scholarly journals Autoimmune Inner Ear Disease: Immune Biomarkers, Audiovestibular Aspects, and Therapeutic Modalities of Cogan’s Syndrome

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Oded Shamriz ◽  
Yuval Tal ◽  
Menachem Gross
Keyword(s):  
Immunosuppressive Agents ◽  
Autoimmune Disorder ◽  
Cogan’S Syndrome ◽  
Sentence Recognition ◽  
Therapeutic Modalities ◽  
Immune Biomarkers ◽  
Cogan's Syndrome ◽  
Include Tumor Necrosis Factor ◽  
Inner Ear Disease ◽  
Necrosis Factor

Cogan’s syndrome (CS) is a rare autoimmune disorder characterized by audiovestibular dysfunction and ocular inflammation. Currently, there is no specific serum autoantibody used in the diagnostic workup of CS. Treatment is based on immunosuppressive agents, mainly corticosteroids as first-line choice. Recently, novel therapeutic modalities in CS have emerged. These include tumor necrosis factor-α inhibitors and other biologicals. Despite medical treatment, hearing loss may progress to irreversible bilateral profound SNHL in approximately half of CS patients resulting in candidacy for cochlear implantation (CI). Due to the inflammatory nature of the disease that is causing endosteal reaction with partial obliteration or complete neoossification of the intracochlear ducts, early CI is recommended. CI provides excellent and stable hearing rehabilitation with high score of word and sentence recognition. In this review, we will discuss different aspects of CS including clinical presentation, diagnosis, treatment, and future directives.

scholarly journals Cogan's syndrome: An autoimmune inner ear disease

2013 ◽  
Vol 12 (3) ◽  
pp. 396-400 ◽  
Author(s):  
A. Greco ◽  
A. Gallo ◽  
M. Fusconi ◽  
G. Magliulo ◽  
R. Turchetta ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease

Use of immunosuppressive agents in the treatment of severe ocular and vascular manifestations of Cogan's syndrome

1990 ◽  
Vol 88 (3) ◽  
pp. 296-301 ◽  
Author(s):  
Nancy B. Allen ◽  
C. Christine Cox ◽  
Mary R. Jacobs ◽  
Rex M. McCallum ◽  
Barton F. Haynes ◽  
...  
Keyword(s):  
Immunosuppressive Agents ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome

Autoimmune reactivity in Cogan's syndrome: A preliminary report

1983 ◽  
Vol 91 (1) ◽  
pp. 24-32 ◽  
Author(s):  
Gordon B. Hughes ◽  
Sam E. Kinney ◽  
Barbara P. Barna ◽  
Robert L. Tomsak ◽  
Leonard H. Calabrese
Keyword(s):  
Inner Ear ◽  
Test Results ◽  
Cogan’S Syndrome ◽  
Therapeutic Implications ◽  
Humoral Immune ◽  
Cogan's Syndrome ◽  
Inner Ear Disease ◽  
Autoimmune Reactivity ◽  
Total Deafness ◽  
Autoimmune Inner Ear Disease

Autoimmune inner ear disease was first described by McCabe in 1979. The diagnosis is supported by cell-mediated immune responses to inner ear membrane antigen stimulation. Cogan's syndrome consists of vestibuloauditory dysfunction, ocular inflammation, and nonreactive serologic tests for syphilis. The ocular disease can be controlled by steroids; unfortunately, some patients ultimately become totally deaf. An autoimmune etiology has long been suspected but only recently confirmed by McCabe. In our study two patients with Cogan's syndrome were studied for autoimmune inner ear disease with the use of both cellular and humoral immune tests. Results were compared to normal control subjects. On the basis of test results, preliminary conclusions were that (1) the vestibuloauditory symptoms of Cogan's syndrome are autoimmune in origin; (2) the autoimmune process is mediated through cellular rather than humoral (antibody) pathways; (3) systemic steroids may suppress positive test results; and (4) test results are more likely to be positive when symptoms are acute. Therapeutic implications are significant: the addition of cytotoxic drugs to steroids in selected cases may help prevent total deafness that otherwise might be inevitable.

scholarly journals Autoimmune ear disease: clinical and diagnostic relevance in Cogan’s sydrome

2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Luigi Maiolino ◽  
Salvatore Cocuzza ◽  
Angelo Conti ◽  
Luisa Licciardello ◽  
Agostino Serra ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Systemic Vasculitis ◽  
Clinical Syndrome ◽  
Inflammatory Disorder ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease

The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated to rapidly progressive hearing loss that, especially at the early stages of disease, may be at monoaural localization, although more often it is at binaural localization. It usually occurs as a sudden deafness, or a rapidly progressive sensorineural hearing loss. In this study a particular form of autoimmune inner ear disease is described, Cogan’s syndrome. Cogan’s syndrome is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis, vestibular and auditory dysfunction. Associations between Cogan’s syndrome and systemic vasculitis, as well as aortitis, also exist. We report a case of a young woman who presented audiological and systemic characteristics attributable to Cogan’s syndrome. In the description of the case we illustrate how the appearance and evolution of the disease presented.

scholarly journals Case Report: Effective and Safe Treatment With Certolizumab Pegol in Pregnant Patients With Cogan’s Syndrome: A Report of Three Pregnancies in Two Patients

2021 ◽  
Vol 11 ◽  
Author(s):  
Nils Venhoff ◽  
Jens Thiel ◽  
Markus A. Schramm ◽  
Ilona Jandova ◽  
Reinhard E. Voll ◽  
...  
Keyword(s):  
Inflammatory Diseases ◽  
Treatment Options ◽  
Certolizumab Pegol ◽  
Immunosuppressive Agents ◽  
Immunosuppressive Treatment ◽  
Cogan’S Syndrome ◽  
Safe Treatment ◽  
Tnf Α ◽  
Cogan's Syndrome ◽  
Systemic Glucocorticoids

Cogan’s syndrome is a rare autoimmune disease characterized by ocular inflammation and audiovestibular manifestations. Treatment consists of systemic glucocorticoids and other immunosuppressive agents including methotrexate, cyclophosphamide and TNF-α-inhibitors. Due to potential ovarian or fetal toxicity immunosuppressive treatment options are limited during pregnancies. Thus far there is a paucity of reports on pregnancies in Cogan’s syndrome. With minimal transplacental transfer, Certolizumab pegol is considered to be safe for the use in pregnant patients with underlying inflammatory diseases. However, there is no literature on the use of this TNF-α-inhibitor in Cogan’s syndrome in general and especially during gestation. Here we report three pregnancies in two Cogan’s Syndrome-patients treated with Certolizumab pegol. Treatment with Certolizumab pegol was effective and well tolerated in patients with Cogan’s syndrome and seems to be a safe treatment option during pregnancy.

Cogan’s Syndrome: Atypical Ophthalmic Manifestations

2014 ◽  
Vol 2 (3) ◽  
Author(s):  
MONTEIRO TC ◽  
BIANCARDI AL ◽  
REIS L ◽  
MORAES JR HV
Keyword(s):  
Cogan’S Syndrome ◽  
Ophthalmic Manifestations ◽  
Cogan's Syndrome

scholarly journals Interstitial keratitis in patients with Cogan’s Syndrome

2017 ◽  
Vol 76 (1) ◽  
Author(s):  
Thiago Gonçalves dos Santos Martins ◽  
Ana Luiza Fontes de Azevedo Costa ◽  
Thomaz Gonçalves dos Santos Martins
Keyword(s):  
Cogan’S Syndrome ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis

Atypical Cogan's syndrome: A case report and summary of current treatment options

2015 ◽  
Vol 79 (3) ◽  
pp. 428-431 ◽  
Author(s):  
Debora Jančatová ◽  
Karol Zeleník ◽  
Pavel Komínek ◽  
Petr Matoušek
Keyword(s):  
Case Report ◽  
Treatment Options ◽  
Current Treatment ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome

scholarly journals Maintenance Therapy in Crohn's Disease

2000 ◽  
Vol 14 (suppl c) ◽  
pp. 23C-28C ◽  
Author(s):  
Hillary Steinhart
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Maintenance Therapy ◽  
Immunosuppressive Agents ◽  
Human Antibody ◽  
Chimeric Mouse ◽  
Wide Range ◽  
Systemic Glucocorticoids ◽  
Necrosis Factor

Symptoms of active Crohn’s disease may respond to one or more of a number of classes of drug therapies. These include systemic glucocorticoids, budesonide, sulphasalazine, mesalazine (5-aminosalicylates), immunosuppressive agents and antibiotics. More recently, a chimeric mouse-human antibody to tumour necrosis factor (infliximab) has been shown to induce clinical remission and endoscopic improvement in patients with moderately active Crohn’s disease refractory to other therapies. Despite this wide range of existing therapies and the potential of emerging biological therapies, recurrent Crohn’s disease continues to be a major impediment to the fulfilment of a normal lifestyle and optimal quality of life for patients with Crohn’s disease. Many drugs known to be effective for the treatment of active disease have been tried as maintenance therapy to prevent disease relapse or recurrence following medical or surgical therapy. The available evidence suggests that most of these drugs are not as useful in maintaining remission as they are in inducing it. Systemic glucocorticoids, budesonide, mesalazine (5-aminosalicylates), sulphasalazine and antibiotics are all associated with either marginal therapeutic gain in the setting of maintenance therapy or unacceptable long term toxicity. The immunosuppressive agents azathioprine, 6-mercaptopurine and methotrexate have been shown to have a beneficial effect in maintaining remission and may be helpful as steroid-sparing agents. Repeated infusions of antitumour necrosis factor antibody maintain the improvements observed after one or two initial infusions. The relative long term safety, efficacy and cost effectiveness of the various choices of maintenance therapy remain to be determined.

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