Recent clinical studies have shown that mutations in the human gene KCNJ10 encoding Kir4.1 cause EAST (epilepsy, ataxia, sensorineural deafness, and tubulopathy) or SeSAME (seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance) syndrome consisting of generalized tonic-clonic (GTC) seizures, ataxia, hearing loss, and abnormal renal excretion of electrolytes (Bockenhauer et al., 2009; Scholl et al., 2009). Here, KCNJ10 is linked to cerebellar ataxia.