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Cogan’s Syndrome (CS)

  • What is it?
    • Cogan’s Syndrome (CS) is a rare inflammatory disorder that affects primarily the eyes and ears. Inflammation of the eyes and ears over extended periods of time can cause vision blurriness, hearing loss and dizziness.
    • Individuals with CS, may also have inflammation in other organs such as the heart and other large blood vessels throughout the body.
    • Approximately 70% of diagnosed CS patients have some type of inflammation in other blood vessels besides the eyes and ears. While the inflammation tends to be found in large and/or medium vessels, any sized vessels can be affected. This can cause damage to those organs where the blood vessels are found and are in extreme cases, death1
    • Other symptoms as a result of CS include: hemiparesis (weakness on one side of the body) or hemiplegia (paralysis on one side of the body) as a result of ischemia (reduce blood flow via the blood vessels) caused by either inflammation or injury 2. In addition, diarrhea, abdominal pain and melena (black feces as a result of upper intestinal bleeding) are seen in patients with CS.3
    • While CS is typically found in young adults of both sex between the age of 20-30s, there have been a few cases where children have been diagnosed.
  • Currently, it is unknown what the cause of CS is. However, there are evidence that suggests that it may be an autoimmune disease (a disease where the immune system, which normally defends against foreign cells and diseases, begin to produce antibodies that attacks the body’s own self cells). As a result, the immune system begins to damage tissues and cells found in certain areas of the body, in this case specifically the inner ear and the eyes.
  • Some studies have shown that some patientshave specific antigens (foreign substances or toxins that causes our bodies’ immune system to react to) expressed in their ear4that are similar to the toxins that are seen in viruses and other bacteria.
  • Some researchers have suggested that the bacteria Chlamydia pneumoniae may have caused the autoimmune disease since some patients with CS, was infected with Chlamydia pneumoniae. However, this is only an association and most patient who have been infected with pneumoniae never developed CS.
  • The median age for CS onset is 22 years old where the majority of patients were between the age of 20s-30s. However, there are cases where children and older adults were diagnosed; from as young as 5 to 63 years of age.
  • While all races can develop this disease, it is more commonly found among Caucasians than other races
  • Click here for more detailed information on classifying and diagnosing Cogan’s Syndrome.
  • Click here for more detailed information on suggested treatment protocols
  1. Cheson, B.D., A.Z. Bluming, and J. Alroy, Cogan’s syndrome: a systemic vasculitis. Am J Med, 1976. 60(4): p. 549-55.
  2. Vollertsen, R.S., et al., Cogan’s syndrome: 18 cases and a review of the literature. Mayo Clin Proc, 1986. 61(5): p. 344-61.
  3. Thomas, H.G., Case report: clinical and radiological features of Cogan’s syndrome–non-syphilitic interstitial keratitis, audiovestibular symptoms and systemic manifestations. Clin Radiol, 1992. 45(6): p. 418-21.
  4. Lunardi, C., et al., Autoantibodies to inner ear and endothelial antigens in Cogan’s syndrome. Lancet, 2002. 360(9337): p. 915-21.
  5. Greco, A., et al., Cogan’s syndrome: an autoimmune inner ear disease. Autoimmun Rev, 2013. 12(3): p. 396-400.
  6. Grasland, A., et al., Typical and atypical Cogan’s syndrome: 32 cases and review of the literature. Rheumatology (Oxford), 2004. 43(8): p. 1007-15.
  7. Sugimoto, K., et al., Childhood Cogan syndrome with aortitis and anti-neutrophil cytoplasmic antibody-associated glomerulonephritis. Pediatr Rheumatol Online J, 2014. 12: p. 15.
  8. Ndiaye, I.C., S.J. Rassi, and S.R. Wiener-Vacher, Cochleovestibular impairment in pediatric Cogan’s syndrome. Pediatrics, 2002. 109(2): p. E38.
  9. Orsoni, J.G., et al., Cogan syndrome in children: early diagnosis and treatment is critical to prognosis. Am J Ophthalmol, 2004. 137(4): p. 757-8.
  • CS is commonly diagnosed by examining 3 conditions:
    • (1) scarring of the cornea (the transparent outer layer of the eye) due to chronic inflammation
    • (2) ear problems such as sudden onset of ringing/buzzing of the ear, loss of balance and gradual hearing loss
    • (3) a time delay of less than 2 years between when the eye and ear symptoms were first revealed.5
  • To provide a definitive diagnosis of CS, usually details regarding the characteristics of the eye and inner ears is needed. Since a very few percentage of patients (<5%) have clear indications of inflammation in major blood vessels, a definitive diagnosis is usually not made until both the eyes and inner ear are affected.
  • The main symptoms of CS include:
    • Red, painful, blurred vision or light-sensitive eyes
    • Gradual Hearing loss
    • Vertigo (Dizziness) & poor balance
    • Nausea, Vomiting, Fatigue & Weight Loss
    • Fever, Headache
  • In some rare cases, CS can cause:
    • Enlarged lymph nodes
    • Skin Rashes
    • Arm and Chest pain
    • Shortness of breaths
  • Occular (Eye) Disease
    • Main feature seen for individuals with CS is interstitial keratitis (an inflammation of the cornea -the transparent layer covering the eyes) which results in photophobia, blurry vision, pain and redness.
  • Inner Ear Disease
    • Sudden onset of dizziness, nausea and vomiting (Usually resolved a few days)
    • Variability in hearing loss
    • Audiologic Test
    • Vesitvular Testing
    • Earth Vertical Axis rotation

While there are no laboratory studies included in the criteria for CS, and most laboratory tests do not provide a definitive diagnosis, certain laboratory tests can help in the diagnosis of CS.

  • Erythrocyte Sedimentation Rate (ESR)
    • CS patients typically have elevated ESR
    • ESR is used to detect and monitor inflammation in the body
  • Leukocytosis (also known as White-Blood Cells)
    • CS patients have typically elevated Leukocytes
    • Leukocytes are elevated due to increased inflammation or infection
  • C-Reactive proteins (CRP)
    • CS patient have elevated CRP
    • CRP is produced in the liver and its level rises when an inflammation occurs in the body
  • Complete Blood Count (CBC)
    • Some CS patients (~30%) have decrease CBC
    • Decreased CBC may be an indication of anemia (deficiency in red blood cells, resulting in weariness
  • Urea/Creatinine/Glomerular Filtration rate7
    • These tests are used to evaluate the kidney function and urine excretion
    • There has been a reported case of children with complication in renal function
  • Slit-Lamp Examination
    • One of the most crucial examinations needed to help make a more definitive diagnosis of CS
    • Performed by optometrists/ophthalmologists to provide a detailed and magnified view on the eye. Slit-examination allows for anatomic diagnosis of CS.
  • High resolution Magnetic Resonance Imaging(MRI)& antibodies to inner ear antigens
    • MRI scans provide a high quality image of the anatomy and physiological process of the ear and eyes. Studies have shown that high resolution MRI can help differentiate between active and inactive stages of CS.
    • Antibodies can be used to determine if the cornea and inner ear tissues have certain markers that the immune system attacks as ‘foreign’.
    • Magnetic resonance angiogram (MRA); a type of MRI has been used to confirm aortitis (inflammation of the aorta artery, the main artery that leaves the heart supplying the rest of the body with oxygenated blood) in children

 

  1. Cheson, B.D., A.Z. Bluming, and J. Alroy, Cogan’s syndrome: a systemic vasculitis. Am J Med, 1976. 60(4): p. 549-55.
  2. Vollertsen, R.S., et al., Cogan’s syndrome: 18 cases and a review of the literature. Mayo Clin Proc, 1986. 61(5): p. 344-61.
  3. Thomas, H.G., Case report: clinical and radiological features of Cogan’s syndrome–non-syphilitic interstitial keratitis, audiovestibular symptoms and systemic manifestations. Clin Radiol, 1992. 45(6): p. 418-21.
  4. Lunardi, C., et al., Autoantibodies to inner ear and endothelial antigens in Cogan’s syndrome. Lancet, 2002. 360(9337): p. 915-21.
  5. Greco, A., et al., Cogan’s syndrome: an autoimmune inner ear disease. Autoimmun Rev, 2013. 12(3): p. 396-400.
  6. Grasland, A., et al., Typical and atypical Cogan’s syndrome: 32 cases and review of the literature. Rheumatology (Oxford), 2004. 43(8): p. 1007-15.
  7. Sugimoto, K., et al., Childhood Cogan syndrome with aortitis and anti-neutrophil cytoplasmic antibody-associated glomerulonephritis. Pediatr Rheumatol Online J, 2014. 12: p. 15.
  8. Ndiaye, I.C., S.J. Rassi, and S.R. Wiener-Vacher, Cochleovestibular impairment in pediatric Cogan’s syndrome. Pediatrics, 2002. 109(2): p. E38.
  9. Orsoni, J.G., et al., Cogan syndrome in children: early diagnosis and treatment is critical to prognosis. Am J Ophthalmol, 2004. 137(4): p. 757-8.
  • Glucocorticoid Therapy
    • Glucocorticoid therapy (drugs that reduces inflammation) are the first line of treatment
    • Prednisoloneor other types of steroids are commonly used
    • They are applied topically for the eyes & ingested orally for decreased hearing
  • Aspirin
    • Aspirin with known anti-inflammatory properties (to reduce fever and swelling) and anti-platelet effects (prevent blood clots)
    • Aspirin is used commonly in conjunction with glucocorticoid therapy (eg: Prednisolone)
  • Steroids & nonsteroidal anti-inflammatory drugs (NSAIDs)
    • Those with mild eye-inflammation can be treated with NSAIDs
    • Celecoxib, diclofenac, aspirin, ibuprofen, etodolac
  • Immunosuppressive Drugs
    • In cases where large amount of steroid medication is needed and/or the disease is very severe where the steroid therapy is not effective, immunosuppressive drugs are recommended in addition to the steroid therapy
    • In children, it has been reported that early immunosuppressive treatment has help prevent progression of the disease and prevention of vision impairment and deafness9
    • Methotrexate, Ciclosporin, Azathioprine, Tacrolimus, Cyclophosphamide
  • Eye-Dilating Drops
    • Used in conjunction with anti-inflammatory drugs to prevent Iris (colored part of the eye) from getting fixed onto the cornea
  • Anti-Tumor Necrosis Factor (TNF) Therapy
    • Class of drugs used to treat inflammatory conditions and reduce inflammation or stop disease progression
    • TNF is chemical compound produced by immune system to cause inflammation and better help clear infection
    • Excess TNF as the case with autoimmune diseases and CS, greater inflammation and long-lasting symptoms occurs
  • Surgery
    • Occasionally, inflammation may damage blood vessels and surgery is usually done to help resolve it.
    • In some cases,the cornea (transparent part of the eye) may be damaged and corneal transplant surgery is necessary to replace the damage cornea with a health donor.
  • Cochlear Implants
    • If hearing is impaired after treatment, cochlear implants may also be necessary
      • Cochlear Implants is a device that helps convert sound waves to electrical signals so the brain can understand and process it

Below you can find downloadable PDF files of suggested treatment protocols for patients with Cogan’s Syndrome.

Suggested Treatment Protocols:

  1. (These numbers depend on the types of treatment protocols provided for Cogan’s Syndrome)
  2. (These numbers depend on the types of treatment protocols provided for Cogan’s Syndrome)
  3. (These numbers depend on the types of treatment protocols provided for Cogan’s Syndrome)
  1. Cheson, B.D., A.Z. Bluming, and J. Alroy, Cogan’s syndrome: a systemic vasculitis. Am J Med, 1976. 60(4): p. 549-55.
  2. Vollertsen, R.S., et al., Cogan’s syndrome: 18 cases and a review of the literature. Mayo Clin Proc, 1986. 61(5): p. 344-61.
  3. Thomas, H.G., Case report: clinical and radiological features of Cogan’s syndrome–non-syphilitic interstitial keratitis, audiovestibular symptoms and systemic manifestations. Clin Radiol, 1992. 45(6): p. 418-21.
  4. Lunardi, C., et al., Autoantibodies to inner ear and endothelial antigens in Cogan’s syndrome. Lancet, 2002. 360(9337): p. 915-21.
  5. Greco, A., et al., Cogan’s syndrome: an autoimmune inner ear disease. Autoimmun Rev, 2013. 12(3): p. 396-400.
  6. Grasland, A., et al., Typical and atypical Cogan’s syndrome: 32 cases and review of the literature. Rheumatology (Oxford), 2004. 43(8): p. 1007-15.
  7. Sugimoto, K., et al., Childhood Cogan syndrome with aortitis and anti-neutrophil cytoplasmic antibody-associated glomerulonephritis. Pediatr Rheumatol Online J, 2014. 12: p. 15.
  8. Ndiaye, I.C., S.J. Rassi, and S.R. Wiener-Vacher, Cochleovestibular impairment in pediatric Cogan’s syndrome. Pediatrics, 2002. 109(2): p. E38.
  9. Orsoni, J.G., et al., Cogan syndrome in children: early diagnosis and treatment is critical to prognosis. Am J Ophthalmol, 2004. 137(4): p. 757-8.