Stapedotomy in osteogenesis imperfecta: a prospective study of 32 consecutive cases.

Abstract

To prospectively evaluate hearing outcomes in patients with osteogenesis imperfecta undergoing primary stapes surgery and to isolate prognostic factors for success. A nonrandomized, open, prospective case series. A tertiary referral center. Twenty-five consecutive patients who underwent 32 primary stapedotomies for osteogenesis imperfecta with evidence of stapes fixation and available postoperative pure-tone audiometry. Primary stapedotomy with vein graft interposition and reconstruction with a regular Teflon piston or bucket handle-type piston. Preoperative and postoperative audiometric evaluation using conventional 4-frequency (0.5, 1, 2, and 4 kHz) audiometry. Air-conduction thresholds, bone-conduction thresholds, and air-bone gap were measured. The overall audiometric results as well as the results of audiometric evaluation at 3 months and at least 1 year after surgery were used. Overall, postoperative air-bone gap closure to within 10 dB was achieved in 88% of cases. Mean (standard deviation) gain in air-conduction threshold was 22 (9.4) dB for the entire case series, and mean (standard deviation) air-bone gap closure was 22 (9.0) dB. Backward multivariate logistic regression showed that a model with preoperative air-bone gap closure and intraoperatively established incus length accurately predicts success after primary stapes surgery. Stapes surgery is a feasible and safe treatment option in patients with osteogenesis imperfecta. Success is associated with preoperative air-bone gap and intraoperatively established incus length.