Review of hair cell synapse defects in sensorineural hearing impairment.

  title={Review of hair cell synapse defects in sensorineural hearing impairment.},
  author={Tobias Moser and Friederike Predoehl and A. M. Schrauwen L. Sequeira L. Stange R. C. Starr},
  journal={Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology},
  volume={34 6},
OBJECTIVE To review new insights into the pathophysiology of sensorineural hearing impairment. Specifically, we address defects of the ribbon synapses between inner hair cells and spiral ganglion neurons that cause auditory synaptopathy. DATA SOURCES AND STUDY SELECTION Here, we review original publications on the genetics, animal models, and molecular mechanisms of hair cell ribbon synapses and their dysfunction. CONCLUSION Hair cell ribbon synapses are highly specialized to enable… CONTINUE READING
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Mutations in CABP4, the gene encoding the Ca2+-binding protein 4, cause 9 HAIR CELL SYNAPSE DEFECTS

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Restoration of hearing in VGLUT3 knockout mice using virally-mediated gene therapy

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