A GENE RESPONSIBLE FOR A SENSORINEURAL NONSYNDROMIC RECESSIVE DEAFNESS MAPS TO CHROMOSOME 2P22-23

The recessive mode of transmission accounts for similar to 75% of inhe rited non syndromic deafness cases. We have previously designed the co nditions for linkage studies of this highly heterogeneous disorder [Gu ilford et al. (1994) Nature Genet. 6, 24-28]. Here, using a similar ap proach, we have studied the segregation of a gene responsible for cong enital, profound and fully penetrant sensorineural deafness in a consa nguineous family living in an isolated region of Lebanon. A maximum lo d score of 8.03 (theta = 0.00) was detected with a new polymorphic mar ker, AFMa052yb5 (D2S2144). Observed recombinants and homozygosity mapp ing define a maximum interval of 2 cM for this gene, DFNB6, which lies between AFMb346ye5 (a new polymorphic marker) (D2S2303) and AFM254vc9 (D2S174) on chromosome 2p22-23.