Amelogenesis imperfecta with taurodontism.

Reports of families having a combination of amelogenesis imperfecta and taurodontism are limited. This study of members of three families shows that the combination is inherited as an autosomal dominant trait. In each of the patients examined, neither condition was seen without the other. The enamel was rough and dysplastic and varied in color from white to yellow. Radiographically, taurodontism was present in the deciduous and permanent dentitions. The pulp chambers of the incisor teeth were larger than is usually seen at all ages. All patients had normal-appearing hair, fingernails, and bones. The distinction between amelogenesis imperfecta with taurodontism and the tricho-dento-osseous syndrome is discussed.