KCNJ10 and Ataxia: SeSAME syndrome (OMIM#612780), characterized by seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance, otherwise known as EAST (Epilepsy, Ataxia, Sensorineural deafness, Tubulopathy) syndrome is predominantly caused by homozygous or compound heterozygous mutations in KCNJ10 gene [12, 13] encoding Kir4.1, an inwardly rectifying potassium channel.