Bidirectional Transport of IgE by CD23 in the Inner Ear of Patients with Meniere's Disease.

Meniere's disease (MD) is a disorder of the inner ear characterized by episodes of spontaneous vertigo, fluctuating hearing loss, and tinnitus. Recent studies have demonstrated that IgE may play a role in the pathogenesis of MD. Patients with MD (n = 103), acoustic neuroma (n = 5), and healthy subjects (n = 72) were recruited into the study. Serum from the participants was analyzed for IgE and type 2-related cytokines. IgE and CD23 expression levels in vestibular end organs of patients, C57BL/6 mice, or mouse HEI-OC1 cells were analyzed. Finally, the role of CD23 in IgE transcytosis was assessed using HEI-OC1 cells. Serum IgE was elevated in patients with MD and positively correlated with clinical symptoms. IL-4, IL-5, IL-10, IL-13, and CD23 levels were increased in patients with MD compared with the control group. In the transcytosis assay, mouse IgE was found to be bidirectionally transported across the HEI-OC1 cell monolayer. Additionally, CD23 downregulation using a small interfering RNA approach significantly reduced the efficiency of IgE transcytosis, suggesting that IgE is transported by CD23. Furthermore, exposure to IL-4 increased CD23 expression and enhanced IgE transcytosis in the HEI-OC1 cells and primary vestibular end organs. Our study indicated that IgE may play a role in the pathophysiology of MD. In addition, CD23-mediated IgE transcytosis in the hair cells may play a critical role in initiating inflammation in the inner ear. Thus, reducing the level of IgE may be a potentially effective approach for MD treatment.

High level of IgE in acute low-tone sensorineural hearing loss: A predictor for recurrence and Meniere Disease transformation.

BACKGROUND: Autoimmunity may play an important role in sudden onset sensorineural hearing loss. However, little is known about the relationship between immunoglobulin E (IgE) and acute low-tone sensorinerual hearing loss (ALHL). OBJECTIVES: To investigate the relationship between IgE level and endolymphatic hydrops and outcomes of ALHL. METHODS: A total of 242 subjects with sudden onset hearing loss, including 115 with ALHL and 127 with idiopathic sudden sensorineural hearing loss (ISSHL), were included in this study. Peripheral venous blood samples of 242 subjects were collected for detection. Clinical data, IgE level, and distribution of allergens were compared between the ALHL and ISSHL groups. The ALHL group received an electrocochleogram (ECochG) test and a follow-up in the outpatient unit or by telephone to evaluate outcomes. RESULTS: Compared to the values in the ISSHL group, a significantly younger onset age (42.30±14.33 years old), higher female onset proportion (72/115, 62.61%), increased total IgE level (median: 66.47, interquartile range: 24.56, 180.96, IU/mL) and specific IgE level (median: 9.42, interquartile range: 1.42, 22.23 IU/mL) were noted in the ALHL group. A clear difference in allergen distribution was noted between the ALHL and ISSHL groups (p=.001). Total IgE and specific IgE levels were factors that contributed to the SP/AP ratio in the electrocochleogram (ECochG) (R2=0.413) in ALHL group. Finally, during the follow-up (17.61±3.46 months) for the ALHL group, 37 subjects recurred, and 17 subjects developed Meniere Disease. In the ROC curve for ALHL recurrence, the area under the curve (AUC) of total IgE was 0.709 and that of specific IgE was 0.679. For MD transformation, the AUC of total IgE was 0.736 and that of specific IgE was 0.716. CONCLUSIONS: High IgE levels correlated with an enhanced SP/AP ratio in ALHL. High IgE levels could be used as a predictor of ALHL recurrence and MD transformation.

Current Understanding and Clinical Management of Meniere's Disease: A Systematic Review.

Meniere's disease (MD) is a set of uncommon disorders with core phenotype of tinnitus, episodic vertigo, and sensorineural hearing loss. MD shows a genetic predisposition and a family history is found in 10% cases, with an autosomal dominant inheritance pattern. It is a multifactorial condition whose onset and development are triggered by the combined effect of genetic and environmental factors. Histopathological studies have associated MD with the accumulation of endolymph in the cochlea and the vestibular organs. However, endolymphatic hydrops does not fully explain the persistence of tinnitus, hearing loss progression, or the frequency of vertigo attacks.There are several comorbidities associated with MD, such as migraine, anxiety, autoimmune, and autoinflammatory disorders, adding more complexity to the phenotype. This "extended phenotype" can make the diagnosis and clinical management more complex, but it could also lead to a better characterization, understanding, and treatment of MD patients.We have conducted a systematic review on MD to update current knowledge, focusing on its mechanisms, diagnosis, comorbidities, and practical management.

Successful treatment of a child with definite Meniere's disease with the migraine regimen.

Emerging evidence suggests substantial overlap between the symptoms of Meniere's disease (MD) and migraine-related cochlear/vestibular disorders. We report a 5-year-old girl with a 6-month history of left-sided hearing loss followed by daily episodes of vertigo, headache, and vomiting who met the criteria for definite MD. The patient became symptom-free and gained near normal hearing levels after starting on a 6-week migraine diet/lifestyle regimen with riboflavin and magnesium. We believe that the symptoms of MD may be primarily due to a vestibular migraine phenomenon. Pediatric MD patients may benefit from migraine lifestyle/dietary changes with control of both cochlear and vestibular symptoms.

Clinical Assessments of Balance in Adults with Concussion: An Update.

Postural instability is a cardinal indicator of concussion. Assessments of the postural control system range from clinical to laboratory tests that assess the balance of the individual. In a previous article regarding clinical assessment of balance in adults with concussion, we reviewed the importance of balance as a component in concussion evaluations. The purpose of this review article is to update the information previously published in 2014. Since 2014, research has provided evidence for the incorporation of dynamic methods for evaluating balance postconcussion with particular emphasis on sensory system integration and dual tasking. Therefore, this review will examine the current state of knowledge on how concussion injuries affect postural control, advancements in evaluating balance postconcussion, such as novel eye-tracking techniques, and current recommendations for best practices for balance assessment.

[Vascular mechanisms in Meniere's disease]. / Sosudistye mekhanizmy v razvitii bolezni Men'era.

Meniere's disease (MD) is chronic multifactorial medical condition caused by endolymphatic hydrops, which etiology is unclear. This review highlights possible vascular mechanisms of MD. Impairment of vascular regulation, further ischemic damage of labyrinth and venous drainage pathology could lead to endolymphatic hydrops. Epidemiologic studies reveal high comorbidity of MD and migraine. Both diseases could be the result of trigeminovascular dysfunction. Betahistine, the medication with vascular effect, is widely used in treatment of MD, the effectiveness of calcium channel blockers is evaluated. Keywords: vertigo, Meniere's disease, endolymphatichydrops, migraine, vascular mechanisms, betahistine.

Diagnostic yield of MRI of the brain and IAC in patients with neurotologic complaints.

OBJECTIVES: Quantify number of MRI scans obtained in a tertiary neurotology practice and identify likelihood of pathologic findings. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary neurotology center. SUBJECTS AND METHODS: A retrospective analysis of all adult patients over 20 months (3/2012-10/2013) where MRI was deemed necessary for evaluation of neurotologic complaints. Demographics, clinical history, physical examination, and audiometric findings were used to categorize new patients into 7 groups: definite Meniere's disease (MD), probable MD, possible MD, vague dizziness, tinnitus only, asymmetric hearing loss (HL), and other symptoms to stratify risk for retrocochlear tumor and other relevant pathology. RESULTS: 1537 MRI scans were performed, 932 of these were for a new diagnosis. Discovering retrocochlear tumors was rare (1.4%). Patients with HL had a 0.3% (1/314) chance of retrocochlear tumor and 3.2% (10/314) chance of relevant pathology. Patients with only unilateral tinnitus had no evidence of retrocochlear tumors, and 3.8% chance of finding relevant pathology. Patients with "definite" or "probable" MD had no evidence of retrocochlear tumor or other relevant findings. All discovered acoustic neuromas were in the "possible MD" category, which had a 9.3% chance of finding all relevant pathology. CONCLUSIONS: In a tertiary neurotology center, the likelihood of finding a retrocochlear tumor on MRI is rare. In the current study, unilateral tinnitus exclusively, "definite MD," and "probable MD" failed to yield a single example of retrocochlear tumor. Patients with "possible MD" had the highest probability of finding retrocochlear tumors and other relevant pathology.

[Diagnostic criteria for Menière's disease according to the Classification Committee of the Bárány Society]. / M. Menière: Diagnostische Kriterien des Internationalen Klassifikationskomitees der Bárány-Gesellschaft.

This paper presents diagnostic criteria for Menière's disease jointly formulated by the Classification Committee of the Bárány Society, The Japan Society for Equilibrium Research, the European Academy of Otology and Neurotology (EAONO), the Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) and the Korean Balance Society. The classification includes two categories: definite Menière's disease and probable Menière's disease. The diagnosis of definite Menière's disease is based on clinical criteria and requires the observation of an episodic vertigo syndrome associated with low- to medium-frequency sensorineural hearing loss and fluctuating aural symptoms (hearing, tinnitus and/or fullness) in the affected ear. Duration of vertigo episodes is limited to a period between 20 min and 12 h. Probable Menière's disease is a broader concept defined by episodic vestibular symptoms (vertigo or dizziness) associated with fluctuating aural symptoms occurring in a period from 20 min to 24 h.

Intralabyrinthine schwannomas : Surgical management and hearing rehabilitation with cochlear implants.

Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss and vertigo. In an own case series of 12 patients, 6 tumors showed an intracochlear, 3 an intravestibular, 1 a transmodiolar including the cerebellopontine angle (CPA), 1a transotic including the CPA, and 1 a multilocular location. The tumors were removed surgically in 9 patients, whereas 3 patients decided for a "wait-and-test-and-scan" strategy. Of the surgical patients, 3 underwent labyrinthectomy and cochlear implant (CI) surgery in a single-stage procedure; 1 patient had extended cochleostomy with CI surgery; 3 underwent partial or subtotal cochleoectomy, with partial cochlear reconstruction and CI surgery (n = 1) or implantation of electrode dummies for possible later CI after repeated MRI follow-up (n = 2); and in 2 patients, the tumors of the internal auditory canal and cerebellopontine angle exhibiting transmodiolar or transmacular growth were removed by combined translabyrinthine-transotic resection. For the intracochlear tumors, vestibular function could mostly be preserved after surgery. In all cases with CI surgery, hearing rehabilitation was successful, although speech discrimination was limited for the case with subtotal cochleoectomy. Surgical removal of intracochlear schwannomas via partial or subtotal cochleoectomy is, in principle, possible with preservation of vestibular function. In the authors' opinion, radiotherapy of ILS is only indicated in isolated cases. Cochlear implantation during or after tumor resection (i. e., as synchronous or staged surgeries) is an option for hearing rehabilitation in cartain cases and represents a therapeutic approach in contrast to a "wait-and-test-and-scan" strategy.

Acute vestibular syndrome in cerebellar stroke : A case report and review of the literature.

The current paper reports on a patient with recurrent rotational vertigo and persistent dizziness and imbalance lasting several weeks, who underwent extensive neuro-otological and radiological examinations. Pathological findings initially included right-sided benign paroxysmal positional vertigo (BPPV), persistent horizontal spontaneous nystagmus (SPN) to the left, and a pathological bedside and video head impulse test (HIT) on the left. The pathological HIT on the left and the SPN to the left indicated a central origin. Therefore, cranial magnetic resonance imaging was performed which revealed a left-sided ischemic stroke in the territory of the medial branch of the posterior inferior cerebellar artery (mPICA).

[Recent surgical options for vestibular vertigo]. / Aktuelle operative Therapie des vestibulären Schwindels.

Vertigo is not a well defined symptom but a heterogenous entity diagnosed and treated mainly by otolaryngologists, neurologists, internal medicine and primary care physicians. Most vertigo syndroms have a good prognosis and management is predominantly conservative, whereas the need for surgical therapy is rare, but for a subset of patients often the only remaining option. In this paper, we describe the development of surgical therapy for hydropic inner ear diseases, Menière disease, dehiscence syndroms, perilymphatic fistulas, and benign paroxysmal vertigo. At the end, we shortly introduce the most recent development of vestibular implants. Surgical vestibular therapy is still indicated for selected patients nowadays when conservative options did not reduce symptoms and patients are still suffering. Success depends on the correct diagnosis and indication for the different procedures going along with an adequate patient selection. In regard to the invasiveness and the possible risks due to the surgery, in depth individual counselling is necessary. Ablative and destructive surgical procedures usually achieve a successful vertigo control, but go along with a high risk for hearing loss. Therefore, residual hearing has to be included in the decission making process for a surgical therapy.

The physiologic role of corticosteroids in Ménière's disease.

Multiple options exist to manage Ménière's disease (MD), ranging from dietary modifications to ablative surgery. Corticosteroids (CS) have long been used to manage MD, but their exact mechanism for disease alleviation is relatively uncertain. Glucocorticoid receptors have been shown to exist in the human inner ear and several studies propose they influence mechanisms of blood flow, fluid regulation, and ion regulation, with recent evidence describing the latter two. Corticosteroids have been shown to upregulate aquaporins and ion channels in the inner ear, and may have a positive effect on labyrinthine blood flow. Additionally, processes have been described in genomic and non-genomic manners. This text will review the literature on the actions of CS on the inner ear relevant to MD.

[The current problems related to Meniere's disease]. / Sovremennye problemy bolezni Men'era.

Over 150 years have elapsed since Prosper Meniere's described for the first time the clinical symptoms of the disease that is called by his name at the present time. Nevertheless, the etiology of this pathological condition remains unknown. It has been believed during many decades that the cause of Meniere's disease Is the presence of an excess fluid (hydrops) in the endolymphatic space of the inner ear leading to the rupture of membranes responsible for hearing impairment and vestibular disorders. The recent histopathological studies have demonstrated that endolymphatic hydrops (RH) is not necessarily associated with the clinical manifestations of Meniere's disease. During the last decades, certain authors have undertaken attempts to elaborate new theories of etiology, pathogenesis, and mechanisms underlying the development of dizziness associated with Meniere's disease. These are the theory of genetic predisposition to this condition, the viral and autoimmune theories, and the theory of disturbance of the longitudinal endolymph flow. However, none of these theories was adequately substantiated. The ongoing experimental and morphological studies appear to give evidence that the aquaporin water channels may play a role in the formation of hydrops. Moreover, the mechanisms underlying episodes of dizziness remain to be elucidated. In the light of recent progress in normal physiology and pathological physiology of the ear, the theory of ruptures proposed by H. Schuknecht tends to be considered increasingly less attractive and appears to be replaced by new hypotheses providing an alternative explanation for the mechanism behind the development of Meniere's disease giving priority to the role of contamination of perilymph with calcium ions.

Jugular bulb abnormalities in patients with Meniere's disease using high-resolution computed tomography.

Temporal bone abnormalities such as hypoplasia of the vestibular aqueduct or hypopneumatization of the mastoid have been described in Meniere's disease (MD). Jugular bulb abnormalities (JBA) are one of the most common temporal bone abnormalities. The aim of this study was to evaluate the frequency of JBA in MD. Radiological data obtained by temporal bone high-resolution computed tomography of 200 ears of 167 MD patients (MD group) and 218 ears of 109 patients with normal inner ear function (control group) were analyzed retrospectively. The frequencies of high jugular bulb (HJB), jugular bulb diverticulum (JBD), inner ear adjacent jugular bulb (IAJB) and jugular bulb related inner ear dehiscence (JBID) were evaluated and compared between MD group and control group. IAJB was differentiated into vestibular aqueduct adjacent jugular bulb (VAAJB), cochlear aqueduct adjacent jugular bulb and posterior semicircular canal adjacent jugular bulb. JBID was further analyzed by differentiating into jugular bulb related vestibular aqueduct dehiscence (JBVAD), jugular bulb related cochlear aqueduct dehiscence and jugular bulb related posterior semicircular canal dehiscence. The frequencies of HJB, JBD and IAJB were higher in MD group compared to control group (21, 13.3 %, p = 0.036; 8.5, 3.7 %, p = 0.037; 13.5, 4.6 %, p = 0.001). No differences between both groups were seen in JBID (4.0, 2.3 %, p = 0.315). Most IAJB and JBID were seen in VAAJB and JBVAD. There is a higher frequency of JBA in patients with MD than in patients without inner ear symptoms. Temporal bones of MD patients might be constituted anatomically different, carrying predisposing factors for the development of clinically apparent MD.

[Menière's Disease]. / Morbus Menière.

Menière`s disease is one of the most common inner ear and vestibular disorders. It is defined as the idiopathic syndrome of endolymphatic hydrops (ELH). Despite the development of several different animal models of ELH, its etiology and pathogenesis is still unresolved. In humans, endolymphatic hydrops may occur spontaneously or as a consequence of specific disorders with distinct inner ear pathologies, e. g., infectious labyrinthitis, noise induced hearing loss or vestibular schwannoma. Recent imaging studies using MRI have shown that hydropic ear disease is associated not only with the full triad of vertigo, hearing, loss and tinnitus/aural pressure, but also with inner ear symptoms that do not fulfill the clinical criteria of definite Menière's disease as set forth by the AAO-HNS. Therefore, terms like "atypical" or "cochlear"/"vestibular" Menière's disease or "forme fruste" should be avoided and the term "Menière's disease" should universally be applied according only to these guidelines. Besides that, the recent possibility of visualizing endolymphatic hydrops on MRI and thereby ascertaining the diagnosis in difficult cases and new audiovestibular function tests for the indirect detection of endolymphatic hydrops show promising results. Evidenced based reviews of currently available therapeutic options still reveal many uncertainties with regard to efficacy, with the exception of the ablative therapies, e. g., intratympanic gentamicin application.

The role of caffeine in otorhinolaryngology: guilty as charged?

Caffeine is implicated as causing or aggravating numerous otorhinolaryngological conditions, including tinnitus, Ménière's disease, laryngopharyngeal reflux, globus pharyngeus and dysphonia. We address caffeine's effects in such conditions and to determine whether such implications are founded. The defined search limits of data sources included human trials and either randomised control trials, meta-analyses, editorials, letters, clinical trials, case reports, comments or journal articles over the last 40 years. MEDLINE, EMBASE and CINAHL databases were searched using 'otorhinolaryngological diseases' and 'caffeine' as a duplicate filter. PubMed databases were searched using 'caffeine' in combination with 'tinnitus', 'Ménière's', 'vertigo', 'motion sickness', 'imbalance', 'vestibular migraine', 'voice', 'vocal hygiene', 'reflux', 'ear', 'nose', 'throat' and 'head neck cancer', respectively. Searches were not limited to the English language. MEDLINE, EMBASE and CINAHL database searches identified 417 papers. Of these, 200 abstracts were chosen for further scrutiny, following which 30 full manuscripts were chosen for full review. The PubMed database search identified 275 abstracts of which 33 were reviewed. Of the total 692 studies searched, 63 studies were reviewed and 36 were finally used. At present, there is little evidence in the literature to support the notion that caffeine causes or aggravates otorhinolaryngological conditions. In tinnitus, its withdrawal may actually worsen symptoms whereas in motion sickness, there is some clinical evidence for its benefit. More research is needed into the role caffeine plays in otorhinolaryngological conditions to allow clinicians to give informed advice to their patients.

[Differential diagnosis "vertigo and dizziness"]. / Differenzialdiagnose "Schwindel"

Vertigo and dizziness are symptoms of interdisciplinary dimension. However, the differentiation and classification of vertigo syndromes also require experience and multidisciplinary knowledge. Since the clinical syndrome is subjective, a detailed analysis of the complaints underlying is required. International disease definitions are an indispensable tool in the differential diagnosis of vertigo syndromes today. With simple diagnostic tools eye movement disorders and nystagmus can be examined and assigned to specific vestibular disorders today. Screening tests (e.g. head impulse test) are now an important instrument in the investigation of patients with vertigo syndromes in case of emergency. With objective diagnostic methods (caloric irrigation, video head impulse test, vestibular evoked myogenic potentials) the degree of functional impairment of the five vestibular receptors can be assessed quantitatively. Furthermore, in vestibulopathies, a receptor and side-specific diagnostic assessment can be performed even with regard to dynamic aspects.

[Cochleovestibular disorders in the patients suffering from migraine of the basilar type].

The objective of the present study was to improve diagnostics of basilar type migraine (BTM) based on the results of neurological, vestibulometric, and audiological studies of 11 patients. Peculiarities of the clinical picture of this disease and the medical histories of these patients are described. The pathogenetic role of labyrinthine hydrops and the difficulties encountered in diagnostics of Meniere's disease are discussed.

Association between polymorphisms in genes encoding methylenetetrahydrofolate reductase and the risk of Ménière's disease.

Folate metabolism is essential for cellular functioning. Despite extensive research on the roles of folate-metabolism-related gene polymorphisms in the pathophysiology of many diseases, such as cardiovascular disease, cancers, and sudden sensorineural hearing loss, little is known about their association with Ménière's disease (MD). The aim of this study was to investigate the effect of methylenetetrahydrofolate reductase (MTHFR) gene polymorphisms (C677T and A1298C) on the risk of MD in a Japanese population. We examined the C677T and A1298C (rs1801133 and rs1801131) polymorphisms in the MTHFR gene and compared them between 1946 adults (986 men and 960 women) participating in the National Institute for Longevity Sciences Longitudinal Study of Aging and 86 cases of MD. A multiple logistic regression was performed to obtain odds ratios (ORs) for the risk of MD regarding the MTHFR polymorphisms before (model 1) and after (model 2) adjustment for age and sex factors. The OR of MTHFR C677T for the risk of MD was 0.669 (95% confidence interval [CI], 0.479-0.934) in model 1 and 0.680 (95% CI, 0.484-0.954) in model 2. In contrast, the OR of MTHFR A1298C for the risk of MD was 1.503 (95% CI, 1.064-2.123) in model 1 and 1.505 (95% CI, 1.045-2.167) in model 2. Our results imply that the MTHFR C677T and A1298C polymorphisms are associated with the risk of MD.

Ménière disease and gluten sensitivity: recovery after a gluten-free diet.

We report the case of a 63-year-old female with definite unilateral Ménière disease, osteoarthritis of the distal finger joints with mucous cysts and Heberden's nodes, and constipation with recurrent abdominal pain whose symptoms remitted after 6months of a restrictive gluten-free diet.