Jones Syndrome
| Classification according to ICD-10 | |
|---|---|
| H90.3 | Bilateral hearing loss due to sensorineural disturbance |
| ICD-10 online (WHO version 2019) | |
The Jones syndrome is a very rare congenital disease with the main features of a fibromatosis of the gingiva and an increasing sensorineural hearing loss .
It is a special form of hereditary gingival fibromatosis .
The name refers to the first author of the first description from 1977 by G. Jones .
Spread and cause
The frequency is given as less than 1 in 1,000,000, so far two families have been described over several generations. The inheritance is autosomal dominant .
A non-familial variant has been described.
Clinical manifestations
Clinical features are:
- Manifestation in adulthood
- Slow overgrowth of the gums with the appearance of permanent teeth with possible displacement of teeth
- Slow increase in hearing loss
Individual evidence
- ↑ a b c Fibromatosis, gingival - progressive hearing loss. In: Orphanet (Rare Disease Database).
- ↑ G. Jones, RS Wilroy, V. McHaney: Familial gingival fibromatosis associated with progressive deafness in five generations of a family. In: Birth defects original article series. Vol. 13, No. 3B, 1977, pp. 195-201, PMID 890092 .
- ↑ B. Gita, S. Chandrasekaran, P. Manoharan, G. Dembla: Idiopathic gingival fibromatosis associated with progressive hearing loss: A nonfamilial variant of Jones syndrome. In: Contemporary clinical dentistry. Vol. 5, No. 2, April 2014, pp. 260-263, doi: 10.4103 / 0976-237X.132387 , PMID 24963260 , PMC 4067797 (free full text).
- ↑ O. Kasaboglu, C. Hallows, S. Balci: Hereditary gingival fibromatosis and sensorineural hearing loss in a 42-year-old man with Jones syndrome. In: Genetic counseling (Geneva, Switzerland). Vol. 15, No. 2, 2004, pp. 213-218, PMID 15287422 .
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