Autoimmune reactivity in Cogan's syndrome: A preliminary report

Hughes, Gordon B.; Kinney, Sam E.; Barna, Barbara P.; Tomsak, Robert L.; Calabrese, Leonard H.

doi:10.1177/019459988309100106

Cited by 85 publications

(25 citation statements)

References 13 publications

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“…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%

“…7,28 Most often vestibular damage is detected by clinical examination only, and instrumental vestibular testing is seldom realized, particularly in children. Cochlear damage is usually better documented.…”

Section: Cochleovestibular Lesions: Labyrinthitismentioning

confidence: 99%

“…5 Cogan's syndrome is generally considered an autoimmune disease despite the frequent lack of direct immunologic evidence. 6,7 The efficacy of steroid treatment in the acute phase provides a compelling argument for this hypothesis.Diagnosis of Cogan's syndrome is often missed or delayed because it is a rare disease and its clinical signs at onset are not specific (banal conjunctivitis). This is dangerous for the patients because steroid treatment should begin as early as possible to allow functional restoration of the inner ear and the eyes before irreversible lesions develop.…”

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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: Arguments Supporting An Autoimmune Origin For Cogan's Syndromementioning

confidence: 99%

Section: Cochleovestibular Lesions: Labyrinthitismentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Cochleovestibular Impairment in Pediatric Cogan’s Syndrome

2002

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“…Ototoxic antibiotics, meningitis, tumours, bilateral endolymphatic hydrops and systemic autoimmune disorders (e.g. lupus erythematosus, polychondritis, Cogan syndrome [12], rheumatoid arthritis) are among the many causes of BV. Despite comprehensive investigations, its aetiology still remains obscure in about 20-30% of cases (so-called idiopathic bilateral vestibulopathy, IBV).…”

Section: Introductionmentioning

confidence: 99%

Serum antibodies against membranous labyrinth in patients with „idiopathic“ bilateral vestibulopathy

Arbusow¹,

Strupp²,

Dieterich³

et al. 1998

J Neurol

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To investigate the possibility of an autoimmune mechanism in idiopathic bilateral vestibulopathy (IBV), we screened patients' sera for antibodies against inner ear structures. IgG antibodies against membranous labyrinth (ampulla, semicircular canals, saccule and utricle) were detected in 8 of 12 patients by immunofluorescence on rat inner ear cryosections. All but one serum of 22 healthy controls and the sera of 6 patients with known autoimmune disorders showed only background staining. Low-titre anti-nuclear IgM antibodies were present in three control sera and one IBV serum. High-titre anti-nuclear IgM was found in a patient with lupus erythematosus and in one with scleroderma. Anti-nuclear IgM was not organ-specific. No human serum used contained detectable anti-vascular preformed antibodies. Cross-reactivity to sections of liver, kidney, cornea, brain and skeletal muscle was absent. Double-staining for IgG and F-actin, the primary constituent of hair cell cilia, did not show predominant Ig-coating of sensory hair cells. Immunosuppressive therapy in 3 IBV patients did not improve the disorder, probably owing to irreversible loss of sensory and neural structures. These data suggest that the bulk of anti-labyrinthine autoantibodies may be an epiphenomenon, yet a small subgroup of organ-specific autoantibodies may synergize with a cellular response in the development of vestibular lesions.

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