Background: Patients with Cogan syndrome typically present with nonsyphilitic interstitial keratitis and acute onset of sensorineural hearing loss. Neurological manifestations have been reported, but various frequencies and mechanisms have been proposed.
Review summary: We critically reviewed the English literature of Cogan syndrome to determine the nature, frequency, and most likely mechanisms of its neurological manifestations.
Conclusions: On the basis of our review, we believe that Cogan syndrome can be associated with neurological manifestations. Our conclusion is based on reported tissue evidence of vasculitis involving the dura, brain, optic nerve, cochleovestibular nerve, and muscle, in patients with referable symptoms. However, we believe that the frequency of neurological manifestations may have been over reported due to lack of confirmatory testing in many of these cases.