EAST/SeSAME syndrome is an extremely rare condition, with a prevalence of 1 out of 1,000,000 individuals, primarily affecting the central nervous system (CNS), kidneys, and inner ear (Abdelhadi et al. 2016). In 2009, two separated studies, one by Scholl et al. (2009) and the other by Bokenhauer et al. (2009), reported an unrecognized syndrome and according to its manifestations named it SeSAME and EAST in their studies, respectively. They found out that defects in potassium channels (Specifically Kir4.1) may cause EAST/SeSAME syndrome. The common characteristics of the syndrome include tubulopathy and electrolyte imbalance (e.g., hypomagnesemia, hypocalciuria, and hypokalemic metabolic alkalosis), sensorineural deafness, along with intellectual disability, seizure, epilepsy, and ataxia. Typically, the syndrome is first noticed due to neurological impairments in infancy and later on by electrolyte imbalances and renal waste salting in childhood (Abdelhadi et al. 2016) (Fig. 1).