Ruthberg Jeremy S, Kocharyan Armine, Farrokhian Nathan, Stahl Maximilian C, Hicks Kayla, Scarborough Jessica, Murray Gail S, Wu Shannon, Manzoor Nauman, Otteson Todd
Case Western Reserve University School of Medicine, Cleveland, OH, USA.
Department of Otolaryngology-Head and Neck Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA.
Int J Pediatr Otorhinolaryngol. 2022 May;156:111072. doi: 10.1016/j.ijporl.2022.111072. Epub 2022 Feb 22.
To reveal hearing loss patterns in patients with enlarged vestibular aqueduct (EVA) syndrome according to demographic and clinical characteristics.
A retrospective, longitudinal study design was utilized to identify patients with EVA. Ears of patients were categorized into one of four cohorts: progressive fluctuating, progressive non-fluctuating, stable fluctuating, and stable non-fluctuating patterns. Pairwise and group comparisons were performed with non-parametric tests to assess vestibular aqueduct (VA) morphology, clinical, and demographic variables between hearing loss pattern cohorts. Rates of hearing loss in the subgroups were determined utilizing a mixed linear effects model.
44 patients (25 female, 19 male, median diagnosis age: 8.06 years) met inclusion criteria. 16 individuals demonstrated unilateral EVA and 29 had bilateral EVA, resulting in 74 total ears with EVA. Amongst the four cohorts, differences in operculum widths amongst groups were statistically significant (p = 0.049) while VA midpoint widths were not (p = 0.522). Progressive hearing loss ears without fluctuations demonstrated a 3.20 dB per year (p < 0.001) progression while progressive hearing loss ears with fluctuations reported a rate of 3.52 dB loss per year (p < 0.001).
Hearing fluctuations occur similarly in EVA patients with stable and progressive hearing loss. With the exception of increased rates of hearing loss progression for fluctuating progressive hearing loss patients, vestibular aqueduct morphology, demographic, and clinical characteristics commonly reported are likely not strong predictors for whether patients will or will not experience fluctuating patterns of hearing loss.
根据人口统计学和临床特征揭示大前庭导水管（EVA）综合征患者的听力损失模式。
采用回顾性纵向研究设计来确定EVA患者。患者的耳朵被分为四个队列之一：进行性波动型、进行性非波动型、稳定波动型和稳定非波动型。采用非参数检验进行成对和组间比较，以评估听力损失模式队列之间的前庭导水管（VA）形态、临床和人口统计学变量。利用混合线性效应模型确定亚组中的听力损失率。
44例患者（25例女性，19例男性，中位诊断年龄：8.06岁）符合纳入标准。16例表现为单侧EVA，29例为双侧EVA，共有74只耳朵患有EVA。在四个队列中，各组间的骨盖宽度差异具有统计学意义（p = 0.049），而VA中点宽度差异无统计学意义（p = 0.522）。无波动的进行性听力损失耳朵每年进展3.20 dB（p < 0.001），而有波动的进行性听力损失耳朵每年损失率为3.52 dB（p < 0.001）。
EVA患者中，稳定型和进行性听力损失患者的听力波动情况相似。除了波动性进行性听力损失患者的听力损失进展率增加外，通常报告的前庭导水管形态、人口统计学和临床特征可能不是患者是否会经历波动性听力损失模式的强预测指标。
