Ectodermal Dysplasia And Neurosensory Deafness

Mikaelian et al. (1970) described brother and sister whose parents were first cousins and who had hidrotic ectodermal dysplasia, sensorineural hearing loss (due probably to a defect of cells of the organ of Corti which are of ectodermal origin), and contracture of the fifth fingers. The sister also had thoracic scoliosis.