Young Yee Man Catherine, Leung Ho Sang, Chang Wai Tsz, Yu Suet Mui, Wang Ki, Wong Ka Tak, Chu Chiu Wing Winnie
Department of Imaging and Interventional Radiology, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, New Territories, Hong Kong.
Department of Otorhinolaryngology, Head and Neck Surgery, The Chinese University of Hong Kong, Shatin, New Territories, Hong Kong.
Eur Radiol. 2025 May 14. doi: 10.1007/s00330-025-11663-5.
Sennaroglu's classification is the most accepted classification worldwide for inner ear and/or nerve malformation, although only limited data is available for the Asian population. This study aims to assess their relative frequency, summarise their radiological features, and determine their correlation with hearing impairment.
Retrospective analysis of patients with imaging-proven inner ear and/or nerve malformation in a single tertiary hospital in Hong Kong in 2014-2023. Clinical data were reviewed along with relevant imaging findings.
A total of 155 abnormal ears from 94 patients were included. All cases of Michel deformity (n = 4), rudimentary otocyst (n = 5), cochlear aplasia (n = 6) and common cavity (n = 3) show severe/profound hearing loss. Cochlear hypoplasia Type I (n = 2) and Type II (n = 6), and incomplete partition Type I (n = 5) also show severe/profound hearing loss; while cochlear hypoplasia Types III (n = 4) and Type IV (n = 8) and incomplete partition Type II (n = 28) show variable degrees of hearing deficit. Cochlear and vestibulocochlear nerve abnormalities were detected in 66% and 54% of cases, respectively. Generalised linear model analyses showed a correlation between the degree of hearing deficit with the presence of cochlear aperture atresia (OR = 2.562, p = 0.015), cochlear nerve atresia (OR = 2.599, p = 0.014), and vestibulocochlear nerve atresia (OR = 2.747, p = 0.064); and remained significant adjusting for the presence of cochlear abnormalities.
A higher relative frequency of vestibulocochlear nerve and cochlear nerve abnormalities is observed in our study when compared with the existing literature. Cochlear nervous system abnormalities are most predictive of the degree of hearing impairment.
Question Accurate assessment of the inner ear/cochlear nerve is important for guiding treatment, but literature on their relative frequency and correlation with hearing impairment is lacking. Findings Cochlear system abnormalities are more frequent than reported in the literature. Cochlear aperture atresia, cochlear, or vestibulocochlear nerve aplasia are significant predictors of hearing impairment. Clinical relevance This study highlights the importance of assessment of the cochlear nervous system with the use of high-resolution MRI in the evaluation of paediatric sensorineural hearing loss.
森纳罗格鲁分类法是全球范围内最被广泛接受的内耳和/或神经畸形分类方法，尽管针对亚洲人群的数据有限。本研究旨在评估其相对频率，总结其放射学特征，并确定它们与听力障碍的相关性。
对2014年至2023年香港一家三级医院中经影像学证实存在内耳和/或神经畸形的患者进行回顾性分析。回顾临床数据以及相关影像学检查结果。
共纳入94例患者的155只异常耳。所有米歇尔畸形（n = 4）、原始耳囊（n = 5）、耳蜗发育不全（n = 6）和共同腔畸形（n = 3）的病例均表现为重度/极重度听力损失。I型（n = 2）和II型（n = 6）耳蜗发育不全以及I型不完全分隔（n = 5）也表现为重度/极重度听力损失；而III型（n = 4）和IV型（n = 8）耳蜗发育不全以及II型不完全分隔（n = 28）表现出不同程度的听力缺陷。分别在66%和54%的病例中检测到耳蜗和前庭蜗神经异常。广义线性模型分析显示，听力缺陷程度与耳蜗孔闭锁（OR = 2.562，p = 0.015）、耳蜗神经闭锁（OR = 2.599，p = 0.014）和前庭蜗神经闭锁（OR = 2.747，p = 0.064）之间存在相关性；在对耳蜗异常情况进行校正后，这种相关性仍然显著。
与现有文献相比，本研究中观察到前庭蜗神经和耳蜗神经异常的相对频率更高。耳蜗神经系统异常最能预测听力障碍的程度。
问题准确评估内耳/耳蜗神经对于指导治疗很重要，但缺乏关于它们的相对频率以及与听力障碍相关性的文献。发现耳蜗系统异常比文献报道的更为常见。耳蜗孔闭锁、耳蜗或前庭蜗神经发育不全是听力障碍的重要预测因素。临床意义本研究强调了在评估小儿感音神经性听力损失时，使用高分辨率MRI评估耳蜗神经系统的重要性。
