CHARGE综合征与听力相关分析  

CHARGE Syndrome and Hearing Loss

作　　者：张丰珍 张杰[1] 陈敏[1] 张亚梅[1] 刘世琳[1] 倪鑫[1] 

机构地区：[1]首都医科大学附属北京儿童医院耳鼻咽喉头颈外科北京市儿童耳鼻咽喉头颈外科重点实验室

出　　处：《中华耳科学杂志》2015年第3期450-453,共4页Chinese Journal of Otology

摘　　要：目的探讨CHARGE综合征的临床特点、听力学特征及人工耳蜗植入疗效。方法通过对我科收治的CHARGE综合征的患儿临床资料收集,结合相关文献分析其临床特征及耳部解剖特点、听力学特点,探讨其人工耳蜗植入的价值及要点。结果两例患儿临床上诊断CHARGE综合征基本明确,均有不同程度的耳部畸形及听力障碍,病例一因其他一些严重的功能障碍于生后5个月时死亡;病例二经过后期的充分及时的治疗,恢复良好,随访听力1年,言语发育良好。结论 CHARGE综合征的诊断基于临床表现,对于合并重度耳聋的患儿,在全身状况允许的情况下进行人工耳蜗植入是听力康复的最佳选择。Objective To investigate clinical and audiological features of CHARGE syndrome and efficacy of cochlear implant. Methods Clinical data of 2 children with CHARGE syndrome seen in our department,including clinical and audiological features, were analyzed in reference to relevant literatures. The value of cochlear implant as treatment and related key points were discussed. Results Both children showed varying degrees of ear malformations and hearing disorders. One child died five months after birth from unrelated serious conditions, while the other recovered well after full and timely treatment, with good speech development in the following year. Conclusion Diagnosis of CHARGE syndrome is primarily based on clinical manifestations. Cochlear implants is the best option for patients with severe sensorineural hearing loss.

关 键 词：CHARGE综合征 后鼻孔闭锁 感音神经性耳聋 人工耳蜗植入 

分 类 号：R762.5[医药卫生—耳鼻咽喉科]