scholarly journals Atypical Cogan's Syndrome

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
João Queirós ◽  
Sofia Maia ◽  
Mariana Seca ◽  
António Friande ◽  
Maria Araújo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Treatment Strategies ◽  
Rare Clinical Entity ◽  
Cogan’S Syndrome ◽  
Peripheral Facial Palsy ◽  
Systemic Corticosteroids ◽  
Cogan's Syndrome ◽  
Biological Tests ◽  
Appropriate Therapy ◽  
Bilateral Deafness

Background. Cogan’s syndrome is a rare clinical entity whose etiopathology is still unknown, and the treatment strategies are not clearly defined.Case. A 23-year-old male presented with symptoms of headache, peripheral facial palsy, persistent right hearing loss and bilateral papillitis. Workup excluded all infectious, granulomatous, neoplastic, and immune causes. The diagnosis of atypical Cogan’s syndrome was established, and the patient was treated with systemic corticosteroids and later on with cyclophosphamide and methotrexate. There were improvement of visual symptoms and stabilisation of left hearing.Conclusion. Cogan’s syndrome is a very rare disease with no specific biological tests for the diagnosis. The diagnostic exams are mostly important to exclude other etiologies. The atypical ocular and audiovestibular manifestations make the diagnosis difficult, delaying the institution of appropriate therapy which may result in profound bilateral deafness.

Side selection for cochlear implantation in a case of Cogan's syndrome

2007 ◽  
Vol 122 (3) ◽  
pp. 310-313 ◽  
Author(s):  
G J Im ◽  
H H Jung
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Steroid Treatment ◽  
Radiological Findings ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis ◽  
Selection For ◽  
Bilateral Deafness ◽  
Selection Of

AbstractCogan's syndrome is a rare clinical entity that is characterised by non-syphilitic interstitial keratitis and audiovestibular symptoms. The cause of Cogan's syndrome is considered to be autoimmune disease, which is supported by the resolution of hearing loss after steroid treatment, and the association with other autoimmune diseases. The sensorineural hearing loss of Cogan's syndrome is progressive over a few months, and sudden, bilateral deafness often occurs, which may be an indication for cochlear implantation. This paper presents the case of a young woman suffering from Cogan's syndrome and sudden, bilateral deafness. With reference to this case, we describe problems regarding cochlear implantation for Cogan's syndrome and radiological findings aiding selection of cochlear implantation side.

scholarly journals Sudden sensorineural hearing loss in atypical Cogan's syndrome: A case report

2018 ◽  
Vol 30 ◽  
pp. 50-53
Author(s):  
José Luis Treviño González ◽  
German A. Soto-Galindo ◽  
Rafael Moreno Sales ◽  
Josefina A. Morales Del Ángel
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome

Late onset Cogan's syndrome

1989 ◽  
Vol 103 (5) ◽  
pp. 512-514 ◽  
Author(s):  
H. Fidler ◽  
N. S. Jones
Keyword(s):  
Young Adults ◽  
Hearing Loss ◽  
Sedimentation Rate ◽  
Late Onset ◽  
Sudden Hearing Loss ◽  
Older Patient ◽  
Cogan’S Syndrome ◽  
Prompt Treatment ◽  
Erythrocyte Sedimentation ◽  
Cogan's Syndrome

AbstractPrevious reports describe Cogan's syndrome occurring in young adults this case shows that it can be diagnosed in an older patient, and that prompt treatment is often successful, making it a worthwhile diagnosis to consider in patients with sudden hearing loss. The erythrocyte sedimentation rate was a useful marker for disease activity in this patient.

scholarly journals Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan’s Syndrome

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Ahad Azami ◽  
Nasrollah Maleki ◽  
Mohammadreza Kalantar Hormozi ◽  
Zahra Tavosi
Keyword(s):  
Hearing Loss ◽  
Systemic Vasculitis ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Conjunctival Hyperemia ◽  
Cogan’S Syndrome ◽  
Auditory Brain Stem Response ◽  
Chronic Inflammatory Disorder ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis

Cogan’s syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan’s syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. 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. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement.

scholarly journals Successful treatment of sudden hearing loss in cogan's syndrome with corticosteroids

1981 ◽  
Vol 24 (3) ◽  
pp. 501-503 ◽  
Author(s):  
Barton F. Haynes ◽  
Anita Pikus ◽  
Muriel Kaiser-Kupfer ◽  
Anthony S. Fauci
Keyword(s):  
Hearing Loss ◽  
Successful Treatment ◽  
Sudden Hearing Loss ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome

scholarly journals Rituximab Not Effective for Hearing Loss in Cogan’s Syndrome

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Daniel R. Bunker ◽  
Leslie Dubin Kerr
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Refractory Disease ◽  
Disease Course ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome

Importance. Rituximab was not effective in ameliorating the hearing loss in a patient with atypical Cogan’s syndrome.Observations. We report the case of a patient who developed acute bilateral uveitis and sensorineural hearing loss. A diagnosis of atypical Cogan’s syndrome was made. The patient’s hearing loss did not improve despite high dose steroids and azathioprine. Rituximab was administered given a recent report of its efficacy in a patient with refractory disease; however, our patient’s hearing loss did not improve.Conclusion. Hearing loss in Cogan’s syndrome is difficult to treat. Though rituximab was ineffective in our case, earlier administration in the disease course could be effective for future patients.

scholarly journals Sensorineural Hearing Loss of Suspected Autoimmune Etiology: Two Cases of Cogan’s Syndrome

2021 ◽  
Vol 64 (12) ◽  
pp. 943-948
Author(s):  
Jungmin Ahn ◽  
Brian Kim ◽  
Kyoung Rai Cho ◽  
Young-Soo Chang
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Immunosuppressive Drugs ◽  
Sensorineural Hearing ◽  
Systemic Steroid ◽  
Ocular Involvement ◽  
Cogan’S Syndrome ◽  
Steroid Administration ◽  
Cogan's Syndrome ◽  
Sparing Effect

Cogan’s syndrome is a rare inflammatory disease characterized by non-syphilitic keratitis and vestibulo-auditory symptoms including hearing loss, tinnitus, and vertigo. Although its precise pathogenesis is not known, Cogan’s syndrome is generally considered an autoimmune disease. This hypothesis is supported by the frequently successful remission of hearing loss after steroid administration and the association with other autoimmune disorders such as rheumatoid arthritis. Medical treatment of Cogan’s syndrome depends on disease severity and on how extensive the disease is. The involvement of inner ear pathology requires systemic corticosteroid therapy. In cases of treatment failure or the need for a corticosteroid-sparing effect, other immunosuppressive drugs can be used. We experienced two patients with typical Cogan’s syndrome, presenting bilateral progressive sensorineural hearing loss and dizziness with ocular involvement, which we have successfully treated with systemic steroid administration and immunosuppressive therapy.

Corneal Autoimmunity in Cogan's Syndrome?

1992 ◽  
Vol 101 (8) ◽  
pp. 679-684 ◽  
Author(s):  
Maarten H. J. M. Majoor ◽  
Frans W. J. Albers ◽  
Frits Gmelig-Meyling ◽  
Ruth van der Gaag ◽  
Egbert H. Huizing
Keyword(s):  
Corticosteroid Therapy ◽  
Cogan’S Syndrome ◽  
Systemic Corticosteroids ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis ◽  
High Doses ◽  
Autoimmune Reactivity ◽  
Autoimmune Pathogenesis

Autoimmune reactivity against corneal antigens is described in two patients with Cogan's syndrome, a nonsyphilitic deep interstitial keratitis with vestibuloauditory symptoms. In both cases corneal antibodies were found at the beginning or during an exacerbation of the disease. After administration of high doses of corticosteroids the corneal antibodies diminished. Interstitial keratitis can generally be controlled by local or systemic corticosteroids. The effect of corticosteroid therapy on the audiovestibular symptoms is variable. The possibility of an autoimmune pathogenesis of Cogan's syndrome is discussed.

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