Cogan Syndrome: The Audiological Picture

Djupesland, G; Flottorp, Gordon; Hansen, Egill; Sjaastad, Ottar

doi:10.1001/archotol.1974.00780030226014

Cited by 17 publications

(6 citation statements)

References 11 publications

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“…First described by Cogan [7], Cogan's syndrome is associated with the sudden onset of a sensorineural hearing loss and ocular symptoms, which are classicaly described as an interstitial keratitis [2,10,18]. Nevertheless, atypical Cogan's syndrome can include other ophthalmic manifestations such as scleritis, episcleritis, exophthalmia and conjunctival hemorrhage [17,23,24].…”

Section: Introductionmentioning

confidence: 99%

Cochlear implantation in patients with Cogan's syndrome: a review of four cases

Minét

Deggouj

Gersdorff

1997

Eur Arch Otorhinolaryngol

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Cogan's syndrome is a rare disorder that is characterized by a rapid onset of bilateral severe sensorineural hearing loss associated with vertigo and tinnitus, ocular involvement (most commonly an interstitial keratitis) and variable systemic symptoms. We review our experience with four cases treated in our department. We describe their symptoms, the evolution of their disease, the diagnosis, and the radiological investigations with computed tomography and/or magnetic resonance imaging. When medical treatment with corticosteroids failed and no benefit could be obtained with hearing aids at each patient's end stage, all patients received a cochlear implant with excellent results. The considerations for implantation in these patients are discussed.

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Section: Introductionmentioning

confidence: 99%

Cochlear implantation in patients with Cogan's syndrome: a review of four cases

Minét

Deggouj

Gersdorff

1997

Eur Arch Otorhinolaryngol

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“…The glycerol test may be positive . 7 With advanced disease, hearing loss is usually progressive, nonfluctuating, and severe; ataxia (especially in darkness) may replace episodic vertigo. Vestibular evaluation usually reveals reduced or absent vestibular response on caloric stimulation.…”

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confidence: 99%

Autoimmune reactivity in Cogan's syndrome: A preliminary report

Hughes¹,

Kinney²,

Barna³

et al. 1983

Otolaryngol.--head neck surg.

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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.

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“…LFABGs were postoperatively detected in many of our cases (81.3%, 13 of 16). It has been previously reported that LFABG was detected in about 30% of cases of Ménière disease without any other surgical procedures15 and was also detected in patients suffering from Cogan syndrome 16. The authors suggested that LFABGs might be a result of endolymphatic hydrops or perilymphatic hypertension, which dampened stapes foot plate mobility.…”

Section: Discussionmentioning

confidence: 94%