Mouse tales from Kresge: the deafness mouse.

  title={Mouse tales from Kresge: the deafness mouse.},
  author={Stacy Drury and Bronya J.B. Keats},
  journal={Journal of the American Academy of Audiology},
  volume={14 6},
  • S. DruryB. Keats
  • Published 1 August 2003
  • Biology
  • Journal of the American Academy of Audiology
Mouse models for human deafness have not only proven instrumental in the identification of genes for hereditary hearing loss, but are excellent model systems in which to examine gene function as well as the resulting pathophysiology. One mouse model for human nonsyndromic deafness is the deafness (dn) mouse, a spontaneous mutation in the curly-tail (ct) stock. The dn gene is on mouse Chromosome 19 and it was recently shown to be a novel gene called Tmc1. A mutation in Tmc1 is also found in… 

Topographic map refinement and synaptic strengthening of a sound localization circuit require spontaneous peripheral activity

Collectively, activity deprivation confined to peripheral protein loss impairs functional MNTB–LSO refinement during a critical prehearing period.



Dominant and recessive deafness caused by mutations of a novel gene, TMC1, required for cochlear hair-cell function

A locus for dominant deafness is reported, DFNA36, which maps to human chromosome 9q13–21 in a region overlapping the DFNB7/B11 locusfor recessive deafness, and eight mutations in a new gene, transmembrane cochlear-expressed gene 1 (TMC1), are identified.

Beethoven, a mouse model for dominant, progressive hearing loss DFNA36

The phenotype of the Beethoven (Bth) mouse mutant and a missense mutation in Tmc1 (transmembrane cochlear-expressed gene 1) are described, which cause progressive hearing loss and profound congenital deafness.

The deafness locus (dn) maps to mouse Chromosome 19

The identification of the defective gene in the mouse will help to explain the mechanism that causes hair cell degeneration and is likely to identify a homologous gene for deafness in humans.

The nature of inherited deafness in deafness mice

Deafness mice are studied in order to determine the nature of their inherited deafness, and data indicate that stimulus-related cochlear potentials do not develop even though hair cells are present in the young animal.

Inherited hearing defects in mice.

  • K. Steel
  • Biology
    Annual review of genetics
  • 1995
The distribution of candidate genes for deafness across the mouse genome is presented, together with a summary of the key features of the mutants involved, and the effects of mutations in orthologous genes involved in the auditory system in humans and mice are compared.

A human recessive neurosensory nonsyndromic hearing impairment locus is potential homologue of murine deafness (dn) locus.

A locus for recessive neurosensory nonsyndromic hearing impairment maps to chromosome 9q13-q21 in two regionally separate consanguineous families from India and the dn mouse is a potential model for the hearing impairment found in both these families.

Inner ear pathology in the deafness mutant mouse.

The data suggest that the deafness gene affects the organ of Corti and that cochlear hair cells in deafness mice are never functional.