A NOVEL LOCUS FOR AUTOSOMAL-DOMINANT NONSYNDROMIC HEARING-LOSS, DFNA13, MAPS TO CHROMOSOME 6P

Nonsyndromic hearing loss (NSHL) is the most common type of hearing im pairment in the elderly. Environmental and hereditary factors play an etiologic role, although the relative contribution of each is unknown. To date, 39 NSHL genes have been localized. Twelve produce autosomal dominant hearing loss, most frequently postlingual in onset and progre ssive in nature. We have ascertained a large, multigenerational family in which a gene for autosomal dominant NSHL is segregating. Affected individuals experience progressive hearing loss beginning in the 2d-4t h decades, eventually making the use of amplification mandatory. A nov el locus, DFNA13, was identified on chromosome 6p; the disease gene ma ps to a 4-cM interval flanked by D6S1663 and D6S1691, with a maximum t wo-point LOD score of 6.409 at D6S299.