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Annu Rev Med. 2009;60:339-53. doi: 10.1146/

The kidney and ear: emerging parallel functions.

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Departments of Medicine, Faculty of Medicine, McGill University, Montreal, Quebec, Canada.


The association between renal dysplasia and minor malformations of the external ear is weak. However, there is a remarkable list of syndromes that link the kidney to the inner ear. To organize these seemingly disparate syndromes, we cluster representative examples into three groups: (a) syndromes that share pathways regulating development; (b) syndromes involving dysfunction of the primary cilium, which normally provides critical information to epithelial cells about the fluid in which they are bathed; (c) syndromes arising from dysfunction of specialized proteins that transport ions and drugs in and out of the extracellular fluid or provide structural support.

[Indexed for MEDLINE]

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