In mouse, mutations in genes expressed by marginal cells of the SV, including Slc12a2, Atp1b2, Kcnq1, and Kcne1, result in a loss or reduction of EP and deafness (Delpire et al., 1999; Dixon et al., 1999; Wangemann, 2002; Chang et al., 2015; Faridi et al., 2019; McNeill et al., 2020; Mutai et al., 2020). The gene discussed is SLC12A2; the disease is deafness.