Cogan Syndrome

Diagnosis

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
• Tests are used to rule out specific diseases and/or demonstrate inflammation
  • Initial assessment
    • Erythrocyte sedimentation rate and C-reactive protein – elevated in ~50% of patients with active disease
  • Rule out other disease processes
    • Renal disorders – urinalysis, urea nitrogen, creatinine
    • Vasculitis – anti-neutrophil cytoplasmic antibody (ANCA)
    • Connective tissue disease – anti-nuclear antibody (ANA), CBC
  • Rule out specific infections
    • Treponema pallidum testing
      • Presence of interstitial keratitis and/or acute sensorineural hearing loss combined with negative testing for syphilis is highly suggestive of Cogan syndrome
    • HIV testing

Imaging Studies

• Angiogram – identify aortitis if symptoms present 

Differential Diagnosis

• Autoimmune sensorineural hearing loss
• Brain tumor – meningioma
• Ménière disease
• Treponema pallidum
• Other vasculitic disease
  • Microscopic polyangiitis
  • Polyarteritis nodosa

Clinical Background

Cogan syndrome is a rare vasculitis that typically manifests as an ophthalmic disorder (interstitial keratitis) and/or an audiovestibular disorder. Although no definitive trigger has been identified, a history of Chlamydia or influenza-like infection has been associated with the onset of Cogan syndrome.

Epidemiology

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

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 10% of cases)

Treatment

• Corticosteroids
  • Administration early in the course of disease may prevent deafness
  • Topical steroids for keratitis
• Immunosuppressives
  • Methotrexate
  • Cyclophosphamide
• Cochlear implant
  • 60% of patients experience significant hearing loss
  • Implant therapy may be effective for severe loss

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

Diagnostic Algorithm

  Vasculitis in Adults Testing Algorithm