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  3. An unusually powerful mode of low-frequency sound interference due to defective hair bundles of the auditory outer hair cells

An unusually powerful mode of low-frequency sound interference due to defective hair bundles of the auditory outer hair cells

  • Proc Natl Acad Sci U S A. 2014 Jun 24;111(25):9307-12. doi: 10.1073/pnas.1405322111.
Kazusaku Kamiya 1,  Vincent Michel 2,  Fabrice Giraudet 3,  Brigitte Riederer 4,  Isabelle Foucher 2,  Samantha Papal 2,  Isabelle Perfettini 2,  Sébastien Le Gal 2,  Elisabeth Verpy 2,  Weiliang Xia 4,  Ursula Seidler 4,  Maria-Magdalena Georgescu 5,  Paul Avan 6,  Aziz El-Amraoui 7,  Christine Petit 8
Affiliations

Affiliations

  • 1 Institut Pasteur, Génétique et Physiologie de l'Audition, 75015 Paris, France;Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche, UMR-S 1120 Paris, France;Sorbonne Universités, UPMC Univ Paris 06, Complexité Du Vivant, 75252 Paris Cedex 05, France;Department of Otorhinolaryngology, Juntendo University Faculty of Medicine, Juntendo University, Tokyo 1138421, Japan;
  • 2 Institut Pasteur, Génétique et Physiologie de l'Audition, 75015 Paris, France;Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche, UMR-S 1120 Paris, France;Sorbonne Universités, UPMC Univ Paris 06, Complexité Du Vivant, 75252 Paris Cedex 05, France;
  • 3 Laboratoire de Biophysique Sensorielle, Faculté de Médecine, and Biophysique Médicale, Centre Jean Perrin, Université d'Auvergne, 63000 Clermont-Ferrand, France;
  • 4 Department of Gastroenterology, Hepatology, and Endocrinology, Hannover Medical School, 30625 Hannover, Germany;
  • 5 Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030; and.
  • 6 Laboratoire de Biophysique Sensorielle, Faculté de Médecine, and Biophysique Médicale, Centre Jean Perrin, Université d'Auvergne, 63000 Clermont-Ferrand, France; aziz.el-amraoui@pasteur.fr christine.petit@pasteur.fr paul.avan@udamail.fr.
  • 7 Institut Pasteur, Génétique et Physiologie de l'Audition, 75015 Paris, France;Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche, UMR-S 1120 Paris, France;Sorbonne Universités, UPMC Univ Paris 06, Complexité Du Vivant, 75252 Paris Cedex 05, France; aziz.el-amraoui@pasteur.fr christine.petit@pasteur.fr paul.avan@udamail.fr.
  • 8 Institut Pasteur, Génétique et Physiologie de l'Audition, 75015 Paris, France;Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche, UMR-S 1120 Paris, France;Sorbonne Universités, UPMC Univ Paris 06, Complexité Du Vivant, 75252 Paris Cedex 05, France;Collège de France, 75005 Paris, France aziz.el-amraoui@pasteur.fr christine.petit@pasteur.fr paul.avan@udamail.fr.
PMID: 24920589 DOI: 10.1073/pnas.1405322111
Abstract

A detrimental perceptive consequence of damaged auditory sensory hair cells consists in a pronounced masking effect exerted by low-frequency sounds, thought to occur when auditory threshold elevation substantially exceeds 40 dB. Here, we identified the submembrane scaffold protein Nherf1 as a hair-bundle component of the differentiating outer hair cells (OHCs). Nherf1(-/-) mice displayed OHC hair-bundle shape anomalies in the mid and basal cochlea, normally tuned to mid- and high-frequency tones, and mild (22-35 dB) hearing-threshold elevations restricted to midhigh sound frequencies. This mild decrease in hearing sensitivity was, however, discordant with almost nonresponding OHCs at the cochlear base as assessed by distortion-product otoacoustic emissions and cochlear microphonic potentials. Moreover, unlike wild-type mice, responses of Nherf1(-/-) mice to high-frequency (20-40 kHz) test tones were not masked by tones of neighboring frequencies. Instead, efficient maskers were characterized by their frequencies up to two octaves below the probe-tone frequency, unusually low intensities up to 25 dB below probe-tone level, and growth-of-masker slope (2.2 dB/dB) reflecting their compressive amplification. Together, these properties do not fit the current acknowledged features of a hypersensitivity of the basal cochlea to lower frequencies, but rather suggest a previously unidentified mechanism. Low-frequency maskers, we propose, may interact within the unaffected cochlear apical region with midhigh frequency sounds propagated there via a mode possibly using the persistent contact of misshaped OHC hair bundles with the tectorial membrane. Our findings thus reveal a source of misleading interpretations of hearing thresholds and of hypervulnerability to low-frequency sound interference.

Keywords

Nherf2; Usher syndrome; hearing impairment; off-frequency detection; tail hypersensitivity.

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