ABSTRACT
Syndromic deafness may account for as much as 30% of all inherited forms of deafness, and the identification of genetic defects associated with specific conditions has been a major goal of biomedical scientists for some time. Although mouse models are commonly exploited to understand human disease, few animal models of syndromic deafness have been developed or identified. However, for two major diseases, Usher and Alport syndromes, there are well-defined mouse models available at this time that harbor mutations in the orthologous genes that cause human deafness, and they are the focus of this chapter.
