Moreover, Pax3 cKOs demonstrated that NC-specific deletion is sufficient to cause DORV/VSDs and death at birth (Olaopa et al., 2011; Koushik et al., 2002), and that restricted deletion within the neuroepithelium causes congenital hydrocephalus (Zhou and Conway, 2016). This evidence concerns the gene PAX3 and congenital hydrocephalus.