Autoimmune inner ear disease

Abstract

The original enthusiasm that was generated by the concept that immune-mediated mechanisms may mediate reversible forms of inner ear pathology has been tempered by the realities conveyed by scientific research. The pathophysiology of AIED is still not well understood. Multiple potential mechanisms have been identified that can result in immune-mediated inner ear pathology. The diagnosis of AIED is based on clinical presentation and response to the administration of corticosteroids. Diagnostic testing may support the diagnosis, and the results of a recent multicenter study should clarify the role of immunologic testing in the diagnosis of this entity. Treatment options are limited, with corticosteroids being the only validated treatment option, and methotrexate offering no significant benefit to patients. The role of cyclophosphamide is restricted to patients willing to endure the attendant risks. With the advent of cochlear implants, the administration of toxic medications to preserve hearing at all costs is a less desirable option.

Avaliação audiológica em pacientes com doenças reumáticas pediátricas

“…This was further illustrated by one of the cases with localized scleroderma en coup de sabre affecting the right side of the face, for whom the audiometry results were normal, while the otoacustic emissions were altered on the right ear (16) . In our study, we found sensorineural hearing loss, which has been observed by different studies in both adults (2,3,9,10,16) and children (12,13) . Audiometry also detected alterations in all frequencies, with thresholds up to 35 dB, however, the JIA group presented mainly high frequency impairments, and the AID group, low frequency impairments.…”

“…The results obtained indicated more otological symptoms and hearing impairment in children and adolescents affected by rheumatic diseases, compared with a control group in which rheumatic diseases were ruled out. A full hearing testing comprising otological symptoms survey combined to otological and audiological systematic assessments revealed comparable rates of hearing impairment in adults with the same rheumatic disease spectrum (1)(2)(3)(4)(5)(6)(7)(8)(9)(10) . Although hearing impairment was more frequent in patients with otological complaints, it was verified that 46% of the patients with no symptoms in the JIA group and 50% of those in the AID group presented some degree of impairment in the hearing tests.…”

“…Evidence of hearing loss or poor sound discrimination, tinnitus and vertigo has been verified in adults affected by autoimmune rheumatic disorders (1)(2)(3)(4)(5)(6)(7)(8)(9)(10) ; however, pediatric studies are scarce (11)(12)(13)(14) . Isolated autoimune hearing loss is reported in both adults and children, with normal otoscopy and clinical hearing assessment, and sudden or progressive asymmetric bilateral hearing loss.…”

“…In autoimune sensorial hearing loss the main histological findings are atrophy of the stria vascularis, loss of spiral ganglion cells, with various degrees of hair cell loss and loss of the membranous labyrinth (15) . Early treatment with prednisone can result in full recovery, and prevent progressive hearing loss for what is considered one of the rare reversible sensorineural hearing loss conditions (2) . Autoimmune rheumatic disorders are usually responsive to prednisone (16) .…”

“…It is possible that systemic autoimmune diseases may similarly affect the inner ear. Autoimmune rheumatic diseases cause structural damage or dysfunction in multiple organs and systems due to inflammation, hence the outcome is variable (1,2,17) . In children, the discrimination of symptoms due to eye or ear involvement is limited.…”