Jones syndrome

What causes Jones syndrome?

The exact, underlying genetic cause of Jones syndrome is not yet known.

Last updated on 05-01-20

How is Jones syndrome inherited?

Jones syndrome is inherited in an autosomal dominant manner. This means that having only one changed (mutated) copy of the responsible gene in each cell is enough to cause signs or symptoms of the condition. When a person with an autosomal dominant condition has children, each child has a 50% (1 in 2) risk to inherit the mutated copy of the gene from the affected parent.

Last updated on 05-01-20

What is Jones syndrome?

Jones syndrome is a very rare condition characterized by gingival fibromatosis (enlargement and overgrowth of the gums) and progressive, sensorineural hearing loss. The onset of gingival fibromatosis usually occurs with the eruption of the permanent teeth. Excessive growth of the gums may cause displacement of teeth, over-retention of primary teeth, and increased spacing. Jones syndrome is inherited in an autosomal dominant manner, but the underlying genetic cause is not yet known. Only a few families with Jones syndrome have been reported.

Last updated on 05-01-20

How might Jones syndrome be treated?

Due to the rarity of Jones syndrome, there are no treatment guidelines available in the medical literature. However, there is information about how the features associated with Jones syndrome might be treated.

Treatment for gingival fibromatosis varies depending on the severity. Maintaining good oral hygiene is very important. Surgery to remove the enlarged gum tissue in the mouth (gingivectomy) may be needed for functional and/or cosmetic reasons. Enlargement may recur to various extents, and repeated surgeries may be needed to reshape the gums. It has been recommended that whenever possible, this treatment should be performed after the complete eruption of permanent teeth.

The goal of treatment for sensorineural hearing loss is to improve hearing. People with sensorineural hearing loss may use hearing aids; telephone amplifiers and other assistive devices; sign language (for those with severe hearing loss); and/or speech reading (such as lip reading and using visual cues to aid communication). A cochlear implant may be recommended for some people with severe hearing loss.

Last updated on 05-01-20

Name: National Association of the Deaf 8630 Fenton Street Suite 820
Silver Spring, MD, 20910 , United States
Phone: +1-301-587-1788 TTY: +1-301-587-1789 Fax : +1-301-587-1791 Email: NADinfo@nad.org Url: https://www.nad.org/
Name: FACES: The National Craniofacial Association PO Box 11082
Chattanooga, TN, 37401, United States
Phone: 423-266-1632 Toll Free: 800-332-2373 Email: faces@faces-cranio.org Url: http://www.faces-cranio.org/
Name: Children's Craniofacial Association 13140 Coit Road Suite 517
Dallas, TX, 75240 , United States
Phone: +1-214-570-9099 Toll Free: 1-800-535-3643 Fax : +1-214-570-8811 Email: contactCCA@ccakids.com Url: https://ccakids.org/
Name: World Craniofacial Foundation P.O. Box 515838
Dallas, TX, 75251-5838, United States
Phone: 972-566-6669 Toll Free: 800-533-3315 Fax : 972-566-3850 Email: info@worldcf.org Url: http://www.worldcf.org/
Name: Hearing Loss Association of America 7910 Woodmont Avenue Suite 1200
Bethesda, MD, 20814, United States
Phone: +1-301-657-2248 Email: info@hearingloss.org Url: https://www.hearingloss.org/
Name: American Speech-Language-Hearing Association ASHA 2200 Research Boulevard
Rockville, MD, 20850-3289,
Phone: 301-296-5700 TTY: 301-296-5650 Toll Free: 800-638-8255 Fax : 301-296-8580 Email: http://www.asha.org/Forms/National-Office-Contact-Form/ Url: http://www.asha.org/
Name: National Institute of Dental and Craniofacial Research Building 31, Room 2C39 31 Center Drive, MSC 2290
Bethesda, MD, 20892,
Phone: 301-496-4261 Toll Free: 866-232-4528 Email: nidcrinfo@mail.nih.gov Url: http://www.nidcr.nih.gov/

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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