KCNQ1 and familial long QT syndrome: The significance of Kv7.1 functions in the heart and inner ear underscore several mutations which result in a cardio-auditory syndrome called Jervell and Lange-Nielsen syndrome (JLNS) (Jervell and Lange-Nielsen, 1957), as well as over 200 mutations that are reported to cause long QT syndrome (LQTS) without the hearing phenotype, as seen in Romano–Ward syndrome (RWS) (Morita et al., 2008; Hedley et al., 2009).