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, neurosenso ry, recessive deafness recently assigned NSRD1 to the centromeric regi on of human chromosome 13. We now report on a dominant form of neurose nsory deafness found in a family of French origin. The deafness is mod erate to severe, has a prelingual onset and affects predominantly the high frequencies. The gene responsible for this form of deafness was f ound by linkage analysis to map to the same region of chromosome 13 as NSRD1. A multipoint analysis gave a maximum lod score of 4.66 with a most likely location close to locus D13S175. This suggests that differ ent mutations in NSRD1 may cause both dominant and recessive neurosens ory deafness.