Cleidocranial dysplasia with hearing loss

Cleidocranial dysplasia is an inherited skeletal anomaly that affects primarily the skull, clavicle, and dentition, which can occur spontaneously, but most are inherited in autosomal dominant mode. The skull findings are brachycephaly, delayed or failed closure of the fontanelles, presence of open s...

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Published inJournal of natural science, biology and medicine Vol. 4; no. 1; pp. 245 - 249
Main Authors Candamourty, Ramesh, Venkatachalam, Suresh, Yuvaraj, Vaithilingam, Kumar, Ganesan
Format Journal Article
LanguageEnglish
Published India Medknow Publications Pvt Ltd 01.01.2013
Medknow Publications and Media Pvt. Ltd
Medknow Publications & Media Pvt. Ltd
Medknow Publications & Media Pvt Ltd
Subjects
Bones
Case Report
Dysplasia
Hearing loss
Scoliosis
Sinuses
Spinal manipulation
Teeth
delayed tooth eruption
hearing loss
hypoplastic clavicle
Cleidocranial dysostosis
supernumerary teeth
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Summary:Cleidocranial dysplasia is an inherited skeletal anomaly that affects primarily the skull, clavicle, and dentition, which can occur spontaneously, but most are inherited in autosomal dominant mode. The skull findings are brachycephaly, delayed or failed closure of the fontanelles, presence of open skull sutures and multiple wormian bones with pronounced frontal bossing. The syndrome is notable for aplasia or hypoplasia of the clavicles. The neck appears long and narrow and the shoulders markedly droop. Oral manifestations exhibit a hypoplastic maxilla with high-arched palate. Crowding of teeth is produced by retention of deciduous teeth, delayed eruption of permanent teeth, and the presence of a large number of unerupted supernumerary teeth. We report a case of CCD in a 12-year-old girl who presented with an unaesthetic facial appearance, unerupted permanent dentition with hearing loss.
ISSN:0976-9668
2229-7707
DOI:10.4103/0976-9668.107318