To ascertain if population variation in genes underlying monogenic long QT SCD syndromes is associated with SCA, tagSNPs spanning KCNQ1 (LQT1), SCN5A (LQT3), ANK2 (LQT4), KCNE1 (LQT5), KCNE2 (LQT6), KCNJ2 (LQT7), CACN1AC (LQT8), and CAV3 (LQT9) were examined. This evidence concerns the gene KCNJ2 and autosomal dominant cerebellar ataxia.