A gene responsible for a dominant form of neurosensory non-syndromic deafness maps to the NSRD1 recessive deafness gene interval

The first localization of a gene responsible for autosomal,neurosensory, recessive deafness recently assigned NSRD1 tothe centromeric region of human chromosome 13. We now reporton a dominant form of neurosensory deafness found In a familyof French origin. The deafness is moderate to severe, has aprellngual onset and affects predominantly the high frequencies.The gene responsible for this form of deafness was found bylinkage analysis to map to the same region of chromosome 13as NSRD1. A multipoint analysis gave a maximum lod score of4.66 with a most likely location close to locus D13S175. Thissuggests that different mutations in NSRD1 may cause both dominantand recessive neurosensory deafness.