4.4 Review

Advantages of a Mouse Model for Human Hearing Impairment

Journal

EXPERIMENTAL ANIMALS
Volume 61, Issue 2, Pages 85-98

Publisher

INT PRESS EDITING CENTRE INC
DOI: 10.1538/expanim.61.85

Keywords

hearing impairment; inner ear hair cell; model mouse; stereocilia

Funding

  1. Japan Society for the Promotion of Science [20300147, 23300160]
  2. Takeda Science Foundation
  3. Grants-in-Aid for Scientific Research [20300147, 23300160] Funding Source: KAKEN

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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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