Hearing Loss & 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 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.

labeled and illustrated Diagram of the outer and inner 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 amplified 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 loss 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 genetic variations, infection, trauma, etc. Hearing loss may also be grouped into types, related to the cause or mechanism of the loss, the ranges of severity, described by the decibels below which the child cannot hear or discriminate sounds, and the pattern of alteration by frequency on the audiogram. These groupings are detailed below under Clinical Classification. [Lieu: 2020] provides a current and comprehensive review of hearing loss in children.
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.

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, which 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 loss, 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, especially congenital rubella and meningitis. Almost 400 genes have been implicated in hereditary hearing loss (Hereditary Hearing Loss Homepage). With the exception of GJB2-related hearing loss each of these genes is responsible for only a small percentage of cases. Genetic testing 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] [Mehta: 2016] [Downie: 2021] For example, when the cause of a patient’s hearing loss is found to be mutations in the gene, GJB2, encoding the gap junction protein, connexin 26 (Cx26), the child's clinician can be reasonably assured that there will be no comorbid symptoms, like retinitis pigmentosa or renal abnormality. Patients with GJB2-related hearing loss uncommonly have progressive hearing loss and are also known to do well with cochlear implantation.
Pathogenic variations in GJB2 are responsible for approximately 10 to 20% of all childhood genetic hearing loss and cause about 50% of the loss 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 GJB2 gene sequencing are available clinically and should be offered to families affected by non-syndromic hearing loss of unknown cause. The American College of Medical Genetics and Genomics also recommended consideration of hearing loss gene panel tests using next-generation sequencing testing for children with sensorineural hearing loss of unknown cause. [Alford: 2014] More recently some authors have suggested proceeding to exome sequencing (ES) in the setting of a child with no known cause of the hearing loss or deafness; [Downie: 2021] however obtaining insurance coverage in the US for ES can be more difficult than a gene panel. If there are findings on history and physical exam which suggest a syndromic etiology for hearing loss, directed gene testing for the syndrome is indicated. [Alford: 2014] Currently, the genes known to cause specific syndromes (e.g., PAX3 in Waardenburg syndrome) are available on most commercially available hearing loss gene panels or on specific panels covering the particular genes causing the syndrome. More than 10 commercial or University-based laboratories in North America offer gene panels for hearing loss and deafness that include over 100 or more genes using next-generation sequencing technologies. These laboratories can be accessed through the Genetic Testing Registry.
Common recessive types of hearing loss are Usher syndrome (16 genes, ~3-6% of congenital hearing loss) and SLC16A4-related hearing loss including Pendred syndrome. Common autosomal dominant syndromes causing hearing loss include are Waardenburg syndromes 1-4, branchio-oto-renal (BOR) syndrome, and CHARGE syndrome. All of these syndromes have current comprehensive reviews available in GeneReviews, the open access NIH/NLM online resource (GeneReviews (NLM)).

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] Other 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 2019 position statement: Principles and guidelines for early hearing detection and intervention programs.
Journal of Early Hearing Detection and Intervention. 2019. / 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

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

Liming BJ, Carter J, Cheng A, Choo D, Curotta J, Carvalho D, Germiller JA, Hone S, Kenna MA, Loundon N, Preciado D, Schilder A, Reilly BJ, Roman S, Strychowsky J, Triglia JM, Young N, Smith RJ.
International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient.
Int J Pediatr Otorhinolaryngol. 2016;90:251-258. PubMed abstract

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 child with D/HH 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 consider 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: 2019] 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: 2019] This approach has been referred to as the 1-3-6 Newborn Hearing Checklist (AAP) ( PDF Document

105 KB).
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 loss 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, congenital cytomegalic virus (CMV) syndrome), 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 or more formal diagnostic ABR testing 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 Bright Futures/AAP Periodicity Schedule. 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 regular testing through school age. In Utah, testing is recommended every 3 months until 3 years of age then every 6 months until 6 years than annually afterwards.
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 12-18 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 Usher Syndrome Type I (GeneReviews) and Usher Syndrome Type II (GeneReviews). 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 (GeneReviews), 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.

Presentations

Children who are D/HH and who are not detected in the newborn period may present with:

Parental/caregiver concern for hearing loss
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:

Congenital, prelingual, or later-onset hearing loss is based on age of diagnosis
The causes of hearing loss are classified as genetic, environmental, and cryptogenic (unknown etiology)
Sensorineural hearing loss (SNHL) results from disorders of the cochlea, often involving the hair cells.
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, CHL causes a higher proportion of pediatric cases (e.g. otitis media with effusion).
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 (This finding can be due to a hypoplastic or aplastic cochlear nerve. This result has clinical implications if a cochlear implant is being considered).
Central auditory processing disorder or central hearing loss may be more common than recognized; prevalence in school-age children has been reported to be 2-3%. These children have difficult understanding speech despite audiometrically normal hearing.
Hearing loss may be stable, fluctuating and/or progressive
The severity of hearing loss is commonly divided into five levels: [Clark: 1981]
- Mild: 20 - 40 dB
- Moderate: 41-55 dB
- Moderately severe: 56-70 dB
- Severe: 71-90 dB
- Profound: >90 dB

Hearing may may be low frequency, high frequency, predominantly in mid frequencies (“cookie bite”) , or across all frequencies.
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:

Language disorder (expressive, receptive, or mixed)
Autism Spectrum Disorders; see Autism Spectrum Disorder
Attention deficit disorder, with or without hyperactivity; see Attention-Deficit/Hyperactivity Disorder (ADHD)
Intellectual Disability; see Intellectual Disability & Global Developmental Delay

Medical Conditions Causing Hearing Loss and Deafness

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. Missing link with id: d90b91f.xml 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:

Congenital Cytomegalovirus (CMV) Related Hearing Loss
Hearing loss associated with renal disease (Alport syndrome, Branchio-Oto-Renal syndrome) - Hearing loss syndromes are often associated with abnormal kidney function/morphology stemming from molecular similarities between the cochlea and the kidneys.
- Alport Syndrome: A diagnosis of autosomal dominant or X-linked Alport syndrome should be entertained in patients (especially boys) who have sensorineural hearing loss and hereditary nephritis suggested by proteinuria and microscopic hematuria. Consultation with a pediatric nephrologist and geneticist is recommended for individuals with hearing loss and persistent hematuria/proteinuria. Vision may also be affected.
- Branchio-Oto-Renal Syndrome: Children with sensorineural, conductive or mixed hearing loss and branchial arch anomalies (neck masses, branchial cleft cysts or fistulae), should undergo renal ultrasonography to evaluate for anomalies associated with Branchio-Oto-Renal syndrome. Renal anomalies vary in this condition and patients are often asymptomatic. Genetic testing is available and should be coordinated through a geneticist or genetic counselor .
Usher sydrome - At least 3 distinct types of Usher syndrome have been described:
- Type 1 is characterized by profound hearing loss, retinitis pigmentosa with onset before age 10 years, and vestibular abnormalities. These children may be delayed in their acquisition of early motor milestones.
- Type 2 displays sloping, high-frequency sensorineural hearing loss, normal vestibular function, and later onset of retinitis pigmentosa than that seen in Type 1 disease.
- Type 3 is characterized by progressive audio-vestibular dysfunction over decades and variable-onset of retinitis pigmentosa. Not every patient with both retinitis pigmentosa and sensorineural hearing loss has Usher syndrome. Some authors have suggested that all children with severe to profound hearing loss should be screened by ophthalmologic exam and electroretinogram for Usher syndrome. In one study of consecutive patients over 2 years of age with severe to profound preverbal hearing loss, 10.4% (5 out of 48) were diagnosed with Usher syndrome after undergoing complete ophthalmologic evaluation that included an electroretinogram. [Mets: 2000]

History & Examination

The initial approach to the child with suspected hearing loss should focus on prompt evaluation by a pediatric audiologist for identification and quantification of hearing loss 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:

History of prematurity and related complications including administration of aminoglycosides (see Pregnancy and Perinatal History below)
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, Treacher Collins syndrome, Goldenhar syndrome, Cornelia de Lange syndrome, branchio-oto-renal syndrome)
Syndromes associated with progressive hearing loss (neurofibromatosis type 2, osteopetrosis, renal tubular acidosis)
Neurodegenerative disorders, such as Hurler syndrome, 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 Jervell and Lange-Nielson syndrome)
Visual impairment, especially retinitis pigmentosa (Usher syndrome)
Chemotherapy

Family History

A three-generation pedigree should be obtained, with particular emphasis on hereditary hearing loss, consanguinity, developmental delays, and genetic syndromes associated with hearing loss. The question of consanguinity between the parents should be asked and if present the degree of relationship should be documented. 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. 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 loss. The following perinatal risk factors place an infant at risk for progressive or delayed-onset hearing loss:

Perinatal infections such as bacterial meningitis, cytomegalovirus and sepsis
NICU stay 5 days or longer
Hyperbilirubinemia requiring exchange transfusion
Persistent pulmonary hypertension of the newborn
Extracorporeal membrane oxygenation (ECMO)
Administration of aminoglycosides or loop diuretics

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 loss.
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 loss in an infant or child can cause significant parental distress and families may need assistance in working through coping and 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 loss.

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 Stickler syndrome.

Skin

Pigmentary abnormalities of skin and/or hair may signify a genetic syndrome associated with hearing loss (cafe au lait macules in multiple lentigines syndrome, hypopigmented patches and/or white forelock in Waardenburg syndrome). The nails should be examined for hypoplasia and leukonychia totalis.

HEENT/Oral

Assess for craniofacial abnormalities such as wide-spaced eyes (hypertelorism, telecanthus), 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 and thyroid enlargement (goiter) in older children.

Extremities/Musculoskeletal

Musculoskeletal malformations may suggest a genetic syndrome associated with hearing loss (e.g., thumb defects in Townes-Brocks 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 loss:

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 a loss 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

CMV-testing of urine in the first 3 weeks of life should be the first test ordered in any infant who fails newborn screening to identify congenital CMV infection. The time of testing is important to avoid confusion from a postnatal infection which does not cause hearing loss. Saliva CMV testing can be more convenient to obtain; however, false positive results have been reported. For that reason, a positive saliva CMV result needs to be confirmed by a urine CMV test before the infant is 3 weeks of age. Urine PCR or culture obtained after 3 weeks of age cannot differentiate between congenital and postnatal infection. If congenital CMV is being considered in a child older than 3 weeks of age, a neonatal archived dried blood spot sample can be used to test for this virus. If positive, a diagnosis of congenital CMV can be established. A landmark study by Boppana et al. reported that DBS PCR had low sensitivity. [Boppana: 2010] A more recent interim study by Dollard et al. suggest a relatively high analytic sensitivity from DBS compared to previous studies. [Dollard: 2021]
Children with asymptomatic congenital CMV infection should undergo a diagnostic auditory brainstem response study. If the result is normal, the child still needs to undergo regular hearing screening given the higher risk for late onset and progressive hearing loss.

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] aand brain (e.g. lesions associated with congenital CMV infection). 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] The International Pediatric Otolaryngology group suggested performing an imaging study following CMV testing in unilateral hearing loss in children. [Liming: 2016]

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 medical 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, GJB2, is the most common molecular investigation. Genetic testing will often predict which children need to be followed closely for retinal, renal, cardiac, and other problems. The result can inform surveillance; for example if a child has gene changes for an Usher syndrome type 1 gene, annual assessment by an ophphamologist familiar with retinal conditions would be recommended after the child is 10 years of age. Depending on the result, the identification of certain genes can predict likely progression or lack of progression of the hearing loss. When positive for a genetic basis, the family receives counseling regarding the recurrence risk in future pregnancies for the couple and also for the child when she/he is of child-bearing age.

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 congenital bilateral severe-profound sensorineural hearing loss of unknown etiology.

Specialty Collaborations & Other Services

Medical Genetics (see NV providers [5])

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 or confirm hereditary causes of hearing loss.

Genetic Testing and Counseling (see NV providers [12])

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

Pediatric Otolaryngology (ENT) (see NV providers [5])

Performs a comprehensive evaluation of the head and neck and can help in the identification of craniofacial malformations and middle ear pathology (e.g. serous otitis media) 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 of a tympanic membrane perforation or aural atresia, cochlear implantation, bone-anchored hearing aids).

Audiology (see NV providers [8])

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

Pediatric Ophthalmology (see NV providers [6])

Every child with confirmed hearing loss without an etiologic diagnosis should undergo annual evaluation by an ophthalmologist to determine visual acuity and evaluate for concomitant vision disorders (e.g., Usher Syndrome). All children with hearing loss should have at least one evaluation to assure optimal vision.

Speech - Language Pathologists (see NV providers [11])

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

Developmental Assessments (see NV providers [5])

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 NV providers [30])

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

Special Education/Schools (see NV providers [9])

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

Treatment & Management

Pearls & Alerts for Treatment & Management

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

photo of infant wearing Hearing aid — Child with hearing aid

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 loss, and family choice. Additional information about most of the following can be found by clicking on the name link.

different models of behind the ear 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.
Cochlear implants have several internal and external components including an electrode array implanted into the cochlea, a receiver and magnet set into the bone behind the ear, a transmitter coil and a microphone worn behind the ear, and a speech processor carried in a pocket or fanny pack. The microphone receives speech and an electrical signal is sent to the speech processor through a connecting cable. The speech processor converts the electrical signal into a code that has been optimized for speech recognition. This code is then sent back over the cable to the headpiece and transmitted via radio waves to the implanted receiver. The code is then passed to the electrode array that stimulates the afferent auditory neurons within the cochlea. The integrity of the cochlear implant is tested at the time of surgery, immediately after surgery, and during routine follow-up visits. In addition to assuring parents return for ongoing audiologic testing and adjustment, the primary care physician should closely monitor and inquire about the child's language development and school performance during routine well child checks and follow-up appointments.
Bacterial Meningitis Prevention
Children with cochlear implants are at greater risk for bacterial meningitis. This risk can be higher with those with cochleovestibular dysplasias. Streptococcus pneumoniae (pneumococcus) is the major cause of bacterial meningitis in people with cochlear implants. With the widespread use of pneumococcal conjugate vaccine (Prevnar® or "PCV13") and the pneumococcal polysaccharide vaccine (Pneumovax® 23 or "PPSV23"), the incidence has decreased dramatically. To reduce the risk of bacterial meningitis, the Centers for Disease Control and Prevention recommend the following vaccinations for individuals with cochlear implants. Please look at the CDC site for recommendations and additional information on vaccination.13-valent pneumococcal conjugate (PCV13) (Prevnar 13®) 23-valent pneumococcal polysaccharide (PPSV23) (Pneumovax®) Haemophilus influenzae type b conjugate (Hib) (ActHIB®, Hiberix®, PedvaxHIB®, and Pentacel®) Meningococcal conjugate (Menactra® and Menveo®) Serogroup B meningococcal (Bexsero® and Trumenba®) There is no evidence that people with cochlear implants are more likely to get meningococcal meningitis (caused by Neisseria meningitidis) than people without cochlear implants.Frequency modulation (FM) auditory trainers use a speaker-worn microphone to transmit amplified speech to a receiver attached to the patient's hearing aid.
Missing issue with id: 3c1f2353.xml 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 NV providers [8])

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

Pediatric Otolaryngology (ENT) (see NV providers [5])

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.

Early Intervention for Children with Disabilities/Delays (see NV providers [30])

For developmental assessment and interventions with the family and child to optimize development and functional outcomes.

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 NV providers [30])

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 NV providers [11])

Evaluate and form a treatment plan for infants and children with language delay associated with hearing loss. 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 regularly through school age to identify and treat hearing loss in a timely manner.
Research supports antiviral 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 every 3 month hearing screens starting at birth through age 3, then every 6 months until age 6, then annually afterwards.
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 prenatal education and hygiene measures has been a hot topic in recent years. Interestingly, the Committee on Practice Bulletins under the American College of Obstetricians and Gynecologists state that counseling women about prevention of CMV would be difficult to implement because they are impractical or burdensome and that patient instructions are an unproven method to reduce the risk for transmission. [[No: 2015] For more information, see Congenital Cytomegalovirus (CMV) Related Hearing Loss.

Specialty Collaborations & Other Services

Pediatric Infectious Disease (see NV providers [2])

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 NV providers [8])

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

Special Education/Schools (see NV providers [9])

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

Early Intervention for Children with Disabilities/Delays (see NV providers [30])

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

No Related Issues were found for this diagnosis.

Ask the Specialist

What if my patient has not had newborn screening?

Resources for Clinicians

On the Web

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

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 EHDI program and screening guidelines and helps to ensure that newborn screening results are communicated to families and reported according to state laws. Also has links to state chapters, EHDI experts, and resources; American Academy of Pediatrics and the Early Hearing Detection and Intervention Program.

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

Hearing Loss in Children (CDC)
Information, statistics, screening/diagnosis, and treatments; from the Centers for Disease Control and Prevention.

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

Lieu JEC, Kenna M, Anne S, Davidson L.
Hearing Loss in Children: A Review.
JAMA. 2020;324(21):2195-2205. PubMed abstract

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.

Dietary Supplements and Nutraceuticals for Children with Migraines ( PDF Document 327 KB)
A summarized list of supplements and suggested dosing recommended by American Headache Society; Texas Childrens.

Resources for Patients & Families

Hearing Loss and Deafness (FAQ)

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.

Types of Hearing Loss (My Baby's Hearing)
Answers to questions about the causes of genetic and non-genetic hearing loss in children; sponsored by Boystown National Research Hospital.

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, statistics, screening/diagnosis, and treatments; from the Centers for Disease Control and 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 loss, including hearing aids, audiology, speech and hearing therapy, or referral to a specialist. Includes general and state-specific resources.

Family Resources (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 Family-to-Family Health Information Center by state.

National Center for Hearing Assessment and Management (NCHAM)
Extensive compilation of resources and support for families with a child who is deaf or hard of hearing.

Studies/Registries

Childhood Hearing Loss (ClinicalTrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.

Otitis Media with Effusion (ClinicalTrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.

Cochlear Implants and Children (ClinicalTrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.

Auditory Brainstem Implants in Children and Adolescents (ClinicalTrial.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.

Services for Patients & Families in Nevada (NV)

Service Categories	NV	NW		NM	RI
Audiology	8	3	22	24	22
Developmental Assessments	5	1	105	35	54
Early Intervention for Children with Disabilities/Delays	30	3	34	13	51
Genetic Testing and Counseling	12	6	6	8	11
Medical Genetics	5	1	2	4	7
Pediatric Infectious Disease	2		2	4	1
Pediatric Ophthalmology	6	1	6	8	4
Pediatric Otolaryngology (ENT)	5	1	11	7	10
Special Education/Schools	9	3	82	35	43
Speech - Language Pathologists	11	4	23	33	65

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: November 2013; last update/revision: September 2021

Current Authors and Reviewers:

Senior Author:	Albert H. Park, MD
Reviewer:	John C. Carey, MD

Authoring history

2019: update: Janet W. Lee, MD^A; Albert H. Park, MD^SA

2018: update: Jennifer Goldman, MD, MRP, FAAP^R

2013: first version: Jennifer Goldman, MD, MRP, FAAP^A; Terry E. Foust, AuD, CCC-SLP^A; Catherine Jolma, MD^CA; William Kimberling, Ph.D.^CA; Richard Harward, AuD^R; Albert H. Park, MD^A; John C. Carey, MD^R

^AAuthor; ^CAContributing Author; ^SASenior Author; ^RReviewer

Bibliography

[No authors listed].
Practice bulletin no. 151: Cytomegalovirus, parvovirus B19, varicella zoster, and toxoplasmosis in pregnancy.
Obstet Gynecol. 2015;125(6):1510-1525. PubMed abstract

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

Boppana SB, Ross SA, Novak Z, Shimamura M, Tolan RW Jr, Palmer AL, Ahmed A, Michaels MG, Sánchez PJ, Bernstein DI, Britt WJ, Fowler KB.
Dried blood spot real-time polymerase chain reaction assays to screen newborns for congenital cytomegalovirus infection.
JAMA. 2010;303(14):1375-82. PubMed abstract / Full Text

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

Clark JG.
Uses and abuses of hearing loss classification.
ASHA. 1981;23(7):493-500. 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

Dollard SC, Dreon M, Hernandez-Alvarado N, Amin MM, Wong P, Lanzieri TM, Osterholm EA, Sidebottom A, Rosendahl S, McCann MT, Schleiss MR.
Sensitivity of Dried Blood Spot Testing for Detection of Congenital Cytomegalovirus Infection.
JAMA Pediatr. 2021;175(3):e205441. PubMed abstract / Full Text

Downie L, Amor DJ, Halliday J, Lewis S, Martyn M, Goranitis I.
Exome Sequencing for Isolated Congenital Hearing Loss: A Cost-Effectiveness Analysis.
Laryngoscope. 2021;131(7):E2371-E2377. 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

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

Lieu JEC, Kenna M, Anne S, Davidson L.
Hearing Loss in Children: A Review.
JAMA. 2020;324(21):2195-2205. 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

Mehta D, Noon SE, Schwartz E, Wilkens A, Bedoukian EC, Scarano I, Crenshaw EB 3rd, Krantz ID.
Outcomes of evaluation and testing of 660 individuals with hearing loss in a pediatric genetics of hearing loss clinic.
Am J Med Genet A. 2016;170(10):2523-30. PubMed abstract

Mets MB, Young NM, Pass A, Lasky JB.
Early diagnosis of Usher syndrome in children.
Trans Am Ophthalmol Soc. 2000;98:237-42; discussion 243-5. PubMed abstract / Full Text

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

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