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Cogan's syndrome (CS), a rare vasculitis characterized by non-syphilitic, interstitial keratitis and Meniere-like attacks, is classified into "typical" and "atypical" forms, while Takayasu arteritis (TAK) is a rare large-vessel vasculitis associated with human leukocyte antigen (HLA)-B*52. Very few cases meet both the CS and TAK classification criteria. We herein report a 53-year-old woman diagnosed with atypical CS and aortitis similar to TAK. Her 25-year-old daughter manifested TAK without symptoms of CS, and both are HLA-B*52 positive. Our case highlights the difficulties of distinguishing aortitis with atypical CS from aortitis with TAK.

The Cogan's syndrome (CS) is a very uncommon inflammatory condition that appears in young adults without a gender predisposition. It can be presented as typical, with interstitial non-syphilitic keratitis and Ménière-like audiovestibular manifestations. An atypical form of CS involves uveitis, scleritis, episcleritis, and systemic vascuitis symptoms. This is a case of a 41-year old male who reported eye redness, photophobia, and gait instability. His condition progressed to a sudden sensorineural hearing loss, tinnitus, and constant vertigo accompanied by cephalea. He was evaluated by the Otolaryngology, Ophthalmology, and Neurology departments giving rise to CS, Ménière's syndrome, acoustic neuroma, glaucoma, multiple sclerosis, and meningioma as differential diagnoses. The patient was prescribed with oral and intravenous steroids, ophthalmic antibiotics and steroids, as well as oral omeprazole. The patient was discharged, without his eye manifestations, and for his clinical progress and underwent conventional and speech audiometry, otoacoustic emissions, nystagmography, tympanometry, and auditory steady-state response, that showed a vestibular disfunction and a severe sensorineural hearing loss. His follow-up, six months later resulted with a normal vestibular function and an improvement from severe to mild sensorineural hearing loss. The Cogan's syndrome is a rare condition which can leave the patient with permanent incapacitating secuelae including profound hearing loss. Effective treatment is necessary to avoid complications and improve the patient's condition and life quality., Highlights • There are less than three hundred cases reported in the medical literature. • It is vital for physicians to include this pathology in their differential diagnosis. • Early diagnosis of this syndrome is key to avoid permanent incapacitant secuelae. • Early steroid therapy is the first-line treatment.

Introduction. Cogan?s syndrome is a rare variable vessel vasculitis. It can be typical and atypical. Basis of the treatment comprises glucocorticoids, and in patients with systemic manifestations, immunosuppressive drugs. Case report. We wanted to present the experience of the Clinic for Rheumatology and Clinical Immunology of the Military Medical Academy, Belgrade, in diagnosing and treating patients suffering from Cogan?s syndrome. The analysis included 7 patients. Patients? demographic characteristics, disease manifestations, course of the disease, applied treatment and treatment outcome were analysed. Five of the patients were women and 2 were men, with the average age of 39 ? 13 (25?65) years. The typical form of the disease manifested in 1 patient. In 6 patients, the first manifestation was the audiovestibular dysfunction. In 1 patient, systemic manifestations were the first to appear. In the cases where the disease manifested atypically, 3 patients developed conjunctivitis, 2 episcleritis, and 1 uveitis. They all had systemic manifestations. One female patient was diagnosed with aortitis and aortic insufficiency. They all tested positive for inflammatory biohumoral syndrome. Four patients had positive antinuclear antibodies, 3 anticytoplasmic antibodies, and 1 positive rheumatoid factor. They were all treated with glucocorticoid and immunosuppressive drugs. Methotrexate was administered to all the patients in doses up to 20 mg per week. Pulses of cyclophosphamide were administered to 2 female patients. All patients went successfully into remission. The female patient with the typical form of the disease experienced permanent bilateral hearing loss. Conclusion. Patients with a rapidly developed audiovestibular dysfunction should be viewed as suffering from Cogan?s syndrome from the viewpoint of differential diagnosis. A timely treatment with glucocorticoids can prevent hearing loss and the development of systemic manifestations of the disease. Precedence should be given to methotrexate when selecting an immunosuppressive drug.

The paper describes a clinical case of the typical variant of Cogan's syndrome (CS), a new nosological entity in the current classification of systemic vasculitides (SV), which belongs to a group of variable vasculitides. The literature review highlights in detail the problems of the diagnosis and treatment of this rare disease. CS is characterized by inflammatory eye involvement (interstitial keratitis, uveitis, and episcleritis) and hearing problems (sensorineural hearing loss, vestibular disorders) with the possible development of vasculitis at other sites, aortitis, and aortic or mitral lesions. A systemic lesion involving the ears and eyes necessitates to rule out granulomatosis with polyangiitis (Wegener's) and to make a differential diagnosis with a wide range of diseases. The given data underline the need for the interdisciplinary collaboration of rheumatologists, otorhinolaryngologists, audiologists, oculists, and cardiologists in order to improve the diagnosis and treatment of this form of SV.

Cogan's syndrome (CS) is a rare disorder characterized by nonsyphilitic interstitial keratitis (IK) and audio-vestibular symptoms. CS affects mainly young Caucasian adults, mostly during their first three decades of age, and may develop into typical and atypical variants. Typical CS manifests primarily with IK and hearing loss, whereas atypical CS usually presents with inflammatory ocular manifestations in association with audio-vestibular symptoms but mostly different Meniere-like symptoms and, more frequently, with systemic inflammation (70%), of which vasculitis is the pathogenic mechanism. CS is considered as an autoimmune- or immune-mediated disease supported mainly by the beneficial response to corticosteroids. Using well-developed assays, antibodies to inner ear antigens, anti-Hsp70, and antineutrophil cytoplasmic antibodies were found to be associated with CS. Corticosteroids represent the first line of treatment, and multiple immunosuppressive drugs have been tried with variable degrees of success. Tumor necrosis factor-alpha blockers and other biological agents are a recent novel therapeutic option in CS. Cochlear implantation is a valuable rescue surgical strategy in cases with severe sensorineural hearing loss unresponsive to intensive and/or innovative immunosuppressive regimens.

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 37 years [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.

The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated to rapidly progressive hearing loss that, especially at the early stages of disease, may be at monoaural localization, although more often it is at binaural localization. It usually occurs as a sudden deafness, or a rapidly progressive sensorineural hearing loss. In this study a particular form of autoimmune inner ear disease is described, Cogan’s syndrome. Cogan’s syndrome is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis, vestibular and auditory dysfunction. Associations between Cogan’s syndrome and systemic vasculitis, as well as aortitis, also exist. We report a case of a young woman who presented audiological and systemic characteristics attributable to Cogan’s syndrome. In the description of the case we illustrate how the appearance and evolution of the disease presented.

Cogan's syndrome is an inflammatory disease that is characterized by ocular inflammation (typically interstitial keratitis) and is associated with Ménière-like vestibuloauditory dysfunction. Ocular inflammation usually resolves after several weeks or months but deafness is often irreversible. We report on a case of Cogan's syndrome in a 23-year-old woman who initially presented with bilateral anterior uveitis, an unusual clinical feature for this disease. We discuss the clinical aspects, the pathogenic mechanisms, the laboratory investigations, the differential diagnosis, and the treatment of Cogan's syndrome., Le syndrome de Cogan est une maladie inflammatoire caractérisée par une atteinte oculaire (typiquement une kératite interstitielle) associée à une symptomatologie cochléo-vestibulaire similaire à celle de la maladie de Ménière. L'évolution de la kératite interstitielle est habituellement très favorable tandis que la surdité profonde est souvent définitive. Nous rapportons le cas d'une jeune femme de 23 ans dont l'atteinte oculaire initiale était une uvéite antérieure bilatérale, manifestation oculaire atypique du syndrome de Cogan. À la lumière de cette observation, nous discutons les caractéristiques cliniques, la pathogénie, les moyens diagnostiques, le diagnostic différentiel et le traitement du syndrome de Cogan.