Hearing Loss and Deafness

Overview

In this module, we will refer to children with deafness or hearing loss as children who are deaf or hard of hearing, abbreviated D/HH.

Hearing loss, or impairment, results from the interruption of sound transmission, which is a complex process involving the external, middle, and inner ear, as well as the vestibulocochlear nerve, brainstem, and cerebral cortex.
Diagram of the Ear
Diagram of a hearing apparatus
Sound waves are captured by the outer ear and directed through the external auditory canal resulting in vibration of the tympanic membrane. Tympanic membrane vibrations are transferred through the ossicular chain (malleus, incus, and stapes) in the middle ear to the oval window of the cochlea, where they enter the inner ear. Equal pressures on both sides of the tympanic membrane are required for effective conduction of sound vibrations; the eustachian tube ventilates the middle ear to provide that pressure equalization. Ossicular vibrations enter the organ of Corti as fluid pressure waves causing movement of the hair cells along the basilar membrane.

This motion is converted to neural impulses at the auditory nerve which are then transmitted through the brainstem to the auditory cortex for processing. Sound intensity is determined by a complex interaction of inner and outer hair cells. Sound frequency is determined by the interplay between the dynamic properties of the basilar membrane and that of the surrounding fluid. [Moller: 2012] Dysfunction in any component of this system can result in hearing loss.

Hearing impairments may be syndromic (associated with other genetic, medical, or anatomic problems) or non-syndromic (lacking such associations). The distinction between syndromic and non-syndromic may change with the age of the child. For example, children with Usher syndrome may initially be thought to have non-syndromic hearing loss but, as the associated retinitis pigmentosa becomes apparent with age, the syndromic diagnosis becomes apparent. The onset of hearing loss may be congenital (present at birth), prelingual (before the development of speech), or of later onset. Hearing loss at any age may be due to a variety of factors including genetics, infection, trauma, etc. Hearing impairments may also be grouped into types, related to the cause or mechanism of impairment, and ranges of severity, described by the decibels below which the child cannot hear or discriminate sounds. These groupings are detailed below under Clinical Classification.

As hearing loss severity increases, more speech sounds fall below the level of detection causing greater difficulty in communication. Quiet conversation, which averages 30 dB, can be difficult to understand for individuals with even mild hearing loss. Normal conversation, which averages 50 dB, is below the hearing level of some individuals with moderate hearing loss, and even loud conversation, which averages 70 dB, is below the hearing level of individuals with severe to profound loss.

Deafness is often defined as any degree of hearing loss that sufficiently reduces the intelligibility of speech or interferes with learning. However, many professionals reserve the term ‘deafness’ to describe a severe to profound hearing loss.
"In the years after our son's diagnosis, I have been surprised at the number of parents with non-hearing and hearing children who say that they used to test their child's hearing by banging pots and pans together to see if they could hear it. I heard a mom at the pool say that once her son heard [the loud banging noises], she didn't worry about his hearing anymore, even though he was severely delayed in his speech." From a mother of a boy with a late diagnosis of hearing impairment.

Other Names & Coding

Deafness
Hard of hearing
Hearing Impairment
ICD-10 coding

H90.0, Conductive hearing loss, bilateral

H90.1x, Conductive hearing loss, unilateral with unrestricted hearing on the contralateral side

H90.2, Conductive hearing loss, unspecified

H90.3, Sensorineural hearing loss, bilateral

H90.4x, Sensorineural hearing loss, unilateral with unrestricted hearing on the contralateral side

H90.5, Unspecified sensorineural hearing loss

H90.6, Mixed conductive and sensorineural hearing loss, bilateral

H90.7x, Mixed conductive and sensorineural hearing loss, unilateral with unrestricted hearing on the contralateral side

H90.8, Mixed conductive and sensorineural hearing loss, unspecified

H91.0x, Ototoxic hearing loss

H91.3, Deaf nonspeaking, not elsewhere classified

H91.8xx, Other specified hearing loss

H91.9x, Unspecified hearing loss

The symbols x represent additional digits, indicating laterality, that are required for billing. See ICD10Data.com for more information and additional codes.

Prevalence

Prevalence of hearing loss varies according to the type and degree of hearing impairment, as well as the age group being studied. Studies of universal newborn hearing screening programs have reported 1-3/1000 infants are found to have permanent hearing impairment, with a national average of 1.1/1000 infants. [Centers: 2010] [Mehra: 2009] [Barsky-Firkser: 1997] [Finitzo: 1998] [Mehl: 1998] [Johnson: 1997]

Some children will acquire permanent hearing impairment during the first few years of life; hearing loss of mild or greater severity is reported in approximately 3.1% of children and adolescents nationwide. [Mehra: 2009]

For more detail, click Prevalence of Permanent Congenital Hearing Loss (NCHAM) (PDF Document 36 KB).

Genetics

More than half of all childhood hearing loss is genetic in origin and this proportion is growing because of better control of non-genetic causes like Rubella and meningitis. Over 400 genes have been implicated in hereditary hearing loss, each of which is responsible for only a small percentage of cases. Genetic screening has become cost effective and should be considered the standard of care for children with congenital sensorineural hearing loss because a genetic diagnosis may help direct further workup and therapy. [Linden: 2013] For example, when the cause of a patient’s hearing loss is found to be mutations in the gene encoding the gap junction protein connexin 26 (Cx26, now called gap junction beta 2, or GJB2) the child's clinician can be reasonably assured that there will be no co-morbid symptoms, like retinitis pigmentosa or renal abnormality. Connexin 26 patients are also known to do well with cochlear implantation.

Pathogenic variations in connexin 26 are responsible for approximately 10 to 20% of all childhood genetic hearing loss and cause about 50% of cases with more than one affected child amongst offspring with the same parents. Tests for the common 35delG mutation (single nucleotide deletion at site 35) and complete Cx26 gene sequencing are available clinically and should be offered to families affected by recessive non-syndromic hearing loss of unknown cause. The American College of Medical Genetics and Genomics also recommended gene panel tests, or next-generation sequencing testing for children with sensorineural hearing loss and findings on history and physical exam which suggest a possible syndromic etiology for hearing loss. [Alford: 2014] [Alford: 2014]

Other common recessive types of hearing loss are Usher syndrome (9 genes, 8 to 15%), Pendred syndrome (5%), Jervall-Lange-Neilsen (1%), and Otoferlin Hearing Loss (2%). Common dominant forms are Waardenburg syndrome, branchio-oto-renal (BOR) syndrome, Alport syndrome, and Wolfram syndrome.

Prognosis

The expected functional impact of childhood hearing loss is variable and depends on the type of hearing loss, age of the child at time detection and intervention, and the presence of risk factors, such as developmental delays, syndromic hearing loss, and other sensory impairment (e.g., blindness). Language development and educational performance are not, however, predicted by degree of hearing impairment. [Davis: 1986] It is well-documented that earlier intervention (before 6 months of age), with amplification and language/communication therapy, yields better outcomes in receptive and expressive language and processing abilities. [Moeller: 2000] [Yoshinaga-Itano: 1998] Recent data indicate that greater family participation and a language-rich home environment also support better ultimate language ability, particularly for those children diagnosed at a later age and those with more severe hearing impairment. [Watkin: 2007]

Practice Guidelines

Joint Committee on Infant Hearing.
Year 2007 position statement: Principles and guidelines for early hearing detection and intervention programs.
Pediatrics. 2007;120(4):898-921. PubMed abstract / Full Text

US Preventive Services Task Force.
Universal screening for hearing loss in newborns: US Preventive Services Task Force recommendation statement.
Pediatrics. 2008;122(1):143-8. PubMed abstract

Harlor AD Jr, Bower C.
Hearing assessment in infants and children: recommendations beyond neonatal screening.
Pediatrics. 2009;124(4):1252-63. PubMed abstract / Full Text

Roles of the Medical Home

In addition to monitoring the general health and development of the child who is deaf or hard of hearing (D/HH), the Medical Home clinician plays a central role in facilitating coordinated care among the other subspecialists involved, including the otolaryngologist, geneticist, ophthalmologist, and audiologist. The primary care clinician must also closely monitor the hearing-impaired child for other delays, since 30-40% of children with hearing loss will have developmental delays or other disabilities. For children with isolated hearing loss, particularly of genetic cause, awareness and respect of the family's perspectives and preferences is important. Some families and organizations feel that deafness is not a medical problem, object to medical intervention, prefer sign language over amplification, and celebrate the deaf culture. (See Hearing Culture vs. Deaf Culture and Understanding Deaf Culture (Mass.gov))

Clinical Assessment

Overview

The US Preventive Services Task Force and Joint Committee on Infant Hearing (JCIH 2007) recommends universal screening for hearing loss in newborns prior to hospital discharge or before 1 month of age. [Joint: 2007] Programs that test newborns for hearing loss based on risk factors alone (prolonged NICU admission, genetic syndromes, craniofacial abnormalities, family history of hereditary hearing loss, TORCH infections) identify only 40-50% of infants with permanent congenital hearing loss. [Kennedy: 2005]Early identification and intervention (amplification, surgical intervention, language assessment and therapy) are known to improve ultimate receptive and expressive language, as well as cognitive skills, academic skills, and social-emotional functioning. [Kennedy: 2006] [Moeller: 2000] Screening is usually conducted prior to discharge from the newborn nursery using otoacoustic emissions testing (OAE), automated auditory brainstem response test (AABR), or a combination of the two. See the Sensory Testing section below, under for more detail. A two-step screening process in which an AABR is used to confirm abnormal OAE results yields the fewest false-positive results. Any infant who fails initial hearing screening should be referred for a full audiologic evaluation by an audiologist with pediatric/neonatal expertise. All infants with problems significant enough to warrant a stay in a Newborn Intensive Care Unit (NICU) should have an AABR. See Hearing Loss & Deafness.

The JCIH 2007 Position Statement recommends screening all newborns for hearing loss by 1 month of age, diagnostic audiologic evaluation by 3 months of age for those who fail screening, and early intervention with complete medical evaluation by 6 months of age for those with hearing loss. [Joint: 2007]

Many cases of permanent sensorineural hearing loss are detected with universal hearing screening in the newborn nursery. However, hearing loss may develop after the perinatal period (late-onset) or be progressive in nature. Regardless of a normal newborn screening result, any child with suspected hearing loss and/or language or other developmental delay should be assessed for hearing impairment since early intervention is critical to optimal language and cognitive development.

Pearls & Alerts for Assessment

Repeat hearing screen for readmitted infants

For infants readmitted to the hospital during the first month of life for conditions known to be associated with hearing loss (hyperbilirubinemia requiring exchange transfusion, culture positive sepsis, bacterial meningitis), repeat hearing screening is recommended prior to discharge.

When a newborn screen is unavailable

Newborn hearing screening may not be completed with home births or births at centers not offering screening, and occasionally infants will be discharged to home prior to its completion. In these cases, referral should be made for outpatient hearing screening as soon as possible and no later than 1 month of age.

Infants with prolonged NICU stays (>5 days) are at increased risk for auditory neuropathy

This type of hearing loss may not be evident on otoacoustic emission testing. These infants should have an automated ABR as part of their initial hearing screen, with formal audiologic assessment for those who do not pass.

Screening

Though most cases of permanent sensorineural hearing loss are detected through universal hearing screening in the newborn nursery, hearing loss which begins later in life or is progressive may not be identified with newborn hearing screening.

For the Condition

The American Academy of Pediatrics (AAP) recommends subjective assessment of hearing (and assessment of risk for hearing loss) at all well-child examinations and objective hearing screening at school entry. See the 2017 Bright Futures Periodicity Schedule (AAP) (PDF Document 119 KB). Routine audiometric screening may not be reliable in children with developmental abnormalities, poor cognitive function, or behavioral problems (e.g., autism/developmental delay); referral to an otolaryngologist and a pediatric audiologist who have the equipment and expertise to test infants and young children should be considered. Risk factors that may warrant screening beyond that routinely recommended include:
  • Caregiver concern regarding hearing, speech, language, or developmental delay
  • Family history of permanent childhood hearing loss
  • Neonatal intensive care of more than 5 days or, regardless of length of stay, history of: extracorporeal membrane oxygenation (ECMO), assisted ventilation, exposure to ototoxic antibiotics (gentamicin and tobramycin) or loop diuretics (furosemide), or hyperbilirubinemia requiring exchange transfusion
  • Intrauterine infections such as cytomegalovirus (CMV), herpes, rubella, syphilis, and toxoplasmosis. CMV-related hearing loss is often progressive and may develop after the neonatal period. Fluctuating hearing loss is also common. Children with asymptomatic congenital CMV infection should have hearing screens at birth and then periodically until school age.
  • Conditions associated with hearing loss, such as neurofibromatosis type 2, osteopetrosis, and syndromes such as Usher, Waardenburg, CHARGE, Alport, Pendred, and Jervell and Lange-Nielson
  • Neurodegenerative disorders, such as Hunter syndrome, or sensory motor neuropathies, such as Friedreich ataxia and Charcot-Marie-Tooth syndrome [Harlor: 2009]
  • Culture-positive postnatal infections associated with sensorineural hearing loss, including confirmed bacterial and viral (especially herpes viruses and varicella) meningitis
  • Head trauma, especially basal skull/temporal bone fracture that requires hospitalization
  • Chemotherapy
  • Recurrent/persistent otitis media
See Early Childhood Hearing Outreach Initiative (NCHAM) for information on screening young children with oto-acoustic emissions in the office.

Of Family Members

If a child is diagnosed with permanent childhood hearing loss, all siblings should have an audiology assessment and new siblings by 24 to 30 months of age or sooner. Thereafter, routine subjective assessment of hearing should be conducted annually at well child examinations with objective hearing screening at school entry. Other first-degree relatives might consider evaluation if there are any concerns about hearing loss or related problems.

For Complications

Late-onset and progressive vision loss are associated with hearing impairment in several genetic syndromes – see Hearing Loss Associated with Eye Abnormalities and Vision (Usher Syndrome) . Children who are deaf or hard of hearing (D/HH) should have an annual ophthalmologic assessment to determine visual acuity and screen for ocular disorders. Individuals with hearing loss rely more on other sensory input and should be prescribed prompt correction for refractive errors.

Screening with electrocardiogram (EKG) for Jervell and Lange-Nielsen syndrome, a potentially lethal syndromic hearing loss associated with prolonged QT syndrome, should be considered for infants with bilateral profound sensorineural hearing loss or a positive family history of prolonged QT interval, syncope, or sudden cardiac death. See Electrocardiogram (EKG) Screening (Jervell and Lange-Nielsen Syndrome).

Presentations

Children who are D/HH and who are not detected in the newborn period may present with:
  • Parental/caregiver concern for hearing impairment
  • Language/communication delay or unusual quality of voice
  • Behavioral concerns, such as inattentiveness, poor listening, hyperactivity, or tantrums
  • Decline in school performance

Clinical Classification

There are several categories of hearing loss:
  • Sensorineural hearing loss (SNHL) results from disorders of the cochlea, usually involving the hair cells, or the auditory nerve.
  • Conductive hearing loss (CHL) results from interference with the transmission of sound vibrations through the middle to the inner ear. Although this condition causes less than 20% of hearing loss in the general population, it causes a higher proportion of pediatric cases.
  • Mixed hearing loss refers to the presence of both sensorineural and conductive hearing loss in the same ear. This is the least common form of hearing loss in the pediatric population.
  • Auditory neuropathy spectrum disorder (ANDS) is a disorder in which the function of the inner ear is preserved, but the timing (or synchrony) of action potentials in the auditory nerve is disrupted.
  • Central auditory processing disorder or central hearing loss is rare; it involves the brainstem or auditory cortex of the brain.
  • Hearing loss may be fluctuating and/or progressive
  • The severity of hearing loss is commonly divided into five levels:
    • Mild: 20 - 40 dB
    • Moderate: 41-55 dB
    • Moderately severe: 56-70 dB
    • Severe: 71-90 dB
    • Profound: >90 dB
Hearing loss can be unilateral vs. bilateral, symmetrical vs. asymmetrical, or progressive vs. sudden; sudden hearing loss (occurring over <72 hours) should prompt immediate clinical assessment.

Hearing loss can be unilateral vs. bilateral, symmetrical vs. asymmetrical, or progressive vs. sudden; sudden hearing loss (occurring over <72 hours) should prompt immediate clinical assessment.

Differential Diagnosis

When evaluating a child for suspected hearing impairment, consider the following conditions that may present with similar symptoms:

Medical Conditions Causing Condition

The most common medical condition that causes hearing loss is middle ear effusion, which causes conductive loss that may persist long enough to cause secondary impact on hearing and language development. Causes of Hearing Loss, with ICD-10 codes offers an extensive list of conditions that may cause hearing loss. Another compilation of such conditions can be found in chapter 6 of the e-book A Resource Guide for Early Hearing Detection and intervention (NCHAM). The following Portal pages provide additional detail regarding selected causes of childhood hearing loss and those with associated with problems in organ systems:

History & Examination

The initial approach to the child with suspected hearing impairment should focus on prompt evaluation by a pediatric audiologist for identification and quantification of hearing impairment and referral for appropriate intervention. The Medical Home clinician should also assess for the presence of genetic and neurologic syndromes that are associated with hearing loss.

Current & Past Medical History

Inquire about medical conditions associated with hearing loss, including:
  • Serious bacterial infections, such as meningitis (particularly secondary to H. influenza, herpes, and varicella) and sepsis
  • Head trauma
  • Craniofacial malformations or genetic syndromes that may lead to conductive/mixed hearing loss or Eustachian tube dysfunction (cleft palate, Down syndrome, Turner syndrome, Cornelia de Lange syndrome, branchio-oto-renal syndrome)
  • Syndromes associated with progressive hearing loss (neurofibromatosis type 2, osteopetrosis)
  • Neurodegenerative disorders, such as Hunter syndrome
  • Sensorimotor neuropathies (Charcot-Marie-Tooth syndrome, Friedrich ataxia)
  • Syncope, palpitations, history of a possible cardiac arrest (suggesting arrhythmia that could be related to Jervelle-Lange-Nielson syndrome)
  • Visual impairment, especially retinitis pigmentosa (Usher syndrome) (Hearing Loss Associated with Eye Abnormalities and Vision (Usher Syndrome) )
  • Chemotherapy

Family History

A three-generation pedigree should be obtained, with particular emphasis on hereditary hearing loss, developmental delays, and genetic syndromes associated with hearing loss. Inquire about a family history of early arrhythmia or sudden death, which may suggest prolonged QT syndrome, a component of Jervell and Lange-Nielsen syndrome; see Electrocardiogram (EKG) Screening (Jervell and Lange-Nielsen Syndrome). A history of craniofacial or other malformations in family members may also suggest the presence of syndromic hearing loss. A family history of renal failure suggests Alport syndrome, while the presence of a white forelock or iris heterochromia may indicate Waardenburg syndrome. The lack of a family history does not exclude a genetic etiology; in fact, more than ½ of patients with recessive hearing loss have no previously known affected family members.

Pregnancy/Perinatal History

Pregnancy history should include questions about gestational age and maternal use of tobacco, alcohol, and illicit drugs. Maternal infections such as cytomegalovirus, rubella, herpes, and syphilis may also lead to permanent hearing impairment. The following perinatal risk factors place an infant at risk for progressive or delayed-onset hearing loss:
  • Perinatal infections such as bacterial meningitis and sepsis
  • NICU stay 5 days or longer
  • Hyperbilirubinemia requiring exchange transfusion
  • Persistent pulmonary hypertension of the newborn
  • Extracorporeal membrane oxygenation (ECMO)

Developmental & Educational Progress

Parental or caregiver concern for speech, language, or developmental delay is a risk factor for delayed-onset hearing loss and should prompt audiologic evaluation. Early motor delay may indicate a defect in the vestibular apparatus and the child should have a vestibular examination. A decline in school performance may also signify hearing impairment.

The Medical Home clinician should conduct routine developmental screening and surveillance at all well child visits. It is also recommended that an early intervention program assess language, cognitive skills, and social-emotional development at 6-month intervals during the first 3 years of life for children who are D/HH. For the older child, close attention should be paid to educational progress, as a decline in academic functioning may signify a progression of hearing loss or poor fit/function of amplification devices.

Social & Family Functioning

The diagnosis of hearing impairment in an infant or child often causes significant parental distress and families may need assistance in working through grief issues. Many Early Intervention providers offer parent outreach services to assist in optimizing family functioning. It must be emphasized that early intervention is critical to language and cognitive outcomes and should proceed concurrently with parental outreach/support. Cognizance of the family's perspectives on medical interventions is important, particularly for families with known genetic causes of hearing impairment.

Physical Exam

Growth Parameters

Microcephaly and/or short stature may suggest a congenital infection (e.g., CMV, rubella) or underlying genetic disorder. Tall, thin body habitus is associated with Marfan or Stickler syndrome.

Skin

Pigmentary abnormalities of skin and/or hair may signify a genetic syndrome associated with hearing impairment (cafe au lait macules in neurofibromatosis type 1, hypopigmented patches and/or white forelock in Waardenburg syndrome).

HEENT/Oral

Assess for anatomic craniofacial abnormalities such as submucous cleft palate, preauricular tags/pits, and auricular malformations. Pneumatic otoscopy should be performed to assess tympanic membrane mobility and middle ear pressure. Examine the neck for masses, sinuses, and pits.

Extremities/Musculoskeletal

Musculoskeletal malformations may suggest a genetic syndrome associated with hearing impairment (e.g., cubitus valgus in Turner syndrome, fused digits in Apert syndrome).

Neurologic Exam

Abnormalities in tone or sensation may indicate an underlying neurologic disorder.

Testing

Sensory Testing

Children with newly identified sensorineural hearing loss should have appropriate audiological monitoring of the hearing loss every 3 months during the first year, every 6 months during their preschool years, and at least once a year while in school. In children less than 3 years, early intervention programs are mandated to monitor audiologic status at 6-month intervals. Approximately 33% of children with sensorineural hearing loss show progressive loss of auditory sensitivity. A change in auditory threshold of 10 dB at any test frequency may represent a significant change in hearing sensitivity.

The following tests are commonly used by audiologists to diagnose hearing impairment:
  • Otoacoustic Emissions (OAE): Sound entering the ear is amplified by the outer hair cells of the cochlea. When outer hair cells are healthy, this amplification produces a pressure wave which is transmitted from the inner ear to the middle ear, called otoacoustic emissions (OAE). Because OAE are preneural in origin, they reflect only the health of the cochlea and cannot be used to assess function of the auditory nerve and brainstem pathways. In testing, frequency-specific clicks are introduced into the ear canal through a small probe and the resulting OAEs are recorded. Middle ear fluid or debris in the external auditory canal can reduce the emissions and cause a falsely abnormal result. A screening OAE gives a Pass or Fail (Refer) result; the latter indicates a need for further hearing assessment. Diagnostic testing with OAE analyzes responses in further detail with interpretation by the audiologist.
  • Auditory Brainstem Evoked Responses (AABR or ABR): Sound entering the ear is transduced into action potentials in the brainstem that can be recorded using surface electrodes. Evoked potentials generated in the brainstem are referred to as the auditory brainstem response (ABR). ABR testing evaluates the ability of the inner ear to produce action potentials and their transmission through the auditory nerve and brainstem. Clicks are introduced into the ear canal and EEG signals evoked by the clicks are separated from the background EEG signals, recorded, and analyzed. The automated Auditory Brainstem Evoked Response (AABR), used for newborn screening, employs a statistical algorithm to determine the likelihood that the results reflect normal hearing. Like OAE, the AABR generates a Pass or Fail result; the latter indicates a need for further testing/quantification of hearing. In patients with abnormal AABR results, an audiologist with infant/pediatric expertise should perform a frequency-specific, diagnostic ABR, which can often be performed without sedation in infants under 6 months of age.
  • Audiometry
    • In very young infants, typically less than six months of age, behavioral observation is not used to assess hearing sensitivity. [Sabo: 2003] Visual reinforced audiometry (VRA) is the method of choice in children who are developmentally 6 months to 2 years of age. [Gravel: 1992] VRA involves presentation of a sound and rewarding appropriately-timed head turns with a picture or other visual stimulus. [Lidén: 1969] Inserts or headphones can be utilized to obtain ear-specific information, with inserts being preferred to avoid collapsing ear canals. Inserts are more reliably tolerated in children greater than 18 months of age, although a number of pediatric centers have reported excellent success with younger children. [Weiss: 2016] A positive response generally requires a 90-degree head turn, and normative data by age is available for interpretation. [Sabo: 2003]
    • Conditioned play audiometry (CPA) may be used with children developmentally 2 to 5 years of age. In this technique, the child is first briefly conditioned to ensure understanding of the task and that they can reliably participate. The chosen task may vary based on child interest and cooperation – for example, putting a peg in a board, tossing a block in a box, or a digital task in response to hearing a sound. [Nielsen: 1997] [Nielsen: 1997]
    • Conventional Audiometry: the child indicates that he/she can hear a sound by raising a hand or pressing a button. The threshold, or quietest, level at which the child can detect sounds at various frequencies is measured, allowing the type, degree, and symmetry of an impairment to be delineated. Conventional audiometry can usually be performed by the time a child is 3-4 years of age.
    • Acoustic Immittance Testing (aka tympanometry) tests middle ear pressure and tympanic membrane compliance and is used to confirm middle ear conditions, such as a fluid-filled middle ear, perforations in the tympanic membrane, and other disorders. Acoustic immittance testing is performed using a hand-held probe with an airtight seal within the ear canal. The probe emits a stimulus tone, which is reflected back to the probe, while the air pressure in the ear canal is changed from negative to positive. The reflected sound is detected by a microphone and quantified to determine the acoustic immittance, which is a measure of the ease of sound flow through the tympanic membrane and the middle ear ossicles. The probe can also measure tympanic membrane mobility, the volume of the ear canal, and the level of sound stimulation required to make the stapedius muscle contract, known as the acoustic reflex threshold.

Laboratory Testing

Routine blood testing of children with sensorineural hearing loss is of little diagnostic value and is not recommended. [Mafong: 2002]

CMV-testing of urine or saliva in the first 3 weeks of life should be performed in infants who fail newborn screening to identify congenital CMV infection. Studies obtained after 3 weeks of age cannot differentiate between congenital and postnatal infection.

Children with asymptomatic congenital CMV infection should have hearing screens at birth and then periodically until school age.

Imaging

If no genetic or medical cause of hearing loss is identified, an MRI scan of the temporal bones and possibly the brain may be considered to identify isolated malformations of the inner ear [Mafong: 2002] and brain. In some cases, an MRI may be helpful in detecting malformations or absence of the auditory nerve, especially for unilateral auditory neuropathy spectrum disorder or subtle central nervous abnormalities associated with CMV. [Mafong: 2002]

Genetic Testing

Genetic testing should be considered in all children without a known etiology for their hearing loss. Patients requiring genetic testing should be referred to a geneticist, genetic counselor, or interdisciplinary hearing assessment clinic, if available. Genetic testing involving all the commonly involved genes implicated in hearing loss is now available and the cost is commonly covered by insurance. Testing for mutations for the gene encoding connexin-26 is the most common molecular investigation. Genetic testing will often predict which children need to be followed closely for retinal, renal, cardiac, etc. problems.

Other Testing

Electrocardiography is indicated if there is a family history of prolonged QT interval or sudden death, or if medical history is significant for syncope, arrhythmia, or history of ALTE. An EKG should be considered in all infants with severe-profound sensorineural hearing loss of unknown etiology.

Specialty Collaborations & Other Services

Pediatric Genetics (see Services below for local providers)

Reviews medical and family history, performs a thorough physical examination to identify hereditary causes of hearing loss. The genetics team may also order genetic tests to diagnose/confirm hereditary causes of hearing loss.

Pediatric Genetic Counseling (see Services below for local providers)

Provides families with information regarding likelihood of progression, associated disorders, and potential for recurrence in future children.

Pediatric Otolaryngology (see Services below for local providers)

Performs a comprehensive evaluation of the head and neck and can help in the identification of craniofacial malformations associated with hearing loss. Should work closely with the audiology team (including Early Intervention specialist) to determine intervention planning for hearing loss. Performs surgical intervention if necessary (e.g., tympanostomy tubes, reconstruction, cochlear implant, bone-anchored hearing aids).

Audiology (see Services below for local providers)

Performs audiologic testing; some centers perform testing under sedation when ordered by a physician. Prescribes and fits amplification when indicated.

Pediatric Ophthalmology (see Services below for local providers)

Every child with confirmed hearing loss should undergo annual evaluation by an ophthalmologist to determine visual acuity and evaluate for concomitant vision disorders (e.g., Usher Syndrome).

Speech - Language Pathologists (see Services below for local providers)

Evaluates speech and language development, develops and implements language therapy programming for those with language delays.

Developmental Assessment (see Services below for local providers)

Consider a comprehensive developmental evaluation if a child shows delays in other streams of development (social, adaptive, gross- or fine-motor).

Early Intervention for Children with Disabilities/Delays (see Services below for local providers)

Developmental therapies for children under 3 years. When there is an associated fee, private services funded through health insurance may cost less.

Public Special Schools (see Services below for local providers)

May offer a specialized classroom setting or consultation with a classroom teacher regarding modifications to aid the child with hearing impairment. Many programs have infant and parent education programs.

Treatment & Management

Pearls & Alerts for Treatment & Management

Impact of otitis media with effusion

Children with underlying sensorineural or permanent conductive hearing loss are more susceptible to further hearing impairment or speech and language delay when they are afflicted by middle ear fluid (otitis media with effusion, OME). Children with underlying permanent hearing loss should be referred for audiologic assessment and otolaryngology consultation if their middle ear effusion persists for more than one month.

Cochlear implants are approved by the FDA for children 12 months and older

Candidacy for cochlear implant depends on a number of factors including the child’s otologic anatomy, hearing status, caregiver motivation and support.

Unilateral hearing loss

Children with unilateral hearing loss are at risk for academic failure, experience considerable difficulty in understanding speech in a background of noise, experience trouble with localization (with resulting safety issues), and appear to exhibit more behavioral problems in school. For these reasons, amplification should be used for the affected ear, if possible.

Systems

Ears/Hearing

Amplification – Most children who are deaf or hard of hearing (D/HH) benefit from some type of amplification device (e.g., hearing aids, cochlear implantation, personal FM systems for the classroom). Amplification recommendations are individualized based on the age of the child, type and severity of hearing impairment, and family choice. Additional information about most of the following can be found by clicking on the name link.
Hearing aid, infant
Child with hearing aid
assorted hearing aids
Assorted hearing aids

Infants should be fitted with Hearing Aids within one month of identification of hearing loss to achieve maximum benefit, even if diagnostic audiological evaluation is still in process. Selection, fitting, and monitoring of hearing aids should be performed by an audiologist with infant/pediatric experience and expertise.

Cochlear Implantation may be considered if limited benefit is derived from hearing aids, although at times it is recommended as the primary amplification device. Candidacy depends on a number of factors including the child’s otologic anatomy, hearing status, caregiver motivation and support. Most cochlear centers around the country have a multidisciplinary approach to evaluate candidacy. In general, children between 12 and 18 months of age are candidates if they have profound sensorineural hearing loss (90 decibels or greater) in both ears. Children 18 months of age and older are candidates if they have severe to profound sensorineural hearing loss (70 decibels or greater) in both ears. Children younger than 12 months of age should be considered for early implantation if their profound loss was caused by meningitis, which may cause ossification of the cochlear structures making implantation more difficult later.

Frequency Modulation (FM) Auditory Trainers use a speaker-worn microphone to transmit amplified speech to a receiver attached to the patient's hearing aid.

Auditory Brainstem Implants may be considered in children who are not candidates for cochlear implantation due to abnormalities of the auditory nerve.

Bone anchored hearing aids amplify sound through bone vibration and are used in children with permanent conductive hearing loss due to external and/or middle ear disorders, malformations, or trauma.

Other surgical intervention – Children with conductive hearing loss due to otitis media with effusion who have normal baseline hearing should be monitored closely with surgical intervention reserved for those with medical or developmental risk factors or significant changes in hearing. Ventilation tubes are the most common surgical procedure to eliminate middle ear fluid and correct persistent otitis media with effusion. Ossicular reconstruction is indicated for ossicular abnormalities from congenital malformations (e.g., malleus fixation) or defects (e.g., cholesteatoma). Closure of congenital perilymphatic fistula can be associated with cochleovestibular dysplasia and may be recommended to prevent recurrent meningitis but may have little impact on ultimate hearing outcome. [Doyle: 2003]

Specialty Collaborations & Other Services

Audiology (see Services below for local providers)

Monitors audiological status; prescribes amplification and monitors function and fit.

Pediatric Otolaryngology (see Services below for local providers)

Examines the structures of the head and neck (e.g., microscopic otologic examination); performs surgical intervention such as cochlear implantation, ossicular reconstruction, tympanostomy tube placement with surgical follow-up.

Development/Language

Early involvement in family-centered language therapy results in better language and verbal reasoning outcomes at 5 years of age. [Moeller: 2000] Family participation in language/communication therapy is an important factor in ultimate language outcomes, particularly for those children with severe or profound permanent SNHL. [Watkin: 2007]

Specialty Collaborations & Other Services

Early Intervention for Children with Disabilities/Delays (see Services below for local providers)

Federal- and state-funded early intervention services from birth to age three. When there is an associated cost involved, private speech therapy services may be more affordable.

Speech - Language Pathologists (see Services below for local providers)

Evaluate and form a treatment plan for infants and children with language delay associated with hearing impairment. May also assist in teaching other communication strategies, such as sign language and picture-exchange communication systems (PECS).

Immunology/Infectious Disease

Congenital infection with cytomegalovirus (CMV) causes 10-20% of all cases of sensorineural hearing loss (SNHL). [Goderis: 2014] One-third of children with symptomatic CMV (characterized by any combination of the following: low-birth weight, microcephaly, thrombocytopenia, jaundice, hepatosplenomegaly, seizures) and 1:10 children with asymptomatic CMV will develop sensorineural hearing loss. [Goderis: 2014]

CMV-related hearing loss is often progressive and may develop after the neonatal period. Fluctuating hearing loss is also common. Children with asymptomatic congenital CMV infection should have hearing screens at birth and then periodically until school age to identify and treat hearing impairment in a timely manner.

Research supports treatment of newborns with symptomatic congenital CMV infection to prevent or mitigate sensorineural hearing loss. Current recommendations are to start oral valganciclovir within the first month of life. [Rawlinson: 2017] These children should have serial hearing screens starting at birth, then every 6 months until age 3.

In older children, particularly those with a family history of renal failure, consider a urinalysis to assess for hematuria and proteinuria suggestive of Alport syndrome.

Prevention of congenital CMV through pre-conception vaccination has been a hot topic in recent years. The goal is to allow women to develop immunity before they become pregnant so that there is low risk of passing the infection to the fetus. [Schleiss: 2017] For more information, see Congenital Cytomegalovirus (CMV) Related Hearing Loss.

Specialty Collaborations & Other Services

Pediatric Infectious Disease (see Services below for local providers)

Contact infectious disease experts for advice regarding testing and management of congenital infection syndromes.

Funding & Access to Care

Interventions for hearing loss in most children are covered by private health insurance and Medicaid. While Medicaid has a federal mandate to provide screening, diagnostic, and therapeutic hearing-related services, private insurers are under no such regulations. Financing and Reimbursement (NCHAM) provides detailed information regarding appropriate coding and billing for hearing related services to optimize support from third-party payers.

Some states have hearing aid recycling programs or offer other support for those who cannot independently afford amplification devices. Information about funding assistance can be obtained through local Early Intervention providers, hearing assessment centers, and school hearing programs. Funding for Service Delivery (ASHA) provides additional information regarding potential funding sources for audiology and hearing services. Some state Early Hearing Detection and Intervention (EHDI) programs have additional information regarding help with funding for hearing aids.

Specialty Collaborations & Other Services

Audiology (see Services below for local providers)

Monitors audiological status; prescribes amplification and monitors function and fit.

Public Special Schools (see Services below for local providers)

May offer a specialized classroom setting or consultation with a classroom teacher regarding modifications to aid the child with hearing impairment. Many programs have infant and parent education programs.

Early Intervention for Children with Disabilities/Delays (see Services below for local providers)

State-coordinated programs for children from birth to age 3 offering audiologic services as well as developmental therapies.

Issues Related to Hearing Loss and Deafness

Ask the Specialist

Additional answers to common questions about the causes of hearing loss in children can be found at:

Causes of Hearing Loss FAQ (My Baby's Hearing)
Answers to questions about the causes, particularly genetic, of hearing loss in children; sponsored by Boystown National Research Hospital.

More questions and answers can be found at:

Frequently Asked Questions about Childhood Hearing Loss (Desert Voices)
Offers a number of questions and answers from the perspective of parents with children who are deaf or hard of hearing.

What is my patient has not had newborn screening?

Newborn hearing screening may not be completed with home births or births at centers not offering screening, and occasionally infants will be discharged to home prior to its completion. In these cases, referral should be made for outpatient hearing screening as soon as possible and no later than 1 month of age.

Resources for Clinicians

On the Web

Hearing Loss & Deafness
Guidance for clinical follow-up and further testing after a positive newborn screen.

Hearing Development Questionnaire
Questions by age range that reflect typical development of skills related to hearing and communication.

American Academy of Audiology
Resources for professionals who test, treat, and provide care to the deaf or hard of hearing.

Information & Resources on Hearing Loss for Professionals (EHDI-PALS)
Resources for professionals; Early Hearing Detection & Intervention - Pediatric Audiology Links to Services.

Permanent Childhood Hearing Loss (ASHA)
Comprehensive clinical topic review with a focus on family-centered care; American Speech-Language-Hearing Association.

Early Hearing Detection and Intervention (AAP)
Enhances clinical knowledge of the Early Hearing Detection and Intervention (EHDI) program and screening guidelines, and helps to ensure that newborn screening results are communicated to families and reported according to state laws; American Academy of Pediatrics and the Early Hearing Detection and Intervention Program. This site also has links to state chapter EHDI experts and resources.

Financing and Reimbursement (NCHAM)
Details about clinical coding and paying for interventions for hearing impairment; National Center for Hearing Assessment and Management.

Helpful Articles

PubMed search for hearing loss or deafness in children, last 1 year

Gifford KA, Holmes MG, Bernstein HH.
Hearing loss in children.
Pediatr Rev. 2009;30(6):207-15; quiz 216. PubMed abstract

Katbamna B, Crumpton T, Patel DR.
Hearing impairment in children.
Pediatr Clin North Am. 2008;55(5):1175-88, ix. PubMed abstract

Shearer AE, Smith RJ.
Genetics: advances in genetic testing for deafness.
Curr Opin Pediatr. 2012;24(6):679-86. PubMed abstract / Full Text

Clinical Tools

Care Processes & Protocols

Newborn Hearing Screening - Guidelines for the Medical Home (EDHI) (PDF Document 85 KB)
Flowchart and information for assuring documentation of normal newborn hearing screening or appropriate follow-up if negative; Early Hearing Detection & Intervention Program.

Clinical Checklists & Visit Tools

1-3-6 Newborn Hearing Checklist (AAP) (PDF Document 105 KB)
Checklist for assuring early detection and intervention for infants born with hearing loss; American Academy of Pediatrics and the Early Hearing Detection and Intervention Program.

Patient Education & Instructions

'Just in Time' Hearing Resources for Families (NCHAM) (PDF Document 574 KB)
A two-page compilation of valuable resources for families with concerns about hearing loss in their child; National Center for Hearing Assessment and Management.

'Just in Time' Hearing Resources for Families (NCHAM) (Spanish) (PDF Document 639 KB)
Two-page compilation of valuable resources for families with concerns about hearing loss in their child; National Center for Hearing Assessment and Management.

Resources for Patients & Families

Information on the Web

Parents' Guide to Hearing Loss (CDC)
Website with comprehensive information on hearing loss in children, including intervention options, building language, decision making, resources, and a glossary of related terms; from the Centers for Disease Control and Prevention.

Strategies for Keeping Hearing Aids on Young Children (Success for Children with Hearing Loss)
Age-appropriate tips for keeping and checking hearing aids in babies, toddlers, and preschoolers;

Resources for Parents of Children Who are Deaf or Hard of Hearing (EHDI-PALS)
Information and questions to ask of your hearing and health providers; Early Hearing Detection and Intervention-Pediatric Audiology Links to Services.

Hearing Tests (My Baby's Hearing)
Overview of hearing testing in children; Boys Town National Research Hospital.

AUDIENT Alliance for Accessible Hearing Care
The AUDIENT Alliance for Accessible Hearing Care Program is designed for individuals whose income is above the government's established poverty levels, but still find it difficult to afford quality hearing care.

Familiar Sounds Audiogram in English and Spanish (PDF Document 381 KB)
Graphic showing normal hearing to profound hearing loss for loudness and pitch. Adapted from the AAP.

Hearing Loss in Children (CDC)
Information for families, clinicians, public health departments, and others; including questions that families may want to ask various professionals (under the Free Materials link); from the Centers for Disease Control & Prevention.

Universal Newborn Hearing Screening (My Baby's Hearing)
Information about specific aspects of newborn hearing screening, as well as information for families who have recently received a diagnosis; Boys Town National Research Hospital.

National & Local Support

American Society for Deaf Children
Independent nonprofit organization whose purpose is to provide support and information to families raising children who are deaf or hard-of-hearing.

American Speech-Language-Hearing Association
Information for professionals working in audiology, speech-language pathology, and the speech and hearing sciences. Advocate for people with communication disabilities.

American Academy of Audiology
Resources for professionals who test, treat, and provide care to the deaf or hard of hearing.

Alexander Graham Bell Association
One of the oldest and most comprehensive organizations focused on pediatric hearing loss, including information on how to find a provider, funding sources and information, scholarships, and a family support section.

Hard of Hearing and Deaf Services (Easter Seals)
Offers a range of services to assist people with hearing impairment or hearing loss, including hearing aids, audiology, speech and hearing therapy, or referral to a specialist. Includes general and state-specific resources.

Organizations of and for People who Are Deaf and Hard of Hearing (LCNDEC)
Comprehensive information compiled by deaf adults and educators; provided by the Laurent Clerc National Deaf Education Center at Gallaudet University.

National Association of the Deaf
National organization whose goal is the cure and prevention of all forms of hearing loss. They also publish a blog and magazine, provide scholarships, and offer education about disability benefits.

Family Voices
A national, nonprofit, family-led organization promoting quality health care for all children and youth, particularly those with special health care needs. Locate your center or F2F HIC (Family-to-Family Health Information Center) by state on this site.

Family Support for Children Who are Deaf or Hard of Hearing (NCHAM)
Extensive compilation of resources and sources of support for families that have a child who is deaf or hard of hearing; National Center for Hearing Assessment and Management.

Services for Patients & Families in Nevada (NV)

For services not listed above, browse our Services categories or search our database.

* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.

Authors & Reviewers

Initial publication: October 2012; last update/revision: February 2019
Current Authors and Reviewers:
Author: Janet W. Lee, MD
Senior Author: Albert H. Park, MD
Authoring history
2018: update: Jennifer Goldman-Luthy, MD, MRP, FAAPR
2013: update: Jennifer Goldman-Luthy, MD, MRP, FAAPA; Richard Harward, AuDR; Albert H. Park, MDR; John C. Carey, MDR; Terry E. Foust, AuD, CCC-SLPR; Catherine Jolma, MDR
2011: revision: William Kimberling, Ph.D.A
2008: first version: Karl White, Ph DA
AAuthor; CAContributing Author; SASenior Author; RReviewer

Bibliography

Alford RL, Arnos KS, Fox M, Lin JW, Palmer CG, Pandya A, Rehm HL, Robin NH, Scott DA, Yoshinaga-Itano C.
American College of Medical Genetics and Genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss.
Genet Med. 2014;16(4):347-55. PubMed abstract

Barsky-Firkser L, Sun S.
Universal newborn hearing screenings: a three-year experience.
Pediatrics. 1997;99(6):E4. PubMed abstract

Centers for Disease Control and Prevention.
Identifying infants with hearing loss - United States, 1999-2007.
MMWR Morb Mortal Wkly Rep. 2010;59(8):220-3. PubMed abstract

Davis JM, Elfenbein J, Schum R, Bentler RA.
Effects of mild and moderate hearing impairments on language, educational, and psychosocial behavior of children.
J Speech Hear Disord. 1986;51(1):53-62. PubMed abstract

Doyle KJ, Ray RM.
The otolaryngologist's role in management of hearing loss in infancy and childhood.
Ment Retard Dev Disabil Res Rev. 2003;9(2):94-102. PubMed abstract

Finitzo T, Albright K, O'Neal J.
The newborn with hearing loss: detection in the nursery.
Pediatrics. 1998;102(6):1452-60. PubMed abstract

Gifford KA, Holmes MG, Bernstein HH.
Hearing loss in children.
Pediatr Rev. 2009;30(6):207-15; quiz 216. PubMed abstract

Goderis J, De Leenheer E, Smets K, Van Hoecke H, Keymeulen A, Dhooge I.
Hearing loss and congenital CMV infection: a systematic review.
Pediatrics. 2014;134(5):972-82. PubMed abstract

Gravel JS, Traquina DN.
Experience with the audiologic assessment of infants and toddlers.
Int J Pediatr Otorhinolaryngol. 1992;23(1):59-71. PubMed abstract

Harlor AD Jr, Bower C.
Hearing assessment in infants and children: recommendations beyond neonatal screening.
Pediatrics. 2009;124(4):1252-63. PubMed abstract / Full Text

Johnson JL, Kuntz NL, Sia CC, White KR, Johnson RL.
Newborn hearing screening in Hawaii.
Hawaii Med J. 1997;56(12):352-5. PubMed abstract

Joint Committee on Infant Hearing.
Year 2007 position statement: Principles and guidelines for early hearing detection and intervention programs.
Pediatrics. 2007;120(4):898-921. PubMed abstract / Full Text

Katbamna B, Crumpton T, Patel DR.
Hearing impairment in children.
Pediatr Clin North Am. 2008;55(5):1175-88, ix. PubMed abstract

Kennedy C, McCann D, Campbell MJ, Kimm L, Thornton R.
Universal newborn screening for permanent childhood hearing impairment: an 8-year follow-up of a controlled trial.
Lancet. 2005;366(9486):660-2. PubMed abstract

Kennedy CR, McCann DC, Campbell MJ, Law CM, Mullee M, Petrou S, Watkin P, Worsfold S, Yuen HM, Stevenson J.
Language ability after early detection of permanent childhood hearing impairment.
N Engl J Med. 2006;354(20):2131-41. PubMed abstract

Lidén G, Kankkunen A.
Visual reinforcement audiometry.
Arch Otolaryngol. 1969;89(6):865-72. PubMed abstract

Linden Phillips L, Bitner-Glindzicz M, Lench N, Steel KP, Langford C, Dawson SJ, Davis A, Simpson S, Packer C.
The future role of genetic screening to detect newborns at risk of childhood-onset hearing loss.
Int J Audiol. 2013;52(2):124-33. PubMed abstract / Full Text

Mafong DD, Shin EJ, Lalwani AK.
Use of laboratory evaluation and radiologic imaging in the diagnostic evaluation of children with sensorineural hearing loss.
Laryngoscope. 2002;112(1):1-7. PubMed abstract

Mehl AL, Thomson V.
Newborn hearing screening: the great omission.
Pediatrics. 1998;101(1):E4. PubMed abstract

Mehra S, Eavey RD, Keamy DG Jr.
The epidemiology of hearing impairment in the United States: newborns, children, and adolescents.
Otolaryngol Head Neck Surg. 2009;140(4):461-72. PubMed abstract

Moeller MP.
Early intervention and language development in children who are deaf and hard of hearing.
Pediatrics. 2000;106(3):E43. PubMed abstract

Moller A.
Hearing: Anatomy, Physiology, and Disorders of the Auditory System.
3rd ed. San Diego: Plural Publishing; 2012. 978-1597564274

Nielsen SE, Olsen SO.
Validation of play-conditioned audiometry in a clinical setting.
Scand Audiol. 1997;26(3):187-91. PubMed abstract

Rawlinson WD, Boppana SB, Fowler KB, Kimberlin DW, Lazzarotto T, Alain S, Daly K, Doutré S, Gibson L, Giles ML, Greenlee J, Hamilton ST, Harrison GJ, Hui L, Jones CA, Palasanthiran P, Schleiss MR, Shand AW, van Zuylen WJ.
Congenital cytomegalovirus infection in pregnancy and the neonate: consensus recommendations for prevention, diagnosis, and therapy.
Lancet Infect Dis. 2017;17(6):e177-e188. PubMed abstract

Sabo DL, Paradise JL, Kurs-Lasky M, Smith CG.
Hearing levels in infants and young children in relation to testing technique, age group, and the presence or absence of middle-ear effusion.
Ear Hear. 2003;24(1):38-47. PubMed abstract

Schleiss MR, Permar SR, Plotkin SA.
Progress toward Development of a Vaccine against Congenital Cytomegalovirus Infection.
Clin Vaccine Immunol. 2017;24(12). PubMed abstract / Full Text

Shearer AE, Smith RJ.
Genetics: advances in genetic testing for deafness.
Curr Opin Pediatr. 2012;24(6):679-86. PubMed abstract / Full Text

US Preventive Services Task Force.
Universal screening for hearing loss in newborns: US Preventive Services Task Force recommendation statement.
Pediatrics. 2008;122(1):143-8. PubMed abstract

Watkin P, McCann D, Law C, Mullee M, Petrou S, Stevenson J, Worsfold S, Yuen HM, Kennedy C.
Language ability in children with permanent hearing impairment: the influence of early management and family participation.
Pediatrics. 2007;120(3):e694-701. PubMed abstract

Weiss AD, Karzon RK, Ead B, Lieu JE.
Efficacy of earphones for 12- to 24-month-old children during visual reinforcement audiometry.
Int J Audiol. 2016;55(4):248-53. PubMed abstract

Yoshinaga-Itano C, Sedey AL, Coulter DK, Mehl AL.
Language of early- and later-identified children with hearing loss.
Pediatrics. 1998;102(5):1161-71. PubMed abstract