Symptoma

Cogan Syndrome

Stromal Keratitis

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]

A diastolic heart murmur may be present when aortitis is significant. Claudication may be present if limb vessels are affected. [merckmanuals.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 vestibuloauditory manifestations consist of a Ménière’s-like disease including vertigo, nausea, vomiting, tinnitus, and hearing loss. [jrheum.org]

    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]

    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]

    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]

  • Skin Lesion

    The systemic manifestations of Cogan’s syndrome are fever, headache, myalgia, arthralgia, arthritis, gastrointestinal symptoms and skin lesions such as rash, vasculitis and vitiligo. [medcraveonline.com]

  • Subcutaneous Nodule

    Clinically, vasculitis has been reported to affect the skin, kidneys, subcutaneous nodules, distal coronary arteries, and muscles. [ajnr.org]

  • Tinnitus

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

    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 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]

    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]

    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]

  • 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]

  • Blepharospasm

    THE COGAN syndrome is characterized by sudden onset of photophobia, ocular pain, blurring of vision, and blepharospasm. [jamanetwork.com]

  • Eye Irritation

    It can start with eye inflammation (red and painful eyes irritated by the light), with hearing loss and vertigo, or with both within a one month period of time. [vasculitisfoundation.org]

  • Round Face

    Adverse effects of glucocorticoids are well known and include glucose intolerance, diabetes, easy bruising, increased infections, rounded faces, loss of calcium from bones, central nervous system effects (particularly at high dose), and thinning of skin [vasculitisfoundation.org]

  • 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]

    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 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]

    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]

  • 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]

    Along with eye inflammation, Cogan’s syndrome can cause dizziness, balance problems, tinnitus (ringing in the ears) and loss of hearing. [lvhn.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]

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

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

  • 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]

  • Lymphocytic Infiltrate

    Figure 2 Photomicrography of the conjunctiva section showing mild lymphocytic infiltration of the chorion Figure 1 Axial MRI of the inner ear showing hyperintensity within the membranous labyrinth on precontrast T1-weighted images when compared to the [pmj.bmj.com]

  • HLA-B27

    Blood test results were negative for antinuclear antibody, antinuclear cytoplasmic antibody, rheumatoid factor, rapid plasma reagin, and HLA-B27. The hepatitis serology finding was negative. [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]

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]

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

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

This is the first case exhibiting that topical cyclosporin A is an effective treatment for severe anterior segment inflammation associated with Cogan syndrome. [ncbi.nlm.nih.gov]

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

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]

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

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]

* 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]

The etiology is not well defined. [medcraveonline.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]

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

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

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]