Interdisciplinary collaboration in a case of bilateral vestibulocochlear damage as the primary manifestation of Cogan I syndrome – a case report

 

Introduction With less than 300 cases described in the literature, Cogan I syndrome is a rare autoimmune disease with vestibulocochlear and ocular manifestations, showing heterogeneous clinical presentation and systemic involvement.

Material and methods The 55-year-old patient presented with a 3-month history of unilateral and then bilateral hearing loss with tinnitus, vertigo on head movement, and conjunctivitis on both sides. The pure-tone audiometry showed a symmetrical basocochlear sensorineural hearing loss of up to 60 dB on both sides. There was a spontaneous nystagmus I° to the left with vestibular thermal hypoexcitability on the right.

Results Interdisciplinary diagnostics were carried out for suspected Cogan I syndrome. The serologic, ophthalmologic, neurologic and rheumatologic findings were inconclusive. A PET-CT was performed to rule out vasculitis and was unremarkable. 6 months later, the hearing loss worsened with bilateral hearing loss bordering on deafness and arthritic complaints. Prednisolone shock therapy did not improve the hearing loss. A new PET-CT was performed. This time revealing large vessel vasculitis, most likely in the context of Cogan I syndrome, so that immunosuppressive therapy with methotrexate and prednisolone was initiated. In the absence of hearing improvement, CI fitting is planned.

Discussion Bilateral vestibulocochlear lesions even with unspecific ocular symptoms should be investigated for Cogan I syndrome if the surrounding diagnostic findings are unremarkable. Organ manifestations can also occur during the course of the disease and should be treated in an interdisciplinary manner.

Publication History

Article published online:
19 April 2024

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