大前庭小管综合征55例报告

• 作 者: 廉能静 诸小侬 兰宝森 蔡正华 刘铤
• 机 构: 北京市耳鼻咽喉科研究所，北京市同仁医院
• 出 处: 临床耳鼻咽喉科杂志
• 发表日期: 1995年05期
• 关键词: 先天性内耳畸形 言语发育 重度耳聋 感音神经性聋 听骨链畸形 脑外伤史 纯音测听 妊娠早期 混合性聋 后半规管

报告１２７例（２５０耳）先天内耳畸形患儿发现大前庭小管综合征５５例（１０５耳）。该病的临床特征是：病人９０％于５岁以前发病，以重度耳聋、言语发育障碍为主要症状；约１０％有听力波动及反复眩晕发作，发病前常有明显脑外伤史；３４．５％患儿母亲妊娠早期有感冒史。提示遇有该类患儿应尽早做高分辨内耳ＣＴ检查确诊，力求保留患儿原有听力。

Report of 127 cases (250 ears) congenital auricular malformations found in 55 cases of large vestibular canal syndrome (105 ears). The clinical features of the disease are: 90% of patients before the age of 5 onset, with severe deafness, speech disorders as the main symptom; about 10% of hearing loss and repeated vertigo attacks, often before the onset of a clear history of traumatic brain injury; 34.5 % Of mothers have a history of cold during early pregnancy. Prompt encounter such children should be done as soon as possible high-resolution internal ear CT examination confirmed, and strive to retain the original hearing of children.