Otosclerosis associated with Ehlers-Danlos syndrome: report of a case.

Abstract

Hearing loss occurs rarely in Ehlers-Danlos syndrome (EDS). We report a case of a 24-year-old woman with EDS who had conductive deafness due to otosclerosis and scar tissues around the malleus and incus. The scar was considered to develop by inflammatory response specific to EDS that had been induced by otitis media with effusion. Following stapes mobilization and removal of the scar tissues, her hearing was improved for several months but showed deterioration thereafter, probably due to recurrence of ossicular fixation. The tendency toward scar formation, infection, and inflammatory response, especially to foreign bodies, in EDS needs to be kept in mind to determine treatment of choice in patients with EDS and conductive hearing loss.