[卵圆窗闭锁伴面神经畸形患者的内耳开窗策略选择及手术效果]。

Z R Chen, R W Tang, J Xie, J Y Guo, P F Zhao, Z J Yang, G P Wang, S S Gong
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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. <b>Results:</b> 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 (<i>t</i>=7.766, <i>P</i><0.05), and there was no significant difference between the mean bone conductive threshold before and after surgery (<i>t</i>=0.225, <i>P</i>=0.824). 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引用次数: 0

摘要

目的总结卵圆窗闭锁伴面神经畸形患者的临床特征和术后疗效。方法回顾性分析我院自2015年1月至2023年3月收治的先天性中耳畸形伴面神经发育畸形患者的临床资料。共 97 例(133 耳)。其中,有完整随访资料的患者39例(44耳),包括男性患者27例,女性患者12例,年龄7-48岁,平均年龄17.8岁。其中,14 例(16 耳)患者合并有面神经畸变,25 例(28 耳)患者无面神经畸变。对影像学检查、纯音测听、手术策略选择、术中发现和术后听力改善的结果进行了总结和分析。记录了耳郭、切迹、镫骨、卵圆窗和面神经的畸形情况。使用 Prism 9 软件对患者手术前后的平均骨传导和气骨间隙进行统计分析。结果中耳畸形并伴有面神经畸变和卵圆窗闭锁的14名患者(16耳)均表现为听力低下,且自幼无面瘫。手术前进行了颞骨高分辨率 CT(HRCT)检查、纯音测听和 Gelle 测试。记录了耳郭、切迹、镫骨、卵圆窗和面神经的畸形情况。术前颞骨高分辨率 CT(HRCT)检查发现,面神经畸形患者组中有 12 只耳朵有 4 个或更多畸形,占 75.00%。在纯音测听(0.5、1、2、4kHz)中,术前平均骨传导阈值为(15.3±10.4)dB,平均气骨间隙为(46.3±10.6)dB。根据面神经和听小骨畸形的不同程度,我们对14例(16耳)面神经畸形和卵圆窗闭锁患者采取了三种不同的听力重建策略,包括7耳门骨旁路人工耳廓植入术、7耳耳廓切开术(MS)和2耳耳廓-人工耳蜗植入术(MCP)。经过 3 个月至 18 个月的随访,所有患者均未出现面瘫。术后平均骨传导阈值为(15.7±7.9)分贝,气骨间隙为(19.8±8.5)分贝。手术前后的平均气骨间隙有显著差异(t=7.766,Pt=0.225,P=0.824)。面神经畸变患者与无面神经畸变患者的平均气骨间隙缩小程度无明显差异(t=1.412,P=0.165)。三种听力重建策略之间无明显差异。通过 U-HRCT 检查,活塞没有明显移位。结论对于面神经部分覆盖椭圆窗的中耳畸形患者,可根据听骨畸形的具体情况选择切口分流人工镫骨植入术或耳廓成形术(MS);对于面神经完全覆盖椭圆窗区域的患者,可选择耳廓-人工耳蜗成形术(MCP)。对于伴有面神经畸形的卵圆窗闭锁患者,三种镫骨手术均安全可靠。它们之间的疗效没有明显差异。术前对中耳畸形进行 HRCT 评估是有效的。面神经畸形与否,手术效果无明显差异。U-HRCT 可用于术前评估中耳畸形和术后活塞植入情况。由于手术的风险,不想接受手术的患者可以选择人工听力艾滋病,如助听器、振动声桥、骨桥或骨固定助听器。
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[Selection of inner ear fenestration strategy and surgical effect of patients with oval window atresia accompanied by facial nerve aberration].

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.

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