The important role of the Kir4.1 channels is demonstrated by a number of studies identifying mutations in the Kir4.1 gene (KCNJ10) as a reason for symptoms found in the EAST syndrome (epilepsy, ataxia, sensorineural deafness, and tubulopathy) [14]–[18]. The gene discussed is KCNJ10; the disease is Ataxia.