ARTICLE

Early Identification and Intervention for

Children Who Are Hearing Impaired
Katheryn Rupp Bachmann, PhD* and Joan C. Arvedson, PhD
Factors Contributing to
Delays in Diagnosis

IMPORTANT POINTS
1. Parental concern about a childs hearing should precipitate an
immediate referral to an audiologist.
2. Audiologists can test the hearing of children at any age.
3. A child who has suspected or diagnosed global delays or speech and
language delays should be referred promptly for audiologic testing.
4. Children who have severe emotional or neurological impairment can
be tested accurately by using evoked response testing.
5. Early diagnosis and management of children who have all degrees
and types of hearing impairment can be attained through heightened
awareness of physicians and other health professionals to the indicators for hearing loss and the need to develop a strong coalition with
a licensed audiologist.

Introduction
The prevalence of hearing loss
among newborns and infants in the
United States is estimated to be 1.5
to 6 per 1,000 live births. This estimate, however, is based on the number of children who are profoundly
deaf and does not account for infants
who are mildly or moderately to
severely hearing impaired. Thus, the
true prevalence is no doubt much
higher. More alarming is the fact
that the average age at which a child
who has a profound, bilateral, sensorineural hearing loss is identified
is 24 months, while hearing impairments of lesser degrees often are
identified at an average age of 48
months of age. The impact of these
statistics is disturbing because the
critical period for language learning
is within the first 36 months of life.
Thus, undetected or late detection
of significant hearing impairment in
infants and young children results
in lifelong disability.

Early Detection and

Intervention Efforts
Late detection of significant hearing
impairment not only affects a childs
*Associate Director of Audiology, SpeechLanguage-Hearing Department, Childrens
Hospital of Buffalo.
Director, Speech-Language-Hearing
Department, Childrens Hospital of Buffalo,
Buffalo, NY.
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speech and language learning, but

it can result in academic failure,
which in turn severely limits career
options. Ultimately, the independence of the hearing impaired individual is compromised. One nationwide focus of health professionals
and the federal government is to
improve methods for early identification of hearing impairment. In
1990, as one of the initiatives in the
Healthy People 2000 document, the
United States Department of Health
and Human Services stated that by
the year 2000, identification of children who are hearing impaired will
be reduced to 12 months of age or
younger. In accordance with this
initiative, the National Institutes of
Health, in a consensus statement,
and the Joint Committee on Infant
Hearing endorsed universal detection of and intervention for hearing impaired infants. In addition,
amendments to the Education of
the Handicapped Act (PL 99-457)
mandate services for children who
have handicaps (including hearing
loss) as part of a comprehensive,
multidisciplinary early intervention
effort. Despite these initiatives,
many caregivers remain unaware
of the laws that support them in
acquiring early intervention services
for their children. In these cases,
health professionals have a responsibility to inform caregivers of their
rights and to advocate for them and
their child.

Physicians and other health professionals need to work with each

other and with caregivers to identify
hearing loss and provide intervention. Pediatricians have the opportunity to evaluate infants on multiple
occasions in early infancy and several more visits before the age of
24 months. During these visits,
the physician can listen to parental
concerns about a childs speech,
language, and auditory development.
Parental concern is of greater predictive value than the informal
behavioral examination performed
in the physicians office. According
to one investigation, parents are usually 12 months ahead of physicians
in identifying their childs hearing
loss. In addition, knowledge of the
associated hearing loss that occurs
in craniofacial syndromes, among
children who have developmental
(especially speech) delays, and in
other medical conditions that place
children at risk for hearing loss
will ensure the earliest possible
diagnosis and contribute greatly
to successful management of the
hearing disorder. There is no place
for the wait and see approach in
diagnosing hearing loss.
The purpose of this review is to
provide clinicians with information
about: the development of the ear
and the onset of hearing that is nec-

ABBREVIATIONS
ABR:
BOA:
COR:
EAC:
OAE:
SDT:
SNR:
SPL:
SRT:
VRA:

auditory brainstem response

behavioral observation
audiometry
conditioned orientation
reflex
external auditory canal
otoacoustic emissions
speech detection threshold
speaker-to-noise ratio
sound pressure level
speech recognition
thresholds
visual reinforcement
audiometry

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EAR DISORDERS
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TABLE 1. Auditory Structures and Associated Organ Systems that Develop from the
Embryologic Germ Layers
GERM LAYER

ECTODERM

MESODERM

ENDODERM

Auditory structures

Pinna

Ossicles (malleus, incus,

stapes)

Tympanic membrane (TM)

medial layer

Cartilaginous portion
of external auditory
canal

Mastoid process

Eustachian tube

Membranous portion of
cochlea up to spiral
ganglion

Middle layer of TM

Lower half of middle ear

Outer layer of TM

Temporal bone (including

osseous labyrinth of
inner ear)

Central nervous system

Skeletal system

Mucous lining of all body

cavities

Visual system

Circulatory system

Digestive system

Outer layer of skin

Excretory system
Reproductive system

Sinuses
Internal organs
Inner lining of blood vessels

Associated organ systems

essary to identify audiologic health

care needs of infants and children;
indications for referral for audiologic evaluation; the components of
audiologic testing; degrees and types
of hearing loss; and management
of the hearing impaired child.

systems of the body associated with

the three germ layers from which all
systems and structures develop are
shown in Table 1. (See Suggested
Reading for detailed reviews of
auditory development by Peck
and by Northern and Downs.)
ONSET OF HEARING

Development of the Ear

and the Onset of Hearing
in Humans
DEVELOPMENT OF THE EAR

The first signs of ear development

occur at about the third week of
gestation, when the neural tube is
forming. The five branchial arches
noted at this time encompass five
branchial grooves or gill slits and
their corresponding pharyngeal
pouches. As the ear begins to take
its final shape, the outer ear forms
from ectodermal tissue of the first
and second branchial arches. The
middle ear ossicles (malleus, incus,
and stapes) arise from mesodermal
tissue. The malleus and incus arise
from mesoderm of the first branchial
arch, and the stapes arises from the
second branchial arch. The middle
ear lining and eustachian tube
develop from endodermal tissue
of the first pharyngeal pouch.
The auditory structures and organ
156

Around the 20th week of gestation,

the outer ear has developed and
taken on its adult shape. It will
continue to grow in size, however,
until a child reaches age 9 years.
The middle ear ossicles are almost
completely ossified, and the inner
ear has achieved adult size, although
differentiation of the sensory cells
(outer and inner hair cells) in the
organ of Corti is still underway. By
24 weeks, the human cochlea and its
end organ have matured structurally.
Myelination of the ganglion nerves
at this same time may be taken as
evidence that the onset of hearing
probably occurs in the sixth month
of gestation.
Knowledge of normal human
embryology and the associations of
each organ system with its respective developmental germ layer
enables the health-care professional
to be alert to the presence of physical anomalies and the possibility of
related structural defects that include

ear and hearing abnormalities. For

example, hypertelorism and abnormal pigmentation of the skin, hair,
or eyes (eg, Waardenburg syndrome)
may be associated with cochlear
pathology because these anomalies
are related to disturbances of the
surface ectoderm during development. Anomalies associated with the
first and second branchial arches
may include mandibular hypoplasia,
an abnormally formed external ear,
and the presence of preauricular pits
or tags. In one study, nearly 50%
of 46 children who were diagnosed
with permanent hearing loss showed
signs of associated structural deformities and related visible physical
anomalies.

Referral for Audiologic

Evaluation
Children who have structural anomalies or syndromes known to be
associated with hearing impairment
should be referred promptly for
audiologic evaluation. Other referral
criteria include significant perinatal
events and medical history that may
be associated with sensorineural
hearing impairment. Further, children who have suspected global
delays or speech and language
delays should be referred. The

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high-risk indicators identified by the
Joint Committee on Infant Hearing
can guide physicians for referral of
infants and children for hearing
assessment (Table 2). Awareness of
expected speech/language/auditory
milestones is helpful in noting lack
of achievement or delayed attainment of specific milestones (Table
3). A checklist can aid professionals
in obtaining important information
from parents and medical records.

Audiologic Evaluation
Recent advances in technology have
made available an extensive battery
of tests for evaluating all parts of
the auditory system. A test battery,

rather than a single test, is necessary

for pediatric assessment to compare
and perform a cross-check of all
test results.
OUTER EAR

The outer ear is evaluated both visually and by otoscopic examination.

The size and shape of the pinnae
should be observed, as should the
presence or absence of skin tags or
ear pits on or near the pinnae. The
examiner also should note the presence of abnormal skin growths,
debris, or excessive cerumen that
may block the auditory signal from
reaching the inner ear. Finally, the
examiner should visualize the familiar landmarks on the tympanic mem-

brane (TM) (cone of light, umbo,

long and short crus of the malleus).
MIDDLE EAR

The middle ear status and function

are evaluated via acoustic immittance testing. The complete acoustic
immittance test procedure consists
of tympanometry, the physical
volume test, and stapedial reflex
measures.
Tympanometry

Tympanometry measures the energy

transfer of the ear as a function of
air pressure. It involves sealing the
external auditory canal (EAC) with
a tightly fitting probe tip that contains three tubes: one connected to

TABLE 2. High-Risk Indicators for

Hearing Loss*

TABLE 3. Expected Speech-Language-Auditory

Milestones*

Checklist of high-risk indicators for hearing loss in

children birth to 24 months of age. These indicators
are red flags and may assist physicians in making
referrals of children for audiologic testing.

Checklist of selected speech-language-auditory milestones achieved by infants and children who have intact
cognition and hearing. Failure to achieve these milestones by expected age ranges may relate to hearing
loss that necessitates audiologic testing.

Birth to 28 days
___ Family history of sensorineural hearing loss
(SNHL), congenital
___ In utero infection associated with SNHL
(eg, toxoplasmosis, cytomegalovirus, syphilis)
___ Craniofacial anomalies
___ Hyperbilirubinemia at levels requiring
exchange transfusion
___ Birthweight <1,500 g
___ Bacterial meningitis
___ Low APGAR scores, ___ 03, 5 min;
___ 06, 10 min
___ Respiratory distress (meconium aspiration)
___ Prolonged mechanical ventilation >10 d
(eg, persistent pulmonary hypertension)
___ Ototoxic medications (eg, gentamicin) >5 d
or used in combination with loop diuretics
___ Stigmata or features associated with a syndrome
known to include SNHL (eg, Wardenburg
syndrome)
29 days to 24 months
___ Parental concern about: hearing, speech/
language, and/or developmental delay
___ Any of the newborn risk factors listed above
___ Head trauma with fracture of temporal bone
___ Childhood infectious diseases associated with
SNHL (eg, mumps, measles)
___ Neurodegenerative disorders, demyelinating
disease
___ Recurrent or persistent otitis media
*Adapted from Joint Committee on Infant Hearing. 1994
position statement. Audiol Today. 1994;6:69.

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Birth to 3 months
___ Startles to loud noise
___ Awakens to sounds
___ Blink reflex or eye widening to noises
3 to 4 months
___ Quiets to mothers voice
___ Stops playing, listens to new sounds
___ Looks for source of new sounds not in sight
6 to 9 months
___ Enjoys musical toys
___ Coos and gurgles with inflection
___ Says mama
12 to 15 months
___ Responds to his/her name and no
___ Follows simple requests
___ Expressive vocabulary of 3 to 5 words
___ Imitates some sounds
18 to 24 months
___ Knows body parts
___ Expressive vocabulary minimum of 20 to 50 words
(uses two-word phrases)
___ 50% of speech intelligible to strangers
By 36 months
___ Expressive vocabulary of 500 words (uses 4 to
5 word sentences)
___ Speech is 80% intelligible to strangers
___ Understands some verbs
*Adapted from Northern J, Downs M. Hearing in Children. 4th ed.
Baltimore, Md: Williams and Wilkins, 1991.

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an oscillator-receiver for the presentation of a tone, one connected to a
microphone for monitoring sound
pressure level (SPL), and one leading to a pump-manometer that both
varies and measures air pressure in
the ear canal. A fixed-frequency tone
is presented to the ear while ear
canal air pressure is varied from
+200 mm to 400 mm H2O. Sound
reflection is monitored continuously
as a function of TM compliance or
mobility, which is related directly to
the artificial variation of ear canal
air pressure. With extremely positive
or negative ear canal pressures, the
compliance of the TM is reduced
and the majority of the sound energy
presented to the ear is reflected to
the measuring microphone in the
probe. As the pressure in the ear
canal approaches the value of the
pressure in the middle ear, the tympanic membrane becomes more
compliant until it reaches a point of
maximum compliance, where the
pressure in the canal is equal to that
in the middle ear space. At maximum compliance, sound energy is
transmitted through the TM, and
little acoustic energy is reflected.
The measurement of ear canal SPL
as a function of pressure changes
provides a direct assessment of TM
compliance, which is plotted as a
tympanogram.
In 1970, Jerger described a classification system for determining the
status of the middle ear based on
the tympanogram and maximum
compliance values (peak amplitude
measures) (Fig. 1). Type A tympanograms are characterized by a
maximum compliance peak at or
near 0 mm H2O (also measured as
0 daPa) and are measured consistently in normal ears. Type A tympanograms also are measured in
otosclerotic ears, but peak amplitude
measures may be reduced due to the
increased stiffness of the ossicular
chain. These reduced compliance
tympanograms also are referred to
as type AS. In cases of ossicular discontinuity, the compliance of the
TM is increased, often beyond the
limits of the equipment, resulting in
a tympanogram classified as type
AD. Type B tympanograms are characterized by their flat line appearance, which indicates immobility of
the TM, resulting in no recording of
158

FIGURE 1. Tympanometric types based on peak amplitude and pressure values, as

described by Jerger.

a maximum compliance peak in

the tracing. Middle ear effusion is
the most consistent diagnosis associated with a type B tympanogram,
although TM perforations also generate this type. Type C tympanograms are similar to type A, but
the maximum compliance peak is
shifted to the left, beyond 100 mm
H2O. This tympanometric function
indicates significant negative middle
ear pressure and usually is recorded
just prior to, or during resolution
of, otitis media with effusion.
Type C tympanograms also may
be recorded in circumstances in
which eustachian tube dysfunction,
an inability to aerate the middle
ear space efficiently, is suspected.
According to recent studies, and
as stated in the American Academy
of Audiology Position Statement on
identification of hearing loss and
middle ear dysfunction in preschool
and school-age children, measures
of tympanic width in conjunction
with static compliance and peak

pressure measures may improve the

sensitivity of middle ear screening.
Generally, normal tympanic width
measures for children should be less
than 150 daPa. Children who have
a history of middle ear disease,
however, often exhibit wide tympanograms (>150 daPa), even in the
absence of middle ear pathology.
Thus, peak pressure, tympanic
width, and static compliance measures should be viewed together,
along with acoustic reflex data and
physical volume measures, to determine any middle ear pathology.
Physical Volume Test

The physical volume test enables

calculation of the physical volume
of the EAC and is extremely useful
in determining TM integrity or ventilation tube patency. When the ear
canal is sealed, the SPL measured
in the enclosed cavity is related
directly to cavity size. Normal physical volume measures in infants and
children range from 0.3 to 1.0 mL

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and depend on ear canal diameter
and probe tip insertion. Volumes
less than 0.3 mL may indicate either
that the probe tip is malpositioned
against the ear canal wall or that
excessive cerumen is present. In
both instances, a tympanogram that
displays a type B function (flat line)
may be recorded. Physical volumes
larger than 1.0 mL indicate TM perforation or ventilation tube patency.
In these cases, a tympanogram cannot be recorded, yet a type B function still might be drawn. Thus,
knowledge of the normal physical
volumes expected for a childs ear
help to clarify the etiology of a
type B tympanogram recording.
Acoustic Reflex Test

The acoustic reflex test in immittance measurement is a powerful

diagnostic tool used to help determine degree, type, and site of hearing loss. It cannot be substituted for
conventional audiometry, however,
because it is not a test of hearing.
The acoustic reflex threshold is a
measure of the lowest signal intensity that elicits a measurable contraction of the stapedius muscle in
the middle ear. In persons who have
normal hearing, tonal signals of 70
to 100 dB HL presented to one ear
elicit this muscle reflex, while white
noise signals of 65 dB HL induce
contraction of the stapedius muscle.
Because it is a bilateral response,

the acoustic reflex threshold can

be measured with the stimulus presented to one ear and the reflex
measured from either the ipsilateral
or the contralateral ear. Helpful
guidelines for interpretation of
findings for the acoustic reflex
threshold test, physical volume
test, and tympanometry are found
in Table 4.
INNER EAR

The hearing status of the pediatric

patient can be assessed by subjective
(behavioral) or objective testing
(which does not require an overt
behavioral response from the child).
The type of testing used depends
on the childs developmental age
and abilities (Table 5).
Behavioral Testing

The behavioral hearing test allows

graphing of a childs response
thresholds to tonal stimuli of different frequencies or pitches on an
audiogram (Fig. 2A). Frequency is
expressed on the abscissa from low
to high frequencies. The intensity or
loudness level in dB HL (referenced
to the hearing levels of normally
hearing individuals) is plotted on the
ordinate, with the lowest intensity
sounds at the top and the highest
intensity sounds at the bottom. A
key of symbols is on the form. Several different techniques can be used
to obtain audiometric information

from children, depending on the

childs developmental, not chronologic age. Behavioral testing techniques include sound field testing
and conventional audiometry.
Testing in the sound field (a
sound-treated room) is conducted
with the infant seated independently
or held on the caregivers lap and
centered between two loudspeakers.
Primary methods of sound field testing include behavioral observation
audiometry (BOA) and visual reinforcement audiometry (VRA).
BOA is used with neonates and
infants up to 6 months of age. Typically, two audiologists or observers
are needed to determine the presence or absence of a response to
presentations of warbled pure tones,
speech, or white and narrow-band
noise at varying intensity levels
through the loudspeakers. Toy
noisemakers of known frequency
and intensity also may be used,
although the primitive nature of
this test makes it of very little
diagnostic use. Responses, which
depend heavily on the childs physical state prior to stimulation, include
arousal, startle, eye-widening, cessation of movement, and auropalpebral
reflex (closing of the eyes or tightening of the lids if eyes already
are closed).
VRA is the sound field procedure
used most commonly for testing or
screening children from 6 months to

TABLE 4. Guidelines for Interpreting Findings from Immittance Testing*

TYMPANOGRAM

PHYSICAL
VOLUME (mL)

Type A

Type B

Type C

ACOUSTIC REFLEX

ETIOLOGY

0.3 to 1.0

60 to 70 dB above audiometric
threshold, but not exceeding
100 dB HL

Normal middle ear function

0.3 to 1.0

<50 dB above audiometric

threshold (abnormal loudness
growth)

Normal middle ear function

Cochlear pathology

<0.3

Absent

Probe against ear canal wall

Cerumen

0.3 to 1.0

Absent

Middle ear fluid

>1.0

Cannot evaluate

TM perforation
Patent ventilation tube

0.3 to 1.0

Absent or elevated
(>100 dB HL)

Negative middle ear pressure

Eustachian tube dysfunction

*Adapted from Northern J, Downs M. Hearing in Children. 4th ed. Baltimore, Md: Williams and Wilkins, 1991.

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DEVELOPMENTAL
AGE OF CHILD

AUDITORY TEST/
AVERAGE TEST TIME

Birth to 9 mo

Pediatrics in Review

TEST PROCEDURE

ADVANTAGES

LIMITATIONS

Auditory Brainstem
Response (ABR)
90 min test

Electrophysiologic measurement of activity in auditory

nerve and brainstem
pathways

Placement of electrodes on
childs head; auditory
stimuli presented through
earphones one ear at a time

Responses not dependent

on patient cooperation;
ear-specific results

Infant must remain

inactive for the test;
not a true test of
hearing because
it does not assess
cortical processing
of sound

9 mo to 2.5 y

Visual Reinforcement
Audiometry (VRA)
30 min test

Behavioral test measuring

responses of the child to
speech and frequencyspecific stimuli presented
through speakers in a
sound-treated room

Child is seated between

two speakers; child is
conditioned to look toward
active speaker; child is
reinforced for correct
responses by activation
of a lighted toy

Assesses auditory
perception of child

Only assesses hearing

of the better ear; not
ear-specific; cannot
rule out a unilateral
hearing loss

2.5 to 4 y

Play Audiometry
30 min test

Behavioral test measuring

auditory thresholds in
response to speech and
frequency-specific stimuli
presented through earphones and/or bone
vibrator

Child is conditioned to put

a peg in a peg board or
drop a block in a box
when stimulus tone is
heard

Ear-specific results;
assesses auditory
perception of child

Attention span of child

may limit the amount
of information
obtained

4 y to adulthood

Conventional
Audiometry
30 min test

Behavioral test measuring

auditory thresholds in
response to speech and
frequency-specific stimuli
presented through earphones and/or bone
vibrator

Patient is instructed to
raise his or her hand
when stimulus is heard

Ear-specific results;
assesses auditory
perception of patient

None

All ages

Evoked Otoacoustic
Emissions (EOAE)
10 min test

Physiologic test measuring

specifically cochlear (outer
hair cell) function in
response to presentation
of a stimulus

Small probe containing a

sensitive microphone is
placed in the ear canal
for stimulus delivery
and response detection

Ear-specific results; not

dependent on patient
state; quick test time

Not a true test of

hearing because it
does not assess
cortical processing
of sound

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TYPE OF MEASUREMENT

EAR DISORDERS
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TABLE 5. Referral Guide for Audiologic Evaluations Based on Developmental Age of Child

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FIGURE 2. Recording forms used in audiometric testing. A) Audiogram (dB HL);

B) Speech testing, including speech detection threshold (SDT), speech recognition
threshold (SRT), and speech discrimination ability.

2 years of age. It involves conditioning the child to respond, usually

with a head turn toward the active
speaker, when the stimulus is heard.
Responses are reinforced visually
by the examiner activating a lighted
toy animal stationed near or on the
speaker. A variation of VRA testing
is called conditioned orientation
reflex (COR) and involves correct
identification of the speaker from
which the signal is emanating. The
difference between VRA and COR
is that any overt response to the
stimulus (head turning, pointing, or
mimicking the sound) is acceptable
in VRA testing, whereas correct
head turning in response to the
stimulus is required with COR.
One limitation of both techniques
is the inability to assess each ear
separately because both ears can listen in the sound field environment.
Therefore, results reflect the sensitivity of the better hearing ear. Only
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when the child can tolerate wearing

earphones can ear-specific results be
obtained with this method.
The conventional audiometric
test battery includes testing via air
conduction and bone conduction,
obtaining speech recognition thresholds (SRT), and assessing speech
discrimination ability. Air conduction testing involves the presentation
of pure tone stimuli to one ear at a
time through earphones. The intensity of the tones is decreased gradually until the lowest intensity that
elicits a response is determined and
recorded as the childs threshold.
The need for sound waves to travel
through the air of the EAC and middle ear space before reaching the
inner ear is the source of the term
air conduction testing. It is possible
to bypass the EAC and middle ear
and stimulate the cochlea directly
via bone conduction. This method
requires placement of a bone oscilla-

tor on the childs forehead or mastoid. It often is necessary to mask

the ear not being tested because
vibration of the skull may elicit a
response from both cochleas. This
is accomplished by presenting a
narrow band noise to the nontest ear
to keep it busy while responses
from the test ear are being recorded.
Play audiometry may be used
for children 2 to 5 years of age to
obtain threshold information via air
and bone conduction mechanisms.
The child is taught to place a peg in
a board whenever a tone is heard
through the earphones or the bone
oscillator. Thus, ear-specific information can be obtained. Speech
testing (Fig. 2B) also may be initiated in children in this age range.
SRT is the clinical measure of
speech perception obtained most
routinely. It is recorded as the lowest intensity level at which a listener
can repeat 50% of the speech material presented. The SRT should
be in close agreement with pure
tone thresholds at 500, 1,000, and
2,000 Hz because the speech material includes phonemes that have most
of their energy in this frequency
range. Children who cannot repeat
words for SRT testing may point
to pictures on a board to indicate
recognition of the speech stimulus.
Younger children who cannot participate in a pointing or repeating task
still may respond to speech with a
head turn. The lowest level of speech
that elicits this type of response from
the child is recorded as the speech
detection threshold (SDT).
Children ages 5 years or older
usually can respond with the conventional hand raising technique that
is used with adults to obtain ear-specific threshold information across
the frequency range for pure tones
and SRT. Speech discrimination
ability (the ability to understand and
discriminate speech material) also
may be assessed. Typically, phonetically balanced words are presented
to the listener at an average conversational level (45 dB HL) or at
higher levels, and the percentage
of words correctly identified and
repeated by the listener is recorded
(Fig. 2B). Speech discrimination
ability often is reduced significantly
in the presence of sensorineural
hearing loss.

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Objective Testing

Auditory brainstem response (ABR)

and otoacoustic emissions (OAE)
are objective tests of peripheral
function that can be administered
to individuals of all ages. ABR, also
commonly referred to as BAER
(brainstem auditory evoked potentials), and OAE are particularly useful with people who are unable or
unwilling to respond behaviorally or
when ear-specific or reliable results
are not obtained with conventional
audiometric techniques.
ABR testing employs several
electrodes on the surface of the persons scalp to record the synchronous firing activity of the auditory
nerve and brainstem auditory neurons in response to brief tonal or
click stimuli. Over the past 25 years
ABR has achieved universal acceptance as an important screening and
diagnostic tool for assessing peripheral and brainstem auditory integrity.
Because it has high sensitivity and
specificity (98% and 96%, respectively), one of its most recognized
uses is in the detection of hearing
impairment in neonates within the
first few hours of life.
The pediatric patient must sleep
during testing because the ABR is
very sensitive to muscle contraction
and movement. ABR testing may
be scheduled around sleeping times
for infants younger than 6 months.
Children older than 6 months often
require conscious sedation with
chloral hydrate (50 mg/kg or
75 mg/kg administered orally, not
to exceed 100 mg/kg or 1,000 mg
total) or an intramuscular injection
of a mixture of meperidine, promethazine/phenylephrine, and chlorpromazine. Sedation is administered
only under the specific orders of
a physician and when medical
support is available for monitoring
the physical condition of the child
before, during, and after emergence
from sedation.
The measurement of OAE is now
recognized as a powerful method for
assessing the status of the cochlea
and the outer hair cells of the organ
of Corti. OAE are acoustic signals
generated from within the cochlea
that travel in a reverse direction
through the middle ear space and
TM out to the ear canal. These signals are usually inaudible to the
162

unaided human ear, but they may be

detected with a very sensitive microphone/probe system placed in the
ear canal, much like that used in
immittance testing. Although 50%
of normal hearing ears generate
OAE spontaneously, nearly 100%
generate OAE in response to an
evoking stimulus. Thus, evoked
OAE, including transient evoked
OAE and distortion product OAE,
are used most frequently in clinical
settings to determine the status of
the cochlea. Similarly to ABR, OAE
can be measured in infants within
the first few days after birth.
Because OAE testing is relatively
quick, noninvasive, and does not
require sleep or sedation, it is
an attractive technique for infant
screening. Its limitations involve an
inability to quantify hearing loss or
hearing threshold levels. However,
because the response is eliminated
in the presence of a hearing loss
greater than 35 dB HL, middle ear
pathology, or debris in the EAC, it
is an excellent screening measure
for normal versus abnormal hearing.
ABR and OAE are tests of auditory structural integrity, but are not
true tests of hearing. Even in the
presence of a normal ABR and normal OAE, there is no way to guarantee that a child hears until he or
she is mature enough to indicate so
behaviorally. Thus, follow-up evaluations should be scheduled until a
reliable audiogram can be obtained.

Degrees and Types of

Hearing Loss
Normal hearing is defined as thresholds of 15 dB HL or better (lower
intensities) for children who are
18 months to school age (Fig. 2A).
Thresholds greater (worse) than
15 dB HL can handicap a child
educationally. Most children who
present with acute otitis media have
worse thresholds than the 15 dB HL
normal limits for hearing. They frequently appear to ignore people and
activities in the classroom or at
home, often turn up the volume
while watching television, and may
ask constantly for repetition from
persons who are speaking to them.
Children who have greater hearing
loss may become withdrawn and
isolated from peers because of the

difficulty in communication. Hearing loss also causes fatigue because

additional effort is needed to listen
in the typically noisy classroom.
Varied degrees of hearing loss are
associated with characteristic handicapping effects (Table 6).
Three major types of hearing loss
are named for their sites of pathology: sensorineural (involving the
sensory end organ of hearing or
cranial nerve VIII), conductive (of
or having to do with the air conduction pathways of the EAC and/or
middle ear space), and mixed
(sensorineural hearing loss with an
additional conductive component)
(Fig. 3). In cases of normal hearing
or sensorineural impairment, bone
conduction thresholds closely agree
with air conduction thresholds
(within 10 dB) (Figs. 3A and 3C).
When pathology of the EAC or
middle ear space effectively
obstructs the air conduction pathway, air conduction thresholds are
poorer compared with bone conduction thresholds (>10 dB); the difference between the two scores is
described as an air-bone gap, indicating the presence of conductive
pathology (Fig. 3B). In some
instances, bone conduction thresholds may be reduced (<15 dB HL)
in combination with an air-bone gap.
This is characteristic of a mixed
type of hearing loss (Fig. 3D).
Hearing loss rarely affects all
portions of the cochlea equally, producing a flat type of hearing impairment. For example, the majority of
sensorineural hearing impairments
affect the base or high frequency
end of the cochlea first; most conductive pathologies have a greater
effect on low frequency hearing
abilities. Different strategies are
used to manage the hearing impaired
child, depending on the type, degree,
and configuration of the loss.

Management of the
Hearing Impaired Child
Most children have at least some
degree of usable hearing, which
makes deaf an incorrect label
because it implies a lack of any
measurable hearing. Recent
advances in amplification technology have enabled audiologists
to access usable hearing, beginning

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EAR DISORDERS
Hearing Impairment

TABLE 6. The Handicapping Effects of Unmanaged Hearing Impairment and the Educational
Needs Associated with Varying Degrees of Hearing Loss
DEGREE OF HEARING LOSS
(AVERAGE THRESHOLDS
AT 500 TO 2,000 Hz)

ASSOCIATED HANDICAP
AND BEHAVIORS
WITHOUT AMPLIFICATION

0 to 15 dB HL (normal hearing)

Can detect all aspects of speech

signal

No special needs

16 to 25 dB HL (borderline)

May miss up to 10% of speech

in noise (eg, classroom)
May respond inappropriately
Peer social interaction affected

Trial of low-gain personal FM system

Medical management of ears
Make teacher aware of impact on
instruction

26 to 40 dB HL (mild)

May miss up to 50% of speech

Often is labeled as behavior
problem and poor listener
Self-esteem is affected

Hearing aid or personal FM unit and

and preferential seating
May need special language, vocabulary,
and reading help

41 to 55 dB HL (moderate)

50% to 100% of speech may

Amplification
be missed
Speech therapy and special education
Voice and speech quality likely
(auditory skills, reading, and writing)
to be poor; limited vocabulary;
defective syntax
Significantly compromised
communication
Possible low self-esteem

56 to 70 dB HL (moderate
to severe)

100% of speech information

lost unless it is very loud
Delayed speech and poor
intelligibility
Social isolation likely if selfconcept not addressed

Hearing aid essential

Resource teacher or special class likely
needed for all academic areas

71 to 90 dB HL (severe)

Loud voices possibly heard

up to 12 in from ear
Delayed speech and language
if loss is prelingual
Declining speech abilities
and atonal voice if loss is
postlingual

Full-time amplification essential

Special class, with caregivers choice of
method emphasized (auditory/verbal,
signing, or total communication)

90+ dB HL (profound)

Sound vibrations felt rather

than heard
Visual cues primary for
communication
Peer group of hearing impaired
children preferred (may be
issue for family)

Special program for the deaf likely

for all academic skills
Some mainstreaming
Possible candidacy for cochlear implant

EDUCATIONAL NEEDS

*Adapted from Northern J, Downs M. Hearing in Children. 4th ed. Baltimore, Md: Williams and Wilkins, 1991 and Flexer C. Classroom management of children with minimal hearing loss. Hear J. 1995;48:5458.

at the earliest age of identification.

Personal FM amplification devices,
hearing aids, and cochlear implants
are used to access sounds for children who have varying degrees and
types of hearing impairment. A complete discussion of each type of
amplification is beyond the scope of
this article, but amplification alone
will not ensure that the child identiPediatrics in Review

Vol. 19

No. 5

fied as having significant hearing

loss will develop speech, language,
and social/emotional skills normally.
Successful communication is
an active process for the hearing
impaired child, fostered by therapeutic, parental, and educational acceptance, patience, and hard work compared with the ease with which most
children who have normal hearing

learn to communicate. No single

miracle method can be used to
teach a hearing impaired child to
communicate. Educational methods
range from the use of sign language
in which the child does not depend
on any auditory information to the
auditory/verbal method in which the
child is forced to access any and all
usable hearing to communicate and

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163

EAR DISORDERS
Hearing Impairment

FIGURE 3. Examples of a normal audiogram (A) and each of the three major hearing loss types described in the text: (B) conductive, (C) sensorineural, (D) mixed.
Results are shown for right ear only.

is not allowed to lip-read or use

signs to a method of total communication in which auditory information, lip-reading, and signs all are
used to communicate. Although
many biases for or against each
method exist among hearing professionals, caregivers ultimately must
decide which method is best for
their child.
Success with any of the methods
depends on several important variables that include, but are not limited to: 1) the degree and type of
hearing impairment, 2) parent and
family motivation, 3) presence of a
healthy emotional support network
for the family, and 4) development
of realistic goals and expectations
for the parents and the child. The
164

day that a child who has significant

hearing loss receives amplification
should be considered by parents and
professionals to be the first day of
that childs hearing life. Thus, a
child who receives amplification
at 1 year of age or older would be
expected to have speech, language,
and auditory skills equivalent to
that of a newborn.
When children who have severe
hearing impairments reach school
age, they usually are placed in
special classrooms with some integration into regular classrooms. This
is typically part of the individualized
education plan. Some children, however, can be mainstreamed completely, with classroom amplification
or personal FM amplification sys-

tems used to enhance the teachers

voice relative to the background
noise. These amplification systems
can be useful for children who have
all degrees of hearing loss.
The child who has a unilateral
loss or a mild-to-moderate hearing
impairment often is seated preferentially. Seating in close proximity to
the teacher may help the child hear
what is being said, although this
is ineffective by itself unless the
teacher stands in one spot the entire
day. Personal FM amplification
systems require the teacher to wear
a remote microphone approximately
6 inches from the mouth; the signal
is transmitted via FM (radio frequency) waves to a receiver worn
by the child. The receiver may be
connected directly to the childs
hearing aids or the child may wear
some form of headphones, similar
to portable audiocassette headphones. The advantage of the FM
system is that the remote microphone can, in essence, place the
teachers voice 6 inches from the
childs ear (the distance of the
microphone from the teachers
mouth) no matter where the teacher
is located in the room. This enhances
both the speaker-to-noise ratio (SNR)
and the sound quality for the child.
Unfortunately, state and school
officials appear to be reluctant to
accept recommendations for use of
an FM unit for children who have
mild hearing impairments and unilateral losses. Physicians, case workers, parents, teachers, and audiologists must advocate proactively for
these children. Section 504 of the
Rehabilitation Act of 1973 states
that all children have the right to
an appropriate education. With the
support of this legislation, professionals can advocate confidently
for the provision of SNR-enhancing
technology. Flexer recommends
stating that the childs hearing
problem interferes with his or her
access to spoken instruction; therefore, the child is being denied an
appropriate education.

Summary
Early identification of and intervention for all children who have hearing impairments remain unattained
goals in the United States. Physi-

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No. 5

May 1998

EAR DISORDERS
Hearing Impairment
cians typically are the first persons
to obtain the medical and family history of infants and children and are
the primary professionals confronted
with parental concerns about hearing
loss. Heightened awareness of the
common causes of hearing loss in
infants and children can facilitate
prompt and appropriate referrals to
audiologists when hearing loss is
suspected. A strong and interactive
relationship between physician and
audiologist is needed to attain the
common goals of providing the earliest and best possible diagnosis of
and optimal management for hearing
impaired pediatric patients.

PIR QUIZ
4.

SUGGESTED READING
Bess F, Hall JW III, eds. Screening Children
for Auditory Function. Nashville, Tenn:
Bill Wilkerson Center Press; 1992
Flexer C. Facilitating Hearing and Listening
in Young Children. San Diego, Calif:
Singular Publishing Group, Inc; 1994
Hayes D, Northern J. Infants and Hearing.
San Diego, Calif: Singular Publishing
Group, Inc; 1996
Jerger J. Clinical experience with impedance
audiometry. Arch Otolaryngol. 1970;92:
311314
Northern J, Downs M. Hearing in Children.
4th ed. Baltimore, Md: Williams and
Wilkins; 1991
Peck J. Development of hearing. Part I.
Pylogeny. J Am Acad Audiol. 1994;5:
291299
Peck J. Development of hearing. Part III.
Postnatal development. J Am Acad Audiol.
1995;6:113123

5.

You elect to refer the previously noted

infant for evaluation of possible hearing loss. The intactness of the auditory pathways of this infant could be
ascertained best by:
A. Acoustic reflex testing.
B. Auditory brainstem response
(ABR).
C. Play audiometry.
D. The conditioned orientation reflex.
E. Visual reinforcement audiometry.

6.

A mother is concerned about her

18-month-old sons ability to hear as
well as his delayed speech. He has
had recurrent otitis media, and pressure equalizing tubes (PET) were
placed 4 months ago. On examination,
the tympanic membranes are dull,
but the PETs appear to be in place
bilaterally. Acoustic immittance testing reveals the following results:
Type C tympanograms bilaterally;
external auditory canal volumes,
0.6 cc and 0.8 cc, AD and AS,
respectively; acoustic reflex absent
bilaterally. The best interpretation
of these data is that:
A. Large effusions are present
bilaterally.
B. Mixed hearing loss is present.
C. The eustachian tubes are present.
D. The probe was malpositioned.
E. The ventilating tubes are
obstructed.

ACKNOWLEDGMENTS
The authors thank D. Blackmore, B. Lasky,
T. Mancuso, L. Segal-Pallas, S. Seidenberg,
and V. Shields-Haseley, members of the
Speech-Language-Hearing Department,
who contributed to the research for this
manuscript.

Pediatrics in Review

Vol. 19

No. 5

You are examining a 3-month-old girl

for the first time. Her family history
is unremarkable. Gestation was 36
weeks. Delivery was vaginal, vertex;
Apgar scores were 5 and 9 at 1 and
5 minutes, respectively; she received
bag-mask ventilation briefly; and her
birthweight was 2,300 g. Ampicillin
and cefotaxime were administered
for 2 days. Peak bilirubin was
16.4 mg/dL/1.0 mg/dL. There was
no evidence of hemolysis. Phototherapy was administered for 12 hours.
She has grown well subsequently.
No dysmorphic features are noted on
physical examination. The tympanic
membranes are normal. The most
appropriate indication for testing
this infant for hearing loss would be:
A. Absence of cooing.
B. Disregard of sudden loud noises.
C. Exposure to ototoxic drugs.
D. Hyperbilirubinemia.
E. Prematurity.

7.

One month later, the toddler

described previously returns for
re-evaluation. He is alert and
attentive. His expressive vocabulary
is limited to four indistinct words.
Physical examination reveals no
dysmorphic features. His tympanic
membranes generally are translucent, with the exception of sclerosis
around the PETs. Acoustic immittance testing reveals type A tympanograms with normal external
auditory canal volumes and acoustic
reflexes. The most appropriate next
step would be:
A. Measurement of otoacoustic
emissions.
B. Reassurance of the parents.
C. Referral for PET replacement.
D. Referral for play audiometry.
E. Referral for visual reinforcement audiometry.

8.

An example of best practice in the

educational management of a child
who has a hearing disorder is:
A. Advocating that speaker-tonoise ratio (SNR)-enhancing
technology be made available
to patients who have persistent
mild hearing impairment.
B. Avoiding the combination of
signing with other communication methods.
C. Ceding to school officials the
decision about which children
should receive a personal FM
unit.
D. Keeping the profoundly deaf
child out of regular classrooms.
E. Relying on preferential seating
for the child who has unilateral
hearing loss.

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165

Early Identification and Intervention for Children Who Are Hearing Impaired
Katheryn Rupp Bachmann and Joan C. Arvedson
Pediatrics in Review 1998;19;155
DOI: 10.1542/pir.19-5-155

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Early Identification and Intervention for Children Who Are Hearing Impaired
Katheryn Rupp Bachmann and Joan C. Arvedson
Pediatrics in Review 1998;19;155
DOI: 10.1542/pir.19-5-155

The online version of this article, along with updated information and services, is located on the
World Wide Web at:
http://pedsinreview.aappublications.org/content/19/5/155

Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly publication, it has
been published continuously since 1979. Pediatrics in Review is owned, published, and trademarked by the
American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright
1998 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0191-9601.

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