Introduction

  • Cogan syndrome (CS) is a rare, chronic, inflammatory disorder characterized by ocular and audiovestibular manifestations, often associated with systemic vasculitis
  • first described by David Cogan in 1945, the syndrome primarily affects young adults with no gender predilection
  • the etiology remains unknown, but an autoimmune mechanism is suspected due to the presence of autoantibodies and their association with other autoimmune conditions
  • systemic involvement can predispose patients to a variety of complications, including rare cerebrovascular events such as stroke

Clinical presentation

  • CS is clinically classified into two forms:
    • typical form – includes interstitial keratitis and audiovestibular symptoms resembling Ménière’s disease
    • atypical form – involves other ocular inflammatory diseases and systemic symptoms

Ocular symptoms

  • interstitial keratitis – the hallmark of typical CS, presenting as photophobia, pain, and decreased vision. Slit-lamp examination reveals stromal inflammation and corneal opacity.
  • other ocular involvements
    • scleritis and episcleritis
    • uveitis
    • conjunctivitis
Keratitis due to Cogane syndrome

Audiovestibular symptoms

  • sensorineural hearing loss – sudden onset, fluctuating, and progressive; bilateral involvement is typical
  • vestibular dysfunction –  vertigo, tinnitus, and balance disturbances, often mimicking Ménière’s disease

Neurologic manifestations

  • cerebrovascular involvement is very rare and involves:
  • other neurological symptoms
    • headaches
    • cognitive dysfunction (encephalopathy)
    • myelopathy

Systemic manifestations

  • fever, weight loss, fatigue
  • medium and large vessel vasculitis may lead to complications such as aortitis, aortic aneurysms, and aortic insufficiency
  • musculoskeletal symptoms – arthralgia and myalgia

Diagnostic evaluation

  • no test is diagnostic of Cogan’s syndrome; the diagnosis is primarily clinical, based on the characteristic combination of ocular and audiovestibular symptoms
  • laboratory tests are non-specific:
    • elevated inflammatory markers (ESR, CRP), leukocytosis, anemia, and thrombocytosis
    • autoantibodies such as rheumatoid factor and nuclear antibodies are usually negative
  • audiometry confirms sensorineural hearing loss
  • imaging studies
    • CT – possible intralabyrinthine calcifications, CTA may demonstratě vasculitic changes
    • MRI – soft tissue obliteration of the membranous labyrinth,  rarely multiple white matter lesions (WML) consistent with cerebral vasculitis
  • a biopsy of affected tissues can confirm vasculitis

Differential diagnosis

  • autoimmune Inner Ear Disease (AIED)
    • rapidly progressive, bilateral sensorineural hearing loss, often fluctuating with tinnitus
    • episodic or continuous dizziness and balance disturbances
    • response to corticosteroid therapy can be diagnostic
  • Meniere’s disease – results from abnormal endolymphatic fluid dynamics, which leads to hydrops
    • spontaneous, recurrent episodes lasting 20 minutes to several hours
    • sensorineural, often fluctuating, usually unilateral initially
    • tinnitus
    • aural fullness (feeling of pressure or fullness in the ear)
  • syphilis
  • sarcoidosis
  • granulomatosis with Polyangiitis (GPA)

Management

Immunosuppressive therapy

  • the treatment is empiric and not based on any formal therapeutic trial because of the rarity of the disease
  • corticosteroids – high-dose prednisone is the first-line treatment
    • typically starting at 1 mg/kg/day
    • gradually tapered over 2–6 months
  • immunosuppressive agentsmethotrexate, azathioprine, and cyclophosphamide are commonly used for steroid-sparing and refractory cases
  • biologic agents  – anti-TNF agents (infliximab, etanercept) and rituximab have shown efficacy in severe and refractory cases
  • ocular and audiovestibular management
    • topical corticosteroids for keratitis
    • hearing aids or cochlear implants for hearing loss
  • patients with severe aortic or cardiac disease may benefit from aortic valve replacement or vascular bypass surgery

Acute stroke therapy and stroke prevention

  • the standard protocols should be followed

Prognosis

  • early diagnosis and aggressive treatment can prevent complications and preserve organ function
  • untreated or refractory cases may lead to permanent vision and hearing loss, systemic complications, and reduced quality of life
  • the prognosis of stroke in patients with Cogan syndrome depends on the extent of cerebral involvement and the promptness of treatment
    • recurrent strokes and progressive vasculitis can lead to significant morbidity and impact the quality of life

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Cogan syndrome
link: https://www.stroke-manual.com/cogan-syndrome/