KCNJ10 and epilepsy: Indeed, independent clinical studies unraveled the involvement of Kir4.1 in EAST/SeSAME syndrome, that presents with a unique set of symptoms including epilepsy, ataxia, mental retardation, hearing loss, and electrolyte imbalance related to renal salt loss ( Bockenhauer et al., 2009; Scholl et al., 2009; Williams et al., 2010).