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A genetic approach to understanding auditory function

Nature Genetics volume 27, pages 143–149 (2001)Cite this article

Abstract

Little is known of the molecular basis of normal auditory function. In contrast to the visual or olfactory senses, in which reasonable amounts of sensory tissue can be gathered, the auditory system has proven difficult to access through biochemical routes, mainly because such small amounts of tissue are available for analysis. Key molecules, such as the transduction channel, may be present in only a few tens of copies per sensory hair cell, compounding the difficulty. Moreover, fundamental differences in the mechanism of stimulation and, most importantly, the speed of response of audition compared with other senses means that we have no well-understood models to provide good candidate molecules for investigation. For these reasons, a genetic approach is useful for identifying the key components of auditory transduction, as it makes no assumptions about the nature or expression level of molecules essential for hearing. We review here some of the major advances in our understanding of auditory function resulting from the recent rapid progress in identification of genes involved in deafness.

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Figure 1: Schematic illustration of the top, apical surface of a hair cell, showing two of the tens to hundreds of stereocilia that make up the hair bundle and are inserted into the cuticular plate.

Bob Crimi

Figure 2: Schematic illustrations of an inner hair cell (IHC, flask shaped) and an outer hair cell (OHC, cylindrical) showing locations in the basolateral membrane of functionally important gene products.

Bob Crimi

Figure 3: Scanning electron micrographs of the upper surface of outer hair cells, showing the stereocilia bundles.
Figure 4: Illustration of cochlear duct showing the proposed lateral (right) and medial (left) K+ ion recycling pathways from the sensory hair cells within the organ of Corti back to the endolymph48.

Bob Crimi

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Acknowledgements

Supported by the MRC, Defeating Deafness and EC contract QLG2-CT-1999-00988.

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  1. Medical Research Council Institute of Hearing Research, University of Nottingham, Nottingham, UK

    Karen P. Steel

  2. School of Biological Sciences, University of Sussex, Falmer, Brighton, UK

    Corné J. Kros

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Correspondence to Karen P. Steel.

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Steel, K., Kros, C. A genetic approach to understanding auditory function. Nat Genet 27, 143–149 (2001). https://doi.org/10.1038/84758

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