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Health Agencies Update
May 20, 1998

Deafness Gene Identified

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JAMA. 1998;279(19):1513. doi:10.1001/jama.279.19.1513-JHA80003-2-1

A team of researchers from Israel and the United States have identified a new gene that, when mutated, causes a form of progressive deafness (Science. 1998;279:1950-1954). The mutated gene, called DFNA15, was identified in several members of a large Israeli family affected by gradual hearing loss, beginning in their 20s and 30s.

The investigators mapped the newly discovered gene to a region of chromosome 5, near another gene recently found to cause progressive deafness. They found that affected family members, who usually experienced moderate to severe hearing loss by the time they reached their 50s, had a mutation in a gene known to cause progressive deafness in mice. Because the mutation doesn't interfere with normal auditory development and hearing is normal through early adulthood, the hearing loss resembles the age-related hearing loss that many people in the general population experience in their 70s and 80s.