LINKAGE OF CONGENITAL RECESSIVE DEAFNESS (GENE DFNB10) TO CHROMOSOME 21Q22.3

Deafness is a heterogeneous trait affecting similar to 1/1,000 newborn s. Genetic linkage studies have already implicated more than a dozen d istinct loci causing deafness. We conducted a genome search for linkag e in a large Palestinian family segregating an autosomal recessive for m of nonsyndromic deafness. Our results indicate that in this family t he defective gene, DFNB10, is located in a 12-cM region near the telom ere of chromosome 21. This genetic distance corresponds to <2.4 Mbp. F ive marker loci typed from this region gave maximum LOD scores greater than or equal to 3. Homozygosity of marker alleles was evident for on ly the most telomeric marker, D21S1259, suggesting that DFNB10 is clos est to this locus. To our knowledge, this is the first evidence, at th is location, for a gene that is involved in the development or mainten ance of hearing, As candidate genes at these and other deafness loci a re isolated and characterized, their roles in hearing will be revealed and may lead to development of mechanisms to prevent deafness.