title: Advantages of a mouse model for human hearing impairment.
abstract: Hearing is a major factor in human quality of life. Mouse models are important tools for discovering the genes that are responsible for genetic hearing loss, and these models often allow the processes that regulate the onset of deafness in humans to be analyzed. Thus far, in the study of hearing and deafness, at least 400 mutants with hearing impairments have been identified in laboratory mouse populations. Analysis of through a combination of genetic, morphological, and physiological studies is revealing valuable insights into the ontogenesis, morphogenesis, and function of the mammalian ear. This review discusses the advantages of the mouse models of human hearing impairment and highlights the identification of the molecules required for stereocilia development in the inner ear hair cells by analysis of various mouse mutants.
authors.affiliation: Mammalian Genetics Project, Department of Genome Medicine, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Kikkawa
authors.forename: Yuta
authors.initials: Y
authors.lastname: Seki
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Okumura
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Ohshiba
authors.forename: Yuki
authors.initials: Y
authors.lastname: Miyasaka
authors.forename: Sari
authors.initials: S
authors.lastname: Suzuki
authors.forename: Mao
authors.initials: M
authors.lastname: Ozaki
authors.forename: Kunie
authors.initials: K
authors.lastname: Matsuoka
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Noguchi
authors.forename: Hiromichi
authors.initials: H
authors.lastname: Yonekawa
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D006198:Hair Cells, Auditory / Q000502:physiology; D006309:Hearing / Q000235:genetics* / Q000502:physiology; D034381:Hearing Loss / Q000235:genetics* / Q000503:physiopathology; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D013045:Species Specificity; D059547:Stereocilia / Q000502:physiology