Ectodermal dysplasia-sensorineural deafness syndrome is characterized by hidrotic ectodermal dysplasia, sensorineural hearing loss, and contracture of the fifth fingers. It has been described in brother and sister born to consanguineous parents. The girl also presented with thoracic scoliosis. The mode of inheritance is likely to be autosomal recessive. The disease is Monarch Disease Ontology term MONDO_0009146 (ectodermal dysplasia-sensorineural deafness syndrome). Also known as: ectodermal dysplasia-sensorineural hearing loss syndrome.