Janky Kristen L, Patterson Jessie N, Kelly Elizabeth A, Merchant Gabrielle R
*Boys Town National Research Hospital, Omaha, NE.
J Am Acad Audiol. 2025 May 1;36(3):160-171. doi: 10.3766/jaaa.240059. Epub 2025 May 27.
Large vestibular aqueduct syndrome (LVAS) is the most common inner ear malformation in children with hearing loss; however, imaging is required to determine the definitive diagnosis. This study aimed to preliminarily determine the sensitivity and specificity of air-conducted and bone-conducted cervical vestibular evoked myogenic potentials (VEMPs) and ocular VEMPs and wideband acoustic immittance (WAI) as screening tools for LVAS and identify candidates for imaging. This was a prospective observational study. The sample included 10 participants (17 ears) with LVAS and 15 control participants. All participants completed cervical and ocular VEMP testing in response to air-conducted and bone-conducted stimuli and WAI testing. Ears with LVAS had significantly higher air-conducted cervical and ocular VEMP amplitudes but reduced bone-conducted VEMP amplitudes. Because of this pattern reversal, air-conducted amplitudes were subtracted from bone-conducted amplitudes. This novel outcome was the best individual factor for differentiating ears of the control group from ears with LVAS and is a sensitive clinical indicator of LVAS, with 82 percent sensitivity and 80 percent specificity for cervical VEMP and 88 percent sensitivity and 80 percent specificity for ocular VEMP. Ears with LVAS exhibited significant differences in WAI absorbance of control ears at select low, mid, and high frequencies, and they also exhibited characteristic low-frequency peaks in individual responses that could be detected using an automated algorithm with moderate sensitivity (64 percent) and specificity (72 percent). This is the first report of the use of VEMP and WAI responses of individuals as screening tools for LVAS. VEMP and WAI could be used to determine which individuals are good candidates for radiographic imaging and decrease the time required to determine a diagnosis. The test results also demonstrated patterns that differed from those of other third window disorders. Therefore, important physiologic differences between LVAS and other third window disorders require further investigation.
大前庭导水管综合征（LVAS）是导致儿童听力损失的最常见内耳畸形；然而，需要影像学检查来确定明确诊断。本研究旨在初步确定气导和骨导颈前庭诱发肌源性电位（VEMP）、眼VEMP以及宽带声导抗（WAI）作为LVAS筛查工具的敏感性和特异性，并确定影像学检查的候选对象。这是一项前瞻性观察研究。样本包括10名患有LVAS的参与者（17只耳朵）和15名对照参与者。所有参与者均完成了气导和骨导刺激下的颈VEMP和眼VEMP测试以及WAI测试。患有LVAS的耳朵气导颈VEMP和眼VEMP振幅显著更高，但骨导VEMP振幅降低。由于这种模式反转，用骨导振幅减去气导振幅。这一新结果是区分对照组耳朵和患有LVAS耳朵的最佳个体因素，是LVAS的敏感临床指标，颈VEMP的敏感性为82%，特异性为80%；眼VEMP的敏感性为88%，特异性为80%。患有LVAS的耳朵在选定的低频、中频和高频下的WAI吸收率与对照耳朵存在显著差异，并且在个体反应中还表现出特征性的低频峰值，可使用具有中等敏感性（64%）和特异性（72%）的自动算法检测到。这是首次将个体的VEMP和WAI反应用作LVAS筛查工具的报告。VEMP和WAI可用于确定哪些个体适合进行影像学检查，并减少确诊所需的时间。测试结果还显示出与其他第三窗疾病不同的模式。因此，LVAS与其他第三窗疾病之间重要的生理差异需要进一步研究。
