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Otodental syndrome

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....

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Detalles Bibliográficos
Autores principales: Bloch-Zupan, Agnès, Goodman, Jane R
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2006
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1459122/
https://www.ncbi.nlm.nih.gov/pubmed/16722606
http://dx.doi.org/10.1186/1750-1172-1-5
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author Bloch-Zupan, Agnès
Goodman, Jane R
author_facet Bloch-Zupan, Agnès
Goodman, Jane R
author_sort Bloch-Zupan, Agnès
collection PubMed
description 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.
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spelling pubmed-14591222006-05-11 Otodental syndrome Bloch-Zupan, Agnès Goodman, Jane R Orphanet J Rare Dis Review 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. BioMed Central 2006-03-21 /pmc/articles/PMC1459122/ /pubmed/16722606 http://dx.doi.org/10.1186/1750-1172-1-5 Text en Copyright © 2006 Bloch-Zupan and Goodman; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( (http://creativecommons.org/licenses/by/2.0) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review
Bloch-Zupan, Agnès
Goodman, Jane R
Otodental syndrome
title Otodental syndrome
title_full Otodental syndrome
title_fullStr Otodental syndrome
title_full_unstemmed Otodental syndrome
title_short Otodental syndrome
title_sort otodental syndrome
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1459122/
https://www.ncbi.nlm.nih.gov/pubmed/16722606
http://dx.doi.org/10.1186/1750-1172-1-5
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