Cogan's Syndrome: 18 Cases and a Review of the Literature

Vollertsen, Randall S.; McDonald, Thomas J.; Younge, Brian R.; Banks, Peter M.; Stanson, Anthony W.; Ilstrup, Duane M.

doi:10.1016/s0025-6196(12)61951-x

Cited by 223 publications

(160 citation statements)

References 36 publications

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“…Children may present with signs identical to those of adults with typical Cogan's syndrome (as in case 3) such as associated nonsyphilitic interstitial keratitis and cochleovestibular deficits (including vertigo and SNHL); or pediatric cases may involve atypical forms (case 1 with uveitis and case 2 with polyarthritis). Vollertsen et al 17 reported that adult cases consist of 70% typical forms and 30% atypical.…”

Section: Discussionmentioning

confidence: 99%

“…The most commonly accepted hypothesis-an autoimmune mechanism 6,7,17,18 -is supported by the frequently successful remission of hearing loss after steroid administration, the positive transformation of the lymphoblastic test regarding cochlear antigen in some cases, 19,20 and the association of Cogan's syndrome with other autoimmune diseases such as rheumatoid arthritis. [21][22][23] Schuknecht and Nadol 24 published a histologic study of the temporal bone from a patient who had suffered from Cogan's syndrome and later died of another pathology.…”

Section: Arguments Supporting An Autoimmune Origin For Cogan's Syndromementioning

confidence: 99%

“…Cardiovascular manifestations are the most serious and represent 10% of cases of Cogan's syndrome. 17,35 Aortic insufficiency, coronary stenosis, 34,38 pericarditis, and arrhythmia are the most common cardiac lesions. According to Haynes et al, 3 aortic insufficiency is most common in typical Cogan's syndrome, whereas systemic vasculitis occurs in the atypical form.…”

Section: Systemic Manifestationsmentioning

confidence: 99%

“…The prognosis for hearing is usually poor over the long-term. Vollertsen et al 17 reported 78 observations of Cogan's syndrome in the literature, with poor prognosis in 63% of cases, including 43% with profound hearing loss or anacusis, 8% with total visual deficit, 14% with aortic insufficiency, and 9% who died. Cochlear implantation may be suggested in profound hearing loss, especially when it is associated with visual loss because the development of blindness can impede the acquisition of sign language communication.…”

Section: Treatment and Prognosismentioning

confidence: 99%

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Cochleovestibular Impairment in Pediatric Cogan’s Syndrome

2002

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ABSTRACT. Cogan's syndrome is a rare, chronic inflammatory disorder that typically targets the eyes and vestibuloauditory apparatus, but it may also involve other organs. Three pediatric cases of Cogan's syndrome (ages 5, 13, and 18 years) are reported with long-term follow-up and complete and regular cochleovestibular functional evaluation and ophthalmologic and neurologic examinations. One case was a typical form (characterized by an interstitial keratitis and cochleovestibular impairment), whereas the other 2 cases were atypical forms with uveitis and polyarthritis. In all 3 cases, the first clinical sign was nonspecific eye redness misdiagnosed as a banal conjunctivitis, initially or secondarily associated with bilateral endocochlear sensorineural hearing loss and complete bilateral peripheral vestibular deficit. During the acute phase, early steroid treatment (prednisone, 1 mg/kg/day) was effective in treating the ocular lesions (3 of 3 cases) and improving hearing (2 of 3 cases) but less effective for the vestibular loss (2 of 3 cases). Adverse effects and dependence on the steroid occurred in 2 cases, and immunosuppressive drugs were necessary to avoid recurrences in 1 case. Over the longterm, the disease was controlled in 2 cases but continued to progress in the other. Cogan's syndrome in childhood should be suspected in cases of conjunctivitis associated with inner-ear symptoms; a prompt steroid treatment can avoid progressive impairment of multiple sensorineural functions (vision, balance, hearing). Long-term management involves limiting disease recurrences by adaptive therapies, screening for complications (aortitis in particular), and planning rehabilitation for the sensorial deficits. Pediatrics 2002;109(2). URL: http://www. pediatrics.org/cgi/content/full/109/2/e38; children, Cogan's syndrome, sudden hearing loss, imbalance, vertigo, conjunctivitis.ABBREVIATIONS. EVAR, earth vertical axis rotation; OVAR, off vertical axis rotation test; VOR, vestibuloocular responses; SNHL, sensorineural hearing loss; NSAID, nonsteroidal antiinflammatory drugs; MRI, magnetic resonance imaging; EBV, Epstein-Barr virus. C ogan's syndrome is a relatively rare inflammatory disease characterized by nonsyphilitic ocular interstitial keratitis associated with sudden hearing loss and vestibular impairment. However, its early diagnosis and treatment with steroids can prevent loss of inner-ear sensorial function and limit ophthalmologic aftereffects. In 1934, Morgan and Baumgartner 1 published the first case of the syndrome. In 1945, Cogan 2 published the full description of the syndrome as an inner-ear disorder in its typical form. Several authors later reported atypical forms 3,4 that differed from the typical presentation by the type of ocular lesions, which can include superficial keratitis, scleritis, episcleritis, iritis, or choroiditis, as well as variable hearing impairment and often systemic inflammation. 5 Cogan's syndrome is generally considered an autoimmune disease despite the frequent lack of direct immunologic...

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Section: Discussionmentioning

confidence: 99%

Section: Arguments Supporting An Autoimmune Origin For Cogan's Syndromementioning

confidence: 99%

Section: Systemic Manifestationsmentioning

confidence: 99%

Section: Treatment and Prognosismentioning

confidence: 99%

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Cochleovestibular Impairment in Pediatric Cogan’s Syndrome

2002

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“…Other manifestations include conjunctivitis, iritis, and, less commonly, a systemic necrotizing vasculitis (1)(2)(3). The disease can progress to deafness, but usually responds to corticosteroids provided therapy is begun early (4).…”

Section: Methotrexate Therapy For Hearing Loss In Cogan's Syndromementioning

confidence: 99%

Immunochemical methods of studying the mechanism of diclofenac‐induced hepatitis

Richardson

1994

Arthritis & Rheumatism

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1559fibrobullous changes, chest wall restriction, and rare cricoarytenoid joint involvement (1,6). BOOP has been previously linked to many underlying connective tissue diseases (5). Although autopsy studies have suggested a common occurrence between AS and bronchiolitis (7), there have been only 2 reports in the literature that could support a causal relationship (3,4). We present a woman with progressive pulmonary impairment in association with AS and suggest that AS should be considered as a rheumatic disorder possibly associated with BOOP.The opinions and assertions contained herein are those of the authors and are not to be construed as oficial policy of the

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Open trial of methotrexate as treatment for autoimmune hearing loss

Matteson¹,

Fabry²,

Facer³

et al. 2001

Arthritis & Rheumatism

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Objective To assess the efficacy of low‐dose methotrexate (MTX) administered for the treatment of autoimmune hearing loss. Methods This was a prospective, 12‐month, open‐label study of 17 patients with refractory autoimmune hearing loss. All patients had ongoing episodic worsening of hearing in one or both ears prior to enrollment despite traditional medical therapy. The MTX dose was 7.5–25 mg/week. Hearing loss and vertigo were evaluated at baseline and at completion of the study. Hearing improvement was defined as an improvement in pure tone threshold (PT) average of >10 dB or an increase in speech discrimination (SD) of >15%; worsening was defined as a decrease of >10 dB in PT or a decrease of >15% in SD in at least one ear. Results MTX was well tolerated. Among patients with Meniere's disease, 5 of 9 had improvement or resolution of vertigo. Equilibrium improved in all 3 patients with Cogan's syndrome and improved in 2 out of 3 patients with idiopathic hearing loss and this symptom. According to the parameters defined above, hearing improved in 11 patients (65%), was unchanged in 4 patients (23%), and worsened in 2 patients (12%). Conclusion Long‐term low‐dose MTX therapy may be a useful therapy for at least some patients who have hearing loss with a presumptively autoimmune‐mediated component that is refractory to traditional therapies.

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Cogan's Syndrome: 18 Cases and a Review of the Literature

Cited by 223 publications

References 36 publications

Cochleovestibular Impairment in Pediatric Cogan’s Syndrome

Cochleovestibular Impairment in Pediatric Cogan’s Syndrome

Immunochemical methods of studying the mechanism of diclofenac‐induced hepatitis

Open trial of methotrexate as treatment for autoimmune hearing loss

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