An unusually powerful mode of low-frequency sound interference due to defective hair bundles of the auditory outer hair cells - Institut Pasteur Access content directly
Journal Articles Proceedings of the National Academy of Sciences of the United States of America Year : 2014

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

Paul Avan

Abstract

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)
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pasteur-01115196 , version 1 (01-12-2015)

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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⟩
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