続・補聴器装用効果の評価　　聴覚心理検査の見直し　　感音性難聴および平衡機能障害を呈するＦａｂｒｙ病の１症例

• 吉川 智子

  東北大学耳鼻咽喉科

• 工藤 貴之

  東北大学耳鼻咽喉科

• 池田 勝久

  東北大学耳鼻咽喉科

書誌事項

タイトル別名

• A Case of Fabry's Disease with Sensor-ineural Hearing Loss and Imbalance.

抄録

We reported a case of Fabry disease in 40-year old man. The patient complained bilateral hearing loss, tinnitus, non-rotational nystagmus. The disease occurred in virtually all areas of the body, including chronic renal insufficiency, simple angioma, angiokeratoma, and left ventricular hypertrophy. Analysis of the clinical data from DPOAE, ABR and nystagmus test revealed cochlear deafness, retrolabyrinthine deafness and peripheral and central imbalance. These findings suggested that the accumulation of glycosphingolipids in the stria vascularis and nerve cell into the brainstem caused deafness. In addition, it is also possible that deposits in glomeruli and tubules of the kidney were secondary to bilateral hearing loss.

収録刊行物

• ＡＵＤＩＯＬＯＧＹ　ＪＡＰＡＮ

  ＡＵＤＩＯＬＯＧＹ　ＪＡＰＡＮ 44 (3), 135-141, 2001

  一般社団法人 日本聴覚医学会

キーワード

• Fabry病
• リソゾーム病
• グリコスフィンゴリピド
• 感音性難聴

詳細情報

• CRID: 1390282679814479360
• NII論文ID: 130003862984
• DOI: 10.4295/audiology.44.135
• ISSN: 18837301
• ISSN: 03038106
• 本文言語コード: ja
• データソース種別:
  • JaLC
  • Crossref
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可