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Article: Volume 42 Number 10 Page 927 - December 2015

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  Dent Update 2015; 42: 927-932

Paediatric dentistry:  Globodontia in the Otodental Syndrome: A Rare Defect of Tooth Morphology Occurring with Hearing Loss in an Eight-Year-Old

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Abstract: Otodental syndrome is a hereditary disorder comprising globodontia and sensorineural hearing loss. Globodontia is characterized by distinctively bulbous, enlarged crowns of molar and primary canine teeth. Anomalies including taurodontism and hypodontia also occur. We report on the dental treatment and multidisciplinary management of an eight-year-old girl with this rare condition. Referral to Clinical Genetics and Oral Pathology was instrumental in establishing a diagnosis of otodental syndrome for this young patient and her mother, who had similar dental defects.

Clinical relevance: To increase awareness among practitioners of this rare dental disorder and highlight the need for multidisciplinary management of such cases.

Author notes: Sinead Enright, BDentSci, MFDS RCSI, MPaedDent RCPSG, Salaried Dental Services, Bradford District Care Trust, Westbourne Green Community Health Centre, 50 Heaton Road, Bradford, BD8 8RA, A Karen Humphrys, BDS, MFDS RCS(Edin), MPaedDent, FDS(Paed Dent) RCS(Edin), Consultant in Paediatric Dentistry, Royal Belfast Hospital for Sick Children, Royal Victoria Hospitals, Belfast, Gillian Rea, MRCP, MA, Specialist Trainee in Clinical Genetics, Northern Ireland Regional Genetics Centre, Belfast City Hospital, Belfast and Jacqueline A James, BSc, PhD, BDS, FDS RCS(Edin), FRCPath, Senior Lecturer and Consultant in Pathology, Centre for Cancer Research and Cell Biology, Queen’s University Belfast, Belfast, UK.

Objective: To describe the features of otodental syndrome and highlight the need for its multidisciplinary management.