めまい・急激な聴力低下を訴えたＶａｎ　ｄｅｒ　Ｈｏｅｖｅ症候群の一例

DOI 被引用文献3件 参考文献18件

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  • A Case of Van der Hoeve syndrome.

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A 63-year-old man presented with bilateral hearing impairment of 25 years' durution. At 12 years, he complained of severe hearing loss and tinnitus, but no vertigo. Blue sclera was noted and the patient was also suffered from bone fracture four times, and he was diagnosed as having Van der Hoeve syndrome. A stapedotomy was performed in his left ear and subsequently his left hearing recovered. However, 5 years ago, he complained of progressive hearing loss and increased tinnitus again. Six months ago he complained of a sudden severe hearing loss along with vertigo. We operated on his left ear to replace a Teflon piston and performed stapedotomy in his right ear and the vertigo was disappeared. The patient's daughter, 38 years old, complained of unilateral hearing loss and vertigo attacks, and suffered from bone fracture several times in her childhood.<BR>In this case caloric response was diminished to a greater degree in his left ear than that of his right ear. A glycerol test showed positive in the left ear. The results of otoneurologic studies suggested that endolymphatic hydrops caused vertigo and sudden hearing loss in this patient.

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