(See also Hearing Loss.)
In the US, permanent childhood hearing loss is detected in 1.1/1000 infants screened. On average, 1.9% of children reported “hearing trouble.” Hearing impairment is slightly more common among boys than girls; the average male:female ratio is 1.24:1.
Etiology
Neonates
The most common causes of hearing loss in neonates are
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Congenital cytomegalovirus (CMV) infection (1)
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Genetic defects (see table Some Genetic Causes of Hearing Loss)
Congenital CMV infection is the most common intrauterine infection in the US. CMV infection may account for as much as 21% of all sensorineural hearing loss at birth. In addition, because CMV infection also may cause late-onset hearing loss, CMV may account for as much as 25% of sensorineural hearing loss present at 4 years of age.
Some Genetic Causes of Hearing Loss
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Syndrome |
Inheritance Pattern |
Clinical Findings |
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Branchio-oto-renal syndrome (Melnick-Fraser syndrome) |
Most have hearing loss and ear abnormalities Associated with renal abnormalities and branchial cleft cysts or fistulas |
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Jervell and Lange-Nielsen syndrome |
Profound congenital hearing loss Abnormal cardiac rhythms (eg, prolonged QTc interval) Increased risk of syncope and sudden death |
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Pendred syndrome |
Progressive hearing loss Associated with simple nontoxic goiter (euthyroid goiter), congenital goiter, and vestibular symptoms |
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Stickler syndrome |
Hearing loss varies in degree and may become more severe over time Associated with bony and skeletal abnormalities Many have severe nearsightedness |
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Usher syndrome |
Accounts for 3 to 6% of all children who are deaf and another 3 to 6% of children who are hard of hearing Associated with retinitis pigmentosa Three clinical types |
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Waardenburg syndrome |
When hearing loss occurs, it is congenital Associated with pigmentary changes in hair, eyes, and skin Four recognized types, each with different physical characteristics |
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AD = autosomal dominant; AR = autosomal recessive. |
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Risk factors for hearing loss in neonates include the following:
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Low birth weight (eg, < 1.5 kg)
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Apgar score < 5 at 1 minute or < 7 at 5 minutes
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Hypoxemia or seizures resulting from a difficult delivery
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Prenatal infection with rubella, syphilis, herpes, cytomegalovirus, or toxoplasmosis
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Craniofacial anomalies, particularly those that involve the external ear
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Sepsis or meningitis
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Ventilator dependence
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Family history of early hearing loss
Infants and children
The most common causes in infants and children are
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Middle ear effusions, including otitis media with effusion
Other causes in older children include head injuries, loud noises (including loud music), use of ototoxic drugs (eg, aminoglycosides, thiazides), viral infections (eg, mumps), tumors or injuries affecting the auditory nerve, foreign bodies of the ear canal, and, rarely, autoimmune disorders.
Risk factors for hearing loss in children include those for neonates plus the following:
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Skull fracture or traumatic loss of consciousness
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Neurodegenerative disorders, including neurofibromatosis
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Noise exposure
Etiology reference
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1. Goderis J, De Leenheer E, Smets K, et al: Hearing loss and congenital CMV infection: A systematic review. Pediatrics 134(5):972–982, 2014. doi: 10.1542/peds.2014-1173
Symptoms and Signs
If hearing loss is severe, the infant or child may not respond to sounds or may have delayed speech or language comprehension. If hearing loss is less severe, children may intermittently ignore people talking to them. Children may appear to be developing well in certain settings but have problems in others. For example, because the background noise of a classroom can make speech discrimination difficult, the child may have problems hearing only at school.
Not recognizing and treating impairment can seriously impair language comprehension and speech. The impairment can lead to failure in school, teasing by peers, social isolation, and emotional difficulties.
Diagnosis
Screening all infants before age 3 months is often recommended and is legally mandated in most states (1). The initial screening test is evoked otoacoustic emissions testing, using soft clicks made by a handheld device. If results are abnormal or equivocal, auditory brain stem evoked responses are tested, which can be done during sleep; abnormal results should be confirmed with repeat testing after 1 month. If a genetic cause is suspected, genetic testing can be done.
In children, other methods can be used. Speech and overall development are assessed clinically. The ears are examined, and tympanic membrane movement is tested in response to various frequencies to screen for middle ear effusions. In children age 6 months to 2 years, response to sounds is tested. At age > 2 years, ability to follow simple auditory commands can be assessed, as can responses to sounds using earphones. Central auditory processing evaluation can be used for children > 7 years without neurocognitive deficits who seem to hear but not to comprehend.
Imaging is often indicated to identify the etiology and guide prognosis. For most cases, including when neurologic examination is abnormal, word recognition is poor, and/or hearing loss is asymmetric, gadolinium-enhanced MRI is done. If bone abnormalities are suspected, CT is done.
Diagnosis reference
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1. US Preventive Services Task Force: Universal screening for hearing loss in newborns: US Preventive Services Task Force recommendation statement. Pediatrics 122(1):143–148, 2008. doi: 10.1542/peds.2007-2210
Treatment
Reversible causes and abnormalities are treated.
If hearing loss is irreversible, a hearing aid can usually be used. They are available for infants as well as children. If hearing loss is mild or moderate or affects only one ear, a hearing aid or earphones can be used. In the classroom, an FM auditory trainer can be used. With an FM auditory trainer, the teacher speaks into a microphone that send signals to a hearing aid in the nonaffected ear.
If hearing loss is severe enough that it cannot be managed with hearing aids, a cochlear implant may be needed. Children may also require therapy to support their language development, such as being taught a visually based sign language.
Key Points
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Common causes of hearing loss in neonates are cytomegalovirus infection or genetic defects and in infants and older children are cerumen accumulation and middle ear infusions.
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Suspect hearing loss if a child's response to sounds or development of speech and language is abnormal.
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Screen infants for hearing loss, beginning with evoked otoacoustic emissions testing.
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Diagnose children based on results of clinical examination and tympanometry.
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Treat irreversible hearing loss with a hearing aid or cochlear implant and language support (eg, teaching sign language) as needed.
