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Gingival fibromatosis-progressive deafness syndrome

Disease definition

A rare syndrome 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.

ORPHA:2027

Classification level: Disorder
  • Synonym(s):
    • Gingival fibromatosis-progressive hearing loss syndrome
    • Jones syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Adult
  • ICD-10: H90.3
  • OMIM: 135550
  • UMLS: C1851112
  • MeSH: -
  • GARD: 3056
  • MedDRA: -

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