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Cogan Syndrome

Stromal Keratitis


Presentation

  • It’s typical form presents as non-syphilitic interstitial keratitis associated with audiovestibular dysfunction.[medcraveonline.com]
  • Within 8 months, she presented to ophthalmology with a 3-week history of coloured haloes.[nature.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]
  • Cogan syndrome is an uncommon disorder that presents with symptoms involving the eyes and ears. At its onset, it can mimic many common entities.[ncbi.nlm.nih.gov]
Easy Bruising
  • 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]
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]
  • The most common symptoms include red, painful, light-sensitive or blurred vision ; hearing loss (which may become profound and permanent); vertigo (dizziness); poor balance; nausea and vomiting; fever, fatigue and weight loss.[vasculitis.org.uk]
  • 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]
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]
Skin Atrophy
  • Although the basic illness does not affect the long-term hearing outcome, skin-atrophy-related complications in cases with systemic symptoms may occur. Finally, the cochlear implant surgeon should be aware of the possible cochlea obliteration.[ncbi.nlm.nih.gov]
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]
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]
Unilateral Red Eye
  • We report a 10 year-old male child who presented with fever, acute polyarthritis, and unilateral red eye and was diagnosed as acute rheumatic fever.[ncbi.nlm.nih.gov]
Tinnitus
  • Along with eye inflammation, Cogan’s syndrome can cause dizziness, balance problems, tinnitus (ringing in the ears) and loss of hearing.[lvhn.org]
  • 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]
  • 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]
  • Useful Links Media Center Meniere’s Disease Tinnitus stay connected American Hearing Research Foundation 275 N. York Street, Suite 201 Elmhurst, IL 60126 (630) 617-5079 info@american-hearing.org 2019 American Hearing Research Foundation[american-hearing.org]
Anger
  • , Université Angers, 49000 Angers, France. 12 Service de médecine interne, CHU Nîmes, Nîmes, France. 13 Service de médecine interne, Université Paris 13, AP-HP, Avicenne, 93000 Bobigny, France. 14 Service de médecine interne, CHU Limoges, Limoges, France[ncbi.nlm.nih.gov]
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]
Facial Swelling
  • A 16-year-old boy with traumatic brain injury and seizures presented to the emergency department with facial swelling, rash, and back pain several days after increasing lamotrigine dose secondary to a breakthrough seizure.[ncbi.nlm.nih.gov]
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]
  • 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]
  • 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]
  • A 48 year old women developed serous otitis, scleritis, myalgia, vertigo, polyneuropathy, crescentic glomerulonephritis, general cerebral dysrythmia, hilar adenopathy, and retroorbital granulomatous inflammation.[ncbi.nlm.nih.gov]
  • 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]
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]
  • Along with eye inflammation, Cogan’s syndrome can cause dizziness, balance problems, tinnitus (ringing in the ears) and loss of hearing.[lvhn.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]
  • Vestibular symptoms (39.1%) included vertigo, vomiting, and dizziness. Auditory involvement (65.2%) consisted of sensorineural hearing loss, tinnitus, and deafness. Four patients had cardiac valve involvement, and 3 had skin manifestations.[ncbi.nlm.nih.gov]

Workup

  • 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]

Treatment

  • 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]
  • 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]
  • Management and treatment Treatment of CS traditionally consisted of systemic corticosteroids, with the ocular symptoms being more responsive than the vestibular-auditory symptoms.[orpha.net]
  • Without steroid treatment, it can rapidly progress to complete and definitive SNHL.[pediatrics.aappublications.org]
  • Include: Medical therapy Nonoperative treatment Operative treatment - include links to pages with detailed surgical techniques Indications and contraindications Outcome Include functional and prosthetic survivorship data as applicable Complications Include[orthopaedicsone.com]

Prognosis

  • 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]
  • 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]
  • 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]
  • CONCLUSION: Audiovestibular and ocular involvement have a major impact on prognosis in children with Cogan syndrome. Copyright 2012 Mosby, Inc. All rights reserved.[ncbi.nlm.nih.gov]

Etiology

  • Cogan syndrome is an uncommon disorder of unknown etiology characterized by vestibuloauditory dysfunction and nonsyphilitic interstitial keratitis.[ncbi.nlm.nih.gov]
  • * 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]
  • Cogan syndrome is a rare, multisystem, autoimmune disorder of unknown etiology. Little is known about whether it affects pregnancy or whether pregnancy affects the disease.[ncbi.nlm.nih.gov]
  • The etiology is not well defined.[medcraveonline.com]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]

Prevention

  • Prompt steroid therapy for Cogan's syndrome based on a diagnosis made using PET/CT prevented progression of the hypoacusis.[jstage.jst.go.jp]
  • Adequate preimplant counselling is mandatory, to prevent high expectations and to stress the necessity for bilateral implantation. In 60% of patients with Cogan syndrome, CI remains the only treatment option.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • Early diagnosis is essential to commence the appropriate immunosuppressive therapy that may prevent permanent hearing loss and ocular dysfunction.[ncbi.nlm.nih.gov]

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