Volume 24, Issue 105, September - October, 2020

Atypical presentation of Cogan’s syndrome with granulomatous anterior uveitis - A case report

Heba Joharjy¹, Pierre-Jean Pisella¹, David Bakhos², Alexandra Audemard³, Marie Laure Le Lez¹

¹Department of Ophthalmology, University Hospital Bretonneau of Tours, Francois Rabelais Medicine Faculty of Tours, France
²Department of Otorhinolaryngology, University Hospital Bretonneau of Tours, Francois Rabelais Medicine Faculty of Tours, France
³Department of Internal Medicine, University Hospital Bretonneau of Tours, Francois Rabelais Medicine Faculty of Tours, France

ABSTRACT

Introduction: Cogan’s syndrome is a scarce inflammatory disorder that mostly impacts the visual and audiovestibular (AV) function. There are two common classifications of Cogan’s syndrome (CS), typical and atypical CS. The ordinary ocular presentation of atypical CS is non-syphilitic interstitial keratitis that may be associated with other ocular inflammation which includes non-granulomatous anterior uveitis (NAU). Case report: A case of atypical CS with bilateral granulomatous anterior uveitis (GAU) was described. Eighteen years old male patient was referred to the Eye Clinic, University Hospital Bretonneau, Tours, France, complaining of blurred vision in the last 3 weeks in both eyes. For the last 5 months, the patient suffered a bilateral severe progressive hearing loss, tinnitus, and vertigo in addition to repeated episodes of ocular redness. The visual acuity was 0.20 and 0.10 Log MAR in the right and left eye, respectively. Biomicroscopic examination showed GAU, the anterior chamber had +3 inflammatory cell and granulomatous infiltration of the iridocorneal angle with granulomatous keratic precipitatein both eyes. The intraocular pressure and dilated fundus examination were normal in the right and lefteyes. The patient was diagnosed as an atypical CS case with GAU. The patient was treated with intravenous methylprednisolone followed by oral prednisolone in addition to infliximab, topical steroid, and cycloplegic eye drops. The redness had relieved and the anterior uveitis resolved. However, there was no improvement in hearing, cochlear implants planned in a month. Conclusion: To the best of our knowledge, this is the first case of atypical CS with GAU without any systemic involvement.

Keywords: Cogan’s syndrome, granulomatous anterior uveitis, audiovestibular disturbances

Medical Science, 2020, 24(105), 3749-3752

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