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Cochlear implantation for hearing loss due to an A8296G mitochondrial DNA mutation

机译：耳蜗植入治疗由于A8296G线粒体DNA突变引起的听力损失

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ObjectiveTo characterize the clinical findings in a patient with hearing loss harboring an A8296G mitochondrial DNA mutation and the outcome of cochlear implantation.PatientsA case report of a patient with hearing loss caused by an A8296G mitochondrial DNA mutation.InterventionThe patient underwent cochlear implantation (CI).Main outcome and results: Bilateral moderate to severe hearing loss was found at high school age and progressed to severe hearing loss bilaterally at the age of 22. The patient's low-tone hearing was relatively well preserved compared to high frequency, although it eventually declined. Speech perception in silence and at S/N10 improved to 100% and 92% for sentences, respectively, 3 years after CI.ConclusionsWe detailed the case of a patient with hearing loss due to an A8296G mitochondrial DNA mutation. Bilateral progressive hearing loss starting from high frequency was observed. Speech discrimination after CI was very good, indicating that a patient with this mutation is a good candidate for CI.
机译：目的表征具有A8296G线粒体DNA突变的听力障碍患者的临床表现和人工耳蜗植入的结果。患者因A8296G线粒体DNA突变导致听力丧失的患者的病例报告。主要结果和结果：在高中年龄时发现双侧中度至重度听力下降，在22岁时双侧发展为严重听力下降。与高频相比，患者的低音听力得到相对较好的保存，尽管最终会下降。 CI后3年，静默状态和S / N10语音的言语感知能力分别提高到100％和92％。结论我们详细介绍了由于A8296G线粒体DNA突变而导致听力丧失的患者。观察到从高频开始双侧进行性听力损失。 CI后的言语辨别非常好，表明具有此突变的患者是CI的良好候选人。

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