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193

193 Early Detection and Diagnosis of Infant

Hearing Impairment
M. Elise Graham, Kavita Dedhia, Albert H. Park

KEY POINTS with hearings, head trauma, and chemotherapy.6 Subsequent

investigation has shown that this approach identifies at most 50%
•Protocols for newborn hearing screening include a of hearing loss.7
hearing screen by 1 month of age, diagnostic hearing
testing in those who failed the hearing screen by 3 HISTORY OF NEWBORN HEARING SCREENING
months of age, and early intervention for infants
diagnosed with hearing loss by 6 months of age. The importance of hearing and its implications for speech and
language development have been recognized on a national level
•The most common causes of congenital hearing loss in in the United States since the late 1980s. During this decade,
infants without apparent risk factors are recessive then US Surgeon General Dr. C. Everett Koop recommended
genetic hearing loss and congenital human that the detection of hearing loss be made a priority for the
cytomegalovirus infection. “Healthy People 2000” national objectives. In that same decade,
•Hearing screening programs that use only evoked initiatives were introduced in Utah, Rhode Island, and Hawaii with
Otoacoustic emissions will fail to identify children with a goal of statewide screening prior to neonatal hospital discharge.
auditory neuropathy. These pilot projects were designed to test the feasibility of
expanding universal NHS to a national level.
•Surveillance of hearing and speech and language status
is essential for all children, whether the infant passed
universal newborn hearing screening or not. RESISTANCE TO NEWBORN HEARING SCREENING
•Not all conductive hearing losses are temporary or Perhaps not surprisingly, there was initial resistance to the
related to middle ear effusion. Some cases of conductive
implementation of universal NHS within the health care
hearing loss will require early intervention. community. Some felt that it was not feasible, and others felt that
•Delayed diagnosis and intervention for hearing loss can it was not necessary to implement such a large-scale project.8
lead to poor speech and language outcomes. False-positive screen results were felt to be potentially harmful,
and the upfront cost of this project was large, without a demon
stratified benefit. Early results of statewide screening proved
these concerns to be unfounded. False-positive rates, although
initially as high at 6% to 10%, have decreased to a very
EPIDEMIOLOGY OF HEARING LOSS acceptable 1% to 2% as screening methods have improved.9

Neonatal hearing loss is very common. It is estimated to occur in STUDIES OF STATEWIDE SCREENING
1 to 3 per 1000 newborns.1 Certain populations are at increased
risk, including those with a family history of hearing loss, infants The Rhode Island Hearing Assessment Project (RIHAP) began in
with perinatal infection, and those requiring a neonatal intensive 1989 and was the first to demonstrate that universal NHS,
care unit (NICU) stay. The rate of hearing loss in NICU patients including both well babies and NICU patients, was a feasible goal
is nearly double, at 2 to 4 per 1000.2 The World Health prior to hospital discharge.10 Transient-evoked otoacoustic
Organization estimates that 7.5 million children have a clinically emissions (TEOAEs) were used to test 1850 infants, identifying
significant hearing loss.3 nearly 500 who required rescreening, with an 81% follow-up rate
of rescreening in these patients. Ultimately, diagnostic testing of
STATUS OF HEARING LOSS DIAGNOSIS BEFORE 6.2% of the total group identified 11 infants with sensorineural
hearing loss (SNHL). Population-based expansion of the RIHAP
UNIVERSAL NEWBORN HEARING SCREENING over subsequent years demonstrated a reduction in the age of
Newborn hearing screening (NHS) is crucial for the early detection identification of hearing loss from nearly 9 to 3.5 months of age,
of hearing loss. Prior to universal NHS, only about 3% of infants and the age at initiation of amplification decreased from 13.3 to
in the United States were screened for hearing loss. Without 5.7 months of age.11
screening, affected children might not be identified until delayed The Colorado Newborn Hearing Project is one of the best
language development is noted; in milder cases of hearing loss, known statewide initiatives demonstrating the feasibility and
they may remain unidentified as late as school age.4 Studies necessity of NHS. Initially the Colorado project involved voluntary
have shown that earlier detection of hearing loss leads to earlier participation by 26 hospitals committed to screening all infants.
intervention, in turn improving patient outcomes.5 Screening methods were not standardized, and both automated
Before the implementation of NHS, hearing screening involved audit brainstem responses (AABRs) and otoacoustic emissions
a targeted approach based on the presence of risk factors for (OAEs) were used. The initiative demonstrated a rate of newborn
permanent hearing loss, including a family history of hearing loss, hearing loss of 1 in 500, with 47% of cases occurring in children
NICU stay greater than 5 days, certain in utero infections, cra without risk factors and therefore likely to remain unidentified
niofacial anomalies, syndromes known to include permanent without screening.7 Legislation in 1997 mandated the involvement
of all Colorado hospitals in universal NHS. This increased the
hearing loss, neurodegenerative disorders, postnatal infections associated
2887
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CHAPTER 193 Early Detection and Diagnosis of Infant Hearing Impairment2887.e1

Abstract Keywords
193
Congenital hearing loss is one of the most common birth defects. Sensorineural hearing loss
For that reason, the otolaryngologist who treats children must be universal newborn hearing screening
well versed in the evaluation and treatment of this condition. This audit brainstem response testing
chapter reviews universal newborn hearing screening, later cytomegalovirus
screening and diagnostic testing, and current paradigms for temporal bone imaging
evaluating the hearing-impaired infant. Additions to this chapter auditory neuropathy
include sections on an update on hearing screening approaches,
genetic testing, imaging, and the role of cytomegalovirus testing and treatment.
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2888 PART VIII Pediatric Otolaryngology

screening rate from 19% of infants in the voluntary program to channel. Currently two types of EOAEs are used for screening,
87% of infants screened before hospital discharge. transient ones (TEOAE) and distortion product otoacoustic
An initiative in Hawaii was also very successful in implementing emissions (DPOAE). When TEOAE is performed, small micro
universal NHS.12 This initiative began in 1992 and was successful phones are placed in the external auditory channel and a series
in screening 96% of infants. Two-stage AABR was used, leading of clicks at approximately 80 dB SPL are used to elicit the response.
to a false-positive rate of just 0.2% (3.5% with single-stage When emissions are present, responses generally indicate a
screen ing). These three pioneering programs provided the basis cochlear sensitivity of 20 to 40 dB HL or better in the range of
and feasibility of the nationwide implementation of universal NHS. 500 to 4000 Hz.17 When two pure-tone stimuli, f1 (lower
frequency) and f2 (higher frequency), are presented at moderate
NATIONAL NEWBORN HEARING STATEMENTS levels (55 to 65 dB SPL) to the ear, the cochlea will generate
DPOAEs. The DPOAEs travel from the site of generation around
AND CURRENT STATUS OF NEWBORN
the f1 and f2 frequencies to their own frequency. They also travel
HEARING SCREENING in the reverse direction and back into the ear canal, where they can be measured.
National endorsements of universal NHS were released by both The distortion product occurs somewhere between f1 and f2; the
the American Academy of Pediatrics (AAP) Joint Committee on largest DPOAEs in the human ear occur at 2f1 to f2.18 Function
Infant Hearing13 and the National Institutes of Health (NIH)14 of normal outer hair cells and the middle ear is required to obtain
in the early 1990s. By the year 2000, the Joint Committee on normal EOAEs, as the signal is transmitted from the cochlea
Infant Hearing began recommending that all infants be screened through the middle ear.
via an integrated Early Hearing Detection and Intervention (EHDI) When AABRs are used, transient acoustic stimuli are
program.4 Specifically, hearing screening should be achieved by generated and detected with probes placed in the ear canal and
1 month of age, audiometric evaluation obtained by 3 months of surface electrodes placed in the head and neck region (eg vertex,
age for those who “refer” (require further testing) or fail the mastoid region). A click stimulus is presented at 30 to 45 dB HL
screening, and intervention initiated by 6 months of age for those to evaluate the audit pathway. The automated test will give either
with permanent hearing loss. This approach is often referred to a pass or refer result. Infants should be tested in a quiet room to
as the “1-3-6” rule. limit noise, which can affect testing.19,20
By the middle of the year 2000, there was significant success The EOAE test is easier to perform and results are faster than
in implementing a national NHS program. Representative Jim those from AABRs, although the EOAE test is affected more by
Walsh was instrumental in promoting EHDI state programs that middle ear or outer ear pathology such as cerumen or middle ear
incorporated evidence-based research, monitoring, and tracking. effusion.21 On the other hand, AABR testing is more time
Statewide screening programs were initiated across the United consuming but is generally reported to obtain lower referral and
States by 2005, and the screening rate had reached nearly 95% false-positive rates (Table 193.1).22 Most of the outcomes of
by that year.15 In 2007 , the rate was estimated at 97%, compared hearing screening programs are reported using referral rate.
with 46.5% in 1999.16 NHS still poses challenges, including Sensitivity and specificity are not commonly reported because
difficulty with follow-up and early amplification. It is estimated that not all patients who pass the screen undergo diagnostic audit
nearly half of those identified with hearing loss on screening do brainstem response (ABR) testing.23 In a study by Lin and
not receive appropriate diagnostic testing or early intervention colleagues, the referral rate for TEOAE was 5.8%; for two-step
services.15 TEOAE and AABR, it was 1.6%; and for AABR, it was only
0.8%.22 The low referral rate with AABR only as compared with
NEWBORN HEARING SCREENING METHODS two-step rates was attributed to increased repetition and
experience in performing the screening. Clarke et al. compared
All newborns must undergo hearing screening prior to 1 month of the results of AABR and TEOAE in 81 newborns undergoing
age. Objective testing is used to identify hearing loss in newborns NHS. They found that the probability of a newborn passing the
because they are unable to participate in behavioral testing. AABR was much higher than that of passing the TEOAE on both
There are currently two objective screening methods, evoked the first and second screens.24 Despite the low referral rates with
otoacoustic emissions (EOAEs) and AABRs. If the response is AABR only, the increased technical difficulty and cost are barriers
not within the acceptable parameters, then the child will “refer” on to implementation in many settings.
the test, or go on to additional testing. The terms fail or failure are Many audiologists please combining the TEOAE initial test
not used to avoid negative connotations. with the AABR as the second stage of a two-stage protocol. A
EOAEs evaluate the function of the outer hair cells, which number of researchers have reported that this approach reduces
generate energy known as the cochlear amplifier; this energy the false-positive rate and minimizes unnecessary referrals.25–30
exits the cochlea through the middle ear and tympanic membrane, Shang compared two-stage TEOAE with two-stage TEOAE and
where it can be captured and measured in the external auditory AABR screening. Lower referral and false-positive rates were

TABLE 193.1 Comparison of Hearing-Screen Algorithms

Probability of Referral for Probability of Referral for

Type of Test First Screen Second Screen Strengths Limitations

OAE 5.8%–33%22,24,30 1%–6%30 Low cost, quick Affected by middle ear debris or
procedure dysfunction, high referral rate, may miss
auditory neuropathy diagnosis
AABR 0.8%–4%22,24,30 0.32%–1%30 Low referral rate, low High cost, more time-consuming, may
impact of middle miss mild hearing loss, more technically
ear pathology demanding
Two-stage: if OAE fails, OAE AABR Low referral rate Higher cost, potential to miss hearing loss
then AABR 2.4%–9.6%33,115 0.8%–2.8%22,33,115 (especially mild loss), time consuming

AABR, Automated audit brainstem response; OAE, otoacoustic emissions.

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CHAPTER 193 Early Detection and Diagnosis of Infant Hearing Impairment 2889

reported for the group tested with AABR. The rate of diagnosing The type of newborn hearing screen used is dependent on the
permanent hearing loss was the same in both groups.30 By state and/or hospital. Although the Centers for Disease Control 193
optimizing the screens and decreasing the number of false records the adherence to 1-3-6 in each state, there is currently no
positives, there will be less need for subsequent follow-ups, national tracking system for specific screening test outcomes.
superfluous testing and costs will be reduced, and there will also Some states have statewide algorithms, others leave this to the
be a reduced potential for parental anxiety. Additionally, EOAE discretion of the birthing centers. The Joint Committee on Infant
will not detect auditory neuropathy, which can be identified in newbornsHearing
only by recommendations
AABR. note that the type of screening program
Infants who are in the NICU for more than 5 days are at greater will depend on what is practical and suitable based on both cost
risk for auditory neuropathy and thus require screening by AABR. and the availability of resources.15 The first hearing screen should
A pass on TEOAE, AABR, or both tests does not mean that a be performed at least 12 hours after birth, preferably as close to
child will continue to have normal hearing. The literature reports discharge as possible, to decrease the referral rate from amniotic
that up to 22% to 30% of children who passed the NHS were fluid or debris in the ear canal. For infants who refer following the
subsequently diagnosed with hearing loss. The underlying reasons first screen, a repeat screen is recommended prior to diagnostic
include equipment issues, improper testing techniques, insufficient testing. Several studies have shown that testing infants at least
threshold for identifying mild hearing loss, or delayed onset 24 to 48 hours after birth will lead to decreased false-positive
hearing loss.31–35 results.23,24,37
Additional studies have shown that infants who have undergone In developing countries, it may be difficult to test newborns
the two-stage NHS protocol may be at increased risk for a missed after the first day, especially for home births or infants discharged
hearing loss diagnosis.33 Johnson et al. performed a multi early. Cost and hospital discharge as early as 6 hours after birth
institutional study evaluating the number of children who failed have made NHS challenging to implement in South Africa. Due to
EOAE and passed AABR, only later to be definitively diagnosed the lack of equipment and time constraints, only 53% of private
with hearing loss at 9 months of age. They determined that 22.8% hospitals in South Africa have reported performing some form of
of all infants screened met these criteria, for an overall incidence NHS. Given the higher cost of disposables for AABR, most cost
of 0.54 infants per 1000.33 Because the repeat diagnostic test conscious facilities will choose to use OAEs, although the AABR's
was performed at 9 months of age, it is difficult to quantify true lower referral rate can decrease long-term costs. Van Dyk and
false negative versus late-onset hearing loss in this study. Levit colleagues compared the BERAphone MB 11, an alternative to
and colleagues overcame this limitation by performing diagnostic AABR without disposables, to TEOAE in 150 well-baby infants.
testing within 10 to 30 days in patients who failed EOAE and Considering all children screened, AABR had a lower referral rate
passed AABR. In this group, 24% of infants were diagnosed with (16.7% to 37.9%) and lower false-positive rate (4.9% to 37.9%)
hearing loss after failing EOAE and passing AABR.36 The than TEOAE.37 The referral rate of TEOAE in this study is
relatively high intensity stimulus (45 dB HL) on the AABR in this consistent with that in other developing countries, which ranges
study may explain why mild degrees of hearing loss were missed, from 10.5% to 33.2%; However, AABR referral rates were higher
although a delayed onset of hearing loss cannot be ruled out. than what has been reported (2% to 9.1%). Performing the
With exception of children at risk for future SNHL, if a child screening in a nursery that is not soundproof and testing prior to
passes the NHS, there is no further testing until the age of 4 under 24 hours of age may explain the higher AABR referral rates.37
current guidelines. The AAPs recommend repeat hearing The NHS program in China, which was started in 2008, still
evaluations in high-risk children (Box 193.1) at the age of 24 to 30 experiences wide variability in screening rates across regions and
months regardless of NHS results.15 remains held back by scarce resources. Chinese patients in the
rural setting had low follow-up rates, especially for diagnostic
testing (42.2%). Several factors are responsible for the low follow-
up rates: the additional cost of transportation and accommodations
for families not living near a screening center, a perception that
BOX 193.1 Risk Indicators Associated With Permanent testing would not change current care, parental denial of hearing
Congenital, Delayed-Onset, or Progressive Hearing Loss loss, and cost. A recent government program covering the cost of
in Childhood screening has decreased the number of patients lost to follow-up.38
The cost of screening and lack of a national database for failed
Caregiver concern regarding, speech, language, or developmental hearing screens have also affected the NHS program in South
delay Korea.39 In Southwest Iran, rural populations had a higher referral
Family history of permanent childhood hearing loss rate than urban populations. Although they had a lower rate
Neonatal intensive care (NICU) stay of more than 5 days (4.23%) of patients lost to follow-up, they faced some of the same
NICU stay for any period of time with the following: extracorporeal problems with health care disparities and limited resources,
membrane oxygenation, assisted ventilation, exposure to requiring travel to distant diagnostic centers.40
ototoxic medications or loop diuretics, and hyperbilirubinemia
requiring exchange transfusion DIAGNOSTIC AUDITORY BRAINSTEM RESPONSE
In utero infections: toxoplasmosis, rubella, cytomegalovirus,
herpes, syphilis After a referral on universal NHS, diagnostic testing is required to
Craniofacial anomalies including those that involve the pinna, ear confirm and characterize the severity of the loss, enabling
canal, ear tags, ear pits, and temporal bone appropriate amplification or intervention. Evaluation should include
Physical findings associated with a syndrome known to include appropriate medical history of the child, any risk factors for hearing
sensorineural or permanent conductive hearing loss loss, a pregnancy and perinatal history, and family history. Tym
Any syndrome associated with hearing loss panometry and DPOAEs or TEOAEs should also be included.
Neurodegenerative disorders The most important component to establish the degree, with
Culture-positive postnatal infections associated with sensorineural figuration, and nature of the hearing loss is a frequency-specific
hearings tone-burst ABR with both bone and air conduction testing.
Head trauma, especially basal skull/temporal bone fracture ABRs are powerful noninvasive tools allowing the estimation of
requiring hospitalization pure-tone thresholds through comparison of the waveform outputs
Chemotherapy with normative data and the child's contralateral ear.41 The lowest
intensity level that elicits the ABR indicates the pure-tone
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2890 PART VIII Pediatric Otolaryngology

TABLE 193.2 Joint Committee on Infant Hearing Recommendations waveforms elicited from broad-spectrum click or tone-burst stimuli
for Diagnostic Audiometric Testing by Patient Age is removed.46 It also allows delivery of simultaneous frequencies,
potentially decreasing the time required for testing.45 Recent
Birth to 6 Months of Age 6–36 Months of Age
studies comparing ASSR with ABR, however, have reported varying results.
Child and family history Child and family history A 2016 study by Celik et al. demonstrated moderate threshold
Frequency-specific ABR with Parental report of audit/visual correlation between ASSR and ABR, whereas previous studies
air and bone tone bursts behaviors and communication have shown a stronger correlation.47 Data validating ASSR for
milestones bone conduction specifically are also limited, but they may be useful
Click-evoked ABR (especially if Behavioral audiometry (visually in patients with conductive hearing loss between 0.5 and 4 kHz.48
“no response” to tone reinforced vs. conditioned play) At this point ASSR is not recommended as an alternative to ABR,
burst) but it may serve as a complementary test.
Distortion product or transient Pure-tone audiometry with speech
evoked OAEs detection/recognition BEHAVIORAL TESTING
Tympanometry with 1000-Hz OAE
probe tone Visually reinforced audiometry (VRA) is the behavioral diagnostic
method of choice in children who are developmentally 6 months to
Clinical observation of audit Tympanometry/reflexes
2 years of age.49 Very young infants (<5 months of age) are not
behavior (as a cross check)
developmentally ready to perform behavioral testing.50 VRA
ABR testing if behavioral testing not involves presenting a sound to the child and rewarding appropriately
reliable or not previously timed head turns with a picture or other visual stimulus.51 Inserts
performed
or headphones can be used to obtain ear-specific information, with
ABR, Auditory brainstem response; OAEs, otoacoustic emissions. inserts being preferred to avoid collapsing of the channels.
Inserts are more reliably tolerated by children above 18 months of
age, although a number of pediatric centers have reported excellent
threshold in the tested child. ABR testing is ideally performed during success in obtaining individual thresholds in younger children.52
natural sleep to avoid additional resources (ie, operating room time, Ideally 500-, 1000-, 2000-, and 4000-Hz thresholds should be
personnel, and monitoring) and risks associated with sedation. Most obtained if possible. A positive response generally requires a 90-
children under 6 months of age can be tested during natural sleep. degree head turn, and normative data by age are available for
Scheduling of tests near nap times, preventing sleep during interpretation.50
transportation to the appointment and the night before, absence of Conditioned play audiometry (CPA) is a technique available to
siblings at the appointment, and feeding on arrival may all increase children who are developmentally 2 to 5 years of age. In this
the likelihood that the infant will be sleeping at the time of technique, the child is first briefly conditioned to ensure that he or
testing.42,43 ABRs are performed by specific placement of she understands the task and can reliably participate. The chosen
electrodes on the forehead and mastoid processes and either task may vary based on the child's interest and cooperation—for
headphones or inserts for the child's ears. The protocol for ABR example, putting a peg in a board, tossing a block in a box, or a
varies by institution and audiologist, but the goal is to obtain digital task in response to hearing a sound.53 Again, frequencies
thresholds at as many useful frequencies as possible while the are chosen to maximize the useful information obtained prior to
child remains asleep. If the child is sleeping, testing is often started patient fatigue.
at 50 to 60 dB to avoid waking the child. Then these stimuli are
sequentially increased by 10 dB threshold untils can be estimated.44 COMPARISON OF METHODS: STRENGTHS
A survey by Munoz et al. indicates that, despite the EHDI
guidelines, parents may have difficulty accessing the recommended AND LIMITATIONS
diagnostic tools and appropriately trained personnel.42 Of the The various diagnostic testing methods are complementary. By
surveyed institutions, almost half, despite being identified as combining several methods of assessment, one reduces the
hospitals with pediatric capability, were unable to offer the complete limitations of any one test. OAEs are quick and require minimal
battery recommended by the Joint Committee. The complete list of cooperation, but they will fail to detect auditory neuropathy. ABRs
recommended tests by patient age is included in Table 193.2. require more time but give more detailed information, including ear-
The tests with limited availability included bone-conduction ABR, specific thresholds. Behavioral observation is noninvasive but
cochlear microphonic obtained through click ABR, and behaviorally unlikely to give ear-specific thresholds until the child can tolerate
observed audiometry. Wait time to access these appointments also inserts or headphones. It also requires a relatively cooperative child.
tended to be unacceptably long, with some delays ranging up to 3 Testing options vary significantly depending on the age and
to 5 months. Changes in practice and resource utilization are still developmental maturity of the child. VRA and, more likely, CPA
needed to meet the Joint Committee's recommendations. may allow assessment of ear-specific thresholds, and an older child
Although the ABR is considered the gold standard among (over the age of 5 years) may be able to participate in conventional
diagnostic tests for newborn hearing assessment, it requires time pure-tone audiometric testing. It is important to use age-appropriate
and specialized training for interpretation. When testing cannot be testing as soon as hearing loss is identified through screening. If a
completed during natural sleep, sedation or general anesthesia center cannot perform the appropriate testing, the child should be
may be necessary, which can be a significant burden in terms of referred, as delay is unacceptable.
resources in addition to subjecting the child to additional risks.
EVALUATION OF THE INFANT WITH
AUDITORY STEADY-STATE RESPONSE SENSORINEURAL HEARING LOSS
An attractive but less studied alternative to the ABR is audit steady- A multidisciplinary hearing assessment clinic was established at the
state response (ASSR) testing. ASSR delivers continuous sinusoids University of Utah in 2006 to help families whose child had been
rather than tone bursts, as in the diagnostic ABR.45 ASSR may diagnosed with hearing loss. The core team includes pediatric
provide some advantages over ABR in that a statistical algorithm genetics, audiology and otolaryngology, with additional specialists
determines whether a response is present. Thus, most of the as needed, including neurology, ophthalmology, infectious disease,
subjectivity needed for conventional methods of reading and child development. The diagnostic approach is based on early
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CHAPTER 193 Early Detection and Diagnosis of Infant Hearing Impairment 2891

screening for cytomegalovirus (CMV), followed by genetic testing and heterochromia iridis. The standard otolaryngology physical
or imaging as informed by the child's presentation. examination of the pinna for abnormalities includes microtia, pits, 193
In 2014 we proposed that CMV testing should be performed and tags; the caliber of the ear canal; and the appearance of the
first for the child who presents with idiopathic SNHL.54 Early CMV tympanic membrane. The presence or absence of middle ear
testing was found to be of high yield; it also reduced costs based effusion should be noted. Nasal examination should be per
on the avoidance of unnecessary genetic testing or temporal bone formed. Examination of the oral cavity should focus on the size of
imaging. If CMV testing is negative, additional testing is the jaw and the presence of clefts. The neck exam should
recommended based on the laterality of the hearing loss. For specifically assess for branchial anomalies and size of the thyroid.
bilateral hearing loss, genetic testing is recommended. For Cranial examination should also be performed, with particular
focus on facial nerve function.
unilateral or asymmetric hearing loss, imaging is recommended. Fig. 193.1
presents a proposed flowchart developed by an international
pediatric otolaryngology group.55 INTERPRETATION OF MIDDLE EAR FLUID
All patients presenting with hearing loss should be examined
by an otolaryngologist. Initial assessment includes a general IN THE INFANT
examination for obvious abnormal facies, dysmorphic features, Otitis media with effusion (OME) is a common finding in infants
asymmetry, or pigmentary abnormalities such as hair color changes who fail their NHS. Several large studies have reported that between

Sensorineural hearing
loss

Unilateral Bilateral

CMV Testing

Comprehensive
genetic testing (if
available) or single
Progressive or gene testing*
Asymmetric loss
No evidence of
associated genetics
syndrome on physical Temporal bone imaging
exam

Consider
Negative Positive

Not additional Additional Nonsyndromic

physical findings physical findings hearings

Consider:
1. Novel genetics
causes Genetics evaluation;
consider directed Pos Syndromic
2. Other risk factors hearings
genetic testing
for acquired
hearings
3. Enlarged
vestibular
aqueduct Negative

Habilitation, follow up

Fig. 193.1 Flowsheet outlining the recommended testing for the child who presents with sensorineural
hearing loss. Regardless of the laterality, we recommend that cytomegalovirus (CMV) testing be performed
first. If the CMV test is negative, one should consider a gene panel or testing for the connexin 26 mutation in the
child with bilateral sensorineural hearing loss. For unilateral sensorineural hearing loss and no evidence of a
syndromic condition, one should consider obtaining imaging.
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2892 PART VIII Pediatric Otolaryngology

55% and 65% of infants who failed their NHS have OME.56,57 whereas dry saliva CMV PCR has a sensitivity and specificity of
OME may provide false reassurance to the primary care provider 97.4% and 99.9%, respectively.21 However, a prospective study
and family as an explanation of why the newborn was referred on of 20,000 newborns in Finland reported that 15 of the 56 infants
the hearing screening test. One study found that none of the 64 with a positive saliva CMV PCR had a subsequent negative urine
infants with OME who were referred on NHS were found to have CMV PCR result.66 Presumably breast milk from the seropositive
an underlying SNHL.57 In contrast, Boone et al. detected an mothers resulted in the abnormal result. Therefore we recommend
underlying SNHL in 11% of infants who presented with a failed waiting at least 90 minutes after breastfeeding to obtain a saliva
NHS and middle ear fluid.56 Clearly the family should be informed sample; if that is positive, a urine sample should be obtained to
that the presence of a middle ear effusion does not rule out the confirm the diagnosis.
possibility of SNHL. As stated in a clinical practice guideline from As archived dried blood spots (DBS) are obtained in most
the American Academy of Otolaryngology–Head and Neck newborns within the first week of life, the DBS can be tested for
Surgery Foundation, “Clinicians should document in the medical CMV DNA when the child is diagnosed with hearing loss after the
record [when] counseling [of] parents of infants with OME who first 3 weeks of life. However, the sensitivity of DBS testing is
fail a newborn hearing screen the importance of following -up to only 34%, which is likely attributable to the lower CMV viral load
ensure that hearing is normal when OME resolves and to exclude found in blood compared with urine or saliva.67–69 The poor
an underlying SNHL.”58 sensitivity of CMV PCR from DBS makes it unsuitable for mass
The best approach to the infant with OME and failed NHS is screening for congenital CMV. However, the test's high specificity
unclear, as a prolonged period of surveillance may not be ideal. of 99.9% is clinically useful, since a positive DBS result is
Boudewyns et al. noted that only 15 of 64 infants with middle ear diagnosis of congenital CMV infection.67
effusion presenting at an average age of 49 days had resolution Although previous algorithms for the evaluation of idiopathic
of their fluid by 4.8 months of age.57 Doyle et al. have noted that pediatric SNHL have focused on genetic testing or temporal bone
neonatal OME is a risk factor for subsequent chronic otitis media imaging,70,71 we developed a sequential diagnostic paradigm
through the first year of life.59 In addition, the need for repeated that incorporates CMV testing as the first step.54 Eighty-three
clinical visits and delays in treatment are sources of significant children underwent CMV testing, imaging, and a genetic
parental dissatisfaction with the NHS process,60 which likely evaluation. Those with confirmed or probable CMV-induced
contributes to the high rates of infants lost to follow-up in many SNHL comprised 30% of all children tested. A cost-estimate
programs.7,61 analysis demonstrated that early CMV testing had the lowest cost
The approach at our institution is to allow 3 months for OME per positive diagnosis for all types of hearing loss except for
to resolve, proceeding with tympanostomy tube placement if auditory neuropathy spectrum disorder (ANSD). For ANSD,
unilateral or bilateral fluid persists. Infants at high risk for SNHL, imaging had the lowest cost.
such as those with Down syndrome or NICU graduates, also As shown in Fig. 193.2, it is difficult to incorporate CMV testing
undergo tympanostomy tube insertion at this time. The infant is for idiopathic SNHL because a urine sample must be obtained by
typically evaluated 3 to 4 weeks after tube placement and 3 weeks of age. It is simpler to perform CMV testing for any infant
undergoes OAE testing. Although OAEs may be absent in the who fails the NHS before that age (Fig. 193.3).
presence of a patent tympanostomy tube, a normal result will Stehel et al.72 instituted a protocol at a large birthing hospital to
obviate the need for follow-up ABR, and the child will return in 6 perform a urine CMV culture or PCR assay for any infant who did
months for behavioral hearing testing. If the test is abnormal, not pass an AABR before discharge. Over a 5-year period, 24 of
there is still adequate time for the infant to undergo a diagnosed 572 (5%) infants who did not pass the inpatient NHS tested
ABR without the need for sedation. Further evaluation and positive for cCMV. Notably, these children would have been
treatment is determined by the results of the ABR. unlikely to have been diagnosed otherwise. Identification of these
children also provides an opportunity for audiologic rescreening
CONGENITAL CYTOMEGALOVIRUS to monitor for the development of late-onset hearing deficits that
could affect language acquisition and educational achievement.
CMV is the most frequent congenital viral infection in the world. A hearing-targeted CMV (HT-CMV) screening approach has
The incidence for congenital CMV (cCMV) infection ranges from been used in Utah since 2013. A CMV PCR or culture is
0.5% to 1.7% of all newborns.62–64 Of those with cCMV, up to performed whenever an infant fails the NHS and is less than 3
20% have permanent disabilities, among which hearing loss is weeks of age.73 Identifying cCMV is important, since at least
the most common. The spectrum of presentation ranges from 50% of cCMV-infected hearing-impaired infants are at risk for
asymptomatic (aCMV) infection, with no clinically apparent developing progressively worse hearing over time.73–75 Utah
disease, to symptomatic (sCMV), with clinically apparent disease became the first state in the country to implement HT-CMV when
in one or more organ systems. The definition of symptomatic the bill was signed into law in July 2013, based on the efforts of
infection has been confusing, as some authors have included our CMV working group.76
SNHL as a “symptom,” whereas others have not. For the Results from the Utah statewide program found that 62% of
purposes of this chapter, we define symptomatic disease to infants who did not pass their hearing screening underwent CMV
include evidence of thrombocytopenia, petechiae, screening.77 Over the subsequent 5 years, the compliance rate
hepatosplenomegaly, intrauterine growth restriction, hepatitis, has improved to over 90%. In addition, 77% of all infants born
central nervous system (CNS) involving ment, and chorioretinitis. after the legislation received timely diagnostic hearing evaluation
We separate those with isolated SNHL as a distinct category. compared with 56% in the 24 months before the legislation.
Hearing loss has been reported in up to 75% of children with Delays in the diagnosis and treatment of hearing loss are major
symptomatic disease, whereas the incidence of hearing loss in challenges for universal NHS programs. Thus HT-CMV screening
infants with aCMV is 10% to 15%.65 Over half of cCMV-infected has positive implications for all infants with hearing loss.
CMV screening addresses a major concern for families whose
infants who present with hearing loss at birth will develop progressive hearings.65
Since the majority of congenital CMV infections are child has recently been diagnosed with SNHL. In a national
asymptomatic, the diagnosis relies on culture-based or polymerase survey of US parents, 84% of respondents stated that they would
chain reaction (PCR) methods. To distinguish between congenital want to have their baby tested for CMV even if their doctor or
and postnatal CMV infection, a urine or saliva PCR or culture hospital did not perform the test routinely.78 An identical
should be obtained within the first 3 weeks of life. Wet saliva CMV percentage of respondents would want to know if their child had
PCR was previously shown to be 100% sensitive and 99.9% specific, cCMV even if he or she never developed problems and that they would be
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CHAPTER 193 Early Detection and Diagnosis of Infant Hearing Impairment 2893

SNHL 193

History and physical examination,

audiologic findings

Apparent diagnosis Idiopathic diagnosis

CMV urine PCR or

culture
POSITIVE
NEGATIVE
>3 weeks old
POSITIVE
<3 weeks old

Idiopathic
Possible or probable diagnosis
CMV-induced SNHL
Definitive
CMV-induced
SNHL
Genetic testing, imaging,
DBS PCR CMV Assay ophthalmology and EKG

NEGATIVE POSITIVE POSITIVE NEGATIVE

Definitive Genetic or Idiopathic

Possible or probable CMV-induced diagnosis
CMV-induced SNHL inner ear
SNHL dysplasia

MRI brain AND temporal bone; serial audiologic testing

POSITIVE NEGATIVE

Probable CMV Idiopathic

induced SNHL diagnosis

Fig. 193.2 Flowchart illustrating a sequential diagnostic paradigm that incorporates cytomegalovirus (CMV)
and ancillary testing depending on the age of the child. The level of complexity increases when the child is
older than 3 weeks of age. DBS, Dried blood spots; PCR, polymerase chain reaction; SNHL, sensorineural
hearing loss.

willing to pay for screening. In a review of all infants tested for hearing-impaired and one was a twin with normal hearing.80 It is
cCMV in British Columbia over an 8-year period,79 14 of 701 essential to identify sCMV patients in order to provide the option
infants, or approximately 4.2 per 100,000 live births, had a positive of therapy.
test for symptomatic CMV. As 50 cases per 100,000 births would Ganciclovir has been the most studied antiviral medication to
be expected, these results indicate that more than 90% of treat cCMV. It is a nucleoside analog of guanosine that is
expected sCMV infections were not diagnosed when testing was structurally related to acyclovir and blocks viral replication.
based on clinical suspicion. In our series, we detected 4 infants Valganciclovir is converted to ganciclovir via hepatic and intestinal
with cCMV and CNS abnormalities who were not diagnosed clinically; 3esterase
were and has the advantage of oral administration, whereas ganciclovir m
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2894 PART VIII Pediatric Otolaryngology

Newborn hearing
screen: Universal CMV screening
(Uni-CMV)

PASSED FAILED

CMV CMV
Positive Negative
CMV CMV
Positive Negative

Hearing Hearing
NORMAL LOSS
Hearing Hearing
NORMAL LOSS

Fig. 193.4 Flowchart illustrating how a universal cytomegalovirus

Fig. 193.3 Flowchart showing how a hearing-targeted early screening (CMV) screening approach would facilitate a diagnosis of
approach for cytomegalovirus (CMV) can facilitate the diagnosis of congenital CMV.
congenital CMV infection.

be administered intravenously. The National Institute of Allergy been documented with ganciclovir; However, these patients were
and Infectious Disease Collaborative Antiviral Study Group (CASG) frequently taking other nephrotoxic medications, making it difficult
presented a large randomized controlled study comparing 6 weeks to ascribe the toxicity to ganciclovir.84 Studies have demonstrated
of intravenous ganciclovir with no treatment in infants less than 30 impaired fertility and carcinogenic properties following ganciclovir
days of age with CNS involvement.81 They found that infants exposure in mice.85 Infertility or cancer has not been reported to
treated with ganciclovir were more likely to have stable hearing or be associated with ganciclovir or Valganciclovir use in any pediatric
even hearing improvement compared with those infants who patient despite its widespread use in CMV treatment for pediatric
received no treatment. None of the treated infants had hearing transplant recipients.
deterioration at 6 months compared with 41% of the untreated A major question is whether cCMV infected children with mild
infants. At the 1-year follow-up, 21% infants in the treated group disease or those with isolated SNHL would benefit from
had some hearing deterioration in the better ear compared with valganciclovir therapy. A number of consensus and review papers
68% in the untreated group. have recommended no treatment for this group of children, given
Amir et al. reported their results in 23 infants with culture the lack of evidence-based data.86–88 A double-blind placebo
proven congenital CMV treated with ganciclovir for 6 weeks controlled clinical trial has been funded by the NIH to determine
followed by oral valganciclovir given until age 12 months.82 the efficacy and safety of valganciclovir therapy for cCMV-infected
Within these 23 children (46 ears), none of the 25 children with infants with isolated SNHL. Infants between 1 and 6 months of age
normally hearing ears developed worsening of hearing by 1 year diagnosed with cCMV and isolated SNHL will be invited to
of age. For the 21 ears presenting with hearing loss, 12 (57%) participate at one of 30 sites in the United States.
had improved hearing, 8 (38%) had no change, and 1 (5%) had a A European consensus statement recommends targeted CMV
worse threshold at 1-year follow-up. These preliminary results screening for infants less than 3 weeks of age with signs or
indicate potential efficacy with longer duration of therapy. symptoms of sCMV such as microcephaly, intrauterine growth
A recent clinical trial by the National Institute of Allergy and retardation, hepatosplenomegaly, elevated transaminases,
Infectious Disease CASG confirmed greater efficacy with longer petechiae, unexplained thrombocytopenia, or intracranial abnormalities.87
therapy.83 A 6-month course of valganciclovir antiviral treatment By implementing these recommendations in only three NICUs, we
began before the end of the first month of life improved hearing diagnosed eight cCMV-infected infants over the past 2 years.
and neurocognitive outcomes in infants with clinically apparent Universal CMV screening has also been advocated in a recent
symptomatic infection as compared with those treated for only 6 consensus statement (Fig. 193.4)89 to identify all cCMV-infected
weeks.83 newborns, including those who pass the NHS. Fowler and col
The major toxicities associated with ganciclovir and valgan leagues reported that a targeted CMV approach that tests
cyclovir are bone marrow depression, specifically neutropenia.81 newborns who fail their NHS identified the majority (57%) of infants
Of the 46 (63%) ganciclovir-treated patients, 29 developed Grade with CMV-related SNHL at birth. However, this approach failed to
3 or 4 neutropenia. Of the 29 ganciclovir-treated patients identify 43% of the infants with CMV-related SNHL in the neonatal
developing neutropenia, 14 required dosage adjustment and 4 period as well as cCMV-infected infants who are at risk for late-
had the drug permanently discontinued. Neutropenia was observed onset SNHL.90 In this study, the type of NHS and sub sequent
only during the first 3 months of treatment, mainly during the behavioral hearing testing were not presented. Such early
intravenous ganciclovir administration in the study by Amir et al. progressive loss is surprising, since an earlier study by Fowler et
Of the 12 patients who developed neutropenia, 2 required al. reported a smaller percentage of cCMV infants with neonatal
discontinuation of treatment for 2 to 3 days until the absolute SNHL.75 As the HT-CMV approach becomes more widely
neutrophil count normalized. Renal toxicity has also adopted, better data will be available.
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CHAPTER 193 Early Detection and Diagnosis of Infant Hearing Impairment 2895

If universal CMV screening were to be implemented, against transmission to the fetus.64 An effective vaccine must
approximately 4 million newborns in the United States would have provide protection for both seronegative and seropositive mothers. 193
to be screened. Currently there are no data to support early There fore a clinically effective vaccine for cCMV is probably years
identification of asymptomatic CMV-infected infants with normal to decades away.
hearing at birth, especially since most will never develop SNHL.91
For those who develop late-onset SNHL, it is unclear whether early
GENETIC MUTATION
diagnosis would improve their speech and language outcomes.
However, there may be value to patients and families in identifying Genetic factors are a common cause of hearing loss (see Chapters
the etiology of hearing loss, if only to obviate the need for genetic 149 and 150). It is estimated that 50% of congenital hearing loss is
testing. A HT-CMV approach capitalizes on the success of an genetic in nature, among which 70% of cases are nonsyndromic.
ongoing NHS program and would involve additional testing of a In syndromic cases, hearing loss is one of a constellation of
much smaller number of infants. Assuming a 1.5% referral rate, coexisting signs and symptoms in the patient. Over 500 syndromes
approximately 60,000 newborns in the United States would have to are associated with hearing loss; therefore it is not possible to
undergo CMV testing to identify those at greatest risk to develop screen for every genetic etiology. The appropriate screening
progressive SNHL. technique remains a topic of debate among experts, with some
Gantt et al. recently estimated the cost-effectiveness of universal advocating for a directed approach and others for screening with
and targeted newborn cCMV screening programs compared with no larger panels. In general, patients with bilateral congenital hearing
cCMV screening.92 They reported that among all infants born in the loss without an obvious inciting factor should undergo genetic
United States, identification of one case of cCMV infection by testing.102
universal screening has approximately five times the cost of targeted
screening. This disparity between the two approaches reaches 28-
IMAGING
fold compared with the cost of identifying one case of hearing loss
from cCMV infection. Assuming a screening cost of $10 per case, In children with congenital hearing loss, imaging detects inner ear
identifying one case of hearing loss due to cCMV would be $27,460 abnormalities (see Chapter 194) in 27.4% to 39% of cases.70,103,104
by universal screening or $975 by targeted screening. Unfortunately, The most common radiographic abnormality is an enlarged vestibular
at least in Utah, the cost per case is closer to $300, which makes aqueduct, which is associated with progressive SNHL or Pendred
universal screening prohibitively expensive. We also performed an syndrome. The most common radiographic abnormality for unilateral
ex ante cost-benefit analysis of the Utah law directed at HT-CMV SNHL is a hypoplastic cochlear nerve. Imaging abnormalities are
screening. We found, from a societal perspective, that if antiviral typically seen more frequently in children with asymmetric or
therapy was successful in preventing a cochlear implant in one unilateral SNHL compared with those who have bilateral loss.
infant per year, the savings would offset the costs associated with
screening and treatment for those on public insurance.93 HRCT has historically been considered the investigation of choice
for patients with hearing loss.105 It is able to identify cochlear
Once a child is diagnosed with cCMV, a multidisciplinary dysplasia, labyrinthine ossification, dehiscence of the semicircular
approach is recommended to determine the severity and extent of canals, the position of the tegmen and sigmoid sinus, as well as the
disease. A head ultrasound is performed in children without obvious course of the facial nerve and possible dehiscence.106,107
CNS involvement; MRI is performed if ultrasound is abnormal or for However, approximately 57% of soft tissue abnormalities of the
children with CNS signs. The battery includes a complete blood inner ear may not be identified with CT scan,108 which is especially
count with differential, comprehensive metabolic profile, important when the presence or absence of the cochlear nerve is to
ophthalmology evaluation, plus early intervention and diagnostic be determined.107 Clemmens et al . reported that 26% of children
ABR study. We categorize the groups into asymptomatic, isolated with unilateral SNHL will have a hypoplastic cochlear nerve.109
SNHL, or symptomatic (Fig. 193.5). The cochlear nerve is defined as abnormally small if, on a sagittal
One of the most exciting areas of CMV investigation has been in projection of the internal auditory nerve bundle, the nerve is found
therapy. One provocative paper by Nigro et al. in 2003 reported to have a smaller diameter than the facial nerve or if the color of the
positive results from using passive immune globulin during pregnancy nerve is a lighter gray than the color of the facial nerve.109 Although
to prevent transmission of CMV from mother to fetus.94 stenosis of the cochlear nerve canal as measured on HRCT is used
Unfortunately a phase III double-blind randomized clinical trial using to indirectly predict cochlear nerve aplasia, it may not be a sensitive
hyperimmune globulin failed to demonstrate efficacy in the course enough modality.106,110 A bony cochlear nerve canal that is less
of primary CMV infection during pregnancy.95 Two future studies, than 4.7 mm in diameter is a strong predictor of cochlear hypoplasia.
one sponsored by Biotest and the other by the Eunice Kennedy
Shriver National Institute of Child Health and Human Development Currently MRI temporal bone imaging is the imaging modality of
(ClinicalTrials.gov NCT01376778), may provide further insight into choice. Although imaging takes longer and is more expensive than
the possible role of treatment for CMV infection during pregnancy. HRCT, the ability to measure the cochlear nerve's diameter and the
avoidance of ionizing radiation are compelling reasons to prefer
Another approach uses pre-conception immunity to human CMV MRI. The diameter of the cochlear nerve is especially important
to reduce the impact of congenital HCMV infection.96–98 when a cochlear implant is being considered. An absent cochlear
An Institute of Medicine (IOM) report, “Vaccines for the 21st Century,” nerve is a relative contraindication for cochlear implantation, since
characterized HCMV vaccines as the highest-level (Level 1) priority a functioning cochlear nerve is critical for a successful outcome.
for vaccines in the new millennium.99,100 Pass et al. reported Children with hypoplastic cochlear nerves will have poorer hearing
results of their Phase II clinical trial comparing a recombinant CMV outcomes compared with those who have normal sized nerves.111
envelope glycoprotein B administered with MF59 adjuvant with
placebo.101 The recombinant vaccine demonstrated an efficacy of
50% for protection against acquisition of a primary HCMV infection
AUDITORY NEUROPATHY SPECTRUM DISORDER
in young women. Kaplan-Meier analysis showed that the vaccine
group was more likely to remain uninfected during a 42-month period ANSD is a heterogeneous disease characterized by behavioral
than the placebo group (P thresholds that do not match with other auditory measures such as
= .02). However, a major challenge with this approach is that prior ABR or speech discrimination scores. There are multiple etiologies,
immunity to cCMV does not confer complete protection. including genetic (eg mutation of the otoferlin [OTOF]
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2896 PART VIII Pediatric Otolaryngology

If any of the following present:

1) Mother positive for CMV infection 7) AST or ALT >100 U/L OR unexplained
during pregnancy direct bilirubin >1.0 mg/dL
2) Abnormal head size (OFC <10th %ile 8) Petechial rash or blueberry muffin
OR >90th %ile at birth) rash at any time
3) Intrauterine growth restriction (weight 9) Leukomalacia, polymicrogyria,
<10th %ile for gestational age) lissencephaly, pachygyria, schizencephaly
4) Unexplained hydrops 10) Unexplained persistent
5) Intracranial OR intraabdominal thrombocytopenia (platelets < 100k/mm3)
calcifications on first imaging exam 11) Failed hearing screen
6) Unexplained hepatomegaly OR
splenomegaly (>1cm below the right or
left costal margin)

Send urine CMV PCR

(obtained by 21 days of life when possible)

If CMV +, perform all of the following tests:

• CBC + differential • Head ultrasound
• CMP • Hearing: Diagnostic ABR, OAE
• Ophthalmology (inpatient or outpatient) (Tympanometry and Bone if indicated)
within 2 weeks of + test • Refer to Early Intervention

ASYMPTOMATIC if all of: SYMPTOMATIC if ÿ one of:

• Normal • Normal platelet Isolated • Thrombo • Abnormal HUS
ophthalmology count Sensorineural cytopenia • Hepatitis
exam • Non-megaly Hearing Loss • Hepatomegaly • Sensorineural
• Normal ABR* hepatosplen • Splenomegaly the hearings (if
• Normal head • Normal liver • IUGR/SGA also ÿ one of
ultrasound functions • Microcephaly above

*Normal ABR ÿ25 dBHL at all test frequencies (500,

1k, 2k, and 4k whenever possible) with present OAEs

At 3 months of age:
By 4 weeks of age:
Follow-up with audiology • Consult Pediatric ID to discuss antiviral
and otolaryngology treatment
• Contact Pediatric Neurology if abnormal
HUS or microcephaly

Fig. 193.5 Recommended workup for the child diagnosed with congenital cytomegalovirus (cCMV) infection.
The tests can help determine the severity of infection (asymptomatic, isolated sensorineural hearing loss, or symptomatic).
We recommend diagnostic testing of the auditory brainstem response in any child diagnosed with cCMV regardless of the
status of the newborn hearing screening test. ABR, Auditory brainstem response; ALT, alanine aminotransferase;
AST, aspartate aminotransferase; CBC, complete blood count; CMP, comprehensive metabolic panel; dBHL, decibel
hearing level; HUS, head ultrasound; IUGR, intrauterine growth retardation; OAE, otoacoustic emission; OFC, occipital
frontal circumference; PCR, polymerase chain reaction; SGA, small for gestational age.

gene) factors, ototoxic drugs, and anatomic (eg, cochlear nerve avoid high-intensity amplification. Some of these children will
hypoplasia) irregularities. The earliest cases of ANSD were become candidates for cochlear implantation, whereas others will
attributed to “high-risk” infants, specifically premature infants demonstrate hearing threshold improvement over time.112,113
admitted to the NICU or those who sustained a prolonged or Harrison et al. reported that of 75 ANSD patients evaluated at the
difficult delivery. As noted earlier, identification of ANSD requires Hospital for Sick Children in Toronto, one in five showed some
testing by AABR or diagnostic ABR, since TEOAEs may be present. threshold recovery that allowed adequate speech and language
Unlike the typical case of a child with SNHL, hearing aids may development without the need for amplification.114
not be indicated for the treatment of a child with ANSD.
Early intervention and regular audiologic assessments are crucial.
SUMMARY
If the child has normal behavioral and speech reception thresholds,
hearing aids are not indicated. If behavioral responses are poor, Over the past decade, there has been a revolution in the detection
then hearing aids may be considered at a conservative gain to and diagnosis of pediatric SNHL. Nationally, over 90% of infants
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CHAPTER 193 Early Detection and Diagnosis of Infant Hearing Impairment 2897

are being screened for hearing loss. With the implementation of NHS, Children are not receiving timely diagnostic testing or treatment.
congenital hearing loss is confirmed at an earlier age. Recent initiatives on early CMV screening may help overcome the 193
Currently EOAE only, AABR only, or a two-stage combination protocol is challenge posed by loss to follow-up. Hearing-targeted early CMV
acceptable for NHS. There is no gold standard throughout the United screening has shown much promise in diagnosing previously missed
States or even within each state. Multiple studies have reported limitations cCMV-infected infants and in improving the time to diagnosis and
and strengths of each protocol. treatment for all infants who fail their NHS.
In the future, it would be beneficial to have a unified protocol for screening, Better diagnoses and novel interventions are needed for this condition.
with tracking of referral rates from each screening test.
Furthermore, improvements in both the sensitivity and specificity of the
EOAE and AABR tests themselves can decrease the overall cost and Acknowledgments
The author would like to acknowledge the help of Jill Boettger, Roger
false-positive rates.
Faix, Stephanie McVicar, Lonnie Miner, Beth Knackstedt, Elizabeth
Advances in genetic testing, imaging quality, and early CMV screening O'Brien, Betsy Ostrander, Kayla Hirschmugl, and Lindsey Tubaugh.
are providing families with an etiology for their child's hearing loss.
Unfortunately, loss to follow-up is still occurring at various stages of
patient evaluation. As a result, too many For a complete list of references, visit ExpertConsult.com.