Hearing Loss in the Pediatric Population

Congenital hearing loss is often hereditary but may be secondary to an intrauterine insult or infection. Of the hereditary variety, the majority are autosomal recessive and nonsyndromic. Risk factors for congenital hearing loss include family history of hearing loss, facial abnormalities, ICU admission, history of meningitis, syndromes known to be associated with hearing loss, low Apgar scores at birth, medications known to cause hearing loss (e.g., aminoglycosides), elevated bilirubin, some prenatal maternal infections, or suspicion of hearing loss.

Universal newborn hearing screening using otoacoustic emissions and auditory brainstem response allows early identification of impaired children. Intervention by age 6 months appears to improve language development. A temporal bone CT is often obtained to evaluate for inner ear malformations that would predispose the patient to further hearing loss with even mild head trauma. A genetics evaluation and counseling may be indicated. Mutations of the Con-nexin-26 gene, an autosomal recessive disorder, accounts for a significant percentage of nonsyndromic hereditary hearing loss. Hearing loss may coexist with other conditions (e.g., renal, ophthalmologic, thyroid, infectious, cardiac), so other testing may be indicated based on clinical suspicion.

In children with a significant hearing loss, hearing aids are recommended. A cochlear implant may be indicated for those with hearing so poor to be considered unaidable. A cochlear implant is a surgically implanted device that receives sound, converts it to electrical signals, and directly stimulates the cochlea. Results are excellent in properly selected patients.

An important and often unrecognized cause of hearing loss in children is chronic OME. Children with either unilateral or bilateral middle ear effusions refractory to medical treatment for more than 3 months should have audiomet-ric testing. Myringotomy with tube insertion is indicated if bilateral CHL is found and should be considered in some cases of unilateral loss as well.

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Hearing Aids Inside Out

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