Seizures, Sensorineural Deafness, Ataxia, Impaired Intellectual Development, and Electrolyte Imbalance (SESAMES)

Seizures, Sensorineural Deafness, Ataxia, Impaired Intellectual Development, and Electrolyte Imbalance, also known as sesame syndrome, is related to sensorineural hearing loss and hypokalemia, and has symptoms including ataxia, polyuria and seizures. An important gene associated with Seizures, Sensorineural Deafness, Ataxia, Impaired Intellectual Development, and Electrolyte Imbalance is KCNJ10 (Potassium Inwardly Rectifying Channel Subfamily J Member 10), and among its related pathways/superpathways are Transport of inorganic cations/anions and amino acids/oligopeptides and Transmission across Chemical Synapses. The drugs Temsirolimus and Benzocaine have been mentioned in the context of this disorder. Affiliated tissues include kidney and liver, and related phenotypes are intellectual disability and seizure