Stapedotomy in osteogenesis imperfecta: a prospective study of 23 consecutive cases.

OBJECTIVE

To prospectively evaluate the hearing results in surgically treated cases of stapes fixation in patients with osteogenesis imperfecta.

STUDY

DESIGN

A prospective study of osteogenesis imperfecta patients with stapes fixation.

SETTING

One tertiary referral center.

PATIENTS: Eighteen patients (23 ears) who underwent stapes surgery from 1994 to 2004 were prospectively included.

INTERVENTION: Stapedotomy with vein graft interposition and reconstruction with a Teflon piston or a bucket handle (cup) prosthesis.

MAIN OUTCOME MEASURES: 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.

RESULTS

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.

CONCLUSION

This study shows that safe and successful stapedotomy is possible in cases of stapes fixation in patients with osteogenesis imperfecta.