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"Otodental" dysplasia.

R J Chen1, H S Chen, L M Lin

  • 1Department of Oral Pathology, School of Dentistry, Kaohsiung Medical College, Taiwan, Republic of China.

Oral Surgery, Oral Medicine, and Oral Pathology
|September 1, 1988
PubMed
Summary

This case report details a young boy with otodental dysplasia, highlighting unique dental anomalies like globe-shaped and taurodontic molars, and supernumerary teeth. Hearing remained normal throughout the observation period.

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Area of Science:

  • Dentistry
  • Genetics
  • Otolaryngology

Background:

  • Otodental syndrome is a rare genetic disorder linking hearing impairment with dental abnormalities.
  • This report focuses on a specific pediatric case presenting with a unique constellation of otodental dysplasia features.

Observation:

  • A 3-year-old Chinese boy exhibited delayed eruption of globe-shaped molars, bulbous deciduous canines, and double pulp chambers.
  • Follow-up radiographs revealed taurodontic molars, supernumerary microdontic teeth, retarded premolar formation, and possible aplasia of mandibular second premolars.

Findings:

  • The patient presented with significant dental anomalies characteristic of otodental dysplasia.
  • Despite dental abnormalities, the patient's hearing function was normal.
  • Progressive dental anomalies were observed over a four-year period.

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Implications:

  • This case expands the phenotypic spectrum of otodental syndrome.
  • Highlights the importance of comprehensive dental and audiological evaluation in suspected cases.
  • Contributes to understanding the variable expressivity of genetic dental disorders.