Triple semicircular canal occlusion: a surgical perspective with short- and long-term outcomes.

OBJECTIVE: To determine the short- and long-term outcomes of triple semicircular canal occlusion as a potential alternative for patients with intractable Ménière's disease. METHODS: A retrospective case series was performed in university settings, enrolling patients with intractable Ménière's disease with previous maximum treatment, who underwent transmastoid, triple semicircular canal occlusion. The study documented: pre- and post-operative Dizziness Handicap Inventory scores at six weeks and one year post-treatment, pure tone audiometry, and surgical aspects. RESULTS: Two female patients, aged 42 and 65 years, underwent unilateral three-semicircular-canal occlusion. Their respective Dizziness Handicap Inventory scores improved from 88 to 68 and 54 to 30 at six weeks post-operatively, with scores of 66 and 0 at one year post-treatment. The one patient with pre-existing functional hearing maintained her hearing threshold post-operatively. CONCLUSION: Triple semicircular canal occlusion is a safe, hearing-preserving, extracranial alternative technique that can control rotatory vertigo in patients with intractable Ménière's disease, when other measures have failed.

Emissões otoacústicas evocadas na doença de Ménière / Otoacoustic emissions evoked in Ménière's disease

RESUMO Objetivo Verificar as respostas das emissões otoacústicas (EOA) evocadas por estímulo transiente e produto de distorção em indivíduos com doença de Ménière. Métodos Estudo transversal com casuística composta por 60 indivíduos de 19 a 75 anos de idade, distribuídos em dois grupos: grupo estudo, com 32 indivíduos com diagnóstico médico de doença de Ménière, sem outros riscos, e grupo controle formado por 28 indivíduos com perda coclear, sem doença de Ménière, pareado por idade e gênero ao grupo estudo. Critério de elegibilidade: curva tipo A, sem perda condutiva ou mista ou suspeita de alteração retrococlear. A avaliação audiológica foi composta por anamnese, inspeção do meato acústico externo, audiometria tonal limiar, logoaudiometria, medidas de imitância acústica e emissões otoacústicas evocadas por estímulo transiente e produto de distorção. Resultados Os indivíduos com Ménière apresentaram maior ocorrência de perda unilateral, zumbido pitch grave, vertigem e plenitude auricular em relação ao controle. Nesses indivíduos, houve maior incompatibilidade entre os resultados das EOA e da audiometria tonal: nas perdas unilaterais, observaram-se alterações nas EOA nas orelhas com limiares auditivos normais do lado contralateral, caracterizando disfunções cocleares. Nas orelhas com perda coclear, houve presença de EOAT (por estímulo transiente) e ausência de EOAPD (produto de distorção), contrapondo-se ao grupo controle, que apresentou ausência de EOAT e de EOAPD, como o esperado em perdas cocleares de outras etiologias. Conclusão A pesquisa das emissões na doença de Ménière identificou disfunção coclear na orelha contralateral nos casos unilaterais e presença de EOAT com ausência de EOAPD nas orelhas com perda auditiva, diferenciando-se das perdas cocleares de outras etiologias.

ABSTRACT Purpose To verify the responses of Evoked Otoacoustic Emissions by transient stimulus and distortion product in individuals with Ménière's Disease. Methods Cross-sectional study with a sample composed of 60 individuals, aged 19 to 75 years, divided into two groups: study group, with 32 individuals with a medical diagnosis of Ménière's disease, without other risks and a control group formed by 28 individuals with cochlear loss without Meniere's disease, age and sex matched to the study group. Eligibility criteria: type A curve, without conductive or mixed loss or suspected retrocochlear alteration. The audiological evaluation consisted of anamnesis, inspection of the external acoustic meatus, pure tone audiometry, logoaudiometry, measures of acoustic immittance and transient evoked otoacoustic emissions and distortion product. Results Individuals with Ménière's disease had a higher occurrence of unilateral hearing loss, low pitch tinnitus, vertigo and ear fullness in relation to the control. In these individuals, there was greater incompatibility between the results of OAE and pure tone audiometry: in unilateral hearing loss, alterations in OAE were observed in ears with normal hearing thresholds on the contralateral side, characterizing cochlear dysfunctions. In the ears with cochlear loss, there was the presence of TEOAE and absence of DPOAE, in contrast to the control group, which showed the absence of TEOAE and DPOAE, as expected in cochlear losses of other etiologies. Conclusion The investigation of emissions in Ménière's disease identified cochlear dysfunction in the contralateral ear in unilateral cases and the presence of TOAE with absence of DPOAE in ears with hearing loss, differentiating from cochlear losses of other etiologies.

Quantification of endolymphatic hydrops and its correlation with Meniere's disease clinical features.

OBJECTIVE: We aimed to quantitatively evaluate the degree of endolymphatic hydrops and its correlation with the clinical features of Meniere's disease. METHODS: We retrospectively collected data from patients with Meniere's disease who underwent gadolinium-enhanced magnetic resonance imaging (MRI) at our department from January 2018 to December 2019. Mimics software was used to perform three-dimensional modelling of the labyrinth, and volume information was obtained to calculate the endolymphatic hydrops index (EHI). A correlation analysis was conducted with data from pure tone audiometry, electrocochleography (EchoG), vestibular myogenic-evoked potential (VEMP) testing, caloric testing and video head impulse testing (vHIT). A two-dimensional method was also employed to calculate the hydrops ratio (HR) of cochlea and vestibule. The test-retest reliability of EHI/HR from different operators was evaluated. RESULTS: A total of 23 affected ears were examined, and the EHI was significantly correlated with Meniere's disease stage, low-frequency hearing threshold, EchoG summating potential/action potential ratio (-SP/AP) and VEMP binaural asymmetry ratio, but no significant correlation was observed between EHI and the caloric test or vHIT. The Intraclass correlation coefficient (ICC) of EHI data calculated by two otologists was 0.946 (p < .001). And the ICC of cochlea and vestibule HR were 0.844 and 0.832 (p < .001). CONCLUSION: Mimics software can be used to quantitatively evaluate the degree of endolymphatic hydrops and have shown higher test-retest reliability than traditional two-dimensional evaluation method. Endolymphatic hydrops correlates with clinical data, such as Meniere's disease stage, low-frequency hearing threshold, EchoG and VEMP asymmetry ratio.

Differences in responsiveness of intratympanic steroid injection for intractable vertigo in Meniere's disease.

OBJECTIVES: The efficacy of intratympanic steroid (ITS) injection for intractable Meniere's disease has been reported; however, its differences in responsiveness are not fully understood. This study investigated the clinical characteristics of patients who responded to ITS injection treatment. METHODS: This retrospective study included 32 patients with Meniere's disease who were unable to control frequent vertigo attacks despite conservative treatment for at least 3 months. They received an intratympanic injection of dexamethasone (3.3 mg/mL) in the affected side at least three times. We measured hearing threshold, subjective symptom scores, cervical and ocular vestibular evoked myogenic potential (cVEMP and oVEMP), and performed glycerol and bithermal caloric tests. RESULTS: Satisfactory control of vertigo for 1 year after the first round of injection was found in 18 patients (56.3%; the response group). However, the injections failed to control vertigo in the other 14 patients (43.8%; the non-response group), and they were then treated with middle ear micropressure therapy. The response group showed improvement in low-frequency hearing, whereas hearing acuity did not change in the non-response group. Significantly reduced amplitude of cVEMP on the affected side was found in 62.5% of patients in the response group; however, no patients in the non-response group showed reduced amplitude of cVEMP. CONCLUSIONS: ITS injection significantly improved the subjective symptoms for intractable Meniere's disease; however, the long-term effects were heterogeneous. Our results suggest that reduced amplitude in cVEMP is associated with the effectiveness of ITS injection treatment.

Intratympanic steroid versus gentamicin for treatment of refractory Meniere's disease: A meta-analysis.

PURPOSE: Intratympanic steroid injections (ITSI) have become a promising treatment for refractory Meniere's disease due to less cochleovestibular damage. However, whether ITSI would be a good alternative to intratympanic gentamicin injections (ITGI) for refractory Meniere's disease still remains controversial. Here we intended to compare the therapeutic effect of ITSI and ITGI in patients with Meniere's disease refractory to conservative treatments, in terms of vertigo control and hearing outcomes, via a meta-analysis. METHODS: Using MEDLINE, PubMed, and EMBASE databases, we calculated pooled odds ratio (OR) estimates of vertigo control rate (i.e., class A according to AAO-HNS guideline) and standardized mean differences (SMD) of spell count, pure tone audiometry (PTA) threshold and speech discrimination score (SDS) with a 95% confidence interval (CI). The trim-and-fill method and sensitivity analysis were used as post-hoc analyses to verify the integrity of the quantitative analysis results. Furthermore, subgroup analyses were performed according to steroid type (methylprednisolone versus dexamethasone) and follow-up period (>1-year versus <1-year). RESULTS: Five studies involving 332 patients with refractory unilateral Meniere's disease were included. In the pooled analysis, those treated with ITGI showed higher ORs than those treated with ITSI in terms of vertigo control rate (OR: 2.39, 95% CI: 0.84-6.79, P = 0.102) and spell counts (SMD: 0.24, 95% CI: -0.12-0.59, P = 0.195), but it did not reach statistical significance. However, a substantial amount of heterogeneity (I2 = 71.0%, Q = 13.79, P = 0.008) and publication bias was found, suggesting a significant small-study effect. Additionally, ITSI elicited better hearing outcomes of the mean PTA threshold (SMD: 3.08, 95% CI: -1.18-7.35) and mean SDS (SMD: 11.15, 95% CI: -23.21-0.90) compared with ITGI, although no statistical significance. In subgroup analysis, the difference in vertigo control rate between ITGI and ITSI was not significant, regardless of the follow-up period and steroid type. Further, methylprednisolone appeared to be superior to dexamethasone for vertigo control. No significant complications from either treatment were reported in the literature. CONCLUSION: The results of this study further refine the recently proposed efficacy of ITSI for the treatment of refractory Meniere's disease, demonstrating the comparable value of ITGI on vertigo control as well as better hearing preservation. Collectively, ITSI could be a safe and the effective treatment for refractory Meniere's disease. However, the current evidence on efficacy of ITSI for refractory Meniere's disease needs to be further clarified, given the substantial heterogeneity and potential biases.

Altered mapping of sound frequency to cochlear place in ears with endolymphatic hydrops provide insight into the pitch anomaly of diplacusis.

A fundamental property of mammalian hearing is the conversion of sound pressure into a frequency-specific place of maximum vibration along the cochlear length, thereby creating a tonotopic map. The tonotopic map makes possible systematic frequency tuning across auditory-nerve fibers, which enables the brain to use pitch to separate sounds from different environmental sources and process the speech and music that connects us to people and the world. Sometimes a tone has a different pitch in the left and right ears, a perceptual anomaly known as diplacusis. Diplacusis has been attributed to a change in the cochlear frequency-place map, but the hypothesized abnormal cochlear map has never been demonstrated. Here we assess cochlear frequency-place maps in guinea-pig ears with experimentally-induced endolymphatic hydrops, a hallmark of Ménière's disease. Our findings are consistent with the hypothesis that diplacusis is due to an altered cochlear map. Map changes can lead to altered pitch, but the size of the pitch change is also affected by neural synchrony. Our data show that the cochlear frequency-place map is not fixed but can be altered by endolymphatic hydrops. Map changes should be considered in assessing hearing pathologies and treatments.

Imaging of endolymphatic hydrops on a vertigo attack of Meniere's disease.

Meniere's disease (MD) characteristically presents with endolymphatic hydrops (EH), which can be visualized with gadolinium-enhanced inner ear magnetic resonance imaging (MRI). Inner ear membrane rupture has been suspected to cause MD attacks, but this remains controversial. We report a case of MD coincidentally evaluated the EH using 3-Tesla MRI during a vertigo attack. A 78-year-old man with bilateral definite MD visited the hospital outpatient department due to a vertigo attack. To evaluate of endolymphatic hydrops on the attack, inner ear MRI was obtained 4 hours after intravenous injection of gadolinium agent. Vestibular EH in each ear occupied almost all vestibular endolymphatic space in contact with the oval window and herniated into the horizontal semi-circular canal. The endolymphatic space was enlarged, without collapse or mixture of contrast agent. No difference was found between ears. EH on a vertigo attack was associated with significant swelling, without obvious evidence of membranous ruptures on magnetic resonance images.

Cochlear Implantation in Meniere's Disease: A Systematic Review and Meta-Analysis.

OBJECTIVES/HYPOTHESIS: Meniere's disease (MD) is a debilitating condition characterized by hearing loss, vertigo, and tinnitus. The objective of this study was to systematically investigate outcomes in MD after cochlear implantation (CoI), with and without labyrinthectomy. STUDY DESIGN: Systematic review and meta-analysis. METHODS: A systematic review of articles in Medline and Embase was performed to identify all studies of patients with MD who underwent CoI. This analysis evaluates outcomes of speech recognition, pure tone audiometry, vertigo, tinnitus, and quality of life. RESULTS: Of 321 studies identified, 37 were included, involving 216 patients. Mean age at implantation was 61.4 years (range 27-85 years) with average length of follow-up at 1.7 years (range 0-9 years). Forty-four (20.4%) patients underwent labyrinthectomy. Meta-analysis demonstrated significant improvements in audiometric outcomes following CoI. There was a statistically significant improvement in Hearing in Noise Test performance, with a mean difference improvement of 44.7 (95% confidence interval [CI] [8.8, 80.6]) at 6 months and 60.1 (95% CI [35.3, 85.0]) at 12 months. The Freiburger Monosyllabic Test (FMT) and Consonant-Nucleus-Consonant (CNC) also improved significantly, with mean difference improvements of 46.2 (95% CI [30.0, 62.4]) for FMT and 19.3 (95% CI [8.1, 30.4]) for CNC. There was a statistically significant decrease in tinnitus, as measured by a mean difference reduction of 48.1 (95% CI [1.0, 95.2]) in the Tinnitus Handicap Index. CONCLUSIONS: CoI with and without simultaneous labyrinthectomy is a viable treatment option for patients with MD, yielding high rates of tinnitus suppression and vertigo control. Post-CoI MD patients demonstrate similar postoperative speech perception outcomes to non-MD implant recipients. Laryngoscope, 131:1845-1854, 2021.

Intraoperative auditory brainstem response monitoring during semicircular canal plugging surgery in treatment of Meniere's disease.

BACKGROUND: Three semicircular canal plugging (TSCP) is an optimized treatment for intractable Meniere's disease (MD). However, 20-30% of patients experience hearing loss after TSCP, for reasons that remain unclear. OBJECTIVE: To evaluate hearing loss resulting from TSCP. SUBJECTS AND METHODS: This study included 12 patients, which were diagnosed with definite MD and consented to TSCP surgery. Intraoperative auditory brainstem response (ABR) was monitored in each surgical procedure. RESULTS: After opening the mastoid cavity, the ABR threshold increased to 77.08 ± 9.88 dB nHL. The ABR threshold almost recovered to preoperative levels, to 68.33 ± 7.78 dB nHL, after completing TSC outlining. Exposure of three semicircular canal 'blue lines' had little effect on ABR threshold. The most prominent change on hearing loss was observed after mastoid outlining, when 41.67% of patients showed hearing loss ≥10 dB nHL. None of the patients showed a threshold shift ≥10 dB nHL following the last step. CONCLUSIONS: TSCP operation itself caused little hearing damage. SIGNIFICANCE: Ruled out hearing loss as a result of the surgery itself. The reason why 20-30% of patients showed hearing loss in 2-year follow-up visit was not clear, although it may be due to serous fibrous labyrinthitis.

Platform posturography of patients with peripheral vestibular dysfunction in the non-acute phase of vertigo.

OBJECTIVE: Posturography (PG) shows various patterns corresponding to a patient's equilibrium condition; however, PG is not useful for the differential diagnosis of peripheral vestibular diseases (PVDs). The aim of this study was to identify parameters of PG that can distinguish between PVDs. METHODS: The differences in PG parameters between PVDs were evaluated retrospectively. Two hundred and two patients with Ménière's disease (MD), 154 patients with benign paroxysmal positional vertigo (BPPV), 20 patients with sudden sensorineural hearing loss with vertigo (SSNHLwV), and 31 patients with vestibular neuritis (VN) underwent PG during the non-acute phase of vertigo, from January 2010 to March 2017. RESULTS: The velocity of body oscillation of BPPV patients with eyes open and closed were significantly faster than those of MD patients with eyes open (p < 0.001) and closed (p = 0.033). The velocity of body oscillation of VN patients with eyes open was significantly faster than that of MD patients with eyes open (p = 0.0083). There were no significant differences among the other PG parameters between PVDs. Although there were significant differences among the velocity with eyes open and closed between males and females (eye open: p = 0.0009, eye close: p < 0.0001), there was no significant difference in the ratio of males to females among PVDs (p = 0.1834). Therefore, the ratio did not influence the difference in velocity among PVDs. Patient age correlated with the velocity with eyes open (p < 0.001) and with eyes closed (p < 0.001). Post-hoc analysis revealed significant differences in patient age, and comparisons of MD and BPPV, MD and SSNHLwV, BPPV and VN, and VN and SSNHLwV. Therefore, we performed multiple regression analysis to determine whether the significant differences in the velocity of body oscillation among PVDs were caused by the difference in age distribution between PVD groups, rather than by differences in the PVDs themselves. There were correlations between age and the velocity of body oscillation with eyes open (p < 0.001) and with eyes closed (p < 0.001). There also were correlations between MD or VN and the velocity of body oscillation with eyes open (p = 0.0194). CONCLUSION: There were significant differences in the velocity of body oscillation with eyes open between MD and VN patients. The difference between MD and VN was significant regardless of the age distribution. To distinguish between MD and VN, the velocity of body oscillation with eyes open is a useful PG index.

The Impact of the COVID-19 Pandemic on Follow-Ups for Vertigo/Dizziness Outpatients.

OBJECTIVES: In the present report, we aimed to investigate the impact of the coronavirus disease (COVID-19) pandemic on vertigo/dizziness outpatient cancellations in Japan. METHODS: We examined 265 vertigo/dizziness outpatients at the ear, nose, and throat department of the Nara Medical University between March 01, 2020, and May 31, 2020, during the COVID-19 pandemic in Japan. We also focused on 478 vertigo/dizziness outpatients between March 01, 2019, and May 31, 2019, before the COVID-19 pandemic, to compare the number of cancellations between these 2 periods. The reasons for cancellation and noncancellation were investigated using telephone multiple-choice questionnaires (telMCQs), particularly for patients with benign paroxysmal positional vertigo (BPPV) and Meniere's disease (MD). RESULTS: There were many cancellations for medical examinations during the 2020 study period. The total number of vertigo/dizziness outpatients decreased by 44.6% in the 2020 period compared to the same period in 2019. The percent reduction in clinic attendance from 2019 to 2020 (ie, [2019-2020]/2019) for patients with BPPV was higher than that for patients with MD. Compared to the other vertigo-associated conditions, patients with MD exhibited a lower percent reduction in clinic attendance. According to the results of the telMCQs, 75.0% of BPPV cases and 88.2% of MD cases cancelled their appointment and gave up visiting hospitals due to fear of COVID-19 infection, even if they had moderate to severe symptoms. On the contrary, 25.0% and 80.0% patients with BPPV and MD, respectively, did not cancel their appointment; they should not have visited the hospital but stayed at home because they had slight symptoms. CONCLUSIONS: These findings suggest that advanced forms should be prepared for medical care, such as remote medicine. These forms should not only be for the disease itself but also for the mental distress caused by persistent symptoms.

Meniere's disease: Medical management, rationale for vestibular preservation and suggested protocol in medical failure.

Meniere's disease is a peripheral audiovestibular disorder characterized by vertigo, hearing loss, tinnitus, and aural fullness. Management of these symptoms includes medical and surgical treatment. Many patients with Meniere's disease can be managed using nonablative therapy, such as intratympanic steroids and endolymphatic shunt surgery, prior to ablative techniques such as intratympanic gentamicin. Recognition of concurrent migraine symptoms may aid in medical therapy and also underscore the importance of preserving vestibular function where possible. The goal of this review is to explain the importance of nonablative therapy options and discuss treatment protocols after medical failure.

Vestibulo-ocular reflex tests may reflect unilateral Ménière disease progression: A cross-sectional study.

ABSTRACT: Vestibular disorders can manifest several patterns of horizontal vestibulo-ocular reflex (VOR) impairment, which can be detected by caloric test and video head impulse test (vHIT). Several studies have examined the patterns of caloric-vHIT response in Ménière disease (MD). The purpose of this study was to investigate the diversity of caloric-vHIT response and its related factors in unilateral MD patients. We also explore the possibility of assessing the progression of unilateral MD by using the horizontal VOR tests.Ninety-eight patients with unilateral MD were enrolled and underwent a battery of audio-vestibular evaluations, including the pure tone audiogram, caloric test, and horizontal vHIT. Some patients received the electrocochleography and glycerol test. The combined results of caloric test and horizontal vHIT were categorized qualitatively into 4 patterns: Pattern I: normal caloric and vHIT responses; Pattern II: abnormal caloric and normal vHIT responses; Pattern III: normal caloric and abnormal vHIT response; and Pattern IV: abnormal caloric and vHIT responses. The abnormal caloric results were semi-quantitatively subdivided into sub-patterns as mild, moderate, and severe abnormality. The associations between these patterns/sub-patterns and related factors were analyzed.Pattern I was found in 35 cases (35.7%), Pattern II in 57 (58.2%), and Pattern IV in 6 (6.1%). No patient had Pattern III. No significant differences were found between the patterns/sub-pattern distribution and age, electrocochleography, and glycerol test results. Disease duration was not associated with the pattern distribution, while remained a relation with sub-pattern distribution. The pattern/sub-pattern distribution varied significantly across MD stages. The proportion of pattern II or pattern IV increased with the stage of unilateral MD.MD can manifest several patterns of horizontal VOR impairment, of which the impaired caloric response with normal vHIT is the most common pattern. With the progression of unilateral MD, the caloric-vHIT pattern tends to shift, which may reflect the deterioration of endolymphatic hydrops and vestibular hair cells impairments.

Modulation of hearing function following the downgrading or upgrading of endolymphatic hydrops in Meniere's disease patients with endolymphatic duct blockage.

The present study was to investigate the dynamics of endolymphatic hydrops (EH) and hearing function, and explore whether the hearing loss is caused by EH alone and whether the hearing function can be modulated by changes in the EH. The extent of EH visualized by gadolinium (Gd)-enhanced inner ear magnetic resonance imaging, hearing thresholds and the summating potential/action potential ratio (-SP/AP ratio) of electrocochleography (ECochG) were recorded prior to and following surgery in 22 patients with intractable Meniere's disease (MD) who underwent endolymphatic duct blockage (EDB). The difference value of the hearing threshold and -SP/AP ratio was significantly positively correlated with the difference value of the endolymph to vestibule-volume ratio (EVVR) and grading of cochlear hydrops between prior to and following surgery. Among patients with a decreased EVVR, the average hearing threshold and -SP/AP ratio was significantly decreased following surgery, as compared to that prior to surgery. Six out of seven patients with a hearing improvement (≥10-dB decline) and 4/5 patients with a negative conversion of EcochG showed downgrading of their hydrops in the cochlea and/or vestibule. By contrast, among patients with an increased EVVR, the average hearing threshold and -SP/AP ratio tended to increase following EDB, as compared with that prior to surgery. One out of two patients with a hearing deterioration (≥10-dB elevation) showed upgrading of her hydrops in both cochlea and vestibule. The present results showed the downgrading of cochlear and/or vestibular hydrops accompanied by the downregulation of the hearing threshold and -SP/AP ratio of EcochG, as well as the upgrading of cochlear and/or vestibular hydrops that tended to upregulate the hearing threshold and -SP/AP ratio of EcochG; this suggested that hearing loss is likely to be caused by hydrops and that the impaired hearing function be modulated by changes in the hydrops.

Vestibular mapping in patients with unilateral peripheral-vestibular deficits.

OBJECTIVE: To test the hypothesis that patterns of semicircular canal (SCC) and otolith impairment in unilateral vestibular loss depend on the underlying disorders, we analyzed peripheral-vestibular function of all 5 vestibular sensors. METHODS: For this retrospective case series, we screened the hospital video-head-impulse test database (n = 4,983) for patients with unilaterally impaired SCC function who also received ocular vestibular-evoked myogenic potentials and cervical vestibular-evoked myogenic potentials (n = 302). Frequency of impairment of vestibular end organs (horizontal/anterior/posterior SCC, utriculus/sacculus) was analyzed with hierarchical cluster analysis and correlated with the underlying etiology. RESULTS: Acute vestibular neuropathy (AVN) (37.4%, 113 of 302), vestibular schwannoma (18.2%, 55 of 302), and acute cochleovestibular neuropathy (6.6%, 20 of 302) were most frequent. Horizontal SCC impairment (87.4%, 264 of 302) was more frequent (p < 0.001) than posterior (47.4%, 143 of 302) and anterior (37.8%, 114 of 302) SCC impairment. Utricular damage (58%, 175 of 302) was noted more often (p = 0.003) than saccular impairment (32%, 98 of 302). On average, 2.6 (95% confidence interval 2.48-2.78) vestibular sensors were deficient, with higher numbers (p ≤ 0.017) for acute cochleovestibular neuropathy and vestibular schwannoma than for AVN, Menière disease, and episodic vestibular syndrome. In hierarchical cluster analysis, early mergers (posterior SCC/sacculus; anterior SCC/utriculus) pointed to closer pathophysiologic association of these sensors, whereas the late merger of the horizontal canal indicated a more distinct state. CONCLUSIONS: While the extent and pattern of vestibular impairment critically depended on the underlying disorder, more limited damage in AVN and Menière disease was noted, emphasizing the individual range of loss of function and the value of vestibular mapping. Likely, both the anatomic properties of the different vestibular end organs and their vulnerability to external factors contribute to the relative sparing of the vertical canals and the sacculus.

Ictal downbeat nystagmus in Ménière disease: A cross-sectional study.

OBJECTIVE: To determine the mechanism of ictal downbeat nystagmus in Ménière disease (MD), we compared the head impulse gain of the vestibulo-ocular reflex (VOR) for each semicircular canal between patients with (n = 7) and without (n = 70) downbeat nystagmus during attacks of MD. METHODS: We retrospectively analyzed the results of video-oculography, video head-impulse tests, and cervical vestibular-evoked myogenic potentials (VEMPs) in 77 patients with definite MD who were evaluated during an attack. RESULTS: Pure or predominant downbeat nystagmus was observed in 7 patients (9%) with unilateral MD during the attacks. All 7 patients showed spontaneous downbeat nystagmus without visual fixation with a slow phase velocity ranging from 1.5 to 11.2°/s (median 5.4, interquartile range 3.7-8.5). All showed a transient decrease of the head impulse VOR gains for the posterior canals (PCs) in both ears (n = 4) or in the affected ear (n = 3). Cervical VEMPs were decreased in the affected (n = 2) or both ears (n = 2) when evaluated during the attacks. Downbeat nystagmus disappeared along with normalization of the VOR gains for PCs after the attacks in all patients. During the attacks, the head impulse VOR gains for the PC on the affected side were lower in the patients with ictal downbeat nystagmus than in those without (Mann-Whitney U test, p < 0.001), while the gains for other semicircular canals did not differ between the groups. CONCLUSION: Downbeat nystagmus may be observed during attacks of MD due to an asymmetry in the vertical VOR or saccular dysfunction. MD should be considered in recurrent audiovestibulopathy and ictal downbeat nystagmus.

The role of endogenous Antisecretory Factor (AF) in the treatment of Ménière's Disease: A two-year follow-up study. Preliminary results.

PURPOSE: To evaluate the effects of increased endogenous Antisecretory Factor (AF) synthesis using specially processed cereals (SPC) in a sample of patients with defined unilateral Meniere's disease (MD), compared to the results of a treatment protocol of intravenous glycerol and dexamethasone. MATERIALS AND METHODS: Twenty-six patients with unilateral MD were divided in 2 groups and treated with SPC and with intravenous glycerol and dexamethasone for 24 months. Audio-vestibular evaluation was performed before (T0) and every six months. The number of vertigo spells were evaluated before and after therapy and the Efficacy Index (EI) was calculated. Questionnaires for hearing loss, tinnitus and quality of life were administered. RESULTS: EI decreased in the SPC group after 18 (T18) (p = .0017) and 24 (T24) months of therapy (p = .0111). There was a significant reduction for tinnitus score in the SPC group at T24 (p = .0131). No significant differences were found between the two groups at T0 (p = .4723), while a significant difference was found at T24 (p = .0027). Quality of life showed a significant improvement in daily activities in the SPC group (p = .0033) compared to the infusion therapy group. No statistically significant changes in PTA thresholds were found in both groups between T0 and T24. CONCLUSION: The preliminary results of our study show a significant reduction of vertigo spells and a positive effect on tinnitus severity and on quality of life in patients with unilateral MD treated with SPC and when compared to patients treated with intravenous glycerol and dexamethasone. No effects on hearing thresholds were noted in both groups.

Assessment of the Progression of Vertical Semicircular Canal Dysfunction and Increased Vestibular Endolymphatic Hydrops in Patients With Early-Stage Ménière Disease.

Importance: Vertical semicircular canals and endolymphatic hydrops play important roles in the pathophysiological mechanisms of Ménière disease. However, their characteristics and associations with disease progression during medical treatment have not been determined. Objective: To examine the function of both the horizontal and vertical semicircular canals in patients with Ménière disease and to evaluate the change in endolymphatic hydrops volume during medical treatment, including treatment with diuretic therapy, over a 2-year period. Design, Setting, and Participants: This prospective longitudinal observational cohort study included 55 patients with definite unilateral Ménière disease and was performed in a tertiary care hospital in Japan. Participants were enrolled between April 1, 2017, and January 31, 2018, and those with vestibular migraine were excluded. All participants received education regarding diet and lifestyle modifications and treatment with betahistine mesylate (36 mg daily) and/or an osmotic diuretic (42-63 mg daily). Patients were followed up for vertigo and hearing evaluations at least once per month for more than 12 months and were instructed to record episodes of vertigo in a self-check diary. Audiometry was performed monthly, video head impulse testing and caloric testing were performed every 4 months, and magnetic resonance imaging was conducted annually. Data were analyzed from May 15, 2017, to January 31, 2020. Main Outcomes and Measures: Neurootological testing to evaluate vestibuloocular reflex gain over time, magnetic resonance imaging to evaluate the change in endolymphatic hydrops volume over time, and monthly vertigo and hearing evaluations for more than 12 months. Results: Among 55 participants with definite Ménière disease, 32 patients (58.2%) were female, and the mean (SD) age was 59.0 (15.1) years. The median disease duration was 2 years (interquartile range, 0-4 years), with 43 patients (78.2%) having an early stage (ie, disease duration ≤4 years) of Ménière disease. Over the 2-year study period, the vestibuloocular reflex gain decreased from 0.76 to 0.56 in the superior semicircular canals, for a difference of 0.20 (95% CI, 0.14-0.26) and from 0.68 to 0.50 in the posterior semicircular canals, for a difference of 0.18 (95% CI, 0.14-0.22). The maximum slow-phase velocity and vestibuloocular reflex gain in the horizontal semicircular canals were maintained. The volume ratio of vestibular endolymphatic hydrops increased from 19.7% to 23.3%, for a difference of 3.6% (95% CI, 1.4%-5.8%). The frequency of vertiginous episodes decreased, and the hearing level over the study period worsened from 40.9 dB to 44.5 dB, for a difference of 3.5 dB (95% CI, 0.7-6.4 dB). Conclusions and Relevance: In this study, during a 2-year period of medical treatment among patients with Ménière disease, vestibuloocular reflex gain decreased in the vertical semicircular canals but was maintained in the horizontal semicircular canals; the endolymphatic hydrops volume ratio increased, and the frequency of vertiginous episodes decreased. These findings describe the pathological progression of chronic Ménière disease and expand the understanding of its pathophysiological characteristics during the early stage of disease.

Persistence of Non-Vertigo Symptoms in Meniere Disease During Remission - A Preliminary Report.

BACKGROUND: Though the absence of vertigo in Meniere disease is often interpreted as remission, patient-centered subjective assessment of quality of life remains the best indicator of such remission. STUDY OBJECTIVE: To assess the presence and severity of aural pressure/tinnitus, hearing loss, unsteadiness, nausea and vomiting in MD patients during remission. SETTING: Urban tertiary care referral hospital in a developing country. METHODOLOGY: Consecutive patients with diagnosis of Definite Meniere were selected from the Balance and Dizziness Clinic of National Hospital Abuja for the study. Quality of life assessment was carried out using 3 validated tools - Modified MD-POSI, Vertigo Symptom Scale and Tinnitus Handicap Inventory (THI). Patients were included only when they have been vertigo free for at least 4 weeks. Pure tone audiometry was carried out in those with subjective hearing loss at recruitment and 4 weeks later. RESULTS: A total of 26 patients completed the study. All had cinnarizine for acute vertigo control and Betahistine for maintenance of vertigo control. There was female preponderance (17:9). The age range was 32-56 years. The duration of MD ranges from 4 months to 12 years. The total and subscale MD-POSI scores for "between attacks" significantly correlated with hearing, unsteadiness and tinnitus/pressure when compared to during attack. 69.2 per cent of participants experienced symptoms of unsteadiness during remission. 13/26 of participants reported persistent, though less annoying tinnitus that poorly correlated with THI score during remission. CONCLUSION: Our study showed that significant non-vertigo symptoms affect the quality of life during remission. Perhaps there is need to properly define, in future studies, what constitutes remission in patients with MD.

Live imaging and functional changes of the inner ear in an animal model of Meniere's disease.

The symptoms of Meniere's disease (MD) are generally considered to be related to endolymphatic hydrops (EH). There are many recent reports supporting the possibility that vasopressin (VP) is closely linked to the formation of EH in Meniere's disease. Based on this, we developed a clinically relevant animal model of Meniere's disease in which a VP type 2 receptor agonist was administered after electrocauterization of the endolymphatic sac. We report live imaging of the internal structure, and functional changes of the inner ear after electrocauterization of the endolymphatic sac and administration of a VP type 2 receptor agonist. In this model, the development of EH was visualized in vivo using optical coherence tomography, there was no rupture of Reissner's membrane, and low-tone hearing loss and vertiginous attacks were observed. This study suggested that acute attacks are caused by the abrupt development of EH. This is the first report of live imaging of the development of EH induced by the administration of a VP type 2 receptor agonist.