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

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感音難聴と顔面神経麻痺を示したMPO-ANCA関連中耳疾患症例を報告した。耳閉感で初発し、滲出性中耳炎の治療を受けるも両感音難聴が進行した。ステロイドの効果は一時的であり、その後、左顔面神経麻痺が出現した。側頭骨CTでは中耳に軟部陰影を認めたが、骨破壊なし。PR3-ANCA陰性、MPO-ANCA陽性であったが、肺・腎病変はなく、中耳組織に血管炎の所見を認めなかった。ステロイドと免疫抑制薬の投与により、難聴と顔面神経麻痺は改善した。このような感音難聴と顔面神経麻痺を示すMPO-ANCA関連中耳疾患は、MPO-ANCAによる血管炎が関与している可能性があるが、組織検査でANCA関連血管炎と診断できない。また、本疾患はANCA関連血管炎とは臨床的特徴が異なっていた。本疾患はステロイドと免疫抑制薬の併用治療が有効であるため、免疫抑制剤の使用の根拠となる診断名が必要と考え、診断基準とともに提案した。

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.

収録刊行物

  • Otology Japan

    Otology Japan 21(5), 808-815, 2011-12-26

    日本耳科学会

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各種コード

  • NII論文ID(NAID)
    10030285311
  • NII書誌ID(NCID)
    AN10358085
  • 本文言語コード
    JPN
  • 資料種別
    ART
  • ISSN
    09172025
  • データ提供元
    CJP書誌  CJP引用  J-STAGE 
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