The hypothesis that a syndrome of sensorineural hearing loss (SNHL) often accompanied by vestibular symptoms might be of autoimmune origin was first proposed by McCabe (1979), who based his findings on clinical features, presence of abnormal immunological tests, and a positive response to immunosuppressive therapy. Since then, a number of syndromes characterized by SNHL with overlapping clinical features have been described and termed in different ways: autoimmune SNHL, immune-mediated inner ear disease (IMIED), idiopathic progressive bilateral SNHL, sudden SNHL, idiopathic SNHL, bilateral immune-mediated Me´nie`re’s disease, and autoimmune vestibulo-cochlear disorders, generating a great confusion in the identification of patients and in the evaluation of different studies. SNHL in adult patients remains idiopathic in the vast majority of cases (71%); known causes are viral infections (12.8%), inner ear abnormality (4.7%), trauma (4.2%), vascular or hematologic (2.8%), neoplastic (2.3%), and CNS abnormality.
Immune-Mediated Inner Ear Disease
LUNARDI, Claudio;
2014-01-01
Abstract
The hypothesis that a syndrome of sensorineural hearing loss (SNHL) often accompanied by vestibular symptoms might be of autoimmune origin was first proposed by McCabe (1979), who based his findings on clinical features, presence of abnormal immunological tests, and a positive response to immunosuppressive therapy. Since then, a number of syndromes characterized by SNHL with overlapping clinical features have been described and termed in different ways: autoimmune SNHL, immune-mediated inner ear disease (IMIED), idiopathic progressive bilateral SNHL, sudden SNHL, idiopathic SNHL, bilateral immune-mediated Me´nie`re’s disease, and autoimmune vestibulo-cochlear disorders, generating a great confusion in the identification of patients and in the evaluation of different studies. SNHL in adult patients remains idiopathic in the vast majority of cases (71%); known causes are viral infections (12.8%), inner ear abnormality (4.7%), trauma (4.2%), vascular or hematologic (2.8%), neoplastic (2.3%), and CNS abnormality.
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