Orphanet: Otodental syndrome

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Otodental syndrome

Disease definition

Otodental syndrome is a very rare inherited condition characterized by grossly enlarged canine and molar teeth (globodontia) associated with sensorineural hearing loss.


  • Synonym(s):
    • Globodontia
    • Otodental dysplasia
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood
  • ICD-10: K00.2
  • OMIM: 166750
  • UMLS: C1833693
  • MeSH: -
  • GARD: 4168
  • MedDRA: -

Detailed information


Additional information

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