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  • Title: [Selection of inner ear fenestration strategy and surgical effect of patients with oval window atresia accompanied by facial nerve aberration].
    Author: Chen ZR, Tang RW, Xie J, Guo JY, Zhao PF, Yang ZJ, Wang GP, Gong SS.
    Journal: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi; 2024 Sep 07; 59(9):902-908. PubMed ID: 39289957.
    Abstract:
    Objective: To summarize the clinical features and postoperative efficacy of patients with oval window atresia accompanied by facial nerve aberration. Methods: The clinical data of patients with congenital middle ear malformation with facial nerve aberration admitted to our hospital from January 2015 to March 2023 were retrospectively analyzed. There were 97 cases (133 ears) in total. Among them, 39 patients (44 ears) had complete follow-up data, including 27 male patients and 12 females, aged 7-48 years old, with an average age of 17.8 years old. Of these, 14 cases (16 ears) were patients combined with facial nerve aberration, and 25 cases (28 ears) were without facial nerve aberration. The results of imaging examination, pure-tone audiometry, selection of surgical strategy, intraoperative findings and postoperative hearing improvement were summarized and analyzed. The malformations of malleus, incus, stapes, oval window and facial nerve were recorded. Prism 9 software was used to statistically analyze the mean bone conductance and air-bone gap of patients before and after surgery. Results: All the 14 patients (16 ears) with middle ear malformation accompanied by facial nerve aberration and oval window atresia showed poor hearing and no facial palsy since childhood. High resolution CT (HRCT) examination of temporal bone, pure tone audiometry and Gelle test were performed before surgery. The malformations of malleus, incus, stapes, oval window and facial nerve were recorded. Preoperative high-resolution CT (HRCT) examination of temporal bone found 12 ears with 4 or more deformities, accounting for 75.00%, in the group of patients with facial nerve malformation. The preoperative average bone conductive threshold was (15.3±10.4) dB and the average air-bone gap was (46.3±10.6) dB in pure-tone audiometry (0.5, 1, 2, 4kHz). According to the different degrees of facial nerve and ossicle malformation, we performed three different hearing reconstruction strategies for the 14 patients (16 ears) with facial nerve aberration and oval window atresia, including 7 ears of incus bypass artificial stape implantation, 7 ears of Malleostapedotomy (MS) and 2 ears of Malleus-cochlear-prothesis (MCP). After 3 months to 18 months of follow-up, all patients showed no facial paralysis. The postoperative mean bone conductive threshold was (15.7±7.9) dB and air-bone gap was (19.8±8.5) dB. There were significant differences in mean air-bone gap before and after operation (t=7.766, P<0.05), and there was no significant difference between the mean bone conductive threshold before and after surgery (t=0.225, P=0.824). There was no significant difference of mean reduction of air-bone gap between patients with and without facial nerve aberration (t=1.412, P=0.165). There was no significant difference between the three hearing reconstruction strategies. There was no significant displacement of the Piston examined by U-HRCT. Conclusion: For patients of middle ear malformation whose facial nerve cover the oval window partially, incus bypass artificial stape implantation or Malleostapedotomy (MS) can be selected according to the specific condition of auditory ossis malformation, and for patients whose facial nerve completely covers the oval window area, Malleus-cochlear-prothesis (MCP) can be selected. Three types of stapes surgery are safe and reliable for patients with oval window atresia accompanied by facial nerve aberration. There was no significant difference in efficacy between them. Preoperative HRCT assessment of middle ear malformation is effective. There is no significant difference of surgical effect with or without facial nerve aberration. The U-HRCT can be used to evaluate the middle ear malformation before surgery and the Piston implantation status after surgery. Due to the risks of surgery, those who do not want to undergo surgery can choose artificial hearing AIDS, such as hearing aid, vibrating soundbridge, bone bridge or bone-anchored hearing aid. 目的： 总结伴有面神经异常的前庭窗闭锁患者内耳开窗术式的选择，并分析疗效。 方法： 回顾性分析2015年1月至2023年3月首都医科大学附属北京友谊医院就诊的先天性中耳畸形患者的临床资料97例（132耳），其中随访资料完整的患者39例（44耳），男27例，女12例；年龄7~48岁，平均17.8岁；伴有面神经异常者且前庭窗闭锁患者14例（16耳），不伴面神经异常者25例（28耳）。总结分析影像学表现、纯音测听结果、内耳开窗术式的选择、术中发现及术后听力改善情况，使用Prism 9软件对手术前后患者的平均骨导及气骨导差进行统计学分析。 结果： 14例（16耳）伴有面神经异常且前庭窗闭锁的中耳畸形患者均表现为自幼听力差，无面神经麻痹。术前行颞骨高分辨率CT（HRCT）检查及纯音测听、盖莱试验检查。记录统计患者锤骨、砧骨、镫骨、前庭窗及面神经5个部位的畸形情况。HRCT检查发现4个部位及以上畸形者12耳（75.0%）。术前纯音测听（0.5、1、2、4 kHz）骨导听阈为（15.3±10.4）dB HL，气骨导差为（46.3±10.6）dB。根据面神经、听小骨畸形程度的不同，对伴有面神经异常的14例（16耳）前庭窗闭锁患者实施了3种不同的内耳开窗手术，其中砧骨-前庭窗开窗术7例，锤骨-前庭窗开窗术7例，鼓岬耳蜗开窗术2例。术后随访3~18个月，患者无面神经麻痹。术后骨导听阈为（15.7±7.9）dB HL，气骨导差为（19.8±8.5）dB。气骨导差在手术前后的差异有统计学意义（t=7.766，P<0.05），手术前后骨导听阈差异无统计学意义（t=0.225，P=0.824）。伴与不伴面神经异常患者气骨导差缩小值之间差异无统计学意义（t=1.412，P=0.165）。3种术式之间疗效不存在明显差异。10 μm级耳科专用CT评估人工镫骨植入位置无明显移位。 结论： 对于面神经不完全遮盖前庭窗的前庭窗闭锁患者可以依据听小骨畸形具体情况选择砧骨-前庭窗开窗术或锤骨-前庭窗开窗术，对于面神经完全遮盖前庭窗区域的患者可以选择鼓岬耳蜗开窗术。3种内耳开窗手术对伴有面神经异常的前庭窗闭锁患者具有安全可靠的手术疗效，且相互之间疗效无明显差异。HRCT对中耳畸形情况评估效果好。伴与不伴面神经异常对先天性中耳畸形患者手术疗效无明显影响。10 μm级耳科专用CT检查可作为评估术前中耳畸形情况，以及术后人工镫骨植入状态的理想手段。因手术存在风险，对不愿意接受手术者可选择人工助听装置，如助听器、振动声桥、骨桥或骨锚式助听器等。.
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