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            : Autoimmune and Autoinflammatory (Disease) in Otology: What is New in Immune‐Mediated Hearing Loss

Vambutas, Andrea; Pathak, Shresh

doi:10.1002/lio2.28

Cited by 95 publications

(115 citation statements)

References 65 publications

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“…More than 70% of the patients with an autoimmune inner ear disorder do not show clinical manifestations of a systemic autoimmune disease, and no systemic autoimmune disorder was detected in MD patients with circulating autoantibodies and inner ear immune reactions. 26,28 These observations suggest autoimmunity confined to the inner ear could be a possible mechanism for BMD and delayed MD, but the pathological mechanisms may be different between Asian and Caucasian populations. Genetic mechanisms also have been suggested as a cause for the bilaterality of MD.…”

Section: Discussionmentioning

confidence: 96%

Clinical Characteristics of Bilateral Meniere's Disease in a Single Asian Ethnic Group

et al. 2018

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Objectives/Hypothesis To identify the clinical characteristics of patients with bilateral Meniere's disease (MD) in an Asian population. Study Design Cross‐sectional retrospective study. Methods We compared the clinical features of bilateral MD (BMD) and unilateral MD (UMD) in 320 Asian patients with single ethnicity. Demographic variables; age of onset; inner ear function; the coexistence of related disorders such as vestibular migraine, delayed MD, systemic autoimmune diseases, and familial MD; and prognoses were analyzed and compared. Results The overall prevalence of BMD was 5.6%. The mean age of disease onset was 40.3 ± 14.8 and 47.0 ± 14.1 years for patients with BMD and UMD, respectively (P = .07). Demographic variables were not significantly different between patients with BMD and UMD (P > .05). Inner ear function, evaluated by hearing thresholds, caloric tests, and cervical vestibular evoked myogenic potentials, was significantly more deteriorated in the first involved ear of patients with BMD than in the second involved ear or the affected side of patients with UMD (P < .05). Among the comorbid conditions, only the prevalence of delayed MD was significantly higher in patients with BMD than in patients with UMD. Systemic autoimmune disease was found in only three patients with UMD. There was no significant prognostic difference between patients with UMD and BMD (P > .05). Conclusions A low prevalence of BMD, a higher frequency of delayed MD in BMD patients, and a low frequency of systemic autoimmune diseases in both UMD and BMD patients are significant findings in an Asian population. Level of Evidence 4 Laryngoscope, 129:1191–1196, 2019

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Section: Discussionmentioning

confidence: 96%

Clinical Characteristics of Bilateral Meniere's Disease in a Single Asian Ethnic Group

et al. 2018

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“…Inner ear involvement in systemic autoimmune diseases should be distinguished from primary autoimmune inner ear disease, a condition in which the immune response acts directly against the inner ear [6, 7]. …”

Section: Introductionmentioning

confidence: 99%

Audiovestibular Symptoms in Systemic Autoimmune Diseases

Ralli

D’Aguanno

Stadio

et al. 2018

Journal of Immunology Research

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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.

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“…Secreted factors, such as inflammatory cytokines, have been shown to cause cochlea damage and hearing loss via immune-mediated mechanisms (47,48). Activation of the cMET pathway has been shown to mobilize an inflammatory network in the brain microenvironment to promote cancer progression (49,50).…”

Section: Discussionmentioning

confidence: 99%

Targeting the cMET pathway augments radiation response without adverse effect on hearing in NF2 schwannoma models

Zhao

Liu

Zhang

et al. 2018

Proc. Natl. Acad. Sci. U.S.A.

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Neurofibromatosis type II (NF2) is a disease that needs new solutions. Vestibular schwannoma (VS) growth causes progressive hearing loss, and the standard treatment, including surgery and radiotherapy, can further damage the nerve. There is an urgent need to identify an adjunct therapy that, by enhancing the efficacy of radiation, can help lower the radiation dose and preserve hearing. The mechanisms underlying deafness in NF2 are still unclear. One of the major limitations in studying tumor-induced hearing loss is the lack of mouse models that allow hearing testing. Here, we developed a cerebellopontine angle (CPA) schwannoma model that faithfully recapitulates the tumor-induced hearing loss. Using this model, we discovered that cMET blockade by crizotinib (CRZ) enhanced schwannoma radiosensitivity by enhancing DNA damage, and CRZ treatment combined with low-dose radiation was as effective as high-dose radiation. CRZ treatment had no adverse effect on hearing; however, it did not affect tumor-induced hearing loss, presumably because cMET blockade did not change tumor hepatocyte growth factor (HGF) levels. This cMET gene knockdown study independently confirmed the role of the cMET pathway in mediating the effect of CRZ. Furthermore, we evaluated the translational potential of cMET blockade in human schwannomas. We found that human NF2-associated and sporadic VSs showed significantly elevated HGF expression and cMET activation compared with normal nerves, which correlated with tumor growth and cyst formation. Using organoid brain slice culture, cMET blockade inhibited the growth of patient-derived schwannomas. Our findings provide the rationale and necessary data for the clinical translation of combined cMET blockade with radiation therapy in patients with NF2.

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AAO : Autoimmune and Autoinflammatory (Disease) in Otology: What is New in Immune‐Mediated Hearing Loss

Cited by 95 publications

References 65 publications

Clinical Characteristics of Bilateral Meniere's Disease in a Single Asian Ethnic Group

Clinical Characteristics of Bilateral Meniere's Disease in a Single Asian Ethnic Group

Audiovestibular Symptoms in Systemic Autoimmune Diseases

Targeting the cMET pathway augments radiation response without adverse effect on hearing in NF2 schwannoma models

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