Neuro-Sweets' disease in a patient with bilateral recurrent acute sensorineural hearing loss

DOI 10 References
  • Wada Tadashi
    Department of Otolaryngology, Niigata University Faculty of Medicine
  • Satoh Hitoshi
    Department of Otolaryngology, Niigata University Faculty of Medicine
  • Izumi Syuji
    Department of Otolaryngology, Niigata University Faculty of Medicine
  • Kubota Yamato
    Department of Otolaryngology, Niigata University Faculty of Medicine
  • Ueki Yushi
    Department of Otolaryngology, Niigata University Faculty of Medicine
  • Fujisaki Toshiyuki
    Department of Otolaryngology, Niigata University Faculty of Medicine
  • Takahashi Sugata
    Department of Otolaryngology, Niigata University Faculty of Medicine

Bibliographic Information

Other Title
  • 両側急性感音難聴を反復したｎｅｕｒｏ‐Ｓｗｅｅｔ病と考えられた１症例

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Abstract

Sweets' syndrome is an usual disease characterized by fever, leukocytosis, and distinctive skin lesions. The involvement of the central nervous system in Sweets' syndrome is termed neuro-Sweet disease. Only a few reports have mentioned sensorineural hearing loss in neuro-Sweet disease. Here, we present a case of neuro-Sweet disease with sensorineural hearing loss. A 33-year-old man experienced bilateral recurrent acute sensorineural hearing loss with meningitis and encephalitis. Magnetic resonance images showed increased signal intensity in the white matter on T 2-weighted and FLAIR scans. HLA typing revealed B54 and CW1, which are frequently observed in Sweets' syndrome. Steroid therapy was remarkably effective for alleviating the sensorineural hearing loss as well as the meningitis and encephalitis. Analysis of an SISI test, Bekesy test, auditory brain stem response and SPELT abnormality revealed both cochlear and brainstem lesions.<br>Neuro-Sweet disease should be included in a differential diagnosis of unexplainable recurrent acute sensorineural hearing loss complicated by meningitis and encephalitis.

Journal

  • AUDIOLOGY JAPAN

    AUDIOLOGY JAPAN 49 (3), 254-259, 2006

    Japan Audiological Society

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