大前庭水管综合征影像学相关研究进展

Research progress in imaging of large vestibular aqueduct syndrome

大前庭水管综合征（large vestibular aqueduct syndrome,LVAS）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一个最常见的内耳畸形。主要表现为渐进性或波动性听力损失,程度从基本正常到极重度,以高频下降为重,气骨导差多出现在低频;发病前常有使颅压增高的诱因;部分患者可伴有眩晕或不稳感等前庭功能异常。

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＇s 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 didn＇t 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＇s imaging studies.