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Gingival fibromatosis - progressive deafness

Orpha number ORPHA2027
Synonym(s) Jones syndrome
Prevalence <1 / 1 000 000
Inheritance
  • Autosomal dominant
Age of onset Adulthood
ICD-10
  • H90.3
OMIM
UMLS -
MeSH -
MedDRA -
SNOMED CT -

Summary

This syndrome is characterized by gingival fibromatosis associated with progressive sensorineural hearing loss. It has been described in two families (with at least 16 affected members spanning five generations in one of the families, and five affected members spanning three generations in the other family). It is transmitted as an autosomal dominant trait.


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