Advantages of a Mouse Model for Human Hearing Impairment

Yoshiaki Kikkawa; Yuta Seki; Kazuhiro Okumura; Yasuhiro Ohshiba; Yuki Miyasaka; Sari Suzuki; Mao Ozaki; Kunie Matsuoka; Yoshihiro Noguchi; Hiromichi Yonekawa

doi:10.1538/expanim.61.85

Review Series: Frontiers of Model Animals for Human Diseases

Advantages of a Mouse Model for Human Hearing Impairment

Yoshiaki Kikkawa, Yuta Seki, Kazuhiro Okumura, Yasuhiro Ohshiba, Yuki Miyasaka, Sari Suzuki, Mao Ozaki, Kunie Matsuoka, Yoshihiro Noguchi, Hiromichi Yonekawa

Author information

Yoshiaki Kikkawa
Mammalian Genetics Project, Department of Genome Medicine, Tokyo Metropolitan Institute of Medical Science
Yuta Seki
Mammalian Genetics Project, Department of Genome Medicine, Tokyo Metropolitan Institute of Medical Science
Kazuhiro Okumura
Laboratory of Experimental Animals, Chiba Cancer Center Research Institute
Yasuhiro Ohshiba
Mammalian Genetics Project, Department of Genome Medicine, Tokyo Metropolitan Institute of Medical Science
Graduate School of Medical and Dental Sciences, Niigata University
Yuki Miyasaka
Mammalian Genetics Project, Department of Genome Medicine, Tokyo Metropolitan Institute of Medical Science
Graduate School of Medical and Dental Sciences, Niigata University
Sari Suzuki
Mammalian Genetics Project, Department of Genome Medicine, Tokyo Metropolitan Institute of Medical Science
Department of Bioproduction, Tokyo University of Agriculture
Mao Ozaki
Mammalian Genetics Project, Department of Genome Medicine, Tokyo Metropolitan Institute of Medical Science
Department of Bioproduction, Tokyo University of Agriculture
Kunie Matsuoka
Mammalian Genetics Project, Department of Genome Medicine, Tokyo Metropolitan Institute of Medical Science
Yoshihiro Noguchi
Department of Otolaryngology, Tokyo Medical and Dental University
Hiromichi Yonekawa
Basic Research Center, Tokyo Metropolitan Institute of Medical Science

Keywords: hearing impairment, inner ear hair cell, model mouse, stereocilia

JOURNAL OPEN ACCESS

2012 Volume 61 Issue 2 Pages 85-98

DOI https://doi.org/10.1538/expanim.61.85

Details

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.

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