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摘要: 目的:分析大前庭水管综合征(LVAS)住院患者临床资料,探讨诊断与治疗策略。目的:对我院耳内科住院治疗的44 例LVAS合并重度至极重度感音神经性听力损失的患者,进行系统的病史、临床听力学特征、前庭功能及疗效分析,以期进一步完善LVAS患者的诊治策略。结果:44例(87耳)LVAS患者男女比例为1.2∶1,发病年龄(3.39±3.13)岁,93.18%的患者发病年龄在8岁以内,有家族史者5例(11.36%)。67耳(77.01%)表现为极重度听力损失,20耳(22.99%)表现为重度听力损失。经内科治疗后,38耳(43.68%)听力得到不同程度改善。疗效影响因素分析显示:发病类型与疗效相关,表现为突发性听力下降的患者,疗效好于渐进性听力下降者;治疗方案选择与疗效相关,联合使用改善动脉循环及静脉回流药物治疗的患者,疗效好于单一使用改善动脉循环类药物者;疗程与疗效相关,疗程在1周以上效果较好。LVAS患者的性别、发病年龄、病程、听力损失程度以及是否合并Mondini畸形对于其治疗效果无明显影响。结论:LVAS患者经过系统的内科用药可以有效提高听力水平。通过对资料完善的住院患者进行诊疗分析,有助于进一步完善LVAS个性化诊治方案,指导具体临床工作。Abstract: Objective: This study is to investigate the clinical materials of in-patients with the large vestibular aqueduct syndrome(LVAS),and explore the feature, diagnosis and treatment measures of the disease.Method: A retrospective review was conducted including the medical history, audiological examinations, vestibular function examinations, imaging examinations and treatment methods of 44 in patients(87 ears) suffering LVAS admitted to our hospital in the past 4 years(from 2008 to 2012).Result: In the 44 in patients, there were 24 male cases and 20 female cases, and the male-female ratio was 1.2∶1. The average of the onset age was 3.39 years. Five cases(11.36%) had related familial history. The profound hearing loss was found in 67 ears(77.01%), and the severe hearing loss was found in 20 ears (22.99%). After systemic treatment,the hearing of 38 ears improved effectively,but that of 49 ears did not improve obviously. The analysis found that patients suffering sudden hearing loss got better curative effect than those with progressive hearing loss. Patients received combined drug therapy improving arterial circulation as well as venous reflux got better therapeutic effect. There was a significant difference on effect between the patients with course of treatment more than 7 days and those less than 7 days. There was no significant correlation between therapeutic effect and other factors.Conclusion: In part of LVAS patients,the hearing level can be effectively improved through a standard internal medicine treatment. We can improve the personalized and standardized treatment strategy for this disease through analysis of diagnosis and treatment of in-patients with complete clinical data.
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Key words:
- large vestibular aqueduct syndrome /
- in patients /
- curative effect
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