Cochlear implantation through retro-facial approach with congenital microtia malformation with facial nerve deformity: a case report
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摘要: 先天性小耳畸形患者行人工耳蜗植入时通常因为解剖标识不清、面神经畸形使手术难度增加。面神经畸形的常见类型有面神经骨管缺失、面神经走行异常及面神经分叉畸形。面神经分叉畸形可能导致面神经遮蔽卵圆窗挤压镫骨、面神经于前庭窗分叉或遮蔽圆窗，正确识别面神经，选择合理的手术径路对于避免术后并发症有重要意义。本文介绍1例在我中心治疗的先天性外中耳畸形合并面神经畸形极重度感音神经性聋患者，行面后径路人工耳蜗植入手术，植入过程顺利，术后定期开机，按时调机。术后2年无面瘫、面肌痉挛等面神经损伤或功能异常症状，人工耳蜗正常使用。经听觉行为分级(CAP)问卷评分7分，言语可懂度分级(SIR)问卷评分4分。人工耳蜗植入术中无法经面隐窝入路暴露圆窗时，面后径路是可行手段，在为面神经畸形患者行中、内耳手术时，为避免出现面神经损伤可在术前完善颞骨薄层CT明确面神经走行，采用术中面神经监测仪明确神经形态，避免损伤面神经。Abstract: The difficulty of cochlear implantation in patients with congenital microtia is usually increased due to the vague anatomical marks and facial nerve malformation. The common types of facial nerve malformation include facial nerve bony cover loss, aberrant position, and bifurcation malformation. Bifurcation malformation may obscure the oval window, press against stapes, and bifurcate in the vestibular window while obscuring the round window. It is important to correctly identify the facial nerve and choose a reasonable surgical approach to avoid postoperative complications. This article describes a case of profound sensorineural hearing loss due to facial nerve malformation in our institution. The patient underwent cochlear implantation through the retro-facial approach. There was no facial nerve injury or dysfunction symptoms such as facial paralysis and hemifacial spasm 2 years after the operation, and the cochlear implant works well. The score of the categories of the auditory performance(CAP) questionnaire was 7, and the score of the speech intelligibility rating(SIR) questionnaire was 4. When the round window cannot be exposed through the facial recess approach during surgery, the retro-facial approach is a feasible method. To avoid facial nerve injury, a thin-section CT of the temporal bone should be performed before the middle and inner ear surgery for patients with facial nerve malformation, and the intraoperative facial nerve monitor should be used to clarify the course of the facial nerve to avoid injury.
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Key words:
- microtia /
- facial nerve malformation /
- retro-facial approach /
- cochlear implant
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