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Kniest Dysplasia Accompanied with Profound I tearMg Loss:Report of a Case Hiroyosi Sugimura 1 1Department of Otolaryngology, Faculty of Medicine, University of Tokyo pp.111-115
Published Date 1994/2/20
DOI https://doi.org/10.11477/mf.1411900872
  • Abstract
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A 7-year-old boy with Kniest dysplasia was refer-red to us for evaluation of hearing. CT showed bony atresia of the right ear but no abnormality in theinner ear. The behavioral audiometry and auditory brain stem response suggested a profound hearing loss bilaterally : a sensorineural hearing loss in the right and a mixed hearing loss in the left. The damped rotation test showed normal response for his age.

Although he received auditory trainings with fit-ting of a hearing aid in his left ear, he did not develop speech and language.


Copyright © 1994, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1316 印刷版ISSN 0914-3491 医学書院

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