In fact, loss-of-function mutations in the human KCNJ10 gene encoding Kir4.1 were reported to cause the epileptic disorders known as “EAST” (Epilepsy, Ataxia, Sensorineural deafness, and Tubulopathy) or “SeSAME” (Seizures, Sensorineural deafness, Ataxia, Mental retardation, and Electrolyte imbalance) syndrome [10,11,12]. The gene discussed is KCNJ10; the disease is Ataxia.