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Making sense out of sound

Nature Genetics volume 21, pages 347–349 (1999)Cite this article

Sound is a well-understood, physical phenomenon. How we perceive sound and make sense of complex auditory stimuli is a different matter altogether. Genetic approaches, however, have provided a powerful strategy to dissect auditory function and to some extent, overcome the challenge posed by the inaccessibility and scant quantities of cochlear neurosensory tissue lying deep within the temporal bone. Although remarkable progress has been made in identifying genes causing hearing loss, correlating the functions of those genes with specific auditory processes or pathways is not always straightforward. On page 363 of this issue, Shin'ichiro Yasunaga and colleagues report the latest 'deafness' gene1. While its function has yet to be determined, its sequence and expression in the inner hair cells of the cochlea provide some intriguing clues.

There are success stories in which deafness genes have been linked to cochlear function, physiology and development. Recently, a 'deafness' gene that resides at the nonsyndromic dominant deafness locus DFNA2 was identified as one that encodes a novel potassium channel (KCNQ4; ref. 6) and is expressed in the outer hair cells of the cochlea. The connexin genes GJB2 and GJB3 and the potassium channel subunit genes KCNQ1 and KCNE1 probably have important roles in potassium homeostasis in the auditory system; mutations in these genes can cause sensorineural hearing loss. Furthermore, mutations of KCNE1 (JLNS2) and KCNQ1 (JLNS1) can cause cardiac conduction abnormalities in addition to hearing loss7.

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Figure 1: Diagram of the human ear, including cross sections of the cochlea showing three outer hair cells and one inner hair cell in the organ of Corti.
Figure 2: Cytogenetic locations of genes for syndromic and nonsyndromic hearing loss illustrated on a human male karyotype.

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Authors and Affiliations

  1. Laboratory of Molecular Genetics, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 5 Research Court, Rockville, 20850, Maryland, USA

    Andrew J Griffith & Thomas B Friedman

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  1. Andrew J Griffith
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  2. Thomas B Friedman
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Griffith, A., Friedman, T. Making sense out of sound. Nat Genet 21, 347–349 (1999). https://doi.org/10.1038/7668

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