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Linking genes underlying deafness to hair-bundle development and function

Nature Neuroscience volume 12, pages 703–710 (2009)Cite this article

Abstract

The identification of mutations underlying monogenic, early-onset forms of deafness in humans has provided unprecedented insight into the molecular mechanisms of hearing in the peripheral auditory system. The molecules involved in the development and function of the cochlea eluded characterization until recently owing to the scarcity of the principal cell types present. The genetic approach has circumvented this problem and succeeded in identifying proteins and deciphering some of the molecular complexes that operate in these cells. In combination with mouse models, the genetic approach is now revealing some of the principles underlying the development and physiology of the cochlea. Focusing on the hair bundle, the mechanosensory device of the sensory hair cell, we highlight recent advances in understanding the way in which the hair bundle is formed, how it operates as a mechanotransducer and how it processes sound. In particular, we discuss how these findings confer a central role on the various hair-bundle links in these processes.

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Figure 1: Hair bundle structure and function.
Figure 2: Hair-bundle development and hair-bundle phenotypes in mouse mutants.

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Acknowledgements

The authors' research was supported by grants from European Commission FP6 Integrated Project EuroHear LSHG-CT-2004-512063 (C.P. & G.R.), Fondation Raymonde et Guy Strittmatter (C.P.), Usher FAUN Stiftung (C.P.), Ernst-Jung Stiftung für Medizin (C.P.), Louis-Jeantet for Medicine Foundation (C.P.) and the Wellcome Trust (G.R.). The authors would like to thank V. Michel and R. Goodyear for their help in preparing the figures, A. Forge for the scanning electron micrographs in Figure 1, J. Levilliers for help in the preparation of the document and J.-P. Hardelin for criticism of the manuscript.

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  1. Unite de Genetique et Physiologie de l'Audition, INSERM Unité Mixte de Recherche en Santé 587, Institut Pasteur, College de France, Université Pierre & Marie Curie, Paris, France

    Christine Petit

  2. School of Life Sciences, University of Sussex, Brighton, UK

    Guy P Richardson

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Petit, C., Richardson, G. Linking genes underlying deafness to hair-bundle development and function. Nat Neurosci 12, 703–710 (2009). https://doi.org/10.1038/nn.2330

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