Abstract
Many mouse mutants have an apparent deficiency in their responsiveness to sound1. Most of these mutants have other abnormalities in addition to their hearing deficit, and the only two which have been subjected to a detailed anatomical and physiological study, shaker-1 and Ames waltzer, also have motor abnormalities2,3. The existence of such motor abnormalities throws some doubt on the usefulness of these two mutants as possible models for hereditary deafness in man, which is most frequently uncomplicated4. Deol and Kocher have described the deafness mutation in which mice homozygous for the recessive deafness gene (dn/dn) were unresponsive to sound and had no significant behavioural abnormality5. Cochlear hair cells in deafness mice develop normally and then degenerate, and the adult animals are completely deaf6. We have now studied deafness mice in order to determine the nature of their inherited deafness. Our data indicate that stimulus-related cochlear potentials do not develop even though hair cells are present in the young animal. The endocochlear potential is present in the scala media, but behaves abnormally during anoxia.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 51 print issues and online access
$199.00 per year
only $3.90 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
Similar content being viewed by others
References
Deol, M. S. J. med. Genet. 5, 137–158 (1968).
Mikaelian, D. O. & Ruben, R. J. Arch. Otolar. 80, 418–430 (1964).
Osako, S. & Hilding, D. A. Acta oto-lar 71, 365–376 (1971).
Fraser, G. R. The Causes of profound Deafness in Childhood (Johns Hopkins University Press, Baltimore, 1976).
Deol, M. S. & Kocher, W. Heredity 12, 463–466 (1958).
Niaussat, M.-M., Chevance, L.-G. & Adrian, M. C.r. Séanc. Soc. Biol. 171, 991–1002 (1977).
Bosher, S. K. J. Physiol., Lond. 293, 329–345 (1979).
Mikaelian, D. & Ruben, R. J. Acta oto-lar. 59, 451–461 (1965).
Russell, I. J. & Sellick, P. M. J. Physiol., Lond. 257, 245–255 (1976).
Dallos, P. The Auditory Periphery (Academic, New York, 1973).
Konishi, T. Acta oto-lar. 87, 506–516 (1979).
Konishi, T., Salt, A. N. & Hamrick, P. E. Hearing Res. 1, 325–342 (1979).
Johnstone, B. M. & Sellick, P. M. Q. Rev. Biophys. 5, 1–57 (1972).
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Steel, K., Bock, G. The nature of inherited deafness in deafness mice. Nature 288, 159–161 (1980). https://doi.org/10.1038/288159a0
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1038/288159a0
This article is cited by
-
Characterization of TMC-1 in C. elegans sodium chemotaxis and sodium conditioned aversion
BMC Genetics (2020)
-
Ultrastructural localization of the likely mechanoelectrical transduction channel protein, transmembrane-like channel 1 (TMC1) during development of cochlear hair cells
Scientific Reports (2019)
-
Distinct functions of TMC channels: a comparative overview
Cellular and Molecular Life Sciences (2019)
-
Variable number of TMC1-dependent mechanotransducer channels underlie tonotopic conductance gradients in the cochlea
Nature Communications (2018)
-
The emerging framework of mammalian auditory hindbrain development
Cell and Tissue Research (2015)
Comments
By submitting a comment you agree to abide by our Terms and Community Guidelines. If you find something abusive or that does not comply with our terms or guidelines please flag it as inappropriate.