THE DEAFNESS LOCUS (DN) MAPS TO MOUSE CHROMOSOME-19

The deafness mouse has profound sensorineural hearing loss with degene ration of hair cells soon after birth. The mode of inheritance is rece ssive, and there are no associated phenotypic anomalies. Thus, this mo use provides a model for recessive, nonsyndromic, prelingual deafness. We have mapped the gene causing deafness in the mouse to Chromosome ( Chr) 19 by analysis of 230 intersubspecific backcross progeny. No reco mbinants were found with the microsatellite marker D19Mit14. The loci for two guanine nucleotide-binding proteins are tightly linked to this marker, and they are being investigated as possible candidate genes. The identification of the defective gene in the mouse will help to exp lain the mechanism that causes hair cell degeneration and is likely to identify a homologous gene for deafness in humans.