OTOSCLEROSIS

DEFINITION
A primary disease of the otic capsule
characterized pathologically by abnormal
resorption and deposition of bone

HISTOPATHOLOGY
Resorption of bone by osteocytes
Formation of new vascular
spongy bone
Formation of dense sclerotic
bone

AREAS OF PREDILECTION
Fissula ante fenestram (80% to 90%)

OTHER AREAS
Round window, the apex of

the cochlea, the

cochlear aqueduct, the semicircular canals, and

the stapes footplate itself

COCHLEAR INVOLVEMENT

ETIOLOGY
Unknown cause
Positive family history in about 60%
Inherited by autosomal dominant transmission with
incomplete penetration (60%)
Persistent measles virus infection
Detection of measles virus RNA in the affected bone

Detection of measles virus-specific antibodies in the

perilymph

PHYSIOLOGY
Conductive HL: due to fixation of the
stapedial footplate
Mixed HL: due to
Liberation of toxic metabolites into the inner
ear
Vascular compromise from sclerosis
narrowing of vascular channels
Direct extension of lesions into the inner ear

Cochlear otosclerosis

and

Involvement of footplate and cochlea

CLINICAL PRESENTATION

Hearing loss of gradual onset at 15 - 45 years

Slowly progressive course
70% are bilateral
Accelerates with pregnancy (30-40%)
Tinnitus
Paracusis Willisii
Change of the speech pattern
Vestibular symptoms

PHYSICAL EXAMINATION
Normal tympanic membrane
Schwartze sign (Flamingo flush)

PHYSICAL EXAMINATION
Normal tympanic membrane
Schwartze sign (Flamingo flush)
Tuning fork tests

PURE TONE AUDIO

CARHARTS NOTCH
Decrease in bone conduction thresholds
5 dB at 500 Hz
10 dB at 1000 Hz
15 dB at 2000 Hz
5 dB at 4000 Hz

Explanation is not known

Reverses following successful surgery

AUDIOMETRY
Pure tone audiogram
Speech discrimination

AUDIOMETRY
Pure tone audiogram
Speech discrimination
Impedence & tympanometry

CT SCAN

Double ring cochlea or Halos sign

COCHLEAR OTOSCLEROSIS
Isolated pure sensorineural hearing loss
without a conductive component

CRITERIA FOR DIAGNOSIS OF

COCHLEAR OTOSCLEROSIS
Progressive pure cochlear loss beginning at the
usual age of onset for otosclerosis
Unilateral conductive hearing loss consistent with
otosclerosis and bilateral symmetric SNHL
Positive Schwartzes sign
Positive family history
Excellent discrimination
Stapedial reflex demonstrating the on-off effect
CT: demineralization of the cochlea

DIFFERENTIAL DIAGNOSIS
Congenital fixation of the stapes
Middle ear effusion
Chronic OM and ossicular discontinuity
Tympanosclerosis
Malleus head fixation
Systemic diseases

SYSTEMIC DISEASES
Osteogenesis imperfecta
Stapes fixation
Blue sclera
Fractures

SYSTEMIC DISEASES
Osteogenesis imperfecta
Stapes fixation
Blue sclera
Fractures

Pagets disease
Crowding in epitympanum
Elevated alkaline phosphatase
Skeletal bone involvement

TREATMENT
Observation
Hearing aid
Medical treatment
Surgical treatment

OBSERVATION

INDICATIONS OF OBSERVATION
Unilateral
Mild CHL
Young age

HEARING AID

INDICATIONS OF HEARING AID

Refuse surgery
Poor surgical candidate
Following improvement of CHL

MEDICAL TREATMENT

AIM OF MEDICAL TREATMENT

Stabilize the disease by reduction of the
osteoclastic bone resorption and increase
osteoblastic bone formation

MEDICAL MANAGEMENT
Sodium fluoride: 50-75 mg /day/2years
followed by 25 mg for life
Vitamin D
Calcium carbonate

INDICATIONS
Cochlear otosclerosis
Patients with confirmed otosclerosis but
having progressive SNHL disproportionate
to age

CONTRAINDICATIONS
Chronic nephritis
Rheumatoid arthritis
Pregnancy and lactation
Children

SURGICAL TREATMENT

PATIENT SELECTION FOR

SURGICAL TREATMENT
Socially unacceptable conductive or
mixed hearing loss
Good speech discrimination
Age
Lifestyle and occupation

ABSOLUTE CONTRAINDICATION
OF SURGERY

The better or the only functioning ear

OTHER
CONTRAINDICATIONS
? Patients experience frequent changes in

barometric pressure
Malignant otosclerosis
Endolymphatic hydrops
TM perforation
Infections

STAPES SURGERY

Stapedectomy

Stapedotomy

STAMP
(STApedotomy
Minus Prosthesis) or
Stapedioplasty

STAPEDECTOMY
Results probably are the best
More traumatic to the inner ear
Increased post-op vestibular symptoms
Higher incidence of postoperative SNHL

The operation is unavoidable in:

Comminuted fracture of the footplate
Revision surgery

STAPEDOTOMY
Equal

or

better

results

vestibulocochlear side effects

with

less

COMPARISON

STAMP
Preservation of the stapedius
tendon
Reduction in hyperacusis
Reduction in risk for long-term
postoperative inner ear injuries

No prosthesis complications
Very difficult technique

SURGICAL PROCEDURE

The Incision

Permeatal (Transcanal)

Endaural

STAPEDOTOMY

LASER STAPEDOTMY

STAMP

OPERATIVE PROBLEMS &

COMPLICATIONS

TM PERFORATION
Proceed and then repair

CHORDA TYMPANI INJURY

30% of cases
Metallic taste
Symptoms usually resolves

in 3-4 months
More symptoms if bilateral

OBTRUSIVE FACIAL NERVE

0.5 %
Stapedotomy is usually possible

BLEEDING
Mucosal trauma
Active phase
Persistent stapedial artery

Persistent stapedial artery

ROUND WINDOW
OTOSCLEROSIS
About 1% complete (Shuknecht)
If complete:
Abandon surgery
If incomplete or not sure:
Do not remove bone and
proceed

OBLITERATIVE OTOSCLEROSIS
OF THE OVAL WINDOW
A total stapedectomy
is

contraindicated

because of high risk

of surgically induced
SNHL

INCUS PROBLEMS
Subluxation:
Proceed
Dislocation:
Remove incus & use a
malleus-grip prosthesis

FLOATING FOOTPLATE
May be avoided if control
holes are used or by using
laser fenestration

FLOATING FOOTPLATE
May be extracted by needles/hooks with hole
inferior to the oval window

FLOATING FOOTPLATE
In many cases should be left
and surgery is completed
with unpredictable results or
use laser fenestration

MALLEUS ANKYLOSIS
About 0.5%
May be congenital or acquired
Causes about 15-20 dB CHL
Remove malleus head and the incus and
use malleus grip prosthesis

CSF GUSHER
Due to fundal defect of IAM or widened cochlear

aqueduct
Introduce spinal catheter and proceed
Or
Pack with fascia and gauze for 4-5 days with delayed
reconstruction that avoid reopening the fenestra

PERILYMPH FISTULA
Primary or secondary

PREVENTION OF PERILYMPH
FISTULA
Stapedectomy < stapedotomy
Oval window seal
No fat or gel-foam for seal
Prohibit nose blowing, flying, diving, &
lifting heavy objects postoperatively

DIAGNOSIS OF PERILYMPH
FISTULA
Drop or fluctuation in hearing
Vertigo & tinnitus
Audiometry
ENG
Fistula test
Radiology

TREATMENT
Surgical closure

REPARATIVE GRANULOMA
Granuloma formation around the prosthesis and
incus
1-5%
Gradual deterioration 5-15 days postoperativly
Vertigo, tinnitus and deafness
Otoscopy: reddish discoloration of the
posterior TM

REPARATIVE GRANULOMA
Treatment is by emergency
tympanotomy and excision

SNHL
0.2-10%
Serous labyrinthitis high frequencies
Surgical trauma

PERSISTENCE OR
RECURRENCE OF CHL
Prosthesis malfunction
Fibrous adhesion
Incus erosion

PERSISTENCE OR
RECURRENCE OF CHL
Prosthesis malfunction
Fibrous adhesion
Incus erosion
Missed pathology: e.g. malleus fixation, round

window otosclerosis
Otosclerosis regrowth

RARE COMPLICATIONS
Facial paralysis
Acute otitis media
Cholesteatoma

THANK YOU