復發性多軟骨炎合併極重度感覺神經性聽力損失：個案報告

Relapsing Polychondritis with Profound Sensorineural Hearing Loss: a Case Report

Relapsing polychondritis is a rare systemic disease characterized by recurrent inflammation and cartilage tissue destruction. Hearing impairment occurs in approximately 20% of patients with relapsing polychondritis. Most of the reported cases presented with mild to moderate hearing loss, whereas profound hearing loss is rarely seen. A 40-year-old woman with relapsing polychondritis experienced sudden bilateral hearing loss with tinnitus, intense dizziness and vertigo. An audiogram revealed a profound sensorineural hearing loss in the right ear and a moderate sensorineural hearing loss in the left ear. After high-dose corticosteroids and cyclophosphamide therapy, she had good hearing recovery in her left ear, but remained profoundly deaf in her right ear. Relapsing polychondritis may cause severe cochlear damage leading to irreversible profound hearing loss. Proper, timely treatment may provide an opportunity to reverse the hearing loss. 復發性多軟骨炎是一種罕見的全身性自體免疫疾病，臨床特徵為軟骨反覆發炎導致破壞變形。約有20％的復發性多軟骨炎患者會出現聽力受損，文獻報告的案例通常出現輕度到中度的聽力損失，極重度聽力損失的個案則相當少見。我們在此報告一個案，一位40歲女性復發性多軟骨炎患者，出現突發性雙耳聽力損失、耳鳴及眩暈。聽力檢查報告為右耳極重度和左耳中度的感覺神經性聽力損失，經高劑量類固醇及愛得星脈衝治療後左耳聽力有明顯改善，但右耳聽力無任何進步，仍是嚴重耳聾並且有耳鳴及眩暈症狀。復發性多軟骨炎可能導致嚴重且永久性的聽力損失，及早診斷及積極適當治療有助於增加聽力恢復的機率。