| A Case of Relapsing Polychondritis Presenting as a Bilateral Sequential Perilymphatic Fistula |
Soomin Jo1
, Doo Sik Park1
, Haewon Rhi1
, Hyo-Jeong Lee1,2
|
1Department of Otorhinolaryngology-Head and Neck Surgery, Hallym University College of Medicine, Anyang, Korea
2Laboratory of Hearing, Balance and Integrated Neuroscience (HBIN), Hallym University College of Medicine, Anyang, Korea |
| 양측에 순차적으로 발생한 외림프 누공으로 발현된 재발성 다발 연골염 1예 |
| 조수민1
, 박두식1
, 이혜원1
, 이효정1,2
|
1한림대학교 의과대학 이비인후-두경부외과학교실
2한림대학교 의과대학 청각평형융합신경과학 연구소 |
| Correspondence |
Hyo-Jeong Lee ,Tel: +82-31-380-3840, Fax: +82-31-386-3860, Email: hyojlee@hallym.ac.kr
|
|
|
Received: 3 March 2026; Revised: April 1, 2026 Accepted: 3 April 2026. Published online: 24 June 2026. |
|
|
|
| ABSTRACT |
|
Relapsing polychondritis (RPC) is a rare systemic autoinflammatory disease that primarily affects cartilaginous organs. Inner ear involvement occurs in up to 40% of patients, leading to fluctuating or sudden audiovestibular dysfunction. The proposed mechanisms include autoimmune reactions to the membranous labyrinth and immune-mediated vasculitis. We report the case of a 21-year-old man with RPC who presented with sequential bilateral perilymphatic fistula (PLF) and acute audiovestibular loss. He first experienced aggravated vertigo and fluctuating hearing loss (HL) on the left side 5 days before the visit. Exploratory tympanotomy for the suspected PLF produced immediate improvement. Twenty days later, he experienced sudden vertigo, right-sided HL, and bilateral auricular swelling. CT revealed soft-tissue density in the right round-window niche, and perilymph leakage was confirmed surgically. Bilateral auricular chondritis and episcleritis prompted the diagnosis of RPC. This case suggests that PLF may represent an additional mechanism of rapid audiovestibular loss in RPC and that prompt surgical repair with immunosuppression may preserve hearing. |
| Keywords:
Perilymphㆍ Relapsing polychondritisㆍ Sensorineural hearing lossㆍ Vestibular diseases |
|
PDF Links
Full text via DOI
Download Citation 
