Several mutations in Ccdc39, Celsr2, Celsr3, Cetn2, Dvls, FoxJ1, Hydin, Mdnah5, Pkd1, Tg737, Daple, Dnah14, Cfap43 and Cwh43 genes, which were related to the structure or function of motile cilia on ependymal cells, have been reported to develop hydrocephalus in animal models [21, 22, 34, 37–51]. Here, PKD1 is linked to Hydrocephalus.