While most KCNQ1-associated LQTS cases are inherited in an autosomal dominant fashion, homozygous or compound heterozygous KCNQ1 mutations cause the autosomal recessive Jervell and Lange-Nielson syndrome (with a marked QT prolongation and congenital sensorineural deafness) or autosomal recessive Romano–Ward syndrome, without deafness but with severe QT interval prolongation [1,4]. The gene discussed is KCNQ1; the disease is Congenital sensorineural hearing impairment.