Connexin 26 Studies in Patients With Sensorineural Hearing Loss

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Abstract
CONGENITAL sensorineural hearing loss (SNHL) has an incidence in children of 1 to 2 per 1000 for bilateral severe-to-profound losses (>50 dB) and up to 4 per 1000 if mild-to-moderate and unilateral loses are included. Three of every 1000 US schoolchildren have unilateral SNHL of 45 dB or greater; if the threshold is changed to 26 dB, the number increases to 13 per 1000. Sixteen percent of adults have hearing impairment of 25 dB or greater.1 Until recently, the cause of many of these losses has been obscure, with identification of the cause occurring about half of the time, and less frequently if the loss is unilateral or if the child has no family history of hearing loss, no significant medical history, and no dysmorphic features. The known causes of SNHL include genetic (syndromic and nonsyndromic; congenital and "acquired"), viral, bacteriologic, traumatic, immunologic, and drug-related causes and other medical conditions. Many authors2-4 believe that up to 50% of congenital SNHL can be attributed to genetic causes; of these, nonsyndromic recessive causes represent approximately 80%.