Title
Human hereditary hearing impairment: mouse models can help to solve the puzzle Human hereditary hearing impairment: mouse models can help to solve the puzzle
Author
Faculty/Department
Faculty of Pharmaceutical, Biomedical and Veterinary Sciences . Biomedical Sciences
Publication type
article
Publication
Berlin ,
Subject
Biology
Source (journal)
Human genetics. - Berlin
Volume/pages
124(2008) :4 , p. 325-348
ISSN
0340-6717
ISI
000260767100001
Carrier
E
Target language
English (eng)
Full text (Publishers DOI)
Affiliation
University of Antwerp
Abstract
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 identiWed. 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 Weld of age related hearing impairment by the use of mouse models and modiWers of hearing loss genes identiWed in mice
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