Overview
CS occurs when the immune system attacks the tissues and blood vessels of the eyes and ears.
Cogan's syndrome (CS)
The disease can present in two forms:
Typical form
Characterized by Ménière-like attacks (nausea, vomiting, tinnitus, vertigo, and hearing loss) along with interstitial keratitis (inflammation of the cornea tissue).
Atypical form
Consists of audiovestibular dysfunction along with other forms of eye disease distinct from interstitial keratitis such as uveitis, scleritis, conjunctivitis, or retinal vasculitis.
Common Symptoms
Eye pain or irritation, loss of vision or hearing, excessive tear production, light sensitivity, vertigo, joint or muscle pain, tinnitus, blood vessel inflammation, fevers, muscle cramping, headaches, diarrhea, heart murmur, and stomach pain. Typically the disease progresses from just affecting the eyes or the inner ears to affecting both.
Coexisting Diseases and Conditions
Vertigo, tinnitus, myalgia, non-specific skin rashes, urticarial vasculitis, palpable purpura, pyoderma gangrenosum, autoimmune inner ear disease, scleritis, inflammatory bowel disease, thrombocytopenic purpura, HLA-B27-positive spondyloarthritis, and sarcoidosis.
Risk Factors and Prevalence
Studies have found that Cogan’s syndrome typically affects younger, white adults. In addition, research indicates that around 50% of cases are preceded by infections of the upper respiratory tract.
Recent Research
- Acute Limb Ischemia in Cogan Syndrome (2022)
- Autoimmune Vestibulopathy-A Case Series (2022)
- Speech reception after cochlear implantation for Cogan’s syndrome: Case series following CARE guidelines (2023)
- Paediatric Cogan´s syndrome – review of literature, case report and practical approach to diagnosis and management (2023)
Sources
- Article Sources
Cogan’s syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. (n.d.). Https://Rarediseases.Info.Nih.Gov/Diseases/1421/Cogans-Syndrome. Retrieved July 15, 2021, from https://rarediseases.info.nih.gov/diseases/1421/cogans-syndrome.
Cogan’s Syndrome. (n.d.). Vasculitis Foundation. Retrieved July 15, 2021, from https://www.vasculitisfoundation.org/education/forms/cogans-syndrome/.
Grasland, A., Pouchot, J., Hachulla, E., Blétry, O., Papo, T., Vinceneux, P., & for the Study Group for Cogan’s syndrome. (2004). Typical and atypical Cogan’s syndrome: 32 cases and review of the literature. Rheumatology, 43(8), 1007–1015. https://doi.org/10.1093/rheumatology/keh228.
Migliori, G., Battisti, E., Pari, M., Vitelli, N., & Cingolani, C. (2009). A shifty diagnosis: Cogan’s syndrome. A case report and review of the literature. Acta otorhinolaryngologica Italica : organo ufficiale della Societa italiana di otorinolaringologia e chirurgia cervico-facciale, 29(2), 108–113.
Murphy, G., Sullivan, M. O., Shanahan, F., Harney, S., & Molloy, M. (2009). Cogan’s syndrome: Present and future directions. Rheumatology International, 29(10), 1117–1121. https://doi.org/10.1007/s00296-009-0945-0.
Touloei, K., Tongdee, E., Smirnov, B., Favreau, T., & Porges, L. (n.d.). Cogan’s Syndrome with Cutaneous Findings: A Case Report and Review of Dermatologic Manifestations. https://cdn.ymaws.com/www.aocd.org/resource/resmgr/jaocd/Contents/Volume34/34-04.pdf.
U.S. Department of Health and Human Services. (2017, December 5). Cogan’s syndrome. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/1421/cogans-syndrome.