GENETIC DEAFNESS - PROGRESS WITH MOUSE MODELS

Genetic deafness is relatively common, accounting for about half of the 1 in 1000 children born with a significant hearing impairment. Heterogeneity is a particular problem when searching for genes for deafness in humans, and the mouse may prove to be a valuable model not only for investigating the nature of the deafness once the gene is known, but also for finding the gene by positional cloning. Several genes causing syndromic deafness have been identified in humans, but the largest group in the population have autosomal recessive deafness, and identification of homologous genes in the mouse may be the only route to these genes. Progress with positional cloning of the shaker-1 mouse mutation is described.