What is Cogan Syndrome?

Cogan Syndrome is a rare autoimmune disorder characterized by inflammation of the eyes (interstitial keratitis) and inner ears (audiovestibular symptoms), often leading to vision and hearing loss. It can also involve other organs, such as the cardiovascular system and the aorta.

What are the symptoms of Cogan Syndrome?

The primary symptoms include interstitial keratitis, hearing loss, tinnitus, and vertigo

Can Cogan Syndrome be cured?

There is no cure for Cogan Syndrome, but with proper treatment, many patients can manage their symptoms and prevent severe complications. Early diagnosis and treatment are crucial for preserving vision and hearing.

ISCHEMIC STROKE / ETIOLOGY

Cogan syndrome

David Goldemund M.D.
Updated on 27/06/2024, published on 27/06/2024

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

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:
- vasculitis (Albayram, 2001) (Rolon, 2022)
- large-artery infarcts from cardiac emboli secondary to valvular disease
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, supported by 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)
Ménière’s Disease
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 agents – methotrexate, 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

→ Recanalization therapy in acute stroke

→ Ischemic stroke prevention

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