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At least 19 recordsLinked to original sources
PubMed1978

Neurologic manifestations of Cogan syndrome.

Cogan syndrome is a multisystem inflammatory vascular disease, characterized by nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Recent reports have directed attention to involvement of other organ systems. Respiratory, cardiovascular, gastrointestinal, and musculoskeletal problems are common, as are laboratory abnormalities and general symptoms such as fever, chills, and weight loss. Prominent neurologic problems in two patients prompted a review of 79 cases of Cogan syndrome. More than half had nervous system involvement, including electroencephalographic or spinal fluid abnormality, headache, psychosis, coma, convulsion, neuropathy, and stroke. Cogan syndrome should be considered when neurologic deficits are accompanied by eye, ear, and systemic symptoms.

Adult↗
PubMed1995

[Interstitial keratitis and Cogan syndrome].

Cogan's syndrome consists of non syphilitic interstitial keratitis associated with vestibuloauditory dysfunction. This syndrome is rare and very few cases have been reported since Cogan's first characterization in 1945. We present the case of a 24-year-oldman with a history of recurrent access of arthralgia and glomerulonephritis. Bilateral corneal disease consists of peripheral whitish patchy opacities in deep stroma. The patient also shows bilateral severe deafness, fever, meningeal and tetrapyramidal syndrome. Ocular involvement responds well to corticosteroids therapy but hearing loss is severe and often irreversible.

Adult↗
PubMedDate not supplied

[The Cogan syndrome].

Cogan's Syndrom is the clinical entity of non syphilitic interstitial keratitis with vestibuloauditory symptoms. Although the disease is not rate the pathohistological findings in temporal bones are only reported twice. Our communication deals with observations made on a temporal bone out of O. Mayer's collection. The most striking findings are: the endolymphatic hydrops, the degeneration of the organ of Corti, the pathology of the ductus reuniens, the fibrosis within the cisterna perilymphatica, the destruction of the maculae and the most uncommon ossification within the scala vestibuli of the basal turn. The vessels seem to show signs of angitis.

Acoustic Maculae↗
PubMed2003

[Atypical Cogan syndrome].

Atypical Cogan syndrome is a systemic vascular disease close to Cogan syndrome. It is characterized by different ocular manifestations associated with Ménière-like audiovestibulatory dysfunction with symptoms arising more than 2 years before or after the ocular abnormalities. We report the case of a 63-year-old woman whose ocular manifestations involved alternating scleritis, myositis and uveitis associated with audiovestibulatory signs, and the onset of ocular muscle and nerve palsies. In the light of this observation, we discuss the clinical characteristics, diagnostic means, differential diagnoses and therapeutic modalities of this syndrome.

Deafness↗
PubMed2001

The cerebral angiographic findings in Cogan syndrome.

SUMMARY: Cogan syndrome is an uncommon disorder of unknown etiology characterized by vestibuloauditory dysfunction and nonsyphilitic interstitial keratitis. To our knowledge, the case herein is the first report to demonstrate the cerebral angiographic findings of a patient with this syndrome.

Cerebral Angiography↗
PubMed1999

An overlap syndrome with features of atypical Cogan syndrome and Wegener's granulomatosis.

A 48 year old women developed serous otitis, scleritis, myalgia, vertigo, polyneuropathy, crescentic glomerulonephritis, general cerebral dysrythmia, hilar adenopathy, and retroorbital granulomatous inflammation. Pulmonary manifestations were absent and antibodies against neutrophilic cytoplasmic antigens (ANCA) could not be detected. The clinical picture was classified as an overlap syndrome with features of both atypical Cogan syndrome and Wegener's granulomatosis. The patient responded to treatment with high dose corticosteroids and pulse cyclophosphamide.

Cyclophosphamide↗
PubMed2002

Cogan syndrome.

PURPOSE: To lead ophthalmologists to consider Cogan syndrome when managing a patient presenting with keratitis or other ocular inflammation accompanied by sensorineural hearing loss. METHODS: Seven patients affected by Cogan syndrome were studied: two males and five females, ranging from 27 to 65 years of age (mean age: 41 years). Subjects were evaluated for a period ranging from 22 to 46 months (mean follow up time: 29.2 months). All patients were treated with immunosuppressive drug combination therapy (IDCT). RESULTS: Three patients were affected by classic Cogan syndrome (i.e., vestibuloauditory symptoms and later sensorineural hearing loss and interstitial keratitis). Four patients presented atypical Cogan syndrome (i.e., sensorineural hearing loss and chronic ocular inflammation such as uveitis, scleritis, conjunctivitis, retinal vasculitis, etc.). Four of these patients had a late diagnosis. Two of them were diagnosed when they already had a cochlear implant, one with bilateral deafness underwent cochlear implantation 1 year after the beginning of IDCT, one had severe bilateral hearing loss that improved during the first year of IDCT, and then rapidly worsened to total deafness in 1 month following an episode of severe systemic hypotension. Three patients who had an early diagnosis of Cogan syndrome had no worsening of vestibuloauditory dysfunction during the follow up period. CONCLUSION: Diagnosis of Cogan syndrome should not be overlooked by ophthalmologists in all patients with recurrent ocular inflammatory disease associated with vestibuloauditory symptoms. Early diagnosis is essential to commence the appropriate immunosuppressive therapy that may prevent permanent hearing loss and ocular dysfunction.

Adult↗
PubMedDate not supplied

Cogan syndrome: autoimmune-mediated audiovestibular symptoms and ocular inflammation.

BACKGROUND: Cogan syndrome is an uncommon disorder that presents with symptoms involving the eyes and ears. At its onset, it can mimic many common entities. Family physicians should be aware of Cogan syndrome because it can be successfully treated if it is recognized early in its course. It is one of the few treatable causes of deafness. METHODS: MEDLINE files were searched from 1982 to the present for "Cogan's syndrome." Additional references were obtained by cross-referencing bibliographies from available articles. RESULTS AND CONCLUSIONS: As first defined in 1945, Cogan syndrome includes nonsyphilitic interstitial keratitis and attacks of vertigo, tinnitus, and hearing loss. Although it usually begins with only one symptom, most patients have both auditory and ocular findings within 1 year of the onset. If untreated, most patients become deaf within 36 months. Blindness occurs in about 5 percent of patients, but ocular symptoms relapse during a period of years. The disease eventually involves other organs with clinical and pathologic findings that suggest vasculitis. Aortic insufficiency, the most serious complication, develops in 15 percent of patients. The cause of Cogan syndrome remains unknown, but several studies suggest an autoimmune-mediated process. Many reports document an improvement in symptoms with immunosuppressive therapy, particularly if started early in the course of the illness. Family physicians should include Cogan syndrome in their differential diagnosis when a young adult seeks care with audiovestibular symptoms or ocular inflammation.

Adult↗
PubMed2002

[Cogan syndrome or sarcoidosis?].

INTRODUCTION: The Cogan's syndrome is characterized by the association of vestibulo-auditory dysfunction, non syphilitic interstitial keratitis or another significant inflammatory eye lesion. Some authors consider this disease as a vasculitis, because it is frequently associated with systemic manifestations. Based on Cogan's diagnostic criteria, Cogan's syndrome may be part of other systemic diseases, as polyarteritis nodosa or Wegener's granulomatosis. EXEGESIS: We report the case of a patient who presented with a Cogan's syndrome and developed further sarcoidosis. CONCLUSION: If Cogan's syndrome is characterized as systemic disease because of its association with aortitis or other vasculitis, on the other hand, clinical presentation may be part of many other systemic diseases.

Diagnosis, Differential↗
PubMed2006

Cogan syndrome: a retrospective review of 60 patients throughout a half century.

OBJECTIVE: To evaluate the disease manifestations and clinical course of patients affected by Cogan syndrome (a syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms) at a single institution during roughly a half century. PATIENTS AND METHODS: Medical records of all patients diagnosed as having Cogan syndrome at the Mayo Clinic in Rochester, Minn, and who were followed up from 1940 to 2002 were comprehensively reviewed. Otolaryngologic, ophthalmologic, and systemic manifestations of disease were analyzed. Analysis included patient demographics, presenting manifestations, delayed manifestations, laboratory testing, physical examination features, therapeutic interventions, disease course, and hearing and vision outcomes. RESULTS: Sixty patients were identified as having Cogan syndrome, with follow-up from 1940 to 2002. Most patients presented initially with vestibuloauditory symptoms, most commonly sudden hearing loss (50%). The most common inflammatory ophthalmologic condition noted was bilateral interstitial keratitis. Headache (40%), fever (27%), and arthralgia (35%) were the most frequently encountered systemic manifestations. Evidence of aortitis was found in 12% of patients. Complete hearing loss was eventually noted in 52% of affected patients, whereas permanent loss of any degree of vision was uncommon. Cochlear implantation outcomes were uniformly good. Death directly or indirectly attributed to the effects of Cogan syndrome was noted in 4 patients. CONCLUSIONS: The major disease-related morbidities were due to vestibuloauditory disease and only infrequently due to systemic manifestations such as vasculitis, with or without aortitis. Cochlear implantation has been of major benefit in modern hearing rehabilitation for this patient population. We advise caution before institution of protracted courses of high-dose corticosteroids and/or chemotherapy for patients without pronounced systemic disease or severe eye disease unmanageable by topical or periocular corticosteroids alone.

Adolescent↗
PubMed2003

Cochlear implantation and Cogan syndrome.

OBJECTIVE: To evaluate outcomes and issues pertaining to cochlear implantation in a group of subjects affected by Cogan syndrome. STUDY DESIGN: Prospective cohort. SETTING: Department of Ophthalmology and Otorhinolaryngology, University of Parma. PATIENTS: Five postlingually deafened adults suffering from a typical form of Cogan syndrome who underwent cochlear implantation. MAIN OUTCOME MEASURES: Benefit from cochlear implantation as measured by word and everyday sentence recognition tests. Surgical issues and postoperative complications were also evaluated. RESULTS: In two cases, intracochlear electrodes were inserted into the scala vestibuli because of the ossification of the scala tympani. Two patients experienced a recurrence of keratitis the day after surgery. To date, with a follow-up of 1 to 4 years, no patient has experienced flap complications or other local or systemic complications. At the 12-month postoperative evaluation, all patients had gained useful open-set speech perception, achieving a mean score of 91% and 95% on word and everyday sentence recognition tests, respectively. CONCLUSIONS: Patients deafened by Cogan syndrome demonstrated high levels of speech understanding after undergoing cochlear implantation. Obliteration of the cochlea may complicate electrode implantation, requiring modifications of the surgical technique. Stress consequent to the surgical procedure may instigate an acute phase of the basic illness.

Adult↗
PubMed2004

Cogan syndrome in children: early diagnosis and treatment is critical to prognosis.

PURPOSE: To present two cases of pediatric Cogan Syndrome and to highlight the differences between the adult and pediatric forms of the disease, as well as the importance of early diagnosis and treatment. DESIGN: Interventional case report. METHODS: Institutional setting. RESULTS: Corneal lesions were much more diffuse than those observed in adult Cogan syndrome. Immunosuppressive drug combination therapy successfully resolved systemic and ocular inflammation, but the involvement of the pupillary area caused permanent low vision in one case and amblyopia in the other. CONCLUSION: When chronic ocular inflammation is observed in association with sensory neural hearing loss and any systemic signs of autoimmune inflammation, a diagnosis of Cogan syndrome should be suspected. If immunosuppressive treatment is not initiated as soon as possible, permanent low vision and deafness can result.

Amblyopia↗
PubMed1994

MR of the inner ear in patients with Cogan syndrome.

PURPOSE: To determine whether the bony and soft-tissue obliterations of the intralabyrinthine fluid spaces reported in pathologic studies of patients with Cogan syndrome can be detected with MR or CT. METHODS: The inner ears of six patients with Cogan syndrome were studied. High-resolution CT was performed in five patients; all six patients were studied with MR, including T1-weighted spin-echo images with and without gadolinium administration, T2-weighted spin-echo images, and three-dimensional Fourier transform constructive interference in steady state images. RESULTS: In two patients, small calcified obliterations were detected on CT but the three-dimensional Fourier transform constructive interference in steady state images revealed more extensive soft-tissue obliteration in five of the six patients. High signal inside the membranous labyrinth on precontrast T1-weighted images and contrast enhancement inside the membranous labyrinth on the postcontrast T1-weighted images were seen in one patient. CONCLUSIONS: The study showed that calcific obliteration and soft-tissue obliteration of the intralabyrinthine fluid spaces in patients with Cogan syndrome can be demonstrated radiologically and that soft-tissue obliteration is more frequent than calcified obliteration. MR detected the intralabyrinthine disease far more frequently than did CT. The three-dimensional Fourier transform constructive interference in steady state sequence proved to be the most sensitive MR sequence. Hyperintensity inside the membranous labyrinth on the precontrast T1-weighted images and enhancement on the contrast-enhanced T1 images were less frequent and probably represent leakage through the abnormal labyrinthine membrane from active disease.

Adult↗
PubMed2000

Effective treatment with topical cyclosporin A of a patient with Cogan syndrome.

The purpose of this report is to describe the effective treatment of severe anterior segment inflammation due to Cogan syndrome through the use of topical administration of cyclosporin A. A 47-year-old female patient had been experiencing headaches and difficulties with her vision. Subsequent examination revealed the sudden onset of bilateral conjunctival injection and swelling of bilateral auricles. Despite the multiple treatment (systemic and topical corticosteroid and antibiotic therapy), necrotizing scleritis had appeared bilaterally and the scleral wall was thinning. Topical administration of 1% cyclosporin A was applied to both eyes 4 times a day. After 2 months of this therapy, the epithelial tissue covered the necrotizing tissue and her symptom of ocular pain was relieved and her corrected visual acuity was improved. This is the first case exhibiting that topical cyclosporin A is an effective treatment for severe anterior segment inflammation associated with Cogan syndrome.

Administration, Topical↗
PubMed2006

Recurrent aortic aneurysms following thoracic aortic stent-graft repair in a patient with Cogan syndrome.

PURPOSE: To report the need for multiple surgical interventions to treat recurrent aortic aneurysms in a patient with Cogan syndrome. CASE REPORT: A 17-year-old Chinese man with clinical Marfanoid features had a left common carotid artery pseudoaneurysm electively repaired with an autologous saphenous vein graft. Four months later, he presented with acute chest pain. Computed tomography (CT) revealed a 1-cm pseudoaneurysm at the mid descending aorta; a 24 x 100-mm Talent stent-graft was implanted to exclude the pseudoaneurysm. He was also found to have increasing left-sided hearing loss. A month later, the patient was re-admitted with vertigo and keratitis, which were treated appropriately. Nine months following stent-graft insertion, he was admitted with acute hemoptysis. Urgent CT showed a rupture at the proximal end of the stent-graft, with hemorrhage into the lung parenchyma. In an emergent procedure, the stent-graft was removed, and the descending thoracic aorta was repaired. Intraoperatively, a large pseudoaneurysm was found arising from the proximal part of the stented aorta, which appeared thickened. His postoperative recovery was uneventful. Nine months after the thoracotomy, a routine CT revealed an aneurysm at the distal descending thoracic aorta. On re-thoracotomy, a de novo saccular aneurysm was found 2.5 cm from the distal anastomosis. The affected segment was replaced with a Dacron graft. The distal aorta appeared thickened and edematous; histology confirmed aortitis. The patient was subsequently diagnosed with Cogan syndrome and given corticosteroids and methotrexate. There is no evidence of recurrence at nearly 2 years after the last intervention. CONCLUSION: This case highlights the pitfalls of stent-graft repair in a patient with presumed connective tissue disease.

Aneurysm, False↗
PubMed1994

Posterior scleritis--an unusual manifestation of Cogan's syndrome.

Cogan's syndrome is characterized by a non-luetic interstitial keratitis associated with vertigo, tinnitus and profound deafness. Evidence of a systemic vasculitis is found in up to 50% of patients. Atypical forms of Cogan's syndrome have been described in which the ocular inflammatory disease may be more severe. We describe a case of atypical Cogan's syndrome in association with bilateral posterior scleritis. Serial B-scan ultrasound measurements of posterior scleral thickness were found to be useful in assessing disease activity, in combination with clinical findings. Combination therapy with prednisolone and cyclosporin controlled the ocular disease but the deafness was irreversible. The length of follow-up of this case highlights the frequent relapses and difficult management problems which may be faced. This multisystem disease requires the close co-operation of ophthalmologist, physician and otorhinolaryngologist. Aggressive therapeutic intervention with high-dose combined immunosuppressive agents may be necessary to control severe ocular inflammatory disease.

Adult↗
PubMed2002

Cochleovestibular impairment in pediatric Cogan's syndrome.

Cogan's syndrome is a rare, chronic inflammatory disorder that typically targets the eyes and vestibuloauditory apparatus, but it may also involve other organs. Three pediatric cases of Cogan's syndrome (ages 5, 13, and 18 years) are reported with long-term follow-up and complete and regular cochleovestibular functional evaluation and ophthalmologic and neurologic examinations. One case was a typical form (characterized by an interstitial keratitis and cochleovestibular impairment), whereas the other 2 cases were atypical forms with uveitis and polyarthritis. In all 3 cases, the first clinical sign was nonspecific eye redness misdiagnosed as a banal conjunctivitis, initially or secondarily associated with bilateral endocochlear sensorineural hearing loss and complete bilateral peripheral vestibular deficit. During the acute phase, early steroid treatment (prednisone, 1 mg/kg/day) was effective in treating the ocular lesions (3 of 3 cases) and improving hearing (2 of 3 cases) but less effective for the vestibular loss (2 of 3 cases). Adverse effects and dependence on the steroid occurred in 2 cases, and immunosuppressive drugs were necessary to avoid recurrences in 1 case. Over the long-term, the disease was controlled in 2 cases but continued to progress in the other. Cogan's syndrome in childhood should be suspected in cases of conjunctivitis associated with inner-ear symptoms; a prompt steroid treatment can avoid progressive impairment of multiple sensorineural functions (vision, balance, hearing). Long-term management involves limiting disease recurrences by adaptive therapies, screening for complications (aortitis in particular), and planning rehabilitation for the sensorial deficits.

Adolescent↗