KCNJ10 and Ataxia: Biallelic loss-of-function mutations in Kir4.1 have been linked to a syndrome that is referred to by two different acronyms, i.e., EAST syndrome (epilepsy, ataxia, sensorineural deafness, and tubulopathy) and SeSAME syndrome (seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance).