title: Potassium ion movement in the inner ear: insights from genetic disease and mouse models.
abstract: Sensory transduction in the cochlea and vestibular labyrinth depends on fluid movements that deflect the hair bundles of mechanosensitive hair cells. Mechanosensitive transducer channels at the tip of the hair cell stereocilia allow K(+) to flow into cells. This unusual process relies on ionic gradients unique to the inner ear. Linking genes to deafness in humans and mice has been instrumental in identifying the ion transport machinery important for hearing and balance. Morphological analysis is difficult in patients, but mouse models have helped to investigate phenotypes at different developmental time points. This review focuses on cellular ion transport mechanisms in the stria vascularis that generate the major electrochemical gradients for sensory transduction.
authors.affiliation: UCL, Department of Neuroscience, London Epithelial Group, Hampstead Campus, London, United Kingdom. a.zdebik@ucl.ac.uk
authors.forename: Anselm A
authors.initials: AA
authors.lastname: Zdebik
authors.forename: Philine
authors.initials: P
authors.lastname: Wangemann
authors.forename: Thomas J
authors.initials: TJ
authors.lastname: Jentsch
mesh_terms: D000818:Animals; D007758:Ear, Inner / Q000378:metabolism / Q000502:physiology*; D006198:Hair Cells, Auditory / Q000378:metabolism / Q000502:physiology; D006801:Humans; D007477:Ions; D007759:Labyrinth Diseases / Q000235:genetics* / Q000378:metabolism* / Q000503:physiopathology; D051379:Mice; D011188:Potassium / Q000378:metabolism / Q000502:physiology*; D015221:Potassium Channels / Q000502:physiology
chemical_list: D007477:Ions; D015221:Potassium Channels; D011188:Potassium