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REVIEW

Potassium Ion Movement in the Inner Ear: Insights from Genetic Disease and Mouse Models

Anselm A. Zdebik¹, Philine Wangemann² and Thomas J. Jentsch³

¹ UCL, Department of Neuroscience, Physiology, and Pharmacology, and Department of Medicine, London Epithelial Group, Hampstead Campus, London, United Kingdom;
² Anatomy and Physiology Department, Kansas State University, Manhattan, Kansas; and
³ Leibniz-Institut für Molekulare Pharmakologie (FMP) and Max-Delbrück-Centrum für Molekulare Medizin (MDC), Berlin, Germany, a.zdebik{at}ucl.ac.uk

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.