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.

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.

Bilateral Vestibular Hypofunction in the Time of the Video Head Impulse Test.

OBJECTIVES: Bilateral vestibulopathy is a clinical syndrome in which laboratory testing plays a crucial diagnostic role. We aimed to establish the frequency of detection of that finding in a tertiary level hospital considering the new methods of laboratory vestibular examination nowadays in use, with respect to the conventional caloric and rotatory chair test approaches. DESIGN: Two similar time periods (5 years) were retrospectively evaluated, and the demographic, clinical data and test results from 4,576 patients were reviewed. In the first period, the diagnosis was based on caloric and rotatory chair tests and, in the second, on the video head impulse test. RESULTS: Of the patients included, 3.77% in the first period and 4.58% in the second met the criteria for bilateral vestibular hypofunction; there was no significant difference between both periods. CONCLUSIONS: The functional vestibular evaluation to detect bilateral deficiency makes no significant difference to the number of patients diagnosed with a bilateral vestibulopathy. New diagnostic categories probably depend not only on the availability and accessibility of complete vestibular and visual-vestibular evaluation, but also on recent advances in defining vestibular disorders. Bilateral vestibular hypofunction manifests with very different patterns. Progress in more detailed definition (clinical and laboratory) is needed, in particular when all 6 semicircular canals and both maculae are available for testing.

Relevance of Artifact Removal and Number of Stimuli for Video Head Impulse Test Examination.

OBJECTIVE: To evaluate the effect of artifacts on the impulse and response recordings with the video head impulse test (VHIT) and determine how many stimuli are necessary for obtaining acceptably efficient measurements. METHODS: One hundred fifty patients were examined using VHIT and their registries searched for artifacts. We compared several variations of the dataset. The first variation used only samples without artifacts, the second used all samples (with and without artifacts), and the rest used only samples with each type of artifact. We calculated the relative efficiency (RE) of evaluating an increasingly large number of samples (3 to 19 per side) when compared with the complete sample (20 impulses per side). RESULTS: Overshoot was associated with significantly higher speed (p = 0.005), higher duration (p < 0.001) and lower amplitude of the impulses (p = 0.002), and consequent higher saccades' latency (p = 0.035) and lower amplitude (p = 0.025). Loss of track was associated with lower gain (p = 0.035). Blink was associated with a higher number of saccades (p < 0.001), and wrong way was associated with lower saccade latency (p = 0.012). The coefficient of quartile deviation escalated as the number of artifacts of any type rose, indicating an increment of variability. Overshoot increased the probability of the impulse to lay on the outlier range for gain and peak speed. Blink did so for the number of saccades, and wrong way for the saccade amplitude and speed. RE reached a tolerable level of 1.1 at 7 to 10 impulses for all measurements except the PR score. CONCLUSIONS: Our results suggest the necessity of removing artifacts after collecting VHIT samples to improve the accuracy and precision of results. Ten impulses are sufficient for achieving acceptable RE for all measurements except the PR score.

Oscillopsia in Bilateral Vestibular Hypofunction: Not Only Gain But Saccades Too.

OBJECTIVES: Oscillopsia is a disabling condition for patients with bilateral vestibular hypofunction (BVH). When the vestibulo-ocular reflex is bilaterally impaired, its ability to compensate for rapid head movements must be supported by refixation saccades. The objective of this study is to assess the relationship between saccadic strategies and perceived oscillopsia. DESIGN: To avoid the possibility of bias due to remaining vestibular function, we classified patients into two groups according to their gain values in the video head impulse test. One group comprised patients with extremely low gain (0.2 or below) in both sides, and a control group contained BVH patients with gain between 0.2 and 0.6 bilaterally. Binary logistic regression (BLR) was used to determine the variables predicting oscillopsia. RESULTS: Twenty-nine patients were assigned to the extremely low gain group and 23 to the control group. The BLR model revealed the PR score (saccades synchrony measurement) to be the best predictor of oscillopsia. Receiver operating characteristic analysis determined that the most efficient cutoff point for the probabilities saved with the BLR was 0.518, yielding a sensitivity of 86.6% and specificity of 84.2%. CONCLUSIONS: BVH patients with higher PR values (nonsynchronized saccades) were more prone to oscillopsia independent of their gain values. We suggest that the PR score can be considered a useful measurement of compensation.

Wireless inertial measurement unit (IMU)-based posturography.

BACKGROUND: Classical posturography techniques have been recently enhanced by the use of different motion tracking devices, but for technical reasons they are not used to track directly the body spatial position of a subject. OBJECTIVE: To describe and clinically evaluate a wireless inertial measurement unit-based mobile system to track body position changes. METHODS: The developed system used a calculus transformation method using the acceleration data corrected by Kalman and Butterworth filters to output position data. A prospective non-randomized clinical study involving 15 healthy subjects was performed to evaluate the agreement between the confidence ellipse areas synchronously measured by the new developed system and a classical posturography system while performing a modified clinical test of sensory interaction in balance. RESULTS: The overall intra-class correlation index was 0.93 (CI 0.89, 0.96). Grouped by conditions, under conditions 1-4, Pearson's correlation was 0.604, 0.78, 0.882, and 0.81, respectively. CONCLUSION: The developed wireless inertial measurement unit-based posturography system was valid for tracking the sway variances in normal subjects under habitual clinical testing conditions. Further studies are needed to validate this system on patients and also under other posture conditions.

Visual Performance and Perception as a Target of Saccadic Strategies in Patients With Unilateral Vestibular Loss.

OBJECTIVES: To evaluate the ability of saccadic strategies developed during vestibular compensation to reduce the effect of an impaired vestibulo-ocular reflex (VOR) on a retinal smear and image motion sensation. DESIGN: Twenty patients with unilateral vestibular loss were examined with a video head impulse test before and after vestibular rehabilitation (VR) with the use of gaze stabilization and refixation saccades training. Head and eye velocity functions were processed to infer the retinal eccentricity, and through its correlation with visual acuity (VA), several measurements are proposed to evaluate the influence of VR on saccades behavior and visual performance. To isolate the effect of saccades on the findings and avoid bias because of gain differences, only patients whose VOR gain values remained unchanged after VR were included. RESULTS: Improved contribution of covert saccades and reduction of overt saccades latency were measured after VR. We found significant differences when assessing both the interval less than 70% VA (50.25 ms), which is considered the limit of a moderate low vision, and less than 50% VA (39.515 ms), which is the limit for severe low vision. Time to recover a VA of 75% (near normal) was reduced in all the patients (median: 56.472 ms). CONCLUSION: Despite the absence of VOR gain improvement, patients with unilateral vestibular loss are able to develop saccadic strategies that allow the shortening of the interval of retinal smear and image motion. The proposed measurements might be of use to evaluate VR outcomes and visually induced impairment.

Recent onset disequilibrium mimicking acute vestibulopathy in early multiple sclerosis.

The differential diagnosis of patients with acute unilateral vestibulopathy rests in the proper clinical assessment and use of selected tests of vestibular function. In case of a central nervous system lesion as in Multiple Sclerosis, the case shown here, it is of particular importance to observe congruency between severity of symptoms and signs and, of topographic diagnosis. We report a case of a 37-year-old woman with recent onset disequilibrium that after careful analysis of the different test results several incongruences were found; this prompted a radiological study that provided the clue to diagnosis. After treatment the patient recovered completely not only clinically but also in vestibular deficit.

Variants of posterior semicircular canal involvement in benign paroxysmal positional vertigo.

Benign paroxysmal positional vertigo is the most common cause of peripheral vertigo. It is characterized by short and recurrent episodes of vertigo, trigged by specific head movements that displace otoconia within the semicircular canals. The movement of dislodge otoconia from the utricle cause abnormal positional endolymphatic currents. Primary treatment involves reposition maneuvers aimed at moving the displaced otoconia out the affected canal, therefore correct identification of the affected canal is essential for the diagnosis. The posterior semicircular canal (PSC) is the most frequently affected due to its spatial orientation and the force of gravity. Recent technological advances have allowed for better assessment of positional nystagmus during diagnostic and therapeutic maneuvers, revealing various possible scenarios of PSC involvement. Regarding the PSC, otoconia may be found in different parts of the canal, and not just in the expected location, floating in the long arm of the canal. The understanding of these variants is crucial, as the prognosis and the disease progression differ in such cases. This review aims to describe the six possible variants of PSC involvement described so far.

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.

Recent Advances in the Diagnosis and Treatment of Vestibular Disorders.

Vestibular medicine "embraces a wide approach to the potential causes of vestibular symptoms, acknowledging that vertigo, dizziness, and unsteadiness are non-specific symptoms that may arise from a broad spectrum of disorders, spanning from the inner ear to the brainstem, cerebellum and supratentorial cerebral networks, to many disorders beyond these structures" [...].

The vestibular system: Contributions of Lorente de Nó.

BACKGROUND: Rafael Lorente de Nó was a neuroscientist that worked alongside two of the giants of Medicine, the Nobel Prize winners Cajal and Bárány. OBJECTIVE: To describe the contributions of Lorente de Nó to vestibular neuroscience. METHODS: Detailed review of the publications of Lorente de Nó and analysis of the archives from Junta para Ampliación de Estudios e Investigaciones Científicas at Residencia de Estudiantes (Madrid, Spain), Casa de Salud Valdecilla at Hospital Universitario Marqués de Valdecilla (Santander, Spain), Becker Medical Library at Washington University (St. Louis, MO, USA), Rockefeller Archive Center (Sleepy Hollow, New York, NY, USA), Archivo Fernando de Castro (Madrid, Spain), Biblioteca Nacional de España (Madrid, Spain) and Legado Cajal at Instituto Cajal (Madrid, Spain). Most of this material is unpublished and includes over a hundred letters to or from Lorente. RESULTS: Lorente de Nó made a substantial contribution to our understanding of the vestibular system. Amongst these, he meticulously detailed the course of the vestibular nerve and its central projections. He described the vestibulo-ocular reflex as the consequence of an integration of the various nuclei and connections across the vestibular system, rather than a simple three-neuron arc. He also highlighted the role of the reticular formation in the generation of the fast phase of the nystagmus. CONCLUSIONS: Lorente de Nó was a pioneer of modern neuro-otology, having made outstanding contributions to vestibular neuroscience, forging novel discoveries that still burn true today.

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.

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.

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.

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.

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.

Synchronized refixation saccades in enhanced VVOR test. A new application for PR score.

OBJECTIVE: Main objectives for this study were to develop a quantification method to obtain a Perez-Rey (PR) score adapted to the VVOR test and to evaluate the correlation of the PR score obtained with quantified VVOR with the PR score of the vHIT test. METHODS: A new PR score calculation method for quantified VVOR test was developed using the MATLAB computational software based on saccadic response time latency variability between each head oscillation cycle of the VVOR test. Retrospective correlation between PR scores in VVOR and vHIT tests, performed in the same vHIT testing session for patients with vestibular neuritis and vestibular neurectomy, was performed to correlate new PR (VVOR) score with the classic PR (vHIT) score. RESULTS: Thirty patients were included: 11 post-neurectomy and 19 subacute vestibular neuritis. Pearson's correlation coefficient (R2) for the overall sample was 0.92 (p < 0.001) and 95% confidence interval was 0.85 -0.96. In the linear mixed-effects statistical model developed, only PRVHIT and PRVVOR scores showed statistical association in Wald X2 test (p = 0.008). CONCLUSION: The new developed PR score for synchronization measurement of saccadic responses in VVOR testing is a valid method that outputs synchronization values and highly correlates with PR score in vHIT test.

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.