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

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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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 , National Academy of Sciences, 2014, 111 (25), pp.9307-12. ⟨10.1073/pnas.1405322111⟩. ⟨pasteur-01115196⟩

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