Comparison to other mouse lines also indicates that the endolymphatic collapse observed in SpotTg/Tg vestibular sensory epithelia is similar to what has been described in the cochlea of Mitf−/− and Ednrb−/− models of Waardenburg syndrome type 1 and type 4, respectively (Koide et al., 1998; Matsushima et al., 2002; Tachibana et al., 1992). Here, EDNRB is linked to Waardenburg syndrome type 1.