A CASE OF BILATERAL PROGRESSIVE SENSORINEURAL HEARING LOSS CAUSED BY SUPERFICIAL SIDEROSIS OF THE CENTRAL NERVOUS SYSTEM

DOI
  • Kamoshida Risa
    Department of Otorhinolaryngology, Yokohama Sakae Kyosai Hospital
  • Takao Natsumi
    Department of Otorhinolaryngology, Yokohama Sakae Kyosai Hospital
  • Nakagawa Chihiro
    Department of Otorhinolaryngology, Yokohama Sakae Kyosai Hospital

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Other Title
  • 両側感音難聴で発見された脳表ヘモジデリン沈着症の1症例

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Abstract

<p>　Superficial siderosis of the central nervous system (SSCN) is a rare disease of the central nervous system characterized by sensorineural hearing loss, ataxia, and pyramidal tract signs.</p><p></p><p></p><p>　The symptoms are caused by hemosiderin deposition in the leptomeninges, subpial tissue, spinal cord and cranial nerves as a result of chronic bleeding into the subarachnoid space.</p><p></p><p>　Previously, the diagnosis of SSCN could only be made at autopsy or during a neurosurgical procedure. However, the diagnosis of SSCN can now be made non-invasively, by MRI.</p><p></p><p>　We present the case of a 79-year-old male with SSCN who presented with bilateral hearing loss. The diagnosis of SSCN was made by brain MRI, based on identification of diffuse marginal hypointensity of the cerebrum, brainstem and cerebellum on T2-weighted images.</p><p></p><p>　SSCN should be considered in the differential diagnosis of unexplained progressive sensorineural hearing loss. MRI is a useful non-invasive imaging modality for accurate diagnosis of SSCN.</p>

Journal

  • JIBI INKOKA TEMBO

    JIBI INKOKA TEMBO 60 (5), 226-230, 2017-10-15

    Society of Oto-rhino-laryngology Tokyo

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