AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

Hearing loss is more prevalent than diabetes mellitus, myelomeningocele, all pediatric cancers, and numerous other medical conditions. However, medical professionals learn little about hearing impairment, about how to advise parents of children who are deaf or hard of hearing, or about the special considerations needed in the care of children with hearing loss.

Recommendations for universal neonatal hearing screening have resulted in numerous articles regarding the tests, the efficacy of testing, the role of the audiologist in amplification, and the importance of early intervention programs. The importance of the otolaryngologist treating the patient along with the primary care physician cannot be overemphasized. In many instances, the otolaryngologist develops a long-term relationship with patients and their families, caring for the patients through their spectrum of development.

Pediatricians play a crucial role in providing referrals to audiologists, otolaryngologists, and special programs. To do so, they must understand the nature of hearing loss and the equipment that can improve auditory reception, the linguistic and social development of children who have hearing impairment, and the educational and linguistic options available to children who are deaf or hard of hearing.

The goals must always be to integrate the child into the family and into society and to enable the growth and development of a healthy, confident child who is deaf or hard of hearing. To meet these goals, clinicians should use any communication strategy and equipment that is best suited for the individual child and his or her family.

Pathophysiology

Relevant anatomy and physiology

Sound waves arrive to the auricle and are channeled through the external auditory canal to the tympanic membrane. When they strike the tympanic membrane, the waves cause it to vibrate, setting off a chain of vibrations along the ossicles (malleus, incus, and stapes) to the membrane of the oval window at the entrance to the cochlea. This process amplifies the environment sound by approximately 20-fold.

The cochlea is the end organ of hearing and is shaped like a snail shell with 2.5 turns. Inside, 2 membranes longitudinally divide the cochlea into 3 sections: the scala tympani, the scala vestibuli, and the scala media. All 3 are filled with fluid of various ion concentrations (similar to intracellular and extracellular constituents).

Along one of the membranes in the scala media, or cochlear duct, lie the internal and external hair cells. Movement of the stapes on the oval window creates a wave or vibration in the perilymph fluid of the cochlea. This fluid movement, which opens ion channels in the hair cells, displaces the hair cells, triggering an action potential and causing a nerve in the cochlea to fire to the brain.

Thousands of nerves representing more than 20,000 frequencies are located along the length of the cochlea; these nerves account for the hearing range. The microscopic nerves culminate in the cochlear portion of the eighth cranial nerve. The location of the vibration in the cochlea is correlated with the frequency of the original pitch. Low-frequency sounds are near the apex, and high-frequency sounds are near the base.

Types of hearing loss

Conductive hearing loss (CHL) results from anything that prevents the transmission of sound from the outside world to the cochlea. Causes range from impaction of cerumen to middle-ear effusions or dysfunction or fixation of the ossicular chain. Otosclerosis is one of the most common examples.

An important cause of CHL is a cholesteatoma. Another cause is a locally destructive but benign growth. Other neoplasms can affect the middle ear as well. Examples include glomus tympanicum or glomus jugulare, schwannomas of the facial nerve, and hemangiomas. Dehiscence of the roof of the middle ear (tegmen mastoideum), such as is caused by an encephalocele, can result in CHL. In CHL, sounds perceived by the brain are diminished but are generally not distorted.

Sensorineural hearing loss (SNHL) may result from disruptions in transmission after the cochlea. These disruptions may be a result of hair cell destruction in the cochlea or damage to the eighth cranial nerve. Sounds perceived by the brain are both diminished and distorted. The degree of distortion is independent of the degree of hearing loss (eg, mild hearing loss but very poor speech discrimination is possible).

Auditory dyssynchrony should be considered in the setting of no auditory brainstem response (ABR), no middle-ear muscle response, normal otoacoustic emissions, or normal cochlear microphonics.

Mixed hearing loss has components of both CHL and SNHL.

Categories of hearing loss

Regardless of the type, the American National Standards Institute defines hearing loss in terms of decibels (dB) lost, as follows:

• Slight hearing loss - 16-25 dB lost
• Mild hearing loss - 26-40 dB lost
• Moderate hearing loss - 41-55 dB lost
• Severe hearing loss - 71-90 dB lost
• Profound - More than 90 dB lost

Frequency

United States

Hearing loss occurs in 10 per 1000 children in the United States. Roughly 1 in 1000 has profound hearing loss, and 3-5 per 1000 have mild-to-moderate hearing loss that may affect language acquisition unless hearing, language, or both are aided. Acquired hearing loss may add 10-20% to these numbers.

Data from the United States Census show that almost 3% of the population in the workforce reports having some hearing loss, including CHL, SNHL, or mixed loss.

The prevalence of hearing loss requiring intervention among graduates from neonatal intensive care unit (NICU) is 1-4%.

International

SNHL occurs in 9-27 per 1000 children worldwide.

Sex

No sex predilection is known. Some hereditary causes of deafness or acquired deafness may occur more frequently in one sex than the other. However, the overall prevalence of deafness is equal in male and female individuals.

Age

Most hearing loss in children is congenital or acquired perinatally. However, hearing loss may occur at any age. Approximately 10-20% of all cases of deafness are acquired postnatally, although some genetic causes of deafness result in hearing loss that begins during childhood or adolescence.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

General presentation and age at detection

The presentation of patients with hearing loss depends on its degree, the patient's age when the hearing loss begins, the threshold of suspicion by parents' and healthcare providers, and the presence of other identifiable risk factors.

Before neonatal hearing screening was routine, deafness was diagnosed at the mean age of 2.5 years. This age recently improved to a mean of 14 months. The goal is universal screening, in accordance with the Newborn and Infant Hearing Loss: Detection and Intervention policy statement from the American Academy of Pediatrics (AAP).¹

In almost two thirds of patients, parents are the first to suspect hearing loss. Pediatricians detect roughly 10% of cases, and other healthcare providers suspect it first in approximately 15% of patients. The mean time between the first suspicion of hearing loss and its diagnosis is 9 months.

High-risk criteria for hearing loss in neonates and infants

The dissemination of high-risk criteria for neonates and infants in 1990 did not notably alter the mean age at diagnosis. About 50% of children with SNHL do not meet any of the criteria listed, and only 10% of neonates have 1 or more of the high-risk criteria that prompt an evaluation. These rates are among the reasons cited for the need for universal neonatal hearing screening. The goals of such screening are to identify children who are deaf or hard of hearing and to start intervention by age 6 months.

High-risk criteria for neonates (birth to 28 d) are as follows:

• Family history of congenital or early SNHL
• Congenital infection known to be associated with SNHL
• Craniofacial anomalies
• Birth weight of more than 1500 g (<3.3 lb)
• Hyperbilirubinemia over the exchange level
• Exposure to ototoxic medications
• Bacterial meningitis
• Low Apgar scores at birth
• Prolonged mechanical ventilation
• Findings of a syndrome associated with SNHL

High-risk criteria for infants (29 d to 2 y) are as follows:

• Concern about hearing, speech, language, and/or developmental delay
• Bacterial meningitis
• Neonatal risk factors associated with SNHL
• Head trauma, especially with fracture of the temporal bone
• Findings of a syndrome associated with SNHL
• Exposure to ototoxic medications
• Neurodegenerative disorders
• Infectious diseases associated with SNHL

Indications for a hearing evaluation and findings

Concern about a child's hearing loss expressed by a parent or caregiver should prompt immediate evaluation. Hearing loss can now be diagnosed as soon as it is suspected. Delaying the diagnosis in the belief that a child is too young to be tested is unjustified. Little justification supports delaying the diagnosis while language delay or behavioral problems are ruled out, especially because hearing loss may be the cause of these problems.

Children with congenital or perinatally acquired profound SNHL (>90 dB) may present with loss of cooing by the age of 6-9 months and with frank language delay. Those with hearing loss less severe than this may present with minor speech impediments, language delay, behavioral problems, or school failure. The degree of hearing loss or loss of speech discrimination is correlated with the patient's speech and language problems.

Behavioral problems may be major or minor, and they are probably best correlated with the child's personality and with how the parents deal with what they may consider a stubborn child. Before speech impediments, mental retardation, autism, attention deficit, or adjustment disorders are presumed, many children could benefit from a hearing evaluation. 

Upon evaluation, a child who cannot normally hear speech can reproduce only what he or she hears. A child who cannot hear the teacher cannot learn. In addition, a child who is bored fidgets. A child who cannot understand and who is berated by caregivers for lack of cooperation may act out.

Healthcare providers must remember that normal speech volumes are 30-50 dB, whereas typical street traffic is about 60 dB. Standard phones ring and shouts register at approximately 80 dB, and lawnmowers are approximately 90 dB. Therefore, children with a 60-dB hearing loss hear their mother when she yells at them and startle when the telephone rings. However, they do not hear most conversational speech, and they may not always hear the teacher in a classroom.

If a child has poor speech discrimination, the diagnosis is usually made relatively early. If a child has good speech discrimination, hearing loss may go undetected.

Children with mild hearing losses that begin prelingually or postlingually may present with difficulties late in childhood.

In general, children who lose their hearing postlingually have a decline in language skills they previously achieved. In children of this age group, clinically significant hearing loss is usually the result of an obvious medical event. Those who lose their hearing postlingually may be able to describe their loss. However, if the decline is gradual, they may not recognize the deficit.

Worsening speech or school performance may herald long-standing or progressive mild-to-moderate hearing loss.

Physical

Carefully evaluate children with congenital hearing loss for any evidence of other physical stigmata or for possible associated syndromes (ie, a white forelock to suggest Waardenburg syndrome). For a partial list of syndromes related to deafness, see Causes. Because almost every organ system can potentially provide evidence of an associated syndrome, detailed physical examination is necessary.

For patients with acquired hearing loss an otolaryngologist should evaluate their ears by inspecting them for external defects or for obstructions that block sound conduction down the ear canal (eg, cerumen, foreign bodies). In addition, pneumatic otoscopy should be performed to detect any evidence of current or chronic infections, such as perforation or scarring of the tympanic membrane, cholesteatoma, abnormal landmarks, and fluid behind the tympanic membrane.

The remainder of the otolaryngology examination should be focused on the head and neck to carefully rule out other abnormalities that may lead to a diagnosis.

If children are old enough to cooperate, they should undergo tests of balance because dysfunction of the inner ear or vestibular nerve may also be present.

Causes

Genetic causes

Genetic causes account for 30-50% of hearing losses and can be divided into syndromic and nonsyndromic types. As with all genetic syndromes, genetic causes of hearing loss may be autosomal dominant (AD), autosomal recessive (AR), X-linked, or sporadic.

Nonsyndromic deafness accounts for slightly more than half of all cases of genetic deafness. It probably accounts for most cases classified as unknown. Children with nonsyndromic deafness are deaf or hard of hearing; however, they have no other physical abnormalities, no particular risk to other organ systems, and no increased risk of mental deficiency. Some children have a history of deafness in a close or distant family member. Others have new mutations or an AR gene with no known proband. The histories of subsequent siblings and progeny may help to distinguish a genetic cause from developmental arrest or a prenatal insult.

Exciting developments in genetic mapping have revealed approximately 2 dozen abnormal genes that lead to deafness. In some, the molecular or structural defect has been identified (eg, collagen in the basilar membrane, structural defect in a membrane-gating protein). These genes have been classified according to their mode of inheritance: ADs (DFNA1 through DFNA11), ARs(DFNB1 through DFNB12), X-linked recessives (DFN1 through DFN6), or mitochondrial (12Sr RNA and tRNA-Ser UCN). Some of the genes exhibit variable penetrance.

Syndromic deafness accounts for the other cases of genetic deafness. Some syndromes have a particular inheritance pattern (eg, AD for Gernet syndrome, AR for Winter syndrome, X linked for Rosenberg syndrome). Others are sporadic (eg, cat-eye syndrome, Turner syndrome, or Klinefelter syndrome). Physical findings usually indicate the presence of  a syndrome; however, children with some syndromes develop the associated physical findings late in childhood. Other children present with either deafness or the sequelae of a biochemical or metabolic derangement. As noted above, syndromes may affect any single organ or several organ systems.

Syndromic associations

A small sample of syndromes associated with deafness is summarized below. A few may be familiar, although many are not; most are fairly uncommon. For many of these syndromes, good data about their actual prevalences are difficult to find.

The first few syndromes listed for each each organ or system are most commonly known. They may be most widely recognized because their associated findings or illnesses may result in high morbidity or mortality rates, because the physical stigmata are classic and therefore make the syndrome easily identifiable, or because they are overrepresented in test questions on pediatric examinations.

Some Syndromes Associated with Deafness

Prenatal causes

Prenatal causes lead to 5-10% of hearing losses. Congenital infections (eg, cytomegaloviral [CMV] infections, herpes, rubella, syphilis, toxoplasmosis, varicella) can result in SNHL.² Fetal exposure to teratogens (eg, alcohol, cocaine, methyl mercury, thalidomide) may also result in SNHL. All of these perinatal insults result in physical abnormalities, which should prompt the clinician to recognize the diagnosis and perform a confirmatory evaluation. Even if these children pass the neonatal screen, careful follow-up of their hearing is necessary

Perinatal causes

Perinatal causes are responsible for 5-15% of hearing losses. A history of prematurity, low birth weight, anoxia and/or low Apgar scores, hyperbilirubinemia, or sepsis should prompt an evaluation of hearing because these conditions may also result in SNHL.

Postnatal causes

About 10-20% of hearing loses are due to postnatal causes. Childhood infections, such as meningitis or mumps, may result in SNHL. Treatment with ototoxic medications, such as aminoglycosides or furosemide, can lead to SNHL. Otitis media or major head injury may cause SNHL or CHL.

Unknown causes

About 20-30% of deaf children have no certain etiology. Their hearing losses likely result from a maldevelopment of the ear or neurologic system. Such an event may have been a developmental accident or the result of an undiagnosed infectious or exposure to a teratogenic agent. However, many are likely due to previously undiagnosed genetic defects that may represent new mutations or a genetic recessive trait.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

No differential diagnosis for deafness is known, although differential diagnoses are generally considered in determining the etiology of hearing impairment. Alternatively, the differential diagnosis for children who present with language, behavioral, and school difficulties should include hearing loss.

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• Depending on the patient's history and physical findings, biochemical evidence may help to determine the etiology of deafness if a genetic syndrome is suspected.
• Some have recommended that any child with a diagnosis of SNHL should immediately be evaluated for evidence of thyroid and renal disease.
• • Such an evaluation involves testing thyroid function, measuring BUN and creatinine levels, and urinalysis.
  • ECG may be useful in diagnosing an arrhythmia, leading to a diagnosis of Jervell Lange-Nielsen syndrome. In this situation, patients have a prolonged QT interval, with cardiac arrhythmias, recurrent syncopal episodes, and a predisposition to suddenly die.
• Connexin-26 is a marker for genetic deafness; therefore, a test for connexin-26 might be helpful.
• For patients with bilateral hearing loss, markers of general inflammatory disease (eg, erythrocyte sedimentation rate, rheumatoid factor) or specific markers for autoimmune inner ear disease (eg, 68-kDa protein) may be evaluated.
• The yield of positive findings is low; however, laboratory studies are safe and inexpensive. Positive findings raise important considerations in the management of hearing loss.

Imaging Studies

• In the past, the benefit of imaging studies was questioned. Although a positive finding on MRI or CT scanning may help to explain the defect, it does not lead to treatment options. However, some abnormalities uncovered during imaging (eg, enlarged vestibula aqueduct) may indicate a child with a sensitive ear in whom minor head trauma could worsen his or her hearing.
• CT scanning and MRI may help in identifying a malformation of the cochlea or the cochlear nerve. Such information may be critical when cochlear implants are being placed in profoundly deaf individuals. Recent work suggests the superiority of MRI in preoperative planning for candidates for cochlear implants.³

Other Tests

At this time, accurate testing of children of all ages is possible. Therefore, if any adult involved in the care of a child suspects the possibility of hearing loss, an immediate referral should be made for appropriate diagnostic evaluation. Universal newborn screening does not rule out the possibility of a newly acquired hearing loss or a progressive loss that had been previously undiagnosed. ECGs can be used to detect a prolonged QT. Some have recommended that all children with SNHL undergo ECG soon after diagnosis unless a clear deafness-related syndrome is identified. Chromosomal studies may be of benefit in seeking particular genetic syndromes. The syndrome determines whether simple chromosomal analysis is sufficient or whether detailed studies are needed for the patient and, sometimes, the parents as well. 

Specific Tests for Hearing Loss

Prior to reviewing specific test results for hearing loss, examining the Joint Committee on Infant Hearing (JCIH) updated position statement is crucial.⁴ Specifically, the JCIH recommends hearing screening in all infants by age 1 month; those who fail the initial test should have a thorough audiologic evaluation by age 3 months, with appropriate intervention by age 6 months. In this update, the JCIH also included auditory neuropathy and dyssynchrony in the category of neural hearing loss.⁵ Recommendations were also made regarding babies who remain in the NICU for longer than 5 days. These patients should undergo ABR testing.

Specific tests for hearing loss include ABR (formally called the brainstem audio-evoked response [BAER] or automated ABR), otoacoustic emissions (OAEs), and audiometry.

The BAER is occasionally referred to as an ABR when it means audio-evoked brainstem response; in this case, the ABR is then called AABR for automated audio-evoked brainstem response. (BAER and ABR, the most common and least confusing abbreviations, are used in this article.)

ABR and BAER testing

ABR testing is based the same principle as electroencephalography (EEG). When a hearing ear is given a stimulus, the resulting electrographic activity can be followed from the ear to central areas of the brain. In the formal testing procedure for BAER, clicks or specific frequencies at different volumes can be the stimuli. CHL cannot be distinguished from SNHL. The sensitivity and specificity of this testing are near 100%. BAER tests frequently require sedation, and they take time and are expensive. Abnormal brain-wave activity (eg, seizure activity, significant prematurity) can render the results uninterpretable.

Use of the automated testing procedure for ABR has been recommended for universal newborn hearing screening. Sound clicks are presented to each ear, and 2 electrodes placed on the scalp record brain-wave activity. An internal template of what the waveforms should resemble is used to determine if the baby passes the test (waveforms match) or not (waveforms do not match). People with relatively little training can perform the ABR test quickly and inexpensively. It has a sensitivity and specificity of about 100% and 96%, respectively.

Because ABR reflects only nerve impulses that reach the brain, it cannot be used to distinguish CHL from SNHL. In neonatal screening, the false-positive rate is 10-15% because amniotic fluid and cellular debris are retained in the neonate's ear canal. However, on subsequent testing, the test performs as well as it should. This repeat testing is often completed before discharge from the nursery, but it is optimally performed after the fluid clears (in about 1 wk).

As with the BAER, prematurity or seizure disorders may cause failing results on ABR testing because the abnormal brain-wave activity does not match the machine's internal template for passing results. In this case, formal BAER testing may be necessary because the important waves might be distinguishable from the background abnormalities. Use of the OAE is a reasonable alternative because does not depend on brain waves.

OAE testing

The concept of OAE is that certain sounds generated by the inner ear can be recorded. These sounds are present in ears that can hear and likely reflect the presence and function of structures responsible for hearing. The sounds may be spontaneous or evoked. How they are produced and why they are not produced in people with SNHL is unclear, but they are well correlated with hearing loss. Also used for newborn screening, OAE tests can be performed quickly and inexpensively by personnel with relatively little training. An earphone is placed over the ear of a resting neonate, and the machine produces and records the evoked response. The sensitivity and specificity reported with evoked OAE are 100% and 82%, respectively.

By definition, OAE cannot be used to diagnose retrocochlear deafness nor can it be used to distinguish CHL from SNHL. OAE had slightly elevated false-positive rates in most studies of neonatal hearing screening probably because a sound must pass in and out of the obstructed canal to be recorded. OAE also seems to have a high failure rate when it is used in the NICU. Follow-up OAE or ABR testing can be performed before the patient is discharge from the nursery, though it is optimally performed a week later (after fluid and debris clear).

Audiometry

Routine audiometry can be performed by placing headsets over the ears of children whose developmental age is at least 4-5 years and who can be instructed to raise the corresponding hand when a sound is heard. Pure tone sounds can be presented so that specific volumes at specific frequencies can be documented. CHL and SNHL can be differentiated, and speech recognition can also be tested. The only limits to the sensitivity and specificity of the test are the patient's ability to understand the instructions and his or her willingness to cooperate.

Pure-tone audiometry can be performed as a quick and easy screening test. It has proven to be an effective tool in schools. The disadvantages of pure-tone audiometry are that formal evaluation takes time and considerable equipment and that it can be fully performed only in older, cooperative patients.

Behavioral (visual reinforcement) and conditioned play audiometry can be completed in children as young as 6 months. Children can be conditioned to look at a puppet or a light show when a pure sound stimulus is presented or their name is called from one side of the room. If the evaluators are reliable, they can judge whether the child is cooperative and responding to cues other than the sound stimulus. In general, this test is fairly successful for identifying hearing loss in children. Disadvantages are that it requires considerable time and equipment, it cannot be used to distinguish CHL from SNHL, and it succeeds only if the child is cooperating.

Tests to Avoid

Avoid some tests. Assessing responses to clapping, rattling keys, and snapping are poor tests of hearing. A child may respond to visual or tactile stimulation (eg, the slight breeze from a clap, accidental touching of the face with keys or fingers) rather than to the noise. The noise created is frequently more than 50 dB and, therefore, not useful in detecting mild and moderate losses.

A few companies market a small wand that produces white noise or a click at fixed or variable volumes. These wands have limited utility in rapid screening done in the office. However, if the time is taken to use them properly, they may provide some useful information.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical Care

Treatment for CHL

Manage CHL due to otitis media or its sequelae with a course of appropriate antibiotics. A patient with a serous otitis media for longer than 3 months benefit from myringotomy and removal of the fluid in the middle ear. Ventilation tubes may ultimately be necessary. If the hearing loss continues, amplification with a hearing aid may be needed. Speech therapy is rarely necessary unless the loss is prolonged and cannot be corrected with amplification.

CHL that results from obstruction of the auditory canal because of cerumen or a foreign body should be treated by removing the obstruction.

Treatment for SNHL

SNHL cannot be medically treated.⁶ Amplification with hearing aids is used to give the child as much auditory input as possible. Speech therapy may be beneficial. If the child requires special schooling, the program determines how much speech training is routinely part of the school day. Preferential seating and use of FM systems should be discussed with the patient's family and teachers.

In older children and in adults, goals for amplification may be as much as 40-60 dB or whatever achieves a nominal hearing level. The limiting factor is the physical sound pressure exerted on the tympanic membrane, which becomes painful after a certain threshold. Young children with small ear canals perceive pain at amplification volumes as low as 10-15 dB. Modern hearing aids can selectively amplify a specified range of frequencies more than others rather than all frequencies equally.

After the hearing aid is fitted by using proper molds, the hearing aid is tested to see how well it matches the goals for loudness at various frequencies. With an older child, speech recognition can be part of this testing. For a young child, the most important goal is to optimize auditory input without causing pain, which can cause the child to avoid using the hearing aid.

Young children should use their hearing aids because the stimulus helps to connect them to their environment and because it maximizes auditory language development. Older children may choose not to use their hearing aids. Parents should be reasonable. For example, if their child is succeeding in school, the hearing aids may not offer a substantial language benefit. If the child prefer not to wear the hearing aids after school, parents should respect this decision.

No medical disadvantage occurs if children choose to not use hearing aids. In fact, many deaf adults use their hearing aids selectively or not at all because they find that the extraneous noises and distortions they hear are more bothersome than helpful. They may use their hearing aids only when they anticipate a particular benefit.

Surgical Care

Some causes of CHL may be managed or aided surgically.

Children with persistent chronic or recurrent otitis media with resultant effusions may benefit from the placement of myringotomy tubes to ventilate the middle-ear space to prevent negative pressure in this area. If otitis results in the destruction or fixation of the ossicles, surgery may improve ossicular function.

Cholesteatoma is a surgical disease.

Bone-anchored hearing aids (BAHAs) may be useful in some patients. Examples are patients with microtia, those with anotia who are awaiting auricular reconstruction, and patients with persistent otorrhea who cannot use a hearing aid.

SNHL cannot be treated with surgical means other than cochlear implantation. Cochlear implantation may be an option in some children, but it should not be mistaken for a cure. Cochlear implants are discussed in the Cochlear implants section below.

Consultations

• Otolaryngologist: Consulting an otolaryngologist is imperative if the child has CHL. An otolaryngologist can provide advice pertaining to medical and potential surgical interventions. Consultation is also recommended if the child has profound SNHL and is a potential candidate for cochlear implants. The otolaryngologist is a crucial member of the multidisciplinary team needed to help patients with profound SNHL.
• Specialists in early intervention: Early intervention programs are essential to help parents understand how to raise a deaf child or one hard of hearing. Such programs are also needed to begin discussing and implementing language and/or educational programs.
• Audiologist: Consulting an audiologist is essential for evaluating patients for hearing aids and for fitting them.
• Geneticist: Consultation with a geneticist is recommended if the cause of deafness may be syndromic or if the family history suggests a hereditary pattern.
• Ophthalmologist, nephrologist, cardiologist: Consulting these subspecialists is recommended if an identifiable syndrome implicates involvement of the visual, renal, or cardiac organ system or if involvement of an organ system is suspected because a particular syndrome is or may be present.
• Experts in managing hearing loss: Consult physicians with expertise in caring for patients with hearing loss. Some physicians have developed expertise in the field of deafness and may be available for consultation. They can offer information about associated medical conditions if present, as well as perspective about language and education, use of  hearing aids and cochlear implants, and other equipment. The literature is filled with debates about the most appropriate venues for children who are deaf or hard of hearing. Many people involved in early intervention are affiliated with a particular program because it matches their personal biases. Physicians with expertise in deafness may be neutral.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

No medical therapy is specifically available for deafness. Only some etiologies of CHL may be managed medically.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Outpatient Care

• Follow-up of the interventions is as important in hearing impairment as in any other disability or medical condition. Physicians too frequently relegate the care of children who are deaf to audiologists and educators. Children who are deaf need ongoing referrals to an audiologist to monitor the progression of their hearing loss and to refit hearing aids to match changing losses and/or growth of their ears.
• Pediatricians should monitor the child's linguistic and social development. They should ask about language and school performance. The child's placement in school may not be optimal for his or her abilities. For instance, a child who is not successfully learning lip-reading cannot learn math or science. Pediatricians should ask how the child is doing in school and in the family, how family members interact with the child, and how the parents discipline or instruct the child.
• Children who cannot communicate with those around them may be frustrated and, therefore, act out or display withdrawal behaviors. These behaviors may be misinterpreted as behavioral or psychological problems rather than reactions to the child's environment or situation.
• Children who are deaf or hard of hearing are at particular risk for abuse. Physical abuse may be inflicted by parents who are frustrated because their child is not acting as they expect. Also, children who perpetrators perceive as being unable to report misconduct are at high risk for sexual abuse. Furthermore, most children with hearing impairment have no physical disabilities and may be attractive to a potential perpetrator. Because deaf children may be socially isolated because of the communication barrier, they may be susceptible to individuals who give them special attention. Watch for physical signs of abuse or for behavioral manifestations of child abuse.

Patient Education

Assisting and Educating Parents

Remember, a parent's response to having a child with hearing impairment is generally the same as that of a parent whose child has another disability or chronic medical condition. Help parents to identify available resources (such as those listed at the end of this section), or refer them to a social worker or counselor who can provide specialized assistance.

The diagnosis of deafness may be delayed because infants may respond to many sounds and yet be unable to hear normal conversational speech to understand words that they hear. Speech discrimination cannot be tested in very young children or in children without language.

Parents must understand that deafness is not an all-or-nothing categorization and that hard of hearing is not defined by the hearing loss being in the middle ranges (eg, 60 dB).

The nature of the distorted sound in SNHL means that amplification of the deficit may not improve the child's language comprehension in any meaningful way. A radio signal that is mostly static is unintelligible no matter how loud it is.

Communication is the most important loss in deafness. Communication is necessary for socialization and integration into the family and into society. All interventions must have the goal of optimizing the child's ability to successfully communicate and interact. Most children who are deaf are otherwise generally healthy.

Lip-Reading (Oralism) and Sign Language

The debate over lip-reading versus sign language

Considerable debate continues regarding the choice of lip-reading (oralism) versus sign language. Lip-reading may be taught alone or supplemented with cued speech. Sign language can be in the form of American Sign Language (ASL) or Signed English (SE) with Signing Exact English/Seeing Essential English (SEE, which is sometimes distinguished as Signing Exact English [SEE 1] and Seeing Essential English [SEE 2]).

Parents must be able and willing to accept and then participate in the language that they chose for their child. They should be vigilant and remember that school failures and behavior problems are not inherent to deafness but frequently result from a frustrated child who has no language, no ability to communicate, and no ability to connect with others.

Advantages and disadvantages of lip-reading

The greatest advantage of lip-reading is that parents and society are required to make only minimal adjustments for the deaf individual.

The disadvantages are are numerous. First, formal training can begin only at school age. Therefore, language acquisition is delayed beyond the optimal neurobiologic window at approximately 3-5 years of age (though some evidence indicates it may be as young as 18 mo).

Second, almost half of the consonants appear similar when spoken (eg, d-t, f-v, g-k, b-p-m). That is, they look identical on the lips but are distinguishable to hearing people when spoken.

Third, lighting, distance, speech impediments, accents, and foreign objects or motion (eg, food, pencils, mustaches, turning the head) can make lip-reading difficult.

Fourth, lip-reading is hard to master and tiring to perform. To appreciate the difficulty, imagine reading this page with no spaces between the words and with only periods as punctuation. Then imagine the text passing in front of you as if on an electronic billboard.

Finally, typical lip-readers understand only one third of a conversation. The best lip-readers understand about two thirds. In general, children with more hearing and better speech discrimination than others are most successful with lip-reading.

Cued speech

Cued speech aids lip-reading because hand shapes are placed near the mouth. These shapes help in discriminating sounds that are difficult to distinguish by observing the lips alone.

Parents must learn how to cue. The technique is similar to shorthand in that as sounds, not letters, are cued. For example, the sh sound in fish, chard, and nation are all cued and in the same way.

Because cued speech is the language of neither the parents nor society, cued-speech interpreters may be required in situations such as interviews or public events. These interpreters are harder to locate than sign-language or oral interpreters.

Similar to instruction in lip-reading, instruction in cued speech cannot begin at an early age; therefore, language acquisition is delayed.

Systems of manual and visual sign language

In the United States, manual and visual signing systems include SEE and ASL.

SEE uses the signs of ASL but imposes a strict order of the spoken word on the signs. SEE invents prefixes, suffixes, conjugations, and signs that are not necessary in the grammar of ASL. SEE is long and tiresome. However, children who use SEE grow up signing what they learn to read and write, just as hearing children speak and hear what they later read and write. In realistic terms, most people end up omitting or changing some of the signs because SEE is so long. Therefore, children do not benefit from the English-grammar aspect of SEE as much as they might.

ASL has a unique grammar. It requires fewer signs than SEE does to complete most thoughts because it incorporates space and time into the motion of signs in a way that spoken language cannot. It is efficient and beautiful to watch. However, the child must grow up being bilingual. The grammar used for ASL must be translated into English in order to write.

An advantage of ASL or SEE is that instruction may begin immediately when hearing loss is diagnosed. In fact, children of parents who are deaf learn to sign as their first language, and they begin to sign babble as early as 6-9 months, when hearing children begin to babble normally. In addition, signs are clearly visible at distances, and signing is the preferred language of the Deaf community.

A disadvantage is that sign language is not the language of the hearing world; therefore, interpreters are necessary. It is usually not the language of the family, and many families are intimidated by having to learn a new language to communicate with their child. In fact, 20% of children who are deaf who sign have no family members who sign, and 40% have only 1 family member who signs. Parents must be reminded that as long as they are 1 sign ahead of their child in early childhood, they know all they need to maintain communication and linguistic development. With only a few signs, love and discipline can be clearly expressed.

Total communication with sign language and voice

Another debate centers on total communication, the use of signs and the voice simultaneously. 

Total communication allows children with residual hearing to benefit from supplemental auditory information. It may also help students in lip-reading because signs with meaning can be associated with movements of the mouth. (For example, mouth the words "I'm going to bed" to your spouse at bedtime; he or she probably understand because the phrase is short and the context is important.)

The main disadvantage is that speaking English while signing ASL at the same time is almost impossible. As a result, neither of the grammars is effectively or consistently applied.

School placement and schools for children with hearing impairment

Educational placements large depend on the choice of language.

Children who are learning to lip-read attend an oral school where lip-reading is taught. A substantial portion of the day is spent in lip-reading instruction, and other subjects cannot truly be taught until adequate language is established. As children age, they can be placed in a regular hearing classroom ("mainstreamed") if their lip-reading and vocal skills are sufficient. Their experience there depends on the teacher's ability to accommodate the needs of the student  (eg, by not spending much time facing the blackboard and away from the student). Many children who are deaf and who have succeeded to this point do well in school with their peers.

If the child learned cued speech in a special program, placement in a regular classroom may be possible if a cued-speech interpreter is provided. Many children who are entering a school where sign language is used have already learned some signs at home or in early intervention. Because sign language is visual, young children who are immersed in a signing setting rapidly acquire the signs for objects, people, and, ultimately, grammar. Shortly thereafter, formal educational programming can begin. This process is analogous to most kindergarten classes, which tend to be more social than academic. The use of ASL or SEE in schools is debated. Two issues are that most hearing teachers do not use good ASL grammar, and SEE takes too long to perform accurately.

Placing children who are deaf or hard of hearing with hearing children in the least restrictive environment (mainstreaming) may not be as successful for them as it is for children with other disabilities. Many so-called mainstream classrooms are isolated from those for hearing children, and the children who are deaf are instead grouped with children who have learning disabilities or mental retardation. Even in an integrated classroom, instruction happens through the interpreter. During when children are engaging in nonacademic subjects or when no interpreter is present, communication between deaf students and their classmates is limited.

Residential deaf schools are on the decline because of the recent desire to keep deaf children at home with their families. Relatively few independent day schools for the deaf exist. At a deaf school, where everyone signs, students can participate in many activities, such as debate, football, and cheerleading. Many deaf children live in a household that is linguistically isolated, though a school environment that is completely linguistically accessible to the child promotes self-esteem and social skills. Most deaf adults who attended such residential schools look back on that experience as being the best time of their lives.

Summary: Choice of language and school placement

The debates rage on, and all parties can be vehement in their views. No one opinion is right, and little literature of adequate quality strongly supports any particular standpoint. Results of most studies cannot be generalized to apply to the majority of children who are deaf and hard of hearing.

Decisions must almost always be individualized to meet the needs of the particular child, parents, and programs available to them. The goal of pediatricians should be continual monitoring of the child's progress. If the child is not succeeding in one environment or with one choice, suggest a trial in a different one.

Devices to Aid Children With Hearing Impairment

Young children need only hearing aids. As they grow, the family should be encouraged to obtain devices such as strobe lights connected to doorbells, timers, alarm clocks, and fire alarms. Telecommunication Devices for the Deaf (TDDs) and teletypewriters (TTYs) are machines than enable deaf people to use the phone. Computers with modems, cell phones, and hand-held devices permit children to communicate with email and text messages.

These and other aids help children with hearing impairment to develop a sense of independence and accomplishment, just as hearing children do when they complete tasks such as waking up for school using their own alarm clock or baking a cake for the first time.

Schools should also consider using FM amplification systems to transmit the teacher's voice to a small headphone speaker the child wear just behind the hearing aid. This system amplifies the teacher's voice over extraneous noise.

All new televisions are equipped with closed captioning, which decodes the captioning of dialogue and action provided with most television shows, videotapes, and DVDs. This not only makes television accessible but also promotes reading skills in deaf and hearing children alike.

Resources

• Alexander Graham Bell Association for the Deaf and Hard of Hearing
  3417 Volta Place, NW
  Washington, DC 20007
  Voice: (202) 337-5220
  TTY: (202) 337-5221
  Fax: 202-337-8314
  E-mail: info@agbell.org
• American Academy of Audiology
  11730 Plaza America Drive, Suite 300,
  McLean, VA 22102
  Voice: (800) AAA-2336, (703) 790-8466
  Fax: (703) 790-8631
  E-mail: info@audiology.org
• American Deafness and Rehabilitation Association (ADARA)
  ADARA National Office
  PO Box 480
  Myersville, MD 21773
  E-mail: ADARAorgn@aol.com
• American Hearing Research Foundation
  8 South Michigan Avenue, Suite 814
  Chicago, IL 60603-4539
  Voice: (312) 726-9670
  Fax: (312) 726-9695
  E-mail: ahrf@american-hearing.org
• Gallaudet University
  Laurent Clerc National Deaf Education Center
  and
  National Center for Law and the Deaf
  800 Florida Avenue, NE
  Washington, DC 20002
• Hearing Loss Association of America (HLAA, formerly Self Help for Hard of Hearing People [SHHH])
  7910 Woodmont Ave, Suite 1200
  Bethesda, MD 20814
  Phone: (301) 657-2248
• Helen Keller Services for the Blind
  National Center for Deaf-Blind Youths and Adults (HKNC)
  141 Middle Neck Road
  Sands Point, NY 11050
  Voice, TTY: (516) 944-8900
  E-mail: hkncinfo@hknc.org
• House Ear Institute (HEI)
  2100 West Third Street
  Los Angeles, CA 90057
  Voice: (213) 483-4431
  TTD: (213) 483-2642
  Fax: (213) 483-8789
  E-mail: info@hei.org
• National Association of the Deaf (NAD)
  8630 Fenton Street, Suite 820
  Silver Spring, MD 20910-3819
  Voice: (301) 587-1788
  TTY: (301) 587-1789
  Fax: (301) 587-1791
• National Cued Speech Association (NCSA)
  5619 McLean Drive
  Bethesda, MD 20814-1021
  Voice, TTY: (800) 459-3529, (301) 915-8009
• National Fraternal Society of the Deaf
  1188 South Sixth Street
  Springfield, IL 62703
  Voice: (217) 789-7429
  TTY: (217) 789-7438
• Office of Special Education and Rehabilitative Services (OSERS)
  US Department of Education
  400 Maryland Avenue, SW
  Washington, DC 20202-7100
  Voice: (202) 245-7468
• Parmly Hearing Institute
  Loyola University
  6525 North Sheridan Road
  Chicago, IL 60626
  Voice: (773) 508-2710
  Fax: (773) 508-2719
  E-mail: rfay@luc.edu (Richard R. Fay, director)
• Rainbow Alliance of the Deaf (RAD)
  Steven Schumacher, RAD Secretary
  9804 Walker House Road, Suite 4
  Montgomery Village, MD 20886-0506
• Registry of Interpreters for the Deaf, Inc
  333 Commerce Street
  Alexandria, VA 22314
  Phone: (703) 838-0030
• Telecommunications for the Deaf, Inc (TDI)
  8630 Fenton Street, Suite 604
  Silver Spring, MD 20910
  Voice: (301) 589-3786
  TTY: (301) 589-3006
  Fax: (301) 589-3797
  E-mail: info@tdi-online.org
• Triological Society (The American Laryngological, Rhinological, and Otological Society, Inc.)
  555 North 30th Street
  Omaha, NE 68131
  Voice: (402) 346-5500
  Fax: (402) 346-5300
  E-mail: info@triological.org
• USA Deaf Sports Federation (USADSF, formerly American Athletic Association of the Deaf [AAAD])
  102 North Krohn Place
  Sioux Falls, SD 57103-1800
  Voice: (605) 367-5760
  TTY: (605) 367-5761
  E-mail: HomeOffice@usdeafsports.org
• World Recreation Association of the Deaf, Inc (WRAD)
  PO Box 3211
  Quartz Hill, CA 93586
  Videophone: (661) 943-8879

Additional resources

For excellent patient education resources, visit eMedicine's Ear, Nose, and Throat Center. Also, see eMedicine's patient education article Hearing Loss.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Special Concerns

Universal neonatal hearing screening

In 1994, the JCIH recommended that hearing be assessed in all newborns before they are discharged from the neonatal nursery.⁷ The committee recommended a 2-step testing process with ABR, OAE, or a combination. The slight advantages of the OAE in easy and rapid testing are offset by an increased false-positive rate because cellular debris and amniotic fluid affect it more than the ABR.

In 1999, the AAP supported the committee's statement and endorsed the development of universal hearing screening programs.¹ The AAP noted that the goal of testing all newborns is feasible if the referral rate after a 2-step screening process can be maintained more than 4%.

Fluid and debris in the ears frequently cause false-positive results, the fluid might not resolve before discharge. Therefore, retesting may not achieve the stated goal of more than 4%. However, this possibility must be balanced against the loss to follow-up. Approximately 5-20% of parents have a child who may have hearing loss at the time of discharge. If a follow-up test is not performed before discharge, these parents may not return for repeat testing.

To be beneficial, a screening test must identify a problem that is amenable to intervention. Early intervention programs improve the likelihood that children will succeed later in life and were not possible when the mean age at diagnosis was 2.5 years. However, few primary care providers significantly understand deafness. They may not know how to help hearing-impaired children and their parents. Furthermore, specialists in the field of deafness are few. Most advice and support comes from nonmedical sources or otolaryngologists; in general, such information is biased toward one method over another.

Because of the prevalence of hearing loss and the benefit of early intervention, universal screening may still be worthwhile. If the AAP-supported recommendations are implemented, and if deafness is increasingly recognized as a pediatric problem, pediatricians will become more involved than they have been. Pediatricians will be able to gain knowledge in this field and to identify local resources for families with children who are deaf or hard of hearing.

Interpreters

Although the Americans with Disabilities Act (ADA) does not specifically limit the right to a sign-language interpreter to adults alone, children who sign are not usually offered the services of an interpreter.

If practitioners verbally ask children aged 6 years why they are sick, they should consider hiring an interpreter for a similar child who is deaf. Many children who are deaf grow up and know nothing about their families' health histories. For instance, they might be inadvertently excluded from discussions about a family member's illness that their hearing siblings overhear. They often know as little about their own health history. As adults, they may remember being in the hospital when they were aged 15 years, but they never knew why.

If a child requires a procedure, an operation, and/or anesthesia, an interpreter should certainly be hired to help communicate. Parents who know sign language are generally not proficient enough to explain the procedure to their child, even the simplified version of the situation that children are told. Furthermore, practitioners should permit the parents to focus on their roles (eg, as worried parents) and not have to be the child's interpreter. For some routine health-maintenance visits and for minor illnesses, interpreters are highly recommended but not as essential as they are when a child is being hospitalized or undergoing a surgical procedure.

Cochlear implants

Cochlear implants are implantable devices inserted through the skull and into the cochlea by using a drill.⁸ The device is connected, with a wire, to a subcutaneous magnet left behind the ear. The patient wears an exterior magnet connected by wire to a computer processor. Cochlear implants are not invisible. The processor alone is at least the size of a cigarette box or deck of cards, and it is worn on a large harness. However, new technologies are reducing the size of the devices.

Cochlear implants create sound in the brain by directly stimulating the auditory nerve. The processor divides sound in the hearing frequencies into 22 channels (current technology). Sound received by the processor in a given frequency is converted into an electronic impulse across the magnets to the cochlear implant itself, which then stimulates the nerve at 22 corresponding locations along the cochlea. Cochlear implants may be considered for implantation in children with profound hearing loss.

The training required for use is substantial and very important. Unless the child and parents participate in the training, much of the potential benefit of the cochlear implant is lost.

Cochlear implants are frequently mischaracterized as a cure for deafness. Many people believe that they may have potential adverse health effects, in addition to the risks of surgery and infection. Conversely, as the technology of the cochlear implant improves, it could potentially signal the demise of the deaf community. A cure for blindness or spinal cord injury would not destroy the communities of individuals with those disabilities in a similar fashion. Cochlear implants do provide many children with substantial hearing and auditory language benefit. However, predicting who will do well or how well they will do is impossible. Therefore, balanced and realistic counseling is important.

Deaf culture and the deaf community

Deafness creates a language barrier and thus isolates persons who are deaf from their families and communities. As a result, deaf people have formed a community linked by sign language. The core of the deaf community uses ASL more than they use SEE. ASL, folklore, jokes, and puns that are unique to the language highlight the shared experiences of the community.

Some parents fear losing their child to the deaf (signing) community. Sometimes, this fear is part of the reason parents opt to have their child read lips. However, children thrive when they are surrounded by people who can understand them.

Instead of denying the child access to the deaf culture, parents should be encouraged to learn about the deaf community and to participate in it. A person who is deaf participates in the hearing world by default. Parents should be encouraged to meet both oral and signing people who are deaf and to talk to them about their experiences. They should be helped to identify resources in the deaf community. The goal is for families to understand that existence in a world predicated on a signed language is liberating and not isolating.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

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Article Last Updated: Jul 21, 2008