{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T00:05:44Z","timestamp":1756339544344,"version":"3.44.0"},"reference-count":21,"publisher":"BMJ","issue":"8","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,8,19]]},"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:p>A woman in her 20\u2009s with a remote history of chronic iritis and bilateral sensorineural hearing loss presented with proptosis, eye pain, blurred vision and elevated intraocular pressures. Her medical history was significant for end-stage renal disease and congestive heart failure. Imaging was limited due to cochlear implants and a cardioverter defibrillator. Infectious and autoimmune investigations were negative. After initial management with topical pressure-lowering drops, she was started on systemic corticosteroids with rapid improvement in visual acuity and resolution of symptoms. The diagnosis of atypical Cogan syndrome was made by exclusion, based on her exam findings, ocular and auditory history, systemic features and positive clinical response to treatment. This case highlights the diagnostic challenges of atypical Cogan syndrome, particularly in the setting of delayed symptom onset and systemic involvement. Early recognition is essential, as timely corticosteroid therapy may prevent irreversible vision and hearing loss.<\/jats:p>","DOI":"10.1136\/bcr-2025-267523","type":"journal-article","created":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T17:15:35Z","timestamp":1756314935000},"page":"e267523","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Atypical Cogan\u2019s syndrome with delayed auditory and ocular manifestations"],"prefix":"10.1136","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-4140-696X","authenticated-orcid":false,"given":"Iden","family":"Amiri","sequence":"first","affiliation":[{"name":"Ophthalmology, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johnson","family":"Hoang","sequence":"additional","affiliation":[{"name":"Ophthalmology, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Khalid","family":"Aldaas","sequence":"additional","affiliation":[{"name":"Ophthalmology, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Fleischman","sequence":"additional","affiliation":[{"name":"Ophthalmology, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,8,27]]},"reference":[{"key":"2025082710152017000_18.8.e267523.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2014.01.002"},{"key":"2025082710152017000_18.8.e267523.2","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-6196(12)61951-X"},{"key":"2025082710152017000_18.8.e267523.3","doi-asserted-by":"crossref","DOI":"10.1007\/s11882-020-00945-1","article-title":"Cogan\u2019s Syndrome: Clinical Presentations and Update on Treatment","volume":"20","author":"Espinoza","year":"2020","journal-title":"Curr Allergy Asthma Rep"},{"key":"2025082710152017000_18.8.e267523.4","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1016\/S0889-857X(21)01068-1","article-title":"Vasculitis and Cogan\u2019s syndrome","volume":"16","author":"Vollertsen","year":"1990","journal-title":"Rheum Dis Clin North Am"},{"key":"2025082710152017000_18.8.e267523.5","doi-asserted-by":"publisher","DOI":"10.4065\/81.4.483"},{"key":"2025082710152017000_18.8.e267523.6","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-198011000-00003"},{"key":"2025082710152017000_18.8.e267523.7","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keh228"},{"key":"2025082710152017000_18.8.e267523.8","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(90)90157-9"},{"key":"2025082710152017000_18.8.e267523.9","doi-asserted-by":"crossref","DOI":"10.1155\/2018\/1498640","article-title":"Autoimmune Inner Ear Disease: Immune Biomarkers, Audiovestibular Aspects, and Therapeutic Modalities of Cogan\u2019s Syndrome","volume":"2018","author":"Shamriz","year":"2018","journal-title":"J Immunol Res"},{"key":"2025082710152017000_18.8.e267523.10","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1177\/000348940010900802","article-title":"Use of methotrexate for autoimmune hearing loss","volume":"109","author":"Matteson","year":"2000","journal-title":"Ann Otol Rhinol Laryngol"},{"key":"2025082710152017000_18.8.e267523.11","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfh380"},{"key":"2025082710152017000_18.8.e267523.12","unstructured":"Salman EJ , Tripathy K . 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