A 12-year-old boy with characteristic findings of otodental syndrome is presented. Abnormalities of deciduous and permanent dentition included bulbous canines, globe-shaped posterior teeth and agenesis of maxillary premolars. High-frequency sensorineural hearing loss was demonstrated, and results of genetic studies indicated an autosomal dominant trait. This patient presented previously unreported findings, including generalized macrodontia, delayed mineralization of the mandibular premolars, and supplementary permanent maxillary canines.
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Hearing impairments and dental anomalies are found in many genetic syndromes. Otodental syndrome is a rare combination of hearing loss and the presence of a pathognomonic dental phenotype known as globodontia, in which the tooth exhibits an abnormal globe shape. There is no histologic evidence of structural anomalies in the enamel, dentin, or pulp.
View Article and Find Full Text PDFOtodental Syndrome.
Eur J Paediatr Dent
September 2023
Department of Life, Health and Enviromental Science, Paediatric Dentistry, University of L'Aquila, L'Aquila Italy.
Otodental syndrome is a rare autosomal dominant condition characterised by a dental phenotype known as globodontia often associated with high-frequency hearing loss. Globodontia occurs both in the decidous and permanent dentition and affects canine and molar teeth.
View Article and Find Full Text PDFOtodental syndrome: Case report and differential diagnosis with Treacher Collins syndrome.
Eur J Paediatr Dent
March 2022
Department of Pediatric Dentistry, Istituto Stomatologico Italiano, Milan, Italy.
Background: Otodental syndrome and Treacher Collins syndrome are rare diseases that have similar clinical features, which can complicate the diagnostic process. These syndromes cause skeletal and dental abnormalities, the differential diagnosis can be based on clinical signs but only the genetic analysis can confirm it. The aim of this case report is to describe and compare clinical signs of these syndromes.
View Article and Find Full Text PDFThree years of follow-up of otodental syndrome in 3-year-old Chinese boy: a rare case report.
BMC Oral Health
July 2019
Key Laboratory of Oral Medicine, Guangzhou Institute of Oral Disease, Affiliated Stomatology Hospital of Guangzhou Medical University, Guangzhou, 510140, China.
Background: Otodental syndrome is an exceptionally rare autosomal dominant condition characterized by a delayed eruption of posterior teeth, globodontia, lisping, and sensorineural hearing loss. In this case report, we reported a 3-year-old Chinese boy with the otodental syndrome.
Case Presentation: A 3-year-old Chinese boy was referred to our hospital with complaint of no eruption of primary canines and molars.
Clinical, pathological, and genetic evaluations of Chinese patient with otodental syndrome and multiple complex odontoma: Case report.
Medicine (Baltimore)
February 2017
State Key Laboratory of Military Stomatology & National Clinical Research Center for Oral Diseases & Shaanxi Clinical Research Center for Oral Diseases, Department of Pediatric Dentistry, School of Stomatology Department of Dentistry, Hospital of Tangdu, Fourth Military Medical University, Xi'an, Shaanxi Province, China.
Otodental syndrome is a rare autosomal-dominant disease characterized by globodontia, associated with sensorineural, high-frequency hearing loss. Here, we describe the clinical, pathological, and genetic evaluations of a 9-year-old girl with otodental syndrome and multiple complex odontoma.The patient presented with a draining sinus tract in her left cheek, globodontia, and hearing loss.
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