先天性外耳道狭窄症に伴う外耳道真珠腫により蝸牛瘻孔を生じた1例

  • 瀧 正勝
    京都府立医科大学大学院耳鼻咽喉科・頭頸部外科学分野
  • 辻川 敬裕
    京都府立医科大学大学院耳鼻咽喉科・頭頸部外科学分野
  • 藤田 朋己
    京都府立医科大学大学院耳鼻咽喉科・頭頸部外科学分野
  • 任 書晃
    京都府立医科大学大学院耳鼻咽喉科・頭頸部外科学分野
  • 長谷川 達央
    京都府立医科大学大学院耳鼻咽喉科・頭頸部外科学分野
  • 坂口 博史
    京都府立医科大学大学院耳鼻咽喉科・頭頸部外科学分野
  • 山本 聡
    京都府立医科大学大学院耳鼻咽喉科・頭頸部外科学分野
  • 兵庫 美砂子
    京都府立医科大学大学院耳鼻咽喉科・頭頸部外科学分野
  • 鈴木 敏弘
    京都府立医科大学大学院耳鼻咽喉科・頭頸部外科学分野
  • 久 育男
    京都府立医科大学大学院耳鼻咽喉科・頭頸部外科学分野

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  • A case of cochlear fistula caused by cholesteatoma of the external auditory canal with congenital auditory stenosis

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A 10-year-old girl was found a speech delay by her mot he r at age of 4 years.<BR>A local ENT physician diagnosed of bilateral congenital auditory stenosis and referred her to our hospital for further evaluation. At that time CT revealed no anomalies of the middle and inner ear, and auditory brain stem response and play audiometry showed the threshold of 60 dB in both ears. Hearing aids were fitted for her ears, thereafter she was followed up in our hospital and by the referring physician. Since January 2004, otorrhea in both ears was repeatedly observed. On March 2007, she hospitalized for surgery. On the day of admission, the size of bilateral external auditory canals was like a pin-hole. Pulsating otorrhea was observed in the left ear. CT revealed the bony stenosis of bilateral external auditory canals, and soft tissue density in the tympanic cavity to antrum and bone destruction at the promontory in the left ear. Three days after her hospitalization, she underwent planned staged tympanoplasty and meatoplasty in the left ear. Cholesteatoma filled in the tympanic cavity and cochlear fistula was found at the promontory. After the operation, she had neither vertigo nor hearing loss in bone conduction.<BR>Most of inner ear fistula in the cases with cholesteatoma are found on the lateral semicircular canal, but cochlear fistula is rare. Cholesteatoma stored at the lateral side of the promontory might have destructed bone over the cochlear and made a fistula in this patient. Any patient with congenital auditory stenosis who has repeating infection should have the surgical intervention as soon as possible.

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