Autoimmune Hearing Loss: A Diagnostic Challenge.

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 [...

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

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.

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

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.

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

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.

Assessment of Metabolic Markers and Osteoporosis in 250 Patients with Superior Semicircular Canal Dehiscence Treated With Middle Fossa Craniotomy.

Background: Superior semicircular canal dehiscence (SSCD) is caused by bony defects in the osseous shell of the arcuate eminence separating the labyrinth and the intracranial space. This pathologic third window causes hydroacoustic transmission resulting in debilitating symptoms. We examine the pathophysiologic association between metabolic markers, previous medical history, and SSCD symptoms before and after middle fossa craniotomy (MFC) treatment.

Acupuncture in the treatment of Dry Eye Syndrome with anxiety symptoms. A case report.

Dry Eye Syndrome (DES) is an eye disorder caused by alteration of production or composition of tear film. Symptoms include dry eye dryness, burning, itching and pain, and often is associated with emotional stress, anxiety and depression. Evidence of the effectiveness of acupuncture to treat DES has recently appeared in literature but not on acupuncture treatment of DES related anxiety or depression symptoms.

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

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.

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

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.

Autoimmune Vestibulopathy-A Case Series.

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.

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

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.

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

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.

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

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.

Cytokines and Inflammation in Meniere Disease.

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.

Effects of primary Sjögren's syndrome on hearing and vestibular systems.

Objective: This study aimed to evaluate primary Sjögren's syndrome patients in terms of hearing and vestibular functions.

Methods: The patient group consisted of 35 individuals diagnosed with primary Sjögren's syndrome and a control group of 35 healthy individuals similar in terms of age and gender.

Results: The rate of hearing loss in the patient group was significantly higher than in the control group ( = 0.

Labyrinthine calcification in ears with otitis media and antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV): A report of two cases.

Otitis media with antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV) has been proposed as a new type of otitis media. The hearing loss caused by OMAAV can be expected to improve with early detection and intervention, but if it continues to worsen and leads to deafness, it is challenging to recover the patient's hearing. When bilateral deafness occurs, cochlear implant (CI) surgery is the only way to improve hearing.

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

(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.

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

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.

Cochlear Implantation and Perioperative Management in Autoimmune Inner Ear Disease: A Systematic Review and Meta-Analysis.

Objective: To assess outcomes in autoimmune inner ear disease (AIED) after cochlear implantation (CI) through systematic review and meta-analysis.

Databases Reviewed: PubMed, MedLine, Embase, and CINAHL.

Methods: Databases were queried for inclusion of AIED patients who underwent CI with outcomes recorded ≥3 months postoperatively.

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

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).

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

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.

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

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.