title: Treatment of enamel hypoplasia in a patient with Usher syndrome.
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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: School of Dentistry, University of Santiago de Compostela, Dr. Teixeiro 11, 4 dcha., 15701 Santiago de Compostela, A Coruña, Spain. dentalinvestigation@yahoo.es
authors.forename: Victor Alonso
authors.initials: VA
authors.lastname: de la Peña
authors.forename: Martín Caserío
authors.initials: MC
authors.lastname: Valea
mesh_terms: D000293:Adolescent; D003188:Composite Resins / Q000737:chemistry; D003442:Crowns; D003744:Dental Enamel Hypoplasia / Q000628:therapy*; D003764:Dental Materials / Q000737:chemistry; D017267:Dental Prosthesis Design; D004955:Esthetics, Dental; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D016876:Metal Ceramic Alloys / Q000737:chemistry; D008963:Molar / Q000601:surgery; D012390:Root Canal Therapy; D014081:Tooth Extraction; D014095:Tooth, Impacted / Q000601:surgery; D052245:Usher Syndromes / Q000473:pathology*
chemical_list: D003188:Composite Resins; D003764:Dental Materials; D016876:Metal Ceramic Alloys