Cogan's syndrome (CS)

Overview

CS occurs when the immune system attacks the tissues and blood vessels of the eyes and ears. The disease can present either in a typical or atypical form.

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.

Sources

  1. Article Sources and Footnotes

    1. 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.

    2. Cogan’s Syndrome. (n.d.). Vasculitis Foundation. Retrieved July 15, 2021, from https://www.vasculitisfoundation.org/education/forms/cogans-syndrome/.

    3. 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.

    4. 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. 

    5. 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.

    6. 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.

    7. 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.