Dlugaiczyk Julia, Rösch Sebastian, Mantokoudis Georgios
Department of Otorhinolaryngology, Head and Neck Surgery & Interdisciplinary Center of Vertigo, Balance and Ocular Motor Disorders, University Hospital Zurich (USZ), University of Zurich (UZH), Rämistrasse 100, 8091, Zurich, Switzerland.
Department of Otorhinolaryngology, Head and Neck Surgery, Paracelsus Medical University, Salzburg, Austria.
HNO. 2025 Jul 31. doi: 10.1007/s00106-024-01467-2.
The diagnosis of third window syndromes often poses a challenge in clinical practice.
This paper provides an overview of diagnostic procedures in third window syndromes, with special emphasis on superior canal dehiscence syndrome (SCDS), large vestibular aqueduct syndrome (LVAS), and X-chromosomal malformation of the cochlea.
A literature search was performed in PubMed up to December 2023. Furthermore, a selection of the authors' own cases is presented.
Audiovestibular tests for the diagnosis of third window syndromes are most often reported for patients with SCDS in the literature. In this context, cut-off values with different sensitivities and specificities have been defined for different outcome parameters of vestibular evoked myogenic potentials. Current developments include the application of electrocochleography, broadband tympanometry, video head impulse testing, and vibration-induced nystagmus. Genetic analyses are increasingly applied in LVAS.
The diagnosis of third window syndromes is always based on the synthesis of patients' symptoms, clinical signs, audiovestibular test results, and imaging.
第三窗综合征的诊断在临床实践中常常是一项挑战。
本文概述第三窗综合征的诊断程序，特别强调半规管裂综合征（SCDS）、大前庭导水管综合征（LVAS）和耳蜗X染色体畸形。
截至2023年12月在PubMed上进行了文献检索。此外，还展示了作者自己选择的病例。
文献中关于第三窗综合征诊断的听前庭测试大多是针对SCDS患者报告的。在此背景下，针对前庭诱发肌源性电位的不同结果参数定义了具有不同敏感性和特异性的临界值。当前的进展包括耳蜗电图、宽频鼓室图、视频头脉冲测试和振动性眼震的应用。基因分析在LVAS中的应用越来越多。
第三窗综合征的诊断始终基于患者症状、临床体征、听前庭测试结果和影像学检查的综合判断。
