Power stapes: an alternative method for treating hearing loss in osteogenesis imperfecta?

OBJECTIVE

To present power stapes, stapedotomy, and middle ear implantation with Vibrant SoundBridge (VSB) performed in a one-step surgery as an alternative option for hearing rehabilitation in patients with osteogenesis imperfecta (OI).

STUDY DESIGN

Retrospective case series.

SETTING

Tertiary referral ear center.

PATIENTS

A family with genetically proven OI Type I.

INTERVENTIONS

Two patients, mother and son, with severe to profound mixed hearing loss underwent 3 power stapes, 1 unilateral and 1 bilateral sequential.

MAIN OUTCOME MEASURES

Thorough audiological diagnostic batteries including aided and unaided pure-tone and free-field audiometry and Freiburg monosyllabic word test were used to assess the preoperative status and the postoperative hearing outcome. High-resolution computed tomography of the temporal bones was performed as well. Surgical procedure and any special considerations were analyzed in detail.

RESULTS

The hearing outcome was favorable in all cases, showing in comparison to the preoperative values an average improvement of 36.8 dB. Severe intraoperative bleeding of the middle ear mucosa was the only complication and could be easily controlled by allowing short time intervals. Inner ear trauma did not occur in any case.

CONCLUSION

Power stapes represents a safe and promising procedure for treating hearing loss in selected patients with OI. Furthermore, it introduces a new, advantageous VSB application in cases of mixed hearing loss with severe otosclerosis and increased bone-conduction thresholds.