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      Sudden Sensorioneural Hearing Loss and Autoimmune Systemic Diseases

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          Abstract

          Introduction  Several authors have demonstrated the relationship between sudden sensorineural hearing loss (SNHL) and systemic autoimmune diseases (SAD). Immune-mediated SNHL can rarely present as unilateral sudden SNHL and manifests itself in the contralateral ear only after years. It presents clinical relevance for being one of the few SNHL that may be reversible given that early and appropriate treatment is applied.

          Objective  The objective of this study is to describe the clinical presentations and audiological findings from patients with idiopathic sudden SNHL and SAD associated with a probable diagnosis of immune-mediated SNHL. Furthermore, we strive to estimate the prevalence of SAD in patients with sudden SNHL.

          Methods  This is an observational retrospective cohort. We have selected and studied patients with SAD. Revision of available literature on scientific repositories.

          Results  We evaluated 339 patients with sudden SNHL. Among them, 13 (3.83%) patients suffered from SAD. Three patients had bilateral involvement, a total of 16 ears. We evaluate and describe various clinical, epidemiological, and audiological aspects of this sample.

          Conclusion  In our sample of patients with sudden SNHL, the prevalence of SAD was found relevant. The majority had tinnitus and dizziness concomitant hearing loss, unilateral involvement and had experienced profound hearing loss at the time of the installation. In spite of instituted treatment, most cases showed no improvement in audiometric thresholds. Apparently, patients with sudden SNHL and SAD have a more severe initial impairment, higher percentage of bilateral, lower response to treatment, and worse prognosis than patients with sudden SNHL of unknown etiology.

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          Most cited references58

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          Sudden hearing loss: eight years' experience and suggested prognostic table.

          F M Byl (1984)
          The etiology, incidence, acute and late prognosis, and treatment of sudden hearing loss (SHL) are described variously in the literature. In an 8-year prospective study of 225 SHL patients, initiated in July 1973, overall, normal, or complete recovery occurred in 45% of patients and late otologic complications in 28%. Important prognostic indicators were severity of initial hearing loss and vertigo, time to initial audiogram, and elevated erythrocyte sedimentation rate; other indicators were age greater than 60 and less than 15 years, midfrequency audiogram configuration, and hearing status of the opposite ear. A common inflammatory cause is suggested for all degrees of severity in SHL, and a prognostic table is provided to aid the practitioner in predicting recovery. There is still no evidence that treatment achieves a result better than expected with spontaneous recovery.
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            The efficacy of steroids in the treatment of idiopathic sudden hearing loss. A double-blind clinical study.

            Double-blind studies were conducted for the treatment of idiopathic sudden hearing loss (ISHL) with oral steroids. The condition was defined as not less than a 30-dB loss over three contiguous frequencies in three days or less. Follow-up audiograms were obtained four weeks and three months later. Specific audiologic guidelines for the assessment of hearing recovery were used to ensure objectivity. Steroids had a statistically significant effect on the recovery of hearing in patients with moderate hearing losses. The nature of the hearing loss and its susceptibility to improvement with steroid therapy lend support to the hypothesis that viral cochlitis is the primary cause of ISHL.
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              Cogan syndrome: a retrospective review of 60 patients throughout a half century.

              To evaluate the disease manifestations and clinical course of patients affected by Cogan syndrome (a syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms) at a single institution during roughly a half century. Medical records of all patients diagnosed as having Cogan syndrome at the Mayo Clinic in Rochester, Minn, and who were followed up from 1940 to 2002 were comprehensively reviewed. Otolaryngologic, ophthalmologic, and systemic manifestations of disease were analyzed. Analysis included patient demographics, presenting manifestations, delayed manifestations, laboratory testing, physical examination features, therapeutic interventions, disease course, and hearing and vision outcomes. Sixty patients were identified as having Cogan syndrome, with follow-up from 1940 to 2002. Most patients presented initially with vestibuloauditory symptoms, most commonly sudden hearing loss (50%). The most common inflammatory ophthalmologic condition noted was bilateral interstitial keratitis. Headache (40%), fever (27%), and arthralgia (35%) were the most frequently encountered systemic manifestations. Evidence of aortitis was found in 12% of patients. Complete hearing loss was eventually noted in 52% of affected patients, whereas permanent loss of any degree of vision was uncommon. Cochlear implantation outcomes were uniformly good. Death directly or indirectly attributed to the effects of Cogan syndrome was noted in 4 patients. The major disease-related morbidities were due to vestibuloauditory disease and only infrequently due to systemic manifestations such as vasculitis, with or without aortitis. Cochlear implantation has been of major benefit in modern hearing rehabilitation for this patient population. We advise caution before institution of protracted courses of high-dose corticosteroids and/or chemotherapy for patients without pronounced systemic disease or severe eye disease unmanageable by topical or periocular corticosteroids alone.
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                Author and article information

                Journal
                Int Arch Otorhinolaryngol
                Int Arch Otorhinolaryngol
                10.1055/s-00025477
                International Archives of Otorhinolaryngology
                Thieme Revinter Publicações Ltda (Rio de Janeiro, Brazil )
                1809-9777
                1809-4864
                July 2017
                26 July 2016
                : 21
                : 3
                : 213-223
                Affiliations
                [1 ]Department of ENT, Universidade Federal de São Paulo, São Paulo, Brazil
                Author notes
                Address for correspondence Bruno Almeida Antunes Rossini, MD Clinica Otovita de Otorrinolaringologia Rua Cubatão, 86–1407, Sao Paulo 04013000Brazil baarossini@ 123456gmail.com
                Article
                0316or
                10.1055/s-0036-1586162
                5495599
                d3ca5708-32d1-4733-a9b9-39ad93d38afd
                © Thieme Medical Publishers
                History
                : 15 June 2015
                : 03 June 2016
                Categories
                Original Research

                hearing loss,sensorineural,sudden,autoimmune diseases,humans

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