Hearing Loss and Deafness

Description

"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 the mother of a boy with a late diagnosis of hearing impairment, talking about the misperception that any hearing is the same as normal hearing.

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.

Other Names

Deafness, Hearing Impairment

Diagnosis Coding

ICD-10

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 and xx represent required additional specificity and laterality. See ICD10Data.com for more information and additional codes.

Description

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 the hearing apparatus (Virtualmedicalcentre.com).

Sound waves are captured by the pinna and directed through the external auditory canal resulting in vibration of the tympanic membrane. Tympanic membrane vibrations are transferred through the ossicular chain (commonly called the hammer, anvil, and stirrup) 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 it to effectively conduct 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 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, related to decibels below which the child can hear or discriminate sounds – see Clinical Classification under Hearing Loss and Deafness, Clinical Assessment.

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 a speech message to render it inadequate for accurate interpretation or to interfere with learning. However, many professionals often reserve the term ‘deafness’ to describe a severe to profound hearing loss.

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 being 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 because of the impact of a genetic diagnosis on 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. Also, connexin 26 patients have done 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 in families with more than one affected child in a sibship. Tests for the common 35delG mutation (single nucleotide deletion at site 35) and complete Cx26 gene sequencing are available on a clinical basis and should be offered to families affected by recessive nonsyndromic hearing loss of unkown cause. 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 prognosis 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 is 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 indicates 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]

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)

Practice Guidelines

The following guideline is an update of the 2000 Joint Committee on Infant Hearing statement on early hearing loss detection and intervention.

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

Helpful Articles

PubMed search for deafness or hearing loss 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 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) is 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 Hearing Loss and Deafness, Clinical Assessment, 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 our Newborn Disorders page on 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. Hearing loss may, however, occur 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.

Screening

Though most cases of permanent sensorineural hearing loss are detected thru universal hearing screening in the newborn nursery, hearing loss may begin after the perinatal period (late-onset) or be progressive in nature.

For The Condition

The American Academy of Pediatrics recommends subjective assessment of hearing (and assessment of risk for hearing loss) at all well-child examinations and objective hearing screening at school entry. Bright Futures Periodicity Schedule (AAP) (PDF Document 119 KB) Developmental abnormalities, poor cognitive function, or behavioral problems (e.g., autism/developmental delay) may preclude accurate results on routine audiometric screening; referral to an otolaryngologist and a pediatric audiologist who has the necessary 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 any of the following regardless of length of stay: ECMO, assisted ventilation, exposure to ototoxic medications (gentamicin and tobramycin) or loop diuretics (furosemide), and hyperbilirubinemia that requires exchange transfusion
  • Intrauterine infections such as cytomegalovirus, herpes, rubella, syphilis, and toxoplasmosis
  • 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, siblings should have an audiology assessment by 24 to 30 months of age or sooner. Thereafter, routine subjective assessment of screening 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 is 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 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 the following symptoms:
  • 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

Hearing loss can be categorized as:
  • Sensorineural hearing loss (SNHL) results from disorders of the cochlea, usually involving the hair cells or the auditory nerve.
  • Conductive hearing loss 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.
  • Fluctuating and progressive hearing loss
  • The severity of hearing loss is commonly divided into four levels:
    • mild, 20 to 40 dB
    • moderate, 41-70 dB
    • severe, 71-90 dB
    • profound, >90 dB
Hearing loss can be unilateral vs. bilateral, symmetrical vs. asymmetrical, or progressive vs. sudden; the latter should prompt immediate clinical assessment.
Determination of cause of hearing loss and, if genetic, whether it is associated with a syndrome offers additional categorization. Genetic diagnosis/classification is critical to the assessment of risk, guiding intervention(s), and informing genetic counseling. It can also help clinicians avoid unnecessary medical procedures. See the discussion of Genetics under Hearing Loss and Deafness, Description.

Differential Diagnosis

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

Medical Conditions Causing Hearing Loss and Deafness

A number of medical conditions may cause hearing loss in children, the most common being conductive loss due to middle ear effusion, which may persist long enough to cause secondary impact on hearing and language development. List of Causes of Hearing Loss with ICD-9 Codes offers an extensive list of conditions that may cause hearing loss. A compilation of conditions causing hearing loss can also 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:

Pearls & Alerts

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 (greater than 5 days) are at increased risk for auditory neuropathy

This 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.

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.

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 families with recessive hearing loss have no previously known affected family members.

Pregnancy Or 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

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 acute life-threatening event (ALTE)
  • visual impairment, especially retinitis pigmentosa (Usher syndrome)
  • chemotherapy

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; see Roles of the Medical Home under Hearing Loss and Deafness, Description.

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

Assess for anatomic craniofacial abnormalities such as submucous cleft palate, preauricular tags/pits, 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. Between 2 and 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. This amplification produces pressure fluctuations within the cochlea that are then transmitted out through the middle ear and can be recorded as sounds called otoacoustic emissions (OAE). These reflect the functional integrity of the inner ear and are used to assess cochlear function. Because OAE are preneural in origin, they 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 influence OAE results. The OAE gives a Pass or Fail (Refer) result; the latter indicates a need for further quantification of hearing function. A screening OAE gives a Pass or Fail (Refer) result; the latter indicates a need for further quantification of hearing assessment. Diagnostic testing utilizing OAEs tests and analyzes responses in further detail that automated screening and is interpreted by the audiologist.
  • Auditory Brainstem Evoked Responses (AABR or BAER): 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). Auditory brainstem response 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 screening or automated Auditory Brainstem Evoked Response (AABR) uses a statistical algorithm to determine the likelihood that the results reflect normal hearing. AABR tests used for newborn screening generate a Pass or Fail result; the latter indicates a need for further testing/quantification of hearing. An audiologist with infant/pediatric expertise will perform a frequency-specific, diagnostic ABR, which generally requires sedation in infants over 3-6 months (in some centers, all infants requiring diagnostic ABR are sedated to avoid the potential need for follow-up tests).
  • Behavioral Audiometry: Sound stimuli (new or interesting sounds) are used to elicit a behavioral response in infants and children. These may be obvious responses, such as the turn of a child's head toward the source of a sound, or subtle, such as the cessation or initiation of sucking activity in an infant. Observation of these physical responses by a qualified and experienced pediatric audiologist is the basis of behavioral audiometry. A number of age-appropriate behavioral tests have been developed, some of which use reinforcements to encourage observable responses to sound. This type of testing can be used starting in infancy and is particularly helpful in evaluating children who are too young to perform conventional audiometry.
  • Conventional Audiometry: In 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 (middle ear testing/tympanometry) is not used for newborn screening and does not test hearing. Rather, it 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 to manage the air pressure 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; 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] For sensorineural hearing loss detected in the newborn period, consider obtaining a culture of saliva or urine for cytomegalovirus. Congenital CMV is a common cause of SNHL and antiviral treatment with valganciclovir may prevent progression of hearing impairment. The risks and benefits of antiviral therapy are evolving. CMV testing should be obtained prior to 3 weeks of age as those performed later cannot differentiate between congenital and postnatal infection. Postnatal infection is not associated with hearing loss. For more information, see CMV and Congenital Hearing Loss (UDOH) (PDF Document 648 KB) and Congenital Cytomegalovirus (CMV)-Related Hearing Loss. 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.

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.

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 ECG should be considered in all infants with severe-profound sensorineural hearing loss of unknown etiology.

Subspecialist Collaborations & Other Resources

Pediatric Genetics (see Services below for relevant 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 relevant providers)

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

Pediatric Otolaryngology (see Services below for relevant 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 relevant 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 relevant 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 Therapy (see Services below for relevant providers)

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

Developmental Evaluation (see Services below for relevant providers)

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

Early Intervention Programs (see Services below for relevant providers)

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

Schools for the Deaf & Blind (see Services below for relevant 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

Impact of otitis media with effusion

Children with underlying sensorineural or permanent conductive hearing loss may be more susceptible to further hearing impairment or speech and language delay from otitis media with effusion (OME). While watchfully waiting, be reasonable when OME affects a child with underlying normal hearing and speech. Children with underlying permanent hearing loss should be referred for audiologic assessment and otolaryngology consultation for middle ear effusions persisting 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. See Ears/Hearing under Hearing Loss and Deafness, Treatment & Management for details.

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), appear to exhibit more behavioral problems in school. For these reasons, there has been a greater motivation to amplify the affected ear, if possible.

Systems

Ears/Hearing

Evaluation and Treatment/Management

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.

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. The Children's Hearing Aid Program (UDOH) provides hearing aids for qualifying children in Utah.

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.

Children with unilateral hearing loss are at risk for academic failure, may experience considerable difficulty in understanding speech in a background of noise, experience trouble with sound localization (with resulting safety issues), and appear to exhibit more behavioral problems in school. Despite these challenges, clinical practice in terms of intervention for this population is variable. [Fitzpatrick: 2010]

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]

Hearing aid, infant
assorted hearing aids
Infant with a hearing aid Assorted pediatric hearing aids

Subspecialist Collaborations & Other Resources

Audiology (see Services below for relevant providers)

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

Pediatric Otolaryngology (see Services below for relevant 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]

Subspecialist Collaborations & Other Resources

Early Intervention Programs (see Services below for relevant 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 Therapy (see Services below for relevant 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).

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,

Subspecialist Collaborations & Other Resources

Audiology (see Services below for relevant providers)

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

Schools for the Deaf & Blind (see Services below for relevant 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 Programs (see Services below for relevant providers)

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

Frequently Asked Questions

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.


For an extensive list of questions and answers, see
Communication Services and Supports FAQs (ASHA)
Extensive list of questions and answers related to hearing loss and deafness, finding and funding services, and helping children who are deaf or hard of hearing; American Speech-Language-Hearing Association.


What does a "false positive" result on a newborn hearing screening mean?

A "false positive" result on newborn hearing screening is when hearing is normal but the hearing test indicates differently. This may be due to birthing debris in the external auditory canal or transient fluid in the middle ears. Hearing screening tests do not diagnose hearing loss in infants; rather, they are designed to identify all infants who may have hearing loss. In the United States, between 1 to 10 percent of babies do not pass the screening test. Of those, less than 1 percent actually have hearing loss - so, most of the babies referred for diagnostic testing will be shown to have no hearing loss. Because early intervention is critical for infants with hearing loss, it is important for all infants who fail the initial hearing screen to have a complete audiologic evaluation.

What is "minimal hearing loss"?

Normal hearing for children is 15 dB HL or better at all frequencies with normal middle ear function; mild hearing loss is typically considered to start at 20 dB HL. Most people define a minimal or slight hearing impairment as one that occurs from 16 dB HL to 25 dB HL. Minimal hearing loss does put a child at risk for school failure and may require classroom accommodation such as preferential seating and frequency-modulation (FM) systems. Classroom Management for Children with Minimal Hearing Loss (Toronto Hearing Services).

How will we decide what type of hearing support to choose for our child?

A wide range of technology options is available for amplification. Technology selection is individualized, based on factors such as age, level of hearing, and the needs of the child and family. An audiologist and an otolaryngologist with expertise in childhood hearing loss will assist you in the decision making process.

What is a cochlear implant?

Cochlear implants may help some children who are deaf or hard of hearing who do not benefit from hearing instruments or FM systems. A wire containing electrodes is surgically placed into the cochlea (the organ of hearing). Sound is picked up by a receiver which is implanted behind the ear in the mastoid bone. An externally worn receiver sends signals to the electrodes and this creates a sensation of sound. See Cochlear Implantation.

What is an audiogram?

An audiogram is a graphic display of the results of a hearing test. The audiologist completes the audiogram by recording the level of hearing at different frequencies (e.g., low and high pitch sounds). This enables the physician / audiologist to understand the level and type of a hearing loss and advise on the appropriate treatment options. Regular testing also allows the hearing levels to be monitored over time. See Hearing Testing and What is an Audiogram? (baby hearing.org).

Issues Related to Hearing Loss and Deafness

Resources

Information for Clinicians

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

Early Intervention Deaf/Heard of Hearing Resource (CENTe-R)
Web-based training that supports graduate-level professionals serving families with infants and toddlers who are deaf/hard of hearing; Collaborative Early Intervention National Training e-Resource.

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)
Overview of the Early Hearing Detection and Intervention (EHDI) program, including information about EHDI programs, fact sheets, resources and tools, and payment 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.

Genetics in Primary Care Institute (AAP)
The goal of this site is to increase collaboration in the care of children with known or suspected genetic disorders. Includes health supervision guidelines and other useful resources; a collaboration among the Health Resources & Services Administration, the Maternal and Child Health Bureau, and the American Academy of Pediatrics.

Helpful Articles

PubMed search for deafness or hearing loss 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

Algorithms/Care Processes

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.

Information & Support for Families

Family Diagnosis Page

Information on the Web

Parents' Guide to Hearing Loss (CDC)
Comprehensive section on hearing information for families, professionals, and programs. Resources and a glossary of related terms; Centers for Disease Control and Prevention.

Learning About Hearing Loss - A Roadmap for Families (NCHAM) (PDF Document 347 KB)
Graphic representation of the path to learning about hearing loss, from a positive newborn hearing screen to 6 months of age; National Center for Hearing Assessment and Management.

Learning about Hearing Loss - A Roadmap for Families (NCHAM) (Spanish) (PDF Document 287 KB)
Spanish language graphic representation of the path to learning about hearing loss, from a positive newborn hearing screen to 6 months of age; National Center for Hearing Assessment and Management.

Newborn Hearing Screening (My Baby's Hearing)
Information about specific aspects of newborn hearing screening; Boys Town National Research Hospital.

Glossary of Hearing Terms (My Baby's Hearing)
Definitions of more than 110 terms related to hearing and hearing loss; Boystown National Research Hospital.

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.

Nonsyndromic Deafness (Genetics Home Reference)
Wealth of information, and links to more information, about hearing impairments that are not associated with genetic syndromes; sponsored by the National Library of Medicine.

Hearing Loss, Genetics, and Your Child Brochure (ACMG) (PDF Document 1.5 MB)
Two-page brochure for families about children with hearing loss and determining a genetic cause; American College of Medical Genetics.

Hearing Loss, Genetics, and Your Child Brochure (ACMG) (Spanish) (PDF Document 1.4 MB)
Two-page brochure, in Spanish, with information for families about hearing loss in children and determining genetic causes; American College of Medical Genetics.

Hearing Problems in Children (MedlinePlus)
Overview and extensive links to vetted sites with more information; National Library of Medicine.

Trauma Treatment Needs of Deaf Children and the Hearing Children of Deaf Parents (PDF Document 430 KB)
Enhances opportunities for deaf and hard-of-hearing children who experience traumatic stress to receive treatment tailored to their individual, cultural, and communicative needs; National Child Traumatic Stress Network.

Alexander Graham Bell Association
One of the oldest and most comprehensive organizations focused on pediatric hearing loss. Educates the public about technological advances for the deaf and hearing impaired and advocates legislation.

Hearing Health Foundation
National organization whose goal is the cure and prevention of all forms of hearing loss.

Gallaudet University
The world's only university geared specifically to the needs of the deaf. Includes links to other deaf schools in the United States.

National Association of the Deaf
Advocates the civil rights of the deaf and hard-of-hearing in areas including education, employment, health care, social services, and telecommunications.

National Theatre of the Deaf
Information about upcoming performances, workshops, and classroom visits.

World Federation of the Deaf
International organization with goal of eradicating discrimination against the deaf community.

American Academy of Audiology
Resources for professionals who test, treat, and provide care to the 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 Sign Language Teachers Association
Provides ASL professionals with information on deaf culture, instructional methods, materials, and evaluation techniques.

Council on Education of the Deaf (CED)
Dedicated to the exchange of information about deaf education, from recommended teaching strategies to curriculum materials.

Gallaudet Research Institute
Research conducted by Gallaudet Research Institute, one of the world's premier deafness-related research centers, into deaf health care services, demographics, literacy, and more.

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

Handspeak.com
Online ASL dictionary, reportedly the largest on the Web, offers 3,090 animated signs, from "accounting" to "zen."

SignWriting
Award-winning site offers extensive information on how to read and write in signed languages.

Support National & Local

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.

Easter Seals
Nonprofit organization offering services for individuals with disabilities and their families. Primary services include medical rehabilitation, early intervention, physical and occupational therapy, speech and hearing therapy, child care, recreation, and transition.

Studies/Registries

The National Institutes of Health conduct many clinical trials and are often seeking study participants; below are links to lists of relevant studies.

Otitis Media with Effusion (clinicaltrials.org)
Clinical trials related to OME in children; National Institutes of Health.

Childhood Hearing Loss (clinicaltrials.gov)
Clinical trials involving hearing loss. Trials may be recruiting subjects - compensation offered in some cases; registered with the National Institutes of Health.

Cochlear Implants (clinicaltrials.gov)
Listing of clinical trials of which may be recruiting subjects; National Institutes of Health.

Services for Patients & Families

Audiology

See all Audiology services providers (69) in our database.

Developmental Evaluation

See all Developmental Evaluation services providers (55) in our database.

Early Intervention Programs

See all Early Intervention Programs services providers (52) in our database.

Pediatric Genetic Counseling

See all Pediatric Genetic Counseling services providers (5) in our database.

Pediatric Genetics

See all Pediatric Genetics services providers (5) in our database.

Pediatric Ophthalmology

See all Pediatric Ophthalmology services providers (8) in our database.

Pediatric Otolaryngology

See all Pediatric Otolaryngology services providers (9) in our database.

Schools for the Deaf & Blind

See all Schools for the Deaf & Blind services providers (12) in our database.

Speech/Language Therapy

See all Speech/Language Therapy services providers (80) in our database.

For other services related to this condition, browse our Services categories or search our database.

Authors

Reviewing Authors: Richard Harward, AuD - 11/2013
Albert H. Park, MD - 11/2013
John C. Carey, MD - 11/2013
Terry E. Foust, AuD, CCC-SLP - 11/2013
Catherine Jolma, MD - 11/2013
Content Last Updated: 12/2013

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Universal newborn hearing screenings: a three-year experience.
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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.
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Doyle KJ, Ray RM.
The otolaryngologist's role in management of hearing loss in infancy and childhood.
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The newborn with hearing loss: detection in the nursery.
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Fitzpatrick EM, Durieux-Smith A, Whittingham J.
Clinical Practice for Children with Mild Bilateral and Unilateral Hearing Loss.
Ear Hear. 2010. PubMed abstract

Gifford KA, Holmes MG, Bernstein HH.
Hearing loss in children.
Pediatr Rev. 2009;30(6):207-15; quiz 216. 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.
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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.
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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.
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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.
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Mafong DD, Shin EJ, Lalwani AK.
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The epidemiology of hearing impairment in the United States: newborns, children, and adolescents.
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Moeller MP.
Early intervention and language development in children who are deaf and hard of hearing.
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Moller A.
Hearing: Anatomy, Physiology, and Disorders of the Auditory System.
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Shearer AE, Smith RJ.
Genetics: advances in genetic testing for deafness.
Curr Opin Pediatr. 2012;24(6):679-86. PubMed abstract / Full Text

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