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Idiopathic sensorineural hearing disorders in adults—a pragmatic approach

Nature Reviews Rheumatology volume 5, pages 505–512 (2009)Cite this article

Abstract

Acute or progressive sensorineural hearing loss, with or without vertigo and tinnitus, has a considerable impact upon an individual's quality of life. Sensorineural hearing disorders are more commonly observed in patients with rheumatic disease than in the general population, are more likely to be accompanied by the presence of serum autoantibodies, and might respond to steroid therapy. A subgroup of these disorders seem to be immune mediated, but audiologic presentation and serologic testing do not enable this subgroup to be further defined. Rheumatologists should be prepared to manage patients with known rheumatic disease who develop acute hearing loss and to evaluate referred patients without known rheumatic illness experiencing progressive or refractory sensorineural hearing loss. In this article, we summarize the literature and outline a rational approach for the evaluation and treatment of such patients.

Key Points

  • Sensorineural hearing loss can be immune mediated

  • These disorders can be organ specific or be part of a systemic rheumatic disorder

  • The clinical pattern of the hearing loss defines the prognosis and approach to therapy

  • Careful review of organs for systemic rheumatic disease, audiogram and MRI are of greatest diagnostic value

  • Large panels of serologic screening tests are not cost effective and can be misleading

  • Immunosuppressive therapies other than corticosteroids have proved ineffective in most cases

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Figure 1: A schematic representation of management recommendations for patients with sensorineural hearing loss, based on published literature.

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Acknowledgements

The authors would like to thank Dr. Matthew Liang for his review and recommendations.

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Authors and Affiliations

  1. Department of Internal Medicine, The Reading Hospital and Medical Center, West Reading, PA, USA

    David L. George & Sagun Pradhan

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Correspondence to David L. George.

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George, D., Pradhan, S. Idiopathic sensorineural hearing disorders in adults—a pragmatic approach. Nat Rev Rheumatol 5, 505–512 (2009). https://doi.org/10.1038/nrrheum.2009.150

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