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作者报告1例发病初期就表现为重度耳聋及平衡障碍的病例,并结合文献进行了讨论。患者女性,41岁,主诉发作性眩晕。左耳聋、鸣及声嘶。面部无异常,两侧鼻腔及左耳鼓膜、鼻咽部、声门可见坏死性肉芽;左耳骨、气导消失,右耳气导30dB、骨导10dB;平衡机能检查,左侧半规管麻痹;左侧乳突呈硬化型、半规管附近阴影不清;肺无异常,肾功正常;鼻腔活检,可见伴有纤维蛋白样变性的血管炎及肉芽性炎症。开始内服强地松龙50mg/日及环磷酰胺100mg/日,约半月后病变基本消失,左耳听力及半规管麻痹无改变:6周后强的松龙减量,停用环磷酰胺;3个
The authors report a case of early onset of severe deafness and balance disorders, and the literature was discussed. Female, 41, complains of episodic dizziness. Left deaf, Ming and hoarseness. No abnormalities in the face, nasal and left ear on both sides of the tympanic membrane, nasopharynx, glottis visible necrotic granulation; left ear bones, air conduction disappears, right ear air conduction 30dB, bone conduction 10dB; balance function check, left semicircular canal paralysis; Left mastoid sclerosis, semicircular canal near the shadow of unclear; no lung abnormalities, normal renal function; nasal biopsy, with fibrinous degeneration associated with vasculitis and granulomatous inflammation. Begin oral prednisolone 50mg / day and cyclophosphamide 100mg / day, about half a month after the lesions disappeared, left ear hearing and semi-regulatory paralysis no change: 6 weeks after the reduction of prednisolone, disable cyclophosphamide; 3