Professional Documents
Culture Documents
I lheIar
)()(I)(
Part
Eight prominent signs and symptoms
CLINICALMANIFESTATI0NS.
primarily with diseasesof the ear and temporal
fl:errassociated
Otalgia usually is associatedwith inflammation of the external
or middle ear, but it may represent pain referred from involvement of the teeth, temporomandibular joint, or pharynx. In
young infants, pulling or rubbing the ear along with general irritability or poor sleep, especiallywhen associatedwith fever, may
be the only signs of ear pain. Ear pulling alone is not diagnostic
of ear pathology.
Purulent otorrhea is a sign of otitis externa, otitis media with
perforation of the tympanic membrane (TM), drainage from the
middle ear through a patent tympanostomy tube, or, rarely,
drainage from a branchial cleft sinus. Bloody drainage may be
associatedwith acute or chronic inflammation (often with granulation tissue),trauma, neoplasm, foreign bodS or blood dyscrasia. Clear drainage suggestsa perforation of the TM with a serous
middle-ear effusion or, rarely, a cerebrospinal fluid leak draining
through defects (congenital or traumatic) in the external auditory
canal or from the middle ear.
Hearing loss results either from disease of the external or
middle ear (conductive hearing loss) or from pathology in the
inner ear, retrocochlear structures, or central auditory pathways
(sensorineuralhearing loss). The most common cause of hearing
loss in children is otitis media (OM).
Swelling around the ear most commonly is a result of inflammation (e.g., external otitis, perichondritis, mastoiditis), trauma
(e.g., hematoma), benign cystic masses,or neoplasm.
Vertigo is a specific type of dizzinessthat is defined as any illusion or sensation of motion. Dizziness is less specificthan vertigo
and refers to an altered orientation in space.Vertigo is an uncommon complaint in children; the child or parent may not volunteer information about balance unless asked specifically. The
most common cause of dizzinessin young children is eustachian
tube-middle-ear disease,but true vertigo also may be caused by
labyrinthitis, perilymphatic fistula between the inner and middle
ear due to trauma or a congenital inner ear defect, cholesteatoma
in the mastoid or middle ear, vestibular neuronitis, benign paroxysmal vertigo, Meniere disease,or diseaseof the central nervous
system. Older children may describe a feeling of room-spinning
or turning; younger children may expressthe dysequilibrium only
by falling, stumbling, or clumsiness.
Nystagmus may be unidirectional, horizontal, or ierk nystagmus. It is vestibular in origin and usually is associated with
vertlgo.
Tinnitus rarely is described spontaneously by children, but it
is common, especially in patients with eustachian tube-middleear diseaseor sensorineural hearing loss (SNHL). Children may
describetinnitus if asked directly about it, including laterality and
the quality of the sound.
The facial nerve may be dehiscentin its course
FACIALPARALYSIS.
through the middle ear in as many as 50"/" ol patients. Infection
with local inflammation, most commonly in acute OM, may lead
to a temporary paralysis of the facial nerve. It also may be due
EXAMINATION
PHYSICAT
Complete examination with special attention to the head and
neck may reveal a condition that may predispose to or be associated with ear diseasein children. The facial appearanceand the
PaoooseBoard
Figure 63.5-1.Methods of restrainingan infant for examination and for prosr.h as tympanocentesisor myringotomy. (From Bluestone CD' Klein
..irr.,
Media in Infants and Children,2nd ed. Philadelphia' \UB Saunders,
Otitis
lO:
1 , 9 9 5p, 9 r . l
NORMAL
Position-neutral
Color-normal
Translucency-translucent
Mobility-moves brisklywith slight
positiveand negativepressure
NEGATIVEMIDDLEEAR
PRESSURE
Position-retracted
Color-normal
Translucency-translucent
Mobility-moves only with applied
negativepressure
ACUTEOTITISMEDIA
Position-full to bulging
Color-red (can be pink,white,or
yellow)
Translucency-opaque
Mobility-poor when both positive
and negative pressuresare
applied
FLUIDLEVEL
Position-retracted
Color-yellow or amber
Translucency-translucent
Mobility-same as with high
negativepressure,but fluid level
and bubbleschangewith applied
pressure
OTITISMEDIAWITH EFFUSION
Position-usuallyretracted
Color-white (or yellowor blue)
Translucency---opaque(may be
translucent)
Mobility-poor when both positive
and negativepressuresare
applied
PERFORATTON
(ORPATENT
ryMPANOSTOMY
TUBE)
Position-neutralor retracted
Color-white,pink,red,or normal
Translucency-translucent
or
opaque
Mobility-none
Figure 635-2' Common conditions of the middle ear, as assessedwith the otoscope. (From Bluestone CD,
Klein JO: Otitis Media in Infants and Children,
e d . P h i l a d e l p h i aS, T BS a u n d e r s2, 0 0 f , p 1 3 1 . )
andEvaluationr 2619
6it5I GeneralConsiderations
Chapter
with one hand and holding the child's head against the parent's
chest with the other hand. If necessary,the child's legs can be held
between the parent's knees.To avoid ear trauma with movement,
the examiner should hold the otoscope with the hand placed
firmly against the child's head or face, so that the otoscope moves
with the head. Pulling up and out on the pinna straightens the
ear canal and allows better exposure of the TM.
'When
examining the ear, inspection of the auricle and external
auditory meatus for infection may aid in the evaluation of complications of OM. External otitis mav result from acute OM with
iischa.ge, or inflammation of the posterior auricular area may
indicate a periosteitis or subperiostealabscessextending from the
mastoid air cells. The presenceof preauricular pits or skin tags
also should be noted because affected children have a slightly
higher incidence of SNHL.
Cerumen is a protective, waxy, water-repellent coating in the
ear canal that may interfere with examination. Cerumen usually
is removed using the surgical head of the otoscope, which allows
passageof a wire loop or a blunt curette under direct visualization. Other methods include gentle irrigation of the ear canal with
warm water, which should be performed only if the TM is intact,
or instillation of a solution such as diluted hydrogen peroxide in
the ear canal (with intact TM only) for a few minutes to soften
the wax for suction removal or irrigation. Some commercial
preparations such as trolamine polypeptide oleate-condensate
(Cerumenex) may cause dermatitis of the external canal with
chronic use and should be used only under a physician's
suDervrslon.
lnflammation of the ear canal with associated pain often
indicates external otitis. Abnormalities of the external auditory
canal include stenosis (common in children with trisomy 21),
bony exostoses, otorrhea, and the presence of foreign bodies.
Cholesteatoma of the middle ear may manifest in the canal as
intermittent foul-smelling drainage, sometimes associated with
white debris; cholesteatoma of the external canal may appear as
a white, pearl-like mass in the canal skin. White or gray debris
of the canal suggestsfungal external otitis. Newborn ear canals
are filled with vernix caseosa,which is soft and pale yellow and
should disappear shortly after birth.
The TM and its mobility are best assessedwith a pneumatic
otoscope. The normal TM is in a neutral position; a bulging TM
may be caused by increased middle-ear air pressure, with or
without pus or effusion in the middle ear; a bulging drum may
obscure visualization of the malleus and annulus. Retraction of
the TM usually indicates negative middle-ear pressure,but it also
may result from previous middle-ear diseasewith fixation of the
ossicles,ossicular ligaments, or TM. When retraction is present,
the bony malleus appears more prominent, and the incus may be
more visible posterior to the malleus.
The norrnal TM has a silvery-gray, "waxed paper" aPpearance
(Fig. 535-2). A white or yellow TM may indicate a middle-ear
effusion. A red TM alone may not indicate pathology, because
the blood vesselsof the membrane may be engorged as a result
of crying, sneezing, or nose blowing. A normal TM is translucent, allowing the observer to visualize the middle-ear landmarks: incus, promontory, round window niche, and, often, the
chorda tympani nerve. If a middle-ear effusion is present, an airfluid level or bubbles may be visible (seeFig.635-2).Inability to
visualize the middle-ear structures indicates opacification of the
drum, usually causedby thickening of the TM, a middle-ear effusion, or both. Assessmentof the light reflex usually is not helpful,
because a middle ear with effusion reflects light as well as a
normal ear.
TM mobility is helpful in assessingmiddle ear pressuresand
is mixed with air, the TM may still have some mobility. Outward
eardrum movement is less likely in the presenceof severenegative middle-ear pressure or middle-ear effusion.
The TM that exhibits fullness (bulging) moves to applied pos-
(BIRTH-AGE
FoRUsE
W|TH
Ntot{ATts
28D)Wt{EilUiltVERsAr
SCREEIilNG
tSiloTAvAttABtE
(hildhood
Family
history
ofhereditary
sensorineural
hearing
los
Inutero
infection,surh
ascytomegalovirus,
rubella,
syphilis,
herpes
simplex,or
toxoplasmosis
(raniofacial
anomalies,
induding
those
withrnorphologic
pinna
abnormalities
ofthe
anoearcanat
weighr
q (33 lb)
Birth
<1500
Hyperbilirubinemia
ataserum
level
requiring
exchange
trandusion
ftotoxic
medrcationt
including
butnotlimited
totheaminoglyc0sides,
used
inmultiple
cou6es
0rin
combination
withloop
diuretic5
Eacerial
meningitis
Apgar
scores
of0-4at1min0r0-6at5 mjn
Mechanical
ventilation
lasting
>5d
Stigmata
orother
findings
asso(iated
withasyndrome
known
toinclude
asensorineural
and/or
conductive
hearing
loss
(AGT
IORUsE
WITH
INFAIITS
29D-2YR}WHEN
CEflTAIN
HEATTI{
C()NDITIONS
DEVTTOP
THAT
REOUIRE
RESCREET'llt{G
Parent/rareglver
concern
regarding
hearing,speech,
language,and/or
developmental
delay
Bactenal
meningitis
andother
infections
aso(iated
withsensorineural
hearing
loss
Head
trauma
associated
withlosofconsciousnes
orskull
fracture
stigmata
0rother
findings
associated
withasyndrome
known
toindude
asensorineural
and/or
conductive
hearing
los
ftotoxic
medicationtincluding
butnotlimited
tochemotherapeutic
agen6
oraminoglycosides
used
in
(ou6es
multiple
0rincombination
withl00p
diuretics
Rerunent
orpersistent
otitis
media
witheffr.lsion
foratIeast
3 mo
(AGE
TOR
UsE
WITH
INIA}ITS
29D-3YB)WH()
REQUIRT
PERIODIC
MONITORING
OTHEARING
(Some
newborns
andinfants
maypass
initial
hearing
periodi(
screening
butrequire
monitoring
ofhearing
todetect
delayed-onset
sensorineural
and/or
conductive
hearing
loss.lnfants
wilhthese
indkat06
require
hearing
evaluation
atleast
every
6 m0untilage3yr,and
atappropriate
intervals
thereafter.)
I1{DICAT()RS
ASSOCIATED
WITH
DTI.AVED{NsET
SENSORIIIEUf,AI
HEARIIIG
LOSS
Family
history
ofhereditary
childhood
hearing
los
infectron,
lnulero
such
ascytomgalovirus,
rubella,syphilis,
hupes
simplex,
ortoxoplasmosis
Neurofrbromato'sis
type
2andneurodegenerative
disorden
IiIDICATOR5
ASSOCIATED
W]IHCOI{DUOIVI
HHRIIIG
TOSS
Re(urrent
orperistent
otitis
media
whheffusi0n
Anatomic
deformities
andother
dis0rde6
thataffed
eushrhian
tube
fun(tion
Neurodegene{ative
disorde6
Adapted
(ommitEe0nlnfafltHeainqi9g4
fromAmerican
Acadeoy
0fPediitrics,J0int
tommittee
onlnfantHearingi]oint
Porition
slatemenl.Pedlrlri(
1995;95:
l5?.
mcus
GENE
AUDIO
PHENOTYPE
Conductive
hearing
loss
fixati0n
mimicklng
otoselerosis;
dueto5tapes
progressive
superimposed
5NHL
DIAPH1 Low{requency
loss
beginning
inthefirstdecade
andprogressing
t0all
DTNA1
withprofound
loses
frequencies
topmdure
aflataudioprofile
throughout
theauditory
range
K(NO4 5ymmetrical
high-frequency
sensorineural
loss
beginning
inthefint
DFNA2
decade
andprogresing
over
allfrequenries
losbeqinning
inthethird
6l83
Symmetriral
hiqh-frequency
sensorineural
decade
W.SI
Early-onset
low{requency
sensorinerual
loss;about
75%offamilies
DFNA6i14/]8
(arrymissense
mutalions
in
dominantly
segregating
thisaudioprofile
thefterminal
domain
ofwolframin
Progresive
sloplng
DFNAl
O
TYA4
loss
beginning
inthesecond
decade
asaflattogently
audioprofile
thatbecomes
sloping
withage
steeply
(0111
A2 (ongeniul
mid.ftequency
loss
thatshows
DFNAl3
sensorineural
age-related
progression
arross
theauditory
range
progressive
inthesecond
decade
POU4I3 Bilateral
sensorineural
loss
beginning
DINA15
progresive
loss
beginning
inthesecond
decade;
DFNA20/26 A(rG|
Bilateral
sensorineural
withage,the
loss
In(rea5es
withthrsh0ld
shifts
inallfreguenries,
asloping
ismaintained
lnmost
cases
although
confgurati0n
(0mmon
genotype,
loss
varies
frommildtoprofound
Themost
DFNB]
6J82,
Hearing
inabout
isassociated
withsevere
toprofound
5NHL
G]86
35del6/35delG,
in
topmfound
deafness
Bobserved
90%ofaffected
children;severe
carrying
one
only60%ofchildren
whoarecompound
heterozygotes
in
6182
SNHL-causing
allele
variant;
35delG
allele
and
any
other
mutati0ns,
severe
children
canying
two6J82
SNHL<ausing
missense
toprofound
deahess
isnotobserved
(see
Table
DFN84
5LQ6A4 DFN84
andPendredt
syndrome
636J)areallelic
DFNB4
withdilatation
0fthe
ve$ibular
aqueduct
hearing
losisassociated
Inthehigh
frequencies,the
losis
and
can
beunilateral
orbilatetal
varies
degree
ofloss
severe
toprofound;
inthelowftequencies,the
(prelingual),but
progressive
widely
becongenital
0nset
can
postlingual
losalso
rscommon
is
I2SrRNA Degree
ofhearing
losvaries
frommildtoprofound
butusually
mtDNA
pre(ipitous
affe(ted;
>G
symmetlGl;
highfrequencies
arepreferentially
1555A
therapy
losinhearing
can
occur
after
aminoglycoside
DFN]
P)u3f4
loss
5NHL,sensorineufal
hearing
RlH,
Bale
lFJlWhite
KR:
Sens0nBeural
hearing
losinchildren
/ancet2ffi5365:879
From
5mith
INTECTIONS
CONGENITAT
[ytomegalovrrus
virus
choriomeninqitis
Lymphocync
avttus
Rubel
qondii
[oxoplosno
pollidun
Treponeno
INFEOIONs
ACQUIRED
Borrelio
burgdarferi
virus
Barr
Epsteif
tnfluenzoe
Hoenophtlus
V US
LASSA
virus
Measles
vtrus
Mumps
neningittdh
Neisseria
entero\/tru5eS
Non-oolio
folciparum
Plosnodium
5trcatacacut
Dneun1niae
virus
zoster
Var(ella
2005J65:879-890
lan(Pf
hearing
losinchildten
KR:Sensorineural
lFlr.,While
Bifl,Bale
From
5mith
as -50% of SNHL cases(seeTables636-2 and 636-3). Thesedisorders may be associatedwith other abnormalities'may be part
of a named syndrome, or may exist in isolatlon' SNH[. often
occurs with abnormalitiesof the ear and eye and with disorders
of the metabolic, musculoskeletal,integumentarn renal, and
nervous systems.Autosomal dominant hearing lossesaccount for
about 107u of all casesof childhood SNHL. Waardenburg(types
I and II) and branchio-otorenal syndromes represent2 of the
most common autosomal dominant syndromic types of SNHL.
Tvoes of SNHL are coded with a 4-letter code and a number, as
fc,ilows, DFN = deafness, A = dominant, B = recessive, and
number = order of discovery,e.g. DFNA 13. Autosomal dominant conditionsin addition to thosejust discussedinclude:DFNA
I t o 1 1 , 1 3 , 1 5 , 1 7 , 2 0 , 2 2 , 2 8 , 3 6 , 4 8 a n d m u t a t i o n si n t h e c r y s tallin gene (CRYM). Autosomal recessivegenetic SNHL, both
syndromic and nonsyndromic, accounts for about 80% of all
clildhood casesof SNHI-. Usher syndrome (types 1,2, and 3),
Pendredsyndrome, and the Jervell and Lange-Nielsensyndrome
(one form of the long Q-T syndrome)are 3 of rhe most common
GENE
PHENOTYPE
DOMINAI'IT
(W51)
Waardenberg
PAX]
relattve
frst-degree
forelock;
andanaffeoed
irses;
white
los;heterochromic
hearing
congenital
acanthorum;
aiteria
indude
dystop
Major
diagnostic
(W52)
Waardenberq
M/lF,others
Branchio-otorenal
EYAl
RECESSIVE
syndrome
Pendred's
5LC26A4
biateral
The
(40%),
lities
abnorma
ear(3070)
andexternal
renal
ial(7070),
pits{85%),
andbranch
prea
uricular
a incl
udehealngoss(9870),
Diagngstic
iriter
indegree,
andmildtoprofound
otmixed,
sensonneural,
loscanbeconductive,
hearing
and
butcanbelae-onset
cases,
inmany
toprofound
andsevere
n0npr0gressive,
loss
thatrscongenital,
hearing
include
sensorineural
Dlagnostir
cfitetia
perchlorate
test0rg0iter'
dlscharge
andanabnormai
hypop
asia;
cochlear
withorwithout
progresive;
aqueduct
oIthevestlbular
bilaeral
dllation
(ommonly
until
notdiagnosed
pigmentosa
(0nqenital,
areflexia,
andretlnitis
vestibular
loss,
hearing
indude
biatera
Diagnoit
c criteria
1(USH1) USHIA,
MY07A,
USHIC,(DH23,
, andprofound
Ushersyndrometype
USHIE,
P(DHI
5,UsHlG
(U5H2) USH2A,USH28,USH2(,othe$
Ushersyndrometype2
type
3(USH3) USH]
Usher
syndrome
functron
ldnref
2005;365:879-8m
Bale
KR:Sensoaneural
hearinq
loss
inchildren
From
Smirh
RJH,
lFlrWhite
I 169 6r,
2622I PART11;1IX
syndromic recessivetypes of SNHL. Other autosomal recessive
hearing loss. Congenital anomalies causing CHL include malformations of the ossiclesand middle-ear srructures and atresia of
the external auditory canal.
Many genetically determined causes of hearing impairment,
both syndromic and nonsyndromic, do not express themselves
until some time afrer birth. Alport, Alstrom, and Down syndromes, von Recklinghausen disease, and Hunter-Hurler syndrome are genetic diseases that may have SNHL as a late
manifestation.
SNHL also may occur secondary to exposure to toxins, chemicals, and antimicrobials. Early in pregnancy, the embryo is particularly vulnerable to the effects of toxic substances.Ototoxic
drugs, including aminoglycosides,loop diuretics, and chemotherapeutic agents (cisplatin) also may cause SNHL. Congenital
SNHL may occur secondary to exposure to these drugs as well
as to thalidomide and retinoids. Certain chemicals, such as
quinine, lead, and arsenic, may cause hearing loss both pre- and
postnatally.
Trauma, including temporal bone fractures, inner ear concussion, head trauma, iatrogenic trauma (e.g., surgery, extracorporeal membrane oxygenation [ECMO]), radiation exposure, ind
noise, also may cause SNHL. Other uncommon causesof SNHL
in children include immune disease (systemic or limited to the
inner ear), metabolic abnormalities, and neoplasms of the temporal bone.
Sudden hearing loss in a previously healthy child is uncommon
but may be due to otitis media or other middle ear pathologies.
Usually these causes are obvious from the history and physical
examination. Sudden loss of hearing in the absence of obvious
causesoften is due to a vascular event affecting the cochlear apparatus or nerve, such as embolism or thrombosis (secondary to
prothrombotic conditions). Additional causesinclude perilymph
fistula, drugs, trauma, and the first episode of Meniere Jyndrome.
THBTSHOTO
AVERAGI
TEVET
(dB)AT500-2,000
Hz(ANSI) DES$lPTlotl
Normal
0-15
mnge
16-25
Slight
hearing
los
(OMMON
CAU5TS
(IFNOT
DEGRTE
OFHAIJOIffP
ON BEHEARD
WHAT
P(OBABIE
I'if[05
rN15TYRot iltf)
AMPLITICATION TRIATID
WITHOUT
hearinq
Conductive
oss
perforation,
0tltismedia,Tl\4
tympanosclerosis;
Eustachian
tubedysfunctron;
some
SNHt
Allspeech
sounds
may
Vowel
headdearly,
sounds
(0n50nanI
mt55
unvOrceo
so|]nds
N0ne
1n
dy(unoion
Mildauditory
rearn
ng
language
some
inperceiving
Difficulty
speecn
s0!n05
25 30
t\4itd
perforation,
0titis
media,TM
ero5i5,severe
tympanos(
dysfunctron,
SN|1L
eustachian
learnrng
dysfunction
sounds, Auditory
ofspeech
Hears
onlysome
language
retardation
iVild
sounds
voiced
thelouder
ptoblems
Mildspeech
lnalteftr0n
l0 50
Moderate
hearing
los
Chronic
otitis,
earcanaJ/mlddle
ear
anomaly,5Nl-1L
at
so|]nds
|\4isses
mostspeech
level
normal
conversationa
5070
hearlng
los
Severe
sound
ofnormal
Hears
nospeech
SNHL
ormixed
loss
duetoa
ronversation5
combinalon
ofmddle-ear
dsease
andsensorineural
invol\/ement
70+
los
Profound
hearing
ormixed
5NHL
orother
sounds
Hean
nospeech
problems
Speerh
retar0al0n
Language
[eaming
dysfunction
hattentr0n
probLems
speech
Severe
rebrdation
Languaqe
Learning
dysfunction
tnaIIent]0n
problems
Severe
speech
retardation
Language
on
Learninq
dystunct
l0attentr0n
None
aid;
ofneed
forhearing
Consideration
ttaining
speech
reading;
auditoty
5peech
therapy
5urgery
Appr0pnale
Preterentral
seating
Hear
ngaid
Lipreading
Auditory
training
Speech
therapy
surqery
Appr0pflate
plus
corsiderati0n
0t
Alloftheabove,
(lassfoom
situation
special
asstgnment
A| 0fth..above;pr0bable
tospecial
dases
probable
asignment
Alloftheabove;
orschools
classes
tosperial
los;Tlvl,
AN5l,
Aflrerican
Nati0nal
Standards
lnstitut;5NHL,sensorineural
hearing
tympanrr
membrane
fromNonhern
ll,DownsMP:He1in|
in(h/drc4
&Wilkins,1991
It/0dified
4thedBaltimore,Williams
AGT
DIVETOPMENT
IMO) NORMA|'
0-4
Should
startle
toloud
sounds,quiet
tomother!
voi(e,
momentarily
cease
activity
when
sound
ispresented
ataconversational
level
5-6
presented
Should
conectly
plane,
localize
tosound
inahorizontal
begin
tolmrtate
sounds
in
repert0ire
ownspee(h
0ratleast
recjprocally
vocalize
withanadult
7-12
presented
5hould
conectly
localize
tosound
inanyplane
quietly
5hould
respond
toname,
even
when
spoken
1l-15
point
Should
toward
anunexpected
sound
ortofamihar
objects
orpersons
when
asked
16-18
Should
follow
gestural
simple
directions
without
visual
orother
cues;(an
betrained
t0rea(h
toward
aninteresting
toyatmidline
when
asound
ispresented
point
19-)4
parts
Should
play
t0body
when
asked;
by21-24mo,
canbetrained
toperform
audiometry
Frcm
Matkin
ND:Eady
ncognttion
andreferral
0fhearing-impalred
children
PediltRevi984;6:i5jReprodured
bypermis\ionofPediottits
PURE-TONE
AUDIOGRAM
Frequency(cycles/sec)
125
250
500
CI
1000
2000
4000
8000
10
0
10
20
JU
40
50
60
\j
70
80
90
100
AGE
{M0)
12
18
24
l0
36
48
REFTRRAT
GUIDETINTs
F()R(HITDREN
WITH'sPEEfi"DETAY
Nodifferentiated
babbling
0rvocal
imitation
Nouseofsingle
words
Single-word
vocabulary
of<10words
<100words;
noevidence
of2-word
combinatiofls;
unintellioible
<200words;
nouseoftelegraphk
sentenceJ;
clarity
<5t)%
<600words;
nouseofsimple
se$tences
dariry<80%
FromlvatkinND:[arlyrecognitionandrfe(al0fhearing-impairedchildren
pediltRev19g4;6151
Reprodwedbypermrssionol Pediottks
110
AUDIOGRAMKEY
Air
Bone
Right
Left
5WTTP
OTAIRPRTS'URI
sFTED
<50daPa/sec*
200daPa/seC
(hildren
(l-5 yr)
Lower
limit
Median
limit
Upper
Adults
limit
Lower
Median
Upper
limit
030
055
090
056
0,85
136
036
061
106
027
072
138
*Earcanal
tail0llympanoglam
atLowett
based
0nadminance
volume
measutement
tEar
fu adultsi
for(hikkoandat+200daPa
tail0ftympanqram
atlowe$
0nadmifian(e
canal
measurementbased
daPa,detaPastah
(edimr]:fleul|r4
W5
prindples
InRintelnan
applirrmn
0fclini(al
J[Tympanometry:8asi(
RH,5hanl6
fiomMargolis
Adapted
l991,pp
179-245
/$erirnenl2nd
edAuslin,TX,PR0D[D,
*Hfl"::i:,:ii::T::
;l'#i"!'T;1il:'n:?
response.
(MO)PCVT
AGE
ATFIRST
DOSE
2-6
1-11
12-)3
24,59
>60
PCVT
STRIIS
PRIMARY
moapart'
3doses,2
1 r^.^- 1 -^.-.,rI
I U U ) r ) , 1 i l U d p d Tl
I 00se5,
I m0apart
I 00ses,
I m0apan
Notrndi(atedll
D05t
P(v7AD0tTr0r'lAt
1dose
a 12-15moofage'
1d o saet1 2 - 1 5m oo f a g e t
Notindiiated
Notindl(ated
Notindi(ated
a >24moofage!
Indicated
at>24moofageq
hdicated
at>24 moofageq
Indicated
lndicaeds
lndiGted
conjugale
varrine
shofiage
resolved
/i4MWR
2043,52.M-447
Pneumococral
)
'For
chrldren
vaccinated
atage<1 yt minimum
interval
between
doses
is4 wks
+The
(omDleled
afterthe
series
hasbeen
additional
dose
should
beadministered
28 weeks
0fimarv
(NoRfl-9)
MMI&R
2000;49
infants
0ftheAdvisory
[0mmittee
0nlmmunization
Pratti(s
andyoung
children:
Recommendations
tMinimum
intervdl
between
doses
rs8 weeks
:PtV/rsnotrecommended
generally
forchildren
aged
25 yrs
FromPneumocotcalvaccinati0nf0fc0ch|ealimplant(andidate5andrcipien6'Updatedrec0mmendati0n50ftheAdv0[y[0mm|fee0
i , ,,r. :' 'r' ; All cochlear implants share ke,v components! rncluding a
rnrcrophonc, specch proccssor, and transmitter coil' shown ln a behind-the-ear
posrtion in thrs diagram. The mlcrophone/speech processor picks up
environmental sounds and cligitises them into coded signals. The signals are
sent to thc transmitter coil and rela,ved through the skin to the internal device
imbeclclcd in the skull The internal devtce converts the code to electronic
signals, n'hrch are transmitted to the electrode arra,v wrapping arouncl the
-I'he
inset shows the radiographic appearance of the stimulating eleccochlea
trode array. Reprocluced with permission from MED-EL Corporation, Innsb r u c k , A u s t r t a . ( F r o m S m r t h R J H , B a l e J F ' J r , ! i l h r t e K R : S e n s o r i n e u r a lh e a r i n g
loss rn children. I'ancet 2005 365:879-890.)
2623rPARIXXXrTheEar
Capaccio P, Ottaviani F, Cuccarini V, et al: Sudden hearing loss and
MTHFR 677C>T/'1,298A>Cgene polymorphisms. Genet Med 2005;7:206208.
Centers for Disease Control and Prevention: pneumococcal vaccination for
cochlear implant candidates and recipients: Updated recommendations of
the Advisory Committee on Immunization practices. MMWR 2003t
52:739-740.
Cox LC: Otoacoustic emissions as a screening tool for sensorineural hearing
loss. J Pediatr 1,997;130:685-686.
Del Castillo I, Villamar M, Moreno-Pelayo MA, et al: A deletion involving the
connexin 30 gene in nonsyndromic hearing impairments. N Engl J Med
2002t345: 243-249.
Eilers RE, Oller DK: Infant vocalizations and early diagnosis of severehearing
impairment. J Pediatr 1994;124:799-203.
Ftgazzola L, Cerutti N, Mannavola D, er al: Differential diagnoses berween
Pendred and pseudo-Pendredsyndromes: Clinical, radiologic, and molecular studies. Pediatr Res 2002:51,:479484.
Gates GA, Miyamoto RT: Cochlear implants. N Engt ! Med 2003;349:
421,423.
Grote JJ: Neonatal screening for hearing impairment. Lancet 2000;355:
5 13-5 14.
Hinson Jl Fantin VR, Schcinberger
J, et al: Missensemurations in the BCSlL
gene as a cause of Bjiirnstad syndrome. N Engl I Med 2007;356:809-81,9.
Kemper AR, Downs SM: A cost-effectivenessanalysis of newborn hearing
screeningstrategies.Arch Pediatr Adolesc Med 2000;154:484.
Kennedy C, McCann D, Campbell MJ, et al: Universal newborn screeningfor
permanent childhood hearing impairmenr: An 8 year follow up of a controlled trial. Lancet 20051366:660-662.
Mason JA, Herrmann KR: Universal infant hearing screening by automated
auditory brainstem response measurement. pediatrics 1998:1,01:221228.
Moeller MP: Early intervention and language development in children who
are deaf and hard of hearing. Pediatrics 2000;106:E43.
Morell RJ, Kim HJ, Hood LJ, et al: Mutations in the connexin 26 gene(GJB2I
among Ashkenazi Jews with nonsyndromic recessivedeafness.N Engt Med
J
19 9 8 ; 3 3 9 : 1
5 0 0 - 15 0 5 .
Monon CC, Nance WE: Newborn hearing screening-a silent revolution.
N Engl J Med 2006;354:2151-2164.
Nikolopoulos TP, Archbold SM, O'Donoghue GM: Does cause of deafness
influence outcome after cochlear implantation in children? pediatrics
2 0 0 6 ; 11 8 : 1 3 5 0 - 1 3 5 5 .
Nikolopoulos TP, Dyar D, Archbold S, et al: Development of spoken language
grammar following cochlear implantation in perlingually deaf children.
Arch Otolaryngol Head Neck Surg 2004;1.30:629-633.
Niskar AS, Kieszak SM, Holmes A, et al: prevalence of hearing loss among
children 5 to 19 yearsof age.JAMA 7998;279:1.077-1075.
Pirozzo S, Papinczak I Glasziou P: Vhispered voice test for screening for
hearing impairmenr in adults and children: systematic rcview. BMJ i003;
327:967-970.
Ramsden RT: Prognosis after cochlear implantation. BMJ 2004;32g:
419420.
Reefhuis J, Honein MA, Whitney CG, et al: Risk of bacterial meningitis
in children with cochlear implants. N Engl J Med 2003;349:435445.
Schultz JM, Yang I Caride AJ, et al: Modification of human hearing loss by
plasma-membrane calcium pump PMCA2. N Engl
1 Ued ZOOS;SSi:
1 55 7 - 1 . 5 5 4 .
Smith RJH: Deafness: from bedside to bench and back. Lancet
20021360:656-657.
Smith RJH, Bale JF Jr, White KR: Sensorineural hearing loss in children.
Lancet 2005; 365:879-890.
Thomas MA, Der Kaloustian VM, Tewfik TL: Connexin mutation testing of
children wirh nonsyndromic, autosomal recessivesensorineuralhearing llss.
J O to laryngo I 2004 ;33 :189-792.
Thompson DC, McPhillips H, Davis RL, et al: Universal newborn hearing
screening:Summary of evidence.JAMA 200I;296:2000-2010.
Waltzman SB, Roland T Jr: Cochlear implantation in children younger than
12 months. Pediatrics 2005J,6:4g7493.
Willems PJ: Genetic causesof hearing loss. N Engt J Med 2000;342:7101.
Wilson C, Roberts A, StephensD: Aetiological investigation of sensorineural
hearing loss in children. Arch Dis Chitd 205:90:307-309.
Wolf B, SpencerR, Gleason T: Hearing loss is a common feature of symptomatrc children with
profound
biotinidase deficiency. pediairics
2002;'J,40:242-246.
Yoshinaga-Itano C, SedeyAL, Coulter DK, et al: Language of early- and lateridentified children with hearing loss. pediatrics 1.999;1,02:1151-7J,7
l.
The external and middle ears, derived from the 1st and 2nd
branchial arches and grooves, grow throughout puberty, but the
inner ear, which develops from the otocyst, reaches adult size and
shape by mid-fetal development. The ossicles are derived from
the 1st and 2nd arches (malleus and incus), and the stapesarises
from the 2nd arch and the otic capsule. The malleus ind incus
achieve adult size and shape by the 15th wk ofgestation, and the
stapes achieves adult size and shape by the 18th wk of gestation.
Although the pinna, ear canal, and tympanic membrane (TM)
continue to grow after birth, congenital abnormalities of these
structures develop during the 1st half of gestation. Malformed
external and middle ears may be associated with serious renal
anomalies, mandibulofacial dysostosis, hemifacial microsomia,
and other craniofacial malformations. Facial nerve abnormalities
may be associared with any of the congenital abnormalities of the
ear and temporal bone. Malformations of the external and middle
ears also may be associatedwith abnormalities of the inner ear
and both conductive (CHL) and sensorineural hearing loss
(SNHL).
children >5 yr of age, when the pinna has reached about 80% of
its adult size.
nous bacteria.
ET|OI0GY. External otitis (swimmer's ear, although it can occur
without swimming) is caused most commonly by P. aerugin-osa,
but S. aureus, Enterobacter lerogenes' Proteus mirabilis,
Klebsiella pneumoniae, streptococci' coagulase-negative staphylococci,-diphtheroids' and fungi such as Candida and
Aspergitlui alsb may be isolated. External otitis results from
pressure.
. Diffuse exrernal otitis may be confused with furuntis media (OM), and mastoiditis. Furuncles occur in
OTHER
DISEASES
OFTHEEXTERNAT
EAR
FURUNCUL0SIS.
Furunculosis is caused by S. aureus and affects
only the hair-containing outer third of the ear canal. Mild forms
are treated with oral antibiotics active against S. aureus. If an
abscessdevelops, incision and drainage may be necessary.
ACUTECEttULlTlS, Acute cellulitis of the auricle and external
auditory canal usually is caused by group A streptococcus ano
occasionally by S. aureus. The skin is red, hot, and indurated,
without a sharply defined border. Fever may be present with
Iittle or no exudate in the canal. Parenteral administration of
penicillin G or a penicillinase-resistantpenicillin is the therapy of
cnorce.
PEBICH0NDRITIS
AND CH0NDRITIS.
Perichondritisis an infection
involving the skin and perichondrium of the auricular cartilage;
extension of infection to the cartilage is termed chondritis. The
ear canal, especiallythe lateral aspect,also may be involved. Early
perichondritis may be difficult to differentiate from cellulitii
because both are characterized by skin that is red, edematous,
and tender. The main cause of perichondritis/chondritis and cellulitis is trauma (accidental or iarrogenic, laceratron or contusion), including ear piercing, especially when done through the
cartilage. The most commonly isolated organism in perichondritis and chondritis is P. aeruginosa, although other gram-negauve
and, occasionally,gram-positive organisms may be found. Treat-
I Patientage 2 W or older
with diffuseAOE
I
Prescribe
basedon painseverity
analgesics
Prescribesystemicantimicrobialactive
againsl Pseudomonas aeruginosa and
Staphylococcusaureus,with or without
topicaltherapy,plus other management
basedon underlyingcondition
Extension
outside
earcanalor host
factors*requiring
therapy?
systemic
|ruo
I
.Factorsrequiringsystemictherapyinclude
diabetes,immunedeficiency,or inabilityto
effectivelydelivertopicaltherapydespiteauraltoilet
Perforatedtympanic
membrane(knownor
suspected)or
tympanostomytube?
PrescribetopicaltheraPYwith a
non-ototoxicpreparation
Prescribetopicaltherapybasedon
benefits,cost,compliance,preference
obstructedearcanal
Yes
auraltoiletto remove
Perform
> obstructing
debris;Placewickif
drugdelivery
edemaprevents
I v""
Assessdrugdelivery
to therapy,
adherence
needto changetheraPy
Figure 63g-1. Flowchart for managing acute otitis externa.(From RosenfeldRM, Brown L, Cannon CR, et al: Clinical practiceguideline:acute otitis externa.
Ololaryngol Head Neck Surg 2006;154t54-S23. O 2005 American Academy of Otolaryngology-Head and Neck Surgery Foundation, Inc )
GENERAI
CONSIDEBATIONS
matitis is suspected.
Haddad J Jr: Care of the draining ear in children. Emerg peds 1,995;8:75.
Nussinovitch M, Rimon A, Volovitz B, er al: Cotton-tip applicators as
a leading causeof oritis externa. Int J pediatr Otolaryngol 2004;6g:433435
Roland PS,Eaton DA, Gross RD, et al: Randomized,placebo-controlledevaluation of Cerumenex and Murine ."r*"* ...ou"l products. Arch Otol a r y n g o lH e a d N e c h S u r g 2 0 0 4 ; 1 3 0 : t l 7 S - 1 1 7 7 .
Roland PS, StromanDW: Microbiology of acuteotitis exrerna.Laryngoscope
2002;712:1166-1177 .
RosenfeldRM, Brown L, Cannon CR, er al: Clinical practiceguideline:acute
otitis externa. Otolaryngol Head Neck Surg 2006;134:54-523.
Van Balen FAM, Smit WM, Zuithoff pA, et al: Clinical efficacy of three
common treatmentsin acuteotitis exrernain primary care:randomizedcon_
t r o f f e dr r i a l . B M l 2 0 0 3 ; 3 2 1 : 1 2l0- l 2 0 3 .
Otitis media (OM) is second only to rhe common cold among illnessesthat bring a child to the physician's office in the United
States. The peak incidence and prevalence is from 6-20 mo of
age. Otitis media figures importantly in the differential diagnosis
of fever, is the most common reason for prescribing antimicrobial drugs to children, and often is the sole or at least primary
basis for undertaking the operations mosr commonly performed
in infants and young children: myringotomy with insertion of
tympanostomy tubes and adenoidectomy. An important characteristic of OM is its propensiry to become chronic and recur. The
earlier in life a child experiencesthe 1st episode, the greater the
degree of subsequent difficulty he or she is likely ro experience,
in terms of frequency of recurrence, severity, and persistenceof
middle-ear effusion.
Accurate definition and diagnosis in infants and young children
often is difficult (Table 639-l). Symptoms may be absent or not
Chapter
639I otitis Media I 2633
to the mechanics of breastfeedingin a study of infants with cleft
formula) was deliv(2)thepresence
(1) a history
oflvlEE,
ofacute
onset
ofsrgns
andsymptoms,
Adiagnosis
ofA0Mrequires
andsymptoms
ofmiddle-ear
inflammation
and(3)signs
ofAOM
indudes:
Thedefinrtion
earinflammation
andMEE
abrupt,
onset
ofsigns
andsymptoms
ofmiddle
Recent,
usually
indicated
Thepresence
ofMEE,
byanyofthefollowing:
B u l g ionfgt h e T M
Lrmited
orabsent
mobility
0ftheTlil
Air-fluid
level
behind
theIM
Otorrhea
rnflammation,
indicated
byeither
orsymptoms
ofmiddle-ear
5igns
Distinct
erythema
oftheTM,or
(discomfon
wnhorpredudes
ininterference
clearly
referable
totheear[s]
thattesults
Dlstinct
otalgia
normal
artivity
orsleep)
lv1Et,
middle-ear
effrsion;]l\.4,tympanic
membrane
A0[1,
acute
otilrs
media;
PPdirl(J
Otitis
medra
Diagn0srs
and]'ranagment
0facule
0nl\4anagement
0fAcute
Otitrs
[4edia:
From
5ub(ommittee
2004;111.1451-1465
Ji;,T:i:,filiJ'"'iiH.l,'#*Jil
O
techniques. Using polymerase chain
reaction assays,middle-ear fluids have been found to contain evi-
develop in the absence of pre-existing eustachian tube obstruction.is uncertain; if so, the infection undoubtedly would lead
quickly to tubal obstruction as a consequenceof both inflammatory edema and the accumulation of middle-ear secretions.
of the tube in infants and young children may increase the likelihood of reflux from the nasopharynx and impair passive gravitational drainagethrough the eustachiantube.-Anotherpo.ribl.
explanation for improved eustachiantube function and decreased
OM incidence as infants mature is that the luminal diameter
increases, reducing the opportunity for tubal obstruction and
dysfunction.
In children with cleft palate, where OM is a nearly universal
finding, the main factor underlying the chronic middle-ear inflammation is impairment of the opening mechanism of the eustachian
tube, due perhaps ro greater-rhan-nbrmal compliance of the tubal
rent sinopulmonary infection; these deficienciesprobably underlie the susceptibility to infection. Children with recurrent OM
that is not associatedwith recurrent infection at other sites rarely
have a readily identifiable immunologic deficiency.Nonetheless,
evidencethar subtle immune deficits play a role in the pathogenesis of recurrent AOM is provided by studies involving antibody
responses to various types of infection and immunization; by
the observation that breast-milk feeding, as opposed to formula
feeding, confers limited protecrion against the occurrence of OM
in infants with cleft palate; and by studies in which young children with recurrent AOM achieved a measure of protection from
intramuscularly administered bacterial polysaccharide immune
globulin or intravenously administered polyclonal immunoglobulin. This evidence, along with the documented decreaseinlncidence of upper respirarory tract infections and OM as children,s
immune systems mature is indicative of the importance of a
child's innate immune system in the pathogenesisof OV.
Evidence that respiratory allergy is a primary etiologic agent in
OM is not convincing; however, in children who have both allergies and OM, it seemspossible rhat the otitis may be aggravated
by the allergy.
EXAMINATION
OFTHEEARDRUM
0T0SC0PY.For clinicians other than otolaryngologists, who may
use an operating microscope, the eardrum ordinarily is viewed by
means of an otoscope. Two types of otoscope heads are available:
surgical or operating, and diagnostic or pneumatic. The surgical
head embodies a lens that can swivel over a wide arc and an unenclosed light source, providing ready access of the examiner's
instruments to the external auditory canal and tympanic membrane. Use of the surgical head is optimal for removing cerumen
or debris from the canal under direct observation, and is necessary for satisfactorily performing tympanocentesis or myringotomy. The diagnostic head incorporates a larger lens, an enclosed
light source, and a nipple for the attachment of a rubber bulb
'When
an attached speculum is fitted snugly into the
and tubing.
external auditory canal, an airtight chamber is created, consisting of the vault of the otoscope head, the bulb and tubing,
the speculum, and the proximal portion of the external canal.
Although examination of the ear in young children is a relatively
invasive procedure that often is met with lack of cooperation by
the patient, this task can be enhanced if done with as little pain
as possible. The outer portion of the ear canal contains hairbearing skin and subcutaneous fat and cartilage that allow a
speculum to be placed with relatively little discomfort. Closer to
the tympanic membrane the ear canal is made of bone and is lined
only with skin and no adnexal structures or subcutaneous fat; a
speculum pushed too far forward and placed in this area often
causes skin abrasion and pain. Using a rubber-tipped speculum
or adding a small sleeveof rubber tubing to the tip of the plastic
speculum may serve to minimize patient discomfort and enhance
the ability to achieve a proper fit and an airtight seal.
Learning to perform pneumatic otoscopy is a critical skill in
being able to assessa child's ear and in making an accurate diagnosis of OM (seeFig. 635-1). By observing as the bulb is alternately squeezed gently and released, the degree of tympanic
membrane mobility in response to both positive and negative
pressure can be estimated, providing a critical assessmentof
middle-ear fluid, which is a hallmark sign of both AOM and
OME. lfith both types of otoscope heads, bright illumination
also is critical to adequate visualization of the tympanic membrane.
AUDIT0RYCANAI. If the tympanic memCIEARINGTHEEXTERNAT
brane is obscured by cerumen, the cerumen may be removed
under direct observation through the surgical head of the otoscope, using a Buck curette (N-400-0, Storz Instrument Co).
Remaining bits can then be wiped away using a Farrell applicator (N-2001A, Storz Instrument Co), with its tip (triangular in
cross section) wrapped with a bit of dry or alcohol-moistened
cotton to create a dry or wet "mop." Alternatively, gentle suction
may be applied, using a No. 5 or 7 French ear suction tube.
During this procedure it may be most advantageous to restrain
the infant or young child in the prone position, turning the child's
head to the left or right as each ear is cleared. One adult, usually
a parent, can place one hand on each of the child's buttocks and
brace the child's hips against the examining table, using his or
her own weight for additional bracing if necessary.Another adult
can restrain the child's head with one hand and the child's free
arm with the other, changing hands for the opposite ear. In children old enough to cooperate, usually beginning at about 5 yr of
common finding.
DIAGN0SIS.Correct diagnosis of AOM is important to guide clin-
membrane or otalgia (seeTable 639-7). A simplified differentiating schema establiihes a diagnosis of AOM when, in a.ddition to
having MEE, a child gives evidence of recent, clinically import".rt .-ut pain or the tympanic membrane shows marked redness
Or
Bubbles
or air-fluid
interfaces
behindtheTM
Acutepurulentotorrhea
notdueto otitisexterna
No Acute
Inflammation
At leastoneof:
1. Substantial
earpain,including
unaccustomed
tuggingor
rubbingof theear
2. Markedrednessof theTM
3. Distinct
fullnessor bulgingof theTM
(oME)
Figure 639-1. Algorithm for distinguishingbetweenacute otitis media (AOM) and otitis media with effusion (OME). TM,
tympanic membrane.
nometer measures and records the volume of the external auditory canal, and if a tympanic membrane perforation or a patent
tympanostomy tube is present, the volume of the middle ear and
masioid air cells as well. A volume reading of >1.0mL should
TREATMENT
of AOM
ofAcuteOtitisMedia.Individualepisodes
Management
customarily have been treated with antimicrobial drugs. Concern
about increasesin bacterial resistancehas prompted some authors
to recommend withholding antimicrobial treatment in some cases
unless symptoms persist for 2-3 days, or worsen (Table 639-2).
Three faitors argue in favor of routinely treating children who
have bona fide AOM (seeTable 539-1 andFig. 639-2) with an
antimicrobial drug. First, pathogenic bacteria cause a large
majority of cases.Second' symPtomatic improvement and resolution of infection occur more promptly and more consistently
with antimicrobial treatment than without, even though most
untreated caseseventually also resolve. Finally, prompt and adequate antimicrobial treatment may prevent the development of
rt
r.5
mI
1.5
ml
.5
.5
.5
- 606 -300
+3OO
daPa
-600
-300
r300
-609
deP.
-300
+300
drPr
Figure 639-4. Tympanogramsobtained with a Grason-StadlerGSI 33 Middle Ear Analyzer,exhibiting (A) high admittance,steepgradient (i.e., sharp-angled
p."t;, a"d midd'le-iar ^L pr"..r.r. approximating atmosphericpressure(0 decaPascals
[daPa]);(B) low admittanceand indeterminatemiddle-earair pressurel
and (C) somewhat low admittance,gradual gradient,and markedly negativemiddle-earair pressure.
TEMPTRATURT
>39TAND/OR
SEVERE
ATDIAG}IOSIS
TOR
PATIENTS
BEING
TRTATEO
OIATGIA
Iil|rIATI,Y
WITH
ANTIEACTTRIAI.
AGTTITS
No
Yes
CtII{IGITY
DTTIIITD
TREATMENT
FAITURE
AT
48-72HRAITER
IIIIIIAI.MANAGTMENT
I/I'ITH
OBSERVATIOI{
OPTION
O.IiII(AITY
DETITIED
TREATMTNT
TAII,URE
AT
48-72HRATTTR
II{ITIAT
MAI'IAGTMINT
WITH
AiITIBACTERNI
AGEI'IT5
AI.TTR}IATIVE
TOR
ATTERNATIVT
FOR
ATTERl{ATIVE
FOR
PENICITTINAI.TERGY
RE(OMMEI{DED
PENIflTtIN
ATTERGY
REC()MMENDED
PENICILLINATI,ERGY
Non-typel.cefdrnir,
Amoxicillin,80-90mq/kg
Non-type
1:cefdinir;
Amoxicillin-clavulanate,90mg/kg
Non-type
l:(eftriaxong3
(efuroxime,
perday
cefuroxime,
cefpodoxime; per
day
ofamoxirillin
days;
type
1:clindamyrin
cefoodoxlme;type
(omponent,with
typeliazithromycin,
64 mg/kg
1:azithromy(in,
perday
darithromycin
ofdavulanate
clarjthromycin
Amoxiciliin-clavulanate,feftriaxone,
1or3days Amoxicillin,
davulanate, Ceftriaxone,l
or3days
[eftriaxone,3
days
Tympanocentais,
dtnda
mycin
90mg/kgpu dayof
perday
90mg/kg
of
amoxkillin,
wirh64 mgikg
amoxl(itlin,with
6_4
mg/kg
perdayof(lavulanate
perday
ofclavulanate
RT(OMMEI{DTD
Amoxkillin,8O-90
mg/kg
perday
From
sJb(ommittee0n
Management
0fArute
0titisl\,4edia:Diagno5is
andmanagment
0facute
otitismediapediltti,J20Q4;113:145j-1M5
1
A diagnosisot acuteotitismedia
requrres:
1) Historyof acuteonset of signs
and symptoms
Theclinician i
assessespain. i
Yes
Clinicianrecommends
treatmentto reduce
parn.
Amoxicillinat a dose of
80-90 mg/kg/dayis the
initialantibacterialof
choicefor most children
.Criteriafor antibacterial
treatmentor observationin
childrenwith nonsevereillness:t
'1)<6 mo: antibacterial
treatment
treatmentwith certain
2) 6 mo to 2 years:antibacterial
diagnosisor severeillnessor observationwith uncertain
diagnosisand nonsevereillness
3) 2 years and older:antibacterialtreatmentif severe
illnessor observewith nonsevereillnesswith certain
diagnosis;observationfor uncertaindiagnosis.
fOaregiveris informedand agreesto the optionof
observation
Caregiveris able to monitorchild and returnshould
conditionworsen
Systemsare in placefor readycommunicationwith
and obtainingmedication
reevaluation,
the clinician,
if necessary.
Figure 639-5. Management of AOM. (From Subcommittee on Management of Acute Otitis Media: Diagnosis and management of acute otitis media. Pediatrics
Continued
204 :11.3:145 l-1. 4 6 5.1
16
Did patientrespond
to initialtreatment
intervention(either
antibacterial
treatmentor
observation)?
Clinicianreassesses
and confirms
diagnosisof AOM.
18
Assessfor othercauses
of illnessandmanage
appropriately.
Patientfollow-up
as appropriate.
19
Clinicianshouldinitiate
antibacterialtreatmentfor children
initiallymanagedwith observaton
or changeanitbacterialtreatment
for patientsinitiallymanagedwith
antibacterialtherapy
Antibacterialchoice
shouldbe basedon the
likelypathogen(s)
presentand on clinical
expenence.
Figure619-i. s6n1'd
Approximarely 50% of strains of S. pneumonlae are penicillinnonsusceptible,divided approximately equally berweenpenicillinintermediate and, even more difficult to treat. penicillin-resistant
strains. A much higher incidence of resistanci ir r..tt in children
in resistanceto clindamycin, which otherwise is generally effective against resisrant strains-of S. pneumoniae. Uilike ,.rirtr.,..
to BJactam antibiotics, macrolide resisrancecannot be overcome
by.increasing the dose. Although vancomycin previously was a
fail-safe antimicrobial in trearing S. pneum'oniae,clinical casesof
vancomycin-tolerant S. pneumoniae have been identified, further
raising the importance and hazard of antimicrobial resistance.
FIRST-tlNEANTIMICROBIAL
TREATMENT,
Amoxicillin remains the
preferred drug for uncomplicated AOM under mosr circum-
T0 FIRST-LINE
TREATMENT,
UNSATISFACTORY
RE$P0NSE
AoM is
essentiallya closed-spaceinfection, and its resolution depends on
both eradication of the offending organism and restoration of
middle-ear ventilation. Factors contributing to unsatisfactory
response to first-line treatment, in addition to inadequate antimicrobial efficacy, include poor compliance with treatment
regimens, concurrent or intercurrent viral infection, persistent
eustachian tube dysfunction and middle-ear under-aeration, reinfection from other sites or from incompletely eradicated middle
ear pathogens, and immature or impaired host defenses.Despite
these many potential factors, switching to an alternative or
secondline drug is reasonable when there has been inadequate
improvement in symptoms or in middle-ear status as reflected in
the appearance of the tympanic membrane, or when the persistence of purulent nasal discharge suggeststhat the antimicrobial
drug being used has less than optimal efficacy. SecondJine drugs
also may be used appropriately when AOM develops in a child
already receiving antimicrobial therapy or in an rmmunocompromised child, or in a child with severesymptoms whose previous experience with OM has been problematic.
'When
TREATMENT.
SECOND-LINE
treatment of AOM with a firstline antimicrobial drug has proven inadequate, a number of
second-line alternatives are available (see Table 639-3). Drugs
chosen for second-line treatment should be effective against Blactamase-producing strains of H. influenzae and M. catarrhalis
and against susceptible and most nonsusceptible strains of S.
pneumoniae. Only 3 drugs have been shown clearly to meet
that requirement: amoxicillin-clavulanate, cefuroxime axetil, and
intramuscular ceftriaxone. Because high-dose amoxicillin
(80-100 mg/kglza\ is effective against most strains of S. pneumoniae and because the addition of clavulanate extends the
effective antibacterial sDectrum of amoxicillin to include
p-lactamase-producing
high-dose
amoxicillinbacteria,
clavulanate is particularly well suited as a second-line drug for
treating AOM. The 14 : 1 amoxicillin-clavulanate formulation
contains twice as much amoxicillin as the previously available
7 : 1 formulation. Diarrhea, especially in infants and young children, is a common adverse effect, but may be ameliorated in some
cases by feeding yogurt, and usually is not severe enough to
require cessationof treatment. The 2 other drugs have important
limitations for use in young children. The currently available suspension of cefuroxime axetil is not palatable, and its acceptance
is low. Ceftriaxone treatment entails both the pain of intramuscular iniection and substantial cost; the inlection may need to be
repeated once or twice at 2- or 3-day intervals to achieve the
desired degree of effectiveness.Nonetheless, use of ceftriaxone is
appropriate in severecasesof AOM when oral treatment is not
penicillin-nonsusceptibleS. pneumoniae.
Myringotomy is a timeMYRING0T0MYAND TYMPAN0CENTESIS.
honored treatment for AOM but is not commonly needed in chil-
MANAGEMENT
OFOTITIS
MEDIA
WITHEFFUSION
To determine the course of an episode of OME, and to distinguish between persistenceand recurrence,examination should be
conducted monthly until resolution, and hearing should be
assessedif effusion has been present for >3 mo (see Fig. 639-1,1.
Rational management of OME depends to a considerable degree
on understanding its natural history and its possible complications and sequelae.Most cases of OME resolve without treatment within 3 mo; limited data are available concerning the
subsequentlong-term course of children in whom OME does not
VARIABTESINFTUENCING
MANAGEMENTDECISIONS
FOROTITIS
MEDIA WITH EFFUSION,
Patient-related variables that affect decisions on how to manage OME include the child's age; the frequency and severity of previous episodesof AOM and the interval
since the last episode;the child's language development; the presence or absence of a history of adverse drug reactions, concrrrrent medical problems, or risk factors such as daycare attendance
CREA5T
Maybeabsent
Ac|rte
mastoiditis
withperiosteitis
withsubpenostea
absces
Absent
Arute
mastoidrtis
Periosteitis
0fplnna
wrthponauri(ular
extensi0n lnta(t
Inta(t
withp0staurirular
extension
txternai
otitls
ymphadenitis
Inuct
Postauricular
*Postduflcular
(fold)
pinna
area
cfease
between
andposlauticular
the skin of the external auditory canal resulting from contamination by purulent discharge from the middle ear. The skin often
is erythematous, edematous, and tender' Management consists
of proper hygiene combined with systemic antimicrobials and
otoiopical drops as appropriate for treating AOM and tube
otorrnea.
required.
ACUTEMAST0|D|T|S.Technically, all cases of AOM are accompanied by mastoiditis by virtue of the associatedinflammation of
ihe mastoid air cells. However, early in the course of the disease
EXTI
RNAI.(ANAI-INFECTION
ANDSYMPTOM5
POSTAURI(UUA
SIGI{s
TENDERNESS
ERYTHEMA MASS
NO
Usually
NO
Ie5
No
Yes
Yes
Maybe
NO
Usually
No
Ies
Yes
Usually
No
Ie5
No
([ircumscrrbed) Maybe
Yes
NO
p l3l
WB5aunde6,
2001,
edPhiladeLphta,
Media
inlnfanrs
antl(hildren,3i
From
Blrestone
[D Klein
] a kditots).)riils
ly
Usua
Usually
NO
t\0
NO
2644r PART
XXIXr TheEar
centmastoiditis;
righrmasroidis normal.Mastoidsurgerywasperformed
in
thiscase.(FromBluestone
CD, KleinJO [edirors]:
OtitisMediain Infantsand
Children,3rded.Philadelphia,
WB Saunders,
2001.,
p 337.1
osteitis require intravenous antimicrobial treatment and mastoidectomy, with the extent of the surgery depending on the
extent of the disease process. As far as possibl-, choice of the
antimicrobial regimen should be guided by the findings of microbiologic examination.
Each of the variants of mastoiditis also may occur in subacute
o_rchronic form. Symptoms are correspondingly less prominent.
Chronic masroidiris always is accompanied by chronii suppurative OM, and occasionally will respond to the conseivative
regimen recommended for that condition. In most cases.however.
mastoidectomy also is required.
FACIALPABALYSIS.
The facial nerve, as ir traversesthe middle ear
temporal bone, lined by keratinized, stratified squamous epithelium and containing desquamated epithelium and/or keratin
(see Chapter 537). Acquired, as distinct from congenital,
cholesteatoma most often develops as a complication of longstanding chronic OM. However, the condition also may develop
from a deep retraction pocket of the tympanic membrane or
as a consequence of epithelial implantation in the middle-ear
cavity from traumatic perforation of the tympanic membrane or
insertion of a tympanosromy tube. Cholesteatomas tend to
expand progressivelS causing bony resorption, and may extend
intracraniallS with potentially life-threatening consequences.
Cholesteatoma should be suspectedif otoscopy shows a discrete,
whitish opacity of the eardrum or a polyp protruding through a
defect in the eardrum; or white caseousdebris persistently overlies the eardrum, especiallyits superior portion; or persistentmalodorous aural discharge is present. When cholesteatoma is
suspected, consultation with an otolaryngologist should be
sought immediately. Delayed recognition and treatment can have
significant long-term consequences,including the need for more
extensive surgical treatment, permanent hearing loss, facial nerve
injury, labyrinthine damage with loss of balance function, and
intracranial extension. Tympanomastoid surgery is required for
treatment of cholesteatoma.
INTRACRANIAT
COMPTICATIONS
Meningitis, epidural abscess,subdural abscess,focal encephalitis,
brain abscess,lateral sinus thrombosis (also called sigmoid sinus
thrombosis), and otitic hydrocephalus each may develop as a
complication of acute or chronic middle-ear or mastoid infection, through direct extension, hematogenous spread, or thrombophlebitis. Bony destruction adjacent to the dura often is
involved, and a cholesteatoma may be present, In a child with
middle-ear or mastoid infection, the presence of any systemic
symp-tom, such as fever, headache, or lethargS of extreme degree,
or a finding of meningismus or of any central nervous systemiign
on physical examination should prompt suspicion of an intracranial complication.
'When
an intracranial complication is suspected,lumbar puncture should be performed only after imaging studies establishthat
there is no evidenceof mass effect or hydrocephalus. In addition
to examination of the cerebrospinal fluid, culture of middle ear
exudate obtained via tympanocentesismay identify the causative
organism, thereby helping guide the choice of antimicrobial
drugs, and myringotomy should be performed to permit middleear drainage.
PHYSICAL
SEOUETAE
PREVENTION
DEVE[OPMENTAI
SEOUETAE
POSSIBIE
Permanenthearing loss in children has a significant negative
impact on development,particularly delays in speechand languige. The degreeto which OM impactslong-term development
in children is difficult to assess,and there have been conflicting
studies examining this question. However, the developmental
impact is most likely to be significant in children with greater
levils of hearingloss, hearingloss that is sustainedfor longer
periodsof time, and hearingloss that is bilateral,and in those
children who haveother developmentaldifficultiesor risk factors
for developmentaldelay.
IMMUNOPROPHYTAXIS
Heptavalentpneumococcalconiugatevaccinereducesthe overall
number of episodesof AOM by only 6-87", but with a 57%o
reductionin ierotype-specificepisodes.Reductionsof 9:2.3o/oare
seenin children with historiesof frequent episodes,and a 20"/"
reduction is seenin the number of children undergoing rympa-
PROPHYTAXIS
ANTIMICROBIAT
OF
ANDINSERTION
MYRINGOTOMY
TUBES
WMPANOSTOMY
In children with persistent OME, several studies have shown tympanostomy tube insertion to be effective in: reducing their subiequent proportion of time with MEE, improving their hearing
ADENOIOECTOMY
Adenoidectomy is efficacious ro some exrenr in reducing the risk
of subsequent recurrences of both AOM and OME in children
who have undergone tube insertion and in whom, after extrusion
of tubes, OM continues to be a problem. Efficacy appears to be
independent of adenoid size, and probably derives from removal
of a focus of infection. In younger children with recurrent AOM
who have not previously undergone tube insertion, however, adenoidectomy usually is not recommended along with the tube
insertion, unless significant nasal airway obstruction or recurrent
rhinosinusitis is associated, in which case, performing adenoidectomy might be considered.
Genetic factors may affect the anatomy and function of the inner
coccal meningitis, the meta-analysis showed a benefit of dexamethasone only when given early and only for protection against
severe hearing loss, Too few cases of meningococcal meningitis
were included to assessthe effect of dexamethasone on this
entlty.
SYPHftlS. Congenital syphilis, caused by Treponema pallidwm,
may cause SNHL in 3-38% of affected children (see Chapter
215). The exact incidence is difficult to ascertain, because the
hearing loss may not develop until adolescenceor even adulthood. When the condition is identified, treatment with antibiotics
and corticosteroids may improve the hearing loss.
0F THE lt{NEREAR.Labyrinthitis may be a com0THERDISEASES
plication of direct spread of infection from acute or chronic otitis
media (OM) or mastoiditis and also may complicate bacterial
meningitis as a result of organisms entering the labyrinth through
the internal auditory meatus, endolymphatic duct' perilymphatic
duct, vascular channels, or hematogenous spread. Clinical manifestations of labyrinthitis may include vertigo, dysequilibrium,
deep-seatedear pain, nausea, vomiting, nystagmus, and SNHL.
Acute suppurative labyrinthitis, charactetized by abrupt, severe
onset of these symptoms, requires intensive antimicrobial
therapy. If it is secondary to OM, otologic surBery may be
requiied to remove underlying cholesteatomaor drain the middle
ear and mastoid, in addition to antibiotics. Acute serous
labyrinthitis, with milder symptoms of vertigo and hearing loss,
may develop secondary to middle-ear infection as well. It usually
responds well to antibiotics and corticosteroids, with improvement in both vertigo and hearing. Chronic labyrinthitis, most
commonly associatedwith cholesteatoma, presents with SNHL
and vestibular dysfunction that develops over time; surgery is
required to remove the cholesteatoma.Chronic labyrinthitis may
alsb occur uncommonly secondary to long-standing OM' with
the slow development of SNHL, usually starting in the higher frequencies,and possibly with vestibular dysfunction' AdditionallS
jnd more commonly, children with chronic middle-ear fluid often
are unsteady or off balance, a situation that improves immediately when the fluid resolves.
Otosclerosis. an autosomal dominant diseasethat affects only
progresslve.
Osteopetrosis, a very uncommon skeletal dysplasia' may
involve the temporal bone, including the middle ear and ossicles'
resulting in a moderate to severe,usually conductive hearing loss.
Recurrent facial nerve paralysis also may occur as a result of
excessbone deposition; with each recurrence' less facial function
may return (see ChaPter 597).
2648
---dim
Joseph Maddad Jr;
I
I I
!&L&iL.
"'I-"
L~
Hough JVD, Stuart WD: Middle ear iniuries in skull trauma. Laryngoscope
1968; 78:899-937.
Suspicion for tumors of the ear and temporal bone may be raised
by the appearanceof a mass in the middle ear or mastoid, as well
as signs of bleeding or local destruction. Imaging with CT or MRI
allows for biopsy guidance to confirm the diagnosis and establish a treatment regimen.
Benign tumors of the external canal include osteomas and
monostotic and polyostotic fibrous dysplasia. Osteomas present