Otorhinolaryngology Department, Hanover Medical University, Hanover, Germany.
Otol Neurotol. 2011 Jun;32(4):589-95. doi: 10.1097/MAO.0b013e318213b0f1.
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).
Retrospective case series.
Tertiary referral ear center.
A family with genetically proven OI Type I.
Two patients, mother and son, with severe to profound mixed hearing loss underwent 3 power stapes, 1 unilateral and 1 bilateral sequential.
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.
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.
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.
介绍在一步手术中进行功率镫骨、镫骨切开术和中耳植入 Vibrant SoundBridge（VSB）作为成骨不全症（OI）患者听力康复的替代选择。
回顾性病例系列。
三级转诊耳中心。
一个经基因证实的 I 型 OI 家族。
两名患者，母亲和儿子，患有严重至重度混合性听力损失，进行了 3 次功率镫骨，1 次单侧和 1 次双侧连续手术。
使用全面的听力诊断电池，包括辅助和未辅助的纯音和自由场听力测试以及弗莱堡单音节词测试，评估术前状态和术后听力结果。还进行了颞骨高分辨率计算机断层扫描。详细分析了手术过程和任何特殊注意事项。
所有病例的听力结果均良好，与术前值相比，平均提高了 36.8dB。唯一的并发症是中耳黏膜严重术中出血，但通过允许短时间间隔，很容易控制。在任何情况下都没有发生内耳创伤。
功率镫骨是治疗 OI 患者选择听力损失的一种安全且有前途的方法。此外，它在伴有严重耳硬化症和骨导阈值升高的混合性听力损失病例中引入了一种新的、有利的 VSB 应用。
