Jahrsdoerfer R A, Aguilar E A, Yeakley J W, Cole R R
Dept of Otolaryngology-Head and Neck Surgery, University of Texas Medical School-Houston 77030.
Ann Otol Rhinol Laryngol. 1989 Oct;98(10):807-12. doi: 10.1177/000348948909801011.
Patients with Treacher Collins syndrome have severe middle ear malformations that render operation difficult. We have evaluated 43 patients with Treacher Collins syndrome, on whom only 11 were operated. Computed tomography, the single most important study done preoperatively, routinely showed an underdeveloped temporal bone with islands of bone marrow and absent mastoid pneumatization. The middle ear space was often underdeveloped. Ossicular dysjunction was often noted in which the fused malleus/incus remnant was found 3 to 4 mm distant to the stapes. A common finding was severe dysplasia of the stapes-facial nerve complex that often made the middle ear malformation uncorrectable. Hearing results were much less predictable than in patients with isolated atresia/stenosis of the ear.
患有特雷彻·柯林斯综合征的患者存在严重的中耳畸形，这使得手术难度很大。我们评估了43例特雷彻·柯林斯综合征患者，其中仅11例接受了手术。计算机断层扫描是术前进行的最重要的单项检查，通常显示颞骨发育不全，伴有骨髓岛且乳突气化缺失。中耳腔常常发育不全。经常发现听骨脱节，其中融合的锤骨/砧骨残端距镫骨3至4毫米。一个常见的发现是镫骨-面神经复合体严重发育异常，这常常使中耳畸形无法矫正。听力结果比单纯性耳闭锁/狭窄患者更难以预测。
