Timothy C. Hain, MD Page last modified: July 26, 2015 Click here to go to the main autoimmune inner ear disease page
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.
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.
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 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).
A patient with Cogan's syndrome volunteered to put her story on line, and it can be found by clicking here.
See also: Autoimmune Inner Ear Disease