Clinical Review & Education

JAMA | Review

Hearing Loss in Children

A Review
Judith E. C. Lieu, MD, MSPH; Margaret Kenna, MD, MPH; Samantha Anne, MD; Lisa Davidson, PhD

Author Audio Interview

IMPORTANCE Hearing loss in children is common and by age 18 years, affects nearly 1 CME Quiz at
of every 5 children. Without hearing rehabilitation, hearing loss can cause detrimental jamacmelookup.com
effects on speech, language, developmental, educational, and cognitive outcomes
in children.

OBSERVATIONS Consequences of hearing loss in children include worse outcomes in

speech, language, education, social functioning, cognitive abilities, and quality of life.
Hearing loss can be congenital, delayed onset, or acquired with possible etiologies including
congenital infections, genetic causes including syndromic and nonsyndromic etiologies, and
trauma, among others. Evaluation of hearing loss must be based on suspected diagnosis,
type, laterality and degree of hearing loss, age of onset, and additional variables such as
exposure to cranial irradiation. Hearing rehabilitation for children with hearing loss may
include use of hearing aids, cochlear implants, bone anchored devices, or use of assistive
devices such as frequency modulating systems.

CONCLUSIONS AND RELEVANCE Hearing loss in children is common, and there has been
Author Affiliations: Author
substantial progress in diagnosis and management of these cases. Early identification affiliations are listed at the end of this
of hearing loss and understanding its etiology can assist with prognosis and counseling of article.
families. In addition, awareness of treatment strategies including the many hearing device Corresponding Author: Judith E. C.
options, cochlear implant, and assistive devices can help direct management of the Lieu, MD, MSPH, 660 S Euclid Ave,
Campus Box 8115, St Louis, MO 63110
patient to optimize outcomes.
(lieujudithe@wustl.edu).
Section Editors: Edward Livingston,
JAMA. 2020;324(21):2195-2205. doi:10.1001/jama.2020.17647 MD, Deputy Editor, and Mary McGrae
McDermott, MD, Deputy Editor.

H
earing loss in children is common (Box 1); by age 18 years, The multichannel cochlear implant was initially approved in the
it affects nearly 1 of every 5 children in the United States. United States in 1990 for children 2 years or older; the age was low-
Without hearing rehabilitation, hearing loss can cause det- ered to 18 months in 1998, 12 months in 2000, and then 9 months
rimental effects on speech, language, developmental, educational, in March 2020.3 The combination of newborn hearing screening pro-
and cognitive outcomes in children. Hearing rehabilitation can miti- grams (Box 2), advances in cochlear implant and hearing aid tech-
gate those detrimental effects for many children, particularly when nology, and legislative policy changes have allowed more than 75%
identified soon after birth or onset. of children with hearing loss to attend public schools main-
The diagnosis and management of pediatric hearing loss have streamed with normal-hearing students.4 The ability of screenings
undergone significant changes in the past 30 years. In 1993, the to detect hearing loss in infancy, the efficacy of hearing aids and coch-
National Institutes of Health recommended newborn hearing lear implants to mitigate consequences of hearing loss, the prolif-
screening within the first 3 months of life.1 The Joint Committee on eration of genetic studies expanding the understanding of genes in-
Infant Hearing, consisting of representatives from many national volved with hearing, and the knowledge about the interaction
organizations dedicated to ensuring early identification, interven- between hearing and cognition have fundamentally altered the un-
tion, and follow-up care of infants and young children with hearing derstanding about children with hearing loss. This review will sum-
loss, published statements in 1994, 2000, 2007, and 2019 to marize what is known about the current diagnosis and manage-
establish guidelines for newborn hearing screening and for early ment of pediatric hearing loss, with a focus on some of the current
hearing detection and intervention programs, benchmarks for controversies in management.
quality, tracking of outcomes, and initial management of infants
with hearing loss.2 Through the Individuals with Disabilities Act
(2004), Part C provides free intervention services from birth to age
Methods
3 years for any child in the United States identified with hearing
loss, and Part B provides educational assistance for children aged 3 PubMed was searched with the Medical Subject Heading term
through 21 years through individualized educational plans and pro- hearing loss with filters for English language, child (birth-18 years),
grams for hearing disability. and humans from 1993 through July 31, 2020. The search was

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 Clinical Review & Education Review Hearing Loss in Children

Box 1. Definitions of Hearing Loss Box 2. Newborn Hearing Screening

Hearing Loss Technology Used to Perform Screening

Any impairment in the ability to hear sounds at thresholds Otoacoustic Emissions (OAEs)
considered normal. For children, a pure tone threshold average of Sounds produced by outer hair cells in the cochlea in response to
more than 15 dB at 500, 1000, 2000, and 4000 Hz is considered acoustic signals in the ear; this noninvasive test has different
outside the normative range, with larger reductions in hearing forms, known as transient evoked and distortion product OAE.
levels classified by severity. Severity of hearing loss is categorized
Auditory Brainstem Response (ABR)
below. Deaf is often used as an alternative to profound hearing
A noninvasive test of the integrity of the auditory pathway from
loss when a person cannot hear typical conversations without
middle ear, to cochlea, to the vestibulocochlear nerve, and to
hearing amplification. Hard of hearing is a general term for anyone
brainstem, where the response is measured; the ABR can be used
who has some hearing loss that ranges from mild to moderate to
as a pass/fail test for screening, or to identify the threshold
moderately severe and who often benefits from hearing aids.
(softest sound) at which sounds are heard.
Severity of Hearing Loss
Automated Auditory Brainstem Response (AABR)
Slight: hearing thresholds 16 to 25 dB
The hearing screening version of ABR for infants in the neonatal
Mild: hearing thresholds 26 to 40 dB intensive care unit (NICU).
Moderate: hearing thresholds 41 to 55 dB
Protocols
Moderately severe: hearing thresholds 56 to 70 dB Normal Newborn Nursery
Severe: hearing thresholds 71 to 90 dB Hearing screening of full-term newborns usually involves the OAE.
Profound: hearing thresholds more than 90 dB If the infant does not pass the screen in one or both ears (termed
as refer for diagnostic testing), then they may undergo a second
Timing of Hearing Loss screening at their primary care provider visit or the birth hospital
Congenital: identified in the neonatal period before the age of 1 month; a second screening refer should then
Delayed-onset: identified after the neonatal period but attributed cause the infant to undergo diagnostic ABR testing before the age
to etiologies present at birth of 3 months.

Acquired: occurs after the neonatal period and is attributed Neonatal Intensive Care Unit
to etiologies not present at birth Hearing screening of infants from the NICU usually involves the
Sensorineural hearing loss: due to injury or defect within the AABR; a refer usually results in a transitory evoked otoacoustic
cochlea, cochlear nerve, or the brainstem pathways to the audi- emission test to rule out auditory neuropathy spectrum disorder
tory cortex and a diagnostic ABR before the age of 3 months (corrected for
gestational prematurity).
Conductive hearing loss: due to injury or defect within the external
or middle ear, including the external auditory canal, tympanic
membrane, middle ear cavity, and ossicles
hearing loss, the prevalence of hearing loss in children by age 18
Mixed hearing loss: combination of sensorineural and conductive
years has been estimated to be as high as 18%.8 Early identifica-
types of hearing loss
tion allows for early interventions with parent-child programs,
with a benchmark of no later than 3 to 6 months of age estab-
supplemented by literature and policy statements that were lished by the Joint Committee on Infant Hearing, including hearing
known to the authors. aids and intensive speech-language therapy, which in turn leads to
better outcomes, including earlier integration into general educa-
tion (ie, mainstream schooling).2,9
In addition to identifying infants with profound bilateral hear-
Epidemiology
ing loss, the newborn hearing screening programs also identify in-
The prevalence of permanent bilateral severe to profound hearing fants with bilateral mild to moderate or unilateral hearing loss. In the
loss in newborns is 1.1 per 1000 newborns and has not changed past, those children would have been identified much later in child-
significantly over time.5 In addition, another 1 to 2 per 1000 new- hood, often when they presented with speech-language or educa-
borns have bilateral mild to moderate hearing loss or unilateral tional delays. The past 20 years are notable for the proliferation of
hearing loss of any degree.6 However, the age at which hearing studies that have investigated the difficulties that children with any
loss is detected has decreased substantially due to successful degree of hearing loss may encounter.
screening programs.2 In a study from the United Kingdom, the
median age of hearing loss identification for screened children
using objective tests of transient-evoked otoacoustic emissions
Consequences of Hearing Loss in Children
and automated auditory brainstem response with bilateral hearing
loss was 10 weeks of age (n = 151), compared with 12 to 20 months Hearing loss is a well-known prominent risk for speech and lan-
with a health visitor distraction test (behavioral observation for guage developmental delay. The provision of hearing aids and
hearing) performed between ages 7 and 8 months in homes or cochlear implants early in life has been demonstrated to help many
community centers (n = 495).7 Because children continue to lose children attain near-normal speech and language trajectories, as
hearing from multiple etiologies as they age, such as temporal measured by growth curves using standardized language
bone fractures, ototoxic exposures, and delayed onset of genetic scores.10-12 The effectiveness of these interventions are influenced

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 Hearing Loss in Children Review Clinical Review & Education

by factors such as maternal educational level, duration of daily posure, and extracorporeal membrane oxygenation.28 Although
hearing aids use, and nonverbal intelligence.10,13 congenital CMV is usually a primary cause of hearing loss, it is com-
Despite the significant improvements in speech and language, mon; thus, other genetic or structural temporal bone etiologies may
children with hearing loss are still at risk of delays in multiple cogni- also be present as an additional etiology.31,32 Congenital infections,
tive functions, such as working memory and executive functions.14-18 including syphilis and rubella can cause hearing loss.33 Rubella, once
These problems have long-term educational and occupational con- the most common viral cause of congenital sensorineural hearing loss,
sequences. In a Danish population study involving young men ap- is now rare due to maternal vaccination. Congenital syphilis, which had
pearing before a draft board, 51% with normal hearing continued edu- decreased for decades and is still very uncommon, is unfortunately
cation beyond age 16 years compared with 42% with mild to moderate on the rise, especially in urban locations and populations (eg, non-
hearing loss, and 34% with more severe hearing loss.19 Similarly, Hispanic Black, Black, and uninsured people), with an incidence of 23.3
a Norwegian cohort study found that people with hearing loss were per 100 000 live births in 2017.34
half as likely to achieve higher education.20
Hearing loss has also been found to affect a child’s quality of life, Delayed-Onset
particularly in the school and social domains, as well as behavior and Delayed-onset hearing loss should be considered if caregivers raise
behavioral disorders.21,22 One systematic review reported unquan- concerns about their child’s hearing, speech, or language delay.
tified but increased associations between hearing loss and internal- Delayed-onset hearing loss may also occur if there were perinatal
izing behaviors, conduct and hyperactivity disorders, and other emo- risk factors such as congenital CMV infection or extracorporeal mem-
tional problems.23 One study found the prevalence of psychiatric brane oxygenation.
disorder in a group of deaf and hearing-impaired children to be as More than 119 genes are associated with sensorineural or mixed
high as 50%.24 In a US public health survey, hearing loss increased hearing loss.35 Of these, syndromes comprise 30% of all genetic
the likelihood of reporting child behavioral diagnoses (55% for hear- causes and are often associated with delayed onset or progressive
ing loss, adjusted odds ratio [OR] for autism, 2.9; 95% CI, 1.8-4.9), hearing loss, including Pendred, Usher, and Alport syndromes
problems with behavior (95% for hearing loss, adjusted OR for at- (Table 1).41 Pendred syndrome, associated with recessive variants
tention-deficit disorder, 3.1; 95% CI, 2.5-3.9), and difficulties with so- in the SLC26A4 gene, is the most common syndromic form of he-
cioemotional domains (90% for hearing loss, adjusted OR, 3.9; 95% reditary sensorineural hearing loss and is associated with thyroid dys-
CI, 3.2-4.7).25 In addition, 17% to 48% of children with unilateral hear- function, goiter, enlarged vestibular aqueduct, and incomplete par-
ing loss and 50% with cochlear implant have impaired vestibular tition type II cochlear anomaly (Mondini). Usher syndrome is also
function, which can further influence their ability to participate in autosomal recessive and has 3 clinical types, associated with at least
normal childhood activities.26 Long-term longitudinal studies have 9 genes that are differentiated by the severity of the hearing loss,
found significant relationships between childhood hearing loss and vestibular dysfunction, and age of onset of vision loss.35 Alport syn-
decreased well-being and self-esteem as well as anxiety and depres- drome is an X-linked (80%) or recessive disorder (depending on the
sion among women.27 gene) resulting in kidney failure, ocular abnormalities (anterior len-
ticonus, retinopathy), and progressive sensorineural hearing loss de-
tected usually in late childhood.42 As a caveat, many of the syn-
dromic hearing loss etiologies may initially present as nonsyndromic
Etiology of Childhood Hearing Loss
hearing loss in infancy or early childhood.
Congenital Several of the nonsyndromic recessive genes are also associ-
The most common causes of permanent congenital sensorineural ated with progressive sensorineural hearing loss, and children may
and mixed hearing loss are congenital cytomegalovirus (CMV; either pass a newborn hearing screening or present with much milder
5%-20%), structural abnormalities of the temporal bones (30%- loss that worsens over time. These include GJB2 (connexin 26),
40%), and genetic causes (50%)28-30 (See Box 1 for definitions). MYO15A, and STRC. Autosomal dominant nonsyndromic progres-
Additionally, many of the anatomical abnormalities are associated sive hearing loss genes include TMC1 and KCNQ4.
with genetic causes, including branchiootorenal syndrome and Delayed onset of hearing loss can also occur after congenital in-
CHARGE syndrome. Branchiootorenal syndrome is associated fections. Historically, prenatal exposure to the TORCHES (toxoplas-
with abnormalities of the second branchial arch derivatives, mosis, other, rubella, CMV, herpes virus, syphilis) organisms were
external ear malformations, hearing loss, and kidney malforma- common causes of congenital hearing loss. However, epidemiol-
tions. CHARGE syndrome consists of coloboma, heart defects, ogy of these organisms has changed, and only congenital CMV is cur-
atresia of choanae, retardation of growth, genital abnormalities, rently a substantial cause of delayed onset loss in many countries.
and ear abnormalities. Ear abnormalities can include malformed The prevalence of congenital CMV infection is 0.4% to 2.3% of all
external, middle, or inner ears. newborns.43 Of infants with confirmed congenital loss, 6% to 7%
The incidence of hearing loss increases with premature birth and have congenital CMV. However, up to 43% of infants with congen-
decreases with increasing gestational age and birth weight (1.2%- ital CVM will initially pass a newborn hearing screening but then pre-
7.5% born at 24-31 weeks and 1.4%-4.8% with birthweight 750- sent with sensorineural hearing loss later in infancy or childhood.43
1500 g) and increasing numbers of comorbidities. Hearing loss oc- More recently congenital Zika infection has been associated with
curs in 1.2% to 7.5% of infants in neonatal intensive care units hearing loss. A 2019 review of 10 articles including 266 infants and
(NICUs).28 NICU-related hearing loss also increases with combina- children from Brazil, Colombia, and the United States, reported a
tions of hyperbilirubinemia, sepsis, neonatal bacterial meningitis, nec- range of hearing loss from 6% to 68% among tested infants.44 More
rotizing enterocolitis, prolonged ventilation, ototoxic medication ex- study is needed to determine the possibility of progression and more

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 Clinical Review & Education Review Hearing Loss in Children

Table 1. Some Common Nonsyndromic and Syndromic Genetic Hearing Loss Genesa
OMIM Associated
locus genes Common findings Additional diagnostic findings
Nonsyndromic hearing loss
DFNB1 220290 GJB2, GJB6 Congenital mild to Usually normal temporal bone
profound autosomal imaging36; rarer dominant forms
recessive nonsyndromic are associated with skin disease;
hearing loss uncommon digenic inheritance
with both GJB2
and GJB6
DFNB16 603720 STRC Bilateral mild to moderate
(CATSPER2) congenital SNHL; deletion
of both STRC and
CATSPER2 is associated
with SNHL and infertility
in males
DFNA8/12 602574 TECTA Often prelingual, often
milder, and mid- or
high-frequency SNHL
DFNB21 602574 TECTA Prelingual severe to
profound SNHL
DFNB3 600316 MYO15A Progressive bilateral
SNHL
Mitochondrial 561000 MT-RNR1; Maternally inherited There are also many
hearing loss 1555G>A (this nonsyndromic hearing mitochondrial syndromes,
is the most loss, or hearing loss that some of which include
common) occurs after brief hearing loss
exposure to
aminoglycosides
Syndromic hearing loss
Pendred 274600 SLC26A4 Euthyroid (often) goiter, Intracochlear partition defect
syndrome, progressive, often type II (Mondini) deformity in
recessive asymmetric, mild to which the cochlea has less than
moderate sensorineural or the normal 2.5 turns and/or
mixed hearing loss enlarged vestibular aqueduct on
CT or MRI3
Usher 276900 MYO7A Electroretinogram or dark
syndrome, Type I: profound hearing adapted thresholds may show
recessive 276904 USH1C signs of RP earlier than routine
loss at birth, vestibular
601067 CDH23 dysfunction starting at ocular examination; there are
birth, vision problems also few variants that result in
602083 PCDH15 either nonsyndromic RP or
early in life
606943 SANS/USH1G nonsyndromic HL

276901 USH2A Type II: moderate to

severe hearing loss at
605472 ADGRV1 birth, vision problems by
611383 WHRN adolescence with
progression, normal
balance
276902 CLRN1 Type III: progressive
hearing loss, later onset
vestibular dysfunction,
and vision loss starting
later in childhood or
adolescence
Alport 301050 COL4A5 Progressive hearing loss, Kidney biopsy may reveal
syndrome, hematuria, ocular glomerulonephritis
x-linked, 203780 COL4A3 abnormalities (anterior
recessive, 104200 COL4A4 lenticonus, retinopathy)
dominant
Jervell and 220400 KCNQ1 Severe to profound Prolongation of QT interval on
Lange-Nielsen bilateral congenital electrocardiogram (ECG)
syndrome, 612347 KCNE1 hearing loss, syncope, Abbreviations: CT, computed
recessive sudden death tomography; DFNB, nonsyndromic
autosomal dominant deafness gene;
Waardenburg 606597 PAX3-WS1/3 HL generally congenital, Dystopia canthorum (WS1),
syndrome, may be unilateral or synophrys, vitiligo, EVA, enlarged vestibular aqueduct;
dominant or 193510 MITF-WS2 bilateral and can be heterochromia iridis, white HL, hearing loss; OMIM, Online
recessive 602229 SNAI2-WS2D associated with structural forelock; upper limb anomalies Mendelian Inheritance in Man
inner ear anomalies, such (WS3), Hirschsprung disease database; MRI, magnetic resonance
608890 SOX10-WS2E as EVA; WS3, and WS4A/B (WS4)
imaging; RP, retinitis pigmentosa;
277580 4C can be autosomal
dominant or recessive SNHL, sensorineural hearing loss.
EDNRB-WS4A
a
Table was constructed based on
613265 EDN3-WS4B
Heredity Hearing Loss,35 DiStefano
Branchiooto- 601653 EYA1 HL is generally Kidney anomalies may be et al,37 Liming et al,38 Shearer
kidney congenital, ear anomalies structural, functional, or both et al,39 Sloan-Heggen et al,40 and
syndrome, 601205 SIX1 may involve external,
dominant middle, and inner ear the Online Mendelian Inheritance
600963 SIX5
database.36

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 Hearing Loss in Children Review Clinical Review & Education

clarity of the actual prevalence of hearing loss. Other congenital in- cal aspects of syndromic genetic causes, or autoimmune etiologies;
fections that may result in later onset sensorineural hearing loss however, yield from this type of testing was low.33 Currently, the rec-
include toxoplasmosis (1 per 10 000 in the United States) and syphi- ommendation is that testing should be based on family and medi-
lis (23.1 per 100 000 live births in 2018).45,46 cal history, patient age, age of onset of hearing loss, whether hear-
ing loss is unilateral vs bilateral and/or progressive, and type of
Acquired hearing loss. Options for tests for evaluation can include computed
Postnatally acquired causes of hearing loss can be attributed to tomography (CT) or magnetic resonance imaging (MRI) of the tem-
trauma, infection, ototoxic medications, or autoimmune disorders. poral bones, to evaluate anatomical causes; genetic testing; oph-
Much of the prevalence of pediatric hearing loss is due to acquired thalmologic evaluation for coexisting abnormalities; and screening
etiologies, but specific contributions to that global prevalence have for congenital CMV in newborn infants. Tests that may yield a treat-
not been well studied or documented. Of the preventable causes able cause of hearing loss (eg, congenital CMV, Lyme disease, auto-
of childhood hearing loss, the World Health Organization attri- immune hearing loss) or diagnoses that would be important to not
butes 31% to infections, 17% to postnatal birth complications, 4% miss (long QT in a child with bilateral profound hearing loss, kidney
to use of ototoxic medications such as aminoglycosides by preg- failure in Alport syndrome) should be carefully considered based on
nant mothers and infants, and 8% to other causes.47 individual patient presentation. Additional factors for imaging may
Trauma can cause conductive, mixed, or sensorineural hearing include CT vs MRI, need for sedation, and risk of exposure to radia-
loss depending on location and type of injury to the temporal bone. tion. Common causes of hearing loss and possible testing options
Conductive hearing loss can result from tympanic membrane per- are identified in Table 2.
foration or ossicular chain injury.48 Temporal bone fractures can dam-
age the cochlea, injure the cochlear nerve, or cause a perilymphatic
fistula, which often result in severe to profound sensorineural hear-
Management Options
ing loss.49 Concussive injuries to the temporal bone without frac-
ture may also result in temporary or permanent sensorineural hear- Hearing Devices for Children With Bilateral Sensorineural
ing loss.50 Trauma to the cochlea can also be in the form of noise Hearing Loss
exposure damaging the outer hair cells resulting in permanent loss. Both physiological and behavioral evidence suggest bilateral input
Infectious causes of sensorineural hearing loss include measles, to the auditory system, as opposed to unilateral input, facilitates bin-
mumps, varicella zoster, Lyme disease, bacterial meningitis, and aural listening skills necessary for developing spoken language skills,
rarely, otitis media. Measles and mumps disease with subsequent effective communication in daily listening and learning environ-
hearing loss is more common in unvaccinated than vaccinated ments, and ultimately for academic success.55,56 For children with
children.51 Lyme disease is an uncommon but potentially treatable bilateral sensorineural hearing loss, maximizing hearing at each ear
cause of hearing loss. Lastly, hearing loss can often result from bac- is best for developing spoken language, ie, 2 ears each fitted with a
terial meningitis and can be progressive, most commonly after Strep- device are better than 1 device in 1 ear. Device options for children
tococcus pneumoniae infections. Close surveillance for hearing loss with bilateral sensorineural hearing loss consists of 2 hearing aids,
is important because labyrinthine ossification can occur and im- 2 cochlear implants, or a cochlear implant at one ear and a hearing
planting a cochlear device must be expedited for the patients that aid at the opposite ear (referred to as bimodal devices). Decisions
meet audiologic criteria for the implant. for recommending these devices are partially guided by audiomet-
Medications known to be ototoxic and can cause permanent ric hearing thresholds. Table 3 illustrates the progression of hear-
hearing loss include aminoglycosides, antineoplastic agents (par- ing threshold levels as they relate to device recommendations. Hear-
ticularly cisplatin), and loop diuretics. Other medications such as sa- ing levels within the normal limits at each ear serve as the optimal
licylates and macrolides, including azithromycin, can cause hearing listening condition. For children with bilateral sensorineural hear-
loss that is generally reversible. Close monitoring of dosages and se- ing loss, bilateral hearing aids are typically recommended for chil-
rum drug levels can lessen the chance of injury to the inner ear. In dren with sufficient amounts of residual hearing. For children with
addition, certain mitochondrial variants can confer increased sus- severe to profound hearing loss, hearing aids may be insufficient for
ceptibility to aminoglycoside ototoxic effects.52 rehabilitating the hearing loss and cochlear implant technology
Autoimmune-related hearing loss can be due to primary auto- should be considered. For children with intermediate levels of re-
immune dysfunction localized to the inner ear or due to systemic au- sidual hearing or different levels of hearing at each ear, bimodal de-
toimmune disorders such as Cogan syndrome (interstitial keratitis, vices may be considered.
progressive hearing loss, and vestibular dysfunction).53 Hearing loss Compared with hearing aids that amplify acoustic informa-
is often rapidly progressive and is sometimes responsive to immu- tion, cochlear implants bypass the normal transduction mecha-
nosuppressants. Autoinflammatory genes, such as the NLRP3 may nisms of the peripheral auditory system and directly stimulate the
also be associated with syndromic and nonsyndromic hearing loss.54 auditory nerve using an electrical signal. Cochlear implants have an
internal component that is surgically placed, consisting of an elec-
trode array that is advanced into the cochlea and a receiver stimu-
lator (Figure and Box 3). The external components of cochlear im-
Evaluation of Hearing Loss Etiology
plants consist of a microphone, a transmitting coil with a magnet,
Before the availability of high-resolution temporal bone imaging, neo- and a processor. As noted in Table 3, device configurations prog-
natal CMV screening, and genetic testing, assessment included labo- ress from delivering an amplified acoustic signal (bilateral hearing
ratory testing for congenital syphilis or rubella, studies for the clini- aids) to an acoustic and electric signal combined (hearing aids and

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 Clinical Review & Education Review Hearing Loss in Children

Table 2. Diagnostic Studies

Diagnostic studies Etiology Advantages Disadvantages

Urine, saliva or blood Congenital CMV Minimally invasive Testing not definitive for
PCR within first 3 wks congenital CMV after 3 wks of
of life; DBS at birth age unless test DBS in
or later conjunction with symptoms or
imaging findings
Viral antibodies, viral Congenital TORCH infections; Minimally invasive False-positives and
DNA serological postnatal infections false-negatives can occur; work
testing closely with ID department to
order and interpret
Genetic testinga Syndromic or nonsyndromic, Minimally invasive Results may be negative or
genetic inconclusive, but HL could still
be genetic because many genes
still are unrecognized or tests
are not available
CT temporal bones Trauma, ear malformations Rapid, often able to be Radiation exposure
completed without
sedation Abbreviations: CMV, cytomegalovirus;
CT, computed tomography;
MRI of temporal Inner ear malformations Painless, no radiation May need sedation
bones, brain associated with hearing loss DAT, dark adapted visual
threshold; DBS, dried blood spot;
Ophthalmologic Concurrent disorders with eye, Minimally invasive Limited examination often based
evaluation vision on age; may need more detailed DFNB, nonsyndromic autosomal
examinations, such as ERG and dominant deafness gene;
DATs if suspect retinal pathology ERG, electroretinogram; HL, hearing
Electrocardiogram Long QT syndrome, generally Minimally invasive May identify cardiac conditions loss; ID, infectious disease;
associated with bilateral severe unrelated to hearing loss MRI, magnetic resonance
to profound SNHL imaging; PCR, polymerase chain
Urinalysis Alport syndrome Minimally invasive Results may be negative early in reaction; SNHL, sensorineural
disease course hearing loss; TORCH, toxoplasma,
Thyroid function Pendred Syndrome Minimally invasive Thyroid function may be truly other (syphilis), rubella,
studies normal (DFNB4) or normal early cytomegalovirus, herpes.
in course of Pendred syndrome a
See Table 1.

13,61-63
9 months. There is evidence that early receipt of a cochlear
Table 3. Hearing Device Configurations
implant is safe and is associated with a greater likelihood of
Hearing level Device configuration improved spoken language and academic outcomes.58,64,65
Normal (all acoustic hearing) None
Postsurgical complications may be minor (such as infections, skin
Mild to moderate loss (acoustic hearing) Bilateral hearing aids flap break down, hematoma) or major (device failures requiring re-
Moderately severe to profound loss Bimodal cochlear implant + vision surgery, facial paralysis, need for explant).66 Overall, there is
(electric + acoustic) hearing aid
a wide range of complication rates reported in the literature, rang-
Severe or profound hearing loss Bilateral cochlear implants
(all electric hearing) ing from 1% to 5% for major complications and 4.5% to 15% for mi-
nor complications.67 A recent study examined outcomes within the
cochlear implant, bimodal) to an electrical signal only (bilateral coch- patients who had a major complication of device failures requiring
lear implants). another implant (rate of 5.9%, n = 578). Even within these pa-
tients, the rate of complications from surgery was relatively low and
Device Candidacy postsurgical audiological performance was good.68
Audiometric guidelines have been developed to determine the
hearing level (unaided pure tone average), at which point children Children With Hearing Aids
with hearing aids should be considered for a cochlear implant in the Two recent studies examining a variety of spoken language out-
United States. 57 Initially, cochlear implants were only recom- comes for children with mild to severe hearing loss using hearing aids
mended for children with profound sensorineural hearing loss who found that, on average, these children scored lower than their typi-
demonstrated no benefit from conventional hearing aids; however, cally hearing peers.11,69 Scores for receptive language, expressive lan-
the guidelines have been expanded to consider cochlear implants guage, speech production, and vocabulary ranged from 0.5 to 2 SDs
for children with less severe loss. Recent studies have shown lower than the normative mean for typically hearing peers. The de-
improved speech perception and language results with cochlear gree to which children fell behind their typically hearing peers was
implants compared with conventional hearing aids for children moderated by degree of residual hearing; those with worse hear-
with less severe loss.58-60 Current US Food and Drug Administra- ing showed greater deficits. Higher maternal educational levels and
tion (FDA) audiometric criteria for placing a cochlear implant in chil- nonverbal intelligence skills coupled with earlier receipt of hearing
dren with bilateral profound sensorineural hearing is from 9 aids, more consistent device use, and greater audibility were asso-
through 24 months and older than 2 years for children with severe ciated with better language outcomes.
to profound sensorineural hearing loss. The documented benefits
of implanting a device early for spoken language skills have Children With Cochlear Implants
supported the decrease in the age at which the FDA has approved Prior to the clinical availability of cochlear implants, children with bi-
the procedure from a minimum of 2 years to a minimum of lateral severe to profound hearing loss using traditional hearing aids

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 Hearing Loss in Children Review Clinical Review & Education

Figure. Cochlear Implant vs Hearing Aid

Cochlear implant
Cochlear implants convert acoustic signals
A cochlear implant consists of 2 main parts
into electrical signals and transmit them
connected via magnets.
directly to the cochlear nerve. Transmission of sound
• Internal device with a receiver and stimulator through the cochlear implant
is surgically implanted behind the ear. 4 to the cochlear nerve
• External device with a speech processor, 3
microphone, and transmitter, is worn Part of mastoid bone is surgically 1 Microphone
around the ear. removed to allow placement
2 Speech processor
Transmitter of electrode into cochlea.
3 Transmitter
2
Mastoid bone
Cochlear nerve 4 Receiver and stimulator
Receiver and
stimulator 5 Electrode array

Microphone 5
1
Speech
processor
Cochlea

Electrode
array

Hearing aid Bone-conduction hearing aid

A conventional hearing aid is worn externally and consists A bone-conduction aid can be surgically implanted or attached
of 6 main parts. to the skin by a headband or adhesive.

Microphone Receiver Headband

Ear hook
Connector
Amplifier
Tubing
Ear mold Processor

The behind-the-ear device is typically used for small children. The nonsurgical headband option is used for small children.

Transmission of sound 1 Transmission of sound

through the hearing aid through the bone-
to the cochlear nerve 3
conduction aid 1 2
4 to the cochlear nerve
1 Microphone
1 Processor
2 Amplifier 2
2 Connector
3 Receiver 5
4 Ear hook
5 Tubing

Hearing aids amplify acoustic signals received through Bone-conduction hearing aids bypass the middle ear and
the microphone so that sound can reach the cochlea. transmit sound through vibrations to the cochlear nerve.

acquired spoken language skills at approximately half the rate have been associated with higher levels of nonverbal intelligence and
of similarly aged children with normal hearing.70 The advent of maternal education, greater levels of preimplant residual hearing, ear-
cochlear implants has made it possible for many children with bilat- lier receipt of cochlear implant and early intervention services, a fo-
eral severe to profound hearing loss to attain age-appropriate speech cus on auditory and oral instruction, and use of updated cochlear im-
perception, speech production, and expressive and receptive lan- plant processor technology.71 Improved academic attainment as well
guage skills by the time they enter elementary school, although as a higher rated quality of life have been documented for children who
a substantial proportion of children (30%-50%) fail to achieve age- received cochlear implants.72,73 However, among those who re-
appropriate spoken language skills even in the presence of factors that ceived cochlear implants, long-term educational, vocational, and oc-
supportsuccessfullanguagedevelopment.13,61,71 Age-appropriatespo- cupational levels achieved have continued to be significantly worse
ken language outcomes for pediatric recipients of cochlear implants than the referenced population average.74

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 Clinical Review & Education Review Hearing Loss in Children

bilateral stimulation prior to the second surgery (through a lack of

Box 3. Common Types of Hearing Devices Used by Children bimodal device use).
More recently, for speech perception and ultimately language
Frequency Modulated (FM) System development, the benefits of bimodal use prior to a second coch-
Frequency modulated systems are often used in classroom lear implant were found to vary with unaided threshold hearing lev-
settings, where the acoustic signal from a teacher wearing
els. For those with thresholds in the severely impaired range (pure
a microphone is transmitted via an FM signal to a portable speaker
that sits in front of the student, allowing that student to hear the
tone averages of ≈ 73 dB hearing level), a period of bimodal use of
teacher better. Other terms for this technology include hearing 3 to 4 years was found to be advantageous for receptive vocabu-
assistive technology (HAT) or remote microphone HAT. lary and language. For those with profound hearing loss ( ≈ 92 dB
hearing level) the benefits were less apparent, and for those with
Hearing Aid
A personal device that is worn by the child to allow amplification of
the most profound hearing losses ( ≈ 111 dB hearing level) early
a sound; conventional hearing aids are fitted to the child’s ear and bilateral cochlear implant was considered to be the best for speech
use the external auditory canal to transmit the amplified sound to perception and language development outcomes.80 Recommen-
the cochlea; a bone-conduction hearing aid is often worn with dations for early bilateral cochlear implant and bimodal use should
a headband and uses vibrotactile stimulation to transmit the be made in the context that these benefits may vary depending
amplified sound to the cochlea.
on the hearing level. Moreover, benefits may vary across differ-
Implantable Devices ent outcome measures; early receipt of a second cochlear implant
Bone-Anchored Implants may be advantageous for certain binaural processing skills such
Surgically placed abutments are inserted through the skin or under as sound localization and listening in spatially separated noise
the skin to allow transmission of sounds through bone conduction while continued bimodal use may be advantageous for spoken lan-
to the cochlea.
guage skills.55,80
Cochlear Implants
Cochlear implants bypass the normal transduction mechanisms of Hearing Rehabilitation for Children
the peripheral auditory system and directly stimulate the auditory
With Unilateral Hearing Loss
nerve using an electrical signal through a surgically placed
There is increasing evidence that unilateral hearing loss leads to harm-
electrode directly in the cochlea.
ful effects on speech and language development, educational dif-
ficulties including failing a grade, and behavioral issues.81-84 This has
For children with the most profound levels of hearing loss, cli- led to increased efforts for auditory rehabilitation for children with
nicians generally agree that bilateral cochlear implants are the most unilateral hearing loss with options including conventional hearing
viable option for spoken language development.75 For some chil- aids, frequency modulating systems, contralateral routing of signal
dren, both cochlear implants are surgically implanted simultane- aids, bone-conduction hearing aids, and cochlear implant. Fre-
ously while for others, the 2 are received sequentially with varying quency modulating systems transmit sounds from the person wear-
durations (eg, few months to several years). Because some studies ing the transmitter to the hearing aids being worn; this reduces dis-
suggest better binaural processing skills when the interval be- tracting background, listening fatigue, and the distance between the
tween the first and second implant is minimal, the effect of timing speaker and listener. Contralateral routing of signal hearing aids col-
for placement of the second must be discussed when the option is lect sound from the ear with the hearing loss and routes it to the bet-
being considered.76,77 Notably, as cochlear implant candidacy guide- ter hearing ear. Bone-conduction hearing aids bypass the middle ear,
lines in the United States expand to include children with greater lev- directly stimulating the auditory nerve on the same side if it is func-
els of residual hearing in at least 1 ear, many children may present tional or the opposite ear with normal hearing. Cochlear implants
with bimodal device configurations (cochlear implant combined with directly stimulate the auditory nerve through an electrode placed
a hearing aids at the nonimplanted ear). For these children, clini- into the cochlea (Figure).
cians must determine whether to recommend continued bimodal For children with severe to profound unilateral sensorineural
use or progression to bilateral implants.75-77 hearing loss, bone-conduction hearing devices have consistently
shown improved hearing thresholds, speech recognition threshold
Effects of Bimodal Devices and Bilateral Cochlear Implants (lowest level at which a person can identify spoken words), and hear-
on Spoken Language Skills ing in noisy environments.85-88 Recently, the cochlear implant pro-
Studies comparing the benefits of bimodal devices and bilateral coch- cedure has been approved for profound unilateral hearing loss (also
lear implants on spoken language skills (receptive and expressive vo- known as single-sided deafness) for children older than 5 years. Stud-
cabulary or receptive or expressive language) have been mixed. One ies have shown improvement with speech outcome measures in both
study found no significant group differences after accounting for vari- quiet and noisy environments; bimodal speech reception thresh-
ous demographic variables.78 Advantages for earlier receipt of a sec- olds in noise; and sound localization.89,90 Contralateral routing of
ond cochlear implant have been found by some, yet others have signal hearing aids have had mixed outcomes reported in the
noted that a period of bimodal use before the second implant was literature.88 Even when unilateral loss is less severe, frequency modu-
advantageous.56,79 In many of these research studies, pediatric popu- lating systems and conventional hearing aids are often beneficial.
lations are described solely by their currently used hearing devices With conventional hearing aids, children have been shown to expe-
yet their device use prior to the first and second cochlear implant is rience subjective improvement at home and school, as measured
unknown.56 Moreover, in some cases it is unclear whether the ben- with the Children’s Home Inventory for Listening Difficulties (CHILD)
efits of early bilateral implants are exaggerated by deprivation of scores, in speech recognition in noise, word recognition scores in

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 Hearing Loss in Children Review Clinical Review & Education

noise and quiet, and sound localization.91-94 Frequency modulat- Limitations

ing systems similarly have shown improvement in objective mea- This review has several limitations. First, because of the breadth of the
sures of hearing such as the Bamford-Kowal-Bench sentence list and topic, only a few of the frontiers of pediatric hearing loss were cov-
word recognition scores in noisy and quiet environments.93,95 Al- ered. Among the aspects of hearing loss that this article did not ad-
though the evidence is consistent in showing improvement in au- dress are (1) controversies about whether to use antiviral agents to
diological measures, studies are needed to evaluate if auditory re- treat infants with congenital CMV; (2) when cochlear implant ought
habilitation can thwart the overall harmful effects of unilateral hearing to be considered for unilateral hearing loss; (3) the diagnosis and man-
loss on speech and language, communication, educational perfor- agement of auditory neuropathy spectrum disorder; (4) the costs of
mance, and social functioning. rehabilitation, such as devices, surgical placement of the devices, pro-
fessionalfees,andtherapy;(5)experimentaltherapiesforgenetichear-
Costs Associated With Hearing Devices ing loss; and (6) issues of policy, such as whether hearing aids should
Hearing devices used in hearing rehabilitation can be associated with be covered by insurance. Second, the continued need for early de-
significant cost. A pair of hearing aids can cost approximately $6000 tection of hearing loss throughout childhood to identify delayed-
and the cochlear implant device itself can cost nearly $20 000 or onset, progressive, or acquired hearing loss was not reviewed.
more. These costs do not include ongoing costs such as speech-
language therapy, programming of the cochlear implant, and pro-
fessional and surgical fees. On the contrary, costs of untreated hear-
Conclusions
ing loss are substantial; one study reported more than $1 million in
lifetime cost due to special education and reduced work productiv- Hearing loss in children is common, and there has been substantial
ity among children with prelingual severe to profound hearing loss. progress in diagnosis and management of these patients. Early iden-
One cost-utility analysis showed that benefits in quality-adjusted life- tification of hearing loss and understanding its etiology can assist
years (QALYs) varied by the child’s age at the time they received the with prognosis and counseling of families. In addition, awareness of
implant: children younger than 18 months gained 10.7 QALYs on av- treatment strategies including the many hearing aids options, coch-
erage over a lifetime vs 9.0 for those aged 18 through 36 months lear implant, and assistive devices can help direct management of
and 8.4 for those older than 36 months.96 the patient to optimize outcomes.

ARTICLE INFORMATION Davidson all reported being coeditors of Pediatric data/documents/2017-HSFS_Type-and-Severity-

Accepted for Publication: August 27, 2020. Sensorineural Hearing Loss: Clinical Diagnosis and Table.pdf
Management. No other disclosures were reported. 7. Bamford J, Uus K, Davis A. Screening for hearing
Author Affiliations: Department of
Otolaryngology-Head and Neck Surgery, Submissions: We encourage authors to submit loss in childhood: issues, evidence and current
Washington University in St Louis, St Louis, papers for consideration as a Review. Please approaches in the UK. J Med Screen. 2005;12(3):
Missouri (Lieu, Davidson); Department of contact Edward Livingston, MD, at Edward. 119-124. doi:10.1258/0969141054855256
Otolaryngology and Communication Enhancement, livingston@jamanetwork.org or Mary McGrae 8. Wang J, Sung V, Carew P, et al. Prevalence of
Boston Children’s Hospital, Boston, Massachusetts McDermott, MD, at mdm608@northwestern.edu. childhood hearing loss and secular trends:
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