It’s typical form presents as non-syphilitic interstitial keratitis associated with audiovestibular dysfunction. [medcraveonline.com]

It most frequently presents in young adults in their late 20s or early 30s. Typical Cogan syndrome manifests with interstitial keratitis and audiovestibular symptoms similar to Meniere disease (tinnitus, vertigo, and hearing loss). [radiopaedia.org]

Within 8 months, she presented to ophthalmology with a 3-week history of coloured haloes. [nature.com]

Most patients presented initially with vestibuloauditory symptoms, most commonly sudden hearing loss (50%). The most common inflammatory ophthalmologic condition noted was bilateral interstitial keratitis. [ncbi.nlm.nih.gov]

• Recurrent Inflammation

  Cogan syndrome is a rare disorder characterized by recurrent inflammation of the front of the eye (the cornea ) and often fever, fatigue, and weight loss, episodes of vertigo (dizziness), tinnitus (ringing in the ears) and hearing loss. [en.wikipedia.org]

• Nausea

  Usually, within a few hours to a few weeks evidence of eighth nerve involvement appears, manifested by tinnitus, nausea, vomiting, vertigo, and bilateral progressive loss of hearing. [jamanetwork.com]

  The administration of a combined immunosuppressive treatment resolved ocular inflammation, dizziness, nausea, and vertigo but gave little results in controlling progressive hearing loss. [ojrd.biomedcentral.com]

  Otorhinolaryngology, bService of Internal Medicine I, cService of Ophthalmology, dService of Pathology Abstract Typical Cogan's syndrome is a rare disease of young adults consisting of flares of interstitial keratitis and sudden onset of Ménière-like attacks (nausea [pmj.bmj.com]

  The vestibuloauditory manifestations consist of a Ménière’s-like disease including vertigo, nausea, vomiting, tinnitus, and hearing loss. [jrheum.org]

  In the past month she also developed vertigo, nausea, otalgia, diminished appetite, as well as progressively worsening vision. Acetaminophen provided moderate relief from her pain. [webeye.ophth.uiowa.edu]

• Regurgitation

  Cardiac auscultation revealed a murmur of mitral regurgitation. Standard electrocardiography (ECG) was performed, followed shortly by transthoracic echocardiography, which confirms moderate mitral regurgitation ( Figure 3 ). [pubs.sciepub.com]

  Cogan’s syndrome with aortitis, aortic regurgitation, and aortic arch vessel stenoses. Annals of Thoracic Surgery, 52, 1166. CrossRef PubMed Google Scholar Cogan, D. S. (1945). [link.springer.com]

  It may cause proximal aorta dilation, aortic valvular regurgitation, ostial coronary artery disease, and thoracoabdominal aortic aneurysms [11, 14–18]. A coronary arteritis has also been described [3, 19, 20]. [hindawi.com]

• Liver Dysfunction

  Azathioprine was added but subsequently stopped because of liver dysfunction. She declined further immunosuppression, but remained stable and prednisolone was gradually tapered. Figure 1 T2-weighted axial MRI brain scan. [nature.com]

• Musculoskeletal Pain

  The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. [hindawi.com]

  Case Report We report a case of a 12-year-old girl with medical history of vestibular symptoms including vertigo and imbalance associated with nausea, vomiting and musculoskeletal pains, one year ago. [pubs.sciepub.com]

  From the few cases published, it is difficult to determine the presence of systemic disorders, which can include: headache, fever, musculoskeletal pain, splenomegaly, lymphadenopathy and vasculitis affecting the aortic valve, coronary arteries and small [ncbi.nlm.nih.gov]

• Tinnitus

  The audiovestibular symptoms, tinnitus, sensorineural hearing loss and acute episodes of vertigo, are usually bilateral. Cogan's syndrome is a disorder of young adults, the average age of onset being 25 years. [pmj.bmj.com]

  Usually, within a few hours to a few weeks evidence of eighth nerve involvement appears, manifested by tinnitus, nausea, vomiting, vertigo, and bilateral progressive loss of hearing. [jamanetwork.com]

  Shortly following these ocular (eye) symptoms, patients develop bilateral audiovestibular (ear) symptoms, including hearing loss, vertigo (dizziness) and tinnitus (ringing in the ears). [aarda.org]

  She denied any hearing loss, diplopia, fever, cough, rhinorrhea, otorrhea, tinnitus, and vomiting. [webeye.ophth.uiowa.edu]

  Cogan syndrome is a systemic disease manifesting interstitial keratitis, sensorineural hearing loss, tinnitus, and rotatory vertigo. Renal complications of this syndrome are very rare. [ncbi.nlm.nih.gov]

• Progressive Hearing Loss

  The same treatment, however, gave poor results in controlling progressive hearing loss (figure 1 ). [ojrd.biomedcentral.com]

  Autoimmune inner ear diseases (AIEDs) are unusual causes of progressive bilateral sensorineural hearing loss and vestibular defects: three kinds of syndromes have been described so far: progressive bilateral sensorineural hearing loss (which could be [ncbi.nlm.nih.gov]

  Fluctuating hearing loss may occur during the course of the disease, 29, 31 like in case 2. Without steroid treatment, it can rapidly progress to complete and definitive SNHL. [pediatrics.aappublications.org]

• Fluctuating Hearing Loss

  The histopathologic manifestations appear to explain the audiovestibular dysfunction that has been reported in Cogan's syndrome, including bilateral fluctuating hearing loss, tinnitus, and severe vertigo. [ 2, 4 ]. [pubs.sciepub.com]

  However, every attempt to decrease and stop the steroid treatment has been followed by fluctuating hearing loss and conjunctivitis. [pediatrics.aappublications.org]

• Conjunctival Hyperemia

  Slit-lamp examination revealed conjunctival hyperemia and peripheral corneal opacification, with inflammation and crystalline deposits on the corneal stroma, findings that are consistent with interstitial keratitis. [nejm.org]

  Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. [hindawi.com]

• Ciliary Injection

  Examination reveals ciliary injection with mild iritis and discrete fluffy opacities in the deep portion of the corneal stroma. Corneal ulceration does not occur and vascularisation is now unusual. [pubs.sciepub.com]

• Suggestibility

  The existence of a population of deficient naive cytotoxic T cells could suggest a priori a poor response to steroid therapy. [pmj.bmj.com]

  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. [ncbi.nlm.nih.gov]

  Recent work has suggested that high-resolution MRI and antibodies to inner ear antigens may be helpful. Very little has been published in recent years on Cogan's syndrome. [web.archive.org]

  In the other 2 cases vestibular function was more severely impaired, suggesting a lesion of the whole sensorial vestibular apparatus. [pediatrics.aappublications.org]

  Next, they may suggest medications to deal with the inflammation. [healthline.com]

• Vertigo

  At 18, the patient developed rotatory vertigo, a sense of ear fullness, and sensorineural hearing loss. The patient was diagnosed with Cogan syndrome. [ncbi.nlm.nih.gov]

  42-year-old Female with Interstitial Keratitis and Vertigo May 24, 2007, updated Nov 20, 2007 Chief Complaint: Red left eye for the past two months with recent onset of vertigo and otalgia. [webeye.ophth.uiowa.edu]

  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. [ajol.info]

  Vertigo can also be severe. Vasculitis signs and symptoms can be present at presentation or may develop later in the course of the syndrome. [vasculitisfoundation.org]

  The audiovestibular symptoms, tinnitus, sensorineural hearing loss and acute episodes of vertigo, are usually bilateral. Cogan's syndrome is a disorder of young adults, the average age of onset being 25 years. [pmj.bmj.com]

• Dizziness

  Shortly following these ocular (eye) symptoms, patients develop bilateral audiovestibular (ear) symptoms, including hearing loss, vertigo (dizziness) and tinnitus (ringing in the ears). [aarda.org]

  Hain, MD Last update: 10/2002 Cogan's syndrome is defined as nonsyphilitic interstitial keratitis (an inflammation of the eye) and bilateral audiovestibular deficits (hearing problems and dizziness). [web.archive.org]

  Cogan syndrome can lead to vision difficulty, hearing loss and dizziness. [en.wikipedia.org]

Neuroimaging and hypercoagulability workup was unremarkable. Vision and intraocular pressure improved, while uveitis remained recalcitrant. Several months later, the patient developed central serous retinopathy in the right eye (vision 20/30). [ncbi.nlm.nih.gov]

We report a case of a atypical Cogan’s syndrome, the workup of the diagnosis, and treatment results. 2. [pubs.sciepub.com]

Infectious workup for Mycobacterium tuberculosis, Treponema pallidum, Chlamydia, Borrelia burfdorferi, hepatitis B and C viruses, and human immunodeficiency virus was also negative. [reumatologiaclinica.org]

We report a case of a typical Cogan’s syndrome, the workup of the diagnosis, and treatment results. 2. [hindawi.com]

Therefore, a scrutinized physical examination, history taking, and thorough laboratory workups are essential for differentiation of these disorders. [n.neurology.org]

• Chlamydia Trachomatis

  Its etiology includes infection and autoimmunity, Chlamydia psittaci has been isolated from a patient with Cogan's syndrome and serological evidence of a recent Chlamydia trachomatis infection was reported in 4 of 13 patients [ 6 ]. [pubs.sciepub.com]

• HLA-B5

  Additional laboratory tests, including complete blood count, urinalysis, serum electrolytes, creatinine, liver transaminases, angiotensin converting enzyme, HLA-B5, antinuclear antibodies, rheumatoid factor, anti-citrullinated protein antibodies, antiphospholipid [reumatologiaclinica.org]

We report a case of a atypical Cogan’s syndrome, the workup of the diagnosis, and treatment results. 2. [pubs.sciepub.com]

The same treatment, however, gave poor results in controlling progressive hearing loss (figure 1 ). [ojrd.biomedcentral.com]

Without steroid treatment, it can rapidly progress to complete and definitive SNHL. [pediatrics.aappublications.org]

[…] and prognosis Treatment consists of immunosuppression, starting with glucocorticoids and adding other immunosuppressive agents depending on the severity of the condition. [radiopaedia.org]

Treatment There are no clinical trials of treatment in Cogan's Syndrome. Most people with Cogan's Syndrome will need treatment with moderately high doses of prednisolone or other types of steroids. [vasculitis.org.uk]

Immunological tests can help to establish the diagnosis and the prognosis for the recovery of hearing. [pmj.bmj.com]

The prognosis of typical CS is excellent with life-threatening aortic insufficiency (AI) developing in only 10% of reported cases. Serious systemic necrotizing vasculitis (SNV) rarely, if ever, complicates typical CS. [ncbi.nlm.nih.gov]

Atypical CS tends to be more aggressive and has a worse prognosis [1]. [medcraveonline.com]

Prognosis The prognosis is dominated by the risk of definitive deafness and by cardiovascular complications, notably aortic insufficiency. Severe visceral involvement or death related to cardiac or vascular involvement is unusual. [orpha.net]

[…] the intralabyrinthine fluid spaces 2 MRI T1: high signal in the membranous labyrinth of the inner ear T1 C+ (Gd): enhancement of the membranous labyrinth T2: soft tissue obliteration or narrowing of the intralabyrinthine fluid spaces 2 Treatment and prognosis [radiopaedia.org]

* Hearing Loss, Sudden/diagnosis Hearing Loss, Sudden/drug therapy Hearing Loss, Sudden/etiology* Humans Immunosuppression/methods Injections Syndrome Tomography, X-Ray Computed Tympanic Membrane Young Adult Substance Glucocorticoids Supplementary concept [ncbi.nlm.nih.gov]

Etiology Etiology is not known but the mechanism of CS is now considered to be autoimmune and is characterized by the presence of specific anti-Cogan peptide antibodies to inner ear antigens and corneal structures and non-specific auto antibodies (such [orpha.net]

Its etiology includes infection and autoimmunity. Vasculitis is considered the pathological mechanism. We report a case of a atypical Cogan’s syndrome, the workup of the diagnosis, and treatment results. 2. [pubs.sciepub.com]

Introduction Define/describe the condition Anatomy Describe the pertinent anatomy Pathogenesis Describe the biomechanics/biologic basis of the disorder or the mechanism of injury Natural History Describe the natural history, epidemiology and prognosis [orthopaedicsone.com]

Summary Epidemiology CS is a rare disease with several hundred cases reported in the literature since 1945. The disease generally affects Caucasians with no gender specific prevalence. [orpha.net]

Diagnosis: Cogan's Syndrome EPIDEMIOLOGY Predominantly young to middle aged adults Rare SIGNS Interstitial keratitis (limbal stromal opacity or edema, non-inflammed vascular loops in the mid-stroma) Other inflammation of the eye ( episcleritis, scleritis [webeye.ophth.uiowa.edu]

[…] endothelium, resulting in its unusual epithelial-like activity.[1][6] Polymerase Chain Reaction (PCR) testing of corneal endothelial cells from ICE syndrome patients has been found to have high percentages of HSV DNA in comparison to normal controls.[6] Epidemiology [eyewiki.aao.org]

Finally, vigilance and awareness may also offer a better setting to study the pathophysiological mechanisms of this rare but debilitating phenomenon. [zora.uzh.ch]

On the other hand, the application of a study protocol including MRI and a series of immunological tests might facilitate the establishment of the prognosis for the auditory injury, opening new lines of research focusing on the pathophysiological mechanisms [pmj.bmj.com]

Pathophysiology Clinically, the corneal endothelium has been described to have a "hammered silver" or "beaten bronze" appearance in ICE syndrome patients, similar to corneal guttae seen in Fuchs Corneal Endothelial Dystrophy.[1] [2] On a pathological [eyewiki.aao.org]

Early diagnosis is essential to commence the appropriate immunosuppressive therapy that may prevent permanent hearing loss and ocular dysfunction. [ncbi.nlm.nih.gov]

Prompt steroid therapy for Cogan's syndrome based on a diagnosis made using PET/CT prevented progression of the hypoacusis. [jstage.jst.go.jp]

Although CSy is considered to be an autoimmune disease and is associated with IBD, immunomodulatory IBD maintenance treatment and even anti-TNF therapy do not seem to prevent disease onset. [zora.uzh.ch]

There are similar autoimmune diseases such as Menieres, that are more widely recognized and diagnosed, but treatment of Cogan's Syndrome requires an immediate diagnose to prevent the disease from doing permanent damage. [amazon.com]