Cogan Syndrome Vasculitis - Cogan Syndrome


Indications for Testing

  • Rapid onset of sensorineural hearing loss, eye inflammation, symptoms of vasculitis

Laboratory Testing

  • No formal criteria or confirmatory test for the diagnosis of Cogan syndrome (CS)
  • Nonspecific testing – helpful in excluding other diagnoses
    • Initial assessment
    • Rule out other disease processes
      • Renal disorders – urinalysis, urea nitrogen, creatinine
      • Other vasculitis – anti-neutrophil cytoplasmic antibody (ANCA)
      • Connective tissue disease – anti-nuclear antibody (ANA), CBC
    • Rule out infections associated with hearing loss
      • Treponema pallidum
        • Presence of interstitial keratitis and/or acute sensorineural hearing loss combined with negative testing for syphilis is highly suggestive of CS
      • HIV

Imaging Studies

  • Angiography – if symptoms present, use to identify aortitis 

Differential Diagnosis

Clinical Background

Cogan syndrome (CS) is a rare vasculitis that typically manifests as an ophthalmic disorder (interstitial keratitis) and/or an audiovestibular disorder. It is categorized as a variable vessel vasculitis (Chapel Hill 2012).


  • Incidence – rare; ~250 cases reported
  • Age – children (rare) and young adults in 20s-30s
  • Sex – M:F, equal
  • Ethnicity – more common in Caucasians


  • Inflammation in all vessel sizes (small, medium, large)
  • Primary ocular target – small vessels in the vascularized layers of the anterior globe, episclera, sclera and uveitis

Clinical Presentation

  • Constitutional – headache, fever, arthralgia, arthritis
  • Ophthalmologic – interstitial keratitis, iritis, uveitis, episcleritis (generally reversible)
  • Audiovestibular – Ménière-like syndrome (vertigo, tinnitus, ataxia) with or without sudden sensorineural hearing loss; frequently results in deafness
  • Vasculitis – occurs in 10-20% of patients
  • Cardiovascular – aortic disease; life threatening in small percent  of cases

Indications for Laboratory Testing

  • Tests generally appear in the order most useful for common clinical situations
  • Click on number for test-specific information in the ARUP Laboratory Test Directory
Test Name and Number Recommended Use Limitations Follow Up
C-Reactive Protein 0050180
Method: Quantitative Immunoturbidimetry

Preferred test for evaluation of inflammation in vasculitis

Anti-Neutrophil Cytoplasmic Antibody with Reflex to Titer and MPO/PR-3 Antibodies 2002068
Method: Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multiplex Bead Assay

Confirm presence of ANCA-associated vasculitis

Reflex pattern  – if screen is positive, then titer and MPO/PR-3 antibodies testing will be added to aid in antibody determination

Anti-Nuclear Antibodies (ANA), IgG by ELISA with Reflex to ANA, IgG by IFA 0050080
Method: Qualitative Enzyme-Linked Immunosorbent Assay/Semi-Quantitative Indirect Fluorescent Antibody

Evaluate for connective tissue disease

All ELISA results reported as "Detected" are further tested by IFA

Detects antibodies against dsDNA, histone, SS-A (Ro), SS-B (La), Smith, snRNP/Sm, Scl-70, Jo-1, centromere, and an extract of lysed HEp-2 cells

Reflex pattern – if ANA are detected by ELISA, then ANA by IFA titer will be added

ANA ELISA assays have lower sensitivities for antibodies associated with nucleolar and specked ANA-IFA patterns

Rapid Plasma Reagin (RPR) with Reflex to Titer and TP-PA Confirmation 0050478
Method: Semi-Quantitative Charcoal Agglutination/Semi-Quantitative Particle Agglutination

Evaluate for syphilis

Reflex pattern – if RPR is reactive, then a titer to endpoint and TP-PA confirmation will be added

Human Immunodeficiency Virus Types 1 and 2 (HIV-1, HIV-2) Antibodies by CIA with Reflex to HIV-1 Antibody Confirmation by Western Blot 2005377
Method: Qualitative Chemiluminescent Immunoassay/Qualitative Western Blot

Evaluate for HIV

Reflex pattern – if HIV-1 and HIV-2 screen is repeatedly reactive, then Human Immunodeficiency Virus type 1 (HIV-1) antibody confirmation by Western Blot will be added

Additional Tests Available
Click the plus sign to expand the table of additional tests.
Test Name and NumberComments
CBC with Platelet Count and Automated Differential 0040003
Method: Automated Cell Count/Differential

May help in ruling out infectious process

Urinalysis, Complete 0020350
Method: Reflectance Spectrophotometry/Microscopy

Screen for hematuria, proteinuria, and RBC casts

Sedimentation Rate, Westergren (ESR) 0040325
Method: Visual Identification

Evaluate for suspected vasculitis

Urea Nitrogen, Serum or Plasma 0020023
Method: Quantitative Spectrophotometry

Assess for renal disease

Creatinine, Serum or Plasma 0020025
Method: Quantitative Enzymatic

Assess for renal disease