Abstract

D;-2qeafness from childhood is relatively common, with 1.3 per 1000 children affected,1 but until recently the causes have been obscure. It is clear that mutations in many different genes can cause deafness, and that many of these mutations are inherited in a recessive manner. The best estimates suggest that half the cases are attributable to genetic causes and half to environmental insults,2 but as for any disease caused by many different recessive mutations, there will be many families with a single affected child in which determination of cause is a matter of guesswork. The recent finding that mutations in a single gene, GJB2 , encoding the connexin …

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