Human hereditary hearing impairment: mouse models can help to solve the puzzle.

Mouse models are important research tools in the discovery of genes for syndromic as well as non-syndromic forms of hearing impairment. The similarity between the human and mouse inner ear, as well as the high homology between the human and mouse genome, make the mouse an excellent tool for genetic research on hearing loss. Over the past ten years, multiple mouse models for human hereditary hearing loss have been identified. The current review discusses the models according to the biological process in which they are involved. We review mouse models for genes involved in acellular matrix composition, hair bundle formation and maintenance, outer hair cell electromotility, the hair cell ribbon synapse and auditory nerve transmission, regulation of cochlear homeostasis and regulation of transcription. We have opted to put most emphasis on mouse models for non-syndromic forms of human hearing loss. However, we also discuss advances made in the field of age related hearing impairment by the use of mouse models and modifiers of hearing loss genes identified in mice.