A Case of Duane's Retraction Syndrome with Dysplasia of the Cochlea

DOI 16 References
  • Ashizawa Kei
    Department of Otolaryngology, Head and Neck Surgery, Tsukuba University Hospital, Tsukuba
  • Tabuchi Keiji
    Department of Otolaryngology, Graduate School of Comprehensive Human Sciences, University of Tsukuba
  • Nakayama Masahiro
    Department of Otolaryngology, Head and Neck Surgery, Tsukuba University Hospital, Tsukuba
  • Hara Akira
    Department of Otolaryngology, Graduate School of Comprehensive Human Sciences, University of Tsukuba

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Other Title
  • 蝸牛形成不全を合併したDuane症候群の一症例

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Description

Duane's retraction syndrome (DRS) is a congenital ocular motility disorder that may be caused by absence of the VIth nerve nucleus. DRS is usually a sporadic occurrence, but there is significantly increased association with systemic abnormalities such as hearing deficits and spinal anomalies. We describe herein a case of DRS with unilateral hearing loss in a 41-year-old man. He was admitted to our hospital for tonsillectomy. He complained of left congenital ocular immobility and left deafness. The ocular immobility revealed left DRS. A computed tomography (CT) scan of the left ear demonstrated underdevelopment of the bony labyrinth. It is mandatory for otolaryngologists to take DRS into consideration for differential diagnosis of abnormality of ocular mobility, and hearing examinations are to be considered when DRS is diagnosed.

Journal

  • Otology Japan

    Otology Japan 19 (5), 672-675, 2009

    Japan Otological Society

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