A case of Ménière's disease – or more?

Introduction:

In cases of sudden hearing loss, tinnitus und vertigo, the otorhinolaryngologist should not only consider Menière's disease, but also autoimmune disorders as a differential diagnosis – in particular if additional sypmtoms occur.

Case:

A 44-year-old woman presented to our clinic in April 2017 with 3 episodes of sudden hearing loss and tinnitus on the right ear along with vertigo in the past 6 months. Each time, the symptoms had recovered with corticosteroid treatment. However, in March 2017 she experienced a first episode of hearing loss and tinnitus in the left ear together with vertigo. Furthermore, she reported bilateral red eyes and arthralgia. Pure tone audiometry revealed a bilateral sensorineural hearing loss (35 dB on the left and from 40 dB at 0.25 kHz to 80 dB at 10 kHz on the right ear). In summary, vestibular testing showed a bilateral vestibulopathy (chronic on the right and subacute on the left side). On ophthalmologic examination, bilateral uveitis was described, which finally led to the diagnosis of atypical Cogan's syndrome. The patient was started on oral corticosteroids in cooperation with our colleagues from rheumatology. However, hearing on the right ear deteriorated further, so that methotrexate was added to the treatment in June 2017. Currently, the bilateral vestibulopathy has recovered almost completely – apart from a deficit in the right posterior canal. Hearing on the right ear has not recovered, however left-sided hearing thresholds are almost back to normal, as well as the ophthalmologic findings.

Conclusion:

Cogan's syndrome is a rare, but important differential diagnosis in cases of “Menière plus” syndrome. Diagnosis and treatment requires a close collaboration between otorhinolaryngology, ophthalmology and rheumatology.

Publication History

Publication Date:
23 May 2018 (online)