The aforementioned research on ZEB2 in NCC-derived ENS, together with novel insights in causal genes of HSCR around that time (for review, see [175]), and similarities in defects in other syndromes that originate in NCC-derived ENS (e.g., SOX10 in Waardenburg-Hirschsprung disease) inspired for phenotypic analysis of double Sox10;Zeb2 mouse mutants. This evidence concerns the gene ZEB2 and Waardenburg-Shah syndrome.