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Stereocilin-deficient mice reveal the origin of cochlear waveform distortions

Author

Listed:
  • Elisabeth Verpy

    (Institut Pasteur, Unité de Génétique et Physiologie de l'Audition
    Inserm UMRS 587
    Collège de France
    Université Pierre et Marie Curie)

  • Dominique Weil

    (Institut Pasteur, Unité de Génétique et Physiologie de l'Audition
    Inserm UMRS 587
    Collège de France
    Université Pierre et Marie Curie)

  • Michel Leibovici

    (Institut Pasteur, Unité de Génétique et Physiologie de l'Audition
    Inserm UMRS 587
    Collège de France
    Université Pierre et Marie Curie)

  • 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
    Inserm UMRS 587
    Collège de France
    Université Pierre et Marie Curie)

  • Gaelle M. Lefèvre

    (Institut Pasteur, Unité de Génétique et Physiologie de l'Audition
    Inserm UMRS 587
    Collège de France
    Université Pierre et Marie Curie)

  • Jean-Pierre Hardelin

    (Institut Pasteur, Unité de Génétique et Physiologie de l'Audition
    Inserm UMRS 587
    Collège de France
    Université Pierre et Marie Curie)

  • 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
    Inserm UMRS 587
    Collège de France
    Université Pierre et Marie Curie)

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.

Suggested Citation

  • Elisabeth Verpy & Dominique Weil & Michel Leibovici & Richard J. Goodyear & Ghislaine Hamard & Carine Houdon & Gaelle M. Lefèvre & Jean-Pierre Hardelin & Guy P. Richardson & Paul Avan & Christine Peti, 2008. "Stereocilin-deficient mice reveal the origin of cochlear waveform distortions," Nature, Nature, vol. 456(7219), pages 255-258, November.
  • Handle: RePEc:nat:nature:v:456:y:2008:i:7219:d:10.1038_nature07380
    DOI: 10.1038/nature07380
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