Gingival fibromatosis-progressive deafness syndrome

Gingival fibromatosis-progressive deafness syndrome, also known as Jones syndrome or gingival fibromatosis with sensorineural hearing loss, is a rare genetic disorder characterized by the combination of gingival fibromatosis (progressive overgrowth of the gum tissue) and sensorineural hearing loss that worsens over time. The gingival overgrowth typically becomes apparent during childhood, often around the time of eruption of the permanent teeth, and can be severe enough to cover the teeth partially or completely, interfering with dental alignment, chewing, and oral hygiene. The sensorineural hearing loss affects the inner ear or auditory nerve pathways and tends to be progressive, potentially leading to significant hearing impairment. The condition primarily affects two body systems: the oral cavity (gingival tissues) and the auditory system. The gingival fibromatosis is characterized by firm, non-tender, pink enlargement of the gums that may recur even after surgical reduction (gingivectomy). The hearing loss is bilateral and sensorineural in nature, distinguishing it from conductive hearing loss. There is no known cure for this syndrome. Management is symptomatic and includes periodic surgical removal of excess gingival tissue (gingivectomy) to maintain oral function and hygiene, along with hearing aids or other auditory rehabilitation strategies to address the progressive hearing loss. Regular dental and audiological follow-up is essential for optimal management. Genetic counseling is recommended for affected families.

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