Jones syndrome H 90.3

Jones G 1977

Very rare, hereditary (autosomal-dominant inheritance) but also sporadically occurring disease characterized by gingival fibromatosis and a progressive sound sensitivity disorder (Gita S et al. 2014).

Prevalence is given as < 1: 1,000,000. So far, two families have been described over several generations ( Jones RS et al. 1977).

Slow fleshy hyperplasia of the gingiva that begins with the appearance of permanent teeth. Possible displacement of teeth. Gradual reduction of hearing.

1. Gita S et al (2014) Idiopathic gingival fibromatosis associated with progressive hearing loss: A nonfamilial variant of Jones syndrome. Contemporary clinical dentistry 5: 260-263.
2. Jones RS et al (1977) Familial gingival fibromatosis associated with progressive deafness in five generations of a family. Birth defects original article series. 13: 195–201.