This is an extremely rare disorder, characterized by Hidrotic ectodermal dysplasia and sensorineural hearing loss. So far, the association has only been reported in a single Lebanese family.
Mikaelian et al. (1970) described an affected brother and sister, born to cinsanguineous parents. Both patients had hidrotic ectodermal dysplasia, sensorineural hearing loss, and contracture of the fifth fingers.