Ménière's disease is a disorder of the inner ear.

PURPOSE OF REVIEW: Ménière's disease is a disorder characterized by recurrent episodes of vertigo, hearing loss, tinnitus, and pressure in the ear. Its pathogenesis and optimal management continue to be the subject of ongoing debate. Although it is classically believed to be a primary disorder of the inner ear, some clinicians have hypothesized that it is a form of vestibular migraine. RECENT FINDINGS: Evidence supporting vestibular migraine in the differential of Ménière's disease includes overlap of symptoms, high prevalence of migraines in patients with Ménière's disease, and the efficacy of migraine treatments for symptoms of Ménière's disease in some patients. However, there is far more evidence to support Ménière's disease as a disorder of the inner ear, including genetic and histologic changes of hydrops, imaging studies showing hydropic changes, predominance of low-frequency hearing loss, clinical efficacy of treatments targeted at inflammatory change/hydrops, and clinical efficacy of ablating the vestibular end-organ. SUMMARY: Although there is conflicting evidence regarding the cause and treatment of Ménière's disease, current evidence favors it as a disorder of the inner ear. Further research is needed to fully understand the mechanisms of Ménière's disease and how to improve diagnosis and treatment outcomes.

Infectious causes and mimickers of meniere's disease.

PURPOSE OF REVIEW: To review the evidence of a possible viral etiology of Meniere's disease, the role of antiviral therapy, as well as other infectious disease processes that may mimic Meniere's disease. Better understanding of the etiology of Meniere's disease and the role of various infectious disease processes may allow for more effective diagnosis and management. RECENT FINDINGS: There is evidence that viral infections - to Herpes simplex virus, cytomegalovirus, Epstein Barr virus, influenza, adenovirus, Coxsackie virus B, and Varicella Zoster Virus - may be involved in the development of Meniere's disease, however the evidence is inconsistent and the underlying mechanism remains theoretical. Nevertheless, antiviral therapy may be effective for a subset of patients with Meniere's disease. Lastly, other infectious diseases including Lyme disease and syphilis can present with symptoms similar to Meniere's disease. It is important to discern these from Meniere's disease to determine the appropriate treatment. SUMMARY: There is a paucity of high-quality evidence to support a viral etiology of Meniere's disease, and the current evidence appears circumstantial and inconsistent. Additional research is necessary to establish the mechanism and causative pathogens. Antiviral therapy may provide therapeutic benefit for a subset of patients with Meniere's disease. Furthermore, clinicians should be aware of other infectious mimics of Meniere's disease and include them in the differential diagnosis of patients presenting with Meniere's-like symptoms. Research on this subject continues to evolve, and data from these studies represent a growing repository of evidence that can be used to guide clinical decision-making.

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.

Cerebellopontine Angle and Internal Auditory Canal Lipomas: Case Series and Systematic Review.

OBJECTIVES/HYPOTHESIS: Cerebellopontine angle (CPA) and internal auditory canal (IAC) lipomas are rare, benign tumors comprising 0.08% of all intracranial tumors and can be mistaken for other, more common lesions of the CPA/IAC such as vestibular schwannoma. The purpose of this study was to review the literature and assess the evolution of CPA/IAC lipoma diagnosis and management. In addition, we present 17 new lipomas, matching the largest known case series of this rare tumor. STUDY DESIGN: Retrospective case series and systematic review. METHODS: Systematic review of the literature was performed using PubMed and Google Scholar. References from identified articles were also reviewed to identify potential additional manuscripts. Manuscripts and abstracts were reviewed to identify unique cases. For the case series, the charts of all CPA/IAC lipoma patients seen at a single institution from 2006-2019 were manually reviewed. Logistic regression and chi-squared analysis were performed where appropriate. RESULTS: A total of 219 unique lipomas have been reported in the literature, including 17 presented in this study. Surgical management has been performed in 46% of cases and has been conducted less often in recent decades, likely due to improved radiographic diagnostic capabilities and understanding of surgical outcomes. Surgical management is associated with worse neurologic outcomes (P = .002) and has become less common in recent decades. Although growth is unlikely, it has been demonstrated in patients into their 30s. CONCLUSIONS: Accurate radiographic diagnosis is imperative for appropriate patient management, as CPA/IAC lipomas should typically be managed through observation and serial imaging whereas vestibular schwannomas and other CPA/IAC lesions may require microsurgical or radiosurgical intervention depending on growth and symptomatology. Laryngoscope, 131:2081-2087, 2021.

Incidence of Developing Contralateral Ménière's Disease in Patients Undergoing Transmastoid Labyrinthectomy for Unilateral Ménière's Disease.

OBJECTIVES: To analyze the incidence of developing contralateral Ménière's disease (MD) in patients who undergo labyrinthectomy for vestibular dysfunction in unilateral MD. STUDY DESIGN: Retrospective chart review. PARTICIPANTS AND METHODS: Adult patients with a diagnosis of MD who underwent surgical labyrinthectomy with minimum follow-up of 12 months were included. Patients who experienced chemical labyrinthectomy, surgical labyrinthectomy for a diagnosis other than MD, contralateral ear surgery, or bilateral MD before the labyrinthectomy were excluded. The key outcome measure is whether symptoms of MD developed in the contralateral ear post-labyrinthectomy. Statistical analysis was performed using χ2 (Fisher exact) test for discrete variables and the Student t test for continuous variables. A P value < .05 was considered significant. RESULTS: Of the140 patients who underwent labyrinthectomy for intractable vertigo due to unilateral MD, 84 had at least 1 year follow-up appointments. Twelve percent (10/84) of these patients developed contralateral MD, which was diagnosed by a neuro-otologist based on symptoms consistent with MD, including low-frequency sensorineural hearing loss. Average age in years is 63.12 (10.83; mean [SD]) at time of surgery. Average follow-up was 35.57 (15.89) months (range: 12-69 months). CONCLUSION: The incidence of contralateral MD development in patients who underwent labyrinthectomy for unilateral MD is 12%. The current literature states that MD has a 30% bilateral involvement rate. Our incidence is significantly lower when compared to the current literature.

Menière's disease and disorders of the carbohydrate metabolism involving the inner ear

Introduction: Menière's disease was described in 1861, but there are still uncertainties regarding its pathophysiology and treatment. Endolymphatic hydrops is recognized as a fundamental pathological characteristic of the disease, as a result of an inadequate absorption of the endolymph. A milder type of endolymphatic hydrops results from an altered chemical composition of the endolymph, due to disorders of the carbohydrate metabolism. Objective: To describe the association of both types of hydrops in patients with Menière disease. Methods: This was a retrospective study of 98 patients with Menière's disease, 62 of whom also presented disorders of the carbohydrate metabolism, and 5 patients with delayed endolymphatic hydrops, 2 of whom also presented disorders of the carbohydrate metabolism. Results: The follow-up of these patients showed that the correction of the metabolic disorders may help in the clinical treatment of Menière's disease and of delayed endolymphatic hydrops, but this does not happen in the more severe types of the diseases. Conclusion: Patients with Menière's disease may present simultaneous disorders of the carbohydratemetabolism, affecting the inner ear. The correction of these disorders helps the clinical treatment but does not preclude the progression of the more severe cases of Menière disease (AU)

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.

[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 aetiopathologies of Ménière's disease: a contemporary review.

Ménière's disease, a condition first described in the 1800's, has been an advancing area of clinical interest and scientific research in recent decades. Guidelines published by the American Academy of Otolaryngology - Head and Neck Surgery remained nearly static for almost 20 years, although we have certainly expanded our knowledge of the aetiology of the disease since that time. This review of the literature highlights the breadth and detail of the current theories in understanding the pathophysiology of this enigmatic disease. Histopathological specimens providing evidence of many of the aetiologies are presented as well. We aim to provide a centralised and updated resource regarding current and emerging theories for Ménière's disease.

Intraoperative Electrocochleography: A Window Into Endolymphatic Hydrops in a Patient With an Endolymphatic Sac Tumor.

OBJECTIVES: Intraoperative electrocochleography (ECochG) provides valuable information regarding inner ear function during surgical procedures. Here, we report findings from ECochG collected at three time intervals during the management of a patient with symptoms of inner ear hydrops. PATIENTS: A 38-year-old man with an endolymphatic sac tumor who demonstrated classic Ménière's symptoms. INTERVENTIONS: Endolymphatic shunt, tumor biopsy, and definitive resection of an endolymphatic sac tumor. MAIN OUTCOME MEASURES: Intraoperative ECochG measurements at three time intervals, using frequency-specific tone bursts and clicks. RESULTS: Across time intervals, the amplitude of ECochG summation potential (SP) responses decreased for tone bursts of all frequencies. On the other hand, the SP response to the click stimulus, and the ratio of the SP to action potential (AP), basically remained unchanged across time points. CONCLUSIONS: Results suggest that the absolute amplitude of the SP response to frequency-specific tone bursts may be more sensitive to inner ear hydrops than the traditionally used SP/AP ratio as a response to click stimuli.

[Relative analysis of endolymphatic imaging and clinical manifestations in Meniere's patients].

Objective:The research aimed to study the relationship between endolymphatic imaging and clinical manifestations(duration of last attack period to MRI scan) of Meniere's patients.Method:Twenty-six Meniere's patients and 29 affected ears were enrolled in this research. According to clinical manifestations, when performing MRI scan, patients who were not in attack period and the duration of last attach period to MRI scan was longer than 14 days were divided to Group A; and patients who were in attack period or the duration of last attach period to MRI scan was shorter than or equal to 14 days were divided to Group B. Administrate gadolinium to mid-ear affected by MD via non-invasive method through Eustachian tube, and then perform 3D-FLAIR MRI scan the next day. After measurement and calculation, compare RV and RC (area of endolymphatic space divided by total lymphatic space in vestibule and cochlea, respectively) of the two groups. Result:Twenty-nine affected ears of 26 patients were administrated successfully.The endolymphatic spaces were distinct from perilymphatic spaces in vestibule and cochlea in 26 ears. In the images of B6, B12, B14, the cochlea and semicircular canals were enhanced, but the vestibules were not enhanced. For Group A ,the average of RV was 0.37±0.06; the average of RC was 0.15±0.04. As to Group B ,the average of RV was 0.52±0.26; the average of RC was 0.18±0.06. According to ANOVA test,RV of the two groups had significant difference（P<0.05）, RC had no significant difference（P>0.05）. The positive RC and RV in Group A were 7.1% and 42.9% respectively. The positive RC and RV in Group B were 13.3% and 66.7%, respectively. As to 4 patients who were during attack period in Group B, the positive RC and RV were 50% and 100%, respectively. The positive RV of Group B was higher than that of Group A. Conclusion:The clinical manifestations(duration of last attack period to MRI scan) of Meniere's patients had relationship with positive rate of MRI scan. The possibility of positive RV was higher when the duration of attack period to MRI scan was shorter.

Vertiginous crisis following temporomandibular joint athrocentesis: a case report.

Temporomandibular joint arthrocentesis and arthroscopy have recently exceeded open surgeries for disorders that failed to respond to conservative treatment. The efficacy of arthrocentesis in reestablishing normal mouth opening and reducing pain and dysfunctions is now commonly accepted, but in contrast to arthroscopy, there are no large series studies on arthrocentesis complications. We report the major complication occurred in our experience: a case of a patient that complained of a violent vertigo, without hearing disorders, following the procedure.

[Analyses of prognostic factors relevant to acute low-tone sensorineural hearing loss].

Objective: To investigate the prognostic factors relevant to acute low-tone sensorineural hearing loss (ALHL). Methods: 196 adult ALHL patients, including 82 males and 114 females with mean age of (43.1±14.3)years old were included. All patients received the same therapy and were evaluated the curative effect. To evaluate the impact factors on the prognosis of hearing, inclusive of age, gender, time delay before the first visit, degree of deafness, vestibular function, electrocochleogram, and the serum levels of thyroxines by SPSS 18.0 software. Results: Of those 196 patients with ALHL, 124(63.3%) were recovery, 5(2.6%) were excellent better, 42(21.4%) were better, and 25 (12.8%) were poor, with a total effective rate of 87.2%. Among 15 (12.1%) who recurred the hearing loss, 2 developed into Meniere's disease during the follow-up. The mean age of patients with poor hearing effect was significantly older than that of other patients (P<0.05). No relativity was found between gender and hearing curative effect. There existed a statistical difference in total effective rate among subjects with different histories (P<0.05). In addition, the recovery rate was significantly different between groups, i. e., the course of disease was less than 14 days, between 14 days and 6 months, and between 6 months and 2 years (P<0.05). There was no statistical significance in total effective rate among different degrees of deafness (P>0.05). However, in term of the recovery rate, the difference was statistical significance (P<0.05). The recovery rate in patients with mild hearing loss was higher than that in middle or heavy hearing loss (both P<0.05). Among patients with mild deafness, the recovery rate in patients whose history was less than 3 months was significantly higher than that more than 3 months (P<0.05). For moderate deafness patients, the recovery rate in patients whose history was less than 7 days was significantly higher than that more than 1 month (P<0.05). There were statistical differences in hearing effect of 130 (66.3%) patients with abnormal vestibular function in comparison to that with normal vestibular function (χ2=15.1, P<0.05). There were 17(8.7%) patients with abnormal electrocochleogram combined with abnormal vestibular function, and the hearing effects were all poor. There were 45 (23.0%) patients with abnormal thyroxine levels in serum, which was significant higher than that in health adults of 5.9%(χ2=7.26, P<0.01). There was no significant difference in hearing prognosis between patients with abnormal and normal thyroxine levels (χ2=2.51, P>0.05). Conclusions: With respect to ALHL, the hearing effect is associated significantly with the history. The severity of hearing loss is negative prognostic factor for hearing recovery. Age, vestibular function, and electrocochleogram might predict hearing recovery. Gender and thyroxine levels couldn't predict the hearing prognosis, although there is a high incidence rate in patients with ALHL.

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.

[Value of videonystagmography and electrocochleography in prognosis prediction of patients with acute low-tone sensorineural hearing loss].

Objective:To predict the prognosis of acute lowtone sensorineural hearing loss（ALHL）by using the videonystagmography(VNG) and electrocochleography(ECochG).Method:A retrospective study was done in 70 patients with ALHL after long-term follow-up. The recurrence rate and rate of progression to definite Meniere 's disease were determined according to the results of VNG and ECochG tests at the onset of the first episode of hearing loss.Result:Twentynine patients（41.4%）experienced recurrent hearing loss and 5 patients（7.1%）developed to Meniere's disease. In patients with an elevated SP/AP ratio and spontaneous nystagmus, the recurrence rate was 81.8%. However, in those with a normal SP/AP ratio and without spontaneous nystagmus, the recurrence rate was 23.3%.There was a statistically significant difference in the progression rate of Meniere's disease between the groups with(15.2%) and without spontaneous nystagmus.Conclusion:It has a certain reference value to predict prognosiswhen spontaneous nystagmus and an elevated SP/AP ratio are detected on initial examination in patients with ALHL.