KCNE1 also regulates KCNQ1 in the inner ear, which is why some individuals harboring loss-of-function mutants in KCNQ1 or KCNE1 in both alleles succumb to Jervell and Lange-Nielsen Syndrome (JLNS), comprising both LQTS and sensorineural deafness. Here, KCNQ1 is linked to familial long QT syndrome.