Mutations in Kir channel genes have been associated with neurological dysfunction and seizure disorders, including a complex syndrome known as epilepsy, ataxia, sensorineural deafness, tubulopathy/seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (EAST/SeSAME), which results from mutations in KCNJ10 (gene encoding Kir4.1) (9, 10). Here, KCNJ10 is linked to cerebellar ataxia.