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自1983年～1987年作者为7例严重传导性耳聋患者施行中耳探查术时发现前庭窗缺如。认为与面神经发育异常有关。胚胎第五周至六周时,如第一鳃弓发育不全可使第二鳃弓超越移位,引起面神经前移至前庭窗上,遮盖前庭窗区,至使前庭窗缺如。本文7例中有4例面神经畸形,表现为镫骨缺失或仅呈残迹型。作者施行前庭开窗术并用 House 钢丝重建听骨链。2例前庭开窗后用 TORP 重建听骨链,1例因中耳内壁缺乏手术标志仅行手术探查。术后4例初期听力提高20～45dB,之后所获听力进步又大部分丧失。随访2年以上最高语言接受阀(SRT)为40dB。术后无并发症发生。作者将近期报道的一组先天性外耳道闭锁患者术后听力与之比较,发现前庭窗缺如组手术效果差,其原因为面神经畸形及砧骨长突畸形使手术复杂
From 1983 to 1987, the author found seven cases of severe conduction deafness in the middle ear exploration and found that the absence of the vestibular window. That facial nerve development and abnormalities. Embryos from the fifth week to the sixth week, such as the first gill arch hypoplasia can make the second gill arch beyond the shift, causing the facial nerve to move forward to the vestibular window, covering the vestibular window area, to the absence of the vestibular window. In this paper, 4 cases of facial nerve deformity in 7 cases, the performance of tarsal bone missing or only residual type. The author practiced the vestibular fenestration and reconstruction of the ossicular chain with House wire. Two cases of rectal ossicular chain were reconstructed with TORP after fenestration, and only one case was operated because of the lack of surgical sign on the inner ear wall. After 4 cases of early hearing increased by 20 ~ 45dB, after hearing most of the progress was lost. Followed up for 2 years or more The highest language acceptance valve (SRT) was 40dB. No postoperative complications. The authors recently reported a group of patients with congenital external auditory canal atresia compared with the hearing and found that the absence of the vestibular surgery group poor results due to facial nerve deformity and ankle synopsis deformity complicated surgery