Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2018 Apr;32(8):639-642. doi: 10.13201/j.issn.1001-1781.2018.08.021.
Large vestibular aqueduct syndrome is one of the common non-syndromic hearing impairment. It is one of the most common inner ear abnormalities that cause hearing loss in children.The main performance is gradual or fluctuant hearing loss, from basic normal to extremely severe. Frequently seen in high frequencies hearing loss. The air-bone conduction gaps present in pure tone audiometry test with low frequencies. There were some inducements of intracranial pressure increases before premorbid. Some patients could be accompanied by vertigo or instability. So far, there was still no effective way to terminate the patient deafness progress.If there was no effective intervention,the speech developmental delay of children were an inevitable trend,greatly affect their normal social communication learning ability. So, early diagnosis was critical. Imaging examination was the golden criterion for the diagnosis of LAVS.Characteristic audiological performance and gene diagnosis can be the basis of the further diagnosed. Because the structure and anatomical location of vestibular aqueduct is small and deep, normal imaging examination is difficult to display its morphology and structure,so,for a long time, it did not work very well. Until the advent of High-resolution computed tomography and magnetic resonance imaging, there was a breakthrough and a deeper understanding of the fine structure with inner ear. We reviewed the latest progress of large vestibular aqueduct syndrome imaging studies.
大前庭导水管综合征是常见的非综合征性听力障碍之一。它是导致儿童听力损失的最常见内耳异常之一。主要表现为渐进性或波动性听力损失，从基本正常到极其严重。常见于高频听力损失。纯音听力测试中低频出现气骨导差。病前有一些颅内压升高的诱因。部分患者可伴有眩晕或平衡失调。迄今为止，仍没有有效的方法终止患者耳聋进展。如果没有有效干预，儿童言语发育迟缓将是不可避免的趋势，极大影响其正常社交沟通学习能力。所以，早期诊断至关重要。影像学检查是诊断大前庭导水管综合征的金标准。特征性听力学表现及基因诊断可作为进一步诊断的依据。由于前庭导水管结构及解剖位置小而深，常规影像学检查难以显示其形态结构，因此长期以来效果不佳。直到高分辨率计算机断层扫描和磁共振成像出现，才取得突破并对内耳精细结构有了更深入了解。我们综述了大前庭导水管综合征影像学研究的最新进展。
