Antibodies to inner ear antigens, anti-Hsp70 (associated with sensorineural hearing loss), and antineutrophil cytoplasmic antibodies (associated with vasculitis) have been found in patients with Cogan syndrome. Following this, there may be a slowly progressive course in some patients while others have a course of complete remission with intermittent episodes of disease activity. It is a potent chemoattractant for innate immune cells and promotes the expression of adhesion molecules by endothelial cells.82 TNF inhibition causes a decrease in interferon- and an increase in IL-4 produced by T cells, inducing a shift of the immune response, with a reduction of clinical disease activity. In a pediatric patient with tubulointerstitial nephritis and uveitis (TINU) syndrome, transtympanic dexamethasone injections and IFX infusions were initiated at the onset of dizziness and severe bilateral sensorineural hearing loss, with a diagnosis of atypical CS in addition to the TINU syndrome. Vasculitis of small, medium, and large vessels is considered as the underlying pathogenic mechanism for systemic presentation of CS,8,10 which may involve the cardiovascular, neurological, and gastrointestinal systems.6 The most typical cardiovascular manifestation of CS is aortitis,9,11 leading to kidney and aortic insufficiency, reported in ~10% of patients, and congestive heart failure (Figure 1).3,12 Neurological manifestations may include hemiparesis or hemiplegia following cerebral vascular accidents, and aphasia, due to transient ischemic events.13,14 Gastrointestinal manifestations, such as diarrhea, melena, and abdominal pain, have also been reported following a possible involvement of the mesenteric arteritis.15 Inflammatory bowel disease such as ulcerative colitis and Crohns disease are also associated with CS.16,17. If there is no improvement, if the inflammation is very deep, if the ear is affected, or if blood vessel inflammation (vasculitis Overview of Vasculitis Vasculitic disorders are caused by inflammation of the blood vessels (vasculitis). IFX is a chimeric monoclonal IgG1 antibody that specifically binds to both soluble and membrane-bound TNF with high affinity, forming stable nondisassociating immune complexes. When the eyes are examined by an ophthalmologist, swelling of specific tissues of the eye (interstitial keratitis) may be identified. Treatment for GER & GERD in infants. Peripheral ulcerative keratitis A condition that progresses to severe inflammation of the middle layers of the cornea (interstitial keratitis), Inner ear problems These symptoms resemble Menieres disease (e.g., vertigo, nausea and tinnitus) and are present within two years of eye symptoms, Hearing loss This continues to get worse within one to three months of onset, Subconjunctival hemorrhage A popped blood vessel under the clear tissue covering the front part of the eye, Episcleritis Inflammation of the clear layer of tissue lying above the white of the eye, Scleritis Inflammation of the sclera (the white of the eye), Uveitis Inflammation of the uvea (the middle layer of the eye), Optic disc edema Swelling of the optic nerve inside of the eyeball, Central vein occlusion (CRVO) A condition that affects the retina, Vasculitis optic neuropathy Inflammation of the blood vessels within the optic nerve, Retinal vasculitis Inflammation in the eye that affects the retinal vessels, Angle closure glaucoma When the drainage canals of the eye become blocked, Papillitis, or optic neuritis Inflammation of the nerve that sends visual information from the retina to the brain. Autoimmune sensorineural hearing loss as presenting manifestation of paediatric Behet disease responding to adalimumab: a case report. 2006;81 (4): 483-8. Only 12 cases of CS are reported in the literature, treated with intravenous infusion of IFX, previously treated with corticosteroid and other immunosuppressive drugs with poor benefits.43,62,70,82,85 The used dosage ranged between 3 mg/kg and 400 mg total. The syndrome was first described by Dr. David G Cogan in 1945 as a syndrome exhibiting non-syphilitic interstitial keratitis and vestibulo-auditory symptoms, hence the given name. It is characterised by ocular and audiovestibular symptoms similar to those of Meniere's syndrome. Acute Limb Ischemia in Cogan Syndrome - PMC It is important to contact your doctor as soon as possible if you think you are ill. Cogans syndrome is a chronic disease, and it typically progresses from three days to three months. Cogan Syndrome - Eye Disorders - MSD Manual Professional Edition Mohan N, Edwards ET, Cupps TR, et al. March 2023. Healthline has strict sourcing guidelines and relies on peer-reviewed studies, academic research institutions, and medical associations. However, it is very sensitive to touch. sharing sensitive information, make sure youre on a federal Lydon EJ, Barisoni L, Belmont HM. This makes early diagnosis and treatment extremely important. In 72 per cent of the affected patients there was an underlying systemic process, often a vasculitis. These results suggest that cochlear implantation may provide excellent and stable hearing rehabilitation in long-term follow-up in most patients with CS. Please enable it to take advantage of the complete set of features! Other symptoms may include joint or muscle pain or inflammation of the blood vessels. Ringing in ears. Treatment options generally include corticosteroids and immunosuppressive agents. o [ pediatric abdominal pain ] Vogt Koyanagi Harada syndrome. 2022 Nov 29;17(4):551-573. doi: 10.18502/jovr.v17i4.12321. Cochlear implantation in patients with autoimmune inner ear disease including cogan syndrome: a comparison with age- and sex-matched controls. Other systemic symptoms include: Headache After passive transfer of antibodies directed against the Cogan peptide into Balb/c mice, antibodies were found within the cochlea of the tested animals, whereas antibodies against an irrelevant peptide did not bind to cochlear cells,29 six of six Balb/c mice injected with the purified antibodies developed hearing loss found via the recording of auditory brainstem responses in mice, showing that an higher stimulus intensity was necessary to obtain smaller and more delayed responses after immunization compared to pretreatment. Autoantibodies to inner ear and endothelial antigens in Cogans syndrome. Using well-developed assays, antibodies to inner ear antigens, anti-Hsp70, and antineutrophil cytoplasmic antibodies were found to be associated with CS. The course of the disease varies significantly from patient to patient. Cogan's syndrome is thought to be an autoimmune disease of uncertain etiology which causes inflammation of the eye, most typically interstitial kcratitis, and hearing loss. Hemiplegia/hemiparesis. Diminished hearing with gradual hearing loss. However, in contrast to anti-TNF antibodies, it is less effective in inflammatory bowel disease and in autoimmunity of the eye (eg, uveitis).82 The efficacy of the ET was examined in a prospective study including patients with bilateral immune-mediated audio-vestibular disorders; of these, three patients with CS were present.87 In two patients, an improvement was assessed, although ET did not succeed in preventing hearing loss. Cogan syndrome is a rare autoimmune syndrome affecting mainly the ocular and vestibuloauditory systems. Central gain control in tinnitus and hyperacusis. Cogan syndrome 1Department of Sense Organs, Sapienza University of Rome, Rome, Italy, 2Department of Oral and Maxillofacial Sciences, Sapienza University of Rome, Rome, Italy. ET is approved for the treatment of several autoimmune diseases, such as rheumatoid arthritis, ankylosing spondylitis, juvenile rheumatoid arthritis, psoriasis, and psoriatic arthritis. Corticosteroids at high dosages remain the mainstay at the beginning and during the acute phases of the disease.52, Though reported to be the standard of care and of promising outcome, there are no double blind controlled studies to show the beneficial effects of corticosteroids in CS management and treatment.6,7,55 A clinical response to steroids may also help a challenging diagnosis as an ex juvantibus criterion. GARD Rare Disease Information - Cogan's syndrome - National An official website of the United States government. Our experts continually monitor the health and wellness space, and we update our articles when new information becomes available. Cochlear implantation is a valuable rescue surgical strategy in cases with severe sensorineural hearing loss unresponsive to intensive and innovative immunosuppressive regimens. Cogan syndrome affects young adults, with 80% of patients between 14 and 47 years. Therefore, if you are prescribed these medications, it is important to wash your hands regularly, maintain good hygiene, and try to avoid people with colds or other contagious illnesses. Van Doornum S, McColl G, Walter M, Jennens I, Bhathal P, Wicks IP. Ocular involvement includes any combination of the following: Bilateral interstitial keratitis Interstitial Keratitis Interstitial keratitis is chronic, nonulcerative inflammation of the mid-stroma (the middle layers of the cornea) that is sometimes associated with uveitis. Occasionally, if the disease has damaged blood vessels, surgery may need to be done to correct the problem. Weyn T, Haine S, Conraads V. Cogans syndrome with left main coronary artery occlusion. The physician carefully examines all the external symptoms displayed by the patient and records when each sign began to manifest in the body. Autschbach F, Palou E, Mechtersheimer G, et al. Centers for Disease Control and Prevention. The diagnosis of CS is mainly clinical and is based on audio-vestibular symptoms, ocular inflammation, and nonreactive serological tests for syphilis.32 Diagnosis requires a multidisciplinary approach, as also reported in other similar autoimmune conditions involving the auditory system.3335 The variable onset of symptoms and the lack of specific laboratory tests contribute to challenges in making CS diagnosis that is often based on the good response to corticosteroid treatment.9,36 Clinical diagnostic criteria of CS include mandatory, prevalent, and possible additional criteria. Dystonia is a name for writhing and twisting motions due to uncontrollable muscle contractions. Bovo R, Ciorba A, Trevisi P, et al. Vaiopoulos G, Sfikakis PP, Skoumas B, Kavouklis E, Doukas E, Kaklamanis P. Lack of response to corticosteroids and pulse cyclophosphamide therapy in Cogans syndrome. It was initially described by David Glendenning Cogan(1908-1993),American ophthalmologist,in 19455,9. HHS Vulnerability Disclosure, Help Most of the patients maintain normal or near-normal vision, and only few develop some degree of visual loss directly attributable to inflammatory eye disease.9, The pathogenetic basis of CS is still unclear.