Objective:To investigate the relationship between imaging characteristics and intraoperative perilymph gusher in patients with Mondini inner ear malformation in cochlear implantation, in order to provide basis and clinical guidance for predicting of intraoperative perilymph gusher before cochlear implantation. Methods:According to Sennaroglu's classification method, children with severe sensorineural hearing loss screened from January 2020 to December 2021 were divided into Mondini group, simple enlarged vestibular aqueduct group and normal inner ear group according to inclusion criteria strictly. The images of temporal bone HRCT and inner ear MRI were post-processed, some relative indicators were measured, including cochlear height and width of vestibular aqueduct, etc., and the gusher situation during cochlear implantation was recorded. The mean value of each indicator among the three groups were compared respectively, and the differences of each indicator between the gusher group and the non-gusher group were analyzed. Results:There were statistically significant differences in cochlear height, length of cochlear bottom turn, width of cochlear aperture, vestibular length and vestibular width among the Mondini group（24 cases）, simple EVA group（15 cases） and normal inner ear group（28 cases）. The incidence of gusher of Mondini group in cochlear implantation was 30.77%（8/26）. The outer diameter of the VA（[3.10±0.74]mm） and the middle width of the VA（[1.90±0.68] mm） in the gusher group were wider than those in the non-gusher group, and the difference was statistically significant. The incidence of intraoperative gusher in patients with EVA was 20.00%（3/15）, and there was statistically significant difference in the length of endolymph sac between gusher group and non-gusher group（P<0.05）. Conclusion:The causes of intraoperative perilymph gusher in patients with Mondini inner ear malformation are complex. The enlarged vestibular aqueduct may be one of the anatomical basis. Whether it can be used to guide the preoperative assessment of the risk of intraoperative perilymph gusher need to be further confirmed by a large sample of clinical research from multiple centers in the future.
目的：初步探讨Mondini畸形患儿的影像学特征与术中“井喷”的关系，为人工耳蜗植入术前预判术中“井喷”提供依据和临床指导。 方法：将2020年1月—2021年12月筛查的重度-极重度感音神经性聋患儿根据Sennarolu的分类方法，严格按纳入标准分为Mondini畸形组、单纯EVA组和内耳无畸形组。对颞骨HRCT及内耳MRI进行图像后期处理，并完成各指标的测量，包括耳蜗高度、前庭导水管外口宽度等，并记录人工耳蜗植入时的“井喷”情况，分别比较三组间各指标的均值，及组内井喷组与非井喷组各指标的差异。 结果：Mondini畸形组、单纯EVA组和内耳无畸形组的耳蜗高度、耳蜗底周长度、蜗孔宽度、前庭长径和前庭宽径存在组间差异（P<0.05）。Mondini畸形患儿人工耳蜗植入术中“井喷”的发生率为30.77%（8/26），井喷组的前庭导水管外口宽度[（3.10±0.74） mm]和前庭导水管中段宽度[（1.90±0.68） mm]较非井喷组宽，差异有统计学意义（P<0.05）。单纯EVA患儿人工耳蜗植入术中“井喷”的发生率为20.00%（3/15），井喷组与非井喷组间的内淋巴囊长度比较差异有统计学意义（P<0.05）。 结论：Mondini畸形患儿术中发生“井喷”的原因复杂，前庭导水管扩大明显可能为解剖基础之一，能否用于指导术前评估术中“井喷”风险，需要今后多中心大样本的临床研究进一步证实。.
Keywords: Mondini deformity; cochlear implantation; enlarged vestibular aqueduct; intraoperative perilymph gusher.
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