以聽神經瘤表现之神經纖維瘤病－病例報告

Neurofibromatosis Presenting with an Acoustic Neuroma-Case Report

梁漢珍(Han-Chen Liang) ； 洪敬賢(Ching-Hsien Hung) ； 楊玉綉(Yu-Hsiu Yang) ； 张燕良(Yen-Liang Chang)

《中華民國聽力語言學會雜誌》 20期 (2007/06) Pp. 88-96

https://doi.org/10.6143/JSLHAT.2007.06.07

摘要

神經纖維瘤病(neurofibromatosis)分爲第一型(NF1)和第二型(NF2)，兩者最大的不同是NF2會出现雙側或單側的聽神經瘤，鑑别診断還需依據National Institutes of Health (NIH)所訂的標凖。本科發现一例疑似第二型神經纖維瘤病的女性患者，該患者除了有頸部腫塊之外，並主訴聽力漸差、耳鳴，偶有頭暈。聽力檢查結果：患耳呈现高頻聽障，語音分辨不佳；鐙骨肌反射檢查和響音衰退檢查皆顯示有耳蝸俊病變；聽性腦幹反應檢查也顯示右耳的第五波絕對潛時值異常延後；變頻耳聲傅射檢查則排除耳蝸病變的可能。最後，磁振攝影確定患者有單側聽神經瘤及頸椎旁腫瘤。除安排患者手術切除頸部腫瘤外，針對只有1.7×0.8cm的小聽神經瘤，建議以立體定位放射手術治療，以維護患者術俊的生活品質。

關鍵字

第二型神經纖維瘤 ； 聽神經瘤

並列摘要

Two distinct forms of neurofibromatosis have been recognized: neurofibromatosis type 1 (NF1) and type 2 (NF2). The hallmark of NF2 is the development of vestibular schwannomas (VS). The diagnoses of NF1 and NF2 are based on the clinical criteria developed by the National Institutes of Health (NIH). The case presented in this paper is a female patient with neck masses, complaining tinnitus, progressive hearing loss, and dizziness. The routine hearing tests revealed an asymmetric hearing loss and decreased speech discrimination in the right ear. Acoustic reflex and tone decay studies indicated a retrocochlear lesion. Auditory brainstem responses also showed a prolonged absolute latency of wave V. DPOAE tests ruled out the possibility of cochlea pathological change. Therefore, she was highly suspected to be a victim of NF2. Finally, an MRI scan confirmed that the patient had a unilateral vestibular schwannoma (l.7×0.8 cm) and cervical paraspinal tumors. The patient was arranged to receive excision of the neck tumors first. As for the small vestibular schwannoma, stereostatic radiosurgery was suggested to maintain a good quality of life postoperatively.

並列關鍵字

neurofibromatosis type 2 ； acoustic neuroma

參考文獻

Gutmann DH,Aylsworth A,Carey J(1997).The Diagnostic Evaluation and Muitidisciplinary Management of Neurofibromatosis 1 and Neurofibromatosis 2.JAMA.278,51-57.

戴念國、秦凌霄(1997)。多發性神經纖維瘤。臨床醫學。40，356-36。

紀慶鍠(1995)。神經纖維瘤病。台灣醫界。38，991-992。

Evans DCR,Sainio M,Baser ME(2000).Neurofibromatosis type 2.J Med Genet.37,897-904.

Chen AF,Samy RN,Gantz BJ(2001).Cerebellopontine angle tumor composed of schwann and meningeal proliferations.Arch Otolaryngol Head Neck Surg.127,1385-1389.