van der Hoeve 症候群例

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タイトル別名
  • A Case of van der Hoeve Syndrome
  • van der Hoeve ショウコウグンレイ

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A case of van der Hoeve syndrome in a 7-year-old Japanese male is reported here. He demonstrated three symptoms: blue sclera, osteogenesis imperfecta and conductive hearing loss.<br>Pure tone audiometry showed a 61.7dB hearing loss in the left ear. Tympanogram was type Ad and stapedial reflex was absent. Therefore, dislocation of ossicular chain was suspected.<br>At surgery, it was observed that the bilateral stapedial crura had fractured spontaneously, but the stapedial footplate was mobile. Using tympanoplasty IV-i type, we reconstructed the conduction with an artificial ossicle. The patient obtained an improvement in his hearing level in the immediate postoperative period.

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