The vast majority of Kv7.1 variants are associated with dominantly inherited long QT syndrome (LQTS), but some are inherited recessively and also associated with hearing loss in addition to LQTS (Jervell and Lange-Nielsen syndrome, JLNS) (Neyroud et al., 1997). This evidence concerns the gene KCNQ1 and Jervell and Lange-Nielsen syndrome.