大前庭水管综合征与儿童突发感音神经性耳聋的临床观察

目的探讨大前庭水管综合征与儿童突发感音神经性耳聋的关联及临床特点、听力学、影像学改变与治疗。方法对35例儿童大前庭水管综合征患者临床资料进行分析总结。结果本研究均为无原因的突发感音神经性聋,听力学检查中发现,均为高频重度感音性聋,其中56耳患儿在250、500Hz均存在有气骨导差,气骨导差>15dBHL,以250dBHL最为明显,经过多次复诊,多数听力波动性进行性加重。结论大前庭水管综合征是内耳畸形的听力障碍性疾病。可依靠听力学检查、CT和MRI明确诊断。

Large Aquaeductus Vestibuli and Children Sudden Perceptive Nerve Deafness

Objective To discuss the relationship, clinical manifestations, audiology and imaging changes of large aquaeductus vestibuli syndrome （LAVS） and children sudden sound nerve deafness. Metlmds The clinical materials of 35 cases of LAVS patient were analyzed and summarized. Results All of them were sudden perceptive nerve deafness （SPND） in high frequency. There were 56 ears existed more than 15dBHL in air and bone conduction differences at 250, 500Hz and most in 250dBHL who were acoustic fluctuating and progressive aggravation obviously. Conclusion The LAVD might be deformity of inner ear acoustice disturbance and could be diagnosed with acoustic detection, CF and MRI.