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Case Report: Case report of a 51 year old patient diagnosed with atypical Cogan's syndrome. The patient exhibited interstitial keratitis, anterior uveitis, and long-standing profound deafness. The treatment was based on topical and systemic steroids, resulting in a satisfactory evolution and currently in clinical remission., Discussion: Cogan's syndrome is a chronic inflammatory disease that has existed for over 60 years. Because its diagnosis is purely clinical, not many cases have been reported worldwide. Therefore, it is important to be aware of this condition to make a quick diagnosis and provide multidisciplinary treatment, thus preventing irreversible complications., (Copyright © 2024 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

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 (CS) is recognized as a form of variable vasculitis. This report presents the case of a middle-aged woman experiencing recurrent coronary artery stenosis, accompanied by a history of non-syphilis keratitis, vestibular auditory symptoms, and venous thrombosis. Positron emission tomography/computed tomography revealed an elevated uptake of (18)F-fluorodeoxyglucose in the subclavian artery, common carotid artery, aortic arch, and thoracic aorta. A diagnosis of Cogan's syndrome was made. The aim of this study was to increase clinicians' awareness of the vascular manifestations in CS and to emphasize the importance of thorough history taking. CS should be included in the differential diagnosis when patients present with recurrent coronary artery stenosis., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Na, Nan, Jingjing and Lili.)

Cogan's syndrome is characterized by ocular symptoms and auditory vestibular dysfunction. Auditory vestibular dysfunction in Cogan's syndrome is believed to be similar to Ménière's disease, but the cause is not known in detail. We present the case of a 10-year-old boy with Cogan's syndrome. The patient had panuveitis, bilateral hearing loss, and bilateral vestibular dysfunction. MRI revealed no evidence of endolymphatic hydrops, which is a cause of Ménière's disease, and enhanced contrast effects on the bilateral cochlear and vestibular apparatus. The caloric test, the video-head impulse test, and the vestibular evoked muscle potential test also showed severe vestibular dysfunction. Based on the above, the auditory vestibular dysfunction in this patient was considered to be caused by internal otitis. The patient's vision recovered after treatment with steroids and immunosuppressive drugs, but his hearing did not recover. He underwent bilateral cochlear implantation and had a good postoperative course but we encountered difficulty in deciding when to perform cochlear implantation. This case demonstrates the importance of determining the timing of surgery in consideration of the ossification and fibrosis of the inner ear and the drug administration status., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Sasano et al.)

Cogan's syndrome (CS) is a rare chronic inflammatory disease, characterized by interstitial keratitis and vestibular auditory dysfunction. Hypertrophic pachymeningitis (HP) is a rare chronic aseptic inflammatory disease of the central nervous system. This article reports a patient with CS coexisting with HP. The patient was a 66-year-old male with fever, headache, red eyes, hearing loss, and significantly elevated inflammatory markers. Cerebrospinal fluid examination, blood culture, and tests for autoantibodies such as antinuclear antibodies were negative. Pure tone audiology (PTA) indicated bilateral sensorineural deafness. Both Positron emission tomography-computed tomography (PET/CT) and vascular color Doppler ultrasound suggest the presence of vasculitis. Considering Cogan's syndrome, the patient received 40 mg of methylprednisolone intravenously once daily. The brain's magnetic resonance imaging (MRI) revealed slightly thickened and enhanced dura mater, suggesting HP. The dose of methylprednisolone was increased to 40 mg intravenously every 8 hours, leading to the patient's improved symptoms and decreased inflammatory markers. Both CS and HP are rare chronic inflammatory diseases, and their coexistence is even rarer, with only two reported cases in literature up to date. The coexistence of CS and HP should be considered when the CS patients with headaches do not respond well to treatment., Competing Interests: The authors declare that there is no conflict of interest., (© 2024 Lu et al.)

Cogan's syndrome is a rare autoimmune inflammatory disease, characterized by interstitial keratitis and audio-vestibular signs. The syndrome was first described in 1945 by David G. Cogan. Then, it was only in 1980 when Haynes et al. proposed diagnostic criteria for patients with other symptoms and was qualified as atypical form of Cogan's syndrome. Herein, we report a case of a 28-year-old woman with atypical Cogan's syndrome. The patient was treated with corticosteroids and received a cochlear implant., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

A 61-year-old man presented with weight loss, bilateral ocular redness, blurred vision, and sensorineural hearing loss. Fluorodeoxyglucose-position emission tomography/computed tomography demonstrated an uptake in the ascending and descending aorta, abdominal aorta and femoral arteries. Atypical Cogan's syndrome complicated with large-vessel vasculitis (LVV) was diagnosed. He was treated with high-dose prednisolone and subcutaneous tocilizumab (162 mg/week), resulting in successful improvements in his ocular and vascular involvements. Although there is currently no established treatment strategy for LVV associated with Cogan's syndrome, our case and literature review suggest that tocilizumab is a viable treatment option for this rare but life-threatening complication.

Cogan's syndrome (CS) is a rare disorder of an unknown origin characterized by inflammatory eye disease and vestibuloauditory symptoms, primarily affecting young white adults, without a hereditary pattern. The exclusion of other diseases makes diagnosis difficult, and it is likely underreported in the literature. A 74-year-old previously healthy African American male presented with ear and jaw pain, later accompanied by vestibular symptoms, fever of unknown origin, aortitis, and a third-degree heart block. The workup revealed incidental renal cell carcinoma and interstitial keratitis. This case highlights the challenge of diagnosing an atypical presentation of CS with late-onset interstitial keratitis by excluding other complex syndromes., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Kirsch et al.)

Background: Cogan's syndrome (CS) is a rare autoimmune disorder characterized by non-syphilitic interstitial keratitis (IK) and Menière-like cochlear vestibular symptoms, which may also have systemic effects. Corticosteroids are first-line treatment. DMARDs and biologics have been used to treat ocular and systemic symptoms of CS., Case Presentation: This is a case of a 35-year-old female who reported hearing loss, eye redness and photophobia. Her condition progressed to a sudden sensorineural hearing loss, tinnitus, and constant vertigo accompanied by cephalea. CS was diagnosed after excluding other diseases. The patient still developed bilateral sensorineural hearing loss after receiving hormone, methotrexate, cyclophosphamide, and a variety of biological agents. Joint symptoms were relieved after treatment with a JAK inhibitor (tofacitinib), and hearing did not deteriorate further., Conclusions: CS should be involved in the differential diagnosis of keratitis. Early identification and intervention of this autoimmune disease can minimize disability and irreversible damage., (© 2023. The Author(s).)

Cogan's syndrome (CS) is a rare autoimmune disease in which approximately 10%-13% of people with the condition develop neurological symptoms. While glucocorticoids are the standard of care for patients with CS, disease-modifying anti-rheumatic drugs (DMARDs) and biologics agents are more widely used to treat the systemic and vestibular auditory manifestations of CS. Herein, we report a rare case of CS with central nervous system damage who failed to respond to systemic use of glucocorticoids and DMARDs. However, his symptoms were successfully improved by intrathecal injection of methotrexate (MTX) and dexamethasone. To our knowledge, the use of intrathecal injections of MTX and dexamethasone to treat CS has not been reported in any literature. Therefore, the present case may provide a new idea for clinicians to treat central nervous system symptoms in patients with CS., (© 2023 The Authors. International Journal of Rheumatic Diseases published by Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral. Although glucocorticoids are fundamental treatment options, in most cases, hearing loss gradually worsens. Herein we report 2 pediatric cases of CS who were treated with corticosteroids and methotrexate. One patient had a cochlear implant, and the hearing of the other patient improved with treatment. Also, a systematic literature review was conducted for articles including pediatric CS patients. In the literature, 34 articles describing 44 pediatric patients with CS were identified. Sudden hearing loss (95.3%) and ocular symptoms (92.5%) were the most common manifestations in these patients. Also, aortic involvement was present in 19.5% of patients in the literature. Otorhinolaryngologists, ophthalmologists, and pediatricians should collaborate to diagnose and manage CS to prevent progressive hearing loss and eye involvement., (© 2022 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Fever of unknown origin remains a diagnostic challenge. Aortitis, defined as inflammation of the aorta, has multiple infectious and non-infectious causes. We report the case of an elderly woman with vertigo and bilateral hearing loss, presenting with fever of unknown origin. Blood tests were remarkable for leucocytosis with neutrophilia, elevation of C-reactive protein and the erythrocyte sedimentation rate, and positive antinuclear antibodies and rheumatoid factor, and an unremarkable search for multiple infectious causes of fever. During admission the patient developed a bilateral red eye. Abdominal and chest computed tomography was performed and demonstrated signs of aortitis. Due to the coexistence of aortitis, ocular inflammation, vertigo and bilateral hearing loss in a patient with persistent fever and elevation of inflammatory parameters, a presumptive diagnosis of Cogan's syndrome was made, with improvement after initiation of steroid therapy., Learning Points: Fever of unknown origin is a diagnostic challenge with an extensive list of possible causes.The coexistence of fever, aortitis, ocular and vestibulo-cochlear symptoms should raise the suspicion of Cogan's syndrome.Steroid therapy is the first-line treatment for Cogan's syndrome., Competing Interests: Conflicts of Interests: The authors declare there are no competing interests., (© EFIM 2022.)

Cogan's syndrome is a rare form of autoimmune vasculitis that manifests with ocular and audiovestibular symptoms. The clinical presentations of Cogan's syndrome can be classified as typical and atypical, and the auditory symptoms are classically similar to those seen in cases of Meniere's syndrome. Without treatment, Cogan's syndrome could become severe, resulting in sensorineural hearing loss in over 50% of patients. This report describes a rare case of uncontrolled intraocular pressure and choroidal effusion in a patient with atypical Cogan's syndrome. A 51-year-old woman was referred to our clinic on account of bilateral distortion of visual acuity and ocular pain following admission to the internal medicine department for bilateral hearing loss, acute renal failure, and fever. Upon ocular examination, we observed bilateral 2-3+ cells in the anterior chamber and closed-angle glaucoma, and choroidal effusion in the right eye. The intraocular pressure could not be controlled with topical and systemic medications, and a bilateral valve implant surgery was performed subsequently. Two weeks later, after systemic corticosteroid therapy, the choroidal effusion resolved. Cogan's syndrome can provoke angle-closure glaucoma with choroidal effusion in patients with a short axial length and shallow anterior chamber., Competing Interests: The authors have no conflicts of interest to declare., (Copyright © 2022 by S. Karger AG, Basel.)

A 49-year-old Japanese man with a 2-month history of a fever, headache, and bilateral conjunctival hyperemia was admitted. His condition fulfilled the giant cell arteritis classification criteria (new headache, temporal artery tenderness, elevated ESR) and atypical Cogan's syndrome (CS) with scleritis and sensorineural hearing loss (SNHL). The interleukin (IL)-6 serum level was extremely high. Two weeks after his insufficient response of SNHL and scleritis to oral prednisolone, we administered tocilizumab (TCZ); rapid improvements in scleritis and SNHL occurred. Early IL-6 target therapy can help prevent irreversible CS-induced sensory organ damage.

Cogan's syndrome is a rare disorder characterized by inflammatory eye and inner ear/vestibular disease. In some cases patients may present medium and large vessel vasculitis which may produce neurological manifestations. We present the case of a patient who was admitted with clinical manifestations of stroke. After intense study, Cogan's syndrome was diagnosed and treated., Competing Interests: The authors declare no conflict of interest., (© 2022 The Author(s). Published by IMR Press.)

Objective: Cogan's syndrome (CS) is an inflammatory disease classified as variable vessel vasculitis. It is a rare disease with few published series, and therefore we reviewed our experience in the last ten years in two centres., Materials and Methods: Description of 7 diagnosed cases of CS, according to the classification criteria (typical or atypical), their clinical manifestations, treatments used and their complications. A comparative analysis was performed with the series and cases described in the literature., Results: Seven cases were included, three men and four women, with a mean age at diagnosis of 43 years, and an average disease duration of 47 months. Five patients met the typical characteristics according to the 1980 classical criteria, the rest being atypical cases, one due to the absence of interstitial keratitis and another due to a period between the onset of ocular and auditory-vestibular clinical symptoms greater than two years. All received immunosuppressants, methotrexate being the most commonly used, followed by azathioprine. In 5 cases, biological drugs were used, infliximab in 4 times and 2 tocilizumab. One patient died from bacterial endocarditis and septic shock., Conclusion: The characteristics of the series presented are like those published to date, with clinical differences mainly in the involvement of large vessels. Given the low frequency, it seems necessary to create multicentre records to improve the evidence regarding the management of patients with CS., (Copyright © 2020 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

"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.)

Cogan's syndrome is a rare autoimmune disease characterized by ocular inflammation and audiovestibular manifestations. Treatment consists of systemic glucocorticoids and other immunosuppressive agents including methotrexate, cyclophosphamide and TNF-α-inhibitors. Due to potential ovarian or fetal toxicity immunosuppressive treatment options are limited during pregnancies. Thus far there is a paucity of reports on pregnancies in Cogan's syndrome. With minimal transplacental transfer, Certolizumab pegol is considered to be safe for the use in pregnant patients with underlying inflammatory diseases. However, there is no literature on the use of this TNF-α-inhibitor in Cogan's syndrome in general and especially during gestation. Here we report three pregnancies in two Cogan's Syndrome-patients treated with Certolizumab pegol. Treatment with Certolizumab pegol was effective and well tolerated in patients with Cogan's syndrome and seems to be a safe treatment option during pregnancy., Competing Interests: NV, IJ, RV, and CG received payment (less than 5000€) from UCB pharmaceuticals for advisory boards, lectures, or travel grants within the last five years. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Venhoff, Thiel, Schramm, Jandova, Voll and Glaser.)

Cogan's syndrome (CS), a rare vasculitis characterized by non-syphilitic, interstitial keratitis and Ménière-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.

Purpose of Review: Cogan's syndrome (CS) is a rare systemic vasculitis that can severely affect vision and hearing, which may also have significant systemic effects. Early recognition of this autoimmune disorder and intervention can minimize disabling and irreversible damage., Recent Findings: This article will review the varying clinical presentations of CS and emerging information of systemic disease associated with CS. We will also review recently published promising treatment outcomes using immune modulating medications. As our framework for recognizing the markers of CS and the associated systemic disorders expands, more effective guidelines and treatment options may emerge.

Introduction: Cogan's syndrome (CS) is a rare autoimmune disease, characterized by ocular and vestibulo-auditory involvement. Treatment of CS could be challenging, and the only evidence-based data comes from case reports or series., Areas Covered: There have recently been several reports of new treatment strategy involving the use of biological disease-modifying anti-rheumatic drugs such as TNFα inhibitors, anti-CD20 or anti-IL6 receptor antibodies, in cases resistant to first- or second-line drugs., Expert Commentary: Corticosteroids are the cornerstone of CS therapy at disease onset and during acute phases of the disease. Conventional immunosuppressive therapy, such as methotrexate, could be used in relapsing patients or as a glucocorticoid sparing agent, but efficacy is often modest. The anti-TNFα monoclonal antibody Infliximab appears to be the most frequently used, leading to an improvement in hearing loss in 89% of the cases and allow corticosteroid tapering in 86% of the patients. The appropriate timing of Infliximab treatment has yet to be thoroughly investigated, but it seems to be more effective when started at an early stage of the disease. Efficacy of others anti-TNFα agents is controversial. Rituximab and Tocilizumab are a safe option, but results on hearing loss have still to be confirmed on larger patients' cohorts.

Cogan's syndrome is a rare disorder characterized by the coexistence of ocular and audio-vestibular manifestations. Systemic manifestations are quite unusual with pan-vasculitis and cardiac involvement reported in the form of aortitis with aortic aneurysm, dissection, or extremely rare aortic valve perforation. Hereby, we report a case of a 56-year-old woman presented with ocular, audio-vestibular, and systemic manifestations with medium-sized vasculitis in the form of multiple splenic artery aneurysms, superior mesenteric artery thrombosis, and cardiovascular involvement in the form of aortic regurgitation due to noncoronary cusp perforation. To the best of our knowledge, this is the second case to report aortic perforation in the setting of Cogan's syndrome., (© 2019 Wiley Periodicals, Inc.)

Objective: AIED (autoimmune inner ear disease) is an autoimmune process that leads to the dysfunction of the inner ear, resulting in fluctuating, audiovestibular symptoms. Although the pathogenesis is likely heterogeneous, immune processes within the inner ear ultimately lead to histopathologic changes and sensorineural hearing loss. This review will discuss the latest evidence on treatment options., Methods: A literature search on articles pertaining to the treatment of autoimmune inner ear disease was performed on PubMed., Results: Corticosteroid treatment continues to remain as first line therapy for AIED but long-term responsiveness is poor. Cytotoxic chemotherapies can be effective alternatives for steroid nonresponsive patients, but significant side effects may limit their use. Intratympanic steroid injections are beneficial and although there is not enough evidence currently to supplant oral steroid trial they may be a useful adjunct if steroid toxicity is an issue. The efficacy of biologic agents has been variable. Compared to placebo, etanercept does not improve the hearing improvement already attained by steroids alone. However, open pilot studies of other biologic agents show hearing improvements, improvements in tinnitus/aural fullness/vertigo, ability to wean steroid dependency, or benefits in steroid-resistant AIED., Conclusion: There is currently not enough evidence that alternative treatments supersede the use of initial steroid treatment. Biologic agents and intratympanic steroid injections are relatively well tolerated and should be considered as adjunctive therapy. More studies on the efficacy of various biologics and more studies on the treatment of steroid resistant disease especially after initial benefit are still needed. For those who eventually lose their hearing, cochlear implantation remains as a viable option., Level of Evidence: expert opinion.

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.

Cogan's syndrome (CS) is defined by the combination of hearing loss, vertigo, and ocular inflammation of uncertain cause, and can be associated with variable vessel vasculitis. Vasculitic manifestations may include arteritis (affecting large, medium or small arteries), aortitis, and aortic and mitral valvulitis. Cutaneous manifestations including erythema, papules, subcutaneous nodules, and purpura sometimes occur; however, to date, only six cases have been histologically confirmed to have genuine vasculitis. Here, we report two cases of CS, one of which involved a patient who developed the typical symptoms of Takayasu arteritis and purpuric lesions in the legs, with histologic findings consistent with small vessel vaculitis in the dermis. The second case involved a patient who developed subcutaneous nodules in the legs and the axilla, and histologic findings revealed a necrotizing vasculitis of the small arteries in the interlobular area. Both cases were successfully treated with systemic steroid therapy. Based on the clinical features and the examination data, there is a possibility that a Chlamydia trachomatis infection played a pivotal role in the pathogenesis of those vasculitides.

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 Ménière-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., Competing Interests: Disclosure The authors report no conflicts of interest in this work.

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.)

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.

The association of sensorineural hearing loss and vertigo with inflammatory eye disease, usually interstitial keratitis, has been called Cogan's syndrome. The pathogenesis of Cogan's syndrome is unknown, but it has been assumed to be an immune mediated disorder with vasculitis. The histopathology of the inner ear in Cogan's syndrome has been described in 6 case reports. Although common pathologic findings in these reports include degeneration of the auditory and vestibular neuroepithelium, endolymphatic hydrops, fibrosis, and new bone formation, direct pathologic evidence of a vasculitis has not been published. A possible reason for this failure to identify vasculitis was a substantial delay (range, 4-40 years) between the onset of symptoms and examination of the otopathology. In the current case report, the patient had both auditory and vestibular symptoms and interstitial keratitis with a time delay of only 2 to 4 weeks between symptoms and death. Evidence of a vasculitis as a possible underlying etiology included H&E histopathology and anti-CD45 immunostaining of vessels both in the auditory and vestibular systems, supporting the hypothesis of a vasculitis as a mechanism in this disorder., (© The Author(s) 2015.)

Background: Cogan's syndrome (CSy) is a very rare autoimmune disorder, mainly affecting the inner ear and the eye, and is associated with inflammatory bowel disease (IBD)., Methods: This was a European Crohn's and Colitis Organisation (ECCO) retrospective observational study, performed as part of the CONFER project. A call to all ECCO members was made to report concomitant CSy and inflammatory bowel disease (IBD) cases. Clinical data were recorded in a standardized questionnaire., Results: This international case series reports on 22 concomitant CSy-IBD cases from 14 large medical centres. Mean duration of IBD until diagnosis of CSy was 8.7 years (range 0.0-38.0) and mean age at CSy diagnosis was 44.6 years (range 9.0-67.0). Six patients had underlying ulcerative colitis (UC) and 16 had Crohn's disease. Eleven patients (50%) had active disease at CSy diagnosis. Sixteen patients were under IBD treatment at the time of CSy diagnosis, of whom 6 (37.5%) were on anti-tumour necrosis factor (TNF). Seven out of 10 patients, who were treated for CSy with immunomodulators (mostly with corticosteroids), demonstrated at least partial response., Conclusion: This is the largest CSy-IBD case series so far. Although CSy is considered to be an autoimmune disease and is associated with IBD, immunomodulatory IBD maintenance treatment and even anti-TNF therapy do not seem to prevent disease onset. Moreover, IBD disease activity does not seem to trigger CSy. However, vigilance may prompt early diagnosis and directed intervention with corticosteroids at inception may potentially hinder audiovestibular deterioration. Finally, vigilance and awareness may also offer a better setting to study the pathophysiological mechanisms of this rare but debilitating phenomenon., (Copyright © 2015 European Crohn’s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

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.

Cogan's syndrome is a rare chronic vasculitis, characterized in its typical form by progressive sensorineural bilateral hearing loss, vestibular symptoms and non-syphilitic interstitial keratitis. Only a few cases have been reported in children, most of whom have been diagnosed with the typical form. Early diagnosis and treatment are crucial to ensure a favorable prognosis. Systemic treatment usually begins with high dosage corticosteroids. In case the initial treatment fails, other immunosuppressive drugs are used (cyclophosphamide, methotrexate, cyclosporine A and azathioprine). Additional treatment possibilities, such as plasmapheresis, TNF-alpha blockers (etanercept and infliximab), rituximab, tocilizumab and mycophenolate mofetil have been described over the past few years., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

The fibromyalgia syndrome (FMS) is characterized by chronic and widespread musculoskeletal pain and soreness accompanied by sleep disorders, chronic fatigue and affective disorders. FMS is often associated with other forms of immuno-rheumatic diseases. Although FMS pathophysiology is still not fully understood, a number of neuroendocrine, neurotransmission and neurosensitive disorders might generate a mechanism for the elicitation of pain by "central sensitization," which is common to many other painful conditions. The present case describes the success of a therapeutic scheme, which associates two different pharmacological classes, anticonvulsants and new-generation antidepressants, when FMS complicates a rare pathology called Cogan's syndrome. The association of two drugs might noticeably affect the molecular mechanisms of difficult pain, thus solving painful conditions of multifactorial origin., (© 2013 The Authors Pain Practice © 2012 World Institute of Pain.)

Cogan's syndrome is defined as a chronic inflammatory disease of unknown origin, an autoimmune disease, characterized by bilateral sensorineural hearing loss, vestibular symptoms, inflammatory ocular manifestations with variable risk of developing into a systemic disease. The onset of disease is variable but is often characterized by isolated ocular symptoms or acute ear and/or vestibular manifestations, variably associated. The diagnosis of Cogan's syndrome can be a challenge as is evident in the case described here since it is based only on the association between bilateral ocular and vestibuloauditory symptoms with no specific diagnostic tests available.