不同形态类型的大前庭水管对临床发病及人工耳蜗植入的影响

文献信息

中文摘要

目的： 探讨大前庭水管（EVA）的不同形态特征对在我院接受人工耳蜗植入（CI）的儿科患者临床发病及术后言语康复效果的影响。
方法： 对我院诊断为EVA并接受初次CI的儿童进行回顾性分析。回顾临床资料、听力学特征及影像学特点。根据听力损失（HL）对称性对患者进行分类：对称性HL组（双耳阈值差<20 dB）和非对称性HL组（差值≥20 dB）。对称性HL组进一步根据发病年龄分为早发亚组和晚发亚组。根据疾病进展，将患者分为突发性HL和波动性HL亚组。影像学测量包括前庭水管（VA）开口角度和长度。对早发亚组术后言语结果在CI后1-6年进行随访，并与同期接受CI的内耳结构正常儿童匹配对照组进行比较。
结果： 共纳入81例EVA患儿。56例表现为对称性HL，25例表现为非对称性HL。关于进展模式，24例被归类为波动性HL，25例为突发性HL。在对称性HL组中，与晚发亚组相比，早发亚组表现出显著更大的双侧VA开口角度和更短的双侧VA长度。在非对称性HL组中，听力较差耳比听力较好耳具有更大的VA开口角度但更短的VA长度。两组术后言语表现随时间改善；然而，与对照组相比，早发亚组在CI后1-6年的所有四项言语评估指标上持续得分较低。
结论： 宽而浅的前庭水管似乎使儿童更容易受到颅内压波动引起的内耳损伤。这种形态学易感性可能导致听力损失发病更早、程度更重且进展更突然，最终损害术后言语康复效果。

英文摘要

OBJECTIVE: To investigate the influence of different morphological features of the enlarged vestibular aqueduct (EVA) on clinical onset and postoperative speech rehabilitation outcomes in pediatric patients undergoing cochlear implantation (CI) at our institution.
METHODS: A retrospective analysis was conducted on children diagnosed with EVA who underwent primary CI at our hospital. Clinical data, audiological profiles, and imaging characteristics were reviewed. Patients were categorized based on hearing loss (HL) symmetry: symmetric HL group (interaural threshold difference < 20 dB) and asymmetric HL group (difference ≥ 20 dB). The symmetric HL group was further stratified by age of onset into early-onset and late-onset subgroups. Based on disease progression, patients were classified into sudden HL and fluctuating HL subgroups. Radiological measurements included the vestibular aqueduct (VA) aperture angle and length. Postoperative speech outcomes in the early-onset subgroup were followed up at 1-6 years post-CI and compared with a matched control group of children with normal inner ear structures undergoing CI during the same period.
RESULTS: Eighty-one children with EVA were enrolled. Fifty-six cases presented with symmetric HL, while 25 cases exhibited asymmetric HL. Regarding progression patterns, 24 cases were classified as fluctuating HL and 25 as sudden HL. In the symmetric HL group, the early-onset subgroup demonstrated significantly larger bilateral VA aperture angles and shorter bilateral VA lengths compared to the late-onset subgroup. In the asymmetric HL group, the ear with worse HL had a larger VA aperture angle but a shorter VA length than the better-hearing ear. Postoperative speech performance improved over time in both groups; however, the early-onset subgroup consistently scored lower across all four speech evaluation metrics at 1-6 years post-CI compared to the control group.
CONCLUSION: A wide and shallow vestibular aqueduct appears to render children more susceptible to inner ear damage induced by intracranial pressure fluctuations. This morphological predisposition likely contributes to earlier onset, greater severity, and more sudden progression of hearing loss, ultimately compromising postoperative speech rehabilitation outcomes.