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Cogan's syndrome is a condition of unknown origin, classified as a systemic vasculitis. It is characterised by a predilection for the cornea and the inner ear. It mainly affects Caucasian individuals with a sex-ratio close to one. Ophthalmological and cochleo-vestibular involvement are the most common manifestations of the disease. The most frequent ophthalmological type of involvement is non-syphilitic interstitial keratitis. Cochleo-vestibular manifestations are similar to those of Meniere's syndrome. The disease progresses in ocular and ear-nose-throat (ENT) flares, which may occur simultaneously or in isolation. Association with other autoimmune diseases, particularly other forms of vasculitis such as polyarteritis nodosa or Takayasu's arteritis, is possible. Ocular involvement, as well as cochleo-vestibular involvement, can be inaugural and initially isolated. Onset is often abrupt. The characteristic involvement is "non-syphilitic" interstitial keratitis. It is usually bilateral from the outset or becomes so during the course of the disease. It presents as a red, painful eye, possibly associated with decreased visual acuity. Cochleo-vestibular involvement is usually bilateral from the outset. It is characterised by the sudden onset of continuous rotational vertigo associated with tinnitus, rapidly progressive sensorineural deafness. Approximately 30-70% of patients present with systemic manifestations. Deterioration in general status with fever may be present. Laboratory evidence of inflammatory syndrome is associated in 75% of cases. Cogan's syndrome is a presumed autoimmune type of vasculitis, although no specific autoantibodies have been identified. Ocular involvement is usually associated with a good prognosis, with total visual acuity recovery in the majority of cases. In contrast, cochleo-vestibular involvement can be severe and irreversible. Therapeutic management of Cogan's syndrome, given its rarity, lacks consensus since no prospective randomised studies have been conducted to date. Corticosteroid therapy is the first-line treatment. Combination with anti-TNF therapy should be promptly discussed., (Copyright © 2024 The Authors. Published by Elsevier Masson SAS.. All rights reserved.)

Cogan’s syndrome is a rare disorder characterized by ocular and audiovestibular manifestations in its typical form and caries a wide variety of atypical manifestations. It is considered as an autoimmune disease. We present the first case in the literature of a 67 year old woman with the development of low grade non-Hodgkin lymphoma (NHL) in the mastoid bone in a pre-existing history of atypical Cogan’s syndrome. The anatomical development of NHL was to a “target” organ of Cogan’s syndrome, which is the inner ear.

"Typical" Cogan's syndrome is defined as a non-syphilitic interstitial keratitis associated with audio-vestibular resembling Ménière's disease with a 2-year maximum delay between these 2 organ impairment. Cogan syndrome is classified as "atypical" in the absence of interstitial keratitis and the presence of other inflammatory eye manifestations, an audio-vestibular impairment different from typical Menière-like disease, or a delay longer than 2 years between eye and audio-vestibular manifestations. Constitutional signs and large-vessel vasculitis is also possible, mostly affecting the thoracic aorta. The presence of acute-phase reactants is common, but no specific laboratory tests are available. The prognosis is dominated by the audio-vestibular impairment and in particular the risk of deafness, while other complications especially vascular complications being rare. Treatment with glucocorticoids is usually necessary and the combination to other immunosuppressive therapies or biological-targeted drugs needs to be determined., (Copyright © 2020 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Background: Cogan syndrome is mainly treated with steroids. We aimed to determine the place of DMARDs and biologic-targeted treatments., Patients and Methods: We conducted a French nationwide retrospective study of patients with Cogan syndrome (n=40) and a literature review of cases (n=22) and analyzed the efficacy of disease-modifying anti-rheumatic drugs (DMARDs) and tumor necrosis factor α (TNF-α) antagonists., Results: We included 62 patients (31 females) (median age 37years [range 2-76]. At diagnosis, 61 patients (98%) had vestibulo-auditory symptoms, particularly bilateral hearing loss in 41% and deafness in 31%. Ocular signs were present in 57 patients (92%), with interstitial keratitis in 31 (51%). The first-line treatment consisted of steroids alone (n=43; 70%) or associated with other immunosuppressive drugs (n=18; 30%). Overall, 13/43 (30%) and 4/18 (22%) patients with steroids alone and with associated immunosuppressive drugs, respectively (p=0.8), showed vestibulo-auditory response; 32/39 (82%) and 15/19 (79%) ocular response; and 23/28 (82%) and 10/14 (71%) general response. Overall 61 patients had used a total of 126 lines of treatment, consisting of steroids alone (n=51 lines), steroids with DMARDs (n=65) and infliximab (n=10). Vestibulo-auditory response was significantly more frequent with infliximab than DMARDs or steroids alone (80% vs 39% and 35%, respectively), whereas ocular, systemic and acute-phase reactant response rates were similar. Infliximab was the only significant predictor of vestibulo-auditory improvement (odds ratio 20.7 [95% confidence interval 1.65; 260], p=0.019)., Conclusion: Infliximab could lead to vestibulo-auditory response in DMARDS and steroid-refractory Cogan syndrome, but prospective studies are necessary., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objective: The aim of this study was to systematically evaluate the evidences for treatment of Cogan's syndrome (CS), with reference to adult and younger patients described in the literature., Systematic Review Methodology: DATA SOURCES: Studies reviewed were English language original articles ranging from 1990 to 2016 reporting data for subjects with CS (typical and atypical) undergoing any systemic treatment other than steroids alone. Medline/EMBASE, Cochrane Library were searched. The full text of articles meeting selection criteria was reviewed for: study type, diagnosis, number of subjects, treatment type and duration, clinical and/or functional outcomes, possible systemic manifestation or other autoimmune syndromes combined., Results and Conclusions: The authors identified 76 relevant reports: 4 prospective studies, 2 retrospective studies, 12 case series and 58 case reports. The studies included a total of 141 new patients: based on the available data, 46 men and 50 women with a mean age of 33years (range 5-69). In the descriptive analysis adult patients (N=87) were separated from pediatric patients (<18years old) (N=17). Concerning treatment strategies, except for a first-line approach to check for a rapid remission, or for a drug sensibility often supporting the diagnosis, a long-term steroidal monotherapy is no longer recommended in CS. As in other autoimmune and rheumatologic diseases, combined treatment with steroid-sparing immunosuppressant agents, also considering "biological" drugs, is nowadays preferred. However, the evidence of clearly effective or preferable treatment options for CS remains lacking, mostly due to the rarity of the disease and to the consequent difficulty in organizing high-quality prospective trials., (Copyright © 2017. Published by Elsevier B.V.)