MPO-ANCA-associated middle ear disease with sensorineural hearing loss and facial palsy: case report, review of literature, proposed diagnostic criteria

DOI 4 Citations 24 References
  • Takeda Noriaki
    Department of Otolaryngology, University of Tokushima School of Medicine
  • Kamimura Seiichiro
    Department of Otolaryngology, University of Tokushima School of Medicine
  • Chida Izumi
    Department of Otolaryngology, University of Tokushima School of Medicine
  • Kitamura Yoshiaki
    Department of Otolaryngology, University of Tokushima School of Medicine
  • Jinnouchi Osamu
    Department of Otolaryngology, University of Tokushima School of Medicine
  • Abe Koji
    Department of Otolaryngology, University of Tokushima School of Medicine
  • Tamura Koichi
    Department of Otolaryngology, University of Tokushima School of Medicine
  • Udaka Jiro
    Udaka ENT Clinic
  • Endo Aki
    Department of Otolaryngology, Shikoku Central Hospital

Bibliographic Information

Other Title
  • 感音難聴と顔面神経麻痺を示すMPO-ANCA関連中耳疾患の1例: 診断基準案の提案

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Abstract

We reported a case of MPO-ANCA-associated middle ear disease with sensorineural hearing loss and facial palsy. A 62-year-old female first complained of bilateral aural obstruction and the treatment for otitis media with effusion was ineffective. Then, bilateral sensorineural hearing loss developed, and after the transient effect of steroid therapy, left facial palsy appeared. CT demonstrated that the low density area in the middle ear cavity without bone destruction, and its granular tissue surgically resected showed non-specific findings by the histopathological examination. MPO-ANCA was positive, but PR3-ANCA was negative in the serum without pulmonary and renal lesions. Steroid treatment with cyclophosphamaide improved the hearing loss and facial palsy with a decrease of serum MPO-ANCA level.<br> Although it was reported previously that patients with MPO-ANCA-associated middle ear disease with sensorineural hearing loss and facial palsy were successfully treated by combination therapy with steroid and immunosuppressive drug in addition to our case, they could not meet the criteria of ANCA-associated vasculitides such as Wegener's glanulomatosis. Therefore, we proposed the diagnostic term and criteria of MPO-ANCA-associated middle ear disease with sensorineural hearing loss and facial palsy. The underlying pathophysiology of this disease may be MPO-ANCA-induced vasculitis, but review of literature showed that its clinical characteristics are different from those of ANCA-associated vasculitides.<br>The proposed diagnostic criteria were as follows: 1) the onset of otitis media-like symptom, 2) progressed sensorineural hearing loss, 3) complicating facial palsy, 4) lesion limited to the middle ear without pulmonary and renal lesions, 5) MPO-ANCA positive, but PR3-ANCA negative in the serum, 6) no findings of vasculitis in the middle ear lesion.

Journal

  • Otology Japan

    Otology Japan 21 (5), 808-815, 2011

    Japan Otological Society

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