Mice as Models for Human Hereditary Deafness

There is now a large number of mouse mutants with hearing and/or balance defects available for investigating the reasons for the impairment, and these mutants will all contribute to our growing understanding of the complexity of deafness. Many more mouse mutants are candidates for involvement of the auditory system, but their hearing has not yet been investigated in any detail. Some of these are listed in additional tables available at the Web site accompanying this chapter (Steel 2000). However, comparison of the chromosomal locations of these mutations causing deafness in the mouse with the locations of known human deafness mutations reveals that there are many human loci for which no mouse model has yet been discovered. Two major mutagenesis programs are ongoing in Europe, at Neuherberg, Germany and Harwell, UK, and new deaf mouse mutants are being isolated from these screens to help to fill the gap between human deafness and mouse models (Nolan et al. 2000). Large-scale, genome-wide mutagenesis programmes are starting in other countries too, including the US, so there will soon be many more mutants available. Deafness is one of the most heterogeneous diseases known in humans, and study of the many deaf mouse mutants will help unravel the molecular basis of the pathology, an essential first step towards a rational approach to treatment.