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首页> 外文期刊>The Journal of laryngology and otology. >Cochlear implantation in a patient with profound hearing loss with the A1555G mitochondrial DNA mutation and no history of aminoglycoside exposure.
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Cochlear implantation in a patient with profound hearing loss with the A1555G mitochondrial DNA mutation and no history of aminoglycoside exposure.

机译：患有严重听力损失且患有A1555G线粒体DNA突变且无氨基糖苷暴露史的患者进行耳蜗植入。

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摘要

The A1555G mitochondrial deoxyribonucleic acid (mtDNA) point mutation has classically been associated with sensorineural hearing loss in patients following aminoglycoside exposure. More recently, the mutation has been implicated in sensorineural hearing loss in patients without previous aminoglycoside use. In addition, cochlear implantation has been shown to be effective in the group of patients with prior aminoglycoside exposure but, to date, no case of cochlear implantation in a patient with the A1555G mutation and no prior exposure to aminoglycosides has been explicitly described in the literature. We report the case of an 80-year-old woman with the A1555G mtDNA mutation, a 35-year history of bilateral progressive hearing loss and no history of aminoglycoside exposure who underwent successful implantation of a Nucleus 24 Contour device at our institution. Post-operatively, the patient exhibited marked improvement in tests of auditory performance. We conclude that cochlear implantation can be an effective method to restore some sense of hearing in patients with the A1555G mtDNA mutation and sensorineural hearing loss.
机译：传统上，A1555G线粒体脱氧核糖核酸（mtDNA）点突变与氨基糖苷暴露后患者的感音神经性听力损失有关。最近，该突变与以前未使用氨基糖苷的患者的感觉神经性听力下降有关。另外，已证明在先前有氨基糖苷暴露的患者组中，人工耳蜗是有效的，但是迄今为止，文献中没有明确描述具有A1555G突变的患者中的人工耳蜗的病例，也没有明确描述对氨基糖苷的先前暴露。 。我们报道了一个在我们机构成功植入Nucleus 24 Contour装置的80岁女性的案例，该女性患有A1555G mtDNA突变，双边进行性听力丧失35年，无氨基糖苷暴露史。术后，患者的听觉表现测试显示出明显的改善。我们得出结论，人工耳蜗植入可以是一种有效的方法，可以恢复患有A1555G mtDNA突变和感觉神经性听力损失的患者的某种听觉。

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