ABSTRACT
BACKGROUND: Usher syndrome (USH) is a group of autosomal recessive diseases characterized by the association of retinitis pigmentosa with sensorineural hearing loss. There are three types of USH. In addition, in people with USH and hypoplasia, the thickness of the enamel is reduced. CASE DESCRIPTION: The authors describe a case of a patient with USH type II associated with severe enamel hypoplasia and multiple unerupted teeth. The authors placed direct composite crowns and extracted severely affected and impacted molars. CLINICAL IMPLICATIONS: There is little information available on the oral pathologies of USH. Because the authors did not know how the patient's condition would progress and the patient still was growing, the authors treated the patient conservatively by placing direct composite crowns. The treatment has met both esthetic and functional expectations for 10 years.
