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大前庭导水管综合征及II型不完全分隔综合征所致听力损失

Hearing loss in enlarged vestibular aqueduct and incomplete partition type II.

作者信息

Ahadizadeh Emily, Ascha Mustafa, Manzoor Nauman, Gupta Amit, Semaan Maroun, Megerian Cliff, Otteson Todd

机构信息

Ear, Nose, and Throat Institute, University Hospitals Cleveland Medical Center, Cleveland, OH, United States; Case Western Reserve University, School of Medicine, 10900 Euclid Avenue, Cleveland, OH, United States.

Ear, Nose, and Throat Institute, University Hospitals Cleveland Medical Center, Cleveland, OH, United States; Center for Clinical Investigation, Department of Epidemiology and Biostatistics, Case Western Reserve University School of Medicine, Cleveland, OH, United States.

出版信息

Am J Otolaryngol. 2017 Nov-Dec;38(6):692-697. doi: 10.1016/j.amjoto.2017.06.010. Epub 2017 Jun 23.

Abstract

PURPOSE

The purpose of this work is to identify the role of incomplete partition type II on hearing loss among patients with enlarged vestibular aqueduct (EVA).

BACKGROUND

EVA is a common congenital inner ear malformation among children with hearing loss, where vestibular aqueduct morphology in this population has been shown to correlate to hearing loss. However, the impact of incomplete partition between cochlear turns on hearing loss has not been, despite meaningful implications for EVA pathophysiology.

METHODS

A retrospective review of radiology reports for patients who had computed tomography (CT) scans with diagnoses of hearing loss at a tertiary medical center between January 2000 and June 2016 were screened for EVA. CT scans of the internal auditory canal (IAC) for those patients with EVA were examined for evidence of incomplete partition type II (IP-II), measurements of midpoint width and operculum width a second time, and patients meeting Cincinnati criteria for EVA selected for analysis. Statistical analysis including chi-square, Wilcoxon rank-sum, and t-tests were used to identify differences in outcomes and clinical predictors, as appropriate for the distribution of the data. Linear mixed models of hearing test results for all available tests were constructed, both univariable and adjusting for vestibular aqueduct morphometric features, with ear-specific intercepts and slopes over time.

RESULTS

There were no statistically significant differences in any hearing test results or vestibular aqueduct midpoint and operculum widths. Linear mixed models, both univariable and those adjusting for midpoint and operculum widths, did not indicate a statistically significant effect of incomplete partition type II on hearing test results.

CONCLUSIONS

Hearing loss due to enlarged vestibular aqueduct does not appear to be affected by the presence of incomplete partition type II. Our results suggest that the pathophysiological processes underlying hearing loss in enlarged vestibular aqueduct may not be a result of cochlear malformation, and instead are more likely to involve vestibular aqueduct or cellular and molecular-level mechanisms of hearing loss.

摘要

目的

本研究旨在确定II型不完全分隔在大前庭导水管(EVA)患者听力损失中的作用。

背景

EVA是儿童听力损失中常见的先天性内耳畸形,该人群的前庭导水管形态已显示与听力损失相关。然而,尽管对EVA的病理生理学有重要意义,但耳蜗螺旋之间不完全分隔对听力损失的影响尚未得到研究。

方法

回顾性分析2000年1月至2016年6月在一家三级医疗中心进行计算机断层扫描(CT)且诊断为听力损失的患者的放射学报告,筛选出EVA患者。对这些EVA患者的内耳道(IAC)CT扫描进行检查,以寻找II型不完全分隔(IP-II)的证据,再次测量中点宽度和骨盖宽度,并选择符合辛辛那提EVA标准的患者进行分析。根据数据分布情况,采用卡方检验、Wilcoxon秩和检验和t检验等统计分析方法来确定结果和临床预测因素的差异。构建所有可用听力测试结果的线性混合模型,包括单变量模型以及调整前庭导水管形态特征的模型,并随时间设置耳特异性截距和斜率。

结果

在任何听力测试结果、前庭导水管中点宽度和骨盖宽度方面均无统计学显著差异。单变量线性混合模型以及调整中点和骨盖宽度后的模型均未显示II型不完全分隔对听力测试结果有统计学显著影响。

结论

大前庭导水管所致的听力损失似乎不受II型不完全分隔的影响。我们的结果表明,大前庭导水管听力损失的病理生理过程可能不是耳蜗畸形的结果,而更可能涉及前庭导水管或听力损失的细胞和分子水平机制。

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