“…In the mouse, maintenance of the endocochlear potential depends on expression of the potassium channel Kcnj10 in intermediate cells (Marcus et al, 2002;Wangemann et al, 2004). Melanocytes also populate vestibular regions and the endolymphatic apparatus in the mammalian ear (Masuda et al, 1994;Escobar et al, 1995;Peters et al, 1995;Stanchina et al, 2006), but their role here is less clear; for example, mutations in Kcnj10 have no effect on vestibular endolymph (Marcus et al, 2002). Analysis of the inner ear phenotype in murine models of WS and other auditory-pigmentary disorders has focused on the presence of intermediate cells in the stria vascularis; deafness is usually attributed to the loss of intermediate cells in this tissue, leading to a reduction or collapse of endolymph volume, a loss of the endocochlear potential and subsequent hair cell degeneration (Tachibana et al, 1992;Cable et al, 1994;Matsushima et al, 2002;Stanchina et al, 2006) (reviewed by Tachibana, 1999;Tachibana et al, 2003).…”