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Hereditary hearing impairment affects about 1 in 1000 newborns. In most cases hearing loss is non-syndromic with no other clinical features, while in other families deafness is associated with specific clinical abnormalities. Analysis of large families with non-syndromic and syndromic deafness have been used to identify genes or gene locations that cause hearing impairment. The present report describes a large Norwegian family with autosomal dominant non-syndromic, progressive high tone hearing loss with linkage to 1q21–q23. A maximum LOD score of 7.65 (θ = 0.00) was obtained with the microsatellite markerD1S196.Analysis of recombinant individuals maps the deafness gene (DFNA7) to a 22 cM region betweenD1S104andD1S466.The region contains several attractive candidate genes. This report supports the idea of extensive genetic heterogeneity in hereditary hearing impairment and represents the first localization of a deafness gene in a Norwegian family.

Identification of a New Locus for Autosomal Dominant Non-Syndromic Hearing Impairment ( DFNA7 ) in a Large Norwegian Family