GJB2 and Vascular dilatation: Although morphological brain anomalies have not been reported in HED2 patients, on occasion patients with connexin 26 (Cx26; also known as GJB2 when referring to the gene)-linked keratitis-ichthiosis-deafness (KID) syndrome present with Dandy–Walker malformation resulting in cystic dilatation of the fourth ventricle and hydrocephalus (Todt et al., 2009), linking connexin status to CSF homeostasis.