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首页> 外文期刊>The Laryngoscope: A Medical Journal for Clinical and Research Contributions in Otolaryngology, Head and Neck Medicine and Surgery, Facial Plastic and Reconstructive Surgery .. >Sensorineural hearing loss, diabetes, and mitochondrial DNA mutation in Taiwan.
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Sensorineural hearing loss, diabetes, and mitochondrial DNA mutation in Taiwan.

机译：台湾的感音神经性听力丧失，糖尿病和线粒体DNA突变。

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Patients with maternally inherited diabetes and deafness (MIDD) usually were young at diabetes onset.1 They were characterized by a point mutation at nucleotide pair (np) 3243 of the mitochondrial DNA (mtDNA). The auditory defect is a progressive bilateral sensorineural hearing loss that first affects high frequencies. The most characteristic audiographic feature is sloping, with flat profiles in advanced cases.2 The incidence of sensorineural deafness for patients harboring the mtDNA A3243G mutation and DM is about 15%.3 Our previous literature reported the clinical features of 67 diabetic patients with unilateral sudden sensorineural hearing loss (SSNHL).4 The patients had a mean age of 60.1 years. The most commonly seen audiologic change was profound hearing loss. The sudden unilateral hearing loss could recover, though not completely, after treatment. The hearing loss in our patients is obviously different from what observed in MIDD. In chart review, none of our patients had family history or otherclinical characteristics suggesting mitochondrial disease.
机译：患有母亲遗传性糖尿病和耳聋（MIDD）的患者通常在糖尿病发作时才年轻。1他们的特征是线粒体DNA（mtDNA）的核苷酸对（np）3243处有点突变。听觉缺陷是首先影响高频的进行性双侧感觉神经性听力损失。听力特征最典型的特征是倾斜，在晚期病例中呈扁平轮廓。2携带mtDNA A3243G突变和DM的患者的感音神经性耳聋发生率约为15％。3我们以前的文献报道了67例单侧突然糖尿病患者的临床特征感觉神经性听力损失（SSNHL）。4患者的平均年龄为60.1岁。最常见的听觉改变是严重的听力损失。治疗后突然的单侧听力损失可以恢复，尽管不能完全恢复。我们患者的听力损失明显不同于MIDD。在图表审查中，我们的患者均无家族病史或其他临床特征提示线粒体疾病。

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