Otodental syndrome

Otodental dysplasia, a form of ectodermal dysplasia, is characterized by abnormal dental crown morphology and sensorineural hearing loss. A variety of dental abnormalities are prominent features of this syndrome--most notably gigantic, bulbous posterior teeth. In this case, a sibling is identifies in a family with otodental syndrome that had been diagnosed earlier. A unique opportunity is provided to view the intact adult dentition and possible complications for dental treatment.