Search

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.

Ci Lu,1,* Panpan Lv,2,* Xiaoying Zhu,1 Yongmei Han1 1Department of Rheumatology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, People’s Republic of China; 2Department of Ultrasound, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, People’s Republic of China*These authors contributed equally to this workCorrespondence: Xiaoying Zhu; Yongmei Han, Department of Rheumatology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, 3# Qingchun East Road, Hangzhou, 310016, People’s Republic of China, Email 3202091@zju.edu.cn; 3408235@zju.edu.cnAbstract: 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.Keywords: Cogan’s syndrome, hypertrophic pachymeningitis, vasculitis

Abstract 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.

Le syndrome de Cogan est rare avec environ 250 cas décrits dans la littérature. Il s'agit d'une maladie supposée auto-immune bien qu'aucun anticorps spécifique n'ait été identifié à ce jour. Elle est classée parmi les vascularites systémiques pouvant toucher les vaisseaux de différents calibres. Le syndrome de Cogan est défini dans sa forme typique par une kératite interstitielle non syphilitique associée à des manifestations audiovestibulaires comparables au syndrome de Ménière. Le délai entre l'apparition des deux symptômes est inférieur à 2 ans. Il est dit atypique lorsque le délai dépasse ces 2 ans et/ou s'il est associé à d'autres manifestations oculaires ou systémiques. Le pronostic visuel est le plus souvent favorable avec une réversibilité des lésions. À l'inverse, l'atteinte audio-vestibulaire peut être sévère et irréversible. Il n'existe à ce jour aucun consensus concernant la prise en charge du syndrome de Cogan. Le traitement repose sur une corticothérapie en association dans certaines formes avec un autre immunosuppresseur (DMARDs, biothérapie). Cogan syndrome is rare with about 250 cases described in the literature. It is a suspected autoimmune disease although no specific antibodies have been identified to date. It is classified as a systemic vasculitis that can affect vessels of various sizes. Cogan's syndrome is typically defined as non-syphilitic interstitial keratitis associated with audio-vestibular manifestations comparable to Meniere's syndrome. The delay between the onset of the two symptoms is less than 2 years. It is said to be atypical when the delay exceeds 2 years and/or if it is associated with other ocular or systemic manifestations. The visual prognosis is usually favourable with reversibility of the lesions. Conversely, the audio-vestibular damage can be severe and irreversible. To date, there is no consensus on the management of Cogan syndrome. The treatment is based on corticosteroid therapy in association in certain forms with another immunosuppressant (DMARDs, biotherapy). [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

ObjectiveAIED (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.MethodsA literature search on articles pertaining to the treatment of autoimmune inner ear disease was performed on PubMed.ResultsCorticosteroid 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.ConclusionThere 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 evidenceexpert opinion.

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. [ABSTRACT FROM AUTHOR]

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.

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.)

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.

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. [ABSTRACT FROM AUTHOR]

A 74-year-old hypertensive male presented with redness of both the eyes, bilateral hearing loss and on and off fever since 1 month. A diagnosis of Cogan's syndrome was made, and treatment with systemic steroids and immune-suppression led to complete reversion of his symptoms, red eyes and hearing loss.

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. [ABSTRACT FROM AUTHOR]

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 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 autoimmune vasculitis that can have significant systemic consequences and progress with progressive and inflammatory eye (interstitial keratitis, scleritis, episcleritis, uveitis) and audiovestibular (sensorineural hearing loss, vertigo, tinnitus) involvement. Vasculitis is observed in approximately 15% of patients with CS and may present as aortitis, large-vessel vasculitis (Takayasu-like), moderate arteritis, or other vasculitic syndromes. In this case report, we present the development of severe aortic and mitral valve insufficiency in a patient diagnosed with CS who was admitted to the hospital with episcleritis and sensorineural hearing loss. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

"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. [ABSTRACT FROM AUTHOR]

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.

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. [ABSTRACT FROM AUTHOR]

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. Summary: As our framework for recognizing the markers of CS and the associated systemic disorders expands, more effective guidelines and treatment options may emerge. [ABSTRACT FROM AUTHOR]

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.

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.

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. [ABSTRACT FROM AUTHOR]

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 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.)

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.

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 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. [ABSTRACT FROM AUTHOR]

Cogan's syndrome is a rare disease from a group of systemic vasculitides with variable vascular involvement. It is a concurrence of ophthalmic symptoms and audiovestibular disorders (Meniere's disease and sensorineural hearing loss). There is often fever, arthralgia or arthritis; neurological disorders and aortitis with the development of aortic aneurysms are encountered. Hearing loss is frequently irreversible. Changes in the organ of vision usually occur favorably.The paper describes a case of Cogan's syndrome in a 33-year-old patient. The disease is manifested by papilledema, conjunctivitis, acute sensorineural deafness with rapid loss of hearing, and microfocal changes in the brain, as detected by computed tomography. Pulse therapy with glucocorticoids and cyclophosphamide led to a rapid and obvious regression of all symptoms. The case is of interest due to the rare occurrence of the disease and to the success of performed therapy. The description stresses the importance of a rheumatologist's consultation and the need for the collective management of young patients with clinical presentations of acute sensorineural hearing loss.

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.

We reported a Jordanian case of Cogan’s Syndrome (CS). A 22-year old male patient presented with interstitial keratitis. The patient was treated successfully with topical steroids but over the following months, he developed vertigo, sensorineural hearing loss (SNHL) and generalized vasculitis. This is the first reported case of CS in Jordanian population.

Cogan's syndrome is a chronic inflammatory disorder of unknown cause affecting mostly young adults. Two main observation of the disease are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between Cogan's syndrome and systemic vasculitis as well as aortitis are exist. The diagnosis of the disease is based upon the presence of inflammatory eye disease and vestibuloauditory dysfunction . In this article, the classic Cogan's syndrome has been reported in a 47-year-old woman. Two months prior to admission, the patient had been suffering from headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss and blindness. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally and on slit lamp examination, there was a conjunctival hyperemia, bilateral cataract and interstitial keratitis. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The patient was initially treated with pulse intravenous methylprednisolone and was followed by oral prednisolone and cyclophosphamide, which in follow-up showed partial improvement.

The article presents a case study of a 42-year-old female with a history of Cogan syndrome, sensorineural hearing loss, and coronary vasculitis presented with three days of right-eye pain and photophobia, then was diagnosed with posterior scleritis and treated with intravenous steroids and transitioned to oral prednisone, infliximab, and methotrexate with a resolution of her ocular symptoms.

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. [ABSTRACT FROM AUTHOR]

Background: Cogan's syndrome is a very rare autoimmune disease characterized by the coexistence of inflammatory lesions in the eyeball and inner ear dysfunction. The symptoms of Cogan's syndrome within the inner ear appear suddenly and resemble Meniere's disease symptoms: severe vertigo, nausea, vomiting, and usually bilateral, fluctuating sensorineural hearing loss with concomitant tinnitus. Case report: A 59-year-old woman was admitted to the Otolaryngology Head and Neck Surgery Clinic to undergo cochlear implantation. At 57 years of age, the patient had been diagnosed with scleritis. About a year later, vertigo and nausea arose, followed by rapidly progressing hearing loss - at first in the left ear and subsequently in the right, accompanied by tinnitus. At the time of implantation, the patient's speech discrimination score was 0% for both ears. Taking into consideration the audiometric tests results and imaging scans, promontorial cochle- ostomy was carried out during the cochlear implantation procedure. In the postoperative period, no complications were observed. After 24 months of using the speech processor, the subjective assessment of speech intelligibility given by the patient on a 0-10 scale was 8.5 in quiet and 5.0 in a noisy environment. The patient could identify 95% of monosyllabic words in silence and 35% in noise (in conditions of SNR +10 dB, speech level at 70 dB HL, and noise level at 60 dB HL). The benefits obtained were confirmed by results of an Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire. The patient is presently eligible for cochlear implantation in the other ear. Conclusions: Cochlear implantation was effective in improving hearing in a patient with Cogans syndrome. [ABSTRACT FROM AUTHOR]

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 33 years (range 5–69). In the descriptive analysis adult patients (N = 87) were separated from pediatric patients (< 18 years 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. [ABSTRACT FROM AUTHOR]

Cogan’s Syndrome is a rare autoimmune disease that presents with multi-system involvement including the eyes and ears. It is typically characterized by attacks of vertigo resembling Meniere, sensorineural hearing loss and interstitial keratitis. Ocular findings may vary widely, other than interstitial keratitis in the manner of atypical form which is less frequently seen and has a less favourable prognosis. Here, a 52-year-old male patient with atypical Cogan’s Syndrome is reported. In Cogan’s Syndrome, especially if diagnosis is delayed, hearing diminishes progressively and deafness usually occurs despite therapy, while ocular findings usually respond to topical therapy and persistent blindness does not occur. Persistent sensorineural hearing loss can be prevented by early diagnosis and immediate high dose systemic steroid administration with other immunosuppressive agents if required.

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.)

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. [ABSTRACT FROM AUTHOR]