Autoantibodies against inner ear proteins in patients with delayed endolymphatic hydrops and unilateral juvenile deafness

Suzuki, Mikio; Hanamitsu, Masakazu; Kitanishi, Tsuyoshi; Kohzaki, Hideaki; Kitano, Hiroya

doi:10.1080/00016480500266008

Cited by 5 publications

(6 citation statements)

References 18 publications

(18 reference statements)

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“…Damage to the inner ear can cause profound hearing loss by exposing the immune system to previously sequestered inner ear proteins, which may result in the production of antibodies against the inner ear, as the inner ear contains thousands of proteins. 22 Animal models used to investigate AIED have shown that there is no anatomic barrier to the systemic circulation within the cochleovestibular compartment. 11 The endolymphatic sac contains immunocompetent tissues capable of immune responses.…”

Section: Pathophysiologymentioning

confidence: 99%

“…3 The inner ear antigens targeted by the immune system have not yet been identified, even though numerous reports of progressive hearing loss associated with systemic autoimmune diseases have been published. 22 Cogan's syndrome, Behcet's disease, relapsing polychondritis, systemic lupus erythematosus, rheumatoid arthritis, polyarteritis nodosa, and inflammatory bowel disease have all been linked with SNHL and dizziness. Audiovestibular disease control or management with immunosuppressive drugs has been effective in reversing or stabilizing hearing loss in some of these patients.…”

Section: Pathophysiologymentioning

confidence: 99%

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Autoimmune Inner Ear Disease

Samy¹

2011

Semin Hear

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Autoimmune inner ear disease (AIED), first reported by McCabe in 1979, describes a disease process in which cochleovestibular is compromised by one's own immune system. Only indirect laboratory evidence exists regarding the underlying immune reaction, which can only be confirmed histopathologically in postmortem studies. Diagnosis involves a thorough history and complete head and neck, otomicroscopic, and audiometric evaluation. AIED classically presents with bilateral, fluctuating, or rapidly progressive asymmetric sensorineural hearing loss (SNHL), which typically occurs over weeks to months but can occur suddenly over a period of a few hours or days. Fifty percent of patients have vestibular symptoms as well, which can be unilateral or bilateral. A lengthy serological workup is unwarranted given the absence of a reliable diagnostic laboratory test. Antibodies to the 68-kDa protein (heat shock protein-70) is marker-specific but not sensitive for AIED and may correlate with steroid responsiveness. In cases where the diagnosis remains unclear, a prolonged course of steroids, with repeat audiometric testing at 1 month, can be undertaken. Treatment aims to inhibit the detrimental immune response using immunosuppressant drugs; however, no standardized treatment regimen has been found. Cochlear implantation remains a viable last resort for patients with progressive SNHL.KEYWORDS: Sensorineural hearing loss, autoimmune inner ear disease, endolymphatic hydrops, dizziness Learning Outcomes: As a result of this activity, the participant will be able to (1) list how to diagnose autoimmune inner ear disease and (2) describe how to treat autoimmune inner ear disease.

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Section: Pathophysiologymentioning

confidence: 99%

Section: Pathophysiologymentioning

confidence: 99%

Autoimmune Inner Ear Disease

Samy¹

2011

Semin Hear

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“…Suzuki reported contralateral DEH in an adolescent with unilateral deafness 10 . This contralateral occurrence of hydrops may have been due to autoimmune attack on the DEH ear 10 …”

Section: Introductionmentioning

confidence: 96%

“…9 Suzuki reported contralateral DEH in an adolescent with unilateral deafness. 10 This contralateral occurrence of hydrops may have been due to autoimmune attack on the DEH ear. 10 However, these studies are still inadequate to determine the underlying causes and genetic factors associated with DEH pathology.…”

Section: Introductionmentioning

confidence: 98%

“…In recent years, DEH patients have been described among cases of familial unilateral deafness, raising the possibility that genetic factors may sometimes be responsible for this unusual syndrome 9 . Suzuki reported contralateral DEH in an adolescent with unilateral deafness 10 . This contralateral occurrence of hydrops may have been due to autoimmune attack on the DEH ear 10 …”

Section: Introductionmentioning

confidence: 99%

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The transcriptome characteristics of vestibular organs from delayed endolymphatic hydrops patients (Meniere’s disease)

Chen

Luo

Zhang

et al. 2021

Clinical Otolaryngology

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Objectives To identify genes that are related to delayed endolymphatic hydrops (DEH) in patients by RNA‐Seq analysis. Design Observational study. Setting Eye & ENT Hospital, Fudan University (Shanghai, China). Participants We collected the entire vestibular system from four patients with DEH who underwent labyrinthectomy. Three control samples were collected from patients with acoustic neuroma or facial neuroma treated via the translabyrinthine approach. High‐throughput RNA‐Seq analysis was performed to investigate gene expression in the pathological vestibular system. Main outcome measures Our bioinformatic analysis identified 17 genes that were upregulated and eight genes that were downregulated in patients with DEH compared with the controls. Results The altered gene expression profile suggested that DEH is closely related to neuropathy and autoimmune disease. In addition, many of the differentially regulated genes were involved in cell adhesion, suggesting a role of cell adhesion in DEH. Immunofluorescence analysis confirmed the expression of PMP2 and CLDN19 in the cytoplasm of hair cells and scattered expression of MPZ at cell junctions. The protein expression levels were higher in specimens from patients with Ménière's disease and DEH compared with controls. Conclusions The protein expression profile of vestibular organs in patients with endolymphatic hydrops exhibited a degree of similarity to that of Ménière's disease. Endolymphatic hydrops is characterised by autoimmune abnormalities. DEH and Ménière's disease are likely to be different manifestations of the same disease, with disparate clinical symptoms. RNA‐Seq is a useful analytical tool to characterise the vestibular pathology based on its transcriptome.

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Endolymphatic Hydrops Detected by 3-Dimensional Fluid-Attenuated Inversion Recovery MRI following Intratympanic Injection of Gadolinium in the Asymptomatic Contralateral Ears of Patients with Unilateral Ménière’s Disease

et al. 2015

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BackgroundThe aim of this study was to identify the incidence of endolymphatic hydrops using 3-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI) in the contralateral ear in patients with unilateral Ménière’s disease (MD).Material/MethodsThis was a prospective study. 3D-FLAIR MRI was performed with a 3 Tesla (3 T) unit 24 h after the intratympanic administration of gadolinium (Gd) in 30 unilateral MD patients with an asymptomatic contralateral ear. The incidence of contralateral involvement in unilateral MD patients and the potential correlations between the affected and contralateral ears were analyzed.ResultsEndolymphatic hydrops was observed in 7 of the 30 (23.3%) asymptomatic ears. The mean PTA of the asymptomatic ears in the contralateral hydrops patients (33.0±6.1 dB) was significantly higher compared with the non-hydrops patients (17.8±5.7 dB). The patients with observed contralateral hydrops exhibited a significantly longer duration of the disease compared with the non-hydrops patients (6.7±6.3 vs. 2.9±3.1 years, respectively). Furthermore, the patients with contralateral hydrops had a worse hearing level in the affected ears compared with the non-hydrops patients (70.3±7.4 vs. 52.5±3.8 dB, respectively).ConclusionsEndolymphatic hydrops is closely related to hearing loss but does not necessarily result in Ménière’s symptoms. Patients with a long history of MD and severe hearing loss in the affected ear are more likely to exhibit endolymphatic hydrops in the asymptomatic contralateral ear. Adequate attention should focus on unilateral MD patients with contralateral ear hydrops because of the potential to develop bilateral MD.

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Autoantibodies against inner ear proteins in patients with delayed endolymphatic hydrops and unilateral juvenile deafness

Cited by 5 publications

References 18 publications

Autoimmune Inner Ear Disease

Autoimmune Inner Ear Disease

The transcriptome characteristics of vestibular organs from delayed endolymphatic hydrops patients (Meniere’s disease)

Endolymphatic Hydrops Detected by 3-Dimensional Fluid-Attenuated Inversion Recovery MRI following Intratympanic Injection of Gadolinium in the Asymptomatic Contralateral Ears of Patients with Unilateral Ménière’s Disease

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