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Deafness gene is identified

Scientists say they have identified a gene that can cause deafness in children, a step that may aid in diagnosis and eventually finding a treatment.

Two independent teams of scientists report finding the gene that, when defective, causes Waardenburg's syndrome.

The inherited syndrome is blamed for 3 percent of childhood deafness, said Dr. Aubrey Milunsky, co-author of the report on the finding.

People with the syndrome often have a distinctive appearance that can include a shock of white hair at the front of the head, wide spacing of the eyes, or one eye a different color from the other. Most patients do not have all these features, Milunsky said.

Only about 20 percent of patients have total hearing loss, Milunsky said. But anybody with the defective gene has a 50 percent chance of transmitting it to each offspring, in whom it may produce deafness, Milunsky said.

Dr. David Edelstein of the Manhattan Eye, Ear and Throat Hospital, who was not connected with the study, said the finding may aid in diagnosis and point the way to a treatment.

The finding of the gene is reported in today's issue of the journal Nature.