Dr. David Cogan.

Cogan's syndrome (CS) is defined as nonsyphilitic interstitial keratitis (an inflammation of the
eye) and bilateral audiovestibular deficits (hearing problems and dizziness). It was first
described in Morgan and Baumgartner in 1934, and Cogan reported 5 more cases in 1945. Dr.
David Cogan was a prolific writer and his name is also on an ocular sign involving ocular
deviation under closed eyelids. Only about 100 cases of the Cogans syndrome discussed here
have been described in the world literature. This disease primarily affects caucasians and is
believed not to be hereditary.
Cause:
Cogan's syndrome is generally assumed to be an immune mediated disorder with vasculitis.
The pathology has been described in a few case reports. Recently, Jung et al (2015) reported
vasculitis.
Signs and Symptoms:
Typical CS is characterized by interstitial keratitis and vestibuloauditory dysfunction.There is
generally a brief episode of inflammatory eye disease (interstitial keratitis) followed by
bilateral audiovestibular symptoms. The interstitial keratitis usually occurs with sudden onset
and is characterized by photophobia, lacrimation, and eye pain. The vestibuloauditory
dysfunction is usually bilateral, presenting with tinnitus, sensorineural hearing loss, and acute
episodes of vertigo.
The symptoms typically progressively deteriorate within days. While the ESR, WBC, C-reactive
protein test may be abnormal and there may be thrombocytosis or anemia, none of these
findings are reliable indicators of the disease. A slit-lamp examination is essential.
Recent work has suggested that high-resolution MRI and antibodies to inner ear antigens may
be helpful. We are dubious about the antibodies, but a high-field MRI with a local ear coil
seems worth considering, as fibrous material appears to close off the labyrinth in this disease
and the cochlea may enhance in Cogans (Fugate et al 2009). Cogans children can occur in
children, and is particularly difficult to recognize in that situation (Massinger and Keilmann,
2003).
"Atypical" Cogans syndrome is the association of the typical hearing symptoms with other
ocular symptoms including scleritis or episcleritis, retinitis, optic neuritis, glaucoma, dry eye,
ptosis, etc. (Grasland et al, 2004; Cundiff et al, 2006). The diagnosis of "atypical Cogans",
should be considered with much greater skepticism than Cogans with interstitial keratitis, as
combining (for example), Meniere's with glaucoma could potentially generate a large number
of falsely diagnosed patients. The author's position is that the diagnosis of Cogan's should be
referred for patients with the so-called "classic" syndrome.
Treatment
Treatment is with vigorous immune suppression to avoid deafness and visual deterioration.
Steroid treatment is usually first attempted (1 mg/kg/day equivalent of prednisone), over 2-4
weeks. If this is successful, then additional immunosuppression is often used. As high dose
steroids are associated with the possibility of severe complications, other drugs that may be
substituted or added on are methotrexate, cyclophosphamide, azathiprine, mycophenolate
moefetil, and cyclosporin. Anti-TNF drugs such as entanercept are also thought to be
sometimes helpful (Murphy et al, 2009). The deafness in Cogan's syndrome can be reasonably
well treated with the Cochlear Implant (Bacciu et al, 2014).

Cogan's Syndrome Neurology ,Otolaryngology

DISEASE OVERVIEW
combination of inflammatory eye disease and vestibuloauditory dysfunction
rare autoimmune disorder affecting the inner ear
described as Meniere's-like fluctuating hearing loss combined with impaired caloric response
young adults commonly affected
As many as 15% of CS patients have a large- to medium-vessel vasculitis
As a helpful mnemonic device think of a man shooting a gun (rhymes with 'gan' in Cogans; see the side effects the shooter
experiences in the figures below)

SIGNS / SYMPTOMS
acute nonsyphilitic interstitial keratitis
sensorineural hearing loss (see figure 3 below)
Medium- and large-vessel arteritis (aortitis, aortic regurgitation, coronary artery inflammation, mesenteric vasculitis, and limb
claudication)
severe vertigo (see figure 3 below)
nausea/ vomiting
tinnitus
diarrhea and/or bloody stools (10% patients with CS)

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DIAGNOSIS
clinical suspicion (make sure to consider more common diseases before committing to a diagnosis of CS)
TREATMENT
systemic corticosteroids for inner ear disease (high doses)
topical steroids for interstitial keratitis
cytotoxic agents for cases refractory to corticosteroids
September 13, 2006