A new locus for late-onset, progressive, hereditary hearing loss DFNA20 maps to 17q25

We report the localization of DFNA20, a gene causing dominant, nonsyndromic , progressive hearing loss in a three-generation Midwestern family, to chro mosome 17q25, Affected family members show a bilateral, sloping, progressiv e, sensorineural hearing loss, first evident at 6000 and 8000 Hz, that can be identified in some family members in the early teens and is clearly evid ent by the early twenties. As age increases, the degree of hearing loss inc reases with threshold shifts seen at all frequencies. Linkage to known here ditary hearing loss loci was excluded. A genome-wide screen detected positi ve linkage to D17S784 (LOD(Z) = 6.62; theta = 0), Haplotype analysis refine s the DFNA20 critical region to 12 cM between D17S1806 and D17S668, Radiati on hybrid mapping with Stanford G3 and TNG panels was used to evaluate the genes ACTG1, GRIN2C, FKHL13, P4HB, SPARC, and ARHGDIA as candidates for DFN A20, (C) 2000 Academic Press.