970 results match your criteria Inner Ear Autoimmune Disease


Use of biologics for treatment of autoimmune inner ear disease.

Am J Otolaryngol 2022 Aug 5;43(5):103576. Epub 2022 Aug 5.

Department of Otolaryngology-Head and Neck Surgery Texas Medical Branch, Galveston, TX, United States of America.

Objective: Biologic medications are novel therapeutics in the treatment of Autoimmune Inner Ear Disease (AIED), an etiology of Sensorineural Hearing Loss (SNHL). The goal of this study is to review the currently available literature on the efficacy of biologic medications on autoimmune-mediated hearing loss and associated symptomology among patients with AIED.

Methods: A systematic review of Pubmed, Scopus, Cochrane, and Web of Science databases was conducted to identify studies investigating the impact of biologic medications on hearing outcomes. Read More

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Autoimmune Hearing Loss: A Diagnostic Challenge.

J Clin Med 2022 Aug 7;11(15). Epub 2022 Aug 7.

1st Otolaryngology Department, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, AHEPA Hospital, 54636 Thessaloniki, Greece.

Autoimmune hearing loss (AIHL) is a clinical disease and may involve the deposition of immune complexes in the labyrinth vessels, the activation of the complement system, the functional alteration in T-cell subpopulations, or an inflammation process in the inner ear [... Read More

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IgG4-Related Sclerosing Disease of the Temporal Bone: A Systematic Review.

Otol Neurotol 2022 Sep 9;43(8):856-863. Epub 2022 Aug 9.

School of Medicine, University of Glasgow, University Avenue.

Objective: IgG4-related disease (IgG4-RD) involving the temporal bone is an uncommon and underrecognized pathology often mistaken for malignancy. This systematic review is the first that aims to thoroughly analyze IgG4-RD of the temporal bone.

Databases Reviewed: Ovid MEDLINE, EMBASE, Cochrane Library, and Google Scholar. Read More

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September 2022

Rare case of sudden onset sensorineural hearing loss in a patient diagnosed with sarcoidosis.

BMJ Case Rep 2022 Jul 12;15(7). Epub 2022 Jul 12.

Department of Ear, Nose and Throat, West Suffolk Hospitals NHS Trust, Bury Saint Edmunds, UK.

We report the case of a woman in her 30s who was referred to the ear, nose and throat department with sudden onset left-sided sensorineural hearing loss (SNHL), left anterior uveitis and erythematous lower limb lesions with bilateral pitting oedema. Based on her symptoms, an underlying inflammatory systemic disease was suspected. Autoantibodies were negative but an X-ray and high-resolution CT scan of the chest were suggestive of sarcoidosis, which was confirmed on endoscopic bronchial biopsy. Read More

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Clinical characteristics of patients with dizziness/vertigo showing a dissociation between caloric and video head impulse test results.

Ear Nose Throat J 2022 Jul 11:1455613221113790. Epub 2022 Jul 11.

Department of Neurology, 457651The First Affiliated Hospital of Jinzhou Medical University, Jinzhou, China.

Objective: To explore the clinical characteristics of patients with dizziness/vertigo who showed a dissociation between the results of the caloric test and video head impulse test (vHIT).

Methods: A total of 327 patients who complained of dizziness/vertigo were continuously included. All patients underwent both the horizontal vHIT (h-vHIT) and caloric tests. Read More

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Lack of NHE6 and Inhibition of NKCC1 Associated With Increased Permeability in Blood Labyrinth Barrier-Derived Endothelial Cell Layer.

Front Cell Neurosci 2022 12;16:862119. Epub 2022 Apr 12.

Department of Biomedicine, University Hospital Basel, University of Basel, Basel, Switzerland.

Acoustic trauma, autoimmune inner ear disease, and presbycusis feature loss of the integrity of the blood-labyrinth barrier (BLB). Normal BLB function depends on endothelial structural integrity, which is supported and maintained by tight junctions and adherens junctions within the microvascular endothelial layer. When these junctions are disrupted, vascular leakage occurs. Read More

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Role of Inner Ear Macrophages and Autoimmune/Autoinflammatory Mechanisms in the Pathophysiology of Inner Ear Disease.

Front Neurol 2022 6;13:861992. Epub 2022 Apr 6.

Department of Otolaryngology-Head and Neck Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

Macrophages play important roles in tissue homeostasis and inflammation. Recent studies have revealed that macrophages are dispersed in the inner ear and may play essential roles in eliciting an immune response. Autoinflammatory diseases comprise a family of immune-mediated diseases, some of which involve sensorineural hearing loss, indicating that similar mechanisms may underlie the pathogenesis of immune-mediated hearing loss. Read More

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Autoimmune thyroiditis in patients with sudden sensorineural hearing loss.

Laryngoscope Investig Otolaryngol 2022 Apr 10;7(2):571-577. Epub 2022 Feb 10.

Department of Otolaryngology Head and Neck Surgery The Sixth Affiliated Hospital of Sun Yat-sen University Guangzhou China.

Objective: This study aims to evaluate the association between autoimmune thyroiditis and Sudden Sensorineural Hearing Loss (SSNHL).

Methods: Hundred and five patients with SSNHL were enrolled. Audiometric tests, serum thyroid autoantibodies (TPOAb, TgAb) were studied. Read More

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Combination Immunosuppressive Therapy in Primary Autoimmune Inner Ear Disease in Pregnancy.

Case Rep Otolaryngol 2022 18;2022:9210780. Epub 2022 Mar 18.

Department of Otolaryngology, Nepean Hospital, Kingswood, NSW 2747, Australia.

Objective: Autoimmune inner ear disease (AIED) is a rare disorder characterized by rapidly progressive, sensorineural hearing loss that demonstrates good responsiveness to corticosteroid and immunosuppressive therapy. The pathophysiology is likely driven by chronic trafficking of immune cells into the inner ear, targeting inner ear proteins to coordinate inflammation. Suppression or modulation of the immune response can minimize cochleitis allowing for potential recovery of hearing. Read More

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Autoimmune Vestibulopathy-A Case Series.

Brain Sci 2022 Feb 24;12(3). Epub 2022 Feb 24.

Department of Neuro-Otology, Royal National ENT and Eastman Dental Hospitals, UCLH, London WC1E 6DG, UK.

Autoimmune inner ear disease (AIED) is a rare clinical entity. Its pathogenicity, heterogenous clinical presentation in the context of secondary systemic autoimmune disease and optimal treatment avenues remain poorly understood. Vestibular impairment occurring in the context of AIED is rarely subject to detailed investigation given that the auditory symptoms and their responsiveness to immunosuppression are the focus of the few proposed diagnostic criteria for AIED. Read More

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February 2022

NaCl exposure results in increased expression and processing of IL-1β in Meniere's disease patients.

Sci Rep 2022 03 23;12(1):4957. Epub 2022 Mar 23.

The Feinstein Institutes for Medical Research, Manhasset, NY, USA.

Meniere's disease (MD) is a chronic disease that causes episodic vertigo, fluctuating hearing loss, and aural fullness, initially managed by dietary salt reduction, and use of diuretics. Our prior research in autoimmune inner ear disease (AIED) demonstrated that in peripheral blood mononuclear cell (PBMC) from corticosteroid-resistant AIED patients, increased production, processing and release of interleukin-1β (IL-1β) is observed and hearing could be improved with use of anakinra, an interleukin-1 receptor antagonist. We have further identified that in these AIED patients, IL-1β is uniquely processed to a 28 kDa pro-inflammatory product by caspase-7. Read More

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Cervical vestibular evoked myogenic potentials and video head impulse test studies: alternative methods for detecting brainstem involvement in multiple sclerosis.

Acta Otolaryngol 2022 Feb 24;142(2):168-174. Epub 2022 Feb 24.

Department of Otorhinolaryngology, Sakarya University Training and Research Hospital, Sakarya, Turkey.

Background: Brainstem involvement (BSI) has been reported as a major predictive factor for future disability in Multiple Sclerosis (MS).

Aims/objectives: To evaluate whether Cervical Vestibular Evoked Myogenic Potentials (cVEMPs) and Video Head Impulse Test (vHIT) can be used to detect demyelinating lesions in vestibular pathways in MS.

Material And Methods: Fifty three people with MS and 40 controls were evaluated with Dizziness Handicap Inventory (DHI), vHIT and cVEMP. Read More

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February 2022

Single-Cell Analysis of Steroid-Responsive Gene Targets in the Mammalian Cochlea.

Front Neurol 2021 25;12:818157. Epub 2022 Jan 25.

Department of Otolaryngology-Head and Neck Surgery, Georgetown University School of Medicine, Washington, DC, United States.

Background: Treatment of many types of hearing instability in humans, including sudden sensorineural hearing loss, Meniere's disease, and autoimmune inner ear disease, rely heavily on the utilization of corticosteroids delivered both by oral and transtympanic routes. Despite this use, there is heterogeneity in the response to treatment with corticosteroids in humans with these diseases. The mechanisms by which corticosteroids exert their effect and the cell types in which they exert their effects in the inner ear remain poorly characterized. Read More

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January 2022

Pathogenesis and Etiology of Ménière Disease: A Scoping Review of a Century of Evidence.

JAMA Otolaryngol Head Neck Surg 2022 04;148(4):360-368

Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston.

Importance: Ménière disease is a rare chronic benign disorder of the inner ear with a natural history of multiple clinical phenotypes of variable severity and a tendency to burnout with time. Although multiple treatment modalities have been shown to improve the disease process-some adversely affecting cochleovestibular function-it remains uncertain whether one, several separate, or a combination of pathophysiologic mechanisms affect the disease process. A scoping review of the evidence underlying proposed pathophysiologic mechanisms of Ménière disease is needed to determine which processes are most likely to be etiopathogenic factors. Read More

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Cytokines and Inflammation in Meniere Disease.

Clin Exp Otorhinolaryngol 2022 Feb 8;15(1):49-59. Epub 2022 Feb 8.

Otology and Neurotology Group CTS495, Department of Genomic Medicine, Centre for Genomics and Oncological Research (GENYO), Pfizer-University of Granada-Junta de Andalucía, PTS, Granada, Spain.

Meniere disease (MD) is a rare set of conditions associated with the accumulation of endolymph in the cochlear duct and the vestibular labyrinth with a decrease of endocochlear potential. It is considered a chronic inflammatory disorder of the inner ear with a multifactorial origin. The clinical syndrome includes several groups of patients with a core phenotype: sensorineural hearing loss, episodes of vertigo, and tinnitus with a non-predictable course. Read More

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February 2022

Effectiveness of Various Treatments for Sudden Sensorineural Hearing Loss-A Retrospective Study.

Life (Basel) 2022 Jan 10;12(1). Epub 2022 Jan 10.

Institute of Sensory Organs, Nadarzyn, 05830 Warsaw, Poland.

(1) Background: A retrospective clinical study was conducted to compare the effectiveness of different pharmacological and non-pharmacological regimens for treating sudden sensorineural hearing loss (SSNHL). (2) Methods: Adult patients ( = 130) diagnosed with sudden sensorineural hearing loss (SSNHL) and hospitalized between 2015 and 2020 were enrolled in this study. Depending on the treatment regimen applied, patients were divided into five groups. Read More

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January 2022

Susac's syndrome diagnostic difficulties - the neurological point of view.

Neurol Neurochir Pol 2022 5;56(2):141-147. Epub 2022 Jan 5.

Department of Neurology, Bielański Hospital, Warsaw, Poland.

Susac's syndrome is a rare microangiopathy affecting small vessels of the retina, inner ear and brain. It is characterised by a triad of symptoms: encephalopathy, visual defects, and sensorineural hearing loss. The disease is probably caused by an autoimmune process. Read More

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Cochlear implant in immune mediated inner ear diseases: Impedance variations and clinical outcomes.

Cochlear Implants Int 2022 Mar 30;23(2):70-79. Epub 2021 Nov 30.

Department of Sense Organs, "Sapienza" University of Rome, Rome, Italy.

Objective: Immune-mediated inner ear disease (IMIED) might cause severe/profound hearing loss and these patients are considered ideal candidates to cochlear implant (CI) surgery. The aim of the study was to evaluate impedance changes over time.

Method: The Study Group (SG) was composed of CI IMIED patients (31 ears) and a Control Group (CG) of CI patients with hearing loss not related to their immune system (31 ears). Read More

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Cochleovestibular involvement in patients with Fabry disease: data from the multicenter cohort FFABRY.

Eur Arch Otorhinolaryngol 2022 Mar 26;279(3):1639-1644. Epub 2021 Nov 26.

Internal Medicine Department, Hotel-Dieu University Hospital, Nantes, France.

Purpose: Fabry disease (FD) is a lysosomal storage disease responsible for cochleovestibular involvement. Exact prevalence and pathophysiological mechanisms behind ENT affections are still poorly known. Treating FD with enzyme replacement therapy (ERT) does not seem to significantly improve the ENT symptoms, while the impact of migalastat has yet to be determined. Read More

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Unilateral Sensorineural Hearing Loss in Children Associated With Sjögren's Syndrome.

Cureus 2021 Oct 17;13(10):e18832. Epub 2021 Oct 17.

Pediatrics, Oita University School of Medicine, Yufu, JPN.

The occurrence of unilateral sensorineural hearing loss (SNHL) during school age is relatively rare and accounts for approximately 6% of all deafness in childhood. We present two cases involving children who were diagnosed with SNHL associated with Sjögren's syndrome (SS). Case 1: An eight-year-old girl with an approximately two-year clinical history of gradual hearing loss was diagnosed with SNHL associated with SS based on histological findings of inflammation in the salivary glands and the presence of serum anti-Sjögren's syndrome-A antibody. Read More

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October 2021

LCCL peptide cleavage after noise exposure exacerbates hearing loss and is associated with the monocyte infiltration in the cochlea.

Hear Res 2021 12 23;412:108378. Epub 2021 Oct 23.

Department of Otorhinolaryngology, Graduate School of Medical Science, Brain Korea 21 Project, Yonsei University College of Medicine, Seoul, Republic of Korea. Electronic address:

Acoustic trauma induces an inflammatory response in the cochlea, resulting in debilitating hearing function. Clinically, amelioration of inflammation substantially prevents noise-induced hearing loss. The Limulus factor C, Cochlin, and Lgl1 (LCCL) peptide plays an important role in innate immunity during bacteria-induced inflammation in the cochlea. Read More

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December 2021

Central positional nystagmus: an update.

J Neurol 2022 Apr 20;269(4):1851-1860. Epub 2021 Oct 20.

Department of Neurology and German Center for Vertigo and Balance Disorders, Hospital of the Ludwig Maximilians University Munich, Munich, Germany.

Clinically, central positional nystagmus (CPN) is often suspected when atypical forms of its peripheral counterpart, i.e., benign paroxysmal positional vertigo (BPPV), are observed, namely a linear horizontal nystagmus as in horizontal canal BPPV or a downwardly and torsionally beating nystagmus as in anterior canal BPPV. Read More

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Bilateral sudden sensorineural hearing loss in patient with Behçet disease.

Ear Nose Throat J 2021 Oct 18:1455613211051660. Epub 2021 Oct 18.

Department of Otolaryngology-Head and Neck Surgery, 63452Tri-Service General Hospital, Taipei, Taiwan.

Approximately 80% to 85% of sudden sensorineural hearing loss (SSNHL) is idiopathic, but immune-mediated mechanisms are thought to be involved. Behçet disease is an autoinflammatory vasculitis that may involve vessels of the inner ear. It can cause sensorineural hearing loss (SNHL) and an increased risk of SSNHL. Read More

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October 2021

Rifle Blast Exacerbating Hearing Loss in a Patient Treated With Teprotumumab for Thyroid Eye Disease.

Ophthalmic Plast Reconstr Surg 2022 Mar-Apr 01;38(2):e41-e43

Texas Oculoplastics Consultants (TOC) Eye & Face, Austin, Texas, U.S.A.

Teprotumumab is a promising new immunomodulatory therapy for thyroid eye disease. One unique adverse effect observed in clinical trials was hearing impairment; however, all adverse auditory effects in prior clinical trials resolved spontaneously after completion of teprotumumab therapy. The authors present a case of a patient on teprotumumab for thyroid eye disease who experienced sustained hearing loss secondary to shooting a rifle without ear protection. Read More

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Rapidly progressive hearing loss occurring over weeks: review of differential diagnoses.

J Laryngol Otol 2022 Aug 13;136(8):775-782. Epub 2021 Oct 13.

Department of Otolaryngology - Head and Neck Surgery, Sunderland Royal Hospital, Sunderland, UK.

Background: Sudden hearing loss, or progressive hearing loss occurring over months to years, are well-established presentations. However, little is described in the medical literature on how to approach patients presenting with a rapidly progressive hearing loss occurring over weeks. This study aimed to evaluate the clinical significance of patients presenting with rapidly progressive hearing loss. Read More

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[Analysis of clinical characteristics and risk factors in patients with benign paroxysmal positional vertigo].

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2021 Oct;35(10):905-909

The Third District,Department of Neurology,the First People's Hospital of Chenzhou City,Chenzhou,423000,China.

〓 Objective: To analyze the clinical characteristics of patients with benign paroxysmal positional vertigo, summarize experience for diagnosis and treatment activities and to explore the risk factors related to the onset of BPPV and provide reference for early intervention to reduce the risk factor exposure of BPPV. One hundred and twelve patients with BPPV were included in the study, and clinical data including age, gender, onset symptoms and duration, past medical history and family history were analyzed. A One-way ANOVA was performed on 16 variables using age, gender, diabetes, hyperuric acid, hyperlipidemia, osteoporosis, hypertension, coronary heart disease, stroke, Meniere's disease, suppurative otitis media, vestibular neuronitis, sudden deafness, head injury, ear nose/maxillofacial surgery and autoimmune thyroiditis. Read More

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October 2021

Construction of an MRI-based decision tree to differentiate autoimmune and autoinflammatory inner ear disease from chronic otitis media with sensorineural hearing loss.

Sci Rep 2021 09 27;11(1):19171. Epub 2021 Sep 27.

Department of Radiology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, 82, Gumi-ro 173 Beon-gil, Bundang-gu, Seongnam, 13620, Republic of Korea.

Autoimmune and autoinflammatory inner ear diseases (AIED/AID) are characterized by the symptom of sensorineural hearing loss (SNHL). To date, standardized diagnostic tools for AIED/AID are lacking, and clinically differentiating AIED/AID from chronic otitis media (COM) with SNHL is challenging. This retrospective study aimed to construct a magnetic resonance imaging (MRI)-based decision tree using classification and regression tree (CART) analysis to distinguish AIED/AID from COM. Read More

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September 2021

Clinical and Cytokine Profile in Patients with Early and Late Onset Meniere Disease.

J Clin Med 2021 Sep 7;10(18). Epub 2021 Sep 7.

Otology and Neurotology Group CTS495, Department of Genomic Medicine, GENYO-Centre for Genomics and Oncological Research-Pfizer/University of Granada/Junta de Andalucía, PTS, 18016 Granada, Spain.

Background: Meniere disease (MD) is an inner ear disorder associated with comorbidities such as autoimmune diseases or migraine. This study describes clinical and cytokine profiles in MD according to the age of onset of the condition.

Methods: A cross-sectional study including 83 MD patients: 44 with early-onset MD (EOMD, <35 years old), and 39 with late-onset MD (LOMD, >50 years old), 64 patients with migraine and 55 controls was carried out. Read More

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September 2021

Audiological Patterns in Patients with Autoimmune Hearing Loss.

Audiol Neurootol 2022 10;27(4):336-346. Epub 2021 Sep 10.

1st Academic ENT Department, Aristotle University of Thessaloniki, AHEPA Hospital, Thessaloniki, Greece.

Introduction: The aim of this study was to illustrate clinical and audiological patterns of hearing impairment in patients with autoimmune hearing loss (AIHL).

Methods: Fifty-three patients with AIHL were retrospectively recruited, and a tapering schema of steroid treatment was administered in all these patients. The diagnosis of AIHL was essentially based on clinical symptoms, such as recurrent, sudden (sensorineural hearing loss [SSHL]), fluctuating, or quickly progressing (<12 months) SSHL (uni-/bilateral), in association with the coexistence of autoimmune diseases, high antinuclear antibodies (ANA) and the presence of human leukocyte antigen (HLA) B27, B35, B51, C04, and C07. Read More

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Cyclophosphamide inhibits the progression of Meniere's disease by reducing the generation of circulating immune complex.

Exp Ther Med 2021 Oct 13;22(4):1177. Epub 2021 Aug 13.

Department of Otolaryngology, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, Inner Mongolia 010110, P.R. China.

Endolymphatic hydrops is a characteristic pathological manifestation of Meniere's disease (MD) that has been previously associated with autoimmunity. Interest in the circulating immune complex (CIC) has increased due to its reported role in the occurrence of MD. The present study aimed to investigate the potential value of serum CIC concentration in the diagnosis of MD and the therapeutic potential of cyclophosphamide (CTX) for the treatment of MD. Read More

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October 2021