Stapedotomy in Osteogenesis Imperfecta: A Prospective Study of 23 Consecutive Cases

Abstract

To prospectively evaluate the hearing results in surgically treated cases of stapes fixation in patients with osteogenesis imperfecta. A prospective study of osteogenesis imperfecta patients with stapes fixation. One tertiary referral center. Eighteen patients (23 ears) who underwent stapes surgery from 1994 to 2004 were prospectively included. Stapedotomy with vein graft interposition and reconstruction with a Teflon piston or a bucket handle (cup) prosthesis. Preoperative and postoperative audiometric evaluation using conventional audiometry. Air-bone gap, bone-conduction thresholds, and air-conduction thresholds were measured. Postoperative audiometry was performed at 6, 9, 12, 18, and 24 months after surgery and at a yearly interval thereafter. Overall, a postoperative air-bone gap closure to within 10 dB was achieved in 85.7% of cases. Postoperative improvement of air-conduction thresholds superior to 20 dB was found in 57% of cases. The postoperative bone-conduction thresholds were unchanged. This study shows that safe and successful stapedotomy is possible in cases of stapes fixation in patients with osteogenesis imperfecta.