy. J Laryngol Otol 1991;105:729-731.
Rassekh CH, Harker LA. The prevalence of migraine in Meniere's disease. Laryngoscope 1992;102:135-138.
Report of the Committee on Hearing and Equilibrium. Trans Am Acad Ophthalmol Otolaryngol 1972;76:1462-1464.
Rosenberg S, Silverstein H, Flanzer J, Wanamaker H. Bilateral Meniere's disease in surgical versus nonsurgical patients.
Am J Otol 1991;12:336-340.
Ruth RA. Trends in electrocochleography. J Am Acad Audiol 1990;1:134-137.
Schuknecht HF. Cochleosacculotomy for Meniere's disease: internal endolymphatic shunt. Oper Tech Otolaryngol
1991;2:35-37.
Schuknecht HF. Myths in neurotology. Am J Otol 1992;13:124-136.
Schuknecht HF. Pathology of Meniere's disease as it relates to the sac and tack procedures. Ann Otol Rhinol Laryngol
1977;86:677-682.
Schuknecht HF. Pathology of the Ear. Cambridge, MA: Harvard Univ Press, 1974.
Schuknecht HF. Transcanal labyrinthectomy. Oper Tech Otolaryngol 1991;2:17-19.
Schwaber MK, Kveton J. Transmastoid labyrinthectomy. Oper Tech Otolaryngol 1991;2:20- 22.
Schwaber MK, Whetsell WO. Cochleovestibular nerve compression syndrome. II. Vestibular nerve histopathology and
theory of pathophysiology. Laryngoscope 1992;102:1030-1036.
Shambaugh GE Jr. Decompression of the endolymphatic sac for hydrops. Otolaryngol Clin North Am 1968;1:613-621.
Shea JJ. Medical and surgical treatment of Meniere's disease. Ann Acad Med Singapore 1991;20:686-689.
Shea JJ, Norris CH. Streptomycin perfusion of the labyrinth. Acta Otolaryngol Suppl 1991;485:123-130.
Shotton JC, Gresty MG, Ludman H, Metcalfe T. Clinical and vestibulometric status following labyrinthectomy or
vestibular nerve section for Meniere's disease. Clin Otolaryngol 1992;17:332-336.
Silverstein H, Norrell H. Retrolabyrinthine vestibular neurectomy. Otolaryngol Head Neck Surg 1982;90:778-782.
Silverstein H, Wanamaker H, Flanzer J, Rosenberg S. Vestibular neurectomy in the United States - 1990. Am J Otol
1992;13:23-30.
Sperling NM, Paparella MM, Yoon TH, Zelterman D. Symptomatic versus asymptomatic endolymphatic hydrops: a
histopathologic comparison. Laryngoscope 1993;103:277-285.
Spindler J. Negative relationships between responses from head-shake and caloric tests. Ann NY Acad Sci 1992;656:892http://www.bcm.edu/oto/grand/41494.html (6 of 8) [5.11.2005 10:43:16]
894.
Stouffer JL, Tyler RS, Kileny PR, Dalzell LE. Tinnitus as a function of duration and etiology: counselling implications.
Am J Otol 1991;12:188-194.
Suzuki M, Kitahara M. Immunologic abnormality in Meniere's disease. Otolaryngol Head Neck Surg 1992;107:57-62.
Teixido M, Wiet RJ. Hearing results in retrolabyrinthine vestibular neurectomy. Laryngoscope 1992;102:33-38.
Telischi FF, Luxford WM. Long-term efficacy of endolymphatic sac surgery for vertigo in Meniere's disease. Otolaryngol
Head Neck Surg 193;109:83-87.
Thomsen J, Bretlau P, Tos M, Johnsen NJ. Endolymphatic sac - mastoid shunt surgery: a nonspecific treatment modality?
Thomsen J, Bretlau P, Tos M, Johnsen NJ. Placebo effect in surgery for Meniere's disease. Arch Otolaryngol 1981;107:271277.
Uemura K. Some neurosurgical and clinical neurophysiological notes on selected neurootological problem, with special
reference to acoustic neurinoma, brain death and NVC. Acta Otolaryngol Suppl 1993;503:174-187.
Viscomi GJ, Bojrab DI. Use of electrocochleography to monitor antigenic challenge in Meniere's disease. Otolaryngol
Head Neck Surg 1992;107:733-737.
Wackym PA, Storper IS, Fu YS, House WF, Ward PH. Differential diagnosis of virus-like particles in the human inner ear.
Am J Otol 1992;13:431-437.
Wolf SR, Christ P, Haid CT. Patient use of telemetric ENG to register nystagmus in the private sphere. Laryngoscope
1993;103:704-707.
Wolf SR, Christ P, Haid CT. "Telemetric" electronystagmography: a new method for examination of nystagmus outside the
clinic. Acta Otolaryngol Suppl 1991;481:374-381.
Wolfe GI, Taylor CL, Flamm ES, Gray LG, Raps EC, Galetta SL. Ocular tilt reaction resulting from vetibuloacoustic nerve
surgery. Neurosurgery 1993;32:417-421.
Yoo TJ, et al. Type II collagen autoimmunity in otosclerosis and Meniere's disease. Science 1982;217:115301155.
Yoshida GY, Woods TR, Barrs DM. Mastoid pneumatocele: unusual complication after transmastoid translabyrinthine
labyrinthectomy. Otolaryngol Head Neck Surg 1991;105:877- 880.


medical literature.
MYRINGOPLASTY AND TYMPANOPLASTY

February 16, 1995
Rance W. Raney, M.D.
Introduction
Tympanoplasty without mastoidectomy is performed to control infection through eradication of disease
and to reconstruct the sound conducting mechanism. Tympanic membrane grafting may or may not be
required. This presentation focuses on tympanoplasty types I through III and ossiculoplasty. It is not a
detailed discussion of otitis media, mastoidectomy or stapedectomy.
Anatomy
Horst Wullstein (1956) said, "The tympanic membrane has two functions, sound pressure transformation
for the oval window and sound protection of the round window." The outer ear functions to capture air
pressure waves. The middle ear functions to convert air pressure waves efficiently into endolymphatic
fluid waves, and the inner ear (cochlea) serves to convert fluid waves into nerve impulses. This soundtransforming mechanism includes the ossicles whose lever action adds 3 dB to the sound level at the oval
window. The hydraulic principle explains how the difference in the surface area of the tympanic
membrane and the stapedial footplate increases the hearing level by 27 dB. The tympanic membrane also
serves to protect the round window from sound by efficiently conducting the pressure waves to the oval
window and delaying the arrival of pressure waves to the round window.
Middle ear pathology associated with perforation or a conductive deficit includes otitis media, otitis
externa, granulation tissue and trauma including myringotomy. Other disorders can interfere with the
ventilatory or conducting function of the middle ear without producing perforations. These include
tympanosclerosis, otosclerosis, congenital cholesteatoma, and Eustachian tube dysfunction.
The Eustachian tube, or pharyngotympanic tube, originates in the middle ear and extends into the
nasopharynx. It is normally closed, but opens for 0.1 to 0.2 seconds during swallowing to allow air to
move between the nasopharynx and middle ear to equalize the pressure across the tympanic membrane.
There is no doubt that Eustachian tube dysfunction can lead to negative pressure in the middle ear and
retraction of the tympanic membrane. But, we cannot distinguish which patients have retractions from
previous tubal dysfunction and which patients have persistent tubal dysfunction and will tend to reform
retractions after surgery.
Preoperative Evaluation
The examiner should make note of otorrhea, hearing loss, otalgia, vertigo and facial weakness when
evaluating a patient with middle ear pathology. A hearing loss of 30 dB or more may represent ossicular
disruption. Progressive loss with no obvious middle ear pathology on exam may represent
tympanosclerosis or otosclerosis.
A microscopic exam should be performed and pneumatic otoscopy used to assess the mobility of the
tympanic membrane and the malleus. A Fistula Test can be performed if there is a history of dizziness or
a marginal perforation.
Surgical Technique
The particular technique that will best repair the tympanic membrane is impossible to determine
preoperatively with confidence. The final decision regarding medial or lateral placement of the graft or
the ossiculoplasty to be performed must be reserved until the ear has been examined under anesthesia.
Wullstein created a classification scheme in 1956 identifying five basic types of tympanoplasty. Type I is
myringoplasty. The Type II tympanoplasty is for tympanic membrane perforations with erosion of the
malleus. It involves grafting on to the incus or the remains of the malleus. The Type III tympanoplasty is
indicated for destruction of the lateral ossicles, but with an intact and mobile stapes. It involves placing a
graft onto the stapes and providing protection for the round window. The Type IV tympanoplasty is used
for ossicular destruction including destruction of all or part of the stapes arch. It involves placing a graft
onto or around a mobile stapes footplate. The resulting middle ear consists of the hypotympanum and the
Eustachian tube orifice only. The impedance matching system is abandoned. The Type V tympanoplasty
is used when the footplate of the stapes is fixed. The Paparella modification Type Va with fenestration of
the horizontal canal, has largely been abandoned in favor of Type Vb with stapedectomy. Farrior (1971)
proposed subclassifications based on the resultant ossicular/ prosthesis arrangement.
There are two basic approaches to tympanoplasty without mastoidectomy. The retroauricular and the
transaural approaches are equally successful, but most surgeons choose a retroauricular approach to
anterior perforations.
Tympanic membrane grafting can be accomplished by medial or lateral grafting. Medial grafting, also
known as the underlay technique, involves creating a tympanomeatal flap via a canal incision and
elevation of the annulus and tympanic membrane. Graft material is then secured between the tympanic
membrane and a bed of Gelfoam placed in the middle ear. Lateral grafting can be performed with a
variety of materials. One technique uses an overlay graft of temporalis fascia and free canal skin. This
produces a thick, well-vascularized graft that will clean itself by epithelial migration, but care must be
taken to maintain the acute angle between the drum and the canal anteriorly, which is essential for a good
hearing result. Recurrent retraction pockets can be prevented for the most part, by cartilage grafting and
scutumplasty.
Graft Materials
Materials used for tympanic membrane repair include autologous grafts (from the same person),
homografts (from other humans), xenografts (from animals), or allografts (synthetic materials). Rambo
(1958) described the use of autologous muscle in a procedure he called a musculoplasty. Fat patch
myringoplasty is an autologous tissue technique. Autologous temporalis fascia and canal skin are used
for medial and lateral grafting. Replacing the tympanic membrane with cartilage prevents subsequent
retractions, but obviously shielding large portions of the drum with cartilage causes a substantial hearing
loss. A composite cartilage-perichondrium graft taken from the tragus was evaluated by Poe and Gadre
(1993), who found that reinforcing the posterior quadrants and pars flaccida with cartilage did prevent
retractions.
Homograph materials used in tympanoplasty have included dura, tympanic membranes, ossicles, corneas
and banked cadaveric materials such as tympanic membranes with attached mallei, including sclera,
infant dura, and nasal septal cartilage. One potential drawback of homografts is the theoretical risk of
transmitting pathogens such as HIV. The fixation process inactivates viral particles and the remaining
material is organic but non-viable. The grafts are implants not transplants. Yet, the fear of litigation has
decreased the number of hospitals willing to bank organic materials. Other potential disadvantages
include the additional time required to sculpt and the cost of tissue banking.
Allograft membranes incorporating wire, metal, ceramic, and plastic, have been largely unsuccessful
according to Campbell (1990). The disadvantage of using allografts is their tendency for extrusion.
Ossiculoplasty
The ossicles may become fixed or lose continuity. They may be eroded by a mass or inflammatory
process, or they may be congenitally malformed. Any of these can prevent the transmission of sound to
the inner ear.
Austin, in 1971, devised a system for classifying ossicular defects. He reasoned that loss of the malleus
head or neck was not important to reconstruction, and that the incus would be mobilized for
reconstruction. So, the condition of the handle of the malleus and the stapes superstructure is the relevant
issuein ossiculoplasty. He classified the four possible defects as types A through D, and noted that most
commonly both the incus and stapes are present. The next most common situation is to have an isolated
defect in the stapes arch. This scheme can aid in planning reconstruction.
Since 1964, homograft ossicles have been used to reconstruct the ME mechanism. The incus could be
prepared in two ways. The notched incus with short process can connect the malleus to the capitulum of
the stapes. The notched incus with long process can connect the malleus to the stapes footplate when the
stapes superstructure was missing or removed. In 1986, hydroxylapatite prostheses became available in
two lengths as an incus or incus-stapes substitute.
Several maneuvers are key to the success of an ossiculoplasty. Special attention should be given to
dislocating the incudostapedial joint, ensuring mobility of the footplate, removing mucosa from the
malleus handle, and popping the prosthesis in place. Malleus head fixation is addressed by dislocation of
the I-S joint followed by dislocation of the I-M joint and removal of the malleus head. Erosion of the
manubrium of the malleus can be addressed by reconstruction with a homograft tympanic membrane and
malleus or use of a PORP (malleus-incus) prosthesis. If the malleus handle and the stapes arch are
absent, a TORP (malleus-incus-stapes) can be used.
There are a wide variety of prostheses available to the surgeon. The stapes prostheses are either wire
loops or pistons. Some of the pistons have a cup rather than a crook to hold the incus. The incus
prostheses come as struts or struts with side arms. Incus-stapes prostheses come in more than 9 types.
They are struts or pistons of stainless steel alone or together with a polymer.
PORP's (partial ossicular reconstruction prostheses) are for lateral chain reconstruction in the absence of
the malleus handle. They have a broad lateral platform designed to minimize point pressure on the TM to
resist extrusion. There are 13 TORP's (total ossicular reconstruction prostheses), available for use as
malleus-incus-stapes substitutes. Ossicular prostheses range in price from about $39.00 to $263.00, and
are constructed of stainless steel, platinum, tantalum, polyethylene, Plasti-pore, fluoroplastic, and
hydroxylapatite.
Staging Tympanoplasty
In 1991, James Sheehy reviewed the current thinking regarding staging of tympanoplasty in badly
diseased ears. Staged operations have been preferred since the mid-1960's at the House Institute and
elsewhere. The first operation would eliminate disease and aerate the middle ear cleft, and the second
would reconstruct the sound pressure transfer mechanism. There are three requirements for a successful
functional result in tympanoplasty: 1) a tympanic membrane; 2) an air-filled, mucosa-lined middle ear
space; and, 3) a secure connection between the vibrating tympanic membrane and the inner ear fluids.
The need for staging is determined in part, by the second of these: a mucosa lined middle ear space. If
there is minimal mucosal disease at the time of the initial operation, there is no need for staging and the
conductive mechanism may be reconstructed immediately. Often large areas of mucosa are diseased.
Infection must be controlled before healthy mucosa can regenerate. Edematous mucosa and uninfected
granulations are reversible. Mucosa should not be stripped if there is any possibility of rejuvenation,
because denuded areas are prone to formation of synechiae or adhesions, scarring or granulation. Such
scarring can impair drum and ossicular mobility. Another obstacle to the regeneration of the mucosal
lining is the presence of residual squamous epithelium in the middle ear. Remnants from a cholesteatoma
or epithelium migrating medially through a perforation may take root on denuded bone in the middle ear.
If removal of the middle ear disease denudes large areas of the medial wall, staging is necessary to
prevent fibrosis of the middle ear cleft. Staging may also enhance the hearing result by allowing the new
drumhead to "settle" before ossiculoplasty, thereby reducing the risk of lateralization (Donaldson and
Snow, 1992).
Some believe that although staged procedures may produce slightly better hearing result, they are often
unnecessary. This is because many patients with extensive middle ear disease heal amazingly well after
the first procedure. The inability to predict who will heal well leads some to reconstruct the conductive
mechanism in a single procedure hoping for a good result. In this way, those who do well are spared a
second operation (Wehrs, Schuknecht, Austin, Gacek, Jansen, and Shea).
Middle Ear Stents
Exogenous and synthetic materials have been used to prevent adhesions within the middle ear after
resection of mucosa for disease. Rambo (1961) recognized this problem and began filling the middle ear
cleft with paraffin. In the mid-1960's, thin plastic sheets of polyethylene, Teflon and silicone rubber
(silastic) were used. Occasionally, this sheeting was pushed laterally by a bed of scar tissue and extruded.
This led to the use of thicker pieces of 0.04 inch silicone rubber or 0.3mm Supramid.
More recently, biodegradable substances such as Gelfilm and Gelfoam (purified gelatin matrix) have
been used. They do not require removal at a later time and will not extrude. The disadvantage comes
when the material is absorbed and no longer stents the middle ear. Some do not stent the middle ear
space at all, but rather avoid adhesions by leaving the perforation ungrafted until the second stage
procedure.
Donaldson and Snow (1992), in a prospective study of 71 patients found a better long-term hearing result
in patients who had mastoidectomies and silastic implants as a part of their first stage procedure. Some
suggest that these measures improve middle ear ventilation by increasing the "air-cushion" available to
resist eustachian reflux.
Surgical Indications and Contraindications
The indications for surgery are conductive hearing loss due to TM perforation or ossicular dysfunction;
chronic or recurrent otitis media and recurrent otitis media due to contamination through a perforated
tympanic membrane; progressive hearing loss due to chronic ME pathology; perforation or hearing loss
persistent for more than three months due to trauma, infection or surgery; and, the inability to bathe or
participate in water sports safely due to perforation of the TM. Normal hearing is not a contraindication
to surgery in the presence of disease, because secretions and granulations may close a perforation or
protect the round window thus improving a conductive deficit, or a cholesteatoma might conduct sound
as a functional portion of the ossicular chain.
Glasscock (1976) listed four absolute contraindications and seven relative contraindications to
tympanoplasty. Absolute contraindications are uncontrolled cholesteatoma, malignant tumors, unusual
infections, and complications of chronic ear disease such as meningitis, brain abscess or sinus
thrombosis. The relative contraindications include Eustachian tube insufficiency, uncooperative patients,
a dead ear, a better hearing or only hearing ear, elderly patients, young children and cases of repeated
failure.
He stated that the factors considered in the surgeon's decision to close the ear or leave it open constitute
the indications or contraindications for tympanoplasty. He stressed that tympanoplasty has been
controversial from the start because filling an infected cavity with foreign material and then closing it off
to the outside, flies in the face of classical surgical training.
Much has been made of the presence or absence of discharge in the ear at the time of surgery. Several
studies found that the secretion type (dry vs. mucoid or purulent) in the middle ear at the time of surgery
had no effect on the final success rate for myringoplasty. Poor eustachian function as evidenced by
contralateral effusion or atelectasis predicts poorer results (60% success).
Post-Operative Care
One goal of post-operative care is to keep the patient comfortable. Infection is generally prevented by
antibiotic-soaked canal and middle ear packing and host defenses. In order for the graft to heal it must
remain in contact with organic matrix or stroma, be free from infection, and not experience shearing
forces or excessive tension. Aural hygiene is aimed at achieving this environment. Maneuvers that
change the transtympanic pressure are forbidden, such as sneezing with the mouth shut, using a straw to
drink, valsalva or heavy lifting or nose blowing.

Complications
Possible complications of tympanoplasty include: failure of the graft to heal resulting in residual or
recurrent perforation, lateralization of a graft, canal stenosis, scarring or adhesions in the middle ear,
perilymph fistula and hearing loss, erosion or extrusion of a prosthesis, dislocation of a prosthesis, facial
nerve or chorda tympani injury.
Other problems may or may not be surgical complications, such as recurrence of cholesteatoma or
atelectasis of the middle ear space with retraction of the TM. The patient should be informed of these
preoperatively, and understand the importance of aural hygiene and follow-up care.
Pediatric Tympanoplasty
Timing of repair in the pediatric population is very controversial. Glasscock (1976) gave young age as a
relative contraindication to tympanoplasty because children under three or four are prone to upper
respiratory infections and otitis media. Koch et al (1990) reported an 81% success rate for children age 8
and older, but only a 30% success rate in younger patients. They concluded that tympanoplasty before
age 8 results in a high rate of failure because of poor Eustachian tube function and frequent URI's. Smyth
(1992) agreed, noting that patients less than 10 years old had a higher failure rate for myringoplasty than
older children. This was independent of secretion type, perforation site, and graft material.
Yet, others such as Lau and Tos (1986) found no significant difference in outcome between the 2 to 7 age
group and those children ages 8 to 14. They suggested that early operation may prevent progression of
ossicular chain resorption. Ophir et al (1987) reported a 79% overall success rate, and their success in
younger children (5-8) was comparable to the rate for older children. They concluded that myringoplasty
had a good chance of success at any age. Kessler et al (1994) reviewed the results of 209 myringoplasties
and concluded that even in young patients (2-6 years) myringoplasty has a high success rate (75-94%),
and that age alone could not be considered a contraindication to surgery.
Surgical Outcome
In his 1992 Toynbee Memorial Lecture, Gordon Smyth of Belfast stated that any hope for cost-effective
health care reform required urgent self-audit and the provision of more realistic advice to patients. He
took exception to the generally quoted success rate for myringoplasty of 90%. When the success rate is
corrected for the length of follow-up, using Survival Life-table Analysis, the success rate for maintaining
a healed TM diminished to 81% at 11 years. He also suggested that the success of ossiculoplasty should
be measured as the effect on binaural hearing, rather than the air-bone gap on the operated side. This is
because the operated ear must reach an air conduction level of 30 dB at speech frequencies or be within
15 dB of the other ear for the patient to benefit (Belfast rule of thumb) (Smyth and Peterson, 1985).
Smyth warned that, "Fiction and fact need untangling, otherwise surgeons are little better than gossips."
Summary
In summary, tympanoplasty without mastoidectomy is performed to eradicate middle ear disease and
reconstruct the conductive hearing mechanism. Priorities in tympanoplasty are to prevent recurrence, to
improve hearing and to minimize ear after care.
Though most would agree that myringoplasty has a high success rate and that staged operations optimize
the hearing result, controversy abounds in other areas. Specifically we need to standardize outcome
measures. To say that a tympanoplasty failed or succeeded in a given case is meaningless if our criteria
are different. 1) It seems reasonable to use post-operative sensorineural hearing level rather than the preoperative level to calculate closure of the "air-bone gap." 2) When reporting pure tone average, "air-bone
gaps," these results should be tabulated in increments of 5 dB. 3) We should agree to report effusions or
retractions as numbers without lumping them into surgical failures.
We need studies with longer follow-up times. This is difficult for three reasons. 1) The population is
highly mobile in this country. 2) Patients no longer choose which specialist they will see or when they
will see him. Attempts to control medical costs may arbitrarily limit follow-up for asymptomatic
individuals. 3) Patients who are doing well may not see the benefit of follow-up, and conversely, patients
with poor results may blame their surgeon and seek referral to a different otolaryngologist. The solution
to this problem lies in improved physician to physician communication. Pre-operative patient counseling
that stresses the importance of follow-up may help as well.
We need more studies that limit the variability of the population by focusing on a single type of
procedure or pathology. The solution to this problem lies in cooperative studies to increase the
population in each study.
Finally, we need improved means of measuring Eustachian tube function. It may be necessary to measure
small gradual pressure changes on an ambulatory basis, like esophageal pH monitoring, in order to gain
clinically relevant information.
Case Presentation
A 20-year-old man with a history of chronic otitis media as a child, requiring the placement of
tympanostomy tubes on three separate occasions, presented with a left myringotomy that failed to heal
after the last set of tympanostomy tubes. He developed a chronic perforation but rejected surgical repair
in favor of expectant management and meticulous aural hygiene. He has had no ear infections in the last
year. He has no complaints of decreased hearing, otorrhea, tinnitus, dizziness, or ear pain.
Recently, he returned for follow-up examination and requested a tympanoplasty so that he could swim,
bathe and exercise without concern for ear protection.

He has no family history of chronic middle ear disease. He has seasonal allergies, but otherwise has no
medical problems. He takes no medications and has no drug allergies.
On physical examination he has a 3cm x 3cm hemangioma of the soft palate on the right. Examination of
the left ear reveals a normal external auditory canal; the tympanic membrane has a 30% posterior
marginal perforation with ragged edges and an adjacent posterior-central monomer. The middle ear
mucosa appears normal. The long process of the incus and stapes appear normal.
He underwent left lateral graft tympanoplasty via a retroauricular approach. The middle ear exploration
revealed no pathology. A type I tympanoplasty was performed using a temporalis fascia and free canal
skin overlay technique.
Bibliography
Alford BR, McFarlane JR, Neely JG. Homograft replacement of the tympanic membrane. Laryngoscope 1976;86:199-208.
Austin DF. Ossicular reconstruction. Arch Otolaryngol 1971;94: 525-535.
Austin DF. Reporting results in tympanoplasty. Am J Otol 1985;6:85-88.
Bellucci RJ. Dual classification of tympanoplasty. Laryngoscope 1973;83:1754-1758.
Black B. A universal ossicular replacement prosthesis: Clinical trials of 152 cases. Otolaryngol Head Neck Surg
1991;104:210-218.
Bluestone CD, Cantekin EI, Douglas GS. Eustachian tube function related to the results of tympanoplasty in children.
Brackman DE, Sheehy JL, Luxford WM. TORPS and PORPS in tympanoplasty: a review of 1042 operations. Otolaryngol
Campbell EE. Homograft tympanoplasty: a long-term review of 477 ears. Am J Otol 1990;11:66-70.
Chalet NI. Tympanic membrane transplant. Harper Hosp Bull 1964;22:27-34.
Cohen AM, Schwaber MK, Anthony LS, Jerger JF. Eustachian tube function and tympanoplasty. Ann Otol Rhinol
Laryngol 1979;88:339-347.
Committee on conservation of hearing of the American Academy of Ophthalmology and Otolaryngology. Standard
classification for surgery of chronic ear disease. Arch Otolaryngol 1965;81:204-205.

Deguine C. Staging in cholesteatoma surgery. Ear Nose Throat J 1993;72:197-200.
Donaldson I, Snow DG. A five year follow up of incus transposition in relation to the first stage tympanoplasty technique.
J Laryngol Otol 1992;106:607-609.
Frootko NJ. Reconstruction of the ear. In: Booth JB, Kerr AG, eds. Scott-Brown's Otolaryngology, 5th ed, Vol. 3. The Ear.
London: Butterworth, 1987:238-263.
Glasscock ME 3d. Contraindications to tympanoplasty II: an exercise in clinical judgment. Laryngoscope 1976;86:70-76.
Glasscock ME 3d. Ossicular chain reconstruction. Laryngoscope 1976;86:211-1221.
Green JD, Shelton C, Brackmann DE. Iatrogenic facial nerve injury during otologic surgery. Laryngoscope 1994;104:922926.
Gyo K, Hinohira Y, Hirata Y, Yanagihara N. Incidence of attic retraction after staged intact canal wall tympanoplasty for
middle ear cholesteatoma. Auris Nasus Larynx 1992;19:75-82.
Halik JL, Smith GDL. Long term results of tympanic membrane repair. Otolaryngol Head Neck Surg 1988;98:162-169.
House WF, Patterson ME, Linthicum FH. Incus homografts in chronic ear surgery. Arch Otolaryngol 1966;84:148-153.
Igarashi M, Konishi S, Alford BR, Guilford FR. The pathology of tympanosclerosis. Laryngoscope 1970;80:233-243.
Kaddour HS. Myringoplasty under local anaesthesia: day case surgery. Clin Otolaryngol 1992;17:567-568.
Kessler A, Potsic WP, Marsh RR. Type 1 tympanoplasty in children. Arch Otolaryngol Head Neck Surg 1994;120:487490.
Koch WM, Friedman EM, McGill TJI, Healy GB. Tympanoplasty in children: The Boston Children's Hospital experience.
Lau T, Tos M. Tympanoplasty in children: an analysis of late results. Am J Otol 1986;7:55-59.
Levenson MJ, Michaels L, Parisier SC. Congenital cholesteatomas of the middle ear in children: origin and management.
Levinson RM. Cartilage-perichondrial composite graft tympanoplasty in the treatment of posterior marginal and attic
retraction pockets. Laryngoscope 1987;97:1069-1074.
Matt BH, Miller RP, Meyers RM, Campbell JM, Cotton RT. Incidence of perforation with Goode T-tube. Int J Pediatr
Ophir D, Porat M, Marshak G. Myringoplasty in the pediatric population. Arch Otolaryngol Head Neck Surg
1987;113:1288-1290.
Parisier SC, Weiss MH. Recidivism in congenial cholesteatoma surgery. Ear Nose Throat J 1991;70:362-364.
Poe DS, Gadre AK. Cartilage tympanoplasty for management of retraction pockets and cholesteatomas. Laryngoscope
1993;103:614-618.
Rambo JHT. Musculoplasty: a new operation for suppurative middle ear deafness. Trans Am Acad Ophthalmol
Otolaryngol 1958;62:166-177.
Rambo JHT. The use of paraffin to create a middle ear space in musculoplasty. Laryngoscope 1961;71:612-619.
Sheehy JL, Shelton C. Tympanoplasty: To stage or not to stage. Otolaryngol Head Neck Surg 1991;104:399-407.
Smyth GDL. Toynbee Memorial Lecture 1992: facts and fantasies in modern otology: the ear doctor's dilemma. J Laryngol
Otol 1992;106:591-596.
Silverstein H, McDaniel AB, Lichtenstein R. A comparison of TORP, PORP, and incus homograft for ossicular
reconstruction in chronic ear surgery. Laryngoscope 1986;96:159-165.
Stewart MG, Troendle-Atkins J, Starke JR, Coker NJ. Nontuberculous mycobacterial mastoiditis. Arch Otolaryngol Head
Neck Surg 1995;121:225-228.
Tos M, Lau T. Stability of tympanoplasty in children. Otolaryngol Clin North Am 1989;22:15-28.
Vartiainen E. Results of surgical treatment for chronic noncholesteatomatous otitis media in the pediatric population. Int J
Pediatr Otolaryngol 1992;24:209-216.
Vartiainen E. Findings in revision myringoplasty. Ear Nose Throat J 1993;72:201-204.
Wehrs RE. Homograft ossicles in middle ear surgery. Am J Otol 1985;6:33-34.
Wullstein H. Theory and practice of tympanoplasty. Laryngoscope 1956;66:1076-1095.


medical literature.
OSTEODYSTROPHIES OF THE MIDDLE EAR AND TEMPORAL BONE

February 9, 1995
Carla M. Giannoni, M.D.
Five major osteodystrophies that affect the middle ear and temporal bone are presented: Paget's Disease,
Osteogenesis Imperfecta, Osteopetrosis, Fibrous Dysplasia, and Osteitis Fibrosa Cystica. The
epidemiology, pathophysiology, clinical features, histopathology, diagnosis, treatment and prognosis of
these five disorders are discussed.
Paget's Disease
The classic description of a patient with Paget's disease is that of a deaf old man with a bent back, bowed
legs, curved tibia, a large skull and a wobbling gait. However, these advanced changes are not frequently
seen. Czerny (1873) was first to describe this disease, but it was Sir James Paget (1877) who correlated
and described the clinical and pathological features of this disease. Paget's disease is a chronic,
progressive disorder and is believed to be AD or X-linked. The disease primarily affects older people and
commonly presents in 6th decade. There is evidence for a viral etiology, possibly a slow virus. Diagnosis
is based on history, exam, radiographic and laboratory findings. Frequently, the alkaline phosphatase is
often grossly increased; the acid phosphatase is often elevated - prostate cancer should be ruled out. The
calcium is usually normal. Demineralization of bone is seen radiographically, such as the well-known
osteoporosis circumscripta and cotton-wool patches of the skull. The histology characteristically shows
osteoclastic bone resorption and a mosaic pattern of new, lamellar bone interspersed with marrow spaces
that become obliterated with fibrous tissue. Typically, pagetoid bone and microfractures are seen in the
temporal bone.
Symptoms are mainly due to the excess osteoid and resulting soft, easily deformed bones. Hearing loss
occurs in 5% - 44%: mixed, CHL or SNHL. CHL component is very rare due to ossicular fixation and
surgery is not recommended. Treatment consists of symptomatic relief. Salmon calcitonin can promote
healing of osteolytic lesions, and stabilize hearing. Prognosis is generally good; however, there is a 20fold risk for developing osteosarcoma in the pagetoid lesions and these malignancies are very aggressive
lesions and portend a poor prognosis.
Osteogenesis Imperfecta
Osteogenesis Imperfecta results from a defect in the biosynthesis of the alpha 1 or alpha 2 chains of type
I collagen. This results in bone fragility with multiple fractures, bone deformities, thin skin, and capillary
fragility with increased bleeding tendency. Histopathologically, those with the most severe form possess
thin, immature cortical bone with small, irregular spicules of bone, decreased osteoid matrix and
increased fibrous tissue and vascular spaces. A thin, fragile stapes is typical. Those with less severe
forms have hypercellular, nonlamellar (immature) bone which fails to mature; there are large remnants of
cartilage in the endochondral bone and delay in ossification of the endochondral layer. Radiographically
this disorder appears similar to osteoporosis with demineralization / deossification of bone and
sometimes with bowing of long bones, thinning of the cortices and exuberant calus formation at
fractures. In the skull, embryonic microfusion lines or "wormian bone" is seen. A "halo sign" of
demineralization of the otic capsule, similar to otosclerosis, may be found.
Previously in two groups (congenita and tarda), they are now grouped in four main categories: 1. blue
sclera and an associated CHL; 2. most severe form; 3. blue sclera and an associated CHL, more severe
than 1, and associated with skeletal deformities; and, 4. similar to 1, but associated with grey sclera and
normal hearing or SNHL. Severe SNHL occurs in 40% of cases and correlates with gray or normal
sclera. CHL reflects structural changes in the ossicles and usually correlates with patient with blue sclera.
The CHL may be due to soft or missing stapes crural arch, due to otosclerosis or due to a combination of
the two. Otosclerosis commonly occurs in association with OI and the histologic independence of these
two disorders has been clearly demonstrated. Otosclerosis in these patients tends to be more aggressive
and there is a higher incidence of SNHL and window obliteration. Medical treatment with calcium,
vitamin D, calcitonin, and anabolic steroids have not been shown to be helpful. Treatment is aimed at
function; bracing and orthopedic stabilization is provided as needed, and physical therapy to maintain
good range of motion and muscular tone (e.g. swimming) is encouraged. Stapedectomy may yield good
results for CHL.
Osteopetrosis
Osteopetrosis is due to defective osteoclast function with failure of normal bone resorption, while normal
osteoblastic bone formation continues with excess deposition of mineralized osteoid and cartilage and
displacement of marrow spaces. Radiographically, one sees increased density of all bones with loss of
marrow spaces and obliteration of mastoid air cells and paranasal sinuses. Later, the periosteal bone
becomes sclerotic without marrow spaces, resulting in dense, brittle bones and anemia. In infants, the
temporal bone is primarily dense, calcified cartilage; the stapes exists in fetal form; dehiscence of VII is a
consistent finding.
Patients with the "tarda" form may be asymptomatic or they may have syndactyly, easy fracturing of
bones, bone pain, osteomyelitis, and skull thickening, and, possibly, narrowing of foramina of optic,
trigeminal, facial, and auditory cranial nerves. CHL is due to encroachment of bone on the middle ear
space with entrapment or ankylosis of the ossicles. Those with the congenital form die at an early age
from anemia or sepsis (none live more than 20 years). Bone marrow transplantation from HLA-identical
sibs has been used successfully with reversal of anemia & osteosclerosis.
Fibrous Dysplasia
Fibrous dysplasia usually presents in the 2nd or 3rd decades and is a chronic, slowly progressive, but not
self-limiting disease of unknown cause and without a known genetic predisposition. It is pathologically
and radiologically similar to osteitis fibrosa cystic. Monostic, polyostic and syndromal (Mc-Cune Albright) types are described. Histopathology shows replacement of normal cancellous bone by a fibrous
stroma arranged in a whorled pattern with "C or Y shapes" of immature, woven bone with scattered
islands of cartilage. The marrow is replaced with fibrous tissue. There is active simultaneous osteoblastic
and osteoclastic activity. Radiographic studies show thickening +/- sclerosis of bones, especially of the
femur, tibia, ribs, jaw and skull; the paranasal sinuses may be obliterated. Variable amounts of the
irregularly arranged spicules of bone lead to the "ground glass" appearance on x-ray. The typically
unilateral distribution may resemble malignant growth and biopsy may be indicated for confirmation of
the diagnosis. Temporal bone involvement is unusual, but it can cause SNHL, vertigo & VII palsy due to
erosion. CHL due to EAC stenosis or cholesteatoma can also occur.
Major otologic findings include: unilateral CHL; swelling of mastoid or temporal regions; SNHL
(erosion otic capsule); stenosis of EAC; cholesteatoma due to stenosis; pain (associated with
cholesteatoma); vertigo (erosion otic capsule) or VII paralysis (erosion fallopian canal). Partial surgical
removal can be performed for functionally or cosmetically deforming masses. XRT is contraindicated in
the treatment of FD because a 0.4% transformation rate to osteo-, chondro-, or fibro-sarcomas (400 time
the de novo risk) has been observed.
Osteitis Fibrosa Cystica (von Recklinghausen's disease)

This disease is due to hyperparathyroidism and the resultant hypercalcemia and loss of bone calcium.
Elevated calcium (96% of patient) and elevated radioimmunoassay for iPTH (carboxyl end) provide the
diagnosis. The cause is usually a parathyroid adenoma (85-90% cases), occasionally associated with
MEN I syndrome. It may also be due to chief cell hyperplasia. (MEN I or Wermer's syndrome consists of
hyperparathyroidism, pancreatic islet cell tumors and pituitary tumors.) The hypercalcemia caused by
parathryoid hormone influenced release of calcium from bone causes a clinical symptom complex of
"Bones, Stones, Moans, Groans, and Hypertones." This refers to the complications of hypercalcimia that
are observed: skeletal deformities; muscle weakness and wasting; bone pain and fractures; anemia; renal
stones (nephrocalcinosis); abdominal pain, ulcer disease and pancreatitis; and impaired mentation,
depression, hyperreflexia, and rarely coma.
Histopathology shows tongues of fibrous tissue with leading osteoclasts; the resulting abnormal bone is
demineralized and made up of loosely arranged trabeculae of varying sizes interspersed with marrow
spaces containing fibrous tissue. Cystic degeneration of fibrous tissue may occur and lead to the
formation of brown tumors. Temporal bone shows similar changes and some cases of SNHL have been
observed. Radiographic studies show subperiosteal erosions ("pepper-pot skull"), chondrocalcinosis and
nephrocalcinosis. Neck exploration are generally curative with restoration of normal calcium balance.
Surgery should be entertained especially in those patients that exhibit one of the following five criteria:
Ca >11.0, x-ray evidence for metabolic bone disease, presence of a complication of hypercalcemia,
active nephrolithiasis, or decreasing renal function.
Case Presentation
A 72-year-old Hispanic man presented in April 1992 with right hip and back pain. A routine lumbarspine
series revealed marked degenerative changes of the lumbar spine with narrowing of the intervertebral
spaces and some increased bone densities of the right ilium and lumbar spine. The findings were
consistent with degenerative change of the spine or possibly Paget's disease. Further evaluation was
significant for an elevated alkaline phosphatase and normal levels of calcium, ionized calcium,
phosphorous, magnesium and PSA. A 24-hour urine calcium collection was significantly elevated (416
mg/24 hr). He was seen by the Endocrinology service and started on Indomethacin and Didronel with
partial pain relief; salmon calcitonin was then begun with good alleviation of his symptoms. With
treatment, his alkaline phosphatase levels decreased to the high normal range. He did well, and in May of
1993 a taper of his calcitonin was commenced. Unfortunately, in November 1993 he re-presented with a
compression fracture of L5. He was restarted on calcitonin with good response and subsequently retapered off calcitonin over six months.
The patient was referred to the Otolaryngology service in September 1994 with a complaint of hearing
loss of several years' duration. The loss was greater in the left ear than the right. He also noted mild
episodic dizziness over the previous two months. Routine audiometric evaluation revealed a right downsloping sensorineural hearing loss and a left mixed hearing loss with a significant conductive component.
Tympanograms were normal. Laboratory evaluation showed an alkaline phosphatase again elevated
(115) and he was restarted on calcitonin with relief of his pain and dizziness. The patient has been
referred for amplification and is currently doing well on a tapering dose of calcitonin.
Bibliography
Andreoli TE, ed. Cecil Essentials of Medicine, 3rd ed. Philadelphia: Saunders, 1993:1415-1468.
Armstrong BW. Stapes in patients with osteogenesis imperfecta. Ann Otol Rhinol Laryngol 1984;93:634-636.
Berger G, Hawke M, Johnson A, Proops D. Histopathology of the temporal bone in osteogenesis imperfecta congenita - a
report of 5 cases. Laryngoscope 1985;95:193-199.
Coccia PF, Krivit W, Cervenka J, Clawson C, Kersey JH, Kim TH. Successful bone-marrow transplantation for infantile
malignant osteopetrosis. N Engl J Med 1980;302:701-708.
Davies DG. Paget's disease of the temporal bone, a clinical and histopathological survey. Acta Otolaryngol Suppl
1968;242:1-47.
Edeiken J, Dalinka M, Karasick D. Edeiken's Roentgen Diagnosis of Diseases of the Bone, 4th ed, vol. 2. Baltimore:
Williams & Wilkins, 1990.
Harersma H. Total decompression of the facial nerve in osteopetrosis. ORL J Otorhinolaryngol Relat Spec 1973;36:21-25.
Hawke M, Jahn AF, Bailey D. Osteopetrosis of the temporal bone. Arch Otolaryngol 1981;107:278-282.
Khetarpal U, Schuknecht HF. In search of pathologic correlates of hearing loss and vertigo in Paget's disease, a clinical
and histopathologic study of 26 temporal bones. Ann Otol Rhinol Laryngol Suppl 1990;145:1-16.
Kritzinger EE, Wright BE. Color Atlas of the Eye and Systemic Disease. Chicago: Year Book Medical Publishers, 1984.
Lindsay JR, Suga F. Sensorineural deafness due to osteitis fibrosa. Arch Otolaryngol 1976;102:37-42.
Marcover RC, Arlen M. Atlas of Bone Pathology with Clinical and Radiographic Correlations. Philadelphia: Lippincott,
1992.
Mills BG, Singer FR. Nuclear incisions in Paget's disease of bone. Science 1976;194:201-202.
Milroy CM, Michaels L. Temporal bone pathology of adult type osteopetrosis. Arch Otolaryngol Head Neck Surg
1990;116:79-84.
Mirra JM. Pathogenesis of Paget's disease based on viral etiology. Clin Orthop 1987;217:162-170.
Myers EN, Stool S. The temporal bone in osteopetrosis. Arch Otolaryngol 1969;89:460-469.
Nadol JB Jr, Merchant SN. Systemic disease manifestations in the middle ear and temporal bone. In: Cummings, CW,
Harker LA, editors. Otolaryngology - Head and Neck Surgery, 2nd ed, vol 4. St Louis: Mosby, 1993:2906-2925.
Nager GT. Osteogenesis imperfecta of the temporal bone and its relation to otosclerosis. Ann Otol Rhinol Laryngol
1988;97:585-593.
Nager GT, Holliday MJ. Fibrous dysplasia of the temporal bone: update with case reports. Ann Otol Rhinol Laryngol
1983;93:630-633.
Parons V. Color Atlas of Bone Disease. Chicago: Year Book Medical Publishers, 1980.
Patterson CN, Stone HB 3d. Stapedectomy in van der Hoeve's syndrome. Laryngoscope 1970;80:544-558.
Sando I, Myers D, Harada T, Hinojosa R, Myers E. Osteogenesis imperfecta tarda and otosclerosis: a temporal bone
histopathology report. Ann Otol Rhinol Laryngol 1981;90:199-203.
Schuknecht H. Pathology of the Ear, 2nd ed. Philadelphia: Lea & Febiger, 1993:365-414.
Schwartz DT, Alpert M. The malignant transformation of fibrous dysplasia. Am J Med Sci 1964;247:1-.
Spranger JW, Langer LO, Wiedemann H-R. Bone Dysplasias: An Atlas of Constitutional Disorders of Skeletal
Development. Philadelphia: Saunders, 1974.
Suga F, Lindsay JR. Temporal bone histopathology of osteopetrosis. Ann Otol Rhinol Laryngol 1976;85:15-29.


medical literature.
OTOTOXICITY
Andrew T. Lyos, MD
August 20, 1992
Ototoxicity is defined as the tendency of certain therapeutic agents to cause functional impairment and
cellular degeneration of the inner ear and of the eighth cranial nerve. Ototoxicity may be reversible or
irreversible. Ototoxicity is differentiated from neurotoxicity where the site of action is central to the
eighth cranial nerve.
The aminoglycosides are a class of broad-spectrum antibiotics capable of producing profound,
irreversible sensorineural hearing loss (SNHL). Morphologic studies demonstrate that the inner row of
outer hair cells (OHCs) of the basal turn of the cochlea are affected first, followed by the other two rows
of OHCs and the inner hair cells. Prospective studies demonstrate an incidence ranging from 4% to 24%.
Clinically, patients frequently develop symptoms following the cessation of therapy. SNHL is primarily
high frequency and may be unilateral. Risk factors for aminoglycoside ototoxicity include: therapy
lasting more than seven days, elevated serum levels, prior exposure to aminoglycosides, noise exposure,
high daily dose, and use in neonates.
Loop diuretics, including furosemide and ethacrynic acid, are capable of producing SNHL which is
frequently reversible. The site of action is thought to be the stria vascularis, with inhibition of potassium
chloride co-transport. Risk factors include renal insufficiency, use in neonates, intravenous
administration, and co-administration with aminoglycosides.
Cis-platinum is a relatively new cytoreductive agent used to treat a variety of malignancies. The
mechanism of ototoxic action is uncertain; however, it produces a loss of OHCs in the basal turn of the
cochlea in a fashion similar to the aminoglycosides. Morphological changes have not been noted in the
vestibular organs. Clinical studies demonstrate an irreversible ototoxicity incidence ranging from 9% to
91%, depending on the criteria used and the cumulative dose. Symptoms are frequently present at the
onset of measurable hearing loss. Risk factors for ototoxicity include renal insufficiency, intravenous
bolus, co-administration with aminoglycosides, and increased cumulative doses. An audiogram should be
obtained at the onset of therapy, before each successive dose, and with the onset of symptoms.
Salycilates have been used for more than 100 years and have long been known to produce reversible
hearing loss associated with tinnitus. The mechanism of action is thought to involve vasoconstriction of
capillaries in the stria vascularis. Symptoms typically resolve within 24 hours following cessation of
therapy; however, cases of irreversible SNHL and tinnitus have been reported.
Following a single dose in sensitive individuals or, more typically, after a prolonged course, the
antimalarial agent quinine can produce the symptom complex of hearing loss, tinnitus, vertigo, nausea,
and vomiting (cinchonism). Symptoms resolve following cessation of therapy. However, several cases of
irreversible SNHL and tinnitus have been reported.
Monitoring for ototoxicity should be individualized to the drug and to the patient. The early symptoms
seen with some drugs, such as the salycilates and quinine, obviate the need for formal monitoring. The
profound, irreversible effects seen with drugs such as the aminoglycosides and cis-platinum require much
more formal monitoring.
Case Presentation
A 64-year-old man was initially referred to the Otolaryngology service for evaluation prior to initiation
of aminoglycoside therapy. He had no prior otologic history, including surgery, noise exposure, or
ototoxic drugs. Past medical history was significant for a right total knee replacement seven months prior
to consultation. His postoperative course was complicated by wound dehiscence and osteomyelitis. He
underwent removal of the hardware and debridement. Cultures grew Serratia marcescens. The Infectious
Disease service was consulted and a six-week course of gentamycin was initiated.
A baseline audiogram was obtained which demonstrated a mild sensitivity loss on the right with normal
sensitivity on the left to 1 kHz, at which point he had a severe sensorineural loss. An audiogram
performed eight days later demonstrated no change in sensitivity; however, the PB performance dropped
from 95% to 76% on the right, and from 70% to 28% on the left. The patient denied symptoms of hearing
loss, tinnitus, or vertigo at that time. However, he noticed progressive difficulty understanding speech
during the next several days. On physical examination, the tympanic membranes were clear and there
was no evidence of nystagmus.
The dose of gentamycin was decreased and serum levels were monitored closely. He completed five
weeks of therapy prior to discontinuation due to elevation of his blood urea nitrogen and creatinine.
Serial audiograms performed weekly during the course of therapy, as well as those performed during the
following five years, have failed to demonstrate any changes in sensitivity or speech understanding.
Bibliography
Bernard PA. Freedom from ototoxicity in aminoglycoside treated neonates: a mistaken notion. Laryngoscope
1981;91:1985-1994.
Bernstein J, Weiss A. Further observations on salicylate oto-toxicity. J Laryngol Otol 2967;82:915-927.
Brown RD, Henley CM, Penny JE, Rupetz S. Link between functional and morphological changes in the inner ear functional changes produced by ototoxic agents and their interaction. Arch Toxicol Suppl 1985;8:240-250.
Fausti SA, Rappaport BZ, Schechter MA, Frey RH, Wart TT, Brummett RE. Detection of aminoglycoside ototoxicity by
high-frequency auditory evaluation: selected case studies. Am J Otolaryngol 1984;5:177-182.
Fee WE Jr. Aminoglycoside ototoxicity in the human. Laryngoscope 1980;90(Suppl 24):1-19.
Fowler EP Jr, Seligman E. Otic complications of streptomycin therapy. JAMA 1947;133:87-91.
Gallagher KI, Jones JK. Furosemide-induced ototoxicity. Ann Int Med 1979;91:744-745.
Goycoolea MV, Paparella MM, Goldberg B, Carpenter AM. Permeability of the round window membranae in otitis media.
Arch Otolaryngol 1964;206:430-433.
Hart CW, Naunton RG. Ototoxicity of chloroquine phosphate. Arch Otolaryngol 1964;80:407-412.
Hawkins JR Jr. Drug ototoxicity. In: Keidel WD, Neff WD, editors. Handbook of sensory physiology. Volume V, Auditory
system. Berlin: Springer-Verlag, 1976:707-748.
Hawkins JR Jr, Lurie MH. The ototoxicity of streptomycin. Ann Otol 1952;61:789-806.
Henley CM. Postnatal developmental changes in inner ear ornithine decarboxylase (ODC). Hear Res 1991;55:45-49.
Henley CM, Schacht J. Pharmacokinetics of aminoglycoside antibiotics in blood, inner ear fluids and tissues and their
relationship to ototoxicity. Audiology 1988;27:136-146.
Henley CM, Rybak LP. Ototoxicity in developing mammals. (Manuscript in preparation)
Hinshaw HC, Feldman WH. Streptomycin in the treatment of clinical tuberculosis: a preliminary report. Proc Mayo Clin
1945;20:313-318.
Jackson GG, Arcieri G. Ototoxicity of gentamicin in man: a survey and controlled analysis of clinical experience in the
United States. J Inf Dis 1971;124(Suppl):130-137.
Johnsson LG, Hawkins JE Jr, Kingsley TC, Black FO, Matz GJ. Aminoglycoside-induced cochlear pathology in man. Acta
Otolaryngol Suppl 1981;383:1-19.
Kapur YP. Ototoxicity of acetylsalicylic acid. Arch Otolaryngol 1965;81:134-138.
Karmody CA, Weinstein L. Reversible sensorineural hearing loss with intravenous erythromycin lactobionate. Ann Otol
Koegel L Jr. Ototoxicity: a contemporary review of aminoglycosides, loop diuretics, quinine, erythromycin, and cisplatinum. Am J Otol 1985;6:190-199.
Komune S, Asakuma S, Snow JB. Pathophysiology of the ototoxicity of cis-diamminedichloroplatinum. Otolaryngol Head
Neck Surg 1981;89:275-282.
Lenoir M, Marot M, Uziel A. Comparative ototoxicity of four aminoglycoside antibiotics during the critical period of
cochlear development in the rat. Acta Otolaryngol Suppl 1983;405:1-16.
Lerner SA, Matz GJ. Aminoglycoside ototoxicity. Am J Otolaryngol 1980;1:169-179.
Lerner SA, Reyes MP, Cone LA et al. Randomized, controlled trial of the comparative efficacy, auditory toxicity, and
nephrotoxicity of tobramycin and netilmicin. Lancet 1983;1:1123-1126.
Lerner SA, Matz GH, Hawkins JE Jr. Aminoglycoside ototoxicity. Boston: Little, Brown, 1981.
Lerner SA, Seligshon R, Matz GH. Comparative clinical studies of ototoxicity and nephrotoxicity of amikacin and
gentamicin. Am J Med 1977;62:919-923.
Lim DJ. Effects of noise and ototoxic drugs at the cellular level in the cochlea: a review. Am J Otolaryngol 1986;7:117119.
Marot M, Uziel A, Romand R. Ototoxicity of kanamycin in developing rats: relationship with the onset of auditory
function. Hear Res 1990;2:111-113.
Meyerhoff WL, Morizono T, Wright CG, Shaddock LC, Shea DA, Sikora MA. Tympanostomy tubes and otic drops.
Laryngoscope 1983;93:1022-1027.
New HC, Bendush CL. Ototoxicity of tobramycin: a clinical overview. J Inf Dis 1976;134(Suppl):5206-5218.
Paloheimo JA, Thalmann R. Influence of "loop" diuretics upon Na-K-ATPase and adenylate cyclase of the stria vascularis.
Arch Otorhinolaryngol 1977;217:347-359.
Pujol R. Periods of sensitivity to antibiotic treatment. Acta Otolaryngol 1986;249:29-33.
Reddel RR, Kefford RF, Grant JM, Coates AS, Fox RM, Tattersall MH. Ototoxicity in patients receiving cis-platin:
importance of dose and method of drug administration. Cancer Treat Rep 1982;66:19-23.
Rybak LP. Cis-platinum associated hearing loss. J Laryngol Otol 1981;95:745-747.
Rybak LP. Drug ototoxicity. Ann Rev Pharmacol Toxicol 1986;26:79-99.
Rybak LP. Pathophysiology of furosemide ototoxicity. J Otolaryngol 1982;11:127-133.
Rybak LP, Whitworth C, Scott V, Weberg A. Ototoxicity of furosemide drug development. Laryngoscope 1991;101:11671174.
Schacht J. Molecular mechanisms of drug-induced hearing loss. Hear Res 1986;22:297-304.
Silverstein J, Bernstein J, Davies DG. Salicylate ototoxicity: a biochemical and electrophysiological study. Ann Otol
Smith BM, Myers MG. The penetration of gentamicin and neomycin into perilymph across the round window membrane.
Head Neck Surg 1979;87:888-891.
Strauss M, Towfighi J, Lord S, Lipton A, Brown HA. Cis-platinum ototoxicity: clinical experience and temporal bone
histopathology. Laryngoscope 1983;93:1554-1559.
Stupp H, Kupper K, Lagler F et al. Inner ear concentration and ototoxicity of different antibiotics in local and systemic
application. Audiology 1973;12:350-363.
Tange RA, Dreschler WA, van der Hulst RJAM. The importance of high-tone audiometry in monitoring for ototoxicity.
Arch Otorhinolaryngol 1985;242:77-81.
Toone EC Jr, Hayden GD, Ellmann HM. Ototoxicity of chloroquine. Arthritis Rheum 1965;8:475-476.
Weatherly RA, Owens JJ, Catlin FI, Mahoney DH. Cis-platinum ototoxicity in children. Laryngoscope 1991;101:917-924.
Wright CG, Meyerhoff WL. Ototoxicity of otic drops applied to the middle ear in the chinchilla. Am J Otolaryngol
1984;5:166-176.
Wright DB, Schaefer SD. Inner ear histopathology in patients treated with cis-platinum. Laryngoscope 1982;92:14081413.


OTOTOXICITY
April 25, 1996
John S. Oghalai, M.D.
Aminoglycoside ototoxicity is associated with the total daily dose, duration of
treatment, previous treatment with aminoglycosides, and renal disease. The
ototoxicity has a delayed onset (months to years later). Aminoglycosides enter the
fluids of the inner ear via the bloodstream or via the round window with topical
application. The drug is cleared more slowly from perilymph and endolymph than
from serum, hence the once-daily dosing theory to reduce ototoxicity. A review of
29 clinical trials (Barclay et al, 1994) found that if the same total daily dose of drug
is given with a once-daily regimen as opposed to the traditional 2-4 doses/day, the
efficacy of antibiotic activity was the same, but there tended to be less ototoxicity.
Symptoms of aminoglycoside ototoxicity include irreversible high-frequency

sensorineural hearing loss, decreased otoacoustic emissions, and disequilibrium.
With further toxicity, this can lead to complete deafness and/or oscillopsia. Studies
of the organ of corti reveal widespread loss of outer hair cells. Potential
mechanisms of toxicity include:
1. Aminoglycosides binding to the cell membrane and blocking
transmembrane signaling systems.
2. Inhibition of the enzyme, ornithine decarboxylase, leading to decreased
polyamine synthesis (Henley, 1994).
3. An unknown drug metabolite may be responsible for the ototoxicity.
(Schacht, 1993).
Briefly stated, topical antimicrobials (particularly Cortisporin) are severely ototoxic
in guinea pigs (Barlow et al, 1994). However, there is no evidence for toxicity of
topical agents in humans. There has never been a controlled study that
demonstrates hearing loss, and there has never even been a case report of hearing
loss after treatment with a topical antimicrobial.
Salicylates and NSAID's cause reversible tinnitus and hearing loss. Also decreased
otoacoustic emissions have been noted. Theories of salicylate ototoxicity include:
1. Vasoconstriction of vessels in stria vascularis, leading to decreased cochlear
blood flow (Didier et al, 1993).
2. Reversible ultrastructural changes in outer hair cells that correlate with
decreased electromotility (Dieler et al, 1991).
In summary, aminoglycosides cause permanent structural damage to hair cells, and
lead to permanent hearing loss and disequilibrium. Salicylates cause reversible
changes in hair cells or the cochlea, and lead to reversible symptoms.
Case Presentation
A 41-year-old female presented with complaints of high-pitched ringing in her ears
for one week. She stated the sound was worse in quiet environments, and was
particularly noticeable when trying to fall asleep. She had no complaints of hearing
loss, aural fullness, unsteadiness, or vertiginous symptoms. She had no history of
ear infections or ear surgery. Family history was negative. There was no history of
autoimmune disease, skin rashes, or arthritis. The patient denied use of diuretics,
intravenous antibiotics, or chemotherapy medications. She did state that she had
been taking 3 aspirin tablets 3 to 4 times per day for stress headaches recently.
Physical examination, including complete neuro-otologic exam, was unremarkable.
A preliminary diagnosis of salicylate ototoxicity was made, and discontinuing the
use of aspirin was recommended to see if her tinnitus resolved. In addition, a
neurology appointment was made for evaluation of her headaches. After one week
the patient returned for follow-up and stated that her tinnitus, as well as her
headache, had resolved. She had no further complaints and was discharged from
clinic.
Bibliography
Aran JM. Current perspectives on inner ear toxicity. Otolaryngol Head Neck Surg 1995;112:133144.
Aran JM, Hiel H, Hayashida T, et al. Noise, aminoglycosides, and diuretics. In: Dancer A,
Henderson D, Salvi RJ, Hamemik RP, eds. Noise Induced Hearing Loss. Philadelphia: B.C.
Decker, 1991:188-195.
Barclay ML, Begg EJ. Aminoglycoside toxicity and relation to dose regimen. Adverse Drug
Reactions & Toxicological Reviews 1994;13:207-234.
Barclay ML, Begg EJ, Hickling KG. What is the evidence for once-daily aminoglycoside
therapy? Clin Pharmacokinetics 1994;27:32-48.
Barlow DW, Duckert LG, Kreig CS, Gates GA. Ototoxicity of topical otomicrobial agents. Acta
Otolaryngol 1994;115:231-235.
Barlow DW, Duckert LG, Kreig CS, Gates GA. Ototoxicity of topical otomicrobial agents. Acta
Oto-Laryngologica 1995;115:231-235.
Brown AM, Williams DM, Gaskill SA. The effect of aspirin on cochlear mechanical tuning. J
Acoust Soc Am 1993;93:3298-3307.
Brownlee RE, Hulka GF, Prazma J, Pillsbury HC, 3d. Ciprofloxacin. Use as a topical otic
preparation. Arch Otolaryngol Head Neck Surg 1992;118:392-396.
Didier A, Miller JM, Nuttall AL. The vascular component of sodium salicylate ototoxicity in the
guinea pig. Hear Res 1993;69:199-206.
Dieler R, Shehata-Dieler WE, Brownell WE. Concomitant salicylate-induced alterations of outer
hair cell subsurface cisternae and electromotility. J Neurocytol 1991;20:637-653.
Dieler R, Shehata-Dieler WE, Richter CP, Klinke R. Effects of endolymphatic and perilymphatic
application of salicylate in the pigeon. II: Fine structure of auditory hair cells. Hear Res
1994;74:85-98.
Duckert LG, Rubel EW. Morphological correlates of functional recovery in the chicken inner ear
after gentamycin treatment. J Comp Neurol 1993;331:75-96.
Ernst A, Reuter G, Zimmermann U, Zenner HP. Acute gentamicin ototoxicity in cochlear outer
hair cells of the guinea pig. Brain Res 1994;636:153-156.
Fitzgerald JJ, Robertson D, Johnstone BM. Effects of intra-cochlear perfusion of salicylates on
cochlear microphonic and other auditory responses in the guinea pig. Hear Res 1993;67:147-156.
Forge A, Li L, Corwin JT, Nevill G. Ultrastructural evidence for hair cell regeneration in the
mammalian inner ear. Science 1993;259:1616-1619.
Garetz SL, Altschuler RA, Schacht J. Attenuation of gentamicin ototoxicity by glutathione in the
guinea pig in vivo. Hear Res 1994;77:81-87.
Garetz SL, Rhee DJ, Schacht J. Sulfhydryl compounds and antioxidants inhibit cytotoxicity to
outer hair cells of a gentamicin metabolite in vitro. Hear Res 1994;77:75-80.
Hawkins JE. Drug Ototoxicity. In: Anonymous Handbook of Sensory Physiology, 1st ed. Berlin:
Springer-Verlag, 1976:707-739.
Henley CH. Kanamycin depletes cochlear polyamines in the developing rat. Otolaryngol Head
Neck Surg 1994;110:103-109.
Henley CM, Rybak LP. Developmental ototoxicity. Otolaryngologic Clin North Am 1993;26:857871.
Henley CM, Rybak LP. Ototoxicity in developing mammals. Brain Res 1995;20:68-90.
Hiel H, Erre JP, Aurousseau C, Bouali R, Dulon D, Aran JM. Gentamicin uptake by cochlear hair
cells precedes hearing impairment during chronic treatment. Audiology 1993;32:78-87.
Hotz MA, Harris FP, Probst R. Otoacoustic emissions: an approach for monitoring
aminoglycoside-induced ototoxicity. Laryngoscope 1994;104:1130-1134.
Hutchin T, Cortopassi G. Proposed molecular and cellular mechanism for aminoglycoside
ototoxicity. Antimicrobial Agents & Chemotherapy 1994;38:2517-2520.
Hyde GE, Rubel EW. Mitochondrial role in hair cell survival after injury. Otolaryngol Head
Neck Surg 1995;113:530-540.
Jung TT, Miller SK, Rozehnal S, Woo HY, Park YM, Baer W. Effect of round window
membrane application of salicylate and indomethacin on hearing and levels of arachidonic acid
metabolites in perilymph. Acta Otolaryngol Suppl 1992;493:81-87.
Jung TT, Rhee CK, Lee CS, Park YS, Choi DC. Ototoxicity of salicylate, nonsteroidal
antiinflammatory drugs, and quinine. Otolaryngologic Clin North Am 1993;26:791-810.
Matz GJ. Aminoglycoside cochlear ototoxicity. Otolaryngol Clin North Am 1993;26:705-712.
Meiteles LZ, Raphael Y. Scar formation in the vestibular sensory epithelium after
aminoglycoside toxicity. Hear Res 1994;79:26-38.
Mukhopadhyay S, Baer S, Blanshard J, Coleman M, Carswell F. Assessment of potential
ototoxicity following high-dose nebulized tobramycin in patients with cystic fibrosis. J
Antimicrob Chemother 1993;31:429-436.
Pollice PA, Brownell WE. Characterization of the outer hair cell's lateral wall membranes. Hear
Res 1993;70:187-196.
Schacht J. Biochemical basis of aminoglycoside ototoxicity. Otolaryngol Clin North Am
1993;26:845-856.
Schuknecht HF. Pathology of the Ear,2nd ed. Philadelphia: Lea & Febiger, 1993.
Shehata-Dieler WE, Richter CP, Dieler R, Klinke R. Effects of endolymphatic and perilymphatic
application of salicylate in the pigeon. I: Single fiber activity and cochlear potentials. Hear Res
1994;74:77-84.
Spongr VP, Boettcher FA, Saunders SS, Salvi RJ. Effects of noise and salicylate on hair cell loss
in the chinchilla cochlea. Arch Otolaryngol Head Neck Surg 1992;118:157-164.
Tunstall MJ, Gale JE, Ashmore JF. Action of salicylate on membrane capacitance of outer hair
cells from the guinea-pig cochlea. J Physiol 1995;485:739-752.
Warchol ME, Lambert PR, Goldstein BJ, Forge A, Corwin JT. Regenerative proliferation in inner
ear sensory epithelia from adult guinea pigs and humans. Science 1993;259:1619-1622.
Weisleder P, Rubel EW. Hair cell regeneration after streptomycin toxicity in the avian vestibular
epithelium. J Comp Neurol 1993;331:97-110.
Wersall J. Ototoxic antibiotics: a review. Acta Otolaryngol Suppl 1995;519:26-29.


OTITIS EXTERNA
October 12, 1995
John S. Oghalai, MD
Acute Otitis Externa is an infection of the skin of the cartilaginous portion of the
ear canal. Contributing factors include moisture, canal occlusion, local trauma, and
allergic disease. These factors lead to a loss of the protective wax layer, causing
edema of the squamous epithelium with plugging of the glandular secretory ducts.
Consequent scratching induces local trauma, allowing bacteria to invade through
the skin, leading to inflammation and production of exudate.
The pathogens in acute otitis externa are pseudomonas (41%), peptostreptococcus
(22%), Staph. aureus (15%), and Bacteroides (11%). The differential diagnosis
includes malignant external otitis, chronic external otitis, furunculosis, otomycosis,
herpes zoster oticus, bullous external otitis, granular external otitis, chondritis,
cellulitis, and eczematoid dermatitis.
Malignant external otitis is a complication of acute otitis externa. The disease starts
as a local infection of the external auditory canal and spreads through the fissures
of Santorini (in cartilage of ear canal) towards the parotid gland and mastoid. This
can lead to facial nerve paralysis. As the disease progresses, there is spread along
the skull base to the jugular foramen (paralysis of CN IX, X, and XI), and finally to
the hypoglossal canal (paralysis of CN XII).
Symptoms are similar to acute otitis externa, however, the main difference is that
the disease is not responsive to several weeks of conventional local therapy. On
physical exam, there may be granulation tissue present in the ear canal, cranial
nerve dysfunction, or a palpable bony defect in the anterior wall of the ear canal.
Risk factors include diabetes, elderly, and immunocompromised status. The
pathogen in malignant external otitis is nearly always Pseudomonas (99.2%).
Relevant laboratory studies include an erythrocyte sedimentation rate (ESR) and a
fasting glucose level. A bone scan is important to demonstrate osteomyelitis. It will
remain positive for years after a full recovery, so it is not useful in following
response to therapy. A gallium scan will demonstrate active inflammation in either
soft tissue or bone, and it will return to normal if the disease is effectively treated.
A CT scan is useful to determine bony erosion and soft tissue involvement,
however its sensitivity for detecting osteomyelitis is low (30%).
Therapy for malignant external otitis includes meticulous control of blood glucose,
local debridement of granulation tissue, and possibly hyperbaric oxygen (Davis et
al., 1992). Traditional antibiotic therapy is 6-8 weeks of IV drugs (usually an antipseudomonal penicillin or cephalosporin, and an aminoglycoside). Recent studies
have demonstrated that oral ciprofloxacin has been 96.4% successful in treating
mild to moderate malignant external otitis (Gehanno, 1994). In severe disease, IV
therapy is recommended initially, and then a long course of oral ciprofloxacin is
needed. Therapy is continued until the gallium scan is clear.
Case Presentation
A 59-year-old female was referred from the General Medicine Clinic for a 1 month
history of bilateral ear pain and otorrhea. She had been treated with Cortisporin otic
drops without benefit for several weeks. Her past history was unremarkable. She
had no history of diabetes or immunosuppressed states. Physical exam revealed the
left ear canal to be erythematous and tender. There was scant mucoid discharge and
no granulations. The right ear canal was also erythematous, swollen, and tender.
There was abundant mucoid discharge, and granulation tissue was present at the
cartilaginous/bony junction posteriorly. Both tympanic membranes were normal.
The rest of the exam was unremarkable. Laboratory studies revealed a slightly
elevated WBC of 12.7, a greatly elevated ESR of 92, a normal random blood
glucose of 100, and a slightly elevated glycosylated Hgb of 7.4.
The patient was admitted to the hospital with the diagnosis of malignant external
otitis. Her ears were debrided and cultures were sent, which eventually grew
Pseudomonas aeruginosa. A CT scan done on admission showed soft tissue
thickening around the ear canals, but was otherwise normal. A technetium bone
scan was negative, and a gallium scan was positive for bilateral temporal
inflammation. The patient was placed on ceftazidime and gentamicin otic drops.
Her ear canals were regularly debrided. Twice daily blood glucose checks during
hospitalization were all normal. After 10 days, her ear canals had no granulation
tissue or discharge, and she was discharged to home on ciprofloxacin. In follow-up,
she has been seen in at 2 weeks and 4 weeks post-discharge and has a normal
exam. She is still taking ciprofloxacin.
Bibliography
Agius AM, Reid AP, Hamilton C. Patient compliance with short-term topical aural antibiotic
therapy. Clin Otolaryngol 1994;19:138-141.
Barrow HN, Levenson MJ. Necrotizing 'malignant' external otitis caused by Staphylococcus
epidermidis. Arch Otolaryngol Head Neck Surg 1992;118:94-96.
Benecke JE Jr. Management of osteomyelitis of the skull base. Laryngoscope 1989;99:12201223.
Brody T, Pensak ML. The fluoroquinolones. Am J Otol 1991;12:477-479.
Brook I, Coolbaugh JC. Changes in the bacterial flora of the external ear canal from the wearing
of occlusive equipment. Laryngoscope 1984;94:963-965.
Brook I, Frazier EH, Thompson DH. Aerobic and anaerobic microbiology of external otitis. Clin
Infect Dis 1992;15:955-958.
Chandler JR. Malignant external otitis and osteomyelitis of the base of the skull. Am J Otol.
1909;10:108-110.
Cohen D, Friedman P. The diagnostic criteria of malignant external otitis. J Laryngol Otol
1987;101:216-221.
Cohen D, Friedman P, Eilon A. Malignant external otitis versus acute external otitis. J Laryngol
Otol 1987;101:211-215.
Cummings CW, Fredrickson JM, Harker LA, Krause CJ, Schuller DE. Otolaryngology - Head
and Neck Surgery. St Louis: Mosby, 1993.
Davis JC, Gates GA, Lerner C, Davis MG Jr, Mader JT, Dinesman A. Adjuvant hyperbaric
oxygen in malignant external otitis. Arch Otolaryngol Head Neck Surg 1992;118:89-93.
Dekker PJ. Alternative method of application of topical preparations in otitis externa. J Laryngol
Otol 191;105:842-843.
Dingle AF. Fistula between the external auditory canal and the temporomandibular joint: a rare
complication of otitis externa. J Laryngol Otol 1992;106:994-995.
Driscoll PV, Ramachandrula A, Drezner DA, Hicks TA, Schaffer SR. Characteristics of cerumen
in diabetic patients: a key to understanding malignant external otitis? Otolaryngol Head Neck
Surg 1993;109:676-679.
el-Silimy O, Sharnuby M. Malignant external otitis: management policy. J Laryngol Otol
1992;106:5-6.
Farrior J. Osteomyelitis of the skull base. South Med J 1989;82:719-722.
Gehanno P. Ciprofloxacin in the treatment of malignant external otitis. Chemotherapy
1994;40(Suppl 1):35-40.
Gherini SG, Brackmann DE, Bradley WG. Magnetic resonance imaging and computerized
tomography in malignant external otitis. Laryngoscope 1986;96:542-548.
Giamarellou H. Malignant otitis externa: the therapeutic evolution of a lethal infection J
Antimicrob Chemother 1992;30:745-751.
Gordon G, Giddings NA. Invasive otitis externa due to Aspergillus species: case report and
review. Clin Infect Dis 1994;19:866-870.
Guy RL, Wylie E, Hickey A, Tonge KA. Computed tomography in malignant external otitis. Clin
Radiol 1991;43:166-170.
Hickey SA, Ford GR, O'Connor AF, Eykyn SJ. Sonksen PH. Treating malignant otitis with oral
ciprofloxacin. BMJ 1989;299:550-551.
Hirsch BE. Infections of the external ear. Am J Otolaryngol 1992;13:154-155.

Hunsaker DH. Conchomeatoplasty for chronic otitis externa. Arch Otolaryngol Head Neck Surg
1988;114:395-398.
Koriwchak M. Temporal bone cancer. Am J Otol 1993;14:623-626.
Kraus DH, Rehm SJ, Kinney SE. The evolving treatment of necrotizing external otitis.
Levenson MJ, Parisier SC, Dolitsky J, Bindra G. Ciprofloxacin: drug of choice in the treatment
of malignant external otitis (MEO). Laryngoscope 1991;101:821-824.
Lucente FE. Fungal infections of the external ear. Otolaryngol Clin North Am 1993;26:995-1006.
Lucente FE, Parisier SC, Som PM. Complications of the treatment of malignant external otitis
Martin-Hirsch DP, Smelt GJ. Conchal flap meatoplasty. J Laryngol Otol 1993;107:1029-1031.
Murray ME, Britton J. Osteomyelitis of the skull base: the role of high resolution CT in
diagnosis. Clin Radiol 1994;49:408-411.
Ostfeld EJ, Kupferberg A. Biocompatible implantable antimicrobial release for necrotizing
external otitis. J Laryngol Otol 1991;105:252-256.
Raza SA, Denholm SW, Wong JC. An audit of the management of acute otitis externa in an ENT
casualty clinic. J Laryngol Otol 1995;109:130-133.
Rubin J, Curtin HD, Yu VL, Kamerer DB. Malignant external otitis: utility of CT in diagnosis
and follow-up. Radiology 1990;174:391-394.
Rubin J, Yu VL. Malignant external otitis: insights into pathogenesis, clinical manifestations,
diagnosis, and therapy. Am J Med 1988;85:391-398.
Rubin J, Yu VL, Kamerer DB, Wagener M. Aural irrigation with water: a potential pathogenic
mechanism for inducing malignant external otitis? Ann Otol Rhinol Laryngol 1990;99:117-119.
Russell JD, Donnelly M, McShane DP, Alun-Jones T, Walsh M. What causes acute otitis
externa? J Laryngol Otol 1993;107:898-901.
Senturia BH, Marcus MD, Lucente FE. Diseases of the External Ear. New York: Grune and
Stratton, 1980.
Shpitzer T, Stern Y, Cohen O, Levy R, Segal K, Feinmesser R. Malignant external otitis in
nondiabetic patients. Ann Otol Rhinol Laryngol 1993;102:870-872.

Strashun AM, Nejatheim M, Goldsmith SJ. Malignant external otitis: early scintigraphic
detection Radiology 1984;150:541-545.
Strauss M. Current therapy of malignant external otitis. Otolaryngol Head Neck Surg
1990;102:174-176.
Swartz JD, Harnsberger HR. Imaging of the Temporal Bone. New York: Thieme Medical, 1992.
Uri N, Gips S, Front A, Meyer SW, Hardoff R. Quantitative bone and 67Ga scintigraphy in the
differentiation of necrotizing external otitis from severe external otitis. Arch Otolaryngol Head
Neck Surg 1991;117:623-626.
Weinroth SE, Schessel D, Tuazon CU. Malignant otitis externa in AIDS patients: case report and
review of the literature. Ear Nose Throat J 1994;73:772-778.
Wilde AD, England J, Jones AS. An alternative to regular dressings for otitis externa and chronic
supperative otitis media? J Laryngol Otol 1995;109:101-103.
Wormald PJ. Surgical management of benign necrotizing otitis externa. J Laryngol Otol
1994;108:101-105.


medical literature.
PATHOLOGY AND PATHOGENESIS OF OTITIS MEDIA

April 21, 1994
Philip A. Matorin, M.D.
Pathology
Understanding the pathology and pathogenesis of otitis media (OM) is important in predicting the
prognosis and sequelae of the disease. In this way treatment can be tailored to individual needs.
There have been several attempts to gain a consensus definition of OM. In 1980, the Ad Hoc Committee
on the Classification of Otitis Media defined OM based on the temporal sequence: Acute < 3 weeks;
Subacute 4-8 weeks; Chronic >9 weeks. In 1989, the Task Force of the 4th International Symposium on
Otitis Media published a working classification. Acute Suppurative OM refers to an identifiable infection
of the middle ear of short duration and sudden onset. Secretory OM refers to the presence of a middle ear
effusion without acute signs or symptoms. Chronic Suppurative OM refers to a chronic middle ear
discharge through a perforated tympanic membrane.
Much of our understanding of the pathology of OM comes from experimental animal models and from
temporal bone preparations. After Eustachian tube obstruction in the cat and chinchilla, edema
hyperemia, subepithelial hemorrhage with PMN infiltration are noted, modeling acute Suppurative OM.
In addition mucoperiosteal thickening and osteitis can be seen. Mild metaplasia from squamous to
cuboidal epithelium is noted.
Continued Eustachian tube obstruction models secretory OM. Early transudation of serum into the
subepithelial space is seen within 18-24 hours. More metaplasia is seen with increased numbers of
pseudostratified, ciliated columnar epithelial cells, and goblet cells. Resorption of water is thought to
promote thickening of the effusion converting thin serous fluid into a thick mucoid effusion. In temporal
bone preparations, thickening and fibrosis of the tensor tympanic is seen. This is felt to be important in
the development of spasm and contracture of the muscle resulting in retraction and fixation of the
tympanic membrane.
In chronic OM models, columnar metaplasia is greatest. fibroblastic density is increased. A lymphoid
infiltrate is noted with polypoid changes present in the mucosa. Osteitis and osteoneogenesis are also
commonly noted. Friable immature granulation tissue with neovascularization and fibroblastic
proliferation is seen. Mature granulation with subepithelial fibrosis and decreased vascularity are found
in long standing disease. The sequelae of COM including tympanosclerosis, cholesterol granuloma, and
cholesteatoma can also be encountered.
Paparella's group has proposed that OM exists as a continuum of mucoperiosteal disease. Epidemiologic
evidence comes from studies showing the overlap and progression of disease from acute suppurative OM
to secretory OM or chronic OM. One out of 5 cases of Acute OM is superimposed on a chronic mucoid
OM. Temporal bone histology also demonstrates the continuum of mucosal disease. Lastly, experimental
animal models of Eustachian tube obstruction followed longitudinally have demonstrated the gradual
change from suppurative, to secretary, to chronic histopathology.
Pathogenesis
Since the time of Politzer, the Eustachian tube has been identified as central to the pathogenesis of OM.
For OM to occur, three conditions must be met. Bacteria must adhere to the nasopharyngeal mucosa. The
bacteria must the enter the Eustachian tube and finally, the bacteria must be able to multiply in the
middle ear environment.
Pathogenic bacteria bind to cell surface receptors in a highly specific fashion. The adhesin receptor
binding mechanism prevents colonizing bacteria from being swept away by the mechanical cleansing
action in the nasopharynx. Adhesion through a receptor mechanism is suggested by the fact that
adherence can be saturated by an increase in bacterial concentration. In general, the more adherent an
organism is to the nasopharyngeal mucosa, the more likely it will be a successful pathogen. In children,
the adenoid bed is an important source of pathogenic bacteria. In addition, Influenza A can cause
destruction of the normal tubal mucosa promoting bacterial entry. One study showed a 5-fold increase in
the rate of pneumococcal OM in chinchillas infected with the virus.
The Eustachian tube itself is cartilaginous in its lower 2/3 portion. It is normally in the closed position.
The main dialator of the tube is the tensor veli palatini innervated by the mandibular division of the
trigeminal nerve. Comparing the infant to the adult tube, the Eustachian tube is shorter (13mm vs 3138mm), shallower (10 deg. vs 45 deg from horizontal), and has abundant goblet cells. These anatomic
features make the pediatric Eustachian tube much more likely to become dysfunctional.
Eustachian tube function can be divided into ventilation, drainage, and protection from nasopharyngeal
secretions. Bluestone has described 4 functional abnormalities radiographically by instilling contrast
material into the nasopharynx. The abnormalities include retrograde obstruction, abnormal distensibility,
middle ear reflux, and retrograde obstruction with abnormal distensibility. The patulous Eustachian tube
predisposes to middle ear reflux. The abnormally compliant Eustachian tube could result in middle ear
reflux with slight increases in nasopharyngeal pressure. On the other hand, rapid changes in pressure
could cause locking of the tube and functional obstruction.
The ventilatory function of the Eustachian tube is also affected by the gaseous equilibrium of the middle
ear space. Large rapid changes can occur because the middle ear space is a relatively small pocket of gas
surrounded by vascular mucosa. Gas is exchanged between the middle ear cavity and the mucosa. Recent
evidence suggests that the diffusion gradient is due to the nitrogen partial pressure gradient.
Anatomically, neoplasms, cleft palate, and large adenoids can cause obstruction of the Eustachian tube.
In children, it appears that adenoid size does not correlate with the incidence of OM. Adenoids act as a
source of pathogenic bacteria rather than anatomic obstruction in the pathogenesis of OM.
The final stage in the development of OM is bacterial replication in the middle ear space. Therefore, the
immunology of OM has generated great interest. In human studies, IgG is the predominant
immunoglobulin involved. Several clinical series have shown lower levels of IgG2 in otitis prone
children. These children have greater susceptibility to non-typeable H.Flu. Higher levels of interleukin-1
have been found in both younger children and those with cleft palate with Eustachian tube dysfunction
and OM. Increased levels of specific hydrolase activity and higher levels of lipoxygenase products have
also been found in otitis media effusions.
Case Presentation
An 11-month-old full term white male child presented with a history of otitis media for the past 5
months. He completed six courses of several different antibiotics without resolution. Physical exam
showed dull, slightly retracted tympanic membranes and no other abnormalities. Visual reinforcement
audiometry showed normal sensitivity for age. Acoustic immittance measurements showed type C
tympanograms. In view of the persistence of the effusion, the patient underwent bilateral myringotomy
and placement of pressure equalization tubes. Thin viscous fluid was aspirated from both middle ear
cavities. One month postoperative assessment revealed that the child was doing fine with well-placed,
patent PE tubes.
Bibliography
Beachey EH, Courtney SH. Bacterial adherence: attachment of group A streptococci to mucosal surfaces. In: Lim DJ, ed.
Proceedings of the Fourth International Symposium Recent Advances in Otitis Media, June 1-4, 1987, Bal Harbour,
Florida. Toronto: B.C. Decker, 1988:291-296.
Bernstein JM. Immunologic reactivity in the middle ear in otitis media with effusion. Otolaryngol Clin North Am
1991;24:845-858.
Biegink GS, Ripley-Petzoldt ML, Juhn SK, Aeppli D, Tomasz A, Tuomanen E. Contribution of pneumococcal cell wall to
experimental otitis media pathogenesis. Ann Otol Rhinol Laryngol 1988;97:28-30.
Bluestone CD, Paradise JL, Beery QC. Physiology of the eustachian tube in the pathogenesis and management of middle
ear effusion. Laryngoscope 1972;82:1654-1670.
Bluestone CD, Rood SR, Swarts JD. Anatomy and physiology of the eustachian tube. In: Cummings, DW, Harker LA, eds.
Otolaryngology -Head and Neck Surgery, Volume 4, 2nd ed. St. Louis: Mosby, 1993:2548-2565.
Cantekin EI, Casselbrant ML, Doyle WJ, Brostoff LM. Eustachian tube function: prospective study of eustachian tube
function and otitis media. In: Lim DJ, ed. Proceedings of the Fourth International Symposium Recent Advances in Otitis
Media, June 1-4, 1987, Bal Harbour, Florida. Toronto: B.C. Decker, 1988:58-60.
Collins ME, Coker NJ, Igarashi M. Inflammatory disease of the anterior epitympanum. Am J Otol 1991;12:11-15.
Diven WF, Doyle WJ, Vietmeier B. Hydrolytic enzymes in otitis media pathogenesis. Ann Otol Rhinol Laryngol
1988;97:6-9.
Diven WF, Doyle WJ, Vietmeier BN. Studies in otitis media, Pittsburgh Otitis Media Research Center. Progress report
1988: Hydrolase activity in experimental middle ear effusion. Ann Otol Rhinol Laryngol Suppl 1988;133:26-27.
Diven WF, LaMarco KL, Doyle WJ, Glew RH. Studies in otitis media, Pittsburgh Otitis Media Research Center. Progress
report 1988: Role of bacterial hydrolases in the pathologic changes caused by otitis media. Ann Otol Rhinol Laryngol
Suppl 1988;133:23-24.
Diven WF, Scanlon KL, Glew RH. Studies in otitis media, Pittsburgh Otitis Media Research Center. Progress report 1988:
Purification and properties of a neuraminidase from streptococcus pneumoniae culture filtrates. Ann Otol Rhinol Laryngol
Suppl 1988;133:24-25.
Diven WF, Vietmeier BN. Studies in otitis media, Pittsburgh Otitis Media Research Center. Progress report 1988: Isolation
and characterization of chinchilla a1-antitrypsin. Ann Otol Rhinol Laryngol Suppl 1988;133:24.
Gates GA, Muntz HR, Gaylis B. Adenoidectomy and otitis media. Ann Otol Rhinol Laryngol Suppl 1992;155:24-32.
Giebink GS. Otitis media update: pathogenesis and treatment. Ann Otol Rhinol Laryngol 1992;101:21-23.
Goycoolea MV, Buah DB, Bequer N. General surgical approach based on pathogenesis: an overall approach. Otolaryngol
Clin North Am 1991;24:957-966.
Goycoolea MV, Hueb MM, Ruah C. Definitions and terminology. Otolaryngol Clin North Am 1991;24:757-761.
Goycoolea MV, Nuchow DC, Goycoolea HG. Otitis media: 16 years of pathogenesis approach. Otolaryngol Clin North
Am 1991;24:967-980.
Hellstrm S, Goldie P. Mechanisms of otitis medial development: involvement of neurogenic inflammation. Otolaryngol
Clin North Am 1991;24:829-834.
Johnson MD, Fitzgerald JE, Leonard G, Burleson JA, Dreutzer DL. Cytokines in experimental otitis media with effusion.
Laryngoscope 1994:104:191-196.
Juhn SK, Tolan CJ, Antonelli PJ, Giebink GS, Goycoolea MV. The significance of experimental animal studies in otitis
media. Otolaryngol Clin North Am 1991;24:813-827.
Jung TT. Arachidonic acid metabolites in otitis media pathogenesis. Ann Otol Rhinol Laryngol 1988;97:14-17.
Jung TT, Park YM, Schlund D, Weeks D, Miller S, Wong O, et al. Effect of prostaglandin, leukotriene,m and arachidonic
acid on experimental otitis media with effusion in chinchillas. Ann Otol Rhinol Laryngol 1990;99:28-32.
Kenna MA. Etiology and pathogenesis of chronic suppurative otitis media. Ann Otol Rhinol Laryngol Suppl 1988;131:1624.
Klein JO, Naunton RF, Tos M, Ohyama M, Hussl B, van Cauwenberge PB. Recent advances in otitis media: definition and
classification. Ann Otol Rhinol Laryngol Suppl 1989;139:10.
Lim DJ, Demaria TF, Bakaletz LO. Current concepts of pathogenesis of otitis media: a review. Acta Otolaryngol Suppl
1988;458:174-180.
Meyerhoff WL, Biegink GS. Pathology and microbiology of otitis media. Laryngoscope 1982;92:273-277.
Meyerhoff WL, Shea DA, Giebink GS. Experimental pneumococcal otitis media: a histopathologic study. Otolaryngol
Head Neck Surg 1980;88:606-612.
Morizono T, Tono T. Middle ear inflammatory mediators and cochlear function. Otolaryngol Clin North Am 1991;24:824852.
Paparella MM, Schachern PA, Yoon TH, Abdelhammid MM, Sahni R, da Costa SS. Otopathologic correlates of the
continuum of otitis media. Ann Otol Rhinol Laryngol 1990;99:17-22.
Senturia BH, Paparella MM, Lowery HN, Klein JO, Arnold WJ, Lim DJ, et al. Report of Research Conference. Recent
Advances in Otitis Media with Effusion: Modified report of the ad hoc committee on definition and classification of otitis
media. Ann Otol Rhinol Laryngol Suppl 1980;69:6.
Sando I, Takahashi H. Otitis media in association with various congenital diseases: preliminary study. Ann Otol Rhinol
Laryngol 1990;99:13-16.
Schachern P, Paparella MM, Sano S, Lamey S, Guo Y. A histopathological study of the relationship between otitis media
and mastoiditis. Laryngoscope 1991;101:1050-1055.
Schuknecht H. Pathology of the Ear, 2nd ed. Philadelphia: Lea and Febiger, 1993.
Weir N. Otolaryngology: An Illustrated History. London: Butterworths, 1990: 14, 18, 42, 81, 206, 211-212, 218.
Yoon TH, Paparella MM, Schachern PA, Lindgren BR. Morphometric studies of the continuum of otitis media. Ann Otol
Yoon TH, Schachern PA, Paparella MM, Aeppli DM. Pathology and pathogenesis of tympanic membrane retraction. Am J
Otol 1990;11:10-17.


medical literature.
THE DIAGNOSIS AND MANAGEMENT OF PERILYMPHATIC

FISTULAS
Troy A. Callender, MD
October 21, 1993
A perilymphatic fistula is an abnormal communication between the inner ear fluids and the middle ear.
Perilymphatic fistulas can be classified into four broad categories: iatrogenic, traumatic, spontaneous, and
congenital. Symptoms of perilymphatic fistula are variable and include sensorineural hearing loss,
vertigo, dysequilibrium, unsteadiness, motion intolerance, tinnitus and aural fullness. Simmons in 1968
first postulated that sensorineural hearing loss could result from intracochlear membrane breaks. In 1971
Goodhill stated that the labyrinth is hydrostatically loaded with intimate relations to hydrodynamic forces
in the carotid arterial system, intracranial venous-sinus systems and the CSF pressure gradients in the
subarachnoid space. He proposed that the membranous labyrinth could be ruptured by an explosive route
via the external auditory canal and eustachian tube and concluded that membrane ruptures could occur
throughout the membranous labyrinth with both acoustic and/or vestibular system sequelae. Simmons
later expounded on his original theory and proposed the "double membrane break theory" in the
development of sensorineural hearing loss. In this theory he postulated that the second membrane break
was the result of a pressure gradient created by the first break, profound sensorineural hearing loss
resulted from the mixing of perilymph and endolymph, healing of intracochlear breaks halts the mixing of
fluid and the widespread hearing loss disappears resulting in hearing loss at the specific site where local
tissue damage occurred along the membrane.
The diagnosis of perilymphatic fistula is very difficult to make since there is no diagnostic test for this
disorder. The diagnosis depends upon a high degree of clinical suspicion and ruling out other possible
causes for the symptoms. In addition to the hearing loss, vestibular dysfunction, tinnitus and aural
fullness, patients may also have recruitment, the Tullio phenomenon and a positive fistula test. Evaluation
of these patients should include a careful history and physical examination, audiogram,
electronystagmography, fistula test, imaging to rule out an intracranial tumor and exclusion of other
possible causes for the symptoms (i.e., autoimmune diseases, vascular diseases, infectious diseases and
endolymphatic hydrops).
The treatment of suspected perilymphatic fistulas is controversial as are most aspects of this disorder and
consists of conservative medical therapy or surgical exploration and closure of the fistula. Medical
therapy consists of strict bed rest, head elevation, and refraining from all strenuous activity. Some authors
advocate this treatment for all cases of suspected perilymphatic fistula if the patient presents early in the
course of the disease, whereas others use it to treat patients with symptoms consistent with a
perilymphatic fistula but no antecedent history of trauma or surgery. After 5 to 7 days, the patients are reevaluated and if symptoms persist, surgical exploration is undertaken. Surgical treatment consists of a
middle ear exploration and careful examination of the oval and round window niches, looking for crystal
clear fluid that persistently wells up after careful suctioning. Special maneuvers such as trendelenburg,
internal jugular vein compression and valsalva may be required to adequately visualize a fistula. In
addition to fistulas of the oval and round windows, fistulas can also develop from microfissures around
the oval and round windows as well as from Hyrtl's fissure and the fissula ante fenestram. Most authors
agree that whether a fistula is found or not, a tissue seal with fat, fascia, perichondrium or loose areolar
tissue should be made around both the oval and round windows since there is a possibility that the fistula
is only intermittently patent or is too small to be visualized. Some authors feel that surgery as an initial
form of therapy is indicated in all patients with symptoms for greater than one month, whereas others
would proceed only if there is a clear antecedent history of trauma or surgery associated with the typical
symptoms of perilymphatic fistula. There are several large studies reporting the results of surgical
treatment of perilymphatic fistulas. The percentage of patients with fistulas found surgically ranges from
24% to 93%. Vestibular symptoms had the best response to surgical treatment, with 60% to 90% of cases
improving. Hearing loss responded much less favorably except in fluctuating or progressive sensorineural
hearing loss in which case surgery would often stabilize or slightly improve the hearing. Postoperative
management consists of bed rest, head elevation and no straining for the first 5 days, followed by 4 to 6
weeks of light, non-strenuous activity. The objective assessment of treatment results awaits the
development of an objective test for diagnosing perilymphatic fistulas.
Case Presentation
A 59-year-old white man presented to the VAMC Otolaryngology Clinic 4 days after he developed
sudden right hearing loss. The hearing loss developed shortly after he sneezed and was associated with a
headache. He denied vestibular symptoms, otalgia, fever or a history of otologic surgery. His past medical
history was unremarkable except for left hearing loss of probable sudden onset in 1976 for which he was
extensively evaluated while in the Navy. Physical examination was unremarkable and significant
laboratory findings included a ESR of 3 mm/hr, rheumatoid factor < 30, negative ANA, nonreactive RPR
and mildly elevated liver function tests. Audiogram obtained on admission revealed a profound SNHL on
the left with no word discrimination and moderate to severe mixed hearing loss on the right with 35%
word discrimination. Tympanograms were type A.
The patient was admitted to the VAMC hospital for bed rest and treated with carbogen, intravenous
steroids and diuretics. MRI scan of the brain, posturography, and fistula test were all negative. Because
the patient's history was suggestive for a perilymphatic fistula, exploratory tympanotomy was performed
on the second hospital day. At surgery, clear fluid was noted to collect around the oval window niche
consistent with a perilymphatic fistula. Both windows were sealed with loose areolar tissue and the
patient recovered unremarkably. A repeat audiogram obtained 2 months after surgery revealed a 10 dB
improvement in the PTA and a 10% improvement in speech discrimination score on the right side.
Bibliography
Allen GW. Fluid flow in the cochlear aqueduct and cochlea - hydrodynamic considerations in perilymph fistula, stapes
gusher, and secondary endolymphatic hydrops. Am J Otol 1987;8:319-322.
Althaus SR. Perilymph fistulas. Laryngoscope 1981;91:538-562.
Althaus SR, House HP. Long-term results of perilymph fistula repair. Laryngoscope 1973;83:1502-1509.
Althaus SR. Spontaneous and traumatic perilymph fistulas. Laryngoscope 1977;87:364-371.
Bassiouny B, Hirsch BE, Kelly RH, Kamerer DB, Cass SP. Beta2 transferrin application in otology. Am J Otol
1992;13:552-555.
Black FO, Lilly DJ, Nashner LM, Peterka RJ, Pesznecker SL. Quantitative diagnostic test for perilymph fistulas.
Black FO, Pesznecker S, Norton T, Fowler L, Lilly DJ, Shupert C, Hemenway WG, et al. Surgical management of
perilymphatic fistulas: a Portland experience. Am J Otol 1992;13:254-262.

Bhmer A. On the pathomechanism of cochlear dysfunction in experimental perilymph fistulas. Laryngoscope
1991;191:1307-1312.
Bojrab DI, Shansali SA. Fluorescein use in the detection of perilymphatic fistula: a study in cats. Otolaryngol Head Neck
Surg 1993;103:348-355.
Campbell KC, Savage MM. Electrocochleographic recordings in acute and healed peilymphatic fistula. Arch Otolaryngol
Head Neck Surg 1992;118:301-304.
Daspit CP, Churchill D, Linthicum FH Jr. Diagnosis of perilymph fistula using ENG and impedance. Laryngoscope
1980;90:217-223.
Davis RE. Diagnosis and management of perilymph fistula: the University of North Carolina approach. Am J Otol
1992;13:85-89.
Fee GA. Traumatic perilymphatic fistulas. Arch Otolaryngol 1968;88:477-480.
Flint P, Duckert LG, Dobie RA, Rubel EW. Chronic perilymphatic fistula: experimental model in the guinea pig.
Otolaryngol Head Neck Surg 1988;99:380-388,
Flood LM, Fraser JG, Hazell WP, Rothera MP. Perilymph fistula: four year experience with a new audiometric test. J
Laryngol Otol 1985;99:671-676.
Glasscock ME III, McKennan KX, Levine SC. Persistent traumatic perilymph fistulas. Laryngoscope 1987;97:860-864.
Glasscock ME III, Hart MJ, Rosdeutscher JD, Bhansali SA. Traumatic perilymphatic fistula: how long can symptoms
presist? A follow-up report. Am J Otol 1992;13:333-338.
Glasscock ME III. The stapes gusher. Arch Otolaryngol 1973;98:82-91.
Goodhill V. Leaking labyrinth lesions, deafness, tinnitus and dizziness. Ann Otol Rhinol Layrngol 1981;90:99-106.
Goodhill V. Sudden deafness and round window rupture. Laryngoscope 1971;81:1462-1474.
Goodhill V. The conductive loss phenomenon in post-stapedectomy perilymphatic fistulas. Laryngoscope 1967;77:11791190.
Goodhill V. Traumatic fistulae. J Laryngol Otol 1980;94:123-128.
Grundfast KM, Bluestone CD. Sudden or fluctuating hearing loss and vertigo in children due to perilymph fistula. Ann Otol
Harris I. Sudden hearing loss: membrane rupture. Am J Otol 1984;6:484-487.
Harrison WH, Shambaugh GE, Derlacki EL, Clemis JD. Perilymph fistula in stapes surgery. Laryngoscope 1967;77:836849.
Hazell JW, Fraser JG, Robinson PJ. Positional audiometry in the diagnosis of perilymphatic fistula. Am J Otol 1992;13:263269.
House HP. The fistula problem in otosclerosis surgery. Laryngoscope 1967;77:1410-1426.
Hughes GB, Sismanis A. Is there consensus in perilymph fistula management? Otolaryngol Head Neck Surg 1990;102:111117.
Jackler RK, Hwang PH. Enlargement of the cochlear aqueduct: fact or fiction? Otolaryngol Head Neck Surg 1993;109:1425.
Kamerer DB, Sando I, Hirsch B, Takagi A. Perilymph fistula resulting from microfissures. Am J Otol 1987;8:489-494.
Kaseki Y, Nakashima T, Fukuta S, Suzuki T, Yanagit N. Effects of persistent perilymph fistula on the inner ear.
Laryngoscope 1991;101:1085-1090.
Kohut RI, Hinojosa R, Budetti JA. Perilymphatic fistula: a histopathologic study. Ann Otol Rhinol Laryngol 1986;95:466471.
Kohut RI. Perilymph fistulas: clinical criteria. Arch Otolaryngol Head Neck Surg 1992;118:687-692.
Kohut RI. Peilymphatic fistulae: more than a century of notions, conjectures, and critical studies. Am J Otol 1982;13:38-40.
Kohut RI, Waldorf RA, Haenel JL, Thompson JN. Minute perilymph fistulas: vertigo and Hennebert's sign without hearing
loss. Ann Otol Rhinol Laryngol 1979;88:153-159.
Lehrer JF, Poole DC, Sigal B. Use of the glycerin test in the diagnosis of post-traumatic perilymphatic fistulas. Am J
Luntz M, Frank I, Yurovitzki I, Berger G, Sad J. Large perilymph fistulas. Am J Otol 1986;7:282-286.
Meyerhoff WL, Pollock KJ. A patient-oriented approach to perilymph fistula. Arch Otolaryngol Head Neck Surg
1990;116:1317-1319.
Meyerhoff WL, Yellin MW. Summating potential/action potential ratio in perilymph fistula. Otolaryngol Head Neck Surg
1990;102:678-682.
Narula A, Marks NJ. Acoustic trauma and perilymph fistula. J Laryngol Otol 1985;99:909-911.
Nomura Y, Hara M, Young Y-H, Okuno T. Inner ear morphology of experimental perilymphatic fistula. Am J Otol
1992;13:32-37.
Parell GJ, Becker GD. Results of surgical repair of inapparent perilymph fistulas. Otolaryngol Head Neck Surg
1986;95:344-346.
Petroff MA, Simmons FB, Winzelberg J. Two emerging perilymph fistula "syndromes" in children. Laryngoscope
1986;96:498-501.
Pillsbury HC III, Postma DS. The Tullio phenomenon, fistula test, and Hennebert's sign: clinical significance. Otolaryngol
Clin North Am 1983;16:205-207.
Poe DW, Gadre AK, Rebeiz EE, Pankratov MM. Intravenous fluorescein for detection of perilymphatic fistulas. Am J Otol
1993;14:51-54.
Poe DS, Rebeiz EE, Pankratov MM. Evaluation of perilymphatic fistulas by middle ear endoscopy. Am J Otol 1992;13:529533.
Pullen FW II. Perilymphatic fistula induced by barotrauma. Am J Otol 1992;13:270-272.
Pyykk I, Ishizaki H, Aalto H, Starck J. Relevance of the Tullio phenomenon in assessing perilymphatic leak in vertiginous
patients. Am J Otol 1992;13:339-342.
Rizer FM, House JW. Perilymph fistulas: the House Ear Clinic experience. Otolaryngol Head Neck Surg 1991;104:239243.
Schuknecht HF, Witt RL. Suppressed sneezing as a cause of hearing loss and vertigo. Am J Otolaryngol 1985;6:468-470.
Seltzer S, McCabe BF. Perilymph fistula: the Iowa experience. Laryngoscope 1986;94:37-49.
Shea JJ. The myth of spontaneous perilymph fistula. Otolaryngol Head Neck Surg 1992;107:613-616.
Shelton C, Simmons FB. Perilymph fistula: the Stanford experience. Ann Otol Rhinol Laryngol 1988;97:105-108.
Shepard NT, Telian SA, Niparko JK, Kemink JL, Fujita S. Platform pressure test in identification of perilymphatic fistula.
Am J Otol 1992;13:49-54.
Silverstein H. Rapid protein test for perilymph fistula. Otolaryngol Head Neck Surg 1991;105:422-426.
Simmons FB. Theory of membrane breaks in sudden hearing loss. Arch Otolaryngol 1968;88:67-74.
Simmons FB. Fluid dynamics in sudden sensorineural hearing loss. Otolaryngol Clin North Am 1978;11:55-61.
Singleton GT. Diagnosis and treatment of perilymph fistulas without hearing loss. Otolaryngol Head Neck Surg
1986;94:426-429.
Singleton GT, Post KN, Karlan MS, Bock DG. Perilymph fistulas: diagnostic criteria and therapy. Ann Otol Rhinol
Laryngol 1978;87:797-803.
Stroud MH, Calcaterra TC. Spontaneous perilymph fistulas. Laryngoscope 1970;80:479-487.

Supance JS, Bluestone CD. Perilymph fistulas in infants and children. Otolaryngol Head Neck Surg 1983;91:663-671.
Thompson JN, Kohut RI. Perilymph fistulae: variability of symptoms and results of surgery. Otolaryngol Head Neck Surg
1979;87:898-903.
Weider DJ. Treatment and management of perilymphatic fistula: a New Hampshire experience. Am J Otol 1992;13:158166.


medical literature.
LESIONS OF THE PETROUS APEX

August 4, 1994
Philip A. Matorin, M.D.
The petrous apex is the most inaccessible portion of the temporal bone. The petrous apex can be involved
in congenital, infectious, inflammatory, and neoplastic processes. Since direct examination is not
possible, careful attention to the often subtle signs and symptoms, as well as keeping a high index of
suspicion, is necessary to diagnose these entities. The true incidence of these rare lesions is difficult to
estimate, but the House group has reported that acoustic neuromas are about 30 times more common in
their practice.
The petrous apex is a truncated pyramid forming the medial portion of the temporal bone. The base is
bounded by the bony labyrinth and the carotid artery anteriorly. The superior surface is the middle cranial
fossa, Meckel's Cave, and the ascending carotid artery. The posterior surface is the posterior cranial fossa
and Dorello's canal transmitting CNVI. The inferior surface is the jugular bulb and the inferior petrosal
sinus. The petrous apex is marrow filled in 84%, pneumatized in 9%, and sclerotic in 7%.
Clinical presentations can range from subtle to dramatic. Involvement of the Eustachian tube can result in
aural fullness or otitis media. Compression at the foramen ovale can cause V3 paresthesia or numbness.
Stretching of the dura can cause headache of eye pain. In addition, ophthalmoplegia, tinnitus, vertigo,
hearing loss, and facial paralysis can occur.
CT and MRI have revolutionized the preoperative work-up of petrous apex lesions. An accurate
differential diagnosis is critical in that it may influence the surgical approach for specific lesions. The two
modalities may provide complimentary information based on density, bone erosion, contrast
enhancement, and imaging characteristics with T1 and T2 weighting.
Several benign anatomic variants may produce radiographic abnormalities that can mimic pathologic
conditions. Thirty to thirty-five percent of petrous apices are pneumatized and 6.8% may have
asymmetric pneumatization that can be demonstrated by CT. On MRI, the less pneumatized, marrowfilled apex would appear bright on T1 and T2, mimicking a lesion. A giant petrous air cell can cause
distortion of the IAC appearing similar to an expansile lesion. Lastly, simple effusions, mucoceles, and
mucus retention cysts of the petrous apex appear as soft tissue density with preservation of the air cell
septae. Expansile mucoceles may have eroded margins. On MRI, these appear low on T1 and bright on
T2.
Cholesterol granuloma is the most common lesion of the petrous apex. It is approximately 10 times more
common than cholesteatoma and 40 times more common than mucocele. The cyst wall lacks the
keratinizing squamous epithelium of cholesteatoma. On CT, the lesion appears as a homogeneous soft
tissue ovoid lesion posterior to the horizontal carotid canal. On MRI, it appears bright on T1 and T2
without enhancement with gadolinium. The surgical treatment is re-establishment of aeration of the apex
by using a silicone drain into the middle ear or mastoid.
Cholesteatoma of the petrous apex can be congenital or acquired. Acquired cholesteatomas are rare and
occur by extension from the middle ear space along the supralabyrinthian air cell system along the
anterior epitympanic space. Facial nerve dysfunction occurs in 20% - 50% of cases. On CT,
cholesteatoma appears as a smooth, expansile lesion hypodense to brain without contrast enhancement.
On MRI, it appears as low intensity on T1 and hyperintense on T2, similar to CSF. The translabyrinthiantranscochlear and the middle fossa approaches are used most often for extirpation of these lesions.
Metastatic lesions of the temporal bone are uncommon. However, the petrous apex and clivus are two of
the most common sites for metastasis in the head and neck. The most common primary sites are the
breast, lung, kidney, stomach, prostate, and larynx. Metastases to the skull base in isolation are
uncommon and infraclavicular metastases are usually present. A bone scan should be performed when
metastasis is suspected. Most tumors are erosive, but some stimulate sclerosis from osteoblastic activity.
The MRI appearance is variable depending on the type of lesion.
Other lesions of the petrous apex that should be in the differential include: petrous apicitis usually due to
pseudomonas and rarely TB; intrapetrous carotid artery aneurysm, chondroma/chondrosarcoma,
meningioma, schwannoma, and chordoma.
Case Presentation
A 64-year-old white lady presented with a nine-month history of asymmetric left-sided hearing loss of
sudden onset. The hearing loss was accompanied by tinnitus. She denied headache, vertigo, and otalgia.
She denied any significant medical or surgical history and had no history of otologic disease, nor previous
otologic surgery. She did have a history of left Bell's Palsy one year before presentation, which had
resolved completely. She admitted having a 75 pack/year smoking history. Audiometry revealed an
asymmetric, moderate to profound, left sensorineural hearing loss, absent acoustic reflexes and
significantly depressed speech audiometry. CT scanning of the temporal bones showed a well defined,
lobulated, expansile lesion of the left petrous apex. By MRI, the lesion was bright on T1 and T2. CT
scanning of the chest and a screening mammogram were negative. The petrous apex was entered after
complete mastoidectomy via the retrofacial air cell tract. A cyst filled with a brown exudate consistent
with a cholesterol granuloma was drained into the mastoid using a pediatric feeding tube. Two months
post operatively, she was asymptomatic, doing well, and had minimal change in her hearing.
Bibliography
Appling D, Jenkins HA, Patton GA. Eosinophilic granuloma in the temporal bone and skull. Otolaryngol Head Neck Surg
1983;91:358-364.
Arriaga MA, Brackmann DE. Differential diagnosis of primary petrous apex lesions. Am J Otol 1991;12:470-474.
Atlas MD, Moffat DA, Hardy DG. Petrous apex cholesteatoma: diagnostic and treatment dilemmas. Laryngoscope
1992;102:1363-1368.
Benecke EE, Kuczer A, Cooper MH. Anatomic importance of the retrofacial air tract. Am J Otol 1992;13:499-500.
Behnke EE, Schindler RA. Dermoid of the petrous apex . Laryngoscope 1984;94:779-782.
Brown RV, Sage MR, Brophy BP. CT and MR findings in patients with chordomas of the petrous apex. AJNR Am J
Neuroradiol 1990;11:121-124.
Charachon R, Crouzet G, Vasdev A, Gratacap B. Tumours of the petrous apex: a clinical series. J Neuroradiol 1988;15:186201.
Chole RA. Petrous apicitis: surgical anatomy. Ann Otol Rhinol Laryngol 1985;94:251-256.
Dalley RW, Robertson WD, Nugent RA. Computed tomography of calvarial and petrous bone sarcoidosis. J Comput Assist
Tomogr 1987;11:884-886.
Davis AE, Robin PE. Transpalatal approach to the petrous apex. J Laryngol Otol 1989;103:94-96.
DeLozier HL, Parkins CW, Gacek RR. J Laryngol Otol 1979;93:177-180.
Franklin DJ, Jenkins HA, Horowitz BL, Coker NJ. Management of petrous apex lesions. Arch Otolaryngol Head Neck
Surg 1989;115:1121-1125.
Frates MC, Oates E. Petrous apicitis: evaluation by bone SPECT and magnetic resonance imaging. Clin Nucl Med
1990;15:293-294.
Gacek RR. Diagnosis and management of primary tumors of the petrous apex. Ann Otol Rhinol Laryngol Suppl 1975;18:120.
Gacek RR. Evaluation and management of primary petrous apex cholesteatoma. Otolaryngol Head Neck Surg 1980;88:519523.
Gates GA. The lateral facial approach to the nasopharynx and infratemporal fossa. Otolaryngol Head Neck Surg
1988;99:321-325.
Gherini SG, Brackmann DE, Lo WW, Solti-Bohman LG. Cholesterol granuloma of the petrous apex. Laryngoscope
1985;95:659-664.
Ghorayeb BY, Jahrsdoerfer RA. Subcochlear approach for cholesterol granulomas of the inferior petrous apex. Otolaryngol
Giddings NA, Brackmann DE, Kwartler JA. Transcanal infracochlear approach to the petrous apex. Otolaryngol Head
Neck Surg 1991;104:29-36.
Glasscock ME 3d, Woods CI, Poe DS, Patterson AK, Welling DB. Petrous apex cholesteatoma. Otolaryngol Clin North
Am 1989;22:981-999.
Goin DW. Surgical management of petrous apex meningioma. Laryngoscope 1979;89:204-213.
Goldsmith AJ, Myssiorek D, Valderrama E, Patel M. Unifocal Langerhans' cell histiocytosis (eosinophilic granuloma) of
the petrous apex. Arch Otolaryngol Head Neck Surg 1993;119:113-116.
Heinrich DE, Gantz BJ, Moore SA, Schubkegel AJ. Undifferentiated small-cell neoplasm of the petrous apex: a case report.
Hendershot EL, Wood JW, Bennhoff D. The middle cranial fossa approach to the petrous apex. Laryngoscope 1976;86:658http://www.bcm.edu/oto/grand/8494.html (4 of 6) [5.11.2005 10:44:18]
663.
Horn KL, Hankinson HL, Erasmus MD, Beauparalant PA. The modified transcochlear approach to the cerebellopontine
angle. Otolaryngol Head Neck Surg 1991;104:37-41.
Jackler RK, Parker DA. radiographic differential diagnosis of petrous apex lesions. Am J Otol 1992;13:561-574.
Jahn AF, Farkashidy J, Berman JM. Metastatic tumors in the temporal bone - a pathophysiologic study. J Otolaryngol
1979;8:85-94.
Kearns DB, Coker NJ, Pitcock JK, Jenkins HA. Tuberculous petrous apicitis. Arch Otolaryngol 1985;111:406-408.
Lempert J. Complete apicectomy (mastoidotympano-apicectomy): a new technique for the complete exenteration of the
apical carotid portion of the petrous pyramid. Arch Otolaryngol 1937;25:144-177.
Lipkin AF, Coker NJ, Jenkins HA, Alford BR. Intracranial and intratemporal facial neuroma. Otolaryngol Head Neck Surg
1987;96:11-77.
Montgomery WW. Cystic lesions of the petrous apex: transsphenoid approach. Ann Otol Rhinol Laryngol 1977;86:429435.
Prasad S, Janecka IP. Efficacy of surgical treatments for squamous cell carcinoma of the temporal bone: a literature review.
Roland PS, Meyerhoff WL, Judge LO, Mickey BE. Asymmetric pneumatization of the petrous apex. Otolaryngol Head
Neck Surg 1990;103:80-88.
Samy LL, Girgis IH. Transzygomatic approach to the petrous apex with a record of three cases. J Laryngol Otol
1967;81:277-289.
Smith PG, Leonetti JP, Kletzker GR. Differential clinical and radiographic features of cholesterol granulomas and
cholesteatomas of the petrous apex. Ann Otol Rhinol Laryngol 1988;97:599-604.
Solodnik P, Som PM, Shugar JM, Sachdev VP, Sacher M, Lanzieri CF, et al. Intraosseous petrous apex neuroma: CT
findings. J Comput Assist Tomogr 1986;10:1027-1029.
Thedinger BA, Nadol JB Jr, Montgomery WW, Thedinger BS, Greenberg JJ. Radiographic diagnosis, surgical treatment
and long-term follow-up of cholesterol granulomas of the petrous apex. Laryngoscope 1989;99:896-906.
Umansky F, Valarezo A, Elidan J. The microsurgical anatomy of the abducens nerve in its intracranial course.
Laryngoscope 1992;102:1285-1292.



Causes of Failure and Complication in Surgery for

Otosclerosis
Mas Takashima, M.D.
November 11, 1999
The restoration of hearing in patients with conductive hearing loss secondary to

otosclerosis has, as some may say, been the most important development in
otology. A number of techniques for opening the oval window and reconstructing
http://www.bcm.edu/oto/grand/111199.htm (1 of 15) [5.11.2005 10:44:32]
the ossicular chain have been developed since Rosen first mobilized the stapes in
1953. Excellent hearing improvement is possible with most of the techniques in use
today. However, a significant number of complications or failures continue to
compromise the expected hearing results and necessitate revision surgery. The best
chance of success is at the time of the initial procedure, with revision surgery
successful in less than 80% of cases. It is the purpose of this grand rounds to
review the most common pitfalls incurred in stapedectomy, as well as to report
surgical complications of this procedure.
The pertinent anatomy includes the tympanic membrane, the anterior and posterior
crus of the stapes, the capitulum, the long process of the incus, the tendon and
stapedius muscle, the footplate of the stapes, the tympanic segment of the facial
nerve, the cochlea, and the labyrinth.
The earliest surgical attempts to improve hearing loss associated with stapes
fixation was by Kessler in 1876. In this procedure, he incised the posterior portion
of the tympanic membrane, separated the incus stapedial joint, removed the bony
canal wall when necessary and attempted to mobilize the stapes by applying
pressure to the capitulum in various directions. If the stapes could not be mobilized,
it was removed. The interest in stapes surgery abruptly stopped in 1900 when at the
International Congress, Politzer and Sutton and other prominent otologists declared
these operations were useless and dangerous. It is surmised that this statement was
prompted by large numbers of complications that had gone unreported.
Next, the area of surgical fistulization began with the work of Barany and
Holmgren. They fistulized the superior semicircular canal in otosclerotic ears,
allowing the dura to rest against the membranous labyrinth. Hearing improvement
was fruitfully noted with this procedure. Sordell studied with Holmgren and
devised a three-stage operation in which the skin-covered horizontal canal fistula
was used to improve hearing. In 1938, Lempert simplified this to a one-stage
operation. After being modified in 1945, this operation became the standard for the
next seven years. The next major advancement occurred in 1952 when Rosen
accidentally mobilized the stapes during palpation on the ossicular chain in a
fenestration operation. The patient noticed remarkable, immediate hearing
improvement, which lasted for years. One year later, when mobilization had
become the standard of care, Rosen reported hearing improvement to at least the 30
decibel level in 22% of 211 operations he performed. In 1954, Shambaugh applied
the operating microscope to stapes mobilization. This paved the way for Shea to
revive the stapedectomy with the modifications of an intact ossicular chain in a
covered oval window. To date, multiple modifications of this procedure have been
performed using various types of prostheses and grafts.
Otosclerosis is the primary focal spongiform disease of the labyrinthine capsule.
Valsalva first described this pathologic process underlying otosclerosis in 1735,

from the autopsy of a deaf patient. Today, there is still some controversy regarding
the etiology and pathogenesis of otosclerosis. Briefly, otosclerosis, otherwise
known as otospongiosis, is thought to be a familial autosomal dominant disorder
with a variable penetrance of 25% to 40%. The mechanism has recently been
suggested to be an autoimmune reaction to cartilaginous remnants and collagen
fiber type II. This is thought to stimulate an inflammatory reaction in lysosomal
proteases in and around the otospongiotic foci.
For a successful surgical outcome, one must begin planning treatment with the
initial physical examination. This begins with an overall observation of the patient.
The age of the patient alone should not be a contraindication to performing surgery.
In patients less than 16 years of age, there is a greater chance that the conductive
impairment is secondary to congenital anomalies rather than otosclerosis. Likewise,
very active diffuse obliterative otosclerosis may be found in a young patient,
predisposing to a higher incidence of complications. Yet surgery should be
considered in the young because hearing acuity is essential during the formative
years of development. Patients older than 75 years are still surgical candidates if
they are in good health. Hearing acuity becomes more important as other senses
decline with age. In order to prepare for surgery and to prevent failures and
complications, infections and anatomic variations must also be considered. The
otologic evaluation may reveal dermatological conditions of the external ear canal
that will predispose to postoperative infections. These include chronic external
otitis, trauma from hearing aides or self-inflicted injury with cotton-tipped swabs.
Likewise, one may see a small, crooked stenotic ear canal that, until corrected, will
restrict surgical movement. Small exostosis usually does not impair access to the
mesotympanum and may be left untouched. Even larger exostosis, which is limited
to the anterior canal wall, usually does not interfere with the surgical approach.
When the removal of the large posterior exostosis is necessary, it may be
impossible to preserve a satisfactory tympanomeatal skin flap. In such cases, it is
best to remove the exostosis first and delay the stapedectomy.
Brahe Pedersen and Felding have postulated a connection between the influenza
viral infection and unexplained sensorineural hearing loss immediately after stapes
surgery. Although without evidence, this cannot be proven, it would seem prudent
to avoid operation when respiratory viral infections are prevalent, especially in the
patient, the patient's family or the surgeon.
The diagnosis of otosclerosis should be confirmed only after other reasons for
progressive hearing loss have been ruled out. The most common differential
diagnoses of conductive losses are malleus fixation, serous otitis media, ankylosis
of the malleus incus joint, Waardenburg's syndrome, Paget's disease,
tympanosclerosis and progressive lysis to the long process of the incus.
Tympanometry provides the most useful method to exclude most of these cases,
particularly serous otitis media and ossicular fixation. Clinical history is of special
value to rule out systemic disease such as Paget's disease, osteogenesis imperfecta
or ankylosing rheumatoid arthritis.
Many of the common anatomic variations, congenital anomalies and pathologic
conditions are not known until seen during surgery. The surgeon must be well
trained and experienced, first in recognizing these malformations, and second, in
skillfully correcting them. A systematic examination of the unusual should be done.
A superiorly located jugular bulb may come into juxtaposition with a tympanic
annulus and in this position is vulnerable to injury during elevation of the tympanic
meatal flap. For this reason, the elevation of the tympanic annulus inferiorly should
not be performed with both strobes of the elevator. Tears of the jugular bulb, of
course, result in profuse bleeding and constitute an alarming, although not serious,
complication. Elevating the head of the operating room table and packing the area
with Gelfoam may control the bleeding. If the bleeding is readily controlled, the
operation may be completed. If the tear is large and the bleeding is difficult to
control, the procedure should be terminated.
The location of the facial nerve should be determined before proceeding with
removal of the stapes. Usually, the facial nerve lies superior to the oval window,
which permits the surgeon to acquire a complete view of the footplate. In this
position, the lateral surface of the nerve is usually covered by bone, although the
inferior surface may be partially dehiscent. When the nerve overhangs the
footplate, there is frequently a lateral as well as an inferior dehiscence to the
fallopian aqueduct. Dehiscence of the facial nerve is not uncommon, and in about
0.5% of middle ears there is a sizable dehiscence, so that the nerve bulges down
and obscures the arch and footplate. Even in these cases, for the experienced
surgeon, it is often possible to remove the footplate, although somewhat blindly,
and the prosthesis can be contoured so as to adapt to the overhanging facial nerve.
Otosclerosis of the round window niche is of no significance unless the round
window opened is completely closed. When obliteration of the niche is severe, only
a dimple may mark its location. From a retrospective study of 30,567
stapedectomies, this has been shown to occur in 300 patients or 1% of cases. In this
situation, the mucosa should be elevated in an attempt to determine that the closure
is complete. When it is not possible to determine with certainly whether the
window is blocked, the stapedectomy should be performed. Patients with round
window closure tend to have an early onset of hearing loss, frequently beginning in
childhood or in adolescence, similar to the hearing losses associated with
obliterative otosclerosis. However, these patients develop a more severe mixed
hearing loss than those patients with disease limited to the oval window. A family
history can be obtained from 50% of the patients with otosclerosis. Shea's series
showed 78% of patients who developed round window closure had a strong family
history consistent with otosclerosis.
The stapedial artery, which is present in the early stage of embryonic life and
persists in many lower animals, is usually absent in the human ear. A pulsating
vessel nearly filling the crural arch will identify a persistent stapedial artery. In
some cases, the stapes can be removed and the prosthesis introduced, but in 0.2%
of the operations it is not feasible to proceed with the surgery. Rupture of the vessel
could result in profuse hemorrhage and is to be meticulously avoided. A small
artery on the footplate is a consistent finding and is not to be confused with the
persistent stapedial artery.
Until the advent of stapedectomy surgery, the high incidence of malleus ankylosis
had not been recognized. Malleus ankylosis occurs with a disease entity unrelated
to, but sometimes associated with, otosclerosis of the oval window. A bridge of
lamellar bone extending from the anterior part of the head of the malleus to the
anterior epitympanic wall fixes the head of the malleus. Presumably, this bridge of
bone results from failure of the malleus to completely separate from the
epitympanic wall during embryonic development. The otologic history, often
secured in retrospect, usually reveals hearing loss in childhood that becomes worse
in adult life. Such cases represent malleus ankylosis with subsequent development
of otosclerosis. When there is a history of hearing loss since early childhood
without progression, the case may represent pure malleus fixation without oval
window otosclerosis. If there is a history of hearing loss dating from childhood or if
the hearing loss is unilateral, the preoperative evaluation could certainly include
tests for malleus mobility. Should examination with the pneumatic otoscope be
inconclusive, mobility should be determined with direct instrumental manipulation
with the aid of magnification. If there is fixation, it is necessary to perform an
atticotomy and widely expose the head of the malleus and the body of the incus.
Proper treatment will consist of amputation of the head of the malleus followed by
ossicular reconstruction.
Congenital anomalies of the stapes and incus are usually suspected because of a
history of hearing problems from early childhood. The anomalies may range from
simple fixation of the footplate due to incomplete resolution of the mesenchymal
tissue during embryologic development, to the absence of the stapes in infancy.
The stapes may be small and articulate with a short, underdeveloped long process
of the incus. The crural arch may contain bone or may be replaced by a single bony
strut. The floor of the oval window niche may consist of a solid layer of bone
without evidence of a footplate or the oval window niche may be completely
missing. The correction of simple congenital stapes fixation may be the same as for
fixation due to otosclerosis. Corrective surgery for anomalies is attended with a
higher incidence of injury to the inner ear because of frequent associations of
anomalies to the membranous labyrinth. For example, the utriculosaccular deck

may be located in the oval window area and may be vulnerable to injury. These
patients should be counseled regarding the greater risk of sensorineural hearing
loss. In very rare cases, the otosclerotic foci may not only obliterate the oval
window niche, but may also extend into the vestibule. This has been shown to
occur in 7%-11% of the cases in which stapedectomies failed. When removing the
bone, the surgeon will find that the growth extends into the vestibule beyond the
footplate level. This is best managed using a slow rotation per minute microdrill to
create a 0.8-mm diameter cylinder. In this situation, laser creates a potential risk to
the patient because of heat transfer to the perilymph of the vestibule and
subsequently to the utriculosaccular.
The gusher is a dramatic complication to stapes surgery. The smallest control hole
in the footplate can produce a perilymph leak so profuse that it fills the middle ear
and internal auditory canal within seconds. The existence of this abnormality is
suggested by an upward sloping air conduction audiogram with best responses at 2
kilohertz. This complication is more common in ears with congenital fixation of the
footplate. Management of the stapes gusher is the same as management for a CSF
leak. Once the surgeon notes the onset of fluid leaking from the aperture in the
stapes footplate, leakage may be controlled with a small piece of Surgicel. A graft
should be placed over the leaking footplate with the completion of the
stapedectomy. A lumbar drain will now effect a diversion of the CSF thereby
decreasing the pressure of the purulence. The head of the patient's bed is elevated
and daily fluid intake is restricted. The flow of CSF may last for several days and a
sensorineural hearing loss is likely to occur. Clearly, if such ears can be identified,
the operation is contraindicated.
Next, we will move on to surgical trauma. Tears of the tympanic meatal flap that
may occur in the following locations. First, a linear tear or buttonhole perforation
may occur in the skin flap. Usually, these tears require no repair. However, when
replacing a flap at the completion of the procedure, care must be taken to avoid
enfolding the margins of the tear. The skin flap may separate from the tympanic
annulus as well. This usually occurs in an inferior location and is due to failure to
elevate the tympanic annulus from the sulcus. Another cause of a tear of this type is
failure to achieve total elevation of skin flap from incision-to-incision. Usually no
repair is required if separation occurs inferiorly. However, if it occurs superiorly,
grafting may be necessary.
Subluxation and luxation of the incus consists of a tear in the capsule of the
incudomalleolar joint, but with a sufficiently intact capsule to maintain the incus in
its normal anatomical position. Although the long process of the incus will be
excessively mobile, the operation may be completed and functional results may be
satisfactory. Luxation of the incus is due to a complete disruption of the
incudomalleolar joint and demands that the removal of the incus and the use of a
malleus over-window prosthesis. Attempts to replace and maintain the incus in its
original position are usually not successful. The incus may be accidentally
dislocating during curetting of the bony annulus and during manipulations of the
oval window. The long process of the incus may be accidentally displaced when
withdrawing the instrument from the oval window niche. Fractures of the long
process of the incus are an unusual complication, but may happen during the wire
tightening procedure. If the fracture appears near the tip of the long process, the
wire may still be placed on the stump. If this is not possible, the procedure must be
altered.
Injury to the facial nerve during stapedectomy is uncommon, but there may be a
direct injury to the nerve in the facial canal by careless use of the instruments,
touching with the drill or heating by a drill if the tip is not cool. An interesting
remark made by Schuknecht states that direct instrumental injury to the facial nerve
is extremely rare and has only occurred once during his numerous stapedectomies.
Since the risk to the facial nerve injury is almost nil, Schuknecht considered that
the possibility of its occurrence need not be mentioned to the patient before
operation.
During a stapedectomy operation, it is frequently necessary to displace the chorda
tympani nerve to gain adequate exposure. There is controversy concerning the
advisability of stretching the nerve or cutting it. Approximately one-fourth of
patients complain of ageusia in the postoperative period. Chorda tympani nerve
dysfunction is usually transient and fewer than 5% of patients experience
permanent deficits. It should be appreciated that if the nerve is cut, it will produce
permanent loss of sensation of taste on the anterior two-thirds of the tongue on the
same side. No reinnervation of the taste buds can take place, either from the chorda
tympani nerve on the opposite side or the posterior third of the tongue supplied by
the glossopharyngeal nerve. Permanent loss of the chorda tympani innervation
results in atrophy of the taste receptors. The dorsum of the tongue becomes smooth
and pale. The patient may not complain of the loss of taste if one nerve is cut, as
long as the tongue retains sensation for 66% of the surface. If the nerve is stretched
during operation, a persistent abnormal sensation in the tongue may occur,
described by the patient as salty or metallic. Bilateral loss of the chorda tympani
nerve produces marked symptoms in the majority of patients. In addition to the loss
of taste, there is loss of secretum motor supply to the submandibular and sublingual
glands that produces an uncomfortably dry mouth. Deficits include a diminution of
taste sensation or a dry mouth. Revision causes cases to deserve special attention.
Sensorineural hearing loss is perhaps the most disappointing and devastating
complication for both the surgeon and patient. Complete sensorineural hearing loss
can occur even in the most meticulously and appropriately performed stapes
procedure. Most commonly, however, are complications due to surgical trauma.

Intraoperative studies performed in stapedectomy indicate that acoustic trauma in
drilling, excessive movement of the stapes producing a hydraulic effect, rupture of
the membranous inner ear, rapid loss in perilymph that may lead to damage at the
membranous structures in the vestibule, footplate fragments or bone death in the
vestibule, and the floating footplate are the great offenders for vestibular and
presumably cochlear damage. The reported incidence of dead ears after operation
varies from 0.5% to 4%. It is important to appreciate that these are figures from a
series of operations performed by expert surgeons with a special interest in
stapedectomy. Results achieved by the occasional operator are not reported and it is
likely that the incidence of cochlear damage is much higher. Mawson has shown
that in a series of 1000 operations, there was a hearing loss at 4% in the first 50
operations, 2% hearing loss after the next 50 operations, no loss in the next 500 and
0.25% of the remaining 400 operations.
An attempt at removal of the floating footplate may result in a dead ear and if this
complication is encountered it must be dealt with correctly. If the footplate is
visible, it may be possible to remove it by manipulation and extraction with a fine
hook, or a small drill hole may be made at the margin of the oval window and a
fine hook used to remove it. If the footplate cannot be removed without excessive
manipulation, it should be left in place and a soft tissue graft placed over the oval
window and the operation abandoned.
The depressed footplate may result from incorrect attempts to remove a floating
footplate or may occur during attempts to carry out a total stapedectomy. Causse
documented this at an incidence of 0.16%. No attempt should be made to retrieve
the submerged footplate by instrument as this may cause severe cochlear damage.
Roche et al have described a method of dealing with this complication that is not
damaging to the contents of the vestibule. Drops of blood are poured laterally into
the vestibule and when the vestibule is full of blood and a clot is formed, the clot is
removed by a lateral application of the sucker and the footplate coated with the clot
is usually removed from the oval window. It is of paramount importance for the
surgeon to understand the relationship of the stapes footplate to the vestibular
contents. Almost all patients have a minimal safe distance of approximately 1-mm
between the medial surface of the footplate and the utricle and saccule. Penetration
of the vestibule by more than 1 mm with the footplate instruments or prosthesis
may perforate the utricle and saccule inducing sensorineural hearing loss, vertigo or
both. In patients with endolymphatic hydrops the saccule may extend to contact the
medial surface of the footplate, thereby placing these patients at a higher risk for
complications from this procedure.
Concerning postoperative complications, prosthesis displacement is the most
common postoperative complication, accounting for 50-70% of revision surgeries.
The hearing deterioration may be acute, as the prosthesis becomes dislodged or
chronic as it gradually displaces out of position. This complication may be

encountered many years after stapes surgery. The diagnosis is definitely made at
reoperation, but may be suspected based on tuning fork and audiometric tests.
Prosthesis loosening due to erosion or notching of the incus and prosthesis
displacement due to necrosis of the incus also occurs. With removal of the stapes
arch, the blood supply to the long process of the incus is divided as it courses over
the incudostapedial joint. Collateral vessels exist from the body of the incus and
maintain the viability of the remaining bone. Circumferential overtightening of the
prosthesis can remove the blood supply to the tip of the incus.
The signs and symptoms of perilymphatic fistula were first described by Lewis in
1961, and this complication, which at one time was thought be unusual, is now
accepted as being one of the more common complications of stapedectomy. The
surgeon creates a fistula at every stapedectomy operation and relies on the natural
process of healing or in some techniques a graft of soft tissue, to seal the opening
that has been made. In most operations, there is enough surgical trauma to the oval
window mucoperiosteum to lead to the production of an inflammatory repair
envelope around the prosthesis, sealing the opening into the oval window. With
symptoms of a perilymphatic fistula or fluctuating hearing loss, tinnitus, a feeling
of fullness in the ear and vertigo, there is no doubt that a small fistula remained
after many stapedectomy operations with incomplete closure of air bone gap, but
the hearing result may be acceptable to the patient. Although a perilymph fistula
usually leads to sensorineural hearing loss, this is not always the case and a
persistent conductive loss after an operation should warn the surgeon that there
might be a fistula. A revision operation to close it would be the best line of
treatment. The fistula may be primary, dating from the time of operation when
there is a failure of the oval window to seal, or it may be secondary, where it can
appear many months or even years after the original operation. Hearing tests
carried out soon after the onset of fistula will show findings similar to those seen in
labyrinthine hydrops. For example, puretone sensorineural hearing loss and low
frequency initially, followed by a flat loss that fluctuates. There may be recruitment
as well. Vestibular tests such as the Hallpike caloric test, EMG, and fistula test with
a pneumatic otoscope may all be helpful, but have been found to be negative in
20% of the cases. Welder described a newer technique, diagnosis by radioactive
Indian-111 that is injected into the lumbar subarachnoid space. The demonstration
of an increased radioactivity in the nasopharyngeal secretions strongly supports the
diagnosis of a fistula.
The treatment of a perilymphatic fistula is a tympanotomy at the earliest possible
moment with an attempt to close the fistula. When the leak is detected, the fistulous
track is excised and the prosthesis removed with great care. The opening of the
vestibule is covered with a soft tissue graft that is held in place by another
prosthesis. Unless early treatment is instituted, the chances of restoration or

improvement of hearing are small. In some cases troublesome dysequilibrium may
remain. It is imperative that the surgeon is fully aware of these complications and
realizes that some techniques are safer than others. The use of Gelfoam to seal the
oval window in stapedectomy produces a very thin membrane and has caused the
highest incidence of fistula formation. The perilymphatic fistula is the only
complication of stapedectomy that is dangerous, and although the risk of meningitis
is small, death from meningitis after this operation has been reported.
As with the perilymphatic fistula, postoperative granulomas can also be a cause of
dysequilibrium, vertigo or progressive sensorineural hearing loss. Granuloma
formation is seen in approximately 1:100 cases of revision stapedectomy. Foreign
body reaction is a suspected etiology of postoperative granuloma formation. Glove,
starch and Teflon have all been implicated. For this reason, direct pullout prosthesis
contact should be avoided. It may also be prudent to rinse the prosthesis prior to its
insertion. The condition usually manifests between the 5th and 15th postoperative
day and is characterized symptomatically by hearing loss after an initial hearing
gain or a sensation of unsteadiness. Associated with the hearing loss are loss of
speech discrimination and a sensation of fullness in the ear. Examination reveals an
edematous, thickened and hypervascular skin flap as well as dullness and reddening
at the posterior part of the tympanic membrane. Audiometric studies show a
combined sensorineural and conductive hearing loss that is worse in the higher
frequency that is also associated with decreased speech discrimination. High-dose
steroids may decrease the inflammatory response and its effects on the inner ear.
Emergency surgical intervention is imperative. Surgical exploration reveals a
granulomatous mass extending from the oval window niche to completely envelop
the prosthesis and the long process of the incus. In about half of the cases the
granuloma extends into the vestibule. The granuloma, including the portion within
the vestibule, must be removed in its entirety along with the prosthesis. They may
also be vaporized with laser after the prosthesis has been removed.
Cholesteatoma following stapedectomy is a rare complication. Mawson reported
only one post-stapedectomy cholesteatoma in a review of nearly 2000 cases. Only
five other cases have been previously reported in the literature. Proposed
mechanisms for formation include prosthesis extrusion, the presence of a squamous
epithelium in the fascial graft, inversion of the tympanomeatal flap and a marginal
perforation associated with a disruptive annulus.
Diplacusis occurs in approximately one-third of the patients. There may be a
variation of distortion of sound, especially music and the human voice, of which
patients frequently complain. For the first few weeks after the operation most
people complain of pure tones appearing higher in pitch when compared with the
unoperated side. It is seldom problematic and usually fades by six weeks
postoperatively. Almost all patients have some degree of phonophobia

postoperatively as well. Reassurance is adequate treatment. A subset of this has
been documented as an abnormal, emotional response. In a 1993 article in
Laryngoscope, Lloyd Stores describes such a case:
"This is the most curious and unexpected phenomena that I have ever encountered.
They brought this girl in that I had performed the stapedectomy a few days prior.
Honestly, her mouth was open and fixed and she had a glassy stare. From the
history, it turns out that her symptoms developed almost immediately after I took
the first packing out of her ear canal. The mother-in-law was a talker and she
continued to talk about how useless her daughter-in-law was. The daughter-in-law,
my patient, had not been hearing all of this for years and she went into a catatonic
trance that was unbelievable. She really freaked."
In conclusion, we have come a long way from the original attempts at mobilization
of the stapes by Kessel, as resurrected by Rosen, which led to the modern day
stapedectomy performed by Shea. A survey of articles in the last 30 years
concerning the complications of stapedectomy indicates that although there are
some differences over the relative importance attributed to a certain problem, there
is general agreement about the direction in which new research should be directed.
The situation is well summarized by Dr. Shea in his 40-year report on over 14,449
stapedectomies, where he makes the observation that although the original
objections to stapedectomy have all but disappeared, it is now increasingly
necessarily to concentrate on the problems arising from the operation itself.
Case Report
I.K. is a 36-year-old Indian male who presents to the BTGH clinic with a several
year history of progressively worsening hearing loss in his left ear. The patient has
previously undergone an unknown middle ear procedure on the right ear,
performed in another state for hearing loss. Of note is that the patient's mother and
father also have had some degree of hearing loss. An audiogram obtained on March
23, 1999 revealed a 40-50 decibel conductive hearing loss in the left ear with a pure
tone average of 67 decibels. On physical exam, the patient had an unremarkable
head and neck examination except for his conductive hearing loss. The patient was
taken to the operating room on April 13, 1999. Upon elevation of the patient's
tympanomeatal flap, there was a narrowed oval window niche. The facial nerve
was in the fallopian canal without dehiscence. The stapes was fixed with diffuse
otosclerosis of the footplate. A 0.8 mm diamond drill was then used to thin the
thickened footplate. The footplate was then perforated and a 4.0 mm Fisch stapes
piston was placed.
Postoperatively, the patient did well, and during the first 15 days, he stated that his
hearing was markedly improved. On post operative day 16, the patient experienced
a sudden decrease of hearing in that ear. There were no associated symptoms of
vertigo, otorrhea or ear pain. A repeat audiogram was performed which revealed
similar results as the preoperative test. The patient was thus taken to the operating
room again on June 17, 1999, for a middle ear exploration, where it was noticed
that the oval window was once again obliterated. It was demonstrated that the
prosthesis was no longer sitting in the oval window, but was surrounded and riding
on top of a thickened and obliterated footplate. A re-drill out of the oval window
was performed, and the vestibule was entered carefully with a sharp pick. A longer
4.5mm Fisch prosthesis was then used. Postoperatively, the patient was started on
sodium fluoride. Unfortunately, the patient never returned for a postoperative visit.
Bibliography
Backous DD, Coker NJ, Jenkins HA. Prospective study of resident-performed stapedectomy. Am
J Otol 1993;14:451-454.
Beales PH. Otosclerosis. Triangle West, Bristol: John Wright & Sons; 1981. pp 85-185.
Belal A Jr, Ylikoski J. Poststapedectomy dizziness. A histopathologic report. Am J Otol
1982;3:187-191.
Bhardwaj BK, Kacker SK. Revision stapes surgery. J Laryngol Otol 1988;102:20-24.
Bracewell A. Diplacusis binauralis--a complication of stapedectomy. J Laryngol Otol 1966;80:5560.
Brackmann DE. Otologic surgery. Philadelphia: Saunders; 1994. pp 347-384.
Burnam JA, Lazar L, Pickard RE. Cholesteatoma: A complication of stapedectomy. Eye Ear
Nose Throat 1971;50:324-326.
Causse J, Causse JB. Eighteen years report on stapedectomy: Problems of fixation. Clin
Clairmont AA, Nicholson WL, Turner JS. Pseudomonas aeruginosa meningitis following
stapedectomy. Laryngoscope 1975;85:1076-1083.
Coker NJ, Duncan NO, Wright GL, Jenkins HA, Alford BR. Stapedectomy trends for the
resident. Ann Otol Rhinol Laryngol 1988;97:109-113.
Cremers CW, Hombergen GC, Wentges RT. Perilymphatic gusher and stapes surgery. A
predictable complication? Clin Otolaryngol 1983;8:235-240.
Derlacki EL. Revision stapes surgery: Problems with some solutions. Laryngoscope
1985;95:1047-1053.
Dornhoffer JL, Bailey HA, Jr., Graham SS. Long-term hearing results following stapedotomy.
Am J Otol 1994;15:674-678.
Doyle PJ, Woodham J. Results of stapes surgery - subjective and objective. J Otolaryngol
1980;9:381-386.
Fenton JE, Turner J, Shirazi A, Fagan PA. Post-stapedectomy reparative granuloma: A
misnomer. J Laryngol Otol 1996;110:185-188.
Ferguson BJ, Gillespie CA, Kenan PD, Farmer JC Jr. Mechanisms of cholesteatoma formation
following stapedectomy. Am J Otol 1986;7:420-424.
Flood LM, Kemink JL, Kartush JM. Pneumocephalus following treatment of a stapes gusher. Am
J Otol 1985;6:508-511.
Franklin DJ, Pollak A, Fisch U. Meniere's symptoms resulting from bilateral otosclerotic
occlusion of the endolymphatic duct: An analysis of a causal relationship between otosclerosis
and Meniere's disease. Am J Otol 1990;11:135-140.
Freeman J. Failures in surgery for stapedial otosclerosis. Laryngoscope 1981;91:1245-1258.
Hammerschlag PE, Fishman A, Scheer AA. A review of 308 cases of revision stapedectomy.
Laryngoscope 1998;108:1794-1800.
Horn KL. Surgical management of otosclerosis: Primary stapes surgery. Oper Tech Otolaryngol
Head Neck Surg1998;9:1-12.
Gibb AG, Pang YT. Surgical treatment of tympanosclerosis. Eur Arch Otorhinolaryngol
1995;252:1-10.
Glasscock ME 3rd, Storper IS, Haynes DS, Bohrer PS. Twenty-five years of experience with
Hough JV, Dyer RK Jr. Stapedectomy. Causes of failure and revision surgery in otosclerosis.
Krieger LW, Lippy WH, Schuring AG, Rizer FM. Revision stapedectomy for incus erosion:
Long term hearing. Otolaryngol Head Neck Surg 1998;119:370-373.
Krmpotic-Nemanic J, Draf W, Helms J. Surgical Anatomy of the Head and Neck. Berlin,
Germany: Springer Verlag, 1985.
Langman AW, Lindeman RC. Revision stapedectomy. Laryngoscope 1993;103:954-958.
Leighton SE, Robson AK, Freeland AP. Audit of stapedectomy results in a teaching hospital.
Clin Otolaryngol 1991;16:488-492.
Man A, Segal S. Serous otitis media as a complication of stapedectomy. Ear Nose Throat J
1980;59:510-513.
Mann WJ, Amedee RG, Fuerst G, Tabb HG. Hearing loss as a complication of stapes surgery.
Mawson SR. Management of complication of stapdectomy. J Laryngol Otol 1975;89:145-149.
Merifield DO. Epithelial cyst as a complication of stapedectomy: A case report. Laryngoscope
1965;75:1893-1896.
Meyer SE. The effect of stapes surgery on high frequency hearing in patients with otosclerosis.
Am J Otol 1999;20:36-40.
Miglets AW, Paparella MM, Saunders WH. Atlas of Ear Surgery. St. Louis, MO: Mosby; 1986.
Millman B, Giddings NA, Cole JM. Long-term follow-up of stapedectomy in children and
adolescents. Otolaryngol Head Neck Surg 1996;115:78-81.
Milstein S. Otosclerosis. Am J Otol 1981;2:283-285.
Morrison AW. Otosclerosis. Br Med J 1962;1:1804.
Parnes LS, Wong L. Pneumo-membranous labyrinth: An unusual complication of a
stapedectomy. Ann Otol Rhinol Laryngol 1998;107:359-361.
Pedersen CB. Revision surgery in otosclerosis--an investigation of the factors which influence
the hearing result. Clin Otolaryngol 1996;21:385-388.
Portmann M, Portmann D. Otologic Surgery: Manual of Oto-surgical Techniques, 2nd ed. San
Diego, CA: Singular Publishing Group; 1998. pp 32-38.
Schuknecht HF. Stapedectomy. Boston, MA: Little Brown; 1971.
Schuknecht HF, Jones DD. Stapedectomy post-mortem findings. Ann Otol Rhinol Laryngol
1979;88:1-43.
Shapira A, Ophir D, Marshak G. Success of stapedectomy performed by residents. Am J

Shea JJ. Stapedectomy - long-term report. Ann Otol Rhinol Laryngol 1982;91:516-520.
Shea JJ, Farrior JB. Stapedectomy and round window closure. Laryngoscope 1987;97:10-12.
Shea JJ Jr. Forty years of stapes surgery. Am J Otol 1998;19:52-55.
Shea JJ Jr. A personal history of stapedectomy. Am J Otol 1998;19:S2-S12.
Sheehy JL, Nelson RA, House HP. Revision stapedectomy: A review of 258 cases. Laryngoscope
1981;91:43-51.
Silverstein H. Laser stapedotomy minus prosthesis (laser STAMP): A minimally invasive
procedure. Am J Otol 1998;19:277-282.
Slattery WH 3rd, House JW. Prostheses for stapes surgery. Otolaryngol Clin North Am
1995;28:253-264.
Smyth GD, Hassard TH. Eighteen years experience in stapedectomy. The case for the small
fenestra operation. Ann Otol Rhinol Laryngol Suppl 1978;87:3-36.
Storrs LA. Complications after surgery for otosclerosis. Laryngoscope 1983;93:265-267.
Wiet RJ, Raslan W, Shambaugh GE Jr. Otosclerosis 1981 to 1985. Our four-year review and
current perspective. Am J Otol 1986;7:221-228.



medical literature.
ISSUES IN STAPEDECTOMY
Douglas D. Backous, MD
February 6, 1992
The history of stapedectomy is one of near discovery, condemnation, reintroduction and refinement.
Prior to the twentieth century poor lighting and magnification, nonstandardized audiometry, and a lack of
understanding of the middle ear conduction mechanism plagued early researchers. Procedures
concentrated on either partial or total removal of the tympanic membrane and ossicles. Mobilization of
the stapes, described by Miot and later by Blake, would eventually be condemned by the otologic world.
Siebenmann, in 1900, capped the early era of stapes surgery by stating, "Clinical experience teaches that
all endeavors at mobilization of the stapes in otosclerosis are not only useless but often harmful."
Between 1900 and 1952 attempts at fenestration of the horizontal semicircular canal came to the
spotlight. Results were reported as excellent but temporary. Microscopy and rotating drills were
introduced. In 1929 the American Otologic Society commissioned a committee to compile a resume of
all literature concerning otosclerosis up to 1928. The result included a 700 page, two volume document
covering histology of the otic capsule, heredity, audiology, and treatment of otosclerosis.
Successful surgery for hearing restoration truly began with Lempert's fenestration operation. Rosen, a
student of Lempert, serendipitously discovered that footplate mobilization improved hearing immediately
in a patient with otosclerosis. He continued to refine his mobilization procedure until Shea, his student,
reopened the era of stapedectomy in 1956. Shea developed the modern technique of footplate
mobilization, soft tissue grafting of the oval window, and ossicular replacement.
Shea is also credited with the first stapedotomy, performed on a young girl in 1960. A 6 millimeter
stapedotomy with piston insertion and soft tissue packing provides equally improved hearing with less
risk for damage to inner ear structures. Higher frequency hearing is preserved better with stapedotomy.
Robinson summed up the stapedectomy/stapedotomy debate best by declaring the "best" procedure is the
operation which provides the individual surgeon with a predictable and safe outcome along with
improved hearing to levels consistent with current otologic standards.
Depletion of the pool of patients with otosclerosis occurred during the "golden age" of stapes surgery
throughout the sixty's and early seventies. Increased numbers of trained otolaryngologists and improved
hearing amplification further contribute to an overall limited exposure in modern practice and residency.
Stapedectomy is the second least performed procedure in American residency, as shown by Harris and
Osborne in 1990.
The issue of training residents to do successful stapedectomy first appeared in the literature in 1983.
Chandler and Rodriguez-Torro revealed a 62% closure of the air-bone gap to within 10 dB in resident
cases, well below the 90-95% standard expected of practicing otologists. Five studies of results from
training programs have since revealed results ranging from 64%-82% closure to within 10 Db in training
programs. Of interest are the results of Shapira, et al. Their residents, in Israel, did stapedectomy in all
four years of training. Results during the first two years of residency compared with American programs.
During the second two years, however, results approached and often equaled those of staff. These
findings further emphasized the critical importance of adequate numbers of procedures to insure
acquisition of competence.
Solutions to the problem of numbers of cases include increased observation of otologic procedures with
correlation in the temporal bone laboratory. Residents should display competence in surgery for chronic
middle ear disease prior to attempting stapedectomy. Finally, all procedures should be supervised by
senior otologic staff and one consistent technique used. For physicians in practice, batching
stapedectomies into groups with review of needed skills in the temporal bone lab followed by
consecutive case completion and review of results could provide valuable feedback in regard to outcome
and maintenance of required skills.
In summary, stapedectomy has a fascinating history. Had Rosen not been open-minded to his first
accidental mobilization, more delay in technique development was inevitable. The best procedure is
surgeon dependent varying with individual skills and experience. Otosclerosis is now at a steady
incidence making increased work in the temporal bone laboratory mandatory for both the acquisition and
maintenance of required skills in footplate manipulation.
Case Presentation
A 68-year-old white male presented with a fifteen year history of progressive hearing loss. He had the
most difficulty hearing in small group conversations. He denied tinnitus, dizziness, otalgia, or chronic
middle ear infections. He did admit to a long history of loud noise exposure. Past surgical and medical
histories were unremarkable. Weber exam showed localization to the right. Audiometry revealed normal
to moderate sensorineural and severe to moderate conductive loss in the right ear with absence of
acoustic reflexes. The left ear had mild to moderate sensorineural loss with normal acoustic immittance
measures. Speech intelligibility scores were within the normal range bilaterally. The patient underwent
right middle ear exploration, stapedectomy, with perichondrial grafting over the oval window and
insertion of a standard well Robinson prosthesis.
Bibliography
Alford BR. Residency program length and structure. Otolaryngol Head Neck Surg 1983;91:1228-230.
Alford BR. Chance favors the prepared mind. The Crowe Lecture, Johns Hopkins School of Medicine, October 2, 1986.
Bailey HA, Pappas JJ, Graham SS. Small fenestra stapedectomy technique: reducing risk and improving hearing.
Benecke JE. Editorial. Am J Otol 1990;11:78.
Chandler JR, Rodriguez-Torro OE. Changing patterns of otosclerosis surgery in teaching institutions. Otolaryngol Head
Neck Surg 1983;91:239-245.
Coker NJ, Duncan NO, Wright GL, Jenkins HA, Alford BR. Stapedectomy trends for the resident. Ann Otol Rhinol
Laryngol 1988;97:109-113.
Conrad GJ. Collective stapedectomy (an approach to the numbers problem). J Laryngol Oto 1990;104:390-393.
Cummings CW, Fredrickson JM, Harker LA, et al. Otolaryngology-Head and Neck Surgery, Vol. IV. St Louis, Toronto:
C. V. Mosby Company, 1986:1095-3113.
Engel TL, Schindler RA. Stapedectomy in residency training. Laryngoscope 1984;94:768-771.

Farrior B. Contraindication to the small hole stapedectomy. Ann Otol 1981;90:636-639.
Farrior JB. Stapedectomy for the home temporal bone dissection laboratory. Otolaryngol Head Neck Surg 1986;94:521525.
Fisch U. Stapedotomy versus stapedectomy. Am J Otol 1982;4:112-117.
Fisch U. Tympanoplasty and Stapedectomy. New York: Theime-Skatton Inc. 1980:57-67.
Glasscock MF, Shambaugh GE. Surgery of The Ear, 4th ed. Philadelphia: WB Saunders, Co., 1980:389-418.
Gristwood RE. The surgical concept for otosclerosis. Adv Oto-Rhino-Laryngol 1988;39:52-64.
Jerger SJ, Jerger JJ. Auditory Disorders: A Manual for Clinical Disorders. Boston: Little, Brown and Company, 1981:131136.
Handley GH, Hicks JN. Stapedectomy in residency - the UAB experience. Am J Otol 1990;11:128-130.
Harris JP, Osborne E. A surgery of otologic training in US residency programs. Arch Otolaryngol Head Neck Surg
1990;116:342-344.
Hillel AD. History of stapedectomy. Am J Otolaryngol 1983;4:131-140.
Hughes GB. The learning curve in stapes surgery. Laryngoscope 1991;101:1280-1284.
Igarashi M, Guilford R, Alford BR. Bilateral vein graft stapedectomy. Acta Otolaryngol 1970;69:9499.
Rosen S. The Autobiography of Dr. Samuel Rosen. New York: Knopf, 1980:48-68.
Levin G, Fabian P, Stahle J. Incidence of otosclerosis. Am J Otol 1988;9:299-301.
Levenson MJ, Bellucci RJ, Grimes C, et al. Otosclerosis surgery in a resident training program. Arch Otolaryngol Head
Neck Surg 1987;113:29-31.
Levy R, Shvero J, Hadar T. Stapedotomy technique and results: ten years' experience and comparative study with
McGee TM. Comparison of small fenestra and total stapedectomy. Ann Otol 1981;90:633-635.
Manu P, Lane TJ, Matthews DA. How much practice makes perfect? A quantitative measure of the experience needed to
achieve procedural competence. Med Teacher 1990;12:367-369.
Marquet J, Creten WL, Van Camp KJ. Considerations about the surgical approach in stapedectomy. Acta Otolaryngol
1972;74:406-410.
Moon CN. Stapedectomy, connective tissue graft and stainless steel prosthesis. Laryngoscope 1968;78:798-807.
Murrant NJ, Gatland DJ. Temporal bone laboratory training for stapedectomy. J Laryngol Otol 1989;103:833-834.
Robinson M. Panel on stapes surgery - total footplate extraction in stapedectomy. Ann Otol 1981;90:630-632.
Robinson M. Is there a "best" stapedectomy technique for the general otolaryngologist? Ear Nose Throat J 1989;68:221224.
Rosen S. Palpation of the stapes for fixation. Arch Otolaryngol 1952;56:610-5.
Shapira A, Ophir D, Marshak G. Success of stapedectomy performed by residents. Am J Otolaryngol 1985;6:388-391.
Shea JJ. Fenestration of the oval window. Ann Otol Rhinol Laryngol 1958;67:932-51.
Shea JJ. Thirty years of stapes surgery. J Laryngol Otol 1988;102:14-19.
Schuknecht HF. Editorial-Training in otolaryngology. Arch Otolaryngol 1979;105:57.
Smyth GDL, Hassard TH. Eighteen years experience in stapedectomy-the case for the small fenestra operation. Ann Otol
Vernick DM. Stapedectomy results in a residency training program. Ann Otol Rhinol Laryngol 1986;95:477-479.
Vernick DM. Who operated on Max? Ear Nose Throat 1988;67:125.
Weir N. Otolaryngology: An Illustrated History. London: Butterworth, 1990:203-212.


medical literature.
SUDDEN SENSORINEURAL HEARING LOSS

Randall S. Zane, MD
March 5, 1992
Sudden sensorineural hearing loss is a confusing and controversial issue in otolaryngology that most of
us will encounter in our practice. The impact of the loss of hearing on the patient can be catastrophic and
most physicians consider it a medical emergency. On the other hand, it usually becomes frustrating for
the physician, as the cause is most often not obvious and the treatment empiric and poorly understood.
There is no accepted definition of the problem, no standard method for evaluating these patients, no
agreement on what the usual etiology is, no easy way to stage the loss and no clearly effective treatment.
Some authors feel strongly that sudden sensorineural hearing loss is just a symptom and not a disease
entity. The most useful definition of sudden sensorineural hearing loss is an idiopathic loss greater than
30 Db in three contiguous frequencies and that occurs in less than 3 days. Most sudden losses occur
within minutes to several hours, and about 1/3 of these patients awaken in the morning with a hearing
loss. Approximately 1/2 note imbalance or vertigo and the intensity of vertigo correlates in general with
the degree of hearing loss. Byl reported an incidence of between 5-20 cases per 100,000 population per
year.
Doug Mattox and Blair Simmons at Stanford reported a 5 year prospective study involving 166 patients,
looking at the natural history of the process. They found that males and females were equally affected
with a mean age of 46 years. There was no seasonal or geographic clustering. They noted that 65% of
patients showed some degree of recovery without any medical or surgical intervention at all. The
likelihood of recovery was influenced by the shape of the audiogram - all but one patient with an upward
sloping audiogram had complete or good recovery and all but two patients with a severely downsloping
audiogram had a fair or poor recovery. Other poor prognostic indicators that are generally agreed upon in
the literature include sensitivity loss greater than 90 Db in the mid-frequencies, advanced age, and the
presence of vertigo. In his triologic thesis, Dr. Frederick Byl compiled data from 225 patients over an
eight year period forming a prognostic table. He also noted the direct relationship between the severity of
the initial hearing loss and potential recovery and recorded several modifying factors. The study
confirmed that the sooner a patient is seen, the better the prognosis for recovery.
Unfortunately, there is no better understanding of the etiology of sudden sensorineural hearing loss than
there was 1 or 2 decades ago. It is certain that there is no single etiology -the loss of hearing is just the
final outcome for many potential insults to the inner ear. The four most accepted hypothetical etiologies
are viral infections, vascular insult, labyrinthine membrane rupture, and autoimmune disease.
Although viral infection of the inner ear is an attractive theoretical explanation for sudden hearing loss,
the supporting evidence is not conclusive and consists of 4 types:
1) coincident symptoms suggesting coincident viral infection;
2) serological data showing coincident viral infection;
3) histopathological studies showing similarities between known viral infections and cases of sudden
hearing loss;
4) extrapolations from the degeneration of the inner ear seen in viral disease such as the prenatal rubella
syndrome.
Uncontrolled studies from Van Dishoeck, Jaffe, and Schuknecht reported "flu-like" symptoms in 20 60% of patients suffering idiopathic sudden sensorineural hearing loss. However, a study by Rowson and
Hinchcliffe in 1975 found the same symptoms of upper respiratory tract infection to be present in
approximately 40% of the general population. Veltri et al looked at 77 unmatched patients with sudden
sensorineural hearing loss and found a conversion rate of 65%. Multiple viruses were implicated
including influenza A and B, rubeola, rubella, mumps, herpes simplex, and CMV. Wilson and coworkers
studied 122 patients, 63% of whom seroconverted compared with 40% of controls. Other studies from
Morrison and Booth as well as Rowson and Hinchcliffe failed to demonstrate a viral titer rise in sudden
hearing loss patients. The most consistent evidence supporting viral etiology is the histopathologic
findings of varying degrees of atrophy of the organ of Corti, stria vascularis, and tectorial membrane with
variable loss of the neuronal population as shown by Schuknecht and others. Perhaps the strongest
evidence for the involvement of viruses in hearing loss comes from the use of immunofluorescent antigen
studies such as those performed by Davis and Johnson, demonstrating the ability of rubeola and mumps
to infect the inner ears of animal models.
A vascular cause for sudden hearing loss is attractive because it is logically consistent with immediate
onset of symptoms and because there are established models of acute hearing loss secondary to vascular
occlusion in hypercoagulable states such as leukemia and sickle cell disease. However, most of the
experimental and clinical evidence casts serious doubt on a vascular etiology. One would expect that
older patients with known peripheral vascular disease would be the ones most often afflicted, but in fact,
most patients are younger and have no stigmata of systemic vascular disease. Perlman and other
investigators have demonstrated permanent loss of the cochlear microphonic and action potential occurs
after only 30 minutes of ischemia. The histopathology of ears affected by sudden hearing loss also differs
significantly from temporal bones examined after experimental vascular occlusion. Permanent
obstruction leads to marked degeneration of the neurons followed by fibrous and osseous proliferation
within the inner ear, changes that are not seen typically in sudden sensorineural hearing loss.
Intracochlear membrane breaks were proposed as a cause of sudden hearing loss by Simmons in 1968,
but the evidence is only coincidental. Schuknecht and Donovan studied 12 temporal bones of patients
afflicted with sudden sensorineural hearing loss, and found no evidence of Reissner's or basilar
membrane rupture.
There are several known autoimmune diseases that are epidemiologically associated with sudden
sensorineural hearing loss, including Cogan's syndrome, systemic lupus erythematosus, and temporal
arteritis. Some of the most convincing evidence has come out of the University of Tennessee by Yoo and
coworkers who showed that monoclonal antibodies specific for type II collagen can activate an
immunologic response in the inner ear of the rat leading to sensorineural hearing loss as documented by
ABR testing. They were able to demonstrate perivascular inflammation and fibrosis and degeneration of
the spiral ganglion on histopathology, and using immunofluorescent techniques they saw immune
complexes in the otic capsule.
Unfortunately, the lack of scientific understanding of the etiology or etiologies of the problem limits its
treatment to simple empiricism. Every proposed treatment has study to support it, but corroborative data
from independent researchers is usually lacking. Perhaps the fatal flaw in most of those studies is that a
single treatment is applied to a symptom that undoubtedly has multiple etiologies.
Wilson and his colleagues did a double blind study of 67 patients with sudden sensorineural hearing loss.
33 were treated with oral steroids and 34 received placebo. 52 additional subjects served as controls. 76%
of patients who had hearing loss greater than 90 Db failed to recover regardless of whether or not they
received steroids. 78% of the steroid treated patients with hearing loss less than 90 Db improved,
compared with only 38% of matched untreated patients. There have been a few other studies supporting
these findings. However, Dr. Wilson returned to his lab and several years later published a report failing
to demonstrate improved recoveries in patients with proven viral antibody seroconversions treated with
steroids.
The use of diuretics is sort of borrowed from their use in the treatment of Meniere's disease. The use of
heparin and warfarin is borrowed from their use in cerebral vascular occlusion in which they seem to
promote better collateral circulation. Dextran is an example of an effective plasma expander that is useful
in reducing red cell sludging and improving microcirculation. Stellate ganglion blocks produce Horner's
syndrome with vasodilation and increased blood flow and is done by making an injection lateral and
inferior to the thyroid gland to the C-7 vertebra, and then backing off slightly to the area of the ganglion
and injecting Lidocaine. Haug, Draper and Haug demonstrated a 70% chance for a patient to recover a 10
Db pure tone gain and 10 dB discrimination improvement with this procedure compared to a 20% chance
of a similar gain if not treated with stellate ganglion blocks. They also noted that the most successful
cases were treated in the first 20 weeks after symptoms appeared. Some of the vasodilators commonly
employed include nylidrin hydrochloride, papaverine hydrochloride, nicotinic acid, histamine, atropine,
and carbogen. Despite the many studies over the years looking at various vasodilators, there has been no
adequately controlled study that supports the use of vasodilators in sudden hearing loss. Suga and Snow
in their 1969 report actually demonstrated experimentally that vasodilators could in fact reduce cochlear
blood flow by causing shunting. Professor Fisch performed several elegant studies and concluded that
carbogen was the safest and most effective.
"Shotgun" therapy includes as many potentially helpful treatment regimens as can be safely combined.
However, there are very few studies looking at the efficacy of shotgun therapy and those that do exist
obviously have so many variables that they are impossible to control for.
The workup and shotgun regimen recommended by the faculty here at Baylor is attached. This treatment
protocol was put together based in part on the experience of the faculty and in part on the information
available in the literature, but again there is no scientific data to show that it is any more effective than
other treatment protocols or even expectant observation.
In summary, sudden sensorineural hearing loss remains a poorly understood and frustrating problem for
the otolaryngologist. More rational treatment will probably only follow the elucidation of the specific
cause or causes of this disease process.
Case Presentation
A 55-year-old healthy white male smoker with no previous otologic history, noted the acute loss of
hearing in his right ear during his usual morning activities. He denied strenuous activity stating that he
had finished brushing his teeth and began to put on his clothes when he suddenly noted a crescendo high
pitched tone in his right ear and inability to hear. There were no other associated symptoms of dizziness
or visual change. After several hours without improvement, he presented to the emergency room and an
otorhinolaryngology consult was obtained. Head and neck examination including otoscopy was
completely normal. An audiogram revealed a severe primarily sensorineural sensitivity loss on the right.
Laboratory studies including SMA-20, CBC, thyroid function tests, and urinalysis were within normal
limits. Syphilis serology was non-reactive and erythrocyte sedimentation rate was minimally elevated at
15 mm/hr. The veteran was admitted and placed on the Baylor sudden sensorineural hearing loss protocol
which will be described.
Bibliography
Alford BR, Shaver EF, Rosenberg JJ, Guildford FR. Physiologic and histopathologic effects of microembolization of the
internal auditory artery. Ann Otol Rhinol Laryngol 1965;74:728748.
Anderson RG, Meyerhoff WI. Sudden sensorineural hearing loss. Otolaryngol Clin N Am 1981;16:189195.
Beal DD, Hemenway WG, Lindsay JR. Inner ear pathology of sudden deafness: the histopathology of acquired deafness in
the adult coincident with viral infection. Arch Otolaryngol 1967;85:591598.
Belal A, Jr. Pathology of vascular sensorineural hearing impairment. Laryngoscope 1980;90:18311839.
Bredenkamp JK, Shelton C. Sudden hearing loss: determining the specific cause and the most appropriate treatment.
Postgrad Med 1989;86:125132.
Byl FM. Seventysix cases of presumed sudden hearing loss occurring in 1973: prognosis and incidence. Laryngoscope
1977;87:817825.
Byl FM. Sudden hearing loss: eight years experience and suggested prognostic table. Laryngoscope 1984;94:647661.
Cole RR, Jahrsdoerfer RA. Sudden hearing loss: an update. Am J Otol 1988;9:211215.
DeKleyn A. Sudden complete or partial loss of function in the octavus system in apparently normal persons. Acta
Otolaryngol 1944;32:407425.
Donaldson JA. Heparin therapy of sudden sensorineural hearing loss. Arch Otolaryngol 1979;105:351352.
Fisch U. Management of sudden deafness. Otolaryngol Head Neck Surg 1983;91:38.
Fowler E. Sudden deafness. Ann Otol Rhinol Laryngol 1950;59:980987.
Goodhill W. The "idiopathic group" and the "labyrinthine window rupture group" approaches to sudden sensorineural
hearing loss. In: Snow JB,Jr., editor. Controversy in Otolaryngology. Philadelphia: Saunders, 1980:1220.
Gussen R. Sudden hearing loss associated with vascular origins: a temporal bone study. Ann Otol Rhinol Laryngol
1976;85:94100.
Hallberg O. Sudden deafness of obscure origin. Laryngoscope 1956;66:12371267.
Haug O, Draper L, Haug SA. Stellate ganglion block for idiopathic sensorineural hearing loss. Arch Otolaryngol
1976;102:58.
Igarashi M, Alford BR, Konishi S, Shaver EF, Guilford FR. Functional and histopathological correlates after
microembolization of the peripheral labyrinthine artery in the dog. Laryngoscope 1969;79:602623.
Igarashi M, Card G, Johnson PE, Alford BR. Bilateral sudden hearing loss and metastatic pancreatic adenocarcinoma.
Arch Otolaryngol 1979;105:196199.
Jaffe BF. Sudden deafness: an otologic emergency. Arch Otolaryngol 1967;86:8186.
Jaffe BF. Sudden deafness a local manifestation of systemic disorders: fat emboli, hypercoagulation, and infection.
Laryngoscope 1970;80:788801.
Jaffe BF. Hypercoagulation and other causes of sudden hearing loss. Otolaryngol Clin N Am 1975;8:395403.
Jaffe BF. Clinical studies in sudden deafness. Adv Otorhinolaryngol 1978;20:221228.
Jaffe BF, Maasab HF. Sudden deafness associated with adenovirus infection. N Engl J Med 1967;276:14061409.
Jenkins HA, Pollack AM, Fisch U. Polyarteritis nodosa as a cause of sudden deafness. Am J Otolaryngol 1981;2:99107.
Jerger J, Allen G, Robertson D, Harford E. Hearing loss of sudden onset. Arch Otolaryngol 1961;73:350357.
Kaplan SL, Catlin FI, Weaver T, Feigin RD. Onset of hearing loss in children associated with bacterial meningitis.
Pediatrics 1984;73:575578.
Lipkin AF, Jenkins HA, Coker NJ. Migraine and sudden sensorineural hearing loss. Arch Otolaryngol Head Neck Surg
1987;113:325326.
McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol 1979;88:585-589.
Mattox DE, Lyles CA. Idiopathic sudden sensorineural hearing loss. Am J Otol 1989;10:242247.
Mattox DE, Simmons FB. Natural history of sudden sensorineural hearing loss. Ann Otol Rhinol Laryngol
1977;86:463480.
Meyerhoff WL. The management of sudden deafness. Laryngoscope 1979;89:18671868.

Meyerhoff WL, Paparella MM. Medical therapy of sudden hearing loss. In: Snow JB, Jr., editor. Controversy in
Otolaryngology. Philadelphia: Saunders, 1980:311.
Nadol J, Wilson W. Treatment of sudden hearing loss is illogical. In: Snow JB, Jr., editor. Controversy in Otolaryngology.
Philadelphia: Saunders, 1980:2332.
Noury KA, Katsarkas A. Sudden unilateral sensorineural hearing loss: a syndrome or a symptom. J Otolaryngol
1989;18:274278.
Ohlms LA, LonsburyMartin BL, Martin GK. Acousticdistortion products: separation of sensory from neural dysfunction in
sensorineural hearing loss in human beings and rabbits. Otolaryngol Head Neck Surg 1991;104:159174.
Rowson K, Hinchicliffe R. A virological and epidemiological study of patients with acute hearing loss. Lancet 1975;1:471474.
Schuknecht HF, Benitez J, Beekhuis J, Igarashi M, Singleton G, Ruedi L. The pathology of sudden deafness.
Laryngoscope 1962;72:11421157.
Schuknecht HF, Donovan ED. The pathology of idiopathic sudden sensorineural hearing loss. Arch Otorhinolaryngol
1986;243:115.
Shaia FT, Sheehy JL. Sudden sensorineural hearing impairment: a report of 1220 cases. Laryngoscope 1976;86:389398.
Shikowitz MJ. Sudden sensorineural hearing loss. Med Clin North Am 1991;75:12391250.
Siegel LG. The treatment of idiopathic sudden sensorineural hearing loss. Otolaryngol Clin N Am 1975;8:467473.
Simmons FB. Theory of membrane breaks in sudden hearing loss. Arch Otolaryngol 1968;88:4148.
Singleton G. Cervical sympathetic chain block in sudden deafness. Laryngoscope 1971;81:734.
Suga F, Preston J, Snow JB, Jr. Experimental microembolization of cochlear vessels. Arch Otolaryngol 1970;92:213220.
Suga F, Snow JB, Jr. Cochlear blood flow in response to vasodilating drugs and some related agents. Laryngoscope
1969;79:19561979.
Van Dishoeck H, Bierman T. Sudden perceptive deafness and viral infection. Ann Otol Rhinol Laryngol 1957;66:963980.
Veltri RW, Wilson WR, Sprinkle RM, Rodman SM, Kavesh DA. The implication of viruses in idiopathic sudden hearing
loss: primary infection or reactivation of latent viruses? Otolaryngol Head Neck Surg 1981;89:137141.
Weber RS, Jenkins HA, Coker NJ. Sensorineural hearing loss associated with ulcerative colitis: a case report. Archives Oto
Laryngol 1984;110:810-812.
Wilkins SA, Jr., Mattox DE, Lyles A. Evaluation of a "shotgun" regimen for sudden hearing loss. Otolaryngol Head Neck
Surg 1987;97:474480.
Wilson WR. Why treat sudden hearing loss? Am J Otol 1984;5:481483.
Wilson WR, Byl FM, Larid N. The efficacy of steroids in the treatment of idiopathic sudden hearing loss: a double blind
clinical trial. Arch Otolaryngol 1980;106:772776.
Yoo TJ, Tomoda K, Stuart JM, Cremer MA, Townes AS, Kang AH. Type II collagen induced autoimmune sensorineural
Yoon TH, Paparella MM, Schachern PA, Alleva M. Histopathology of sudden hearing loss. Laryngoscope
1990;100:707715.


medical literature.
SUDDEN SENSORINEURAL HEARING LOSS

James O. Fordice, MD
November 18, 1993
Sudden sensorineural hearing loss (SHL) is one of the most perplexing and controversial unsolved
mysteries in otolaryngology. SHL has no universally accepted definition, but one useful definition is an
idiopathic hearing loss of sensorineural origin, greater than 30 dB in three contiguous frequencies that
occurs in less than three days. Most patients report onset of hearing loss within minutes to several hours.
About one-third of patients will awaken with the loss; others may discover the problem when they try to
use the telephone, or may describe a brief period of fluctuating hearing before the loss. About 50% of
patients complain of concomitant unsteadiness or vertigo.
Most studies find no seasonal, geographic, ethnic, racial or sexual predilection for SHL. The right and
left ears appear equally vulnerable. In a series of 225 cases, Byl in 1984 noted bilateral SHL in 2% of
patients. Reported overall incidence of SHL ranges from 5% to 20% per 100,000 persons per year. The
mean overall age for SHL is 46 years, according to a large series by Mattox and Simmons.
Sudden hearing loss is most likely a symptom rather than a discrete disease, merely representing the end
result of many insults to the inner ear. Known etiologies of sudden hearing loss include multiple
sclerosis, Cogan's syndrome, Buerger's disease, macroglobulinemia, leukemia, sickle cell disease,
polycythemia, syphilis, bacterial infection, mumps, ototoxic drugs, metastatic tumors, trauma, and lupus.
Ten percent of patients with Meniere's disease and up to 10% of acoustic neuroma patients will present
with sudden hearing loss. In patients with sudden hearing loss, all of these known etiologies should be
considered and ruled out before the most common "diagnosis" - idiopathic sudden hearing loss (SHL) - is
accepted. Among patients with SHL, four etiologic theories remain pre-eminent: viral, vascular,
membrane rupture, and auto-immune.
Viruses have long been suspected as etiologic agents in SHL. Wilson et al, in 1983, studied 122 patients
with SHL in the Boston area, and documented 63% viral seroconversion, compared with 40% of
controls. Conversion rates were statistically significantly higher for mumps, rubeola, varicella-zoster,
influenza, and CMV. Mumps virus was cultured from the perilymph of a patient with SHL by Westmore
in 1979, and CMV has been cultured from the perilymph of a congenitally infected infant. Davis, Davis
and Johnston, and Shimokata et al documented viral infection of the inner ear in animal models with
CMV, mumps, and rubeola, respectively. However, SHL has not successfully been induced in an animal
model using viruses. Schuknecht and Donovan in 1986 studied the temporal bone pathology in twelve
ears with SHL and reviewed the literature for ten others; comparing the histopathologic findings in these
SHL patients with findings from patients with known viral labyrinthitis, they found similar degenerative
changes: atrophy of the organ of Corti, tectorial membrane, and stria vascularis in most cases. They
concluded that viral cochleitis was the most probable cause of SHL.
Simmons in 1968 proposed a double membrane break theory for SHL. Goodhill et al in 1973 discovered
perilymph fistulas in three patients with sudden hearing loss, thus establishing round and oval window
breaks as accepted etiologies for sudden hearing loss. Intracochlear membrane breaks, with and without
round or oval window breaks, as a cause for SHL, have been poorly supported clinically and
histopathologically. Most patients with SHL do not provide a history suggestive of implosive or
explosive forces on the membranous labyrinth; Schuknecht and Donovan found no active or healed
membrane breaks in the twelve temporal bones they examined.
A vascular etiology for SHL is logically appealing, since sudden onset is suggestive of a vascular event.
Sudden hearing loss has been observed in patients with vascular or coagulatory defects. However,
histopathologic findings after permanent occlusion of the labyrinthine vasculature include necrosis of the
membranous labyrinth followed by ossification and fibrosis - these findings are not consistent with those
seen in most cases of SHL. Furthermore, temporary occlusion of the internal auditory artery was studied
by Perlman et al in 1959, and these workers found irreversible loss of cochlear function after about one
hour. They found that the hair cells, ganglion cells, and spiral limbus were the structures most frequently
affected. These histopathologic findings are at odds with those from SHL ears studied. The irreversibility
of the loss is also inconsistent with the reversibility of SHL in most cases. Finally, SHL typically strikes
relatively young patients without systemic manifestation of vascular disease. This population seems
unlikely to suffer isolated labyrinthine vascular compromise.
In recent years autoimmune ear disease has been added to the theoretical etiologic triad of vascular, viral,
and membrane rupture. Lehnhardt in 1958 first theorized that bilateral hearing disorders could result
from autoimmune reactions directed against inner ear antigens. McCabe in 1979 first proposed the
clinical entity of autoimmune sensorineural hearing loss. Yoo et al in 1983 induced autoimmune
sensorineural hearing loss in rats by immunization with type II collagen, and demonstrated the presence
of monoclonal antibody to type II collagen within the otic capsule. Harris in 1983 demonstrated the full
competence of an independent inner ear immune system, capable of producing its own antibodies. In
further work reported this year, Drs. Yamanobe and Harris isolated five autoantibodies to inner earspecific antigens in patients with idiopathic sensorineural hearing loss. No study has yet detected inner
ear-specific autoantibodies in SHL, and no study has proven the development of SHL in animal models
using such antibodies. The role of autoimmunity in SHL has yet to be clarified.
Given the lack of understanding of the etiology or etiologies of SHL, treatment regimens have of
necessity been empirical. One of the most commonly used treatments is vasodilator therapy. Many agents
have been tried, but one of the most popular has been carbogen, which is a gaseous mixture of 5% CO2
in 95% O2. Ugo Fisch in 1983 compared carbogen therapy with papaverine and low molecular weight
dextran therapy in SHL patients. He found no significant short term difference in hearing results, but
discovered significantly better hearing in carbogen-treated SHL patients after one year.
Wilson et al in 1980 reported a double-blinded study of 67 SHL patients treated with steroids or placebo,
and included an additional 52 controls who received no treatment. They found that patients with isolated
midfrequency losses recovered without regard to therapy, and that 76% of patients with losses greater
than 90 dB failed to recover regardless of therapy. However, in 74 patients with hearing loss less than 90
Db not limited to the midfrequencies, 78% treated with steroids improved, compared with 38% of
untreated patients. Subsequent studies have confirmed the benefit of steroids in selected patients. This is
the only controlled study showing conclusive superiority of any treatment for SHL over no treatment.
Other treatment regimens have included diuretics, anticoagulants, plasma expanders, and diatrizoate
meglumine, or Hypaque, an intravenous contrast dye. Controlled studies have not been performed to
show a benefit from any of them. Stellate ganglion block is an invasive technique used in SHL therapy.
Ganglion block causes vasodilation and subsequent increased blood flow through sympathetic blockade.
Haug et al from Houston reported in 1976 that 70% of 56 patients with SHL treated by a series of blocks
experienced greater than or equal to 10 dB pure tone average improvement and over 10% gain in speech
discrimination scores, versus only 20% of 20 patients treated by other means. Because this procedure
involves deep injection of anesthetic in the low neck, it carries obvious risk of lung injury and injury to
vital neural and vascular structures. Many centers, including Baylor, use a so-called "shotgun" approach
to SHL treatment, in an attempt to address all theoretical etiologies. Wilkins et al in 1987 evaluated one
exhaustive shotgun regimen of dextran, histamine, Hypaque, diuretics, steroids, vasodilators, and
carbogen, but found the results no different from those for no treatment.
It is generally agreed that spontaneous recovery is common in SHL, usually occurring within two weeks
of onset. Mattox and Simmons stated that 65% of all SHL patients will have spontaneous recovery of
functional hearing without treatment. Others have estimated that about one-third have spontaneous return
to normal hearing, and another one-third have return to functional hearing with a residual deficit.
Obviously, the ideal treatment protocol would improve on these numbers. Only steroid treatment, as
evaluated by Wilson et al, in 1980, has been proven superior to no treatment, and this success came only
in patients with moderate loss across many frequencies. Byl in 1984 reported a prospective study of 225
SHL patients. He found the most important factors influencing recovery to be severity of initial loss,
degree of vertigo, and time from onset to initial visit. All of these factors were inversely related to degree
of recovery. Other facts identified by Byl and others include patient age, shape of initial audiogram, and
ESR. Byl found that patients under 15 years of age and over 60 recovered less well than their
counterparts within these two age limits. Most researchers agree that SHL patients with upsloping and
midfrequency loss audiograms recover the best, while those with downsloping initial audiograms fare the
worst. Elevated ESR has been shown to be a negative prognostic sign. Tinnitus, present in 70% to 85%
of SHL patients, has no prognostic value.
Sudden sensorineural hearing loss remains a poorly understood and maddening problem for the
otolaryngologist. Prognostic predictors include audiogram shape, severity of loss, patient age, presence
of vestibular symptoms, and duration of symptoms. Viral and autoimmune mechanisms are currently the
best etiologic candidates, but the heterogeneity of patients and findings makes the discovery of a single
pathologic pathway for all SHL unlikely.
Case Presentation
A fifty-two-year-old Latin American man presented to Ben Taub General Hospital with a twelve hour
history of sudden hearing loss in the left ear. The patient had been watching television when he felt a pop
in his left ear, followed by tinnitus and subjective hearing loss. He denied dizziness, vertigo, nausea, or
vomiting, and gave no history of straining, trauma, or barotrauma. He also denied any history of ototoxic
drugs or otologic surgery. Hearing was subjectively normal bilaterally prior to this episode. Past medical
history was remarkable only for insulin-dependent diabetes mellitus, and social history was negative for
drinking, smoking, and drug use. Physical examination was unremarkable except for tuning fork tests
indicating a left sensorineural hearing loss. The patient had a negative fistula test and no spontaneous
nystagmus. Laboratory studies were all within normal limits, including an ESR of 2 and negative syphilis
serologies. Admission audiogram showed a pure tone average of 73 dB on the left and 8 dB on the right,
and speech understanding depressed to 32% on the left, normal on the right. Tympanograms were Type
A bilaterally. The patient was admitted and put to bedrest with a low salt diet. He underwent stellate
ganglion blocks times three, and was started on a prednisone taper from 80 mg and given carbogen
treatments. He experienced subjective improvement in his hearing on day three, and repeat audiogram on
day four showed pure tone average of 42 on the left, with speech discrimination of 60%. The patient was
discharged on day 5 on prednisone taper and low salt ADA diet, with light activity. Outpatient MRI of
the head was normal. At follow-up two months later, the patient reported subjective return of normal
hearing. Audiogram showed pure tone average of 12 dB on the left, 8 dB on the right, with 100% speech
discrimination bilaterally.
Bibliography
Alford BR, Shaver EF, Rosenberg JJ, Guildford FR. Physiologic and histopathologic effects of microembolization of the
internal auditory artery. Ann Otol Rhinol Laryngol 1965;74:728-748.
Anderson RG, Meyerhoff WI. Sudden sensorineural hearing loss. Otolaryngol Clin N Am 1983;16:189-195.
Beal DD, Hemenway WG, Lindsay JR. Inner ear pathology of sudden deafness: the histopathology of acquired deafness in
the adult coincident with viral infection. Arch Otolaryngol 1967;85:591-598.
Belal A, Jr. Pathology of vascular sensorineural hearing impairment. Laryngoscope 1980;90:1831-1839.
Bredenkamp JK, Shelton C. Sudden hearing loss: determining the specific cause and the most appropriate treatment.
Postgrad Med 1989;86:125-132.
Byl FM. Seventysix cases of presumed sudden hearing loss occurring in 1973: prognosis and incidence. Laryngoscope
1977;87:817-825.
Byl FM. Sudden hearing loss: eight years experience and suggested prognostic table. Laryngoscope 1984;94:647-661.
Cole RR, Jahrsdoerfer RA. Sudden hearing loss: an update. Am J Otol 1988;9:211-215.
DeKleyn A. Sudden complete or partial loss of function in the octavus system in apparently normal persons. Acta
Otolaryngol 1944;32:407-425.
Donaldson JA. Heparin therapy of sudden sensorineural hearing loss. Arch Otolaryngol 1979;105:351-352.
Fisch U. Management of sudden deafness. Otolaryngol Head Neck Surg 1983;91:38.
Fowler E. Sudden deafness. Ann Otol Rhinol Laryngol 1950;59:980-987.
Goodhill W. The "idiopathic group" and the "labyrinthine window rupture group" approaches to sudden sensorineural
hearing loss. In: Snow JB Jr, editor. Controversy in Otolaryngology. Philadelphia: Saunders, 1980:12-20.
Grandis JR, Hirsch BE, Wagener MM. Treatment of idiopathic sudden sensorineural hearing loss. Am J Otol 1993;14:1835.
Gussen R. Sudden hearing loss associated with vascular origins: a temporal bone study. Ann Otol Rhinol Laryngol
1976;85:94-100.
Hallberg O. Sudden deafness of obscure origin. Laryngoscope 1956;66:1237-1267.
Harris I. Sudden hearing loss: membrane rupture. Am J Otol 1984;5:484-7.
Harris JP. Immunology of the inner ear: response of the inner ear to antigen challenge. Otolaryngol Head Neck Surg
1983;91:18-23.
Harris JP. Experimental immunology of the inner ear. Adv Otorhinolaryngol 1991;46:26-33.
Harris JP. Autoimmunity of the inner ear. Am J Otol 1989;10:193-5.
Harris JP, Tomiyama S. Experimental immune system of the inner ear. J Otorhinolaryngol -Relat-Spec 1987;49:225-33.
Harris JP, Sharp PA. Inner ear autoantibodies in patients with rapidly progressive sensorineural hearing loss.
Haug O, Draper L, Haug SA. Stellate ganglion block for idiopathic sensorineural hearing loss. Arch Otolaryngol
1976;102:58.
Igarashi M, Alford BR, Konishi S, Shaver EF, Guilford FR. Functional and histopathological correlates after
microembolization of the peripheral labyrinthine artery in the dog. Laryngoscope 1969;79:602-623.
Igarashi M, Card G, Johnson PE, Alford BR. Bilateral sudden hearing loss and metastatic pancreatic adenocarcinoma.
Arch Otolaryngol 1979;105:196-199.
Jaffe BF. Sudden deafness: an otologic emergency. Arch Otolaryngol 1967;86:81-86.
Jaffe BF. Sudden deafness a local manifestation of systemic disorders: fat emboli, hypercoagulation, and infection.
Jaffe BF. Hypercoagulation and other causes of sudden hearing loss. Otolaryngol Clin North Am 1975;8:395-403.
Jaffe BF. Viral causes of sudden inner ear deafness. Otolaryngol Clin North Am 1978;10:63-9.
Jaffe BF. Clinical studies in sudden deafness. Adv Otorhinolaryngol 1978;20:221-228.
Jaffe BF, Maasab HF. Sudden deafness associated with adenovirus infection. N Engl J Med 1967;276:1406-1409.
Jenkins HA, Pollack AM, Fisch U. Polyarteritis nodosa as a cause of sudden deafness. Am J Otolaryngol 1981;2:99-107.
Jerger J, Allen G, Robertson D, Harford E. Hearing loss of sudden onset. Arch Otolaryngol 1961;73:350-357.
Kaplan SL, Catlin FI, Weaver T, Feigin RD. Onset of hearing loss in children associated with bacterial meningitis.
Pediatrics 1984;73:575-578.
Kronenberg J, Almagor M, Bendet E, Kushnir D. Vasoactive therapy versus placebo in the treatment of sudden hearing
loss: a double-blind clinical study. Laryngoscope 1992;102: 65-8.
Lipkin AF, Jenkins HA, Coker NJ. Migraine and sudden sensorineural hearing loss. Arch Otolaryngol Head Neck Surg
1987;113:325-326.
McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 1979;88:585-589.
McCabe BF. Autoimmune inner ear disease:therapy. Am J Otol 1989;10:196-7.
Mattox DE, Lyles CA. Idiopathic sudden sensorineural hearing loss. Am J Otol 1989;10:242-247.
Mattox DE, Simmons FB. Natural history of sudden sensorineural hearing loss. Ann Otol Rhinol Laryngol 1977;86:463480.
Meyerhoff WL. The management of sudden deafness. Laryngoscope 1979;89:1867-1868.
Meyerhoff WL, Paparella MM. Medical therapy of sudden hearing loss. In: Snow JB Jr, editor. Controversy in
Otolaryngology. Philadelphia: Saunders, 1980:311.
Nadol J, Wilson W. Treatment of sudden hearing loss is illogical. In: Snow JB Jr, editor. Controversy in Otolaryngology.
Philadelphia: Saunders, 1980:23-32.
Noury KA, Katsarkas A. Sudden unilateral sensorineural hearing loss: a syndrome or a symptom. J Otolaryngol
1989;18:274-278.
Ohlms LA, LonsburyMartin BL, Martin GK. Acousticdistortion products: separation of sensory from neural dysfunction in
sensorineural hearing loss in human beings and rabbits. Otolaryngol Head Neck Surg 1991;104:159-174.
Rowson K, Hinchicliffe R. A virological and epidemiological study of patients with acute hearing loss. Lancet 1975;1:471474.
Schuknecht HF, Benitez J, Beekhuis J, Igarashi M, Singleton G, Ruedi L. The pathology of sudden deafness.
Laryngoscope 1962;72:1142-1157.
Schuknecht HF, Donovan ED. The pathology of idiopathic sudden sensorineural hearing loss. Arch Otorhinolaryngol
1986;243:1-15.
Shaia FT, Sheehy JL. Sudden sensorineural hearing impairment: a report of 1220 cases. Laryngoscope 1976;86:389-398.
Shikowitz MJ. Sudden sensorineural hearing loss. Med Clin North Am 1991;75:1239-1250.
Siegel LG. The treatment of idiopathic sudden sensorineural hearing loss. Otolaryngol Clin N Am 1975;8:467-473.
Simmons FB. Theory of membrane breaks in sudden hearing loss. Arch Otolaryngol 1968;88:41-48.
Simmons FB. Fluid dynamics in sudden sensorineural hearing loss. Otolaryngol Clin North Am 1978;11:55-61.
Singleton G. Cervical sympathetic chain block in sudden deafness. Laryngoscope 1971;81:7-34.
Suga F, Preston J, Snow JB Jr. Experimental microembolization of cochlear vessels. Arch Otolaryngol 1970;92:213-220.
Suga F, Snow JB Jr. Cochlear blood flow in response to vasodilating drugs and some related agents. Laryngoscope
1969;79:1956-1979.
Van Dishoeck H, Bierman T. Sudden perceptive deafness and viral infection. Ann Otol Rhinol Laryngol 1957;66:963-980.
Veldman JE, Hanada T, Meeuwsen F. Diagnostic and therapeutic dilemmas in rapidly progressive sensorineural hearing
loss and sudden deafness. Acta Otolaryngol 1993; 113:303-6.
Veltri RW, Wilson WR, Sprinkle RM, Rodman SM, Kavesh DA. The implication of viruses in idiopathic sudden hearing
loss: primary infection or reactivation of latent viruses? Otolaryngol Head Neck Surg 1981;89:137-141.
Weber RS, Jenkins HA, Coker NJ. Sensorineural hearing loss associated with ulcerative colitis: a case report. Arch
Otolaryngol 1984;110:810-812.
Wilkins SA Jr, Mattox DE, Lyles A. Evaluation of a "shotgun" regimen for sudden hearing loss. Otolaryngol Head Neck
Surg 1987;97:474-480.
Wilson WR. Why treat sudden hearing loss? Am J Otol 1984;5:481-483.
Wilson WR, Byl FM, Larid N. The efficacy of steroids in the treatment of idiopathic sudden hearing loss: a double blind
clinical trial. Arch Otolaryngol 1980;106:772-776.
Yamanobe S, Harris JP. Inner ear-specific autoantibodies. Laryngoscope 1993;103:319-25.
Yoo TJ, Tomoda K, Stuart JM, Cremer MA, Townes AS, Kang AH. Type II collagen induced autoimmune sensorineural
Yoon TH, Paparella MM, Schachern PA, Alleva M. Histopathology of sudden hearing loss. Laryngoscope 1990;100:707715.

medical literature.
TEMPORAL BONE FRACTURES

Warren E. Morgan, MD
April 2, 1992
Temporal bone fractures may cause a variety of signs and symptoms. These include hearing loss, vertigo,
facial paralysis, cerebrospinal fluid (CSF) otorhinorrhea, tympanic membrane (TM) perforation,
hemotympanum, canal wall laceration, and Battle's sign. Adults and children are similarly affected but
several series have found that the complication rate is lower in children.
Fractures of the temporal bone are commonly classified based on the relationship of the fracture line to
the long axis of the temporal bone. Longitudinal fractures are the most common accounting for 80% of
the temporal bone fractures. This fracture extends along the length of the temporal bone. Transverse
fractures extend directly across the petrous bone and make up about 10% of temporal bone fractures.
Mixed fractures display some characteristics of each.
Longitudinal fractures usually present with classic findings of laceration of the ear canal, tympanic
membrane perforation, ossicular disruption, facial paralysis, and hearing loss. The hearing loss is
predominately conductive but may have a sensorineural component as well. Facial paralysis occurs in
about 10-20% of longitudinal fractures. These fractures result from trauma directed laterally over the
temporal area. Conversely, transverse fractures of the temporal bone show hearing loss, hemotympanum,
facial paralysis, and vertigo. Facial nerve paralysis occurs about 50% of the time. Transverse fractures
result from trauma to the anterior and posterior aspects of the head.
Recently a new classification system was proposed by Ghorayeb and Yeakley at The University of
Texas, Houston. They studied 150 temporal bone fractures radiographically using CT scan with threedimensional reconstruction. Based on their findings they proposed a new classification system. They
identified an oblique fracture type that had originally been described by Voss. In their review this was the
most common fracture type, occurring in 74.7% of the temporal bone fractures in this series. Other
fracture types encountered in this series include transverse, mixed, longitudinal, and fractures confined to
the petrous apex. They differentiate the oblique and longitudinal fractures based on the orientation of the
fracture line on the external aspect of the temporal bone. The oblique fracture crosses the external canal
in a horizontal plane and then extends upward obliquely toward the middle fossa. The fracture misses the
otic capsule and may extend toward the petrous apex where the fracture line may extend to the foramen
lacerum. Conversely, the longitudinal fracture line is oriented in a more vertical plane.
Histopathologic examination of temporal bone fractures reveals several patterns of injury. As previously
mentioned, longitudinal fractures extend anterior to the otic capsule and do not cause direct injury to the
otic capsule. In transverse fractures the otic capsule may be completely disrupted leading to complete
loss of audiovestibular function. Fractures of the otic capsule do not heal, but may have fibrous union
and new bone formation. This lack of healing may lead to infectious complications and the development
of labyrinthitis ossificans. This is an important consideration because of the potential for cochlear
implantation in patients with bilateral temporal bone fractures and profound deafness. Loss of
audiovestibular function results from concussion of the inner ear as well. This may occur in patients with
longitudinal fractures or may occur in patients without a temporal bone fracture. As demonstrated
experimentally in cats by Schuknecht, high frequency hearing loss may occur from the transfer of
vibratory energy to the cochlea. Lindsay described the changes noted in concussion and found the
predominate injury to be hemorrhage into the audiovestibular elements. It is thought that hemorrhage
induces a hyperplastic inflammatory response which may lead to degeneration of neural elements,
fibrosis, and eventual ossification.
The examination of a patient with suspected temporal bone fracture begins with a history and physical
exam. Often times the initial assessment occurs in the emergency room where the patient is also being
evaluated for other more life threatening injuries as well. History about the mechanism of injury should
be obtained along with any audiovestibular symptoms. The physical exam begins with a general
examination of the patient. The head and neck should be examined carefully, looking for the signs and
symptoms of temporal bone fractures. The facial nerve should be carefully examined and any evidence of
weakness documented. When the patient's condition improves audiogram and CT scan of the temporal
bones should be performed with further workup directed by any subsequent complications. These
complications include hearing loss, vertigo, facial nerve injury, and cerebrospinal fluid otorrhea.
Conductive and sensorineural hearing loss can result from temporal bone fractures. Classically,
conductive loss is associated with longitudinal fractures, and sensorineural loss with transverse fractures.
But each may have components of both. The etiology of the conductive hearing loss may result from
tympanic membrane perforation, hemotympanum, and ossicular disruption. Persistent conductive hearing
loss suggests an ossicular disruption. Middle ear injury with ossicular derangement was initially reported
at the time of autopsy by Keleman, and was noted by Thornburn in 1956. Shortly thereafter Hough
described this
as well, and later published his experience with 31 patients. He found that the most commonly damaged
ossicle is the incus. This is thought to result from its relative lack of support compared to the other
ossicles; and its location between the other two ossicles, which subjects it to torsional forces. Treatment
of these injuries requires middle ear exploration and appropriate ossicular reconstruction.
Sensorineural hearing loss may result from disruption of the membranous labyrinth or concussion.
Treatment options for traumatic sensorineural hearing loss are limited. For the majority of patients the
only option is rehabilitative. This consists of hearing aids and cochlear implantation for patients with
profound bilateral hearing loss.
Another sequelae of temporal bone fractures is vertigo. The incidence varies between different studies
with a incidence of 78% by Tuohimaa, while Griffiths reports only 24%. The mechanism of the vertigo
may vary and can result from a variety of causes which may be central or peripheral. Central causes are
related to injury to the brainstem and result from dysfunction of the vestibular nuclei. There are a number
of peripheral causes, the most common being benign positional vertigo. Patients that have associated
vertigo with a fluctuating hearing loss should be suspected of having a perilymph fistula. Disruption of
the labyrinth may lead to a unilateral weakness that causes vertigo. Another cause of vertigo that has
been reported is endolymphatic hydrops. Rivzi describes a patient who sustained a transverse temporal
bone fracture that left the otic capsule intact. This patient developed Meniere's-type symptoms and
several months later died of unrelated causes. On examination of the temporal bones the fracture line was
noted to cross the vestibular aqueduct. Since the majority of these patients' symptoms resolve within 12
months, initial treatment is conservative. Patients who have persistent incapacitating symptoms may
require vestibular nerve section or labyrinthectomy.
Temporal bone fractures may result in facial nerve paralysis. Approximately 20% of longitudinal
fractures and 50% of transverse fractures result in a facial palsy. This derives from edema, intraneural
hemorrhage, bony fragment impingement and dehiscence of the nerve. In a review of 15 cases of
longitudinal temporal bone fractures at Baylor, Coker et al found the perigeniculate region to be the most
common site of injury. This injury occurred there about 93.3% of the time. Surgical findings noted neural
edema present in 93%, intraneural hemorrhage in 40%, bony fragment impingement in 33%, and one
patient with dehiscent geniculate ganglion and dehiscent proximal tympanic segment. In this same
review, three patients with transverse temporal bone fractures underwent surgical exploration and they
were noted to have injury to the labyrinthine and tympanic segments of the facial nerve.
The evaluation of patients' facial paralysis from temporal bone fractures begins with early diagnosis.
Patients with complete paralysis should be followed up with electrical testing. Several tests are available,
with nerve excitability test, ENOG, and EMG being the most commonly used. Audiogram is also
important since hearing status influences the choice of surgical approach. Initial treatment is
conservative, consisting of eye protection and steroids. Facial nerve exploration and decompression is
indicated for patients who show significant neural degeneration. For patients with serviceable hearing,
decompression by a middle cranial fossa/transmastoid approach is best. For those without hearing a
translabyrinthine/transmastoid approach is used.
CSF otorrhea may result from temporal bone fractures, although the incidence is relatively low. In a
review of 1,185 patients with skull fractures Raaf found that 3.6% presented with CSF otorrhea. Of these,
97% closed spontaneously. Of the patients with otorrhea 7.8% developed meningitis, with one patient
dying. Patients with longitudinal fractures usually develop CSF leaks from the tegmen where the fracture
line perforates the dura. Transverse fractures are more likely to cross the vestibule and leakage may
occur through the defect. Also, since the TM may be intact they are more likely to present with CSF
otorhinorrhea. In most cases identification of the fluid is obvious. In questionable cases the most specific
test available is electrophoretic identification of the B2 fraction of transferrin, which is specific for CSF.
For persistent leaks localization may be attempted with CT scan with metrizamide.
Since the majority of these leaks close spontaneously management is conservative. Several studies have
failed to show any benefit from prophylactic antibiotics, and, while it remains controversial, they are
probably not indicated. Initial management includes elevating the head and avoiding elevations of
intracranial pressure. If this fails to resolve the leak the next step in management is insertion of lumbar
drain. Leaks that persist, despite these measures, require surgical closure. While the period of waiting is
controversial, surgical closure should be considered for leaks that have persisted for two weeks despite
conservative therapy. The surgical approach is based on the suspected location and status of hearing. If
the patient has good hearing and the defect is suspected in the tegmen, a middle fossa approach may be
used. For patients with no hearing, obliteration via a translabyrinthine approach may be used.
In a review of 82 temporal bone fractures in 75 patients Ghorayeb et al presented several other more
unusual complications of temporal bone fractures. These include abducens nerve palsy which may be
bilateral or unilateral, trigeminal paralysis, and aseptic sigmoid sinus thrombosis. In their review the
incidence of abducens palsy was 6.67%. The abducens nerve has the longest intracranial course of any
cranial nerve and is intimately associated with the facial nerve and the trigeminal nerve. It appears the
nerve is most vulnerable as it enters Dorello's canal at the end of the petrous apex. Because of the
complex anatomy of this nerve the exact mechanism of injury is unclear and may result from stretching
and contusion alone. Patients present with an inability to abduct the eye. Many of these injuries are
partial and may recover. Treatment consists of alternately patching one eye and if the injury fails to
recover, corrective surgery may be attempted.
Ghorayeb et al found an incidence of trigeminal paralysis in 2.67% of the cases. This injury also occurs
near the end of the petrous apex where the trigeminal ganglion sits in Meckel's Cave. Patients present
with hypesthesias and paresthesia in the trigeminal distribution. They may also have weakness of the
muscles of mastication. No therapy is available for this injury.
Sigmoid sinus thrombosis was found in one patient in this series. Patients may be asymptomatic or
symptoms of increased intracranial pressure may occur. These include headache and retinal changes. The
diagnosis is made with CT scan which shows occlusion of the sigmoid sinus with soft tissue. Clinically
the diagnosis is made with the Tobey-Ayer Test, which consists of measuring changes in the CSF
pressure with compression and release of the jugular veins. For septic sigmoid thrombophlebitis the
treatment of choice is exploration of the sinus via a mastoid approach. For aseptic sigmoid sinus
thrombosis Ghorayeb et al caution against exploration in debilitated patients, but advocate exploration as
part of any other ear procedure. The patient presented in this review underwent facial nerve
decompression and at that time the sinus was explored.
Another rare complication of temporal bone fractures is cholesteatoma formation. This occurs in
fractures that involve the external canal. With the disruption of the bony external canal small amounts of
epithelium may be trapped. Many years later these patients may present with large and extensive
cholesteatomas.
Due to the complex anatomy, temporal bone fractures may cause a variety of disorders. Often the
patients have sustained other more life threatening injuries that take precedence over the evaluation of
temporal bone fracture. Early identification of the fracture, along with careful observation in the recovery
period, is necessary for proper treatment.
Case Presentation
A 21-year-old black male presented to the Emergency Room after suffering blunt trauma to the head. He
reportedly was hit with a vase in the right temporal area and lost consciousness at the scene. He
complained of decreased hearing and bleeding from the right ear, but denied any vertigo. Physical exam
of the right ear revealed a posterior external canal wall laceration and a hemotympanum. The facial nerve
was intact. CT scan of the head and temporal bone was obtained which revealed a longitudinal fracture of
the right temporal bone. He was admitted to the Neurosurgery Service for observation. On the second
day of hospitalization he developed a complete right facial paralysis and he was transferred to the
Otolaryngology Service. Audiogram showed a mild conductive hearing loss in the right ear, and acoustic
reflexes showed a probe effect in the right ear. He was started on steroids and received local care for the
right eye. He was followed daily with nerve excitability tests with the side to side difference remaining
less than 2mA. On the seventh day after the onset of the paralysis, nerve excitability testing revealed a
greater than 3.5 mA difference between sides. ENOG revealed greater than 95% degeneration of the
facial nerve. He subsequently underwent a combined middle cranial fossa/transmastoid exploration and
decompression of the facial nerve. At the time of surgery a bony spicule was found impinging on the
facial nerve at the geniculate ganglion. The facial nerve was intact but edematous in the labyrinthine and
tympanic segments. The postoperative course was uneventful and the patient was discharged to home on
the sixth postoperative day.
Bibliography
Aguilar EA, III, Yeakley JW, Ghorayeb BY, Hauser M, Cabrera J, Jahrsdoerfer RA. High resolution CT scan of temporal
bone fractures: association of facial nerve paralysis with temporal bone fractures. Head Neck Surg 1987;9:162-166.
Alford BR, Sessions RB, Weber SC. Indications for surgical decompression of the facial nerve. Laryngoscope
1971;81:620-635.
Babin RW. Topognostic and prognostic evaluation of traumatic facial nerve injuries. Otolaryngol Head Neck Surg
1982;90:610-611.
Barber HO. Head injury: audiological and vestibular findings. Ann Otol Rhinol Laryngol 1969;78:239-252.
Battle WH. Three lectures on some points relating to injuries to the head. Lancet 1890;1:57-63.
Bellucci RJ. Traumatic injuries of the middle ear. Otolaryngol Clin North Am 1983;16:633-650.
Benitez JT, Bouchard KR, LaneSzopo D. Pathology of deafness and disequilibrium in head injury: a human temporal bone
study. Am J Otol 1980;1:163-167.
Bottrill ID. Posttraumatic cholesteatoma. J Laryngol Otol 1991;105:367-369.
Brodsky L, Eviatar A, Daniller A. Posttraumatic facial nerve paralysis: three cases of delayed temporal bone exploration
with recovery. Laryngoscope 1983;93:1560-1565.
Brookes GB, Graham MD. Posttraumatic cholesteatoma of the external auditory canal. Laryngoscope 1984;94:667-670.
Cannon CR, Jahrsdoerfer RA. Temporal bone fractures. Review of 90 cases. Arch Otolaryngol 1983;109:285-288.
Coker NJ. Management of traumatic injuries to the facial nerve. Otolaryngol Clin North Am 1991;24:215-227.
Coker NJ, Kendall KA, Jenkins HA, Alford BR. Traumatic intratemporal facial nerve injury: management rationale for
preservation of function. Otolaryngol Head Neck Surg 1987;97:262-269.
Eby TL, Pollak A, Fisch U. Histopathology of the facial nerve after longitudinal temporal bone fracture. Laryngoscope
1988;98:717-720.
Eby TL, Pollak A, Fisch U. Intratemporal facial nerve anastomosis: a temporal bone study. Laryngoscope 1990;100:623626.
Fisch U. Current surgical treatment of intratemporal facial palsy. Clin Plast Surg 1979;6:377-388.
Fisch U. Management of intratemporal facial nerve injuries. J Laryngol Otol 1980;94:129-134.
Fredrickson JM, Griffith AW, Lindsay JR. Transverse fracture of the temporal bone: a clinical and histopathologic study.
Freeman J. Temporal bone fractures and cholesteatoma. Ann Otol Rhinol Laryngol 1983;92:558-560.
Ghorayeb BY, Yeakley JW, Hall JW, III, Jones BE. Unusual complications of temporal bone fractures. Arch Otolaryngol
Head Neck Surg 1987;113:749-753.
Glasscock ME, III, House WF, Alford BR. Middle fossa facial nerve decompression. Ann Otol Rhinol Laryngol
1970;79:234-240.
Glasscock ME, III, Wiet RJ, Jackson CG, Dickins JR. Rehabilitation of the face following traumatic injury to the facial
nerve. Laryngoscope 1979;89:1389-1404.
Goodwin WJ, Jr. Temporal bone fractures. Otolaryngol Clin North Am 1983;16:651-659.
Griffin JE, Altenau MM, Schaefer SD. Bilateral longitudinal temporal bone fractures: a retrospective review of seventeen
cases. Laryngoscope 1979;89:1432-1435.
Grobman LR, Pollak A, Fisch U. Entrapment injury of the facial nerve resulting from longitudinal fracture of the temporal
bone. Otolaryngol Head Neck Surg 1989;101:404-408.
Hallpike CS. On a case of deafmutism of traumatic origin. J Laryngol Otol 1937;52:661-674.
Harris JP, Butler D. Recognition of malleus handle fracture in the differential diagnosis of otologic trauma. Laryngoscope
1985;95:665-670.
Hicks GW, Wright JW, Jr., Wright JW, III. Cerebrospinal fluid otorrhea. Laryngoscope 1980;90:125.
Hough JV. Restoration of hearing loss after head trauma. Ann Otol Rhinol Laryngol 1969;78:210-226.
Hough JV. Surgical aspects of temporal bone fractures. Proc R Soc Med 1970;63:245-252.
Hough JVD, Stuart WD. Middle ear injuries in skull trauma. Laryngoscope 1968;78:899-937.
Kamerer DB. Intratemporal facial nerve injuries. Otolaryngol Head Neck Surg 1982;90:612-615.
Kelemen G. Fractures of the temporal bone. Arch Otolaryngol Head Neck Surg 1944;40:333-373.
Khan AA, Marion M, Hinojosa R. Temporal bone fractures: a histopathologic study. Otolaryngol Head Neck Surg
1985;93:177-186.
KoefoedNielsen B, Tos M. Posttraumatic sensorineural hearing loss: a prospective long-term study. ORL J
Kveton JF. Obliteration of mastoid and middle ear for severe trauma to the temporal bone. Laryngoscope 1987;97:13851387.
Lambert PR, Brackmann DE. Facial paralysis in longitudinal temporal bone fractures: a review of 26 cases. Laryngoscope
1984;94:1022-1026.
Lindsay JR, Zajtchuk J. Concussion of the inner ear. Ann Otol Rhinol Laryngol 1970;79:699-709.
May M. Trauma to the facial nerve. Otolaryngol Clin North Am 1983;16:661-670.
Millen SJ. Excellent spontaneous recovery following temporal bone fracture with loss of nerve excitability. Am J Otol
1991;12:235-236.
Millen SJ. Spontaneous recovery following temporal bone fracture in spite of loss of nerve excitability. Am J Otol
1990;11:459-460.
Perlman HG. Process of healing in injuries to the capsule of the labyrinth. Arch Otolaryngol Head Neck Surg 1939;29:287305.
Podoshin L, Pradis M. Hearing loss after head injury. Arch Otolaryngol Head Neck Surg 1975;101:1518.
Rizvi SS, Gibbin KP. Effect of transverse temporal bone fracture on the fluid compartment of the inner ear. Ann Otol
Rohrt T. Fracture of temporal bone, early or retrospective diagnosis, and surgical hearing reconstruction. Acta Otolaryngol
1973;75:355-356.
Schneider RC, Boles R. Combined neurootologic and neurosurgical problems in head trauma. Laryngoscope 1968;78:955972.
Schuknecht HF. A clinical study of auditory damage following trauma to the head. Ann Otolaryngol Chir Cervicofac
1950;59:331-358.
Schuknecht HF. Mechanism of inner ear injury from blows to the head. Ann Otol Rhinol Laryngol 1969;78:253-262.
Schuknecht HF, Neff WD, Perlman HB. An experimental study of auditory damage following blows to the head. Ann Otol
Shapiro RS. Temporal bone fractures in children. Otolaryngol Head Neck Surg 1979;87:323-329.
Vartiainen E, Karjalainen S, Karja J. Auditory disorders following head injury in children. Acta Otolaryngol 1985;99:529536.
Ward PH. The histopathology of auditory and vestibular disorders in head trauma. Ann Otol Rhinol Laryngol 1969;78:227238.
Wennmo C, Svensson C. Temporal bone fractures. Vestibular and other related ear sequelae. Acta Otolaryngol Suppl
1989;468:379-383.
Wiet RJ, Valvassori GE, Kotsanis CA, Parahy C. Temporal bone fractures. State of the art review. Am J Otol 1985;6:207215.
Yanagihara N. Transmastoid decompression of the facial nerve in temporal bone fracture. Otolaryngol Head Neck Surg
1982;90:616-621.


medical literature.
TEMPORAL BONE GUNSHOT WOUNDS:

EVALUATION AND MANAGEMENT
Douglas D. Backous, MD
August 5, 1993
With the significant increase in handgun availability since the 1960s, treatment of gunshot injuries is no
longer limited to major trauma centers. Five thousand Americans under the age of 20 die annually from
gunshot wounds in the United States alone, with many more victims surviving with significant injury.
The easy availability of guns is seen in rural as well as in metropolitan areas across the country.
Wound ballistics is the study of the effects of missiles striking tissues. Kinetic energy is directly
proportional to the mass of the projectile and proportional to the square of the velocity at which the bullet
is traveling. The most efficient missile enters tissues and imparts the majority of its kinetic energy to
surrounding structures without exiting the victim. Traditionally, gunshot injuries could be classified as
civilian or military. Civilian injuries were inflicted with low velocity weapons (90-210 m/sec) while
military wounds typically were caused by high velocity weapons (610 m/sec and up). Due to medium to
high velocity weapons appearing on the streets, more civilians are suffering from military-type injuries.
Gunshot injuries to the temporal bone affect intracranial, vascular, middle or inner ear, cranial nerve, and
external canal structures. Being the toughest bone in the body, the temporal bone deflects missles, often
resulting in hidden injuries. Early intracranial injuries include skull fractures, subdural hematoma,
foreign bodies, pneumocephalus and cerebrospinal fluid leaks. Temporal lobe abscess, meningitis,
aphasia, hemianopsia, and hemiplegia are late intracranial complications. Venous injuries to the
transverse and sigmoid sinuses, jugular system and cavernous sinus have been reported. Acute
transection of internal or external carotid arteries is usually obvious while pseudoaneurysms and carotidcavernous fistulae can develop later during the recovery period. Tympanic membrane perforations,
ossicular disruption, labyrinthine destruction, and mixed longitudinal and transverse temporal bone
fractures are the most commonly associated middle ear injuries. The majority of victims suffer acoustic
injury inflicted either by direct damage from the bullet path or, more commonly, from blast effects. Fifty
percent of victims will have facial nerve involvement with transections by far outweighing contusions.
The vagus, hypoglossal, and abducens nerves are the next most frequently damaged cranial nerves. Bony
and cartilaginous fractures, cicatricial scarring, temporomandibular joint displacement, canal
cholesteatoma, and chronic bony infections can occur after gunshot injury involving the external auditory
canal.
Initial assessment includes a standard trauma workup with attention directed to life-threatening problems
first. General and neurosurgical clearance is mandatory prior to thorough head and neck and otologic
examination. Evaluation of the ear should be done with sterile instruments to avoid further soft tissue
contamination. Computed tomography of the temporal bones is ordered once the patient is stable. Carotid
and vertebral arteriograms should be obtained at the slightest suspicion of potential vascular
involvement. Venous phases of these studies are important. Audiometric testing documents hearing at
baseline and prior to any surgical manipulation of the otic structures and aids in selecting approaches to
the middle ear and facial nerve. Vestibular testing may be obtained to document the nature and location
of lesions in symptomatic patients and is useful to follow vestibular compensation.
Patients are first stabilized from life-threatening injuries. Acute care for intracranial injuries includes
intravenous steroids and osmotic diuretics. Antibiotic prophylaxis is key, especially if dural injury is
suspected. Lumbar drainage and craniotomy should be employed as indicated. Embolization, packing at
the skull base, and vessel ligation in the neck are options for vascular injuries. Mastoidectomy and
tympanoplasty should be considered whenever remaining anatomy permits and residual hearing is
documented. Debridement of the external auditory canal, labyrinthectomy and temporal bone obliteration
should be used depending on the extent of destruction within the temporal bone and depending on the
status of remaining critical structures.
Presentation with a complete facial nerve paralysis signals a transection and should be managed
aggressively. Exploration of the wound to localize damaged nerve endings is best done before 72 hours
when distal nerve segments have not undergone degeneration. Exploration is often delayed while other
injuries are treated. Exploration is then best completed in the subsequent 6 to 12 months. The best results
are accomplished with end-to-end anastomosis. Due to local destruction from blast effects, end-to-end
anastomosis is frequently not feasible mandating interposition grafting techniques. The great auricular
nerve provides 5 cm to 7 cm of graft material while the sural nerve affords up to 30 cm of donor tissue.
Delayed loss of facial nerve function most likely results from nerve edema or contusion and
decompression suffices as definitive operative management. House-Brackmann grade 4 recovery or
worse is expected with interposition grafting while up to grade 3 recovery has been documented from
decompressions. Local eye care is mandatory to avoid exposure keratitis while nerve recovery
progresses. Other cranial nerve injuries are managed symptomatically. Long-term follow-up is required
since many of these patients will need secondary facial reanimation procedures.
In conclusion, multispecialty assessment is required for patients with gunshot wounds involving the
temporal bone. Life-threatening injuries are addressed first, followed by a thorough head and neck
workup. Care must be taken to avoid missing hidden injuries induced secondary to blast effects or due to
deflection of the missile off of the otic capsule. Indications for surgical exploration of the temporal bone
include facial nerve paralysis, vascular injury, cerebrospinal leakage, chronic otitis media, conductive
hearing loss and to debride necrotic tissue.
Case Presentations
A 44-year-old black male presented to the shock room at Ben Taub General Hospital with a single
gunshot wound to the left preauricular area. On admission, he was awake and alert, with a complete left
facial nerve paralysis. He had significant tissue loss on the left side of his face and in the left tonsillar
area. After airway stabilization by endotracheal intubation, emergent computerized tomographic
scanning of the head was negative for intracranial pathology and four vessel arteriograms showed no
vascular injury. A barium swallow revealed no extravasation into the pharynx or esophagus. He was
extubated after 48 hours. The left external auditory canal was remarkable for bloody discharge and
squamous debris, with the Weber lateralizing to the right. The remaining cranial nerves were intact. No
other injuries were found after thorough physical examination. The patient had swallowed the bullet that
was originally presumed to be resting in his posterior oropharynx. Temporal bone CT scans revealed a
comminuted left mastoid tip and fractured external auditory canal and temporomandibular joint.
Audiogram showed no responses in the left ear and a moderate sensorineural loss on the right. No
response could be elicited by nerve excitability testing on the left side. The patient was discharged after
11 days in the hospital. He was readmitted seven days later, at which time he underwent left radical
mastoidectomy, complete facial nerve decompression, sural nerve grafting and meatoplasty. The patient
did well postoperatively and has been followed regularly in clinic. He has a House-Brackmann grade VI
recovery and is now, at two years postrepair, being considered for a secondary procedure to improve his
facial tone and to gain left eye closure.
Bibliography
Brookes GB. Post-traumatic cholesteatoma. Clin Otolaryngol 1983;8:31-38.
Byrnes DP, Crockard HA, Gordon DS, Gleadhill CA. Penetrating craniocerebral missile injuries in the civil disturbances
in Northern Ireland. Br J Surg 1974;61:169-176.
Coker NJ. Management of traumatic injuries to the facial nerve. Otolaryngol Clin North Am 1991;24:215-227.
Coker NJ, Jenkins HA. Traumatic injury to the facial nerve. Exhibit Handout 1990.
Coker NJ, Kendall KA, Jenkins HA, Alford BR. Traumatic intratemporal facial nerve injury: management and rationale
for preservation of function. Otolaryngol Head Neck Surg 1987;97:262-269.
Cushing H. A study of a series of wounds involving the brain and its enclosing structures. Br J Surg 1918;5:558-684.
du Trevou M, Teasdale RB, Quin RO. False aneurysms of the carotid tree due to unsuspected penetrating injury of the
head and neck. Br J Accident Surg 1991;22:237-239.
Duncan NO, Coker NJ, Jenkins HA, Canalis RF. Gunshot injuries to the temporal bone. Otolaryngol Head Neck Surg
1986;94:4755.
Hagan WE, Tabb HG, Cox RH, Travis LW. Gunshot injuries to the temporal bone: an analysis of thirtyfive cases.
Laryngoscope 1979;89:1258-1272.
Holt GR, Kostohryz G. Wound ballistics of gunshot injuries to the head and neck. Arch Otolaryngol 1983;109:313-318.
House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg 1988;93:146-147.
Kerr AG. Gunshot injury of the temporal bone a histological report. J Irish Med Assn 1967;110(366):446-448.
Kinney SE. Trauma to the middle ear and temporal bone. In: Cummings CW, Fredrickson JM, Harker LA, Krause CJ,
Schuller DE, editors. Otolaryngology Head and Neck Surgery. 2nd ed. St. Louis: Mosby, 1993:2873-2884.
Mahmoud NA. Traumatic aneurysm of the internal carotid artery and epistaxis. J Laryngol Otol 1979;93:629-656.
Singh SP, Adeloye A. Hearing loss in missile head injuries. J Laryngol Otol 1971;85:1183-1187.


medical literature.
TUBERCULOUS OTITIS MEDIA

Troy Callender, MD
November 12, 1992
Despite tremendous advances in the treatment and prevention of tuberculosis (TB) it remains one of the
most common, lethal infectious diseases in the United States. Worldwide, TB is the single most important
bacterial infection, with more than 7 million active cases. Fortunately the ear is rarely infected by
mycobacterium tuberculosis; current incidence is estimated to be between 0.9% and 0.04%. The tubercle
bacillus can spread to the middle ear by several routes. The most common route is hematogenous. Other
routes of infection include regurgitation of the tubercle bacillus through the eustachian tube, through
previously existing tympanic membrane perforations; and by direct extension from a nasopharyngeal site
of infection. TB can also be transmitted congenitally and is associated with a high incidence of ear
involvement. However, congenital TB is extremely rare and hardly ever presents with isolated ear
involvement.
Classically, tuberculous otitis media is described as having an insidious onset with painless otorrhea,
multiple tympanic membrane perforations, abundant pale granulation tissue in the middle ear, early
severe hearing loss out of proportion to clinical findings, and bone necrosis. However, the current
literature indicates most patients actually present with a single perforation. Indications of tuberculous
otitis media include hearing loss out of proportion to clinical findings, failure to respond to the usual
medical therapy, post-mastoidectomy recurrence of granulation tissue, slow wound healing, persistent
otorrhea, and the formation of bony sequestra.
The evaluation of these patients should include a history of contact to active tuberculosis, placement of a
PPD skin test with control, culture and stain of ear drainage for AFB, biopsy of granulation tissue for
histology and culture, and evaluation of the immune system if indicated. All patients should be evaluated
with a chest x-ray, urinalysis, sputum for AFB culture and smear, gastric aspirates in children, and lumbar
puncture if CNS involvement is suspected or if the patient has miliary TB. On examination of the ear, the
tympanic membrane will appear dull and thickened with dilated vessels on the surface early and will later
develop perforations from liquification of caseous tubercles. There will also be abundant granulation
tissue in the middle ear. Late complications include facial paralysis, labyrinthitis, postauricular fistulae,
subperiosteal abscess, petrous apicitis, and intracranial extension of infection. Radiologic evaluation of
the temporal bone cannot differentiate TB from non-specific infections. A well-pneumatized mastoid
with chronic otitis media is suggestive of tuberculous otitis media but not diagnostic, as these cases can
also have sclerotic and destructive mastoid lesions.
The differential diagnosis of tuberculous otitis media includes fungal infections, Wegener's
granulomatosis, midline granuloma, sarcoidosis, syphilis, necrotizing otitis externa, atypical
mycobacterial infections, and histiocytosis X.
The treatment of tuberculous otitis media is primarily medical and should include a 6- to 9-month course
of Isoniazid, Rifampin, and Pyrazinamide. Indications for surgical intervention include the late
complications of tuberculous otitis media; cases unresponsive to medical therapy; extensive disease with
bone sequestra or necrotic bone; and reconstruction of the tympanic membrane and ossicular chain after
the middle ear disease has been eradicated.
Case Presentation
A 10-month-old black female, developed persistent otitis media at 4 months, which failed to respond to
multiple courses of antibiotics. On referral to the Pediatric Otolaryngology Service, examination was
remarkable for bilateral dull, thickened, erythematous tympanic membranes with poor mobility on
insufflation. Myringotomy on June 22, 1992 revealed granulation tissue filling the middle ear cleft
bilaterally. Post-operatively she was improved but had persistent scant otorrhea in spite of outpatient
medical therapy. A CT scan of the temporal bones was obtained and showed opacification but no
destructive lesions. The patient was returned to the OR on September 23, 1992. Middle ear tissue was
obtained for routine AFB, fungal cultures, and histologic examination, and her ventilation tubes were
replaced. She was admitted and a pediatric infectious disease consultation was obtained.
Additional historical data obtained at that time revealed the patient to have had close contact with a
relative with active tuberculosis. Complete evaluation during this admission was remarkable for elevated
total IgG and IgG1, + ELISA HIV with an indeterminate western blot, miliary CXR pattern consistent
with TB, CSF analysis showing 62 WBC's, 2 RBC's, 82% monos, a glucose of 36 mg/dl and protein of 38
mg/dl consistent with tuberculous meningitis. A CT scan of the brain revealed tuberculomas in the
posterior fossa and a PPD skin test was positive with 19 mm to 20 mm of induration. ABR was
compatible with a mild conductive loss.
Treatment was instituted with isoniazid, rifampin, pyrazinamide, and kanamycin. She was also treated
with oral prednisone. She had a dramatic otologic response with resolution of granulation tissue over 2 to
3 weeks. Except for occasional fever she was otherwise well. She was kept in the hospital for 4 weeks to
insure compliant therapy and is being closely followed as an outpatient. Final cultures at eight weeks
show no AFB growth.
Bibliography
Austin WK, Lockey MW. Mycobacterium fortuitum mastoiditis. Arch Otolaryngol 1976;102:558-560.
Bate TWP, Sinclair RE, Robinson MJ. Neonatal tuberculosis. Arch Dis Child 1986;61:738-740.
Cunningham MJ, Curtin HD, Jaffe R, Stool SE. Otologic manifestations of Langerhans' cell histiocytosis. Arch
Davidson S, Creter D, Leventon G, Katznelson K. Tuberculosis of the middle ear in an infant. Arch Otolaryngol Head
Neck Surg 1989;115:876-877.
Glover SC, Tranter RMD, Innes JA. Tuberculous otitis media - a reminder. J Laryngol Otol 1981;95:1261-1264.
Grabscheid E. Tuberculosis of the petrous apex. Report of a case. Arch Otolaryngol 1937;25:11-16.
Harbert F, Riordan D. Tuberculosis of the middle ear. Laryngoscope 1984;74:198-204.
Jeang TK, Fletcher EC. Tuberculosis otitis media. JAMA 1983;249:2231-2232.
Kinsella JP, Grossman M, Black S. Otomastoiditis caused by Mycobacterium avium-intracellulare. Pediatr Infect Dis
1986;5:704-706.
Lederer E. Diseases of the Ear, Nose and Throat. Philadelphia: Davis, 1953:216-223.
Li CK, Chan Y-F, Har CMY. Congenital tuberculosis. Aust Paediatr J 1989;25:366-3677.
Lowry WP, Jarvis WR, Oberle AD, Bland LA, Silberman R, Bocchini JA Jr, et al. Mycobacterium chelonae causing otitis
media in an ear-nose-and-throat practice. New Engl J Med 1988;319:978-982.
Lucente FE, Tobias GW, Parisier SC, Som PM. Tuberculous otitis media. Laryngoscope 1987;88:1107-1115.
Lyons AS, Petrocelli RJ. Medicine. An Illustrated History. NY: Abradale Press, 1978:559,590.
Ma KH, Tang PSO, Chan KW. Aural tuberculosis. Am J Otol 1990;11:174-177.
Munzel MA. Tympanoplasty and tuberculosis of the middle ear. Clin Otolaryngol 1978;3:311-313.
Neitch SM, Bydnor JB, Schleupner CJ. Mycobacterium fortuitum as a cause of mastoiditis and wound infection. Arch
Palva T, Palva A, Krj J. Tuberculous otitis media. J Laryngol Otol 1973;87:253-261.
Plester D, Pusalkar A, Steinbach E. Middle ear tuberculosis. J Laryngol Otol 1980;94:1415-1421.
Ramages LJ, Gertler R. Aural tuberculosis: a series of 25 patients. J Laryngol Otol 1985;99:1073-1080.
Sahn SA, Davidson PT. Mycobacterium tuberculosis infection of the middle ear. Chest 1974;66:104-106.
Scully RE, Mark EJ, McNeely WF, McNeely BU. Case records of the Massachusetts General Hospital. Case 21-1991. New
Engl J Med 1991;324:1489-1495.
Shambaugh GE, Glasscock ME. Surgery of the Ear, 3rd edition. Philadelphia: WB Saunders, 1980:208-210.
Slonik PR, Nadol JB Jr, Baker AS. Tuberculosis of the middle ear: review of the literature with an instructive case report.
Rev Infect Dis 1986;8:403-461.
Snider DE, Rieder HL, Combs D, Bloch AB, Hayden CH, Smith MHD. Tuberculosis in children. Pediatr Infect Dis J
1988;7:271-278.
Summary of notifiable diseases, United States 1991. MMWR 1991;40(53):3-63.
Turner AL, Fraser JS. Tuberculosis of the middle ear cleft in children: a clinical and pathological study. J Laryngol Otol
1915;6:207-247.
Vomero E, Ratner SJ. Diagnosis of miliary tuberculosis by examination of middle ear discharge. Arch Otolaryngol Head
Neck Surg 1988;114:1029-1030.
Wallner LJ. Tuberculous otitis media. Laryngoscope 1953;63:1058-1077.
Wardrop PE, Pillsbury HC III. Mycobacterium avium acute mastoiditis. Arch Otolaryngol 1984;110:686-687.
Windle-Taylor PC, Bailey CM. Tuberculous otitis media: a series of 22 patients. Laryngoscope 1980;90:1039-1044.
Wolfowitz BL. Tuberculous mastoiditis. Arch Otolaryngol 1982;95:109-113.
Yaniv E. Tuberculous otitis media: a clinical record. Laryngoscope 1987;97:1303-1306.
Yaniv E, Traub P, Conradie R. Middle ear tuberculosis - a series of 24 patients. Int J Pediatr Otorhinolaryngol 1986;12:5963.


medical literature.
TYMPANOSTOMY TUBES
February 23, 1995
Warren Morgan, M.D.
Otitis media is the most common diagnosis in children, affecting two thirds of all children by the age of
two years. Recurrent otitis media or otitis media with effusion may be treated with tympanostomy tubes,
and approximately 700,000 children undergo this procedure each year. In an article that appeared in
JAMA, Kleinman and colleagues studied the medical appropriateness of tympanostomy tube insertion in
children under the age of 16. After developing clinical indicators, they retrospectively reviewed a large
group of patients. They felt that preoperative indicators for tympanostomy insertion were inappropriate
for 25% and equivocal for 33% of the patients reviewed. Despite several biases in the study, this paper
received a lot of national attention, increasing parental concern about tympanostomy tubes. In addition,
the increase in cost concerns related to health care reform has lead to increased scrutiny as well.
Tympanostomy tubes are indicated for chronic otitis media with effusion, recurrent acute otitis media,
tympanic membrane atelectasis, and complications of acute otitis media in children. A recent health care
panel convened by the US government recommended tympanostomy tubes as a treatment option for otitis
media with effusion that persists for three months with associated hearing loss. If the effusion persists
four to six months despite other treatments, then tympanostomy tubes should be inserted at that time.
Recurrent acute otitis media (5-6 episodes/year) with persistent middle ear effusion is best treated with
tympanostomy tubes. Children with recurrent otitis media without persistent effusion may be treated with
antibiotic prophylaxis or tympanostomy tubes. Children who fail antibiotic prophylaxis are candidates for
tympanostomy tubes.
No guidelines exist for preoperative audiologic testing in normal children undergoing tympanostomy tube
insertion. Preoperative testing may identify undetected hearing loss and document hearing for
medicolegal reasons. Manning et al reviewed the audiologic results of 600 children undergoing
preoperative audiologic testing prior to tympanostomy tube insertion at Children's Medical Center in
Dallas, Texas.
They identified 6 patients (1%) with previously undetected sensorineural hearing loss. Each patient in this
group had risk factors for hearing loss. Also, because the study was done in a tertiary children's hospital,
the rate of hearing loss was higher than in the community. Despite these factors, they still recommended
screening of all patients.
A vast array of tympanostomy tubes have been designed. Tympanostomy tubes have been made out of
Teflon, silicone, polyethylene, stainless steel, titanium, and even gold plated steel. Karlan et al performed
a laboratory and clinical study comparing silicone and Teflon tubes and found the Teflon to be superior.
Metal compounds such as stainless steel, titanium, and gold have been considered good compounds for
tympanostomy tube construction. Several studies have failed to show a significant advantage over
synthetic tubes.
The shape and design of the tympanostomy tube is more important in determining its function. Large
inner flanges increase the duration of middle ear ventilation. Tympanostomy tubes with a large inner
diameter and short length are less likely to occlude but may allow easier passage of water. These
characteristics must be considered during the selection of tympanostomy tubes.
Tympanostomy tubes may be arbitrarily divided based on the duration of ventilation. Short term tubes are
shaped like grommets or bobbins and have and the average duration of intubation is about 10 months. The
tubes are easy to insert, have a low rate of obstruction, and low rate of permanent perforation. Long term
ventilation tubes are typically T-shaped and have the opposite characteristics. The average duration of
ventilation is around 30 months. Both groups of tubes have been modified to provide intermediate
characteristics.
Other operative factors may be important in tympanostomy tube insertion. In the past, several authors
have felt that ear canal preparation was important prior to tympanostomy tube insertion. But Baldwin and
Aland found no advantage with iodine preparation of the external canal prior to tympansotomy tube
insertion. Myringotomy direction and location have been thought to influence the duration of middle ear
ventilation and subsequent healing of the tympanic membrane. Although some authors have reported that
intubation is longer when the tube is inserted in the anterior superior quadrant, a study by April et al
found no difference between the anterior superior and anterior inferior quadrants. Likewise, myringotomy
direction had no effect in a study by Guttenplan et al.
Tympanostomy tubes may be inserted under local or general anesthesia. Some otolaryngologists have
reported successful tympanostomy tube insertion using iontophoresis for local anesthesia in cooperative
children over the age of six. Summerfield and White described insertion of tympanostomy tubes in 103
patients with an age range of 3-12 years using EMLA cream for local anesthesia. EMLA is a mixture of
lignocaine and prilocaine in a high concentration that is absorbed across the epidermis providing local
anesthesia. They were successful in 93.2% of the patients, with all failures occurring in children less than
four years. Many children undergo the tympanostomy tube insertion under general anesthesia. MarkowitzSpence et al reviewed the anesthetic complications in a series of 510 children undergoing general
anesthesia for tympanostomy tubes at the Children's Hospital of Buffalo. No complications were reported
in 423 (82.3%). Minor complications without long-term sequelae occurred in the other patients.
Postoperative topical antibiotic prophylaxis after tympanostomy tubes remains controversial. Many
otolaryngologists use otic drops to prevent blockage of the tube by blood or discharge and to prevent
postoperative otorrhea, despite the theoretical risk of ototoxicity. Garcia et al performed a meta analysis
of the five previous studies on the use of antibiotic drops and found a reduction in otorrhea by 85 %. They
recommend continued use of topical antimicrobial prophylaxis in high risk cases with mucoid or purulent
effusions. For routine cases without effusion and normal middle ear mucosa, they do not advocate routine
use.
Water may pass through a tympanostomy tube and cause a middle ear infection. Consequently, most
otolaryngologists instruct parents to take precautions to avoid water exposure. This usually includes the
use of ear plugs during bathing or water sports, while others forbid water sports. Paradoxically, children
with ear tubes who swim do not have an increased rate of otorrhea, as shown in nine different studies.
For middle ear contamination to occur through a tympanostomy tube, water must reach the tympanic
membrane and then cross the tympanostomy tube into the middle ear. Middle ear contamination with bath
water is much more irritating than other solutions. Studies suggest that middle ear contamination without
immersion underware occurs rarely.
Occasionally, a tympanostomy tube will persistent beyond the desired period of middle ear ventilation.
The otolaryngologist must then decide at what time to remove the tube. Based on an increased rate of
complications in tubes that persist for three years or longer, most tubes are removed at 2-3 years. No other
tests or criteria exist to determine when they should be removed.
Tympanostomy tubes may have significant complications. Meclelland reviewed his series of 307 patients
under the age of 10 years who had been treated with tympanostomy tubes and followed for a minimum of
1 year. The most common complication was otorrhea in 34.5% of patients. This include postoperative and
delayed otorrhea with chronic otorrhea occurring in 5.5% of patients. Tympanic membrane perforation
occurred in 6.2% of patients, but only 2.8% had chronic perforation that required surgical closure.
Persistent tympanostomy tube and cholesteatoma occurred in 0.2 % of patients. Several other studies have
been done on the overall rate of complications, showing similar rates.
Long term complications following myringotomy and tubes was reported by Ben-Ami et al for Afula,
Israel. They retrospectively reviewed and examined 64 patients 10 years after having myringotomy and
tubes. In their review, 77.9% of patients were treated with one procedure, and the remainder required
multiple procedures. Two patients had perforations on follow-up. Myringosclerosis was found in 42.3%
of tympanic membranes with only 17.8% of the patients having myringosclerosis in the area of the
previous tympanostomy tube. Only two patients (1.9%) developed long term otorrhea.
Otorrhea, the most common complication, occurs in around 20% of all patients with tympanostomy tubes.
Postoperative otorrhea is most likely related to the presence of purulent fluid or inflamed middle ear
mucosa. Delayed otorrhea occurs due to another episode of acute otitis media, which may result from
either external contamination through the tube or from nasopharyngeal reflux. Management of post
tympanostomy otorrhea usually begins with ototopical agents and antibiotics. If this fails to clear the
infection, then a culture is taken to identify any resistant organisms and guide further therapy. Sometimes
if all therapy fails, removal of the tympanostomy tube is required.
Chronic tympanic membrane perforation remains problematic with tympanostomy tubes. The rate of
perforation varies widely in reported series and is heavily influenced by tympanostomy tube type and
duration of intubation. In a number of studies, the rate of chronic perforation with short term grommet
type tube is less than 5%, while with long term tympanostomy tubes the rate of perforation is around 1520%. The longer the period of intubation, the greater the risk of persistent perforation. This factor should
be considered during selection of tympanostomy tubes.
Tympanosclerosis, or more accurately myringosclerosis, occurs in around 40-50% of children with
tympanostomy tubes. The incidence of tympanosclerosis in chronic otitis media not treated with
tympanostomy tubes is around 10%. The etiology of the tympanosclerosis is unclear and is related to both
the disease process and the tympanostomy tube. In several studies the myringosclerosis does not cause an
appreciable difference in hearing.
The development of cholesteatoma with tympanostomy tubes is a relatively rare occurrence, reported in
less than 1% in most series. Cholesteatoma may result from squamous debris being trapped in the middle
ear around the tympanostomy tube. In some series, patients developed cholesteatoma in the attic area
away from the tube site.
In conclusion, tympanostomy tubes are an important tool in the treatment of chronic otitis media in
children. Successful management requires the use of appropriate indications and the proper selection of
tube type.
Case Presentation
A 10-month-old female infant was referred to the Otolaryngology Clinic for recurrent otitis media for
which she had been treated six times with antibiotics. Each episode responded to antibiotics, but she
would develop another episode shortly after completion of antibiotic therapy. She was not routinely
exposed to cigarette smoke, and was not in day care. The remainder of her history was normal. Her
physical examination was remarkable for bilateral middle ear effusions. After discussing treatment
options with the family, they elected to proceed with insertion of tympanostomy tubes. Preoperative
audiologic evaluation was normal. She underwent bilateral myringotomies with tympanostomy tube
insertion under general anesthesia. At the time of surgery she had thick middle ear effusions.
Postoperatively she received a 3-day course of Cortisporin otic solution. She recovered well and has had
no further episodes of otitis media.
Bibliography
April MM, Portella RR, Orobello PW Jr. Tympanostomy tube insertion: anterosuperior vs. anteroinferior quadrant.
Arcand P, Gauthier P, Bilodeau G, Chapados G, Abela A, Desjardins R, et al. Post-myringotomy care: a prospective study.
J Otolaryngol 1984;13:305-308.
Armstrong BW. A new treatment for chronic secretory otitis media. Arch Otolaryngol 1954;59:653-654.
Avraham S, Luntz M, Sade J. The influence of ventilating tubes on the surgical treatment of atelectatic ears. Eur Arch
Baldwin RL, Aland J. The effects of povidone-iodine preparation on the incidence of post-tympanostomy otorrhea.
Balkany TJ, Arenberg IK, Steenerson RL. Ventilation tube surgery and middle ear irrigation. Laryngoscope 1986;96:529532.
Becker GD, Eckberg TJ, Goldware RR. Swimming and tympanostomy tubes: a prospective study. Laryngoscope
1987;97:740-741.
Ben-Ami M, Rosen G, Shlezinger T, Konack S, Ben-Ami M. Otitis media with effusion - complications after treatment. J
Laryngol Otol 1983;97:1091-1094.
Bhatnagar RK. Critical evaluation of Per-Lee tube sin children. J Laryngol Otol 1990;104:112-113.
Bright RA, Moore RM, Jeng LL, Sharkness CM, Hamburger SE, Hamilton PM. The prevalence of tympanostomy tubes in
children in the United States, 1988. Am J Public Health 1993;83:1026-1028.
Bulkley WJ, Bowes AK, Marlowe JF. Complications following ventilation of the middle ear using Good T tubes. Arch
Dingle AF, Flood LM, Kumar BU, Hampal S. The mini-grommet and tympanosclerosis: results at two years. J Laryngol
Otol 1993;107:108-110.
Fior R, Veljak C. Late results and complications of tympanostomy tube insertion for prophylaxis of recurrent purulent otitis
media in pediatric age. Int J Pediatr Otorhinolaryngol 1984;8:139-146.
Garcia P, Gates GA, Schechtman KB. Does topical antibiotic prophylaxis reduce post-tympanostomy tube otorrhea? A
meta-analysis. Ann Otol Rhinol Laryngol 1994;103:54-58.
Gates GA, Avery C, Prihoda TJ, Holt GR. Delayed onset post-tympanotomy otorrhea. Otolaryngol Head Neck Surg
1988;98:111-115.
Gates GA, Avery C, Prihoda TJ, Holt GR. Delayed onset post-tympanotomy otorrhea. Otolaryngol Head Neck Surg
1988;98:111-115.
Gates GA, Avery C, Prihoda TJ, Holt BR. Post-tympanostomy otorrhea. Laryngoscope 1986;96:630-634.
Giebink GS, Kaly K, Buran DJ, Satz M, Ayre T. Predictors for postoperative otorrhea following tympanostomy tube
insertion. Arch Otolaryngol Head Neck Surg 1992;118:491-494.
Guttenplan MD, Tom LWC, DeVito MA, Handler SD, Wetmore RF, Potsic WP. Radial versus circumferential incision in
myringotomy and tube placement. Int J Pediatr Otorhinolaryngol 1991;21:211-215.
Handler SD, Miller L, Potsic WP, Wetmore RF, Marsh RR. A controlled study of a 'new' ventilating tube. The gold
standard? Int J Pediatr Otorhinolaryngol 1986;12:33-38.
Handler SD, Miller L, Potsic WP, Wetmore RF, Marsh RR. A prospective study of titanium ventilation tubes. Int J Pediatr
Hughes LA, Warder FR, Hudson WR. Complications of tympanostomy tubes. Arch Otolaryngol 1974;100:151-154.
Hunter LL, Margolis RH, Giebink GS. Identification of hearing loss in children with otitis media. Ann Otol Rhinol
Laryngol 1994;103:59-61.
Jurgens GL, Paparella MM. Three new middle ear ventilation tubes. Trans Am Acad Ophthalmol Otolaryngol
1968;76:1017-1019.
Karlan MS, Skobel B, Grizzard M, Cassisi NJ, Singleton GT, Buscemi P, et al. Myringotomy tube materials: bacterial
adhesion and infection. Otolaryngol Head Neck Surg 1980;88:783-795.
Kleinman LC, Kosecoff J, Dubois RW, Brook RH. The medical appropriateness of tympanostomy tubes proposed for
children younger than 16 years in the United States. JAMA 1994;271:1250-1255.
Kobayashi H, Zusho H. When to remove the ventilation tube - judgment using t-tube on the time to remove the tube. Auris
Nasus Larynx 1985;12(Suppl I):S247-S248.
Le CT, Freeman DW, Fireman BH. Evaluation of ventilating tubes and myringotomy in the treatment of recurrent or
persistent otitis media. Pediatr Infect Dis J 1991;10:2-11.
Levine S, Daly K, Gieink GS. Tympanic membrane perforations and tympanostomy tubes. Ann Otol Rhinol Laryngol
1994;103:27-33.
Levinson SR, Gill AJ. Middle ear semipermeable membrane tubes for prolonged retention. Otolaryngol Head Neck Surg
1986;94:438-440.
Luotonen J, Laitaraki K, Karjalainen H, Jokinen K. EMLA in local anesthesia of the tympanic membrane. Acta Otolaryngol
Suppl 1992;492:63-67.
Luxford WM, Sheehy JL. Myringotomy and ventilation tubes: a report of 1,568 ears. Laryngoscope 1982;92:1293-1297.
Mangat KS, Morrison GAJ, Ganniwalla TM. T-tubes: a retrospective review of 1274 insertions over a 4-year period. Int J
Pediatr Otorhinolaryngol 1993;25:119-125.
Mangat KS, Morrison GAJ, Ganniwalla TM. T-tubes: a retrospective review of 1274 insertions over a 4-year period. Int J
Manning SC, Brown OE, Roland PS, Phillips DL. Incidence of sensorineural hearing loss in patients evaluated for
tympanostomy tubes. Arch Otolaryngol Head Neck Surg 1994;120:881-884.
Markowitz-Spence L, Brodsky L, Syed N, Stanievich J, Volk M. Anesthetic complications of tympanotomy tube placement
in children. Arch Otolaryngol Head Neck Surg 1990;116:809-812.
Matt BH, Miller RP, Meyers RM, Campbell JM, Cotton RT. Incidence of perforation with Goode T-tube. Int J Pediatr
Maws AR. Development of tympanosclerosis in children with otitis media with effusion and ventilation tubes. J Laryngol
Otol 1991;105:614-617.
McLelland CA. Incidence of complications from use of tympanostomy tubes. Arch Otolaryngol 1980;106:97-99.
Meyerhoff WL, Morizono T, Wright CG, Shaddock LC, Shea DA, Sikora MA. Tympanostomy tubes and otic drops.
Laryngoscope 1983;93:1022-1027.
Moore PJ. Ventilation tube duration versus design. Ann Otol Rhinol Laryngol 1990;99:722-723.
Morgan NJ. Penetration of water down the external auditory meatus to the tympanic membrane. J Laryngol Otol
1987;101:536-537.
Pappas JJ. Middle ear ventilation tubes. Laryngoscope 1974;84:1098-1117.
Pashley NRT, Scholl PD. Tympanostomy tubes and liquids - an in vitro study. J Otolaryngol 1984;13:296-298.
Per-Lee JH. A wide flanged middle ear ventilation tube. Trans Am Acad Ophthalmol Otolaryngol 1967;71:358-359.
Prichard AJN, Marshall J, Skinner DW, Narula AA. Long-term results of Good's tympanostomy tubes in children. Int J
Pringle MB. Grommets, swimming and otorrhoea - a review. J Laryngol Otol 1993;107:190-194.
Rothera MP, Grant HR. Long-term ventilation of the middle ear using the Goode T-tube. J Laryngol Otol 1985;99:335-337.
Silverstein H. Malleus clip tube for long-term equalization of middle ear pressure. Trans Am Acad Ophthalmol Otolaryngol
1966;70:640-642.
Smelt GJ, Monkhouse WS. The effect of bath water, sea water and swimming pool water on the guinea pig middle ear. J
Laryngol Otol 1985;99:1209-1216.
Summerfield MJ, White PS. Ventilation tube insertion using topical anaesthesia in children. J Laryngol Otol 1992;106:427428.
Tami TA, Kennedy KS, Harley E. A clinical evaluation of gold-plated tubes for middle-ear ventilation. Arch Otolaryngol
Head Neck Surg 1987;113:979-980.
Tavin ME, Gordon M, Ruben RJ. Hearing results with the use of different tympanostomy tubes: a prospective study. Int J
Tos M, Stanger S-E. Hearing loss in tympanosclerosis caused by grommets. Arch Otolaryngol Head Neck Surg
1989;115:931-935.
Weigel MT, Parker MY, Goldsmith MM, Postma DS, Pillsbury HC. A prospective randomized study of four commonly
used tympanostomy tubes. Laryngoscope 1989;99:252-256.


medical literature.
VESTIBULAR ADAPTATION AND REHABILITATION

Greg Ator, MD
May 21, 1992
A patient presenting with vague disequilibrium and occasional episodic vertigo is a common occurrence
in the general otolaryngology clinic. Typical etiologies include status post head injury and the
vestibulopathy of the elderly. These patients often have mild progressive symptoms without spontaneous
resolution. Many patients, over time, will have resolution of their symptoms, but for the patient who
continues to have symptoms, treatment is frequently required. Traditional treatment methods have relied
upon medication such as Antivert and Valium and perhaps Cawthorne type exercises in an attempt to
encourage central nervous system adaptation and rehabilitation. I will briefly describe vestibular
physiology, as it relates to compensation, and discuss the design and efficacy of a program of vestibular
rehabilitation.
The function of the vestibular system is to transduce head acceleration into a signal the brain can
interpret. This information is used by the central nervous system (CNS) for gaze stabilization on the
retina and posture control. Transduction is performed for rotational motion by the semicircular canals
and for linear motion by the otolith organs. A spontaneous discharge rate is present in the nerve fibers
arising from these sense organs and rotation or displacement is excitatory in one direction and inhibitory
in the opposite direction. Any difference between the discharge rates of the bilateral symmetrical
vestibular apparatus is perceived as motion.
After a unilateral vestibular insult, there is persistent asymmetry in the vestibular nerve discharge rates
leading to the incorrect sensation of vertigo and motion. These static symptoms, present in the absence of
motion, quickly resolve with restoration of symmetrical discharge rates in the vestibular nuclei.
Symptoms brought about by head motion (dynamic) take longer to compensate and the remaining
vestibular organ as well as the visual system and the somatosensory network work in concert to maintain
posture and balance. In most cases by reliance on these and other inputs the CNS can adapt to the
unilateral loss of input and near normal function in all aspects of daily living can be attained. The
mechanisms for the static and dynamic aspects of vestibular compensation to a peripheral deficit are
largely unknown. Several mechanisms have been proposed: commissural pathways between nuclei of the
vestibular system, reactive synaptogenesis, cerebellum nuclei modifications, and denervation
supersensitivity. The most likely mechanism for resolution of static symptoms is, however, that neurons
of the vestibular nuclei have a spontaneous pacemaker activity which takes over after input is eliminated
from the peripheral organ. The compensation mechanism is affected by external manipulations which are
of therapeutic value. It is known that sedative medications may prolong the time course and decrease the
extent of compensation while stimulants such as amphetamines accelerate compensation. Other external
factors can also influence the compensation process. Visual input is important in dynamic compensation
and animals deprived of visual input do not manifest dynamic compensation although this has no effect
on static deficits. Somatosensory and visual dysfunction impair dynamic compensation. The best
example is the diabetic with retinal and somatosensory disease who suffers a new onset vestibular deficit.
Because of the existence of dysfunction in all three limbs of the posture and balance system,
compensation will likely be very minimal. Exercise is important in vestibular compensation in that
immobility has been demonstrated to prevent dynamic compensation from occurring.
The importance of exercise in vestibular rehabilitation is not new, having first been described by
Cawthorne and Cooksey in 1946. They noted that patients who exercised early after a unilateral ablation
did better and they did so faster than patients who did not exercise. This clinical observation in support of
modern experimental evidence provides the basis for rehabilitation; CNS compensation is facilitated by
exercises with visual and somatosensory stimulation. The first step in a rehabilitation program is an
assessment which is typically performed by a physical or occupational therapist. The patient is
questioned about symptoms and a objective clinical assessment of vestibular function is made by
physical examination, rotatory function, and posturography. A specific, tailored program is developed to
address the particular deficits. The treatment strategy relies on the following factors: balance retraining
for postural control mechanisms; eye and head coordination with progressively more difficult visual
tracking tests; habituation therapy; and a general conditioning program. Indications for rehabilitation
therapy include patients with: movement induced dizziness or poor compensation after a unilateral
vestibular injury; status post head injury; the elderly patient with vague disequilibrium; and poor
compensation or recurrent symptoms after surgery.
Several large series have been published recently detailing results from vestibular rehabilitation
programs. Horak and Shemway reported a prospective study of twentyfive subjects with a peripheral
vestibular disorder and symptoms greater than six months duration. These patients were divided into
three treatment groups. The first group underwent a tailored program of twice weekly outpatient
exercises. The second group was given a regiment of general (Cawthorne) exercises. Finally, a third
group was given medications such as Antivert and Valium only. The results are encouraging in that
the
patients who were on medication alone showed no objective improvement on posturography scores, but
in the patients who had a tailored vestibular exercise regiment, there was a statistically significant
increase in the posturography scores indicating improvement in compensation. A subjective dizziness
index also showed significant improvement with the vestibular exercise program while no improvement
was seen with the general exercise program and approximately 50% improvement with the medication
alone group.
A prospective study from Shepard et al at the University of Michigan of 98 patients with diagnoses
including peripheral, central, and mixed etiologies. These patients participated in a tailored program with
twice daily home therapy lasting ten to fifteen weeks. Results showed a reduction in symptoms scores in
87% with complete resolution of symptoms in 36%. Patients with head injury accompanied by postural
problems, but with normal visual and somatosensory systems, and those with secondary gain did the
worst. Patients with episodic motion dysfunction had the best prognosis for improvement with this
protocol.
In summary, vestibular rehabilitation is a important treatment modality for patients with vestibular
dysfunction who are not surgical candidates. An increasing proportion of our society, the elderly, are
often prime candidates for vestibular rehabilitation therapy. It appears that patients with peripheral
vertigo with classical symptoms, especially of an episodic variety will definitely benefit from a vestibular
rehabilitation program but also patients with a vague disequilibrium and probable central etiology for this
condition may also benefit from vestibular rehabilitation therapy.
Case Presentation
A 39-year-old female had benign paroxysmal positional nystagmus, vertigo, and imbalance secondary to
a mild blow to the head. She fell while downhill ski racing and hit her head (no loss of consciousness)
but was able to continue her activities within several minutes. She was previously healthy, with no
significant medical problems, led an active life, and continued employment as a nurse. Symptoms of
vertigo, nausea, and disequilibrium progressed over two weeks post-injury and persisted for ten months.
Neurologic examination was normal; CT and MRI were negative. Tests of horizontal vestibulo-ocular
reflex control (caloric and rotary chair) were within normal limits. On ENG testing, the patient
demonstrated a directionally fixed vertical oblique nystagmus and vertigo in the right Hallpike position.
All other positional tests were normal. Symptoms progressed to the point of interfering with her work
and she sought medical treatment. She received outpatient therapy twice a week for six weeks, and twice
daily performed home exercises targeted at a variety of postural, gaze, and motion perception disabilities.
In addition, she participated in a low-impact aerobics program several times a week. After six weeks of
therapy, repeat platform posturography revealed that she had normal body sway during all six of the
sensory organization tests and her complaints of dizziness had significantly decreased. She still had mild
complaints (intensity two on a scale of zero to ten) in 12 positions. She continued with her exercises, and
within six months she was vertigo-free in all positions and has remained so.
Bibliography
Baloh RW, Honrubia V. Clinical neurophysiology of the vestibular system. 2nd ed. Philadelphia: F. A. Davis, 1989.
Cawthorne T. Vestibular injuries. Proc R Soc Med 1946;39:270-272.
Cohen B. Erasmus Darwin's observations on rotation and vertigo. Human Neurobiol 1984;3:121-128.
Cooksey FS. Rehabilitation in vestibular injuries. Proc R Soc Med 1946;39:273-275.
Galiana HL, Flohr H, Jones GM. A reevaluation of intervestibular nuclear coupling: its role in vestibular compensation. J
Neurophysiol 1984;51:242-259.
Galiana HL, Outerbridge JS. A bilateral model for central neural pathways in vestibulo-ocular reflex. J Neurophysiol
1984;51:211-241.
Grusser OJ. J. E. Purkyne's contributions to the physiology of the visual, the vestibular and the oculomotor systems.
Human Neurobiol 1984;3:129-144.
Hecker HC, Haug CO, Herndon JW. Treatment of the vertiginous patient using Cawthorne's vestibular exercises.
Laryngoscope 1974;84:2065-2072.
Henn V. E. Mach on the analysis of motion sensation. Human Neurobiol 1984;3:145-148.
Herdman SJ. Exercise strategies for vestibular disorders. Ear Nose Throat J 1989;68:961-964.
Horak FB, JonesRycewicz C, Black FO, ShumwayCook A. Effects of vestibular rehabilitation on dizziness and imbalance.
Igarashi M. Vestibular compensation: An overview. Acta Otolaryngol 1984;(Suppl 406):78-82.

Igarashi M, Alford BR, Kato Y, Levy JK. Effect of physical exercise upon nystagmus and locomotor dysequilibrium after
labyrinthectomy in experimental primates. Acta Otolaryngol 1975;79:214-220.
Igarashi M, Ishikawa K, Ishii M, Schmidt KA. Effect of ACTH(410) on equilibrium compensation after unilateral
labyrinthectomy in the squirrel monkey. Eur J Pharmacol 1985;19:239-242.
Igarashi M, Levy JK, OUchi T, Reschke MF. Further study of physical exercise and locomotor balance compensation after
unilateral labyrinthectomy in squirrel monkeys. Acta Otolaryngol 1981;92:101-105.
Ito M. Longterm depression. Ann Rev Neurosci 1989;12:85-102.
Leigh DS, Zee DS. The neurology of eye movements, 2nd ed. Philadelphia: F. A. Davis, 1991
Lowenstein O. Fifty years of vestibular science. In: Dawson WW, Enoch JM, editors. Foundations of Sensory Science.
Berlin: SpringerVerlag, 1984: 513-551.
Norre ME, Forrez GHJ, Beckers AM. Vestibular compensation evaluated by rotation tests and posturography. Arch Otol
Head Neck Surg 1987;113:533-535.
Peppard SB. Effect of drug therapy on compensation from vestibular injury. Laryngoscope 1986;96:878-898.
Semont A, Freyss G, Vitte E. Curing the BPPV with a liberatory maneuver. Adv Otorhinolaryngol 1988;42:290-293.
Shepard NT, Telian SA, SmithWheelock M. Habituation and balance retraining therapy. Neurol Clin 1990;8:459-475.
ShumwayCook A, Horak FH. Rehabilitation strategies for patients with vestibular deficits. Neurol Clin 1990;8:441-457.
Smith PF, Curthoys IS. Mechanisms of recovery following unilateral labyrinthectomy. Brain Res Rev 1989;14:155-180.
Smith PF, Darlington CL. Neurochemical mechanisms of recovery from peripheral vestibular lesions (vestibular
compensation. Brain Res Rev 1991;16:117-133.
SmithWheelock M, Shepard NT, Telian SA. Physical therapy program for vestibular rehabilitation. Am J Otol
1991;12:218-225.
Thach WT, Goodkin HP, Keating JG. The cerebellum and the adaptive coordination of movement. Annu Rev Neurosci
1992;15:403-442.
Westheimer G. Helmholtz on eye movements. Human Neurobiol 1984;3:149-152.
Zee DS. Adaptation to vestibular disturbances: some clinical implications. Acta Neurol Belg 1991;91:97-104.


medical literature.
VESTIBULAR NEURITIS
July 14, 1994
Douglas D. Backous, M.D.
Vestibular neuritis refers to a disorder of the vestibular system without an associated auditory deficit or
other disease of the central nervous system. It is a unilateral peripheral disorder, primarily affecting
patients in their third and fourth decades, which is associated with ipsilateral caloric weakness. Except
for persistent unsteadiness, symptoms usually resolve by 3-6 months. The episode of vertigo usually
consists of a series of attacks within a 10 day period. Of the patients in Coats' series, 38.2% had an
antecedent infection, 20% with sinusitis, and 61% had spontaneous nystagmus. Besides the unilateral
caloric weakness, electronystagmography reveals a directional preponderance and beating nystagmus
away from the affected side. Vestibular neuritis has also been referred to in the literature as vestibular
neuronitis, epidemic vertigo, acute labyrinthitis, vestibular paralysis, and vestibular neuropathy.
A multiple attack variant of vestibular neuritis occurs in a younger age group and consists of recurrent
attacks over months to years. Symptoms of unsteadiness and disequilibrium have shorter duration, and
patients have less severe caloric weakness. Preceding upper respiratory illness is more commonly
reported in this subset of patients.
The differential diagnosis of vestibular neuritis includes Meniere's disease, vestibular schwannoma,
perilymphatic fistula, cerebellar infarction, multiple sclerosis, disequilibrium of aging, basilar artery
insufficiency, vestibular atelectasis, and metastatic carcinoma. Meniere's disease (endolymphatic
hydrops) patients describe tinnitus, fluctuating hearing loss, and frequent episodes of vertigo of duration
limited to hours. Caloric responses are normal early in the course of endolymphatic hydrops. Tinnitus
and hearing losses are much more common in the presentation of vestibular schwannoma than is vertigo.
Patients can have signs of brainstem compression and often have other deficits in cranial nerves,
primarily V and VII. The majority of perilymphatic fistula patients describe "popping" while straining as
a precipitating event to their symptoms. Vertigo is often positional with fluctuating hearing loss and
normal caloric testing. Cerebellar infarction can be caused by hemorrhagic or ischemic events in the
distribution of the anterior inferior or posterior inferior cerebellar arteries. These patients report
symptoms consistent with the single attack variant of vestibular neuritis. Multiple sclerosis is a
demyelinating disease of mainly white matter. Early symptoms include blurred vision, vertigo, cranial
nerve palsies, and awkward extremity use. Nystagmus, scanning speech, and intention tremors occur
later. There is no sex predilection and patients are typically in their third to fourth decades of life when
diagnosed. This disease has an average duration of 20 years from discovery until eventual death.
Findings of oligoclonal banding in CSF and white matter plaques on MRI scanning help establish the
diagnosis. Disequilibrium of aging is due to degenerative changes seen in the vestibular labyrinth and to
interference with fluid motion within labyrinthine sense organs. Sudden head movements exacerbate the
vertigo. Basilar artery insufficiency is caused by occlusion of the vestibular branch of the internal
auditory artery. This most often occurs in an older age group of people who also have high frequency
hearing loss. Additional neurotologic findings are not uncommon. Collapse of vestibular membranous
structures either primarily or as a component of another process such as endolymphatic hydrops is
described as vestibular atelectasis. The most likely etiologies include viral and degenerative changes seen
in aging patients. The principle symptom is unsteadiness with head movement that is not amenable to
medical management but is not severe enough to warrant surgical therapy. The symptoms associated
with metastatic carcinoma to the temporal bone depend on where the metastasis occurs and can include
vertigo or unsteadiness.
Work-up of patients with vestibular neuritis begins with thorough history and physical examination.
Audiometry and vestibular testing are the cornerstones to diagnosis with imaging and laboratory studies
being guided by findings on examination. Bedside examination includes Doll's eye test, head shaking
nystagmus, dynamic visual acuity, caloric testing, rotational testing, past pointing, Romberg and Fukuda
tests, and tandem walking.
Treatment is primarily supportive with reassurance that symptoms will resolve in most cases. Vestibular
suppressants are reserved for patients with severe symptomatology. Medical labyrinthine ablation or
surgical therapy are rarely indicated.
In conclusion, the characteristic symptom complex of unilateral vestibular weakness without auditory or
other central nervous system pathology comprises vestibular neuritis. Vestibular nerve atrophy or
degeneration may occur but vascular etiologies are unlikely. Treatment is primarily conservative and the
majority of patients will recover in 3-6 months. Refractory cases should be investigated for other
etiologies.
Case Presentation
A 65-year-old white man presented to the Otolaryngology clinic with a three-day history of severe
vertigo. He reported nausea and vomiting for the first two days of his illness but successfully ate
breakfast on the day he was seen in clinic. He denied hearing loss and tinnitus. His past medical and
surgical histories were unremarkable. He had no previous exposure to ototoxic drugs and denied further
neurologic symptoms. Physical examination revealed an obviously uncomfortable white male in a
wheelchair. Otologic examination was without abnormality. Weber testing with a 512 hz tuning fork was
to midline. Romberg and Fukuda testing indicated right-sided pathology. Other than a crisp left beating
nystagmus, cranial nerve examination was normal. In light of the nystagmus and severe vertigo, the DixHallpike maneuver was not done. Audiometry revealed a symmetrical mild-to-moderate high frequency
hearing loss. Acoustic reflexes were mildly elevated bilaterally with adequate speech understanding.
Type A tympanograms were present bilaterally. The patient was treated with valium, m q 8 hours. He
returned to the clinic 7 days later, able to walk and with persistent unsteadiness. Electronystagmography
performed one week later revealed 30% unilateral weakness on the right with a 55% directional
preponderance to the right. Rotatory testing revealed a right peripheral disorder. On return clinic followup he had a residual feeling of unsteadiness but no vertigo.
Bibliography
Adour KK, Sprague MA, Hilsinger RL Jr. Vestibular vertigo: a form of polyneuritis? JAMA 1981;246:1564-1567.
Baloh FW, Honrubia V. Clinical Neurophysiology of the Vestibular System, ed. 2. Philadelphia: F.A. Davis, 1990.
Baloh RW, Jacobson K, Honrubia V. Idiopathic bilateral vestibulopathy. Neurology 1989;39:272-275.
Brandt T, Dieterich M. Vestibular falls. J Vestibular Res 1993;3:3-14.
Brantberg K, Magnusson M. The dynamics of the vestibulo-ocular reflex in patients with vestibular neuritis. Am J
Otolaryngol 1990;11:345-351.
Coats AC. Vestibular neuronitis. Tran Am Acad Ophthalmol Otolaryngol 1969;73:395-408.

Davis LE. Infections of the labyrinth. In: Cummings CW, Harker LA, eds. Otolaryngology - Head and Neck Surgery, Vol.
4. St. Louis: Mosby, 1993:2795-2807.
Davis LE, Shurin S, Johnson RT. Experimental viral labyrinthitis. Nature 1975;254:329-331.
Davis LE. Comparative experimental viral labyrinthitis. Am J Otolaryngol 1990;11:382-388.
Disher MJ, Telian SA, Kemink JL. Evaluation of acute vertigo: unusual lesions imitating vestibular neuritis. Am J Otol
1991;12:227-231.
Fluur E. Interaction between the utricles and the horizontal semicircular canals. IV. Tilting of human patients with acute
unilateral vestibular neuritis. Acta Otolaryngol 1973;76:349-352.
Gagey PM, Toupet M. Orthostatic postural control in vestibular neuritis: a stabilometric analysis. Ann Otol Rhinol
Laryngol 1991;100:971-975.
Karmody CS. Viral labyrinthitis: early pathology in the human. Laryngoscope 1983;93:1527-1533.
Kemink JL, Telian SA, El-Kashlan H, Langman AW. Retrolabyrinthine vestibular nerve section: efficacy in disorders
other than Meniere's disease. Laryngoscope 1991;101:523-528.
Lachman J, Stahle J. Vestibular neuritis: a clinical and electronystagmographic study. Neurology 1967;17:376-380.
Lindsay JR, Vital labyrinthitis - histopathologic characteristics. Acta Otolaryngol 1967;63:138-143.
Magnusson M, Brantberg K, Pyykk, Schaln L. Reduction of the time constant in the VOR as a protective mechanism in
acute vestibular lesions. Acta Otolaryngol Suppl 1989;468:329-332.
Montandon PB, Husler R. Relevance of otopathological findings in the treatment of dizzy patients. Ann Otol Rhinol
Laryngol Suppl 1984;112:12-16.
Pfaltz CR. Vestibular compensation: physiological and clinical aspects. Acta Otolaryngol 1983;95:402-406.
Rahko T, Karma P. New clinical finding in vestibular neuritis: high-frequency audiometry hearing loss in the affected ear.
Schuknecht HF, Kitamura K., Vestibular neuritis. Ann Otol Rhinol Laryngol Suppl 1981;78:1-19.
Schuknecht HF, Witt RL. Acute bilateral sequential vestibular neuritis. Am J Otolaryngol 1985;6:255-257.
Yoneda S, Tokumasu K. Frequency analysis of body sway in the upright posture: statistical study in cases of peripheral
vestibular disease. Acta Otolaryngol 1986;102:87-92.


Alford Otology PDF

Uploaded by

Document Information

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Alford Otology PDF

Uploaded by

Copyright:

Available Formats

Grand Rounds Archive - Otology/Neurotology

Grand Rounds Archive

Grand Rounds Archive - Otology/Neurotology

Considerations in the Evaluation of the Hearing Impaired Child [3/11/93]

Laryngology | Neoplasms | Pediatric | Plastics | Rhinology | Others | Main | Home

2001, The Bobby R. Alford Department of Otorhinolaryngology and Communicative Sciences,

http://www.bcm.edu/oto/grand/otology.html (2 of 2) [5.11.2005 10:38:17]

From the Grand Rounds Archive at Baylor

Grand Rounds Archives

**** DISCLAIMER ****

Acute Otitis Media: A Public Health Perspective

From the Grand Rounds Archive at Baylor

months of age with a 3-day history of increasing irritability and

http://www.bcm.edu/oto/grand/031899.html (2 of 6) [5.11.2005 10:38:33]

From the Grand Rounds Archive at Baylor

http://www.bcm.edu/oto/grand/031899.html (3 of 6) [5.11.2005 10:38:33]

From the Grand Rounds Archive at Baylor

http://www.bcm.edu/oto/grand/031899.html (4 of 6) [5.11.2005 10:38:33]

From the Grand Rounds Archive at Baylor

pharmacodynamic studies. Antimicrob Agents Chemother 1997;41:1926-1932.

From the Grand Rounds Archive at Baylor

Click here to view the slides from this presentation

Return to the Grand Rounds Archive Index

&REG; Copyright, 1995-9. All Rights Reserved.

http://www.bcm.edu/oto/grand/031899.html (6 of 6) [5.11.2005 10:38:33]

Acute Otitis Media

http://www.bcm.edu/oto/grand/03-18-99/sld001.htm [5.11.2005 10:39:23]

Grand Rounds Archives

**** DISCLAIMER ****

AUTOIMMUNE INNER EAR DISEASE

http://www.bcm.edu/oto/grand/4893.html (2 of 5) [5.11.2005 10:39:37]

http://www.bcm.edu/oto/grand/4893.html (3 of 5) [5.11.2005 10:39:37]

http://www.bcm.edu/oto/grand/4893.html (4 of 5) [5.11.2005 10:39:37]

Return Grand Rounds Archive Index

http://www.bcm.edu/oto/grand/4893.html (5 of 5) [5.11.2005 10:39:37]

From the Grand Rounds Archive at Baylor

Grand Rounds Archives

**** DISCLAIMER ****

BAROTRAUMA OF THE MIDDLE AND INNER EAR

From the Grand Rounds Archive at Baylor

From the Grand Rounds Archive at Baylor

http://www.bcm.edu/oto/grand/32395.html (3 of 10) [5.11.2005 10:39:56]

From the Grand Rounds Archive at Baylor

From the Grand Rounds Archive at Baylor

From the Grand Rounds Archive at Baylor

The last disorder related to barotrauma is the phenomenon known as alternobaric

http://www.bcm.edu/oto/grand/32395.html (6 of 10) [5.11.2005 10:39:56]

From the Grand Rounds Archive at Baylor

http://www.bcm.edu/oto/grand/32395.html (7 of 10) [5.11.2005 10:39:56]

From the Grand Rounds Archive at Baylor

From the Grand Rounds Archive at Baylor

Return Grand Rounds Archive Index

From the Grand Rounds Archive at Baylor

Return to BCOM Otolaryngology Home Page

http://www.bcm.edu/oto/grand/32395.html (10 of 10) [5.11.2005 10:39:56]

From the Grand Rounds Archive at Baylor

Grand Rounds Archives

**** DISCLAIMER ****

From the Grand Rounds Archive at Baylor

From the Grand Rounds Archive at Baylor

http://www.bcm.edu/oto/grand/72095.html (3 of 11) [5.11.2005 10:40:15]

From the Grand Rounds Archive at Baylor

From the Grand Rounds Archive at Baylor

DISCLAIMER

DISCLAIMER

DISCLAIMER

DISCLAIMER

DISCLAIMER

DISCLAIMER

DISCLAIMER

DISCLAIMER

DISCLAIMER