Stereocilin-deficient mice reveal the origin of cochlear waveform distortions

Elisabeth Verpy (), Dominique Weil, Michel Leibovici, Richard J. Goodyear, Ghislaine Hamard, Carine Houdon, Gaelle M. Lefèvre, Jean-Pierre Hardelin, Guy P. Richardson, Paul Avan and Christine Petit ()
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Elisabeth Verpy: Institut Pasteur, Unité de Génétique et Physiologie de l'Audition
Dominique Weil: Institut Pasteur, Unité de Génétique et Physiologie de l'Audition
Michel Leibovici: Institut Pasteur, Unité de Génétique et Physiologie de l'Audition
Richard J. Goodyear: University of Sussex, School of Life Sciences, Falmer, Brighton BN1 9QG, UK
Ghislaine Hamard: Institut Cochin, Plate-Forme de Recombinaison Homologue
Carine Houdon: Institut Pasteur, Unité de Génétique et Physiologie de l'Audition
Gaelle M. Lefèvre: Institut Pasteur, Unité de Génétique et Physiologie de l'Audition
Jean-Pierre Hardelin: Institut Pasteur, Unité de Génétique et Physiologie de l'Audition
Guy P. Richardson: University of Sussex, School of Life Sciences, Falmer, Brighton BN1 9QG, UK
Paul Avan: Université d'Auvergne, Laboratoire de Biophysique Sensorielle
Christine Petit: Institut Pasteur, Unité de Génétique et Physiologie de l'Audition

Nature, 2008, vol. 456, issue 7219, 255-258

Abstract: The key to sound distortion The mammalian ear, or rather its auditory organ the cochlea, is a remarkably sensitive and finely tuned acousto-electrical transducer. It also markedly distorts sounds, and counter-intuitively it is this latter characteristic that gives rise to the masking effect that improves speech intelligibility in the human ear. Until now, the origin of these distortions was unknown. The mammalian cochlea possesses two types of hair cells — inner and outer hair cells. In experiments in mice, Verpy et al. show that it is the outer hair cells that introduce the distortion; the nonlinearity arises in the 'top connectors' between outer hair cell sterocilia known as, and more specifically, in a protein called stereocilin.

Date: 2008
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DOI: 10.1038/nature07380

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