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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 To understand the hearing status of children with early diabetes through comprehensive audiological examination of children with diabetes. Methods Forty children diagnosed with diabetes from January 2015 to May 2015 in Beijing Children’s Hospital were enrolled. Basic clinical records (history, glycosylated hemoglobin level, presence or absence of hypoglycaemia, urinary albumin) and comprehensive audiological examination were collected. Auditory steady-state response (ASSR), Distortion Product Oto-acoustic Emissions (DPOAE), pure tone audiometry, acoustic impedance. Genetic testing of children with a family history of genetic testing. Results A total of 40 children were selected, including 16 males and 24 females, the oldest being 17.8 years old, the youngest being 0.4 years old and the median age being 10 years old. The shortest course of 2 days, the longest 1642 days, the median duration of 30 days, hearing test hemoglobin level from 6.2 to 18.8%. Among the 40 subjects, one infant with deaf hearing loss, family history of diabetes and family history of hearing loss showed severe deafness of both ears. Gene test results showed that the mitochondrial tRNAleu (AUR) A3243G mutation in the blood, The mutation rate was 42.8%. There was a mutation in A3243G in urine and the mutation rate was 83.2%. The diagnosis was mitochondrial diabetes. There were no significant abnormalities in other children with ABR, ASSR, DPOAE, pure tone audiometry and acoustic conductivity test. However, there was significant difference in V wave latency or IV wave interval between normal and abnormal urinary albumin (P <0.05). Conclusion There is a subclinical change in the audiology of children with early diabetes, which is related to the glycemic control in children with diabetes. It is an early manifestation of peripheral organ damage. Children with diabetes who have a history of hearing loss and a family history should be treated Genetic testing to exclude mitochondrial diabetes.