Cogan's syndrome (CS), described by David G. Cogan in 1945,1 is a disease of unknown etiology, but probably has an autoimmune basis. It is characterized by the association of non-syphilitic interstitial keratitis and audiovestibular symptoms (similar to Meniere's syndrome), occurring in a period of less than 2 years. It occurs predominantly in young adult Caucasians. There is no predominance of gender.2 When the eye or audiovestibular manifestations are different from what is seen, or the interval between them is more than 2 years, it is called atypic CS.3 It may be associated with other systemic manifestations such as aortitis and vasculitis.4 A patient with atypical CS is presented.

The patient is a 44-year-old male, hypertensive, with axial psoriatic arthritis since age 20 and anterior nongranulomatous uveitis developed in the last 4 years. He attended consultation derived from Otorrhinolaryngology for bilateral Ménière's syndrome resistant to standard therapy, of 3 years of evolution. Audiometry revealed cophosis stood in the left ear and moderate hearing loss in the right ear (RE) at 60dB (the patient wore a hearing aid). Physical examination showed psoriasis and redness in the extremities of both ears, which were rubbery to the touch. No peripheral arthritis was found but he had axial involvement (BASMI: 6). No other data were found upon examination. Laboratory tests showed: ESR of 49mm in the 1st hour and CRP 25mg/L. CBC, serum biochemistry and urinalysis were normal. Autoimmunity: rheumatoid factor, antineutrophil cytoplasmic antibodies, antinuclear antibodies and anti-CCP antibody were all negative. HLA B27 antigen was positive. C3 and C4 were normal. Radiographs of the chest, hands and feet were normal. The sacroiliac X-rays showed stage IV sacroiliitis. Electrocardiogram, echocardiography and computed tomography with intravenous contrast were normal. Atypical CS was diagnosed associated with relapsing polychondritis (no biopsy was performed) and psoriatic arthritis. Methotrexate (MTX) was started orally at doses of 12.5mg/week. At 2 months he presented sudden hearing loss in the RE compared to baseline. The bilateral otoscopy was normal. Audiometry revealed a serious 60dB drop in treble in the RE conversational frequencies. ESR and CRP were 86mm and 45.30mg/L, respectively. Treatment was initiated with boluses of methylprednisolone 1000mg every 24h for 3 days, showing a good response, recovering RE hearing to baseline (determined through a new audiometry) and normalization of acute phase reactants. The patient was discharged with prednisone 30mg/day and tapered the dosage of MTX to 12.5mg/week, remaining asymptomatic after 6 months. Since the first description of CS, more than 220 cases have been described, 92 of them appearing atypically.5 Unlike typical CS, the atypical variety is most commonly associated with systemic6 manifestations and other autoimmune diseases, such as sarcoidosis, rheumatoid arthritis, relapsing polychondritis, juvenile idiopathic arthritis, Sjögren's syndrome and inflammatory bowel disease, among others.7 Our case may raise doubts about the diagnosis, given the coexistence of several autoimmune8,9 diseases. Psoriatic arthropathy could justify that the patient presented uveitis. Relapsing polychondritis can also present with hearing loss and vertigo, although generally it is a conductive hearing loss and vestibular dysfunction is not as similar to Meniere's. In this patient, the vestibular episodes were intense, with prolonged and bilateral sensorineural hearing loss, preceded by ocular involvement in less than a two year interval, and in the absence of specific complementary data, made us opt for the diagnosis of atypical CS, fulfilling the criteria established by Haynes et al.,3 with 2 associated autoimmune disorders (psoriatic arthritis and relapsing polychondritis) and showing a good response to corticosteroid and immunosuppressive therapy, something relevant given the poor prognosis of deafness.