Cochlear development: hair cells don their wigs and get wired

Current Opinion in Otolaryngology & Head and Neck Surgery
Donna S Whitlon

Abstract

Hair cells and spiral ganglion neurons form functional pairings in the cochlea that transduce the mechanical energy of sound into signals that are carried to the brainstem. Mutations of genes affecting the development and maintenance of these two cell populations cause deafness in humans and other animals. This review highlights recent findings regarding the development of hair cell stereocilia and spiral ganglion neurons in the cochlea. Genes underlying Usher syndrome 1A have shed light on possible molecular participants in the development and structure of the hair cell stereocilia. Analysis of deaf mouse mutants have uncovered genes involved in stereocilia elongation and the orientation of the stereociliary bundles. Studies on the regulation of spiral ganglion neuronal survival and guidance suggest that the timing of expression of specific growth factors along the cochlear spiral is involved in maintaining the divergence of vestibular and cochlear nerve fibers. Examining human and mouse genes affecting hearing has not only provided insight into causes of human deafness, but has also opened a window into how stereociliary bundles are constructed and spiral ganglion neurons are preserved and guided during development. Synthesis...Continue Reading

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Citations

Feb 17, 2006·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Mathias SenftenUlrich Müller
Feb 12, 2011·Frontiers in Neuroendocrinology·Dennis McFadden
Apr 20, 2007·Developmental Neurobiology·Dongguang WeiMats Ulfendahl
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