Inherited hearing defects in mice.

Mouse mutants with hearing impairment are useful for elucidating the pathological processes underlying auditory system defects, as well as for understanding the normal process of auditory development and sensory transduction. Deaf mouse mutants are also valuable for identifying the responsible genes by positional cloning, and are used to expedite the search for genes involved in human deafness. The distribution of candidate genes for deafness across the mouse genome is presented, together with a summary of the key features of the mutants involved. Genetic defects affecting hearing can be grouped into broad categories according to their pathological features. These categories include middle ear defects, morphogenetic inner ear defects, central auditory system defects, peripheral neural defects, neuroepithelial defects, cochleo-saccular defects, and late onset hearing loss. The biological features and molecular basis of each type of hearing impairment are described. Finally, the effects of mutations in orthologous genes involved in the auditory system in humans and mice are compared.