Audiologic assessment in infants

Yvonne S. Sininger

Purpose
The purpose of this review is to provide the reader with current
information regarding the standards for audiologic assessment
of infants and very young children. The nature of the
appropriate test battery and the need for adjusting test
procedures to meet the specific needs of infants and toddlers
are emphasized.
Recent findings
The basic measures in the audiologic test battery include
frequency-specific threshold tests by air and bone conduction,
predicted by electrophysiologic measures when necessary;
immittance measures including tympanometry and acoustic
reflex using a high-frequency probe tone for infants under
4 months of age; and otoacoustic emissions. The ABR can be
used with frequency-specific stimuli to predict the audiogram
in newborns with a great deal of accuracy. Newer techniques,
such as Auditory Steady State Response, are promising but
need further study before they can be used reliably to predict
hearing levels in infants. Finally, infants with hearing loss can
be fit with amplification using prescriptive formulae, such as
the Desired Sensation Level, which give appropriate hearing
aid characteristics for infants based on their hearing
thresholds. These fittings must be verified using objective
electro-acoustic measures tailored to infants.
Summary
Infants failing newborn hearing screenings can be evaluated by
audiologists to predict all necessary audiologic data and those
found to have hearing loss can be fitted with appropriate
amplification in the newborn period. Procedures must be
carefully tailored to this age group.
Keywords
auditory brainstem response (ABR), otoacoustic emissions
(OAEs), visual reinforcement audiometry (VRA), real-ear to
coupler difference (RECD)
Curr Opin Otolaryngol Head Neck Surg 11:378382. 2003 Lippincott Williams
& Wilkins.

Professor, Division of Head & Neck Surgery, University of California Los Angeles,
David Geffen School of Medicine, Los Angeles, California, USA
Correspondence to Yvonne S. Sininger, PhD, 62-132 Center for the Health
Sciences, Box 951624, Los Angeles, CA 90095-1624, USA
E-mail: ysininger@mednet.ucla.edu
Current Opinion in Otolaryngology & Head and Neck Surgery 2003,
11:378382
Abbreviations
AR
ART
ASSR
OAE

378

acoustic reflex
acoustic reflex threshold
auditory steady-state response
otoacoustic emission

BOA
VRA
OME
RECD

behavioral observation audiometry

Visual reinforcement audiometry
otitis media with effusion
Real-ear to Coupler Difference

2003 Lippincott Williams & Wilkins

1068-9508

Introduction
Early detection of hearing loss is the goal of newborn
hearing screening programs. Early detection must be followed immediately by appropriate early intervention to
maximize development of auditory skills and speech and
language [1]. The infant who does not pass screening
must receive a comprehensive audiologic evaluation, sufficient to characterize the degree and type of hearing loss
in each ear. To avoid errors, the audiologic evaluation
process must use a battery of tests with cross checks. The
test battery must incorporate objective and behavioral
measures and standard audiologic tests must be tailored
to be developmentally appropriate.
Unlike the visual system, the peripheral auditory system
and the hearing sensitivity of a newborn infant are expected to be nearly mature at birth. The human cochlea
reaches adult size and configuration by about 20 weeks
gestation [2]. Cochlear tuning, as measured by distortionproduct otoacoustic emission suppression tuning curves,
is adult-like for high (6000 Hz) and low (1500 Hz) regions by term birth [3]. Allowing a few days for resolution
of amniotic fluid in the middle ear and clearing of vernix
and other debris from the neonatal ear canal [4], the
otoacoustic emission (OAE), generated by active processes in the outer hair cells of the normal newborn, can
be expected to be present and robust in amplitude [5,6].
The auditory nerve and brainstem structures continue to
develop for 18 to 24 after-birth months as reflected in a
gradual shortening of interpeak latencies in the ABR [7].
Although the ABR can only be recorded in response to
high-level sound in neonates as young as 27 to 30 weeks
gestational age [8,9], by a few days after term birth, the
ABR can be recorded with frequency-specific stimuli revealing thresholds that are within 15 dB of those produced by young, normally hearing adult subjects [10].
The ABR is a tool that has the ability to evaluate two
distinct aspects of the auditory system. First, auditory
brainstem development can be studied by determining
patterns of peak latency and amplitude change over
time. Normal developmental processes, such as myelination, or abnormal processes, for example in infants with
compromised auditory system development from prena-

Audiologic assessment in infants Sininger 379

tal exposure to drugs or alcohol, prematurity, hyperbilirubin etc., can be studied using the ABR [11,12]. Also,
the threshold of ABR relative to the level of stimulus
used to elicit the response, can generally be used as an
accurate predictor of auditory sensitivity and is used to
determine the presence of hearing loss in infants who are
too young for subjective assessment [13,14].
Screening programs for detection of hearing loss in newborns are now widespread. Every state in the US either
has legislation mandating screening of newborns or is
screening most infants in birthing hospitals by choice.
This has created a challenge for audiologists who must
evaluate those infants who do not pass the newborn
screening to confirm or deny the presence of educationally significant hearing loss and, if confirmed, to determine the type of loss and frequency-specific thresholds
that define the degree and configuration. There is urgency to determine this information in each infant and to
fit appropriate amplification as soon as possible and before the infant reaches 6 months of age [15].

Predicting the pure-tone angiogram

Tests of pure-tone or frequency-specific threshold in infants and children are either physiologic or behavioral (an
overt reaction is elicited from the child in response to
sound and the action is judged by an audiologist). When
the appropriate technique is applied, physiologic tests,
such as ABR, can predict pure-tone hearing thresholds
within a few dB in infants and young children with all
degrees of hearing loss [14]. It is important to note that
normal hearing newborns will show ABR thresholds for
tone-burst stimuli, representing the full range of audiometric frequencies, that are clinically identical to those
obtained in young adults and clearly within the normal
range of hearing by air and bone conduction [10,16].
The auditory steady-state response (ASSR) is a new
physiologic evoked potential technique that is used to
predict frequency-specific thresholds [17]. ASSR uses
pure-tones stimuli (carriers) that are amplitude-modulated. For pediatric applications the modulation occurs at
a frequency appropriate for infants and children (about
80100 Hz). Scalp-recorded activity is analyzed for evidence of the modulation frequency. Statistical analysis is
used to determine the salience of the modulation frequency activity to judge a response presence or absence
for the carrier frequency. This technique has been
shown to be of value in assessing aided hearing thresholds in the sound field [18]. Hearing thresholds have
been estimated within about 10 to 15 dB in adults with
normal hearing and hearing loss using the multifrequency ASSR [17]. It must be noted that when conditions of neural disease, such as auditory neuropathy
(AN) are found [19] neither the ABR nor the ASSR will
be good indications of hearing level and they may dra-

matically overestimate thresholds. If cochlear function

appears normal as indicated by present OAEs and neural
function appears abnormal, as with absent or abnormal
ABR, neither OAE nor ABR will be useful to predict
hearing thresholds [20]. For an update on AN in infants
see Sininger [21].
One reservation about the use of ASSR for measurement
of hearing is the lack of data in infants and children
[22]. Perez-Abalo et al. [23] found that although they
were able to determine hearing loss in the severe and
profound range, in general, only fair agreement was
found between ASSR thresholds and hearing levels in
children with mild hearing loss or normal hearing. Further research and refinement is needed on ASSR to determine if this technique will produce accurate audiogram predictions in all infants but this is a promising
technique.
Before the age of 6 months, it is possible to obtain unconditioned responses to sound such as a change in sucking behavior, startle reflex, or eye widening. This is
known as behavioral observation audiometry (BOA).
These responses will be supra-threshold and, although
BOA cannot be used to rule out mild or moderate hearing loss, it is a valuable part of the test battery for infants
under 6 months to substantiate overall impressions. At
this young age audiometric thresholds must be inferred
from physiologic tests such as ABR.
Audiometric tests for children under the age of 3 years
are classified in Table 1. A 6-month-old infant with
normal vision will naturally turn their head to find the
source of an interesting sound. Visual reinforcement audiometry (VRA) is an operant conditioning paradigm that
reinforces head turns with a pleasant visual stimulus
(usually a lighted, animated toy). Tones and speech
stimuli can be used. VRA can be administered using
insert earphones for an ear-specific response or with a
bone-conduction vibrator. If a child will not tolerate earphones, the stimuli can be presented through a speaker
into the sound-field of a sound-treated chamber. Generally, a normal-hearing 6-month-old infant will respond
to stimuli of 20 dB HL or better [24]. Finally, depending upon cooperation, a 2-year-old toddler can usually be
tested using Play Audiometry, in which the childs response to pure tones is to stack a ring on a pole or drop
a block into container as part of a game. Thresholds
obtained using Play Audiometry in a cooperative child
are assumed to be adult-like in accuracy.

Immittance measures
Tympanometry and acoustic or middle-ear muscle reflexes are a standard, objective test of tympanic membrane mobility, middle-ear pressure, and brainstem auditory activation. These tests are a very important part of
the test battery used for audiometric assessment of in-

380 Audiology
Table 1. Audiologic tests appropriate for infants and toddlers by age
Age in months

Threshold
Air or
Bone

[------------------------------------------------------------------------------------------------------

ABR

VRA

Tymp 600
1000 Hz
Tymp
226 Hz

Cochlear

OAEs

12

18

24

30

36

[----------------------------------------------------------------]

Play
Middle-ear
function

10

[--------------------
[------------------------------------------------------------------------------------------------------
[---------------------------------------------------------------------------
[------------------------------------------------------------------------------------------------------

VRA, Visual reinforcement audiometry; Tymp, Tympanogram.

fants and toddlers. This is especially important when one

considers the high incidence of otitis media with effusion
(OME) in children in this age group. In a recent study of
over 3000 infants aged 8 to 12 months, receiving followup audiologic examinations, 30% had OME at the time
of testing [25].
Tympanometry for young infants, when performed with
a standard probe tone frequency of 220 or 226 Hz, can be
invalid. Infants with OME can reveal a normal-appearing
tympanogram when tested using this probe frequency
[26]. This finding may be due to extensibility of the skin
of the ear canal in these infants. Valid tympanograms can
be obtained by raising the probe frequency to 600 to
1000 Hz for infants aged 4 months or less [27,28]. The
acoustic reflex (AR) can be a very useful part of the
audiologic evaluation in infants. A present reflex is
added support for normal middle-ear function. Because
the reflex arc involves the seventh and eighth nerve and
the low brainstem, a normal or present reflex can be
useful in ruling out abnormalities such as AN [20]. In
addition, although there is no direct relation between
acoustic reflex threshold (ART) and hearing threshold,
the ART does set an upper bound for hearing level (ie,
the ART will never be elicited at levels below the true
auditory threshold). It is particularly important to use a
high-frequency probe (6001400 Hz) to measure the AR
in infants under 6 months of age [29].

Otoacoustic emissions
Otoacoustic emissions are a noninvasive, objective measure of cochlear functioning. OAEs are generated exclusively by outer hair cells [30]. Outer hair cells are generally more vulnerable to disease and damage than inner
hair cells. Therefore when OAEs are normal, it is reasonable to assume that the inner hair cells are functioning as well. When OAEs are present for a range of frequencies, hearing thresholds are generally 30 to 40 dB
HL or better for those frequency regions. Caution should
be used in over-interpretation of very narrow, lowamplitude regions of OAE, which can be spurious noise.
Also, OAEs are not strong in low-frequency regions
(1000 Hz and below) in infants and toddlers due to

physiologic noise and thus, absent OAEs in low- frequency regions should not be given great weight in
interpretation.
Absence of OAEs can be due to a variety of causes, from
middle-ear dysfunction to sensorineural disorders producing hearing loss of any degree. The absence of an
OAE should not be interpreted as indication of significant hearing loss. In contrast, OAE presence indicates
good hair cell function and generally indicates that the
hearing thresholds should be better than 30 to 40 dB.
However, OAEs cannot be used to determine exact hearing thresholds.
The presence of an OAE alone cannot insure that hearing sensitivity is normal. Disease that spares the cochlea
and impairs function in the auditory nerve or low brainstem (eg, acoustic neuroma or AN) can also cause significant hearing loss. The OAE must be included in a battery of tests for accurate interpretation.

Fitting of amplification in infants

The goal of early identification and characterization of
hearing loss in infants is the appropriate fitting of amplification as early as possible. If an infant is identified
before 6 months of age, the fitting must proceed based
on hearing thresholds obtained by ABR. The infant will
be able to do very little to help in the initial fitting of the
amplification. For that reason, we rely on objective
physical measurements along with behavioral observations and parent report in this age group.
Formulas such as the Desired Sensation Level (DSL)
[31] prescribe the output characteristics needed in a
hearing aid for children based on the childs hearing
thresholds. Infant ear canals are considerably smaller
than those of adults and it is critical to take the acoustic
characteristics of the individual infants ear into consideration when the amplification levels from the hearing
aids are set. Consequently, real-ear measures are an indispensable part of the fitting process in infants.
Hearing aid characteristics (such as gain and maximum
output) can be measured in one of two ways. Tradition-

Audiologic assessment in infants Sininger 381

ally, the hearing aid is connected to a hard-walled coupler with dimensions designed to mimic the size and
acoustic characteristics of the human external ear and
canal. A microphone is connected to the coupler to measure the hearing aid output in approximately the position
that the tympanic membrane would occupy. The hearing
aid is turned on and placed in a test chamber where
calibrated sounds are delivered to the hearing aid microphone. A comparison of the input sound to the output in
the coupler describes the performance characteristics of
the hearing aid.
Another way of measuring hearing aid acoustic performance is termed a real-ear measurement. The measuring microphone is connected to a tiny probe tube that
is placed in the patients ear canal terminating near the
tympanic membrane. The hearing aid is then placed in
the ear with the probe tube undisturbed. The measurement device again presents calibrated sounds directed
toward the subject wearing the hearing aid. By comparing the sound levels at the entrance to the ear and those
amplified by the aid near the tympanic membrane, the
amplification characteristics of that aid on that patient
can be determined. When real-ear measures are used,
the true characteristics of the aid in the subjects ear can
be determined and the aid can be manipulated to obtain
the desired levels according to the prescriptive formula
being used.
Because it can be difficult to get cooperation from infants
for the real-ear measures, a technique known as the
Real-ear to Coupler Difference (RECD) is recommended for small children [32]. The probe tube is fit into
the infants ear followed by an inserted earphone. The
earphone presents a calibrated sound and a quick acoustic measurement is recorded from the probe microphone.
The time needed for infant cooperation is less than a
minute. Next, the earphone is used to present the same
sound via the standard coupler and the sound level is
recorded. By comparing the levels recorded in the coupler to those recorded in the infants ear, the infants
RECD can be determined. Generally, the level recorded
is higher in the infants ear and the degree of difference
is greatest for the high frequencies. Now, hearing aids
can be adjusted and tested for that infant using the coupler (rather than the squirming baby) and RECD values
can be applied to correct coupler measures indicating
how the aid will perform in the infants ear. Whether or
not the infant will cooperate for standard real-ear measures, a physical measurement is used to determine
that the appropriate hearing aid fitting has been achieved.
This allows audiology to move ahead with hearing aid
fittings long before the infant can offer a behavioral response when using the aids. For excellent review of hearing aid fittings and verification for infants see Beauchaine
[33] and Scollie and Seewald [34].

Conclusion
Audiologists have the technology necessary to perform
all the basic audiologic assessments on newborn infants.
It is crucial that time not be lost in identifying hearing
loss and fitting appropriate amplification. Extra care
must be taken to avoid mistakes. For that reason test
batteries with attention to crosschecking results must be
used. (For an excellent review see Gravel [35]). Whenever possible objective measures are used in conjunction
with behavioral measures and test batteries are adjusted
as appropriate for the age of the child as they grow. Once
the audiogram is accurately predicted, electro-acoustic
real ear measures and prescriptive formulae can be applied to accurately fit and monitor amplification. No infant with hearing loss should be misdiagnosed or improperly fit with amplification, if proper protocols are
understood and followed.

References and recommended reading

Papers of particular interest, published within the annual period of review,
have been highlighted as:

Of special interest

Of outstanding interest

Sininger YS, Doyle KJ, Moore JK: The case for early identification of hearing
loss in children. Pediatr Clin North Am 1999, 46:114.

Moore KL, Persaud TVN: The Developing Human, edn 5. Philadelphia:

WB Saunders;1993.

Abdala C: A developmental study of distortion product otoacoustic emission

(2f1-f2) suppression in humans. Hear Res 1998, 121:125138.

Doyle KJ, Burggraaff B, Fujikawa S, et al.: Neonatal hearing screening with

otoscopy, auditory brain stem response, and otoacoustic emissions. Otolaryngol Head Neck Surg 1997, 116:597603.

Sininger YS: Clinical Applications of Otoacoustic Emissions. Advances in

OtolaryngologyHead and Neck Surgery 1993, 7:247269.

Norton SJ, Widen JE: Evoked otoacoustic emissions in normal-hearing infants

and children: Emerging data and issues. Ear Hear 1990, 11:121.

Salamy A, McKean CM, Buda FB: Maturational changes in auditory transmission as reflected in human brainstem potentials. Brain Res 1975, 96:361
366.

Starr A, Amlie RN, Martin WH, et al.: Development of auditory function in

newborn infants revealed by auditory brainstem potentials. Pediatrics 1977,
60:831839.

Galambos R, Hecox KE: Clinical applications of the auditory brainstem response. Otolaryngol Clin North Am 1978, 11:709722.

10

Sininger YS, Abdala C, Cone-Wesson B: Auditory threshold sensitivity of the

human neonate as measured by the auditory brainstem response. Hear Res
1997, 104:2738.

11

Shih L, Cone-Wesson B, Reddix B: Effects of maternal cocaine abuse on the

neonatal auditory system. Int J Pediatr Otorhinolaryngol 1988, 15:245251.

12

Lee JA, Schoener EP, Nielsen DW, et al.: Alcohol and the auditory brain-stem
response, brain temperature, and blood alcohol curves: explanation of a paradox. Electroencephalogr Clin Neurophysiol 1990, 77:362375.

13

Sininger YS, Abdala C: Hearing threshold as measured by auditory brain

stem response in human neonates. Ear Hear 1996, 17:395401.

14

Stapells DR, Gravel JS, Martin BA: Thresholds for auditory brainstem responses to tones in notched noise from infants and young children with normal hearing or sensorineural hearing loss. Ear Hear 1995, 16:361371.

15

Joint Committee on Infant Hearing: Year 2000 position statement. Audiology

Today 2000 (Special Issue 2000):323.

16

Cone-Wesson B, Ramirez GM: Hearing sensitivity in newborns estimated

from ABRs to bone-conducted sounds. J Am Acad Audiol 1997, 8:299307.

17

Dimitrijevic A, John MS, Van Roon P, et al.: Estimating the audiogram using
multiple auditory steady-state responses. J Am Acad Audiol 2002, 13:205
224.

382 Audiology
This paper describes the use of simultaneous multiple test tones (4 per ear) for
rapid measurement of ASSR in both ears at once. The technique is able to predict
hearing levels in adults with normal hearing and hearing loss within 15 dB.
18

Picton TW, Durieux-Smith A, Champagne SC, et al.: Objective evaluation of

aided thresholds using auditory steady-state responses. J Am Acad Audiol
1998, 9:315331.

19

Starr A, Picton TW, Sininger Y, et al.: Auditory neuropathy. Brain 1996,

119:741753.

20

Sininger YS, Oba S: Patients With Auditory Neuropathy: Who Are They and
What Can They Hear. In: Sininger YS, Starr A, editors. Auditory Neuropathy:
A New Perspective on Hearing Disorders. Albany, New York: Thompson
Learning; 2001:1535.

Sininger YS: Auditory neuropathy in infants and children: implications for early
hearing detection and intervention programs. Audiology Today 2002 (Special
Issue):1621.
This article summarizes data from a variety of studies on the incidence and risk
factors involved in infants with auditory neuropathy. Implications for test batteries
appropriate for newborn screening are discussed.
21

22 Stapells DR: The tone-evoked ABR: Why its the measure of choice for young
infants. The Hearing Journal 2002, 55:1418.

This paper is part of an entire issue devoted to the same topic as this paperthe
follow-up evaluations for infants who are referred from newborn hearing screening.
This manuscript discusses tricks on getting a rapid and reliable threshold measure
using ABR and also discusses the prematurity of ASSR for this application.
23

Perez-Abalo MC, Savio G, Torres A, et al.: Steady state responses to multiple

amplitude-modulated tones: an optimized method to test frequency-specific
thresholds in hearing-impaired children and normal-hearing subjects. Ear
Hear 2001, 22:200211.

Widen JE, OGrady G: Using visual reinforcement audiometry in the assessment of hearing in infants. The Hearing Journal 2002, 55:2836.
This is another paper in the issue on follow-up testing that provides audiologists
with excellent practical tips on how and when to use VRA for the assessment of
behavioral thresholds.

24

25

Widen JE, Folsom RC, Cone-Wesson B, et al.: Identification of neonatal hearing impairment: Hearing status at 8 to 12 months corrected age using a visual
reinforcement audiometry protocol. Ear Hear 2000, 21:471487.

26

Paradise J, Smith C, Bluestone C: Tympanometric detection of middle ear

effusion in infants and young children. Pediatrics 1976, 58:198210.

27

Marchant CD, McMillan PM, Shurin PA, et al.: Objective diagnosis of otitis
media in early infancy by tympanometry and ipsilateral acoustic reflex thresholds. J Pediatr 1986, 109:590595.

28

McKinley AM, Grose JH, Roush J: Mulit-frequency tympanometry and evoked

otoacoustic emissions in neonates during the first 24 hours of life. J Am Acad
Audiol 1997, 8:218223.

29

Weatherby M, Bennett M: The neonatal acoustic reflex. Scand Audiol 1980,

9:103110.

30

Schrott A, Puel J-L, Rebillard G: Cochlear origin of 21-2 distortion products

assessed by using 2 types of mutant mice. Hear Res 1991, 52:245254.

31

Seewald RC, Moodie KS, Sinclair ST, et al.: Predictive validity of a procedure
for pediatric hearing instrument fitting. Amer J Audiol 1999, 8:143152.

32

Moodie KS, Seewald RC, Sinclair ST: Procedure for predicting real-ear hearing aid performance in young children. Amer J Audiol 1994:2331.

Beauchaine KA: An amplification protocol for infants. In A Sound Foundation

Through Early Amplification 2001; Proceeding of the Second International
Conference. Edited by Seewald RC, Gravel JS. Phonak AG;2002:10512.
This paper is found in the proceedings of an excellent meeting on amplification for
children sponsored by Phonak. The text gives a step-by-step procedure for transitioning from hearing thresholds to hearing aid fitting for infants.
33

Scollie SD, Seewald RC: Electroacoustic verification measures with modern

hearing instrument technology. In A Sound Foundation Through Early Amplification 2001; Proceeding of the Second International Conference.. Edited
by Seewald RC, Gravel JS. Phonak AG;2002:121127.
This paper is also from the pediatric amplification proceedings. This paper discusses the theoretical and practical aspects of electro-acoustic verification of hearing aid fittings on infants and young children.
34

Gravel JS: Potential pitfalls in the audiological assessment of infants and

young children. In A Sound Foundation Through Early Amplification 2001;
Proceeding of the Second International Conference. Edited by Seewald RC,
Gravel JS. Phonak AG;2002:85101.
The third recommended paper from the Phonak conference, which provides excellent case studies demonstrating the importance of the use of age-appropriate measures and of the test battery crosscheck principle. Many of the points stressed in
this review are emphasized by these case studies.
35