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題 名 | Sensorineural Hearing Loss in Melas Syndrome--Case Report=MELAS症候群之感音性聽力喪失--病例報告 |
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作 者 | 陳俊男; 何坤瑤; 阮愷輝; | 書刊名 | The Kaohsiung Journal of Medical Sciences |
卷 期 | 14:8 1998.08[民87.08] |
頁 次 | 頁519-523 |
分類號 | 416.865 |
關鍵詞 | 感音性聽力喪失; MELAS; SNHL; |
語 文 | 英文(English) |
中文摘要 | 粒線體核糖核甘酸3243的突變造成MELAS症候群的相關性常見於大部份病人。我們 提出一例三十歲女性 MELAS 的病人, 四年前因兩側耳鳴及漸進性聽力喪失前來耳鼻喉科門 診求診。間隔三年半的兩次純音聽力檢查顯示兩側對稱性漸進的感音性聽力喪失,特別在高 頻區。 感音性聽力喪失在 MELAS 病人是一個重要的臨床特徵, 大約可見於百分之三十的 MELAS 病人,也通常是臨床上首先表現的徵狀。因此,當病人表現出成人型兩側的漸進的感 音性聽力喪失及合併其他神經學症狀時,要考慮是否為粒線體病變所引起的疾病。 |
英文摘要 | A mitochondrial tRNA mutation at nucleotide 3,243 is known to be found in most patients with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes). We report a 30 year-old female patient of MELAS, diagnosed 5 years ago, who presented herself to our ENT outpatient department because of her bilateral tinnitus and progressive hearing impairment since 4 years ago. Two sequential pure tone audiograms showed bilateral symmetrical progressive sensorineural hearing loss, especially in the high frequency area in 1993 and 1996. The pure tone average was R-45 dB, L-47 dB in 1993 and R-62 dB, L-67 dB in 1996. Hearing loss is an important feature in MELAS syndrome and reported to be seen in about 30% of patients. It is often the first clinical symptom, too. In any case, mitochondrial cytopathies need to be considered by the otologist in forming a diagnosis of sensorineural hearing loss (SNHL), particularly in cases, which present adult-onset progressive hearing loss and neurologic symptoms before 50 years of age. |
本系統中英文摘要資訊取自各篇刊載內容。