KCNJ10 and EAST syndrome: The deafness and abnormal renal excretion of electrolytes in the EAST/SeSAME syndrome (Table 2) seem to be attributable to Kir4.1 dysfunction in the inner ear and renal epithelial cells, respectively, since Kir4.1 channels are involved in the maintenance of endocochlear potential and the electrolytes transport (e.g., K+) in the scala media and the distal convoluted tubules [55,56].