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). Here, KCNJ10 is linked to EAST syndrome.