Abstract
Immune-mediated inner ear disease can be primary, when the autoimmune response is against the inner ear, or secondary. The latter is characterized by the involvement of the ear in the presence of systemic autoimmune conditions. Sensorineural hearing loss is the most common audiovestibular symptom associated with systemic autoimmune diseases, although conductive hearing impairment may also be present. Hearing loss may present in a sudden, slowly, rapidly progressive or fluctuating form, and is mostly bilateral and asymmetric. Hearing loss shows a good response to corticosteroid therapy that may lead to near-complete hearing restoration. Vestibular symptoms, tinnitus, and aural fullness can be found in patients with systemic autoimmune diseases; they often mimic primary inner ear disorders such as Menière's disease and mainly affect both ears simultaneously. Awareness of inner ear involvement in systemic autoimmune diseases is essential for the good response shown to appropriate treatment. However, it is often misdiagnosed due to variable clinical presentation, limited knowledge, sparse evidence, and lack of specific diagnostic tests. The aim of this review is to analyse available evidence, often only reported in the form of case reports due to the rarity of some of these conditions, of the different clinical presentations of audiological and vestibular symptoms in systemic autoimmune diseases.
Highlights
The inner ear has been considered for a long time an immune-privileged site, spared from organ-specific autoimmunity and rarely involved in systemic autoimmune diseases thanks to the blood-labyrinthine barrier [1]
The evidence of a clear presence of macrophages in the human temporal bone of patients affected by autoimmune diseases [15] support the hypothesis that in Multiple sclerosis (MS), the autoimmunity mechanisms affect the structures of the inner ear; hair cells and auditory and vestibular spiral ganglion neurons may be subject to the attack of lymphocytes, and their damage may present with sensorineural hearing loss (SNHL) and vertigo
Audiovestibular symptoms may be found in a variety of autoimmune diseases, and diagnosis is essential to increase the chances of restoration when specific therapy is promptly initiated
Summary
The inner ear has been considered for a long time an immune-privileged site, spared from organ-specific autoimmunity and rarely involved in systemic autoimmune diseases thanks to the blood-labyrinthine barrier [1]. There is growing interest for inner ear involvement in systemic autoimmune diseases [5, 6]; this condition should be considered in patients with audiovestibular dysfunction presenting a constellation of symptoms consistent with systemic autoimmunity or with a preexisting diagnosis of autoimmune disease [7, 8]. Inner ear involvement in autoimmune diseases is estimated to account for less than 1% of all cases of acquired hearing loss [7] and follows gender and demographic characteristics of autoimmune disorders, with higher prevalence in female patients between their thirties and fifties [5]. A correct identification of inner ear involvement in patients with systemic autoimmune diseases is essential for the possibility of near-complete hearing restoration with appropriate treatment [9]; it is often misdiagnosed due to variable clinical presentation, limited knowledge, sparse evidence, and lack of specific diagnostic tests. The aim of this review is to analyse the available evidence of the different clinical presentations of audiological and vestibular symptoms in systemic autoimmune diseases, this is often only reported in the form of case reports due to the rarity of some of these conditions
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