両側感音難聴を呈したサルコイドーシスの一例

  • 大野 十央
    東京医科歯科大学大学院医歯学総合研究科耳鼻咽喉科学分野
  • 野口 佳裕
    東京医科歯科大学大学院医歯学総合研究科耳鼻咽喉科学分野
  • 戸叶 尚史
    東京医科歯科大学大学院医歯学総合研究科耳鼻咽喉科学分野
  • 畑中 章生
    東京医科歯科大学大学院医歯学総合研究科耳鼻咽喉科学分野
  • 喜多村 健
    東京医科歯科大学大学院医歯学総合研究科耳鼻咽喉科学分野

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  • Bilateral sensorineural hearing loss in a patient with sarcoidosis

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Sarcoidosis is a systemic granulomatous disease of unknown etiology. We report a 27-year-old woman with systemic sarcoidosis and bilateral sensorineural hearing loss (SNHL). Other findings included granulomatous uveitis, dysfunction of the bilateral fifth cranial nerves, and hilar adenopathies. She first suffered from right sudden hearing loss and consulted an otolaryngologist. A pure-tone audiogram revealed a right highfrequency SNHL that spontaneously recovered. One month later, she presented at our department with left sudden hearing loss, tinnitus, and aural fullness. A pure-tone audiogram revealed a left Ushaped SNHL and a right mild SNHL. Further audiological examinations including speech audiometry, self-recording audiometry, distortionproduct otoacoustic emissions, electrocochleography, and auditory brainstem responses revealed cochlear dysfunction with a cochlear nerve conduction block. Steroid therapy for a 10-day period was insufficient, and recovery from the left SNHL was incomplete. The patient was diagnosed as having systemic sarcoidosis based on pulmonological and serological studies. During the course of her illness, her left SNHL normalized four months after onset. Although transient right SNHL occurred, the patient's normal hearing persisted. The clinical course of her hearing loss could not entirely refute a diagnosis of endolymphatic hydrops.

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