Nonsyphilitic Interstitial Keratitis Research Articles

Tocilizumab Successfully Treating Refractory Hearing Impairment in a Patient With Cogan Syndrome: A Case Report and Review of the Literature.

Cogan syndrome (CS) is a rare systemic vasculitis characterized primarily by nonsyphilitic interstitial keratitis and vestibular and auditory dysfunction. In this article, we report the case of a 31-year-old male diagnosed with CS for 1 year. He was admitted to the hospital with fever, dizziness, headache, tinnitus, and hearing loss. After being treated with glucocorticoids, cellular immunosuppressants, and infliximab therapy, his symptoms were greatly relieved except for hearing loss. Then, he attempted to use tocilizumab (TCZ) which was ultimately effective in controlling the auditory dysfunction. In addition, we found 4 cases of TCZ for CS through a literature review and compared them with our patient. Although glucocorticoids are still the first-line treatment for CS, TCZ therapy provides fresh hope for patients who have refractory hearing impairment with hormone resistance, or whose hormone dosages cannot be lowered to maintenance levels.

Cogan’s syndrome is more than just keratitis: a case-based literature review

BackgroundCogan'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 presentationThis 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.ConclusionsCS should be involved in the differential diagnosis of keratitis. Early identification and intervention of this autoimmune disease can minimize disability and irreversible damage.

P158 A case of refractory paediatric Cogan’s syndrome treated with tocilizumab

Background/AimsCogan’s syndrome is a rare vasculitis, characterised by progressive sensorineural bilateral hearing loss, vestibular symptoms and non-syphilitic interstitial keratitis. We present a paediatric case which was refractory to initial treatment and subsequently successfully treated with Tocilizumab.MethodsCase report.ResultsA previously well 14-year-old boy presented with sudden onset hearing loss, tinnitus and vertigo. There was no history of new medication, trauma or tick bite. There was no family history of autoimmune disease or genetic hearing losses. On examination his cranial nerves except for vestibulocochlear were grossly intact. He had a steady gait with no cerebellar signs. Otoscopy was unremarkable. Audiology showed a moderate to severe sensorineural hearing loss bilaterally, worse on the right. He had normal type A tympanograms. An autoimmune screen was carried out which showed normal FBC, inflammatory markers, ACE and Chitotriosidase. ANCA, Rheumatoid factor and lyme serology were negative. MRI of the internal auditory meati was normal. He subsequently developed visual disturbance and was diagnosed with bilateral interstitial keratitis. A unifying diagnosis of Cogan’s syndrome was made. An MRI scan of his head, neck and upper thorax looking for evidence of large vessel vasculitis was normal. Genetics looking for evidence of a monogenic autoinflammatory disorder and primary immunodeficiency were negative. He was initially treated with a weaning course of oral prednisolone with improvement in symptoms. Unfortunately, 4 months later had clinical and audiological deterioration in hearing. He therefore received pulsed IV methylprednisolone and commenced subcutaneous methotrexate and adalimumab. 3 months later, there was both clinical and audiological improvement and he started to wean prednisolone. 7 months later, he presented with a 24-hour history of reduced hearing on the right, confirmed on audiogram. He received a further pulse of IV methylprednisolone and a short course of high-dose oral prednisolone, followed by a slowly weaning course. 3 months later, again he felt his hearing had deteriorated and this was confirmed on audiogram, with a 40-decibel loss in his previously good ear. He received two doses of IV methylprednisolone and background steroids were increased to 20mg daily. Due to frequent relapses, adalimumab was changed to IV tocilizumab at 10mg/kg 2-weekly, alongside methotrexate. IV tocilizumab was changed to the subcutaneous route during the COVID-19 pandemic and was tolerated well. His hearing subsequently improved and tocilizumab interval was extended to 3-weekly in Feb 2021. At last review he was stable and successfully transitioned to adult services.ConclusionEvidence regarding treatment options in paediatric patients is lacking due to the rarity of the condition and consequent difficulty in arranging high-quality trials. This is the first case report of use of tocilizumab for Cogan’s syndrome in children, highlighting it as a well-tolerated and successful treatment modality.Disclosure C. Anderson: None. K. McAllister: None. J. Walsh: None.

Atypical Cogan syndrome as adifferential diagnosis of sudden sensorineural hearing loss

CoganI syndrome is arare disease consisting of vestibulocochlear symptoms and non-syphilitic interstitial keratitis. Although this disease was first described in1945, its pathogenesis is still unknown. An autoimmune vasculitis etiology is currently discussed. Atypical manifestations are characterized by delayed ocular symptoms or variability of inflammatory eye symptoms. Physical examination often reveals bilateral sensorineural hearing loss. Intratympanic corticosteroid application can be successful.

P31 Cogan's syndrome—a neglected autoimmune vasculitis

P31 Cogan's syndrome—a neglected autoimmune vasculitis

Syndrome de Cogan

“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.

Atypical Cogan syndrome; case report

Cogan syndrome is a rare disease characterized by the concomitance of non-syphilitic interstitial keratitis with Meniere-like vestibulo-auditory symptoms. There are criteria for the diagnosis of both typical and atypical Cogan syndrome. We present the case of a 40 years old woman with sudden onset of hearing loss, tinnitus, intense vertigo, instability associated with kerato-conjunctivitis. The concomitance of the symptoms, the onset, and the evolution under treatment are consistent with the diagnosis of atypical Cogan syndrome.

Optimal management of Cogan's syndrome: a multidisciplinary approach.

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.

Late-onset Cogan's syndrome associated with large-vessel vasculitis

Cogan's syndrome is a rare autoimmune disease that usually affects young Caucasian adults and is classically defined as the combination of nonsyphilitic interstitial keratitis and audiovestibular symptoms resembling Meniere's disease, both of them developed in an interval of less than two years. Nevertheless, cases with atypical ophthalmologic and audiovestibular features, with systemic manifestations or affecting children and older patients have also been reported, expanding the clinical spectrum of Cogan's syndrome. Herein, we present the case of a late-onset Cogan's syndrome associated with a large-vessel vasculitis.

Cogan's Syndrome: A Retrospective Study of 22 Years

Introduction: Cogan’s syndrome (CS) is a rare clinical entity, of presumptive autoimmune etiology, typically characterized by a non-syphilitic interstitial keratitis (IK) associated with a Meniere-like vestibuloauditory dysfunction. Objective: Since only few cases have been published to current date and considering the variable presentation, clinical course and absence of specific diagnostic tests, we aim to potentially bring further insight for diagnosis and management. Materials and Methods: We conducted a retrospective study of all cases of CS followed at the Department of Otorhinolaryngology, Voice and Communication Disorders in Santa Maria Hospital, Lisbon, from 1992 to 2013. Patient’s demographics, otolaryngologic, ophthalmologic and systemic manifestations, presenting symptoms, audiovestibular and laboratory testing, imaging assessment, performed treatment, disease course and hearing outcomes were analyzed. Results: We review a group of six patients, two with typical and four with atypical CS. Five patients evolved to profound hearing loss, bilateral in two cases. No patient had permanent visual deficit. The Erythrocyte Sedimentation Rate (ESR) increase was the most consistent laboratory finding. Only one patient had positive anti- Heat Shock Protein (HSP) 70 antibodies. All patients were treated with corticosteroids. Methotrexate was associated in three, with some hearing loss stabilization. Three patients underwent cochlear implantation. Discussion and Conclusions: In our series, morbidity was mainly related to the vestibuloauditory dysfunction. In implanted patients, there was a significant improvement of hearing function. We highlight the need of a multidisciplinary approach to this pathology for better therapeutic outcomes and quality of life.

The Importance of FDG-PET/CT in Cogan's Syndrome.

The present study gives a detailed report of a patient with atypical Cogan’s syndrome with uveitis and sensorineural hearing loss. Cogan’s syndrome is characterized by nonsyphilitic interstitial keratitis and audiovestibular dysfunction. This syndrome can be divided into two groups, typical and atypical, based on the presence of interstitial keratitis. It may sometimes be associated with systemic vasculitis. Fluoro-D-glucose-positron emission tomography/computed tomography (FDG-PET/CT) scanning was used to investigate the presence of vasculitis. With FDG-PET/CT scanning, there is no pathological involvement in the walls of the arteries; thus the patient is protected from aggressive and long term immunosuppressive treatment’s side effects. Hence, we can conclude that FDG-PET/CT may play an important role in excluding the presence of vasculitis.

Síndrome de Cogan

Cogan syndrome (CS) is typified by nonsyphilitic interstitial keratitis and Meniere-like auditory involvement. It can present atypically with other ocular and audiovestibular symptoms and associated systemic manifestations. Its name derives from the author who first described the disease. CS affects adults of both sexes, with a mean age of 30years. The prevalence is higher in Caucasians. The pathogenesis of this syndrome is unknown, but is probably the result of an autoimmune mechanism triggered by an infection. The diagnosis is mainly clinical, using the criteria established by Haynes et al. in 1980 for “typical CS” and “atypical CS”. A differential diagnosis should be performed with other systemic diseases that cause similar eye and inner ear manifestations. The course is variable and deafness is a common complication. Prompt treatment and its maintenance are the basis of a favorable outcome.

Sudden Hearing Loss: Could it be Inflammatory Bowel Disease?

Purpose: Cogan's syndrome (CS) is a rare autoimmune disorder characterized by ocular and vestibuloauditory symptoms. We describe a patient initially diagnosed with CS and was later found to have Crohn's disease (CD). Case: A 36-year-old woman with no medical history initially developed acute eye pain and photophobia. After evaluation by ophthalmology, she was treated for iritis with a topical steroid. A week later, she developed unilateral hearing loss. Rheumatology workup revealed negative antinuclear antibody, antinuclear cytoplasmic antibody, rheumatoid factor, hepatitis serologies and HLA-B27. Erythrocyte sedimentation rate and C-reactive protein were normal. Chest x-ray was negative for lymphadenopathy or other lesions. She was diagnosed with CS and refused Methotrexate due to potential side effects. A month later she developed panuveitis despite topical steroid therapy. Adalimumab was given, but she had an injection site reaction which caused her to stop the medication. She then presented to GI clinic with an 8 lb weight loss and new-onset intermittent, lower abdominal pain. She denied nausea, vomiting, diarrhea, constipation, or rectal bleeding. Colonoscopy was performed, which revealed a normal colon but multiple ulcers in the terminal ileum. Pathology showed chronic active ileitis consistent with Crohn's disease. MR enterography revealed mild wall thickening and enhancement involving a 3 cm segment of terminal ileum. The patient was diagnosed with CD five months after her initial symptoms began. Discussion: Less than 250 case reports of CS have been described. The syndrome is defined in two forms: “typical” or “atypical.” Typical CS is characterized by nonsyphilitic interstitial keratitis affecting one or both eyes. A majority of patients develop symptoms in the eye or ear alone, but rapidly involve both within three months. Atypical CS includes other inflammatory complications such as eye involvement other than interstitial keratitis. In contrast to typical CS, there is often a delay of more than two years between ocular and audiovestibular manifestations. Previous case series show an association with inflammatory bowel disease. In this population, CS symptoms often precede inflammatory bowel disease (IBD) diagnosis. On the other hand, auditory impairment is a rare extraintestinal manifestation of IBD. We use this case to stress the importance of evaluating for IBD in a patient with auditory and visual complaints.

Cogan Syndrome

Patients with Cogan syndrome typically present with nonsyphilitic interstitial keratitis and acute onset of sensorineural hearing loss. Neurological manifestations have been reported, but various frequencies and mechanisms have been proposed. We critically reviewed the English literature of Cogan syndrome to determine the nature, frequency, and most likely mechanisms of its neurological manifestations. On the basis of our review, we believe that Cogan syndrome can be associated with neurological manifestations. Our conclusion is based on reported tissue evidence of vasculitis involving the dura, brain, optic nerve, cochleovestibular nerve, and muscle, in patients with referable symptoms. However, we believe that the frequency of neurological manifestations may have been over reported due to lack of confirmatory testing in many of these cases.

Aortic Root Vasculitis Associated With Cogan's Syndrome

Cogan's syndrome is characterized by nonsyphilitic interstitial keratitis and an audiovestibular disorder resembling Meniere disease. We report a patient with progressive congestive heart failure due to massive aortic and mitral insufficiency coupled with aortitis leading to an ascending aortic aneurysm. The patient underwent successful aortic root replacement and mitral valve repair.

Hearing loss, uveomeningitis, and stroke in a 55‐year‐old man

Hearing loss, uveomeningitis, and stroke in a 55‐year‐old man

Atypical Cogan's syndrome: a case report

Cogan's syndrome is a rare presumed autoimmune disorder characterized by nonsyphilitic interstitial keratitis and progressive audiovestibular symptoms. The initial report by David G. Cogan in 1945 was modified by Haynes et al in 1980 who proposed diagnostic criteria for patients with other ocular or vestibular symptoms and suggested this to be atypical Cogan's syndrome. In a more typical presentation of Cogan's syndrome, ocular and audiovestibular signs and symptoms usually appear alone and are bilateral. We report a case of 50-year-old woman with an atypical Cogan's syndrome manifested by unusual relatively rapid clinical deterioration.

Cochlear implant in Cogan’s syndrome

Post-lingual deafness is a stressful condition which is rendered even more painful by the sudden emotional isolation that the patient suffers. Cogan's syndrome is a rare autoimmune cause for post-lingual deafness characterized by non-syphilitic interstitial keratitis, bilateral audio vestibular deficiencies and systemic vasculitis. World over very few cases of Cogan's syndrome have been reported. Cochlear implant surgery in such a patient is a challenging but highly satisfactory experience due to the multitude of clinical problems the patient faces. This demands a proper work up, meticulous surgery and stringent post-operative follow-up. Here we present a patient with atypical Cogan's syndrome, diabetes mellitus and hypothyroidism. She went into a major depression with suicidal tendency following the complete loss of hearing. We performed cochlear implant surgery in this patient, but not before facing several clinical obstacles, helped by a dedicated team consisting of a rheumatologist, endocrinologist, neurophysician, psychiatrist, anaesthetists and audiologist. The results are extremely satisfying for the patient and all the people involved. This case underlines the prime importance of hearing in maintaining the psychological well being of a human being.

Low-dose oral methotrexate for the management of childhood Cogan’s syndrome: a case report

Cogan's syndrome is a rare inflammatory disease characterized by nonsyphilitic ocular interstitial keratitis associated with hearing loss and vestibular impairment. Although systemic corticosteroids are usually used as the standard therapy, hearing ability in most cases gradually deteriorates. We, herein, describe a patient with childhood Cogan's syndrome who was treated with low-dose oral methotrexate, which successfully helped to taper the doses of the systemic corticosteroids. The serum levels of the complements were good markers for the disease activity in this patient.

Side selection for cochlear implantation in a case of Cogan's syndrome

Cogan's syndrome is a rare clinical entity that is characterised by non-syphilitic interstitial keratitis and audiovestibular symptoms. The cause of Cogan's syndrome is considered to be autoimmune disease, which is supported by the resolution of hearing loss after steroid treatment, and the association with other autoimmune diseases. The sensorineural hearing loss of Cogan's syndrome is progressive over a few months, and sudden, bilateral deafness often occurs, which may be an indication for cochlear implantation. This paper presents the case of a young woman suffering from Cogan's syndrome and sudden, bilateral deafness. With reference to this case, we describe problems regarding cochlear implantation for Cogan's syndrome and radiological findings aiding selection of cochlear implantation side.