Hacettepe University, Faculty of Medicine, Department of Radiology, Turkey.
Hacettepe University, Faculty of Medicine, Department of Radiology, Turkey.
Eur J Radiol. 2021 Dec;145:110064. doi: 10.1016/j.ejrad.2021.110064. Epub 2021 Nov 23.
Large endolymphatic duct and sac (LEDS) is one of the most common imaging abnormalities in congenital sensorineural hearing loss and is frequently seen with coexistent cochlear anomalies, especially incomplete partition type II. However, MRI findings of accompanying cochlear and especially modiolar dysplasias may be subtle. The purpose of this study is to evaluate the imaging findings of LEDS with high-resolution imaging at 3 T and correlate with the audiological data.
3 T temporal bone MRIs of 54 ears in 30 LEDS patients were retrospectively evaluated. The cochlear dysmorphism and modiolar deficiency were assessed qualitatively and quantitatively. The severity of LEDS anomaly, the signal changes within the LEDS and cochlea were also noted. The imaging findings were correlated to the audiological data.
The cochlea was abnormal in 77.8% of the ears with an isolated modiolar deficiency in 11.1%. Cochlea and modiolus were completely normal in 11.1% of the ears. In 63% of the ears X-distance was increased. T2 hypointensity within LEDS and cochlea were detected in 42.6%, and 7.4% of the ears, respectively. The median diameters of LEDS were higher in ears with severe to profound HL than ears with normal to moderate HL (p < 0.05). The X-distance, presence of T2 hypointensity within LEDS, and diameters of modiolus did not show statistical correlation with the audiographic data.
High-resolution 3 T imaging of patients with LEDS anomaly revealed a spectrum of cochlear anomalies, but up to 11.1% of the ears had no underlying anomaly despite severe (endolymphatic duct/sac) dilatation and/or profound HL.
大内淋巴管（LEDS）是先天性感觉神经性听力损失最常见的影像学异常之一，常伴有耳蜗异常，尤其是不完全分隔 II 型。然而，伴随的耳蜗和特别是蜗轴发育不良的 MRI 表现可能较细微。本研究旨在评估 3T 高分辨率成像下 LEDS 的影像学表现，并与听力学数据相关联。
回顾性分析 30 例 LEDS 患者的 54 耳 3T 颞骨 MRI。定性和定量评估耳蜗畸形和蜗轴发育不良。还注意了 LEDS 异常的严重程度、LEDS 内和耳蜗内的信号变化。将影像学发现与听力学数据相关联。
77.8%的耳朵耳蜗异常，其中 11.1%孤立性蜗轴发育不良。11.1%的耳朵耳蜗和蜗轴完全正常。63%的耳朵 X 距离增加。42.6%和 7.4%的耳朵分别检测到 LEDS 内 T2 低信号和耳蜗内 T2 低信号。严重至极重度听力损失的耳朵的 LEDS 直径中位数高于正常至中度听力损失的耳朵（p<0.05）。X 距离、LEDS 内 T2 低信号的存在以及蜗轴直径与听力数据均无统计学相关性。
LEDS 异常患者的 3T 高分辨率成像显示出一系列耳蜗异常，但高达 11.1%的耳朵尽管存在严重的（内淋巴管/囊）扩张和/或极重度听力损失，但没有潜在的异常。
