Abstract
Importance. Rituximab was not effective in ameliorating the hearing loss in a patient with atypical Cogan's syndrome. Observations. We report the case of a patient who developed acute bilateral uveitis and sensorineural hearing loss. A diagnosis of atypical Cogan's syndrome was made. The patient's hearing loss did not improve despite high dose steroids and azathioprine. Rituximab was administered given a recent report of its efficacy in a patient with refractory disease; however, our patient's hearing loss did not improve. Conclusion. Hearing loss in Cogan's syndrome is difficult to treat. Though rituximab was ineffective in our case, earlier administration in the disease course could be effective for future patients.
Highlights
Cogan’s syndrome is a rare disease characterized by inflammatory eye disease and vestibuloauditory dysfunction
Rituximab has been proposed as a treatment option for persistent hearing loss associated with Cogan’s syndrome; we report a case in which a patient’s sensorineural hearing loss was not responsive to this agent
A 43-year-old female was transferred to our institution because of bilateral hearing loss associated with bilateral eye pain and blurry vision
Summary
Cogan’s syndrome is a rare disease characterized by inflammatory eye disease and vestibuloauditory dysfunction. The ocular symptoms are usually amenable to treatment but the vestibuloauditory symptoms can persist despite aggressive therapy. Rituximab has been proposed as a treatment option for persistent hearing loss associated with Cogan’s syndrome; we report a case in which a patient’s sensorineural hearing loss was not responsive to this agent
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