早期糖尿病儿童听力学状况初步研究  

A preliminary audiology study in children with early diabetes

作　　者：刘薇[1] 张杰[1] 李靖[1] 刘海红[1] 吴迪[2] 郝津生[1] 杨杨[1] 张亚梅[1] 刘世琳[1] 葛文彤[1] 陈敏[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科研究所儿童耳鼻咽喉头颈外科疾病北京市重点实验室,北京100045 [2]首都医科大学附属北京儿童医院内分泌科,北京100045

出　　处：《中华耳科学杂志》2015年第3期393-397,共5页Chinese Journal of Otology

摘　　要：目的通过对糖尿病患儿进行全面的听力学检查,了解早期糖尿病儿童的听力情况。方法收集北京儿童医院2015年1月-2015年5月确诊糖尿病患儿40例,进行临床基本资料记录(病史、糖化血红蛋白水平、有无低血糖发作、尿微量白蛋白)及全面的听力学检查听性脑干反应Auditory Brainstem Response(ABR)、稳态多频诱发电位Auditory steady-state response(ASSR)、畸变产物耳声发射Distortion Product Oto-acoustic Emissions(DPOAE)、纯音测听、声导抗。对有家族遗传史患儿行基因检测。结果入选患儿共40名,其中男16名,女24名,年龄最大17.8岁,年龄最小0.4岁,中位年龄10岁。病程最短2天,最长1642天,中位病程为30天,听力测试时糖化血红蛋白水平由6.2-18.8%。40名受试者中,有听力下降主诉及糖尿病家族史及听力下降家族史的1名患儿表现为双耳重度感音神经性耳聋,基因检测结果血液中线粒体t RNAleu(uur)A3243G突变,突变比例为42.8%,尿液中A3243G有突变,突变比例为83.2%,确诊为线粒体糖尿病。其余患儿ABR、ASSR、DPOAE、纯音测听及声导抗检查均未见明显异常;但糖化血红蛋白、尿微量白蛋白正常和异常组之间V波潜伏期或I-V波间期有差异性(P<0.05)。结论早期糖尿病患儿的听力学存在亚临床改变,这种改变与糖尿病患儿的血糖控制情况有关,是一种早期的周围器官损害表现;对于有听力下降主诉及家族史的糖尿病患儿应行基因检测,以除外线粒体糖尿病。Objective This is a comprehensive audiology examination on children with diabetes aimed to determine if diabetes affected their hearings. Methods Data from 40 children with diabetes seen at Beijing Children＇s Hospital from January to May in 2015 were collected, including clinical information and comprehensive audiology examination（e.g. ABR, ASSR, DPOAEs, pure tone audiometry and acoustic immittance）. Mitochondrial genes were also tested in a patient with family history of hearing loss. Results Of the 40 patients, 16 were male and 24 were female, aged from 0.4 to 17.8 years（median = 10 years）. The course of disease was 2– 1642 days（median = 30 days）. Hb A1 c level at the time of audiology testing was 6.2- 18.8%. Audiology results were within normal limits in all patients except one child with reported hearing loss and family history of diabetes, whose genetic tests showed mitochondrial t RNA leu（uur） A3243 G mutation in blood（mutation ratio = 42.8%） and urine（mutation ratio = 83.2%） and was diagnosed with mitochondrial diabetes. ABR wave V latency and I-V interpeak latency in children with normal Hb A1 C and urine trace albumin were different from those showing abnormal results（P〈0.05）. Conclusion Children with diabetes of less than 5 years appear to carry subclinical hearing change, that is related to blood glycemic control and probably represents early peripheral organ damage. Genetic testing is important in children with reported hearing loss and family history of diabetes to identify mitochondrial diabetes.

关 键 词：糖尿病 听力损失 基因突变 

分 类 号：R578.1[医药卫生—消化系统]