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Kennedy병에 의한 개방성 이관 1예

A Case of Patulous Eustachian Tube Associated with Kennedy Disease

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초록

We report a 53-year-old male having Kennedy disease who complained of severe bilateral tinnitus and autophony. He was diagnosed as patulous Eustachian tube based on the observation of fluttering motion of the tympanic membrane associated with respiration, which is presumed to occur secondary to bulbar weakness. The patulous Eustachian tube affected the management of his respiratory problem by impairing his ability to tolerate BiPAP. We report this rare condition with a literature review.

Abstract

We report a 53-year-old male having Kennedy disease who complained of severe bilateral tinnitus and autophony. He was diagnosed as patulous Eustachian tube based on the observation of fluttering motion of the tympanic membrane associated with respiration, which is presumed to occur secondary to bulbar weakness. The patulous Eustachian tube affected the management of his respiratory problem by impairing his ability to tolerate BiPAP. We report this rare condition with a literature review.

참고문헌 (8)

  1. Kennedy WR, "Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait", Neurology, 18, pp.671~680 (1968)
  2. Finsterer J, "Bulbar and spinal muscular atrophy (Kennedy’s disease): a review", Eur J Neurol, 16, pp.556~561 (2009)
  3. Takasaki K, "The patulous Eustachian tube complicated with amyotrophic lateral sclerosis: a video clip demonstration", Laryngoscope, 118, pp.2057~2058 (2008)
  4. Schellenberg K, "Roaring in the ears: patulous Eustachian tube in bulbar amyotrophic lateral sclerosis", Amyotroph Lateral Scler, 11, pp.395~396 (2010)
  5. O'Connor AF, "Autophony and the patulous eustachian tube", Laryngoscope, 91, pp.1427~1435 (1981)
  6. Pitman LK, "The open Eustachian tube", Arch Otolaryngol, 9, pp.494~500 (1929)
  7. Takasaki K, "Histopathologic study of the human Eustachian tube and its surrounding structures following irradiation for carcinoma of the oropharynx", Arch Otolaryngol Head Neck Surg, 126, pp.543~546 (2000)
  8. Tsuji T, "Moriyama H. Mastoid pneumatization of the patulous Eustachian tube", Ann Otol Rhinol Laryngol, 109, pp.1028~1032 (2000)

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