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

Kazusaku Kamiya (1, 2) , Vincent Michel (1) , Fabrice Giraudet (3) , Brigitte Riederer (4) , Isabelle Foucher (1) , Samantha Papal (1) , Isabelle Perfettini (1) , Sébastien Le Gal (1) , Elisabeth Verpy (1) , Weiliang Xia (5) , Ursula Seidler (4) , Maria-Magdalena Georgescu (6) , Paul Avan (3) , Aziz El-Amraoui (1) , Christine Petit (1)

1 Collège de France - Chaire Génétique et physiologie cellulaire
2 Department of Otorhinolaryngology
3 Equipe Biophysique Neurosensorielle [Neuro-Dol]
4 Department of Gastroenterology, Hepatology and Endocrinology
5 School of Management
6 Department of Neuro-Oncology

A detrimental perceptive consequence of damaged auditory sen-sory 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 dis-played OHC hair-bundle shape anomalies in the mid and basal co-chlea, 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 co-chlear 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)

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

Domains: Sensory Organs

Kazusaku Kamiya, Vincent Michel, Fabrice Giraudet, Brigitte Riederer, Isabelle Foucher, et al.. An unusually powerful mode of low-frequency sound interference due to defective hair bundles of the auditory outer hair cells. Proceedings of the National Academy of Sciences of the United States of America, 2014, 111 (25), pp.9307-12. ⟨10.1073/pnas.1405322111⟩. ⟨pasteur-01115196⟩