Contribution of Serology in the Diagnosis of Cogan’s Syndrome

Introduction

Cogan’s syndrome is a rare, systemic inflammatory disorder of presumed autoimmune origin associating recurrent ocular inflammation with Menière-like vestibuloauditory symptoms and severe hearing loss. The syndrome presents with two distinct forms.

The typical form, first described by Cogan in 1945 [2], is characterized by mostly bilateral non-syphilitic interstitial keratitis preceded or followed by severe vestibuloauditory manifestations that often progress to complete hearing loss [4].

In 1980, Haynes et al. [5] broadened the definition of Cogan’s syndrome to include any form of ocular inflammation, such as conjunctivitis, episcleritis, uveitis, optic disc edema or retinal vasculitis and also audiovestibular symptoms other than Menière-like episodes, proposing diagnostic criteria for atypical Cogan’s syndrome.

Concerning vestibuloauditory manifestations, neurosensorial hearing loss, vertigo, tinnitus, ataxia, oscillopsia and gaze-evoked nystagmus are the most common symptoms described. As a rule, hearing loss is sudden, bilateral, fluctuating and progressive with frequent progression to complete deafness [4].

Contrary to vestibuloauditory involvement, ocular symptoms respond well to topical treatment with corticosteroids and severe visual repercussions are rare, with only 10 % of patients having visual deficits directly attributable to their disease [4].

Various treatment approaches have been adopted for Cogan’s syndrome, with some articles advocating aggressive treatment with systemic corticosteroids, and cytotoxic drugs, such as methotrexate and cyclophosphamide for audiovestibular disfunction in order to prevent irreversible deafness.

Controversy exists regarding the role of detecting autoantobodies to the inner ear and cornea for the diagnosis of Cogan’s syndrome. We report here a case of unilateral Cogan’s syndrome with primary ocular involvement and subsequent mild hearing impairment, confirmed by the presence of anti-cochlear autoantibodies in the patient’s serum.

References

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• 2 Cogan D G. Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms.  Arch Ophthalmol. 1945;  33 144-149
• 3 Garcia Callejo F J, Platero Z A, Sebastian G E et al. Atypical Cogan syndrome. Clinical and laboratory spectrum. Report of 2 cases.  Acta Otorrinolaringol Esp. 2002;  53 191-198
• 4 Gluth M B, Baratz K H, Matteson E L et al. Cogan syndrome: a retrospective review of 60 patients throughout a half century.  Mayo Clin Proc. 2006;  81 483-488
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