Stapedotomy in osteogenesis imperfecta patients

Osteogenesis imperfecta (OI) is a connective tissue disorder characterized by osseous fragility, blue sclerae and hearing loss. In order to assess the impact of stapedotomy on improving hearing on OI, a retrospective, one-gro up, pre-test-post-test design was used to compare the pre-operative and pos toperative audiograms of nine OI patients, treated with stapedotomy for the ir mixed hearing loss. Operative findings included fixation or thickening o f the stapes footplate with normal superstructure configuration and hyperva scularization of the promontory mucosa. Immediate post-operative results sh owed a significant improvement (p<0.05) from 250-4000 Hz in air conduction and from 250-2000 Hz in bone conduction. A significant closure of the air-b one gap between 250-2000 Hz was also achieved (p<0.05). The long-term resul ts remained satisfactory with a mean threshold shift of 8 dB HL and an almo st unchanged air-bone gap. These satisfactory results and the lack of compl ications make stapedotomy an appealing method for the management of OI-asso ciated hearing loss.