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. Here, GJB2 is linked to KID syndrome.