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Applicants!
November 3, 2011
Patient’s Audiogram
Normal Audiogram
Classification
 *Conductive→
involving any cause that in some
way limits the amount of external sound that
gains access to the inner ear (usually
temporary)
• More common in children
 Sensorineural→
involving the inner ear,
cochlea, or the auditory nerve (more likely
permanent)
 Mixed→
which is a combination of conductive
and sensorineural hearing loss.
 Central→
involving higher brain centers.
Differential of Hearing Loss
Conductive (outer and
middle ear)
Sensorineural (inner
ear)
Congenital microtia/atresia
Genetic disorders
External otitis
In utero infection (CMV,
measles, mumps, rubella,
varicella, syphilis)
Otitis media +/- effusion
Anatomic abnormality of cochlea
or temporal bone
Trauma
Ototoxic drugs
Foreign Body (cerumen)
Hyperbilirubinemia
*Ossicular disruption (usually
after trauma)
Infection (meningitis, Lyme,
Fifth dz, syphilis)
Cholesteatoma
Noise exposure
TM perforation
Radiation
Temporal bone trauma
Ischemia
Introduction
 Hearing
loss in the first years of life can
cause delays in speech, language, and
cognitive development
1
to 6 per 1,000 newborns and 2 per 1,000
young children
 With
early intervention, hearing-impaired
can reach same skill level of hearing peers
Question
 3-day-old
infant failed otoacoustic emission
testing (OAE). Prenatal and family histories
are negative. Physical exam is normal.
 The
next most appropriate step is:
• A. Confirm the hearing loss with an auditory
brainstem response test
• B. Perform renal ultrasound
• C. Refer to audiology at 1 year of age
• D. Repeat OAE in 6 months
• E. Treat with oral abx for possible middle ear
effusion
Screening
 AAP
recommends congenital hearing loss be
detected by 1 month, diagnosed by 3 months,
and intervention started by 6 months
 Every
state has universal newborn hearing
screening
• Otoacoustic emission testing (OAE) – detects evoked
sound from the cochlea in response to clicks and
tones
• Auditory brainstem response (ABR) – measures the
electroencephalographic waveform response from the
vestibulocochlear nerve

 All
Used to confirm abnormal OAE
children ages 4 years and older should be
screened regularly for hearing loss
Acquired Hearing Loss
 History
• Onset
• Drainage
• Tinnitus, vertigo, disequilibrium
• Kidney or cardiac disease
• Congenital infection, recurrent OM, meningitis
• Trauma – noise exposure
• Prematurity, hyperbilirubinemia
• Family hx of hearing problems
Acquired Hearing Loss
 Physical
exam
• Good head and neck exam
• Ear exam

Pneumatic otoscopy is performed with positive and
negative pressure to evaluate mobility of the tympanic
membrane

Diminished or absent tympanic membrane mobility
can be caused by fluid, mass in the middle ear cavity,
perforation, pressure equalization tube, or sclerosis of
the tympanic membrane.

Increased mobility of the tympanic membrane can
indicate ossicular chain disruption.
Question
 Which
of the following statements regarding
hearing loss in infants and children is TRUE?
• A. Chidren with external ear anomalies have
sensorineural hearing loss more than conductive
hearing loss
• B. Cholesteatoma is the most common cause of
conductive hearing loss
• C. Language delay does not occur unless hearing loss
is severe or profound
• D. Newborn hearing screens should be performed
only on premature infants or those with family hx
• E. Parental concern for language delay is sufficient
cause for auditory testing
Office Hearing Tests
 256
Hz and 512
Hz tuning forks
•
Hearing loss at
256 Hz is
equivalent to
approximately 10
to 15 decibels (dB),
and hearing loss at
512 Hz is
equivalent to 20 to
30 dB
Office Hearing Tests

Pure tone audiometry
• Softest decibel threshold
at which the child can
hear a sound 50 percent
of the time for each
tested frequency
•
Normal hearing
threshold is between 0 to
20 dB
• Accurate results are
dependent upon
cooperation from the
child
• Any threshold >20 dB
suggests a hearing loss
(conductive,
sensorineural, or mixed).
Office Hearing Tests

OAE

Tympanometry
• Valuable in
conjunction with
pneumatic otoscopy
• *Flat tympanograms
(type B
tympanograms)
and/or abnormal
OAE testing may
indicate conductive
hearing loss, usually
due to middle ear
effusion
Referral
 Any
abnormal hearing screenings in the
office should be followed by formal
audiology
Lab work-up
Lab work-up
Etiologies of
Acquired Conductive
Hearing Loss
 *Otitis
media with effusion
• Fluid filling the middle ear space prevents the
tympanic membrane from vibrating adequately
• Persistence of asymptomatic effusion following an
episode of acute otitis media:

40% at 1 month

20% at 2 months

10% at 3 months
• These pts. may benefit from tympanostomy tubes
Etiologies of Acquired
Conductive Hearing Loss
 TM
Perforation
 Foreign
Body
Etiologies of Acquired Hearing
Loss

Cholesteatoma
• Benign growth of
stratified, squamous
epithelium filled with
desquamated cells
and keratin
• Erodes ossicular
chain
• Results from poor
eustachian tube
function
Question
A
newborn who failed hearing screen, also has
microcephaly, enlarged liver, and weight at 5th
percentile. Pregnancy was uneventful, with
normal prenatal labs, and there is no family
history of hearing impairment.
 Most
likely cause of hearing impairment is:
• A. Alport syndrome
• B. Congenital CMV
• C. Middle ear effusion
• D. Prenatal measles exposure
• E. Waardenburg syndrome
Etiologies of Hereditary
Sensorineural Hearing Loss
 Usher
syndrome
 Pendred
syndrome
 Waardenburg
 *Alport
syndrome →
syndrome
• Glomerular disease, ocular abnormalites, SNHL
(progressive), family hx of hematuria, renal failure
and deafness
 Mitochondrial
disorders
Etiologies of Acquired
Sensorineural Hearing Loss
 Bacterial
meningitis
• *Most common cause of acquired deafness in
childhood
• 2.5 to 18%
• Occurs early in the course (within first 48 hours)
• *Prompt and complete hearing evalutaion initially
and regular follow-up
Etiologies of Acquired
Sensorineural Hearing Loss
*Ototoxic drugs
Aminoglycosides (gent, tobra, amikacin, neomycin)
Erythromycin
Vancomycin
Tetracycline
Chemotherapy (cisplatin)
Salicylates (reversible)
Antimalarials
Etiologies of Acquired
Sensorineural Hearing Loss

Noise exposure (acoustic
trauma)
• *High frequency hearing loss
• Constant exposure to loud
noises (personal listening
devices, power tools,
firearms) greater than 85 dB

Or sudden noise
(firecracker) greater than
140 dB
• 12.5% of kids (age 6 to 19)
have hearing loss in one or
both ears
Etiologies of Acquired
Sensorineural Hearing Loss

Trauma
• Temporal bone fracture

Bleeding from ear or
hemotympanum
Treatment
 Multidisiplinary
approach
• Audiology
• ENT
• Speech
• Genetics
 Optho
referral
• Patients rely on sight for communication and
learning
 Treatment
 Hearing
based on etiology
aids
 Cochlear
implant
Noon conference
Toddler Behavior, Dr. Nass
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