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Intrathecal Application of a Fluorescent Dye for the Identification of Cerebrospinal Fluid Leaks in Cochlear Malformation

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Cleidocranial dysplasia with hearing loss.

Ramesh Candamourty¹, Suresh Venkatachalam, Vaithilingam Yuvaraj

¹Department of Oral and Maxillofacial Surgery, Indira Gandhi Institute of Dental Sciences, Mahatma Gandhi Medical College and Research Institute Campus, Pillaiyarkuppam, Pondicherry, India.

Journal of Natural Science, Biology, and Medicine

|May 2, 2013

Summary

Cleidocranial dysplasia (CCD) is a genetic disorder affecting skeletal development, notably the skull, clavicles, and teeth. This case highlights typical CCD features in a young girl, including dental issues and hearing loss.

Keywords:

Cleidocranial dysostosis delayed tooth eruption hearing loss hypoplastic clavicle supernumerary teeth

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Area of Science:

Genetics
Skeletal Biology
Dentistry

Background:

Cleidocranial dysplasia (CCD) is an inherited skeletal disorder.
It typically follows autosomal dominant inheritance but can occur spontaneously.
CCD primarily affects the skull, clavicles, and dentition.

Observation:

Skull abnormalities include brachycephaly, delayed fontanelle closure, open sutures, and frontal bossing.
Clavicular hypoplasia or aplasia is a hallmark feature.
Oral manifestations include a hypoplastic maxilla, high-arched palate, and significant dental anomalies.

Findings:

The case involves a 12-year-old girl with CCD.
She presented with unaesthetic facial appearance and unerupted permanent dentition.
Hearing loss was also noted as a co-occurring condition.

Implications:

This case underscores the diverse clinical manifestations of CCD.
Early diagnosis and management are crucial for addressing skeletal and dental issues.
Further research into associated conditions like hearing loss in CCD is warranted.