- 期刊
Mondini Dysplasia with Recurrent Meningitis
Mondini發育不良併發反覆性腦腦膜炎
摘要
Mondini氏發育不良是一種先天性內耳發育異常，多合併有先天性外淋巴孌管。臨床症狀以反複性腦膜炎，漸進性單側聽力障礙、平衡障礙，及腦脊髓液耳漏來表現居多。診斷方法靠高解析度電腦斷層攝影顯示內耳迷路構造異常。這類病例需要施予中耳探查，進行前庭充填術來阻止腦膜炎的再發和聽力惡化，但往往要進行多次手術才能成功。本文報告兩例病案以反複性腦膜炎就醫，經高解析度電腦斷層攝影證實有內耳先天異常，診斷爲Mondini發育不良，分別經過一、二次中耳手術才成功地阻止了腦膜炎之再發。
並列摘要
Abstract Mondini dysplasia is a congenital malformation of the inner ear, commonly associated with hearing impairment, cerebrospinal fluid otorrhea/rhinorrhea and recurrent meningitis. Two such cases are described, with hearing impairment, cerebrospinal fluid rhinorrhea, and several episodes of meningitis. Diagnosis was confirmed by high-resolution computed tomography. After surgical correction of the malformation, there was no recurrent episode of meningitis at subsequent follow-up. To avoid the suffering and the sequelae of recurrent meningitis, an early diagnosis and prompt surgical intervention are crucial for such patients.
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