Bonnard Åsa, Karltorp Eva, Verrecchia Luca
Department of Clinical Science, Intervention and Technology, Division of Ear, Nose and Throat Diseases, Karolinska Institutet, Karolinska University Hospital, Huddinge, B61 141 86 Stockholm, Sweden.
Department of Otorhinolaryngology Audiology and Neurotology, Karolinska University Hospital, Huddinge, M53 141 86 Stockholm, Sweden.
Audiol Res. 2023 Feb 9;13(1):130-142. doi: 10.3390/audiolres13010013.
This is a single center cohort study regarding the prevalence of vestibular loss in hearing impaired children affected by large vestibular aqueduct syndrome (LVAS) with incomplete cochlear partition malformation type II (IP2), fitted with cochlear implant (CI). Twenty-seven children received CI operations at 0.4-13 years on one or both ears and tested for vestibular loss with head impulse test, video head impulse test, mini ice-water test and cervical VEMP. Vestibular loss was found in 19% of operated ears and in 13.9% of non-operated ears. The difference was not statistically significant and was not significantly modified by age at implantation, age at testing, sex, presence of SLC26A4 gene mutation or bilaterality. However, the presence of anatomic anomalies at the level of the vestibulum or semicircular canals was significantly associated with a higher incidence of vestibular loss in CI operated children but not in those non-operated. No other factors, such as the surgical access, the electrode type, the presence of Gusher perioperatively, or post-operative vertigo modified significantly the prevalence of vestibular loss. In conclusion, LVAS/IP2 appears to be the major determinant of vestibular loss in these children, with a less obvious impact of CI, excluding the cases with vestibulum/canal anomalies: this group might have a higher risk for vestibular loss after CI surgery.
这是一项单中心队列研究，旨在探讨患有大前庭导水管综合征（LVAS）合并不完全性II型蜗管分隔畸形（IP2）且接受了人工耳蜗植入（CI）的听力受损儿童的前庭功能丧失患病率。27名年龄在0.4至13岁之间的儿童接受了单耳或双耳的CI手术，并通过头部脉冲试验、视频头脉冲试验、微量冰水试验和颈肌前庭诱发肌源性电位进行前庭功能丧失检测。在接受手术的耳朵中，19%发现存在前庭功能丧失，在未接受手术的耳朵中这一比例为13.9%。差异无统计学意义，且不受植入年龄、检测年龄、性别、SLC26A4基因突变的存在与否或双侧性的显著影响。然而，在前庭或半规管水平存在解剖学异常与接受CI手术儿童的前庭功能丧失发生率较高显著相关，但在未接受手术的儿童中并非如此。没有其他因素，如手术入路、电极类型、围手术期是否发生“井喷”或术后眩晕，会显著改变前庭功能丧失的患病率。总之，LVAS/IP2似乎是这些儿童前庭功能丧失的主要决定因素，CI的影响不太明显，但不包括存在前庭/半规管异常的病例：这组病例在CI手术后可能有更高的前庭功能丧失风险。
