Mutations in KCNJ10, which codes for the glial‐specific Kir4.1 channel, underlie SeSAME/EAST syndrome (seizures, sensorineural deafness, ataxia, intellectual disability and electrolyte imbalance/epilepsy, ataxia, sensorineural deafness, and tubulopathy) (Bockenhauer et al., 2009; Scholl et al., 2009) and have been detected in patients diagnosed with autism spectrum disorder and epilepsy (Sicca et al., 2011, 2016). Here, KCNJ10 is linked to cerebellar ataxia.