Otic Capsule Dehiscences Simulating Other Inner Ear Diseases: Characterization, Clinical Profile, and Follow-Up-Is Ménière's Disease the Sole Cause of Vertigo and Fluctuating Hearing Loss?

INTRODUCTION: We present a series of six cases whose clinical presentations exhibited audiovestibular manifestations of a third mobile window mechanism, bearing a reasonable resemblance to Ménière's disease and otosclerosis. The occurrence of these cases in such a short period has prompted a review of the underlying causes of its development. Understanding the pathophysiology of third mobile window syndrome and considering these entities in the differential diagnosis of conditions presenting with vertigo and hearing loss with slight air-bone gaps is essential for comprehending this group of pathologies. MATERIALS AND METHODS: A descriptive retrospective cohort study of six cases diagnosed at a tertiary center. All of them went through auditive and vestibular examinations before and after a therapeutic strategy was performed. RESULTS: Out of 84 cases of dehiscences described in our center during the period from 2014 to 2024, 78 belonged to superior semicircular canal dehiscence, while 6 were other otic capsule dehiscences. Among these six patients with a mean age of 47.17 years (range: 18-73), all had some form of otic capsule dehiscence with auditory and/or vestibular repercussions, measured through hearing and vestibular tests, with abnormalities in the results in five out of six patients. Two of them were diagnosed with Ménière's disease (MD). Another two had cochleo-vestibular hydrops without meeting the diagnostic criteria for MD. In two cases, the otic capsule dehiscence diagnosis resulted from an intraoperative complication due to a gusher phenomenon, while in one case, it was an accidental radiological finding. All responded well to the proposed treatment, whether medical or surgical, if needed. CONCLUSIONS: Otic capsule dehiscences are relatively new and unfamiliar entities that should be considered when faced with cases clinically suggestive of Ménière's disease, with discrepancies in complementary tests or a poor response to treatment. While high-sensitivity and specificity audiovestibular tests exist, completing the study with imaging, especially petrous bone CT scans, is necessary to locate and characterize the otic capsule defect responsible for the clinical presentation.

Hearing and Balance in Healthy Aging Project: Characterization of Hearing, Balance, and Other Associated Disorders in Three Population Groups Aged 55 and Over.

INTRODUCTION: Active aging emphasizes optimizing health and participation for a better quality of life as people age. This paper explores the significant impact of hearing loss and balance disorders on the elderly. Age-related hearing loss is thought to contribute to communication breakdown and cognitive dysfunctions. The "hearing and balance in healthy aging" project focuses on early detection, mitigation, and advocacy. Objectives include exploring epidemiological traits, evaluating overall well-being impact, proving positive intervention effects, and advocating societal care for the elderly with hearing loss and balance disorders, aiming to reduce their broader impact on cognition, independence, and sociability. METHODS: This study is observational, prospective study. Subjects over 55 years old with a follow-up every year or every 2 years were divided into three groups, according to their hearing and balance: within the normal range (group A), detected and not treated (group B), and detected and treated (group C). At each visit, they underwent a series of tests or questionnaires, evaluating different areas: hearing, balance, cognition, depression, dependence, tinnitus, loneliness, health. RESULTS: A total of 710 patients were included in the study. The distribution of patients was as follows: group A - 210 patients, group B - 302 patients, and group C - 198 patients. Significant differences were found between the three groups related to age, sex, educational level, bilingualism, and work activity. In group C, there was a higher percentage of males, older than in groups A and B, and the percentage of individuals with a university education was lower (28%), as was the rate of bilingualism (23%). In terms of hearing, significant differences were found in the three groups in the mean PTA, speech discrimination in quiet, and the HINT test, with worse results for group C. Only patients in group C presented a perception of hearing impairment, and the handicap caused by hearing impairment worsened from group A to C. Concerning balance, both tests performed (TuGT and DHI) revealed increased difficulty in maintaining autonomous walking from group A to C, which, again, exhibited the worst results, with statistically significant differences across the group. Analysis about cognition revealed significant differences in DSST questionnaires and in TMT scores, where group C had the worst scores. In HUI3 questionnaire scores, the differences between each and every group were statistically significant, with group C showing moderate disability. CONCLUSION: This extensive analysis, encompassing a considerable number of subjects, reveals significant findings that have important implications for the early prevention of hearing loss and its consequent consequences. At the same time, these data represent an initial exploration, which raises the need for in-depth examinations of additional factors and longer follow-up to continue contributing insights and knowledge for a healthy aging.

Acute histological reactions in the otolith organs to inner ear drug delivery through a cochlear implant.

Introduction: Cochlear implantation is currently regarded as a safe and minimally invasive procedure. However, cochlear implantation can have an impact on vestibular function, despite the lack of correlation between patient symptomatology and damage in vestibular tests. Thus, the present study aims to analyze the presence of hydrops and histological reactions at the level of the vestibule after cochlear implantation with dexamethasone pump delivery in Macaca fascicularis (Mf). Materials and methods: A detailed histological study was conducted on a total of 11 Mf. All 11 Mf were divided into three groups: 5 Mf were implanted with an electrode array HL-14 connected to a pump delivering FITC-dextran for 24 h (Group A); 4 Mf were implanted with a CI electrode array attached to a pump for FITC-dextran delivery for 7 days (Group B); and 2 Mf were considered the control group, without any kind of cochlear device implantation (Group C). After drug deliver, the selected macaques were euthanized to collect tissue samples for histological analysis. An experienced observer, focusing on the utricle and saccule areas, conducted a blinded inner ear histology analysis. Results: Surgical procedures were successfully performed in all cases. No signs of cochlear reaction to the device were observed, including neither collapse nor fibrosis. Endolymphatic sinus dilatation was observed in Mf4A and Mf3B, while cochlear hydrops was observed in Mf3A. The mean areas of the utricle and saccule exhibited some statistically significant differences, specifically, in the saccule between groups C and both groups A (p = 0.028) and B (p = 0.029); however, no significant differences were observed between groups A and B or among comparisons of the utricle. Discussion: A significant concern relates to the safety of cochlear implantation with regard to vestibular preservation and hearing. New advancements in electrode arrays, such as CI devices coupled with delivery pumps, pose a challenge in maintaining minimally traumatic surgical concept-based procedures without affecting the inner ear homeostasis. The implantation of this device may cause vestibular hydrops in the saccule, indicating that the longer the time of substance release, the greater the grade of hydrops evidenced at the saccular level. Apart from this finding, the risk of histological damage to the vestibule is low.

Bone Conduction Implants: Comparative of Audiometric Results and Quality of Life Bonebridge® vs Osia®.

INTRODUCTION: Bone conduction implants have been indicated for patients with conductive hearing loss, mixed hearing loss, and even profound unilateral sensorineural hearing loss. With the introduction of Bonebridge®, new transcutaneous implant options emerged. The latest is Osia®, a direct-drive variant or active systems where the implant directly generates and applies vibration to the bone. MATERIALS AND METHODS: Retrospective study of two cohorts of patients treated with active bone conduction implants at a single center, one with the Bonebridge® device and the other with Osia®. OUTCOMES: Fourteen patients were included, seven in each group (n=14). The Bonebridge® group showed an average hearing gain in tonal intelligibility thresholds of 32.43±21.39 dB and a gain in the average intelligibility threshold (with 50% discrimination) of 26.29±19.10 dB. In the Osia® group, there was a gain in average tonal thresholds of 41.49±14.16 dB and 23.72±6.98 dB in average intelligibility thresholds. Both devices contributed to improvements in patients' quality of life, as assessed with APHAB in all the variables studied in the test. Both devices offer rehabilitation for hearing loss as an alternative to hearing aids. The Osia® system shows statistically significant(p<0.05) improvements in mid and high frequencies, but Bonebridge® slightly outperforms in speech understanding at 50%. Differences in average tonal thresholds and quality of life are not statistically significant. CONCLUSIONS: While auditory improvement is observed post-implantation, other aspects, such as intelligibility thresholds and quality of life, lack statistical significance. Given the limited experience with Osia® and the small sample size, the choice of the device should be personalized. Although the literature is inconsistent due to small sample sizes and variable approaches, some studies suggest potential advantages of the Osia® system, especially in speech comprehension in different environments and greater hearing gain compared to Bonebridge®.

Congruence and incongruence on the radiological and functional examination of inner ear hemorrhage.

BACKGROUND: Inner ear hemorrhage (IEH) is an increasingly recognized cochlear lesion that can cause sensorineural hearing loss (SNHL). Magnetic resonance imaging (MRI) is known to be the best imaging modality for clarifying the causes of SNHL and providing images that point to those causes. AIMS: Evaluate the lesional patterns in patients with presumed Inner ear hemorrhage (IEH) from radiological and functional aspects. MATERIAL AND METHODS: We retrospectively reviewed 10 patients performed in our institution from 2014 to 2020, with suspected labyrinthine hemorrhage based on radiological and functional examination. RESULTS: We included 8 patients with IEH and sensorineural hearing loss (SNHL). The median age was 55 years (range: 3 months - 78 years). The results from the MRI and functional tests were compared for each end-organ. Only three cases (37.5%) showed a correlation between signal abnormalities and dysfunction in the labyrinthine apparatus. CONCLUSIONS: In patients with SNHL inner ear hemorrhage needs to be ruled out in the differential diagnosis, so specific MRI sequences should be requested. It represents a way to a better understanding of the disorder and the variety of findings claim for a complete auditory and vestibular testing.

Histological Reaction in the Round Window Membrane after Cochlear Implant Insertion in Nonhuman Primates.

INTRODUCTION: The primary objective of this article was to determine if any histological alterations occur in the round window (RW) and adjacent anatomical structures of an animal model with normal hearing when a cochlear implant (CI) electrode array is inserted. Furthermore, this article aims to relate these histological alterations to the auditory changes generated. METHODS: Cochlear implantation was performed, following the principles of minimally traumatic surgery (MTS), in 15 ears of nonhuman primates (Macaca fascicularis) (Mf) with normal hearing. Auditory brainstem-evoked potentials (ABR) using clicks and tones were used prior to surgery and during a 6-month follow-up period. Histological evaluation was carried out, analyzing the position of the electrode array with respect to the round window membrane (RWM), its percentage of occupation and integrity, the presence of intracochlear damage, and the tissue reaction provoked, the latter of which was quantified in the temporal bones. RESULTS: Surgery was performed on all 15 ears without relevant incidences. Regarding histology, the electrode array in the RW of all ears presented a lateral position with respect to the modiolus. No lesions affecting the integrity of the RW were observed. The mean value of the array's occupation in the RW was 25%. Tissue reaction, in the form of fibrosis, was observed in all ears and more intensely in the trans-RWM and post-RWM areas. In all ears, the electrodes remained on the scala tympani. No profound hearing impairment was recorded in any ear, being the mean loss of 25.4 dB when comparing presurgical thresholds with those collected 6 months after implantation in ABR click and 24.4 dB in ABR tone burst. CONCLUSIONS: The animal model and Hybrid L-14 (HL14) electrode array were optimal for implementing a surgical technique similar to that routinely performed on humans. Mild histological alterations were observed in the round window membrane and adjacent anatomical structures from the insertion of a cochlear implant electrode array. Following the minimally invasive technique, levels of hearing preservation were satisfactory, reaching a pre-post difference of 25.4 dB in the ABR click and 24.4 dB for a high-frequency tone burst. Complete hearing impairment was not observed in either ear. Correlation between the severity of histological alterations and hearing changes recorded in the ABR studies was observed.

Magnetic Resonance Volumetric Quantification of Vestibular Endolymphatic Hydrops in Patients with Unilateral Definite Meniere's Disease Using 3D Inversion Recovery with Real Reconstruction (3D-REAL-IR) Sequence.

BACKGROUND: The 3D-REAL-IR MRI sequence allows for an in vivo visualization of endolymphatic hydrops. Qualitative assessment methods of the severity of vestibular and cochlear hydrops are the most commonly used. METHODS: A quantitative volumetric measurement of vestibular EH in patients with definite unilateral Ménière's disease using the 3D-REAL-IR sequence and the calculation of the endolymphatic ratio (ELR) was intended. RESULTS: Volumetric calculations of the vestibules, vestibular endolymph and vestibular ELR are performed in 96 patients with unilateral Ménière's disease and correlated with classic qualitative grading scales. CONCLUSIONS: Quantitative volumetric measurement of vestibular hydrops using the 3D-REAL-IR sequence is feasible and reproducible in daily clinical practice. Vestibular ELR values exceeding 60% defined radiologically significant vestibular hydrops, while values below 30% defined radiologically non-significant vestibular hydrops.

A Paradoxical Clinical Coincidence: Benign Paroxysmal Positional Vertigo and Bilateral Vestibulopathy.

Benign paroxysmal positional vertigo (BPPV) and bilateral vestibulopathy (BVL) are two completely different forms of vestibular disorder that occasionally occur in the same patient. We conducted a retrospective review searching for that coincidence in our database of the patients seen over a 15-year period and found this disorder in 23 patients, that is 0.4%. More frequently they occurred sequentially (10/23) and BPPV was diagnosed first. Simultaneous presentation occurred in 9/23 patients. It was subsequently studied, but in a prospective manner, in patients with BPPV on all of whom a video head impulse test was performed to search for bilateral vestibular loss; we found it was slightly more frequent (6/405). Both disorders were treated accordingly, and it was found that the results follow the general trend in patients with only one of those disorders.

Histopathological reaction in the vestibule after cochlear implantation in Macaca fascicularis.

Cochlear implantation surgery (CI) is considered a safe procedure and is the standard treatment for the auditory rehabilitation in patients with severe-to-profound sensorineural hearing loss. Although the development of minimally traumatic surgical concepts (MTSC) have enabled the preservation of residual hearing after the implantation, there is scarce literature regarding the vestibular affection following MTCS. The aim of the study is to analyze histopathologic changes in the vestibule after CI in an animal model (Macaca fascicularis). Cochlear implantation was performed successfully in 14 ears following MTCS. They were classified in two groups upon type of electrode array used. Group A (n = 6) with a FLEX 28 electrode array and Group B (n = 8) with HL14 array. A 6-month follow-up was carried out with periodic objective auditory testing. After their sacrifice, histological processing and subsequent analysis was carried out. Intracochlear findings, vestibular presence of fibrosis, obliteration or collapse is analyzed. Saccule and utricle dimensions and neuroepithelium width is measured. Cochlear implantation was performed successfully in all 14 ears through a round window approach. Mean angle of insertion was >270° for group A and 180-270° for group B. In group A auditory deterioration was observed in Mf 1A, Mf2A and Mf5A with histopathological signs of scala tympani ossification, saccule collapse (Mf1A and Mf2A) and cochlear aqueduct obliteration (Mf5A). Besides, signs of endolymphatic sinus dilatation was seen for Mf2B and Mf5A. Regarding group B, no auditory deterioration was observed. Histopathological signs of endolymphatic sinus dilatation were seen in Mf 2B and Mf 8B. In conclusion, the risk of histological damage of the vestibular organs following minimally traumatic surgical concepts and the soft surgery principles is very low. CI surgery is a safe procedure and it can be done preserving the vestibular structures.

A novel maneuver for diagnosis and treatment of torsional-vertical down beating positioning nystagmus: anterior canal and apogeotropic posterior canal BPPV / Uma nova manobra para diagnóstico e tratamento de nistagmo vertical para baixo com componente de torção: forma apogeotrópica da VPPB do canal anterior e canal posterior

Abstract Introduction In patients with benign paroxysmal positional vertigo, BPPV; a torsional-vertical down beating positioning nystagmus can be elicited in the supine straight head-hanging position test or in the Dix-Hallpike test to either side. This type of nystagmus can be explained by either an anterior canal BPPV or by an apogeotropic variant of the contralateral posterior canal BPPV Until now all the therapeutic maneuvers that have been proposed address only one possibility, and without first performing a clear differential diagnosis between them. Objective To propose a new maneuver for torsional-vertical down beating positioning nystagmus with a clear lateralization that takes into account both possible diagnoses (anterior canal-BPPV and posterior canal-BPPV). Methods A prospective cohort study was conducted on 157 consecutive patients with BPPV. The new maneuver was performed only in those with torsional-vertical down beating positioning nystagmus with clear lateralization. Results Twenty patients (12.7%) were diagnosed with a torsional-vertical down beating positioning nystagmus. The maneuver was performed in 10 (6.35%) patients, in whom the affected side was clearly determined. Seven (4.45%) patients were diagnosed with an anterior canal-BPPV and successfully treated. Two (1.25%) patients were diagnosed with a posterior canal-BPPV and successfully treated with an Epley maneuver after its conversion into a geotropic posterior BPPV. Conclusion This new maneuver was found to be effective in resolving all the cases of torsional-vertical down beating positioning nystagmus-BPPV caused by an anterior canal-BPPV, and in shifting in a controlled way the posterior canal-BPPV cases of the contralateral side into a geotropic-posterior-BPPV successfully treated during the followup visit. Moreover, this new maneuver helped in the differential diagnosis between anterior canal-BPPV and a contralateral posterior canal-BPPV.

Resumo Introdução Em pacientes com vertigem posicional paroxística benigna, VPPB, um nistagmo vertical para baixo com componente de torção pode ser provocado no teste head hanging supino executado na posição reta ou no teste de Dix-Hallpike para qualquer um dos lados. Esse tipo de nistagmo pode ser explicado por uma VPPB do canal anterior ou por uma variante apogeotrópica da VPPB do canal posterior contralateral. Até agora, todas as manobras terapêuticas propostas abordam apenas uma possibilidade, sem antes fazer um diagnóstico diferencial claro entre elas. Objetivo Propor uma nova manobra para nistagmo vertical para baixo com componente de torção com uma lateralização clara que leve em consideração os dois diagnósticos possíveis, VPPB do canal anterior e VPPB do canal posterior. Método Um estudo de coorte prospectivo foi conduzido em 157 pacientes consecutivos com VPPB. A nova manobra foi feita apenas nos pacientes com nistagmo vertical para baixo com componente de torção, com lateralização nítida. Resultados Vinte pacientes (12,7%) foram diagnosticados com nistagmo vertical para baixo com componente de torção. A manobra foi feita em 10 (6,35%) pacientes, nos quais o lado afetado foi claramente determinado. Sete (4,45%) pacientes foram diagnosticados com VPPB do canal anterior e tratados com sucesso. Dois (1,25%) pacientes foram diagnosticados com VPPB do canal posterior e tratados com sucesso com a manobra de Epley após sua conversão para VPPB geotrópica de canal posterior. Conclusão Essa nova manobra mostrou-se eficaz na resolução de todos os casos de VPPB com nistagmo vertical para baixo com componente de torção causada por VPPB do canal anterior. E na mudança de forma controlada dos casos de VPPB do canal posterior do lado contralateral para uma VPPB geotrópica de canal posterior tratada com sucesso durante a consulta de seguimento. Além disso, essa nova manobra auxiliou no diagnóstico diferencial entre a VPPB do canal anterior e a VPPB do canal posterior contralateral.

Apogeotropic Horizontal Canal Benign Paroxysmal Positional Vertigo: Zuma e Maia Maneuver versus Appiani Variant of Gufoni.

Benign paroxysmal positional vertigo (BPPV) is one of the most common disorders that causes dizziness. The incidence of horizontal semicircular canal (HSC) BPPV ranges from 5% to 40.5% of the total number of BPPV cases diagnosed. Several studies have focused on establishing methods to treat BPPV caused by the apogeotropic variant of the HSC, namely, the Appiani maneuver (App). In 2016, a new maneuver was proposed: the Zuma e Maia maneuver (ZeM), based on inertia and gravity. The aim of this study is to analyze the efficacy of App versus ZeM in the resolution of episodes of BPPV produced by an affectation of the horizontal semicircular canal with apogeotropic nystagmus (Apo-HSC). A retrospective, quasi-experimental study was conducted. Patients attended in office (November 2014-February 2019) at a third-level hospital and underwent a vestibular otoneurology assessment. Those who were diagnosed with Apo-HSC, treated with App or ZeM, were included. To consider the efficacy of the maneuvers, the presence of symptoms and/or nystagmus at the first follow up was studied. Patients classified as "A" were those with no symptoms, no nystagmus; "A/N+": no symptoms, nystagmus present during supine roll test; "S": symptoms present. Previous history of BPPV and/or otic pathology and calcium levels were also compiled. From the 54 patients included, 74% were women. The average age was 69. Mean follow-up: 52.51 days. In those patients without previous history of BPPV (n = 35), the probability of being group "A" was 63% and 56% (p = 0.687) when treated with App and ZeM, respectively, while being "A/N+" was 79% and 87% for App and ZeM (p = 0.508). Of the 19 patients who had previous history of BPPV, 13% and 64% were group "A" when treated with App and ZeM (p = 0.043), and 25% and 82% were "A/N+" after App and ZeM, respectively (p = 0.021). In conclusion, for HSC cupulolithiasis, ZeM is more effective than App in those cases in which there is a history of previous episodes of BPPV ("A": 64% (p = 0.043); "A/N+": 82% (p = 0.021)).

Endolymphatic hydrops in the unaffected ear of patients with unilateral Ménière's disease.

PURPOSE: Current studies show that frequency tuning modification is a good marker for the detection of endolymphatic hydrops (EH) employing magnetic resonance imaging (MRI) in patients with Ménière's disease (MD). The purpose of the present study is to analyze the auditory and vestibular function with audiometric and vestibular-evoked myogenic potentials (VEMP) responses, respectively, in both the affected and unaffected ears of patients with unilateral MD using MRI as diagnostic support for the degree of EH. METHODS: We retrospectively reviewed the medical records of 76 consecutive patients with unilateral definite MD (age 55 (28-75); 39 women, 37 men). MRI was used through intravenous gadolinium administration, audiometry, and VEMPs. Functional tests were performed up to a week after the MRI. All were followed up one year after imaging utilizing clinical, auditory, and vestibular testing to rule out bilateral involvement. RESULTS: In the unaffected ear, the mean pure-tone average is normal even in cases with hydrops and, for a similar severity of hydrops is significantly lower than in the affected ear. Significant differences for the amplitude of the response at 0.5 kHz, at 1 kHz between the affected and unaffected ears were found to be lower in the affected ears. The relative amplitude ratio (1 Kz-0.5 kHz) was significantly lower in the affected ear and in the case of the oVEMP response depends on the degree of EH. The response in the unaffected ear was not modified by the presence or the degree of hydrops. CONCLUSION: In the unaffected ear, hydrops is not associated with hearing deterioration. For a similar degree of hydrops, hearing loss is significantly greater in the affected ear. The endolymphatic hydrops in the vestibule induces a frequency bias in the VEMP response only in the affected ear and not in the unaffected ear. Because of these findings we consider that hydrops does not represent an active disorder in the unaffected ear.

Low-Dose Intratympanic Gentamicin for Unilateral Ménière's Disease: Accuracy of Early Vestibulo-Ocular Reflex Gain Reduction in Predicting Long-Term Clinical Outcome.

Background: The number of intratympanic gentamicin (ITG) injections needed to achieve vertigo control in patients with intractable Ménière's disease (MD) may vary from a single dose to several instillations. Changes in different vestibular test results have been used to define an endpoint of treatment, including the decrease of the vestibulo-ocular reflex (VOR) gain elicited by the head-impulse test. Objective: To assess the accuracy of the VOR gain reduction after horizontal canal stimulation, as measured with the video head-impulse test (vHIT) 1 month after the first intratympanic injection, in predicting the need for one or more instillations to control vertigo spells in the long term. Methods: The VOR gain reduction was calculated in 47 patients submitted to (ITG) therapy 1 month after the first instillation. Results: Single intratympanic treatment with gentamicin has a 59.6% efficacy in vertigo control in the long term. Hearing change in the immediate period after treatment (1 month) is not significant to pre-treatment result and is similar for patients who needed multiple doses due to recurrence. Chronic disequilibrium and the need for vestibular rehabilitation were less frequent in patients with a good control of vertigo with just one single injection of gentamicin. A fair accuracy was obtained for the VOR gain reduction of the horizontal canal (area under the curve = 0.729 in the Receiver Operating Characteristic analysis) in predicting the need for one or more ITG. Conclusions: Single intratympanic treatment with gentamicin is an effective treatment for patients with MD. That modality of treatment has very limited damaging effect in hearing. The degree of vestibular deficit induced by the treatment is significant as measured by the reduction in the gain of the VOR but not useful for prognostic purposes.

A Possible Mechanism for Flecainide Induced Dizziness.

OBJECTIVE: Flecainide is an oral class IC antiarrhythmic drug whose most common extracardiac adverse reactions are "dizziness" and "visual disturbances." We describe a case of flecainide associated- bilateral vestibulopathy and a literature review of this drug's effect on the vestibular system. PATIENT: Sixty-nine-year-old man with a 3-month history of unsteadiness and dizziness after an increase in the dose of flecainide. INTERVENTIONS: Otologic examination, video head-impulse test, vestibular evoked myogenic potentials, pure tone audiometry and high-resolution magnetic resonance imaging. RESULTS: Otologic examination, including the head-impulse test, and vestibular testing revealed bilateral vestibulopathy. CONCLUSIONS: Dizziness is a common extracardiac adverse reaction of Flecainide. Based on the clinical case that we present and the literature review carried out, we hypothesized that a possible mechanism by which flecainide might cause dizziness and visual disturbances is bilateral vestibulopathy.

Speech Recognition During Follow-Up of Patients with Ménière's Disease: What Are We Missing?

BACKGROUND: Hearing loss causes a significant reduction in the quality of life of patients with Ménière's disease. Although speech recognition is also affected, it has not been extensively studied. The objective of the study was to describe speech recognition behavior during a prolonged period in patients with unilateral Ménière's disease. METHODS: A prospective case-control study was performed. The case group included patients with defined unilateral Ménière's disease and the control group included patients with progressive non-fluctuating hearing loss. Patients underwent an auditory evaluation periodically. Pure-tone audiometry and speech recognition tests-speech recognition threshold and speech discrimination score-were administered. The dissociation between pure-tone audiometry and speech recognition was assessed through a linear regression analysis. During follow-up, Ménière's disease patients were subdivided into a stable and fluctuating subgroup (a change of >20% in the speech discrimination score with a change no greater than 15 dB in pure-tone audiometry). RESULTS: The average follow-up time was 79.9 months. Fifty-seven patients were included (30 cases, 27 controls). Dissociation between puretone audiometry and speech recognition threshold began to appear in the case group after 21 months, and it was statistically significant at 108 months. Duration of the disease was the only variable studied that influenced the dissociation. The fluctuation subgroup in cluded 56.6% of the cases. CONCLUSION: We described 2 audiological peculiarities in Ménière's disease patients: dissociation between pure-tone audiometry and speech recognition during the evolution of the disease and the fluctuation of speech recognition regardless of the change in pure-tone audiometry. Our results highlight the importance of performing speech recognition tests during follow-up in patients with Ménière's disease.

A novel maneuver for diagnosis and treatment of torsional-vertical down beating positioning nystagmus: anterior canal and apogeotropic posterior canal BPPV.

INTRODUCTION: In patients with benign paroxysmal positional vertigo, BPPV; a torsional-vertical down beating positioning nystagmus can be elicited in the supine straight head-hanging position test or in the Dix-Hallpike test to either side. This type of nystagmus can be explained by either an anterior canal BPPV or by an apogeotropic variant of the contralateral posterior canal BPPV Until now all the therapeutic maneuvers that have been proposed address only one possibility, and without first performing a clear differential diagnosis between them. OBJECTIVE: To propose a new maneuver for torsional-vertical down beating positioning nystagmus with a clear lateralization that takes into account both possible diagnoses (anterior canal-BPPV and posterior canal-BPPV). METHODS: A prospective cohort study was conducted on 157 consecutive patients with BPPV. The new maneuver was performed only in those with torsional-vertical down beating positioning nystagmus with clear lateralization. RESULTS: Twenty patients (12.7%) were diagnosed with a torsional-vertical down beating positioning nystagmus. The maneuver was performed in 10 (6.35%) patients, in whom the affected side was clearly determined. Seven (4.45%) patients were diagnosed with an anterior canal-BPPV and successfully treated. Two (1.25%) patients were diagnosed with a posterior canal-BPPV and successfully treated with an Epley maneuver after its conversion into a geotropic posterior BPPV. CONCLUSION: This new maneuver was found to be effective in resolving all the cases of torsional-vertical down beating positioning nystagmus-BPPV caused by an anterior canal-BPPV, and in shifting in a controlled way the posterior canal-BPPV cases of the contralateral side into a geotropic-posterior-BPPV successfully treated during the followup visit. Moreover, this new maneuver helped in the differential diagnosis between anterior canal-BPPV and a contralateral posterior canal-BPPV.

Endolymphatic Hydrops in Fluctuating Hearing Loss and Recurrent Vertigo.

Background: Endolymphatic hydrops (EH) is the histopathological hallmark of Ménière's disease (MD) and has been found by in vivo magnetic resonance imaging (MRI) in patients with several inner ear syndromes without definite MD criteria. The incidence and relevance of this finding is under debate. Purpose: The purpose of the study is to evaluate the prevalence and characteristics of EH and audiovestibular test results in groups of patients with fluctuating audiovestibular symptoms not fulfilling the actual criteria for definite MD and compare them with a similar group of patients with definite MD and a group of patients with recent idiopathic sudden neurosensory hearing loss (ISSNHL). Material and Methods: 170 patients were included, 83 with definite MD, 38 with fluctuating sensorineural hearing loss, 34 with recurrent vertigo, and 15 with ISSNHL. The clinical variables, audiovestibular tests, and EH were evaluated and compared. Logistic proportional hazard models were used to obtain the odds ratio for hydrops development, including a multivariable adjusted model for potential confounders. Results: No statistical differences between groups were found regarding disease duration, episodes, Tumarkin spells, migraine, vascular risk factors, or vestibular tests; only hearing loss showed differences. Regarding EH, we found significant differences between groups, with odds ratio (OR) for EH presence in definite MD group vs. all other patients of 11.43 (4.5-29.02; p < 0.001). If the ISSNHL group was used as reference, OR was 55.2 (11.9-253.9; p < 0.001) for the definite MD group, 9.9 (2.1-38.9; p = 0.003) for the recurrent vertigo group, and 5.1 (1.2-21.7; p = 0.03) for the group with fluctuating sensorineural hearing loss. Conclusion: The percentage of patients with EH varies between groups. It is minimal in the ISSNHL group and increases in groups with increasing fluctuating audiovestibular symptoms, with a rate of severe EH similar to the known rate of progression to definite MD in those groups, suggesting that presence of EH by MRI could be related to the risk of progression to definite MD. Thus, EH imaging in these patients is recommended.

VEMPs and Dysautonomia Assessment in Definite Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome (CANVAS): a Case Series Study.

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a recently described slowly progressive ataxia with severe imbalance due to the compromise of three of the four sensory inputs for balance, leaving only vision unaffected. Bilateral vestibulopathy is present but saccular and utricular function, measured by vestibular evoked myogenic potentials (VEMPs), has not been widely studied in these patients. Dysautonomia has been reported but is not among the diagnostic criteria. We performed a database analysis to identify patients evaluated between 2003 and 2019 with probable diagnosis of CANVAS by using key words "bilateral vestibulopathy and/or cerebellar ataxia and/or sensory polyneuropathy." Five out of 842 met all conditions. Patients underwent neurological/neurootological exam, brain MRI, visually enhanced vestibulo-ocular reflex (VVOR) exam by high-speed video-oculography using video-Head Impulse Test (vHIT), VEMPs, neurophysiological studies, and genetic tests to exclude other causes of ataxia. Dysautonomia was addressed by the standardized survey of autonomic symptoms. All patients had clinically definite CANVAS as brain MRI showed vermal cerebellar atrophy, neurophysiological studies showed a sensory neuronopathy pattern (absent sensory action potentials), VVOR was abnormal bilaterally, and genetic tests ruled out other causes of ataxia including SCA 3 and Friedreich ataxia. Patients had at least 3 dysautonomic symptoms, including xerostomia/xerophthalmia (5/5). VEMP results varied among patients, ranging from normal to completely abnormal. We found inconsistent results with VEMPs. The utilization of VEMPs in more CANVAS cases will determine its utility in this syndrome. Dysautonomia may be included in the diagnostic criteria.

Cochlear Implantation With a Dexamethasone Eluting Electrode Array: Functional and Anatomical Changes in Non-Human Primates.

OBJECTIVES: The aim of this study was to determine whether the dexamethasone-eluting electrode (DEXEL) has a protective anti-inflammatory effect in a normal hearing implanted cochlea, as well as its performance and safety. DESIGN: Ten healthy and normal hearing cynomolgus macaques (Mf) were divided into two cohorts: DEXEL group (DG) (CONCERTO CI device containing a DEXEL) and conventional CI group (CG) (unmodified CONCERTO CI device). The electrode was inserted into the scala tympani via the round window with an angle of insertion of 270 degrees. Auditory, impedance, electrically evoked compound action potential (eCAP), and recovery of function measurements were recorded monthly until sacrifice at 6 months post-implantation. A histologic analysis was also performed. RESULTS: At 6 months, measurement of auditory brainstem responses revealed a mean threshold shift, as well as a mean impedance value, lower in the DEXEL group. The minimum eCAP for the remaining active contacts in the DEXEL group was 68% of that in the conventional CI group. Also at 6 months, the eCAP amplitude produced by a stimulating current of 800 cu (eCAP) was almost 2.5-fold higher in the DEXEL group than in the conventional CI group (1338.86 ±â€Š637.87 µV versus 545.00 ±â€Š137.37 µV; p = 0.049). Tissue reactions, in particular fibrosis and ossification, were more common in the conventional CI group. CONCLUSIONS: The CI electrode array equipped with a dexamethasone-eluting component tested in macaques evidence that delivery of dexamethasone to the inner ear may produce rapid and long-lasting improvement of hearing with fewer neural tissue reactions.