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Hearing loss in children: Screening and evaluation

Hearing loss in children: Screening and evaluation
Literature review current through: Jan 2024.
This topic last updated: Sep 12, 2023.

INTRODUCTION — Childhood hearing loss that is undetected and untreated can result in speech, language, and cognitive delays. Early identification and effective treatment of hearing loss is associated with improved language and communication skills.

Hearing screening beyond the newborn period and the evaluation of hearing loss in children are reviewed here. Causes of hearing loss in infants and children are summarized in the table and discussed in detail separately (table 1). (See "Hearing loss in children: Etiology".)

Newborn hearing screening and management of hearing loss in children are also discussed in detail separately. (See "Screening the newborn for hearing loss" and "Hearing loss in children: Treatment".)

DEFINITIONS — The following terms are used throughout this topic:

Conductive hearing loss – Conductive hearing loss is caused by a problem of the outer or middle ear that interferes with conduction of sound to the inner ear. It can occur at any location from the outer ear (pinna, external auditory canal) to the stapes footplate and oval window. In children, conductive hearing loss is most often transient (eg, otitis media with effusion), but it can be permanent (eg, aural atresia or chronic adhesive otitis media). (See "Hearing loss in children: Etiology", section on 'Conductive hearing loss'.)

Sensorineural hearing loss (SNHL) – SNHL is hearing loss resulting from damage, disease, or other disorders affecting the inner ear (eg, the cochlea) and/or the auditory nerve (cranial nerve VIII).

SNHL due to disorders of the inner ear – Numerous conditions (acquired or congenital) are associated with SNHL. The causes are discussed separately. (See "Hearing loss in children: Etiology", section on 'Sensorineural hearing loss'.)

Auditory neuropathy spectrum disorder (ANSD) – In ANSD (also called auditory neuropathy, neural synchrony disorder, neural dyssynchrony, and paradoxical hearing loss), the cochlea can detect sound, but signals are not transmitted properly from the inner ear to the brain. ANSD is characterized by normal otoacoustic emissions, which indicate normal outer hair cell function. However, auditory brainstem responses are abnormal, implying an abnormality of the inner hair cells of the cochlea or the cochlear branch of cranial nerve VIII.

"Retrocochlear" hearing loss – "Retrocochlear" refers to lesions proximal to the cochlea. We define "retrocochlear" hearing loss as hearing loss that results from abnormalities of the eighth cranial nerve. The most common retrocochlear lesion is a vestibular schwannoma (also called acoustic neuroma). However, there is overlap between retrocochlear hearing loss and ANSD and the terms are used somewhat inconsistently in the medical literature. Some resources use the term "retrocochlear" more broadly to describe other abnormalities of the auditory central nervous system.

Mixed hearing loss – Mixed hearing loss refers to a combination of conductive and SNHL.

The causes and relative frequencies of different types of hearing loss in children are discussed in a separate topic review. (See "Hearing loss in children: Etiology".)

SCREENING FOR HEARING LOSS IN CHILDREN

Rationale — Hearing screening beyond the newborn period is necessary to identify children with hearing loss that is acquired (eg, from meningitis, trauma, noise exposure), progressive (eg, certain genetic causes), has delayed onset (eg, congenital cytomegalovirus infection), or was not detected on newborn hearing screen [1-3]. (See "Hearing loss in children: Etiology".)

Hearing loss that is undetected and untreated can result in speech, language, and cognitive delays. Early identification and effective treatment of hearing loss is associated with improved language and communication skills [4-7].

Although most children with hearing loss are identified before they begin school [8], some cases are missed [9]. Hearing loss of at least 16 decibels (dB) in either the low- or high-frequency range occurs in approximately 15 percent of children aged 6 to 19 years [10,11].

Additional details about the rationale and evidence supporting newborn hearing screening are provided separately. (See "Screening the newborn for hearing loss", section on 'Rationale for screening'.)

Screening schedule — We agree with the guidelines from the American Academy of Pediatrics (AAP), which suggest a hearing risk assessment at all health maintenance visits and periodic hearing screening throughout childhood and adolescence [3,12].

Children with risk factors – Children with identified risk factors for early hearing loss (table 2) should have at least one formal audiologic assessment in infancy or early childhood even if they passed the newborn hearing screen. For infants with congenital or perinatal risk factors, formal audiology generally should be performed by age 9 months [3]. For those with acquired risk factors, formal audiology should be performed no later than three months after the event or concern was identified [3]. (See 'Risk assessment' below and 'Formal audiology' below.)

Patients with abnormal formal audiology results should be referred to otolaryngology, speech-language pathology, and early intervention (see 'Referrals' below). Those with normal audiology results should have ongoing periodic developmentally appropriate hearing assessments thereafter.

Children without identified risk factors – For children without identified risk factors, office- or school-based hearing screening is suggested at ages 4, 5, 6, 8, and 10 years [12,13]. For children and adolescents >10 years old, the AAP guidelines suggest audiometry screening that includes 6000 and 8000 hertz (Hz) frequencies once between 11 and 14 years, once between 15 and 17 years, and once between 18 and 21 years [13]. This guidance places a high value on the potential for improved outcome with early detection and intervention (compared with the cost, inconvenience, and lost time necessary for follow-up of a potentially false positive result). Abnormal screening results should be followed up with formal audiology. (See 'Formal audiology' below.)

Abnormal results should be explained to the parents/caregivers and there should be a system in place to ensure appropriate follow-up.

Risk assessment — Risk assessment for hearing loss in childhood includes monitoring language milestones (table 3), performing routine developmental surveillance, identifying risk factors for hearing loss (table 2), and eliciting any parental concerns about hearing [3,13].

Risk factors – Risk factors for early childhood hearing loss include (table 2) [3]:

Parent/caregiver concerns regarding hearing, speech, language, or developmental delay – Normal language and communication milestones are summarized in the table (table 3) and discussed in detail separately. (See "Expressive language delay ("late talking") in young children", section on 'Primary care evaluation'.)

Family history of permanent childhood hearing loss – However, many children with genetic hearing loss do not have affected relatives because genetic hearing loss often has an autosomal recessive inheritance pattern. (See "Hearing loss in children: Etiology", section on 'Genetic'.)

Neonatal intensive care unit (NICU) graduates – Important risk factors for hearing loss among NICU graduates include (table 4) (see "Screening the newborn for hearing loss", section on 'Infants with risk factors for hearing loss'):

-NICU stay ≥5 days

-Very low birth weight (<1500 g)

-Perinatal asphyxia and neonatal encephalopathy (see "Clinical features, diagnosis, and treatment of neonatal encephalopathy")

-Requiring mechanical ventilation or extracorporeal membrane oxygenation (ECMO)

-Requiring exchange transfusion for hyperbilirubinemia (see "Unconjugated hyperbilirubinemia in neonates: Risk factors, clinical manifestations, and neurologic complications", section on 'Consequences of severe hyperbilirubinemia')

-Exposure to ototoxic drugs (eg, loop diuretics, aminoglycosides) (See "Hearing loss in children: Etiology", section on 'Ototoxic drugs'.)

Infections – This includes:

-Congenital infections (eg, cytomegalovirus, Zika virus) (see "Overview of TORCH infections" and "Congenital cytomegalovirus infection: Clinical features and diagnosis", section on 'Isolated hearing loss')

-Central nervous system infections (eg, meningitis, encephalitis) (see "Bacterial meningitis in children: Neurologic complications", section on 'Hearing loss' and "Acute viral encephalitis in children: Treatment and prevention", section on 'Neurologic sequelae')

-Chronic or recurrent ear infections (see "Otitis media with effusion (serous otitis media) in children: Clinical features and diagnosis", section on 'Hearing loss' and "Acute otitis media in children: Epidemiology, microbiology, and complications", section on 'Hearing loss')

Ototoxic drug exposure, including chemotherapy. (See "Hearing loss in children: Etiology", section on 'Ototoxic drugs'.)

Head trauma (particularly basal skull and temporal bone fractures).

Syndromes associated with hearing loss (table 5). (See "Hearing loss in children: Etiology", section on 'Genetic'.)

Craniofacial or ear malformations (eg, cleft lip and/or palate, anomalies of temporal bone, congenital microcephaly, microtia, ear dysplasia, white forelock, microphthalmia). (See "Syndromes with craniofacial abnormalities" and "Congenital anomalies of the ear".)

Chronic exposure to loud noises, particularly if the exposure occurs over extended periods of time and at high volume [14] (see "Hearing loss in children: Etiology", section on 'Noise exposure')

Screening questions – In older children and adolescents, the following questions can help identify hearing problems [15]:

Do you have a problem hearing over the telephone?

Do you have trouble following the conversation when two or more people are talking at the same time?

Do others complain that you turn the television volume too high?

Do you have to strain to understand conversation?

Do you have trouble hearing in a noisy background?

Do you find yourself asking other people to repeat themselves?

Do many people you talk to seem to mumble (or not speak clearly)?

Do you misunderstand what others are saying and respond inappropriately?

Do you have trouble understanding the speech of women and children?

Do people get annoyed because you misunderstand what they say?

Screening methods — Screening can be performed in the office or school setting with pure tone audiometry or otoacoustic emission (OAE) [3,16]. Tympanometry is an additional method to screen for conductive hearing loss associated with chronic otitis media with effusion (OME). (See "Otitis media with effusion (serous otitis media) in children: Clinical features and diagnosis", section on 'Diagnosis'.)

Pure tone audiometry and OAE should be performed in a quiet room. While a dedicated soundproof environment (like that used for formal audiology) improves the sensitivity of testing, this is generally not necessary for office-based hearing screening. Clinicians and patients should be aware that screening may fail to detect mild hearing loss (<30 dB).

Pure tone audiometry – Pure tone audiometry involves determining the threshold at which the child can hear a sound 50 percent of the time at each frequency. The accuracy of this screening method depends upon the child’s ability to cooperate; it is generally not feasible in children younger than three or four years of age. Pure tone audiometry generates an audiogram, which is a graph showing hearing thresholds (in dB) plotted according to frequency (in Hz). Pure tone audiometry in the office setting typically involves testing only air thresholds (whereas formal audiology tests both air and bone conduction). The normal hearing threshold is between 0 to 20 dB. A threshold >20 dB suggests hearing loss. Examples of audiograms are shown in the figures, depicting conductive hearing loss (figure 1) and sensorineural hearing loss (figure 2). (See 'Pure tone audiometry' below.)

Otoacoustic emission – OAE testing measures the presence and strength of low-intensity sound produced by the cochlea in response to an acoustic stimulus. One advantage of this screening tool is that testing does not require a behavioral response and thus can be performed in infants and young children. However, a quiet setting is still needed. An abnormal OAE may be due to cochlear dysfunction or conductive hearing loss. (See 'Otoacoustic emissions' below.)

OAE is one of the two methods used for newborn screening, as discussed separately. (See "Screening the newborn for hearing loss", section on 'Otoacoustic emissions'.)

Tympanometry – Tympanometry is most valuable in conjunction with pneumatic otoscopy in patients with chronic or recurrent ear infections. (See 'Tympanometry' below.)

Flat tympanograms (type B tympanograms) (figure 3) are usually due to middle ear effusion (acute otitis media [AOM] or OME). A hearing evaluation and referral to an otolaryngologist may be warranted if the abnormalities fail to resolve with appropriate therapy (for AOM) or during the expected time frame (for OME). These issues are discussed in greater detail separately. (See "Otitis media with effusion (serous otitis media) in children: Management", section on 'Approach to management' and "Acute otitis media in children: Treatment", section on 'Response to antibiotics or observation'.)

EVALUATION OF THE CHILD WITH HEARING LOSS

History — The history may be helpful in distinguishing between acquired versus inherited causes of hearing loss. Important elements of the history include the following [17]:

Prenatal history, including maternal infections (eg, cytomegalovirus, rubella, syphilis) or drug exposures (eg, thalidomide, retinoic acid)

Neonatal history, including premature birth, low birth weight, birth hypoxia, hyperbilirubinemia, sepsis, and exposure to ototoxic medications

Developmental history focusing on motor milestones, including age at sitting and age at walking

Postnatal medical history, including viral illnesses, recurrent otitis media, bacterial meningitis, head trauma, and exposure to ototoxic medications

Age of onset and progression of hearing loss

Associated symptoms (eg, pain or drainage from the ear suggests an acute infectious process; tinnitus, vertigo, or disequilibrium suggest an inner ear process; poor balance or late walking suggest of a vestibular aspect to ear disease)

Associated medical conditions, including:

Kidney disease, which may suggest Alport or branchio-oto-renal syndrome (see "Clinical manifestations, diagnosis, and treatment of Alport syndrome (hereditary nephritis)" and "Renal hypodysplasia", section on 'Genetic disorders')

Vision problems such as night blindness, poor visual acuity, or visual field defect, which may suggest syndromic retinitis pigmentosa (Usher syndrome) (see "Retinitis pigmentosa: Clinical presentation and diagnosis")

Cardiac arrhythmia, which may suggest Jervell and Lange-Nielsen syndrome (see "Congenital long QT syndrome: Epidemiology and clinical manifestations")

Cutaneous or pigmentary abnormalities, which may suggest Waardenburg syndrome or neurofibromatosis (see "The genodermatoses: An overview", section on 'Waardenburg syndrome' and "NF2-related schwannomatosis (formerly neurofibromatosis type 2)")

Family history – The family history focuses on identifying first- and second-degree relatives with hearing loss, known genetic syndromes associated with hearing loss (table 5), or features commonly associated with syndromic hearing loss (eg, pigmentary abnormalities; vision problems; kidney disease; branchial abnormalities such as ear pits, cervical fistulas, or cysts; or arrhythmia or sudden cardiac death). A history of consanguinity suggests an inherited etiology. If the family history is positive, a full three-generation pedigree should be obtained to establish the pattern of inheritance.

Physical examination

General examination — Physical examination should include measurement of growth parameters and assessment of general appearance, while looking for the clinical features of various syndromes (table 5) [18].

Examination of the head and neck is especially important. In a retrospective analysis of 114 children with hearing loss referred to a tertiary care center, 43 percent had head and neck abnormalities that helped to establish the etiology of hearing loss [19]. As examples, a white forelock and heterochromia of the iris are seen in Waardenburg syndrome (picture 1), micrognathia in Apert syndrome and Pierre Robin sequence, and enlarged thyroid in Pendred syndrome. (See "The genodermatoses: An overview", section on 'Waardenburg syndrome' and "Craniosynostosis syndromes", section on 'Apert syndrome' and "Syndromes with craniofacial abnormalities", section on 'Pierre Robin sequence'.)

Ear examination — The physical examination of the outer ear includes inspection for preauricular pits (picture 2) or sinuses, pinna size and shape, and patency of the external auditory canal. The external auditory canal should be cleared of cerumen for more accurate hearing testing. The tympanic membrane should be viewed to ensure that no middle ear abnormality (eg, middle ear fluid, tympanic membrane perforation, tympanic membrane scarring, cholesteatoma or other middle ear mass) exists. (See "Congenital anomalies of the ear" and "The pediatric physical examination: HEENT", section on 'Ears'.)

Pneumatic otoscopy is performed with positive and negative pressure to evaluate mobility of the tympanic membrane. Diminished or absent tympanic membrane mobility can be caused by fluid, mass in the middle ear cavity, perforation, pressure equalization tube, or sclerosis of the tympanic membrane. Increased mobility of the tympanic membrane can indicate ossicular chain disruption.

Simple hearing tests — In older children and adolescents, hearing status can be assessed in the office using 256 and 512 hertz (Hz) tuning forks. Hearing loss at 256 Hz is equivalent to approximately 10 to 15 decibels (dB) and hearing loss at 512 Hz is equivalent to 20 to 30 dB. An ear with normal hearing has air conduction (sound waves traveling to the tympanic membrane and converted into sound in the inner ear) that is louder than bone conduction (sound transmitted via the vibration of the skull into the cochlea). The Weber and Rinne tests examine the relative adequacy of air and bone conduction of sound (figure 4 and table 6).

The Weber test is performed by placing the tuning fork on the bridge of the forehead, nose, or teeth and asking the child if the sound is louder in one ear or the other. The sound is heard equally in both ears in children with normal hearing. Sensorineural hearing loss (SNHL) is suspected if the vibratory sound is louder on the "good" side. Conductive hearing loss is suspected if the vibratory sound is louder on the "bad" side.

The Rinne test compares bone conduction (when the tuning fork is placed on the mastoid bone) with air conduction (when the tuning fork is held near the ear). An abnormal result occurs when sound is at least equally loud or louder when the fork is placed on bone as compared with when it is held next to the ear (bone>air conduction). The Rinne test is considered normal when the vibrating fork placed near the ear is louder than when placed on the mastoid bone (air>bone conduction). An abnormal test is consistent with conductive hearing loss, especially if the Weber test also lateralizes to that side.

As examples, a conductive hearing loss on the right side would demonstrate a Weber test that lateralizes to the right (ipsilateral side), an abnormal Rinne test (bone conduction>air conduction) on the right, and a normal Rinne test on the left. An SNHL on the right side would demonstrate a Weber test that lateralizes to the left (contralateral side) and normal Rinne tests bilaterally. Testing results may vary for mixed hearing loss, severe SNHL (Rinne test on the bad ear will appear to be abnormal because bone conduction crosses over and is detected by the good ear), or conductive hearing loss below the thresholds that are detectable by the tuning forks used.

FORMAL AUDIOLOGY — Formal audiologic assessment is performed by an audiologist in a soundproof environment and provides detailed information about hearing ability. The audiologic assessment typically consists of numerous different studies. It may include pure tone audiometry, speech audiometry, behavioral audiometry, visual reinforcement audiometry, play audiometry, impedance audiometry, tympanometry, and/or electrophysiologic tests (including auditory brainstem response [ABR] and otoacoustic emissions [OAE]). The choice of which tests are performed is based on the child's developmental age, the reason for the referral, the test environment, the equipment available, and the skill of the test administrator. No child is too young to have their hearing evaluated.

Testing in cooperative children

Pure tone audiometry — Audiology measures the ability to hear pure tones of various frequencies as a function of intensity measured in decibels (dB). The pure tone frequencies evaluated in a complete audiogram are 250, 500, 1000, 2000, 3000, 4000, and 8000 hertz (Hz). The threshold for each tone is determined by finding the intensity level at which the child can detect the tone 50 percent of the time.

Hearing is tested with both air and bone conduction. Air conduction, performed with earphones, tests the ability to hear when sound waves travel their normal route through the external auditory canal to the tympanic membrane and the middle ear system. Bone conduction, performed with an oscillator placed on the mastoid bone, tests the ability to hear when the middle ear is bypassed and the inner ear fluid and cochlea are stimulated directly with bone vibration.

Hearing loss is defined by the pure tone threshold: normal hearing has a threshold of 0 to 20 dB, mild hearing loss from 20 to 40 dB, moderate hearing loss from 40 to 60 dB, severe hearing loss from 60 to 80 dB, and profound hearing loss greater than 80 dB. Characteristic patterns that vary with the type of hearing loss are seen:

The audiogram of a child with a conductive hearing loss shows normal bone-conducted pure tone thresholds and abnormal air-conducted pure tone thresholds (figure 1). The difference in the air- and bone-conducted thresholds is termed the air/bone gap.

The audiogram of a child with sensorineural hearing loss (SNHL) shows both air- and bone-conducted pure tone thresholds that are outside of the normal range (figure 2). Different patterns may suggest different etiologies:

Unilateral SNHL usually suggests an inner ear disorder.

Audiograms of children with nonsyndromic genetic hearing loss are typically bilaterally symmetric, although the shape may be highly variable (eg, flat, down-sloping, up-sloping, U-shaped) (figure 5). Audiograms in children with syndromic hearing loss may demonstrate symmetric or asymmetric losses.

Noise-induced SNHL presents initially as high-frequency hearing loss (figure 6).

Speech audiometry — Speech audiometry consists of two parts: the speech threshold (also called the speech reception threshold) and the word discrimination score. The speech threshold is the softest level at which a child can correctly repeat 50 percent of presented "spondee" words. Spondee words are two-syllable words such as airplane, armchair, or pancake. The speech threshold is recorded in decibels and serves as a cross-check for the pure tone air-conduction thresholds. The speech threshold is typically equal to the pure tone air-conduction average, ±10 dB. The pure tone average is the average decibel score at 500, 1000, and 2000 Hz.

The word discrimination score is the percentage of phonetically balanced words that a child can correctly repeat at a given sensation level. Testing is typically performed at 40 dB above the child's speech threshold. This discrimination score serves two purposes: it can establish the prognosis for the use of a hearing aid, and it helps determine the site of the lesion. A poor discrimination score usually indicates significant neural degeneration. These individuals may not be good candidates for hearing aids because the aid will amplify sound but may not permit the child to understand what is being said.

Tests for young and/or uncooperative children — Young children and some children with developmental disabilities may not be able to cooperate with pure tone or speech audiometry testing as described above. Their hearing is assessed using behavioral methods in conjunction with other tests, such as acoustic impedance testing, OAEs, or ABRs, as discussed below. (See 'Electrophysiology' below.)

Behavioral observation audiometry – Behavioral observation audiometry is used to examine auditory function in infants younger than six to eight months, children with multiple handicaps, or adults who are not able to cooperate for other types of testing. Live voice, warbled tones, or narrow-band noises are presented in a sound field environment to elicit reflexive and orienting responses to auditory stimuli. The responses can include head or limb reflex, whole-body startle, sucking, eye blinking, raising of the eyebrows, or cessation of certain behaviors, such as movement or sucking [20,21]. Responses to stimuli are not reinforced.

Behavioral observation is a subjective measure of hearing ability and does not provide ear-specific or frequency-specific information. It is best used in conjunction with objective methods. (See 'Impedance testing' below and 'Electrophysiology' below.)

Visual reinforcement audiometry – Visual reinforcement audiometry is used to evaluate the hearing of infants and young children from approximately six months of age through the second year. Sound stimuli (live voice and tones) are presented in the sound field and via insert earphones. The child is visually rewarded with lighted and animated toys for turning his or her head toward the sound source. The child is conditioned to perform this task repeatedly. An experienced audiologist will use several lighted and animated toys and an intermittent reinforcement schedule to maintain the child's attention. Under ideal circumstances, complete ear-specific information for speech stimuli and interactive frequencies from 250 through 8000 Hz can be obtained.

Play audiometry – Play audiometry is used to evaluate the hearing of children between the ages of 30 months and 5 years. Pure tones and speech information are presented via insert earphones. The child is taught to perform a simple task, such as placing a block in a bucket or a peg on a pegboard each time a sound is heard. They are expected to perform the task over and over again when they hear the sound. The detection of speech sounds is determined by the child's ability to point to simple pictures when instructed by the audiologist.

Speech understanding or speech discrimination scores are obtained in children who are able to perform the tests. Such tests use picture-pointing tasks for younger children and require reading skills for older children. Speech-understanding testing is used to evaluate the child's ability to hear and understand speech in quiet and noisy listening environments.

Impedance testing — Impedance audiometry (also known as admittance audiometry) evaluates the integrity and function of the middle ear system, including middle ear pressure, tympanic membrane mobility, Eustachian tube function, continuity and mobility of the ossicles, and acoustic reflex thresholds. It is a quick, noninvasive test that requires little cooperation from the child.

Full impedance audiometry encompasses tympanometry and stapedial reflex testing. However, stapedial reflex testing is seldom performed in children.

Tympanometry — Tympanometry measures the changes in the acoustic impedance of the middle ear system in response to changes in air pressure. As the pressure increases, the tympanic membrane is pushed medially; as negative pressure is placed, the tympanic membrane protrudes laterally. The point of maximum compliance of the middle ear is identified, indicating the status of air pressure in the middle ear.

Five types of tympanograms can be seen (figure 3) [22]:

Type A – Normal middle ear pressure

Type B – Little or no mobility, suggestive of fluid behind the tympanic membrane or perforation

Type C – Negative pressure in the middle ear, suggestive of a retracted tympanic membrane

Type AS – A very stiff middle ear system that can be caused by myringosclerosis or otosclerosis

Type AD – The highly compliant tympanic membrane seen in ossicular chain discontinuity

Stapedial reflex — The acoustic stapedius reflex is the sound-evoked contraction of the stapedius muscle [23]. It is mediated by a neural network with afferent input from the auditory nerve and efferent output to the facial nerve. The central portion of the reflex pathway is comprised of several centers in the brain. The acoustic stapedius reflex test assesses the presence or absence of ipsilateral and contralateral stapedial contraction with sound stimulation to each ear. Normally, sound stimulation on one side produces a stapedial contraction both on the ipsilateral and contralateral side.

Acoustic stapedius reflex testing can be used to help differentiate different types of hearing loss, particularly retrocochlear hearing loss (ie, due to abnormalities of the eight cranial nerve such as a vestibular schwannoma [also called acoustic neuroma]).

Electrophysiology — Electrophysiologic tests of hearing do not require behavioral responses. They include ABRs, OAEs, and stapedial reflex testing.

Brainstem response — The ABR test (also referred to as auditory brainstem evoked response [ABER], brainstem evoked response audiometry [BERA], or brainstem auditory evoked response [BAER]) uses click stimuli and tone burst stimuli from loud (80 or 90 dB) to soft (0 to 20 dB) to evoke responses from the auditory pathway [24]. These responses are recorded using electrodes placed on the head and ears of the child. A computer averages the responses, and the waveforms generated are compared with normative data. Delayed or absent waves suggest cochlear or neurologic deficits. Normal hearing or conductive hearing loss patterns can also be recognized [18]. Children with auditory neuropathy have absent or severely distorted ABRs with preserved OAEs [25].

Diagnostic ABR testing is distinct from automated ABR (AABR), which is commonly performed to screen newborns. AABR is a screening tool with an automated pass/fail response, whereas diagnostic ABR testing provides quantitative data (eg, waveforms) that are interpreted by trained audiologists. (See "Screening the newborn for hearing loss", section on 'Automated auditory brainstem response'.)

ABR testing estimates auditory sensitivity, and it provides useful clinical information about the integrity of the eighth nerve pathway. However, it is not a true test of hearing and should not be used alone or as a substitute for behavioral hearing testing.

ABR testing can be used to confirm behavioral hearing testing in infants and preverbal children (ie, younger than 30 months), children with developmental delay or intellectual disability, and those who are otherwise difficult to test. ABR testing should generally be performed in infants who fail the newborn hearing screen or if the parents or health care provider are concerned about hearing. ABR testing may not be required if both behavioral audiometry and OAE testing are normal. Sedation may be necessary for ABR testing since excessive movement can disrupt the results.

Otoacoustic emissions — OAEs are faint sounds produced by the normal motion of the outer hair cells of the cochlea in a healthy ear [24,26]. These sounds are transmitted in a retrograde fashion through the middle ear and the tympanic membrane, where they can be measured by a microphone sealed in the external auditory canal. The presence of OAE suggests normal cochlear function. This test is often used as a screening test since it is quick, noninvasive, and does not require sedation. OAE tests are commonly used in newborn hearing screening programs, which are discussed separately. (See "Screening the newborn for hearing loss", section on 'Otoacoustic emissions'.)

Beyond the newborn period, OAE testing can be used in combination with behavioral audiometry to evaluate of infants and preverbal children (ie, younger than 30 months). Normal OAE in conjunction with normal behavioral audiometry in children ages 6 to 30 months old can eliminate the need for ABR, which often requires sedation. In addition, OAE testing can be used to monitor aminoglycoside-induced ototoxicity, to differentiate central from peripheral auditory dysfunction, and to confirm hearing acuity when malingering is suspected by audiometry [25,27].

REFERRALS

For all children with abnormal audiology results – Children who have abnormal formal audiology results should be referred to otolaryngology, speech-language pathology, and early intervention [3].

Additional evaluation for infants and children with SNHL

Genetic evaluation – If there are syndromic features or if the child has bilateral sensorineural hearing loss (SNHL), referral to a clinical geneticist is warranted. (See 'Genetic testing' below.)

Ophthalmologic evaluation – Infants and children with SNHL should have at least one formal evaluation by an ophthalmologist since the prevalence of vision problems is higher among children with SNHL compared with the general pediatric population [28]. In a series of 226 children with SNHL, the prevalence of ophthalmologic abnormalities was 22 percent [29]. This is considerably higher than the prevalence of vision problems in the general pediatric population, which is approximately 5 to 10 percent. Refractive errors (myopia, hyperopia, astigmatism) were more frequent than nonrefractive conditions (eg, strabismus, amblyopia, nystagmus, optic atrophy, retinitis pigmentosa). (See "Vision screening and assessment in infants and children".)

Assessment of vestibular function – The clinician should inquire about vestibular symptoms (eg, whether the child is clumsy or has difficulty walking or balancing) since vestibular abnormalities are common in children with SNHL. Though rarely performed in infants and young children, formal testing of vestibular function can be considered if concerns arise from the history. Formal evaluation of vestibular function can be carried out by measuring cervical vestibular evoked myogenic potentials (cVEMP). In one report of 254 infants with SNHL who underwent vestibular screening with cVEMP, 14 percent had abnormal results [30]. Patients at greatest risk included infants with severe-to-profound hearing loss and those with certain underlying etiologies (eg, meningitis, syndromic SNHL, congenital cytomegalovirus infection, cochleovestibular anomalies). (See "Evaluation of the patient with vertigo", section on 'Diagnostic tests'.)

IDENTIFYING THE ETIOLOGY

Diagnostic approach — Infants and children with hearing loss detected by screening should undergo formal audiologic and electrophysiologic testing, which helps to guide the subsequent diagnostic evaluation. (See 'Formal audiology' above and 'Electrophysiology' above.)

The diagnostic evaluation begins with a history and physical examination. (See 'History' above and 'Physical examination' above.)

In some cases, the etiology is readily apparent from this initial evaluation (eg, a child with unilateral conductive hearing loss who has a history of recurrent otitis media and has a perforated tympanic membrane in the affected ear on otoscopic examination). In other cases, the etiology may be uncertain based on the history and physical examination, and additional evaluation is warranted. Depending on the clinical context, this may include genetic evaluation, testing for congenital infections (eg, cytomegalovirus [CMV]), and/or temporal bone imaging.

The following sections outline our approach to the diagnostic evaluation based on the type of hearing loss (sensorineural versus conductive; unilateral versus bilateral) and age of the patient (algorithm 1). Our approach is generally consistent with recommendations of the International Pediatric Otolaryngology Group and the American College of Medical Genetics and Genomics [17,31]. Whenever possible, the evaluation should be carried out by a multidisciplinary team including otolaryngologists, audiologists, clinical geneticists, and genetic counselors.

Sensorineural hearing loss — Important causes of sensorineural hearing loss (SNHL) in infants and children include genetic causes, which may be syndromic (table 5) or nonsyndromic, and infections, particularly congenital CMV infection. (See "Hearing loss in children: Etiology", section on 'Genetic' and "Hearing loss in children: Etiology", section on 'Congenital'.)

Initial evaluation for infants and children with SNHL includes the following [17]:

Genetic evaluation – The approach to genetic testing depends upon whether the hearing loss is bilateral or unilateral and whether syndromic features are present:

Bilateral SNHL – Comprehensive genetic testing using next-generation sequencing (NGS) has become the preferred method for establishing a genetic diagnosis of hereditary SNHL, and we offer this testing to all children with bilateral SNHL. (See 'Genetic testing' below.)

Unilateral SNHL – For children with unilateral SNHL, we suggest not routinely performing comprehensive genetic testing with NGS to evaluate for nonsyndromic hearing loss. The yield of NGS in this setting is low. However, it is important to consider syndromic forms of hearing loss as syndromes are often variable in their phenotypic presentation. Common syndromic forms of hearing loss include Waardenburg, Usher, Pendred, Alport, hemifacial microsomia, and CHARGE syndromes, among others (table 5). These can often be identified through a detailed history (including three-generation pedigree) and physical examination. Based on the findings of the history and examination, additional testing may be warranted to evaluate for particular clinical concerns (eg, kidney function tests if there is concern for Alport or branchio-oto-renal syndrome; thyroid function tests if there is concern for Pendred syndrome; ophthalmologic examination if there is concern for Usher syndrome; electrocardiogram if there is concern for Jervell and Lange-Nielsen syndrome). If the findings of the evaluation suggest a syndromic cause of SNHL, genetic testing is generally warranted. Most of the comprehensive genetic testing panels designed for nonsyndromic hearing loss now also include the common syndromic forms of hearing loss. (See 'Genetic testing' below.)

Testing for congenital CMV infection – We suggest testing for CMV in all infants <12 months of age with either bilateral or unilateral SNHL. The hearing loss associated with congenital CMV infection may be unilateral or bilateral and may be present at birth or have delayed onset. Thus, newborn hearing screening does not identify all affected infants.

Infants <12 months old – Congenital CMV infection can only be definitively diagnosed if testing is performed within 21 days after birth. Beyond 21 days, the likelihood that a positive test is due to postnatal exposure increases with increasing age. Detection of CMV between 3 weeks to 12 months after birth in conjunction with a finding of SNHL is suggestive of congenital infection, though it is not definitive. Testing for congenital CMV infection at this age is discussed in greater detail separately. (See "Congenital cytomegalovirus infection: Clinical features and diagnosis", section on 'Three weeks to one year'.)

Children ≥12 months old – Congenital CMV infection remains an important consideration in children presenting with delayed onset SNHL after the age of 12 months; however, establishing a diagnosis of congenital CMV infection at this age is generally not feasible. This is because detection of CMV in children ≥12 months is far more likely to represent postnatal infection than congenital infection. (See "Congenital cytomegalovirus infection: Clinical features and diagnosis", section on 'Older than one year'.)

Temporal bone imaging – Temporal bone imaging can be useful in the evaluation of children with unilateral SNHL or if there is evidence of auditory neuropathy (characterized on audiologic testing by the presence of otoacoustic emissions in the setting of absent or abnormal auditory brainstem response). The diagnostic yield of temporal bone imaging in children with bilateral SNHL without auditory neuropathy is low and we do not routinely obtain imaging in these children except for in the setting of preoperative evaluation for children undergoing cochlear implant. (See 'Temporal bone imaging' below.)

Review newborn screening panel – In addition to the above testing, results of the full newborn screening panel should be reviewed as some causes of hearing loss may be identified through newborn screening (eg, congenital hypothyroidism, certain inborn errors of metabolism). (See "Overview of newborn screening" and "Clinical features and detection of congenital hypothyroidism", section on 'Newborn screening'.)

Vision assessment – It is important that children with SNHL have their vision assessed, both to rule out retinitis pigmentosa (Usher syndrome) and to make sure the child does not have deficits in more than one of the major senses. The approach to vision assessment in infants and children is discussed in detail separately. (See "Vision screening and assessment in infants and children".)

Other testing is based on specific clinical concerns. Examples include:

Testing for other congenital infections such as Zika virus, rubella, toxoplasmosis, or syphilis may be warranted depending on maternal exposure history and other clinical findings in the infant (table 7). (See "Overview of TORCH infections".)

In children with bilateral severe to profound hearing loss, family history of sudden death, or personal history of syncope or cardiac arrhythmia, an electrocardiogram should be obtained to assess the QT interval. A prolonged QT interval in a child with SNHL suggests Jervell and Lange-Nielsen syndrome, the autosomal recessive form of congenital long QT syndrome. Early diagnosis is important since patients with Jervell and Lange-Nielsen syndrome are at risk for sudden cardiac death. Definitive diagnosis can be made by comprehensive genetic testing. The approach to diagnosis and other aspects of congenital long QT syndrome are discussed in detail separately. (See "Congenital long QT syndrome: Epidemiology and clinical manifestations".)

If there is a family history of kidney disease (which may suggest Alport syndrome), urinalysis should be performed to assess for microscopic hematuria. Definitive diagnosis can be made by comprehensive genetic testing. (See "Clinical manifestations, diagnosis, and treatment of Alport syndrome (hereditary nephritis)".)

Conductive hearing loss — Common causes of conductive hearing loss in infants and children include middle ear fluid (eg, acute otitis media and otitis media with effusion [OME]), tympanic membrane perforation, obstruction of the external auditory canal (eg, cerumen), and trauma. In most cases, the etiology is readily apparent based on the history and physical examination findings and additional evaluation is not necessary. (See "Hearing loss in children: Etiology", section on 'Conductive hearing loss'.)

If a diagnosis of OME is made and the effusion and hearing loss persist, tympanostomy tube insertion may be warranted. This is discussed separately. (See "Otitis media with effusion (serous otitis media) in children: Management", section on 'Tympanostomy tubes'.)

If the cause of conductive hearing loss is not readily apparent from the history and examination, further evaluation with temporal bone imaging and/or surgical exploration may be warranted. Causes may include external canal atresia, neoplasms, myringosclerosis, or cholesteatoma. (See 'Temporal bone imaging' below.)

Genetic testing — We refer all pediatric patients with SNHL to an otolaryngologist with expertise in genetic hearing loss and/or a clinical geneticist for further evaluation and counselling unless the medical and birth history are highly suggestive of an environmental cause of SNHL. For example, an infant with a history of symptomatic congenital CMV infection generally does not require an evaluation for genetic causes of SNHL. However, in a child with asymptomatic CMV and hearing loss (positive CMV testing in the absence of other clinical signs of congenital CMV infection), genetic testing is still warranted, especially if antiviral therapy is going to be offered to treat the presumed CMV-related hearing loss [32]. Similarly, other risk factors for hearing loss (eg, prematurity, aminoglycoside exposure) do not obviate the need for genetic evaluation unless they can clearly be established as the cause of SNHL.

For infants and children with bilateral SNHL, the evaluation typically includes comprehensive genetic testing of the patient and possibly other family members. By contrast, the yield of comprehensive genetic testing is low in children with unilateral SNHL in the absence of syndromic findings.

If genetic testing reveals a pathologic variant in a hearing loss-related gene, specific genetic counseling should be provided, followed by appropriate medical evaluations and referrals [17].

Genetic testing should be directed by an otolaryngologist with expertise in genetic hearing loss, a genetic counselor, or a clinical geneticist based upon the three-generation pedigree and specific clinical findings in the patient. A general approach is as follows [31]:

Syndromic findings – For patients with clinical findings that suggest a specific genetic syndrome, targeted genetic testing is performed to evaluate for the specific syndrome (table 5). To facilitate testing, comprehensive genetic testing panels for deafness now include testing for the genetic variants associated with syndromic causes of hearing loss in addition to nonsyndromic causes. Some examples include:

SNHL in the setting of a family or personal history of kidney disease, which suggests Alport or branchio-oto-renal syndrome (see "Clinical manifestations, diagnosis, and treatment of Alport syndrome (hereditary nephritis)", section on 'Molecular genetic testing')

SNHL associated with pigmentary abnormalities, which suggests Waardenburg syndrome (see "The genodermatoses: An overview", section on 'Waardenburg syndrome')

SNHL accompanied by progressive vision loss, which suggests Usher syndrome, a form of retinitis pigmentosa (see "Retinitis pigmentosa: Clinical presentation and diagnosis")

SNHL accompanied by thyroid disease or goiter, which suggests Pendred syndrome

Findings of choanal atresia, colobomas, heart defect, intellectual disability, genital hypoplasia, and ear anomalies, which suggest CHARGE syndrome (see "Congenital anomalies of the ear", section on 'Ear anomalies in CHARGE and DiGeorge syndromes')

Findings of mandibular hypoplasia, orbital distortion, and ear anomalies, which suggest hemifacial microsomia (picture 3) (see "Syndromes with craniofacial abnormalities", section on 'Craniofacial microsomia')

No syndromic findings – For patients with bilateral SNHL without syndromic findings, comprehensive genetic testing using NGS should be performed as the initial test [33].

NGS tests can be designed as disease-specific or can use whole-exome sequencing (WES) [17]. As a general rule, a disease-targeted exon-capture approach that restricts sequencing to genes known to be associated with hearing loss is preferred over WES because targeted testing is less costly and faster and the bioinformatic analysis is simpler. Several NGS tests are clinically available and can be found by querying the Genetic Testing Registry website [34].

The overall diagnostic yield of NGS testing for hereditary hearing loss is approximately 40 percent but increases to approximately 60 percent if there is a family history of hearing loss [33,35]. A genetic basis for hearing loss can be identified in up to one-half of patients with bilateral auditory neuropathy [36]. The available NGS panels for hereditary hearing loss differ somewhat with regard to the number and type of genes included and whether copy number variations are examined. Performance characteristics and analytic sensitivity also may vary.

Negative test results do not exclude a genetic etiology of hearing loss. Patients with negative test results should have periodic follow-up with a clinical geneticist to allow for subsequent testing as medical knowledge advances and new genetic tests become available.

NGS testing may identify variants of uncertain clinical significance. When performing NGS with WES, there is also a possibility of incidental findings unrelated to the child's hearing loss that may be medically actionable (eg, BRCA1 or BRCA2 gene mutation). Thus, if WES is being considered, pretest genetic counseling must be provided to all families and should include discussion of these issues.

The principles of NGS technology and issues related to incidental findings are discussed in detail separately. (See "Next-generation DNA sequencing (NGS): Principles and clinical applications" and "Secondary findings from genetic testing".)

Temporal bone imaging — Temporal bone imaging is not routinely necessary in the evaluation of children with hearing loss. However, imaging may be helpful in select circumstances as a complement to other diagnostic testing and to guide therapy [31,37].

We obtain temporal bone imaging in the following circumstances, particularly if hearing loss is progressive:

Fluctuating hearing loss associated with vestibular problems (eg, poor balance, late walking), which may indicate dilated vestibular aqueduct syndrome.

Unilateral or asymmetric SNHL.

Auditory neuropathy.

In addition, temporal bone imaging may be useful in the following settings [37-44]:

Evaluation of trauma to the temporal bone.

If the appearance of the tympanic membrane or middle ear space is abnormal on otoscopy, temporal bone imaging can help evaluate for congenital anomalies of, trauma to, or tumors of the middle ear and ossicular chain.

Evaluation of persistent or progressive conductive hearing loss, when the cause is not readily apparent from the history and examination.

Evaluation of recurrent meningitis.

Preoperative evaluation prior to cochlear implantation.

Imaging can be performed using magnetic resonance imaging (MRI) or computed tomography (CT). The advantaged of MRI is that it avoids radiation exposure, it is more sensitive for most peripheral auditory abnormalities, and it allows for simultaneous evaluation of the brain. The main disadvantage of MRI is that it generally requires sedation in young children. Contrast-enhanced MRI is preferred if there is suspicion of an inflammatory or neoplastic disorder [40]. The anticipated clinical utility of imaging studies should be balanced against the risks associated with radiation exposure (for CT) and sedation (for MRI). The decision of whether to obtain temporal bone imaging is generally made by the otolaryngologist rather than the primary care provider.

In a systematic review of 50 observational studies, the reported diagnostic yield of CT imaging in children with hearing loss ranged from 7 to 74 percent, with a pooled estimate of approximately 30 percent [45]. The most commonly identified findings were enlarged vestibular aqueduct and cochlear anomalies. In one series of 97 children with nonsyndromic SNHL, CT or MRI abnormalities were detected in 39 percent [39]. The most common findings were enlarged vestibular aqueduct, lateral semicircular canal dysplasia, and cochlear dysplasia. In another retrospective report of 116 children with bilateral SNHL, abnormalities on CT imaging were noted in 28 percent [41]. Abnormalities were most common in children with progressive hearing loss, profound hearing loss, and associated craniofacial abnormalities. Among children without any of these characteristics, CT abnormalities were uncommon (7 percent).

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Hearing impairment in infants and children".)

SUMMARY AND RECOMMENDATIONS

Routine screening – Risk assessment for hearing loss in childhood includes monitoring language milestones (table 3), routine developmental surveillance, identifying risk factors (table 2), and eliciting any parental concerns. This assessment should be included at all pediatric health maintenance visits. In addition, we suggest periodic hearing screening for all children starting at age 4 years and continuing through adolescence (Grade 2C). (See 'Risk assessment' above and 'Screening schedule' above.)

An abnormal hearing screen should be followed up with formal audiology. In addition, children with risk factors for early hearing loss (table 2) should have at least one formal audiologic assessment in infancy or early childhood. The rationale for routine screening is to identify children with hearing loss at an early age since earlier treatment of hearing loss is associated with improved language and communication skills. (See 'Formal audiology' above and 'Rationale' above.)

History and physical examination – Children with suspected or confirmed hearing loss should have a complete history and physical examination.

Important aspects of the history include (see 'History' above):

Characterizing the onset and progression of hearing loss

Characterizing any associated symptoms (eg, pain, drainage from the ear, tinnitus, vertigo, disequilibrium)

Identifying underlying medical conditions, particularly those that are associated with hearing loss (eg, congenital infection; prematurity; hyperbilirubinemia; meningitis; recurrent otitis media; exposure to ototoxic drugs, noise, or barotrauma; kidney disease; cardiac dysrhythmia; or decreased visual acuity)

Reviewing the family history

The physical examination should include (see 'Physical examination' above):

Measurement of growth parameters (see "Measurement of growth in children")

Assessment for syndromic features (table 5)

Ear examination (see 'Ear examination' above)

Simple hearing tests (in cooperative children) (see 'Simple hearing tests' above)

Formal audiology – Formal audiologic assessment is performed by an audiologist in a soundproof environment and provides detailed information about hearing ability. The audiologic assessment typically consists of numerous different studies. The choice of which tests are performed is based on the age of the child, reason for the referral, test environment, available equipment, and skill of the test administrator. No child is too young to have their hearing evaluated. Testing may include any of the following (see 'Formal audiology' above):

Pure tone audiometry (see 'Pure tone audiometry' above)

Speech audiometry (see 'Speech audiometry' above)

Behavioral audiometry, visual reinforcement audiometry, or play audiometry (see 'Tests for young and/or uncooperative children' above)

Tympanometry (see 'Tympanometry' above)

Brainstem response (see 'Brainstem response' above)

Otoacoustic emissions (see 'Otoacoustic emissions' above)

Referrals – Children who have abnormal formal audiology results should be referred to otolaryngology, speech-language pathology, and early intervention. Referral to a clinical geneticist may be warranted if there are syndromic features or if the child has bilateral sensorineural hearing loss (SNHL). In addition, infants and children with SNHL should have at least one formal evaluation by an ophthalmologist. (See 'Referrals' above.)

Identifying the cause – Causes of hearing loss in infancy and childhood are diverse (table 1). If the etiology is uncertain based on the history and physical examination alone, additional evaluation is warranted. Depending on the clinical context, this may include genetic evaluation, testing for congenital infections (eg, cytomegalovirus), and/or temporal bone imaging. The approach to the diagnostic evaluation is based on the type of hearing loss (sensorineural versus conductive, unilateral versus bilateral) and the age of the patient (algorithm 1). For infants and children with bilateral SNHL, the evaluation typically includes comprehensive genetic testing, which is directed by an otolaryngologist with expertise in genetic hearing loss or clinical geneticist with appropriate pre- and post-test counseling. (See 'Diagnostic approach' above and 'Genetic testing' above.)

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References

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