Abstract
The otodental syndrome also named otodental dysplasia, is characterised by a striking dental phenotype known as globodontia, associated with sensorineural high frequency hearing loss and eye coloboma. Globodontia occurs in both primary and permanent dentition, affecting canine and molar teeth (i.e. enlarged bulbous malformed posterior teeth with almost no discernable cusps or grooves). The condition appears to be inherited in an autosomal dominant mode, although sporadic cases have been reported. It is a rare disease, a few families have been described in the literature. In the British family, the locus for oculo-oto-dental syndrome was mapped to 20q13.1 within a 12-cM critical chromosomal region. Dental management is complex, interdisciplinary and will include regular follow up, scheduled teeth extraction and orthodontic treatment. Hearing checks and, if necessary, hearing aids are mandatory, as well as eye examination and ad hoc treatment if necessary.
Highlights
Globodontia; the term globodontia refers to the enlarged bulbous fused malformed posterior teeth with almost no discernable cusps or grooves [4,5,6];
The Otodental syndrome has been described under various names:
In some families, an associated ocular phenotype was recognized [7] and it was named as the Oculo-Oto-Dental syndrome (OOD) [8]
Summary
Address: 1Faculté de Chirurgie Dentaire de Strasbourg, Université Louis Pasteur Hôpital Civil, 1 place de l'Hôpital 67000 Strasbourg, France and 2Eastman Dental Institute, University College London, 256 Gray's Inn Road, London, WC1X 8LD, UK. Published: 21 March 2006 Orphanet Journal of Rare Diseases2006, 1:5 doi:10.1186/1750-1172-1-5
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