Introduction
The natural history of Ménière’s disease is defined as the progression of its signs and symptoms over time in the absence of surgical or ablative vestibular treatments. Most studies have overlooked the potential effect of conventional medical therapies, as their efficacy remains uncertainReference da Costa, de Sousa and de Toledo Piza1, Reference Friberg, Stahle and Svedberg2
The effect of Ménière’s disease on quality of life is considerable due to its episodic nature and the uncertainty associated with the onset of vertigo attacks.Reference Green, Verrall and Gates3 The disease typically affects individuals in middle age, a period marked by significant occupational and family responsibilities. The unpredictability of vertigo episodes, often accompanied by panic, contributes to the development of psycho-affective disorders such as anxiety, depression, and agoraphobia. Several studies have correlated these conditions with more severe forms of the disease or with a more aggressive clinical course, as observed in bilateral Ménière’s disease.Reference Savastano, Maron, Mangialaio, Longhi and Rizzardo4–Reference Anderson and Harris6
From a clinical perspective, vertigo is the most disabling symptom of Ménière’s disease, followed by hearing loss and tinnitus. Patients with bilateral Ménière’s disease report a greater degree of perceived disability, and a poorer quality of life compared to those with unilateral Ménière’s disease. Furthermore, dizziness is considered a more limiting symptom in bilateral Ménière’s disease than in unilateral Ménière’s disease.Reference Lopez-Escamez, Viciana and Garrido-Fernandez7
Various tools have been used to assess the quality of life in patients with Ménière’s disease. One of the most widely employed is the perceived severity scale of the American Academy of Otolaryngology Head and Neck Surgery (AAO-HNS), established in the 1985 Ménière’s disease criteria, which evaluates the functional impact of the disease over the previous six months using a six-item ordinal scale.Reference Pearson and Brackmann8 Additionally, other scales such as the Hearing Disability and Handicap Scale, Vertigo Handicap Index, and International Tinnitus Inventory have been utilised. Disease-specific questionnaires, including the Ménière’s Disease Quality of Life (MenQOL) and the Ménière’s Disease Questionnaire (MenQ), have also been developed.Reference Gürkov, Pyykö, Zou and Kentala9, Reference Quimby, Brant, Staab and Ruckenstein10
The natural course of Ménière’s disease is highly heterogeneous, characterised by alternating symptomatic periods and intercritical intervals lasting months or even years. Although the frequency of vertigo episodes tends to decrease over time, their duration and intensity may increase, leading to a greater perception of severity in later stages of the disease. The perception of quality of life in Ménière’s disease patients is influenced not only by the frequency of vertigo episodes but also by their duration and intensity, both of which determine the functional effect of the disease and its natural progression. For this reason, current diagnostic criteria define a typical Ménière’s disease attack as lasting between 20 minutes and 12 hours.Reference Lopez-Escamez, Carey, Chung, Goebel, Magnusson and Mandalà11
However, previous studies have used diverse methodologies to assess the duration and intensity of vertigo episodes, complicating the comparison of results and the interpretation of disease progression.Reference Friberg, Stahle and Svedberg2, Reference Havia and Kentala12–Reference Charachon, Gratacap, Barthez and Benoît-Gonin16 To address this variability, the vertigo index has been proposed as a tool that integrates both the duration and frequency of vertigo episodes. As described by Pérez-Garrigues et al.,Reference Pérez-Garrigues, Lopez-Escamez, Perez, Sanz, Orts and Marco13 this index is calculated by multiplying the number of episodes by a factor reflecting their duration: grade 2 episodes (15 min to 2 h) are multiplied by 1, grade 3 episodes (2–6 h) are multiplied by 2, and grade 4 episodes (> 6 h) are multiplied by 3. Grade 1 episodes (< 15 min) are not included in the calculation, as they do not meet the diagnostic criteria for typical Ménière’s disease attacks. The final index is obtained by summing the products for each category, thereby providing a single numerical value representing the cumulative vertigo burden since the last clinical assessment.
Both perceived quality of life and vertigo severity vary throughout the disease and differ between clinical subtypes. Bilateral Ménière’s disease, with its more aggressive course, is associated with greater functional impairment, while in unilateral Ménière’s disease, different evolutionary patterns have been identified based on the number and duration of vertigo episodes. One way to classify unilateral Ménière’s disease is by the number of episodes experienced in the first 10 years of the disease. The classification proposed by Pérez-Carbonell et al. allows patients to be stratified into different models according to the frequency of vertigo episodes over time.Reference Pérez-Carbonell, Orts-Alborch, Pérez-Guillén, Tenías-Burillo, Pla-Gil and Marco-Algarra17 Given that this classification is based on vertigo burden, differences in quality-of-life effects have been observed across groups, with greater functional impairment in those exhibiting a more active disease course.
This study aimed to analyse the evolution of the vertigo index and its relationship with the perceived level of disability in patients with both unilateral and bilateral Ménière’s disease. The progression of these parameters were compared over time, differentiating within the unilateral Ménière’s disease group those patients with follow-up data from disease onset who have been classified into previously described evolutionary models.
Materials and methods
A longitudinal descriptive study was conducted on patients diagnosed with Ménière’s disease, both unilateral and bilateral, who were treated at two referral centres. Data were collected and stored in a database using the MEN software, covering the period from 1977 to 2023. All patients included in the cohort met the updated diagnostic criteria for Ménière’s disease and bilateral Ménière’s disease. Given the evolution of these criteria over time, a retrospective review was performed to ensure that all cases adhered to the most recent definitions. Patients with incomplete phenotypic forms of the disease, such as possible or probable Ménière’s disease, were excluded.
At the first visit to each centre, patients were advised to attend follow ups every six months, regardless of whether they were asymptomatic or if their symptoms were controlled with the prescribed treatment. At each visit, clinical and evolutionary variables were recorded, including the date of the first vertigo episode, the number of episodes in the previous 12 months, and the degree of disability over the past six months, assessed using the six-item ordinal scale defined in the 1985 AAO-HNS criteria.Reference Pearson and Brackmann8 For analysis, responses were grouped into two categories: mild disability (patients selecting one of the first three options) and moderate/severe disability (patients selecting one of the last three options). Additional variables recorded included the emergence of new symptoms, the number of episodes in the previous six months, and their duration, classified as follows: grade 1 for episodes lasting less than 15 minutes; grade 2 for episodes lasting between 15 minutes and 2 hours; grade 3 for episodes lasting between 2 and 6 hours; and grade 4 for episodes lasting more than 6 hours. Based on these data, the vertigo index was calculated,Reference Pérez-Garrigues18–Reference Belinchon, Pérez-Garrigues and Tenias20 allowing quantification of the vertigo burden by integrating both episode duration and frequency.
From the first visit, patients were instructed to record in a provided log the frequency and duration of their vertigo episodes, accompanying symptoms (e.g., tinnitus, fluctuating hearing loss), and the effect of vertigo on daily life, aiming to minimise recall and reporting bias. Before data analysis, medical records were reviewed to confirm Ménière’s disease laterality, compliance with diagnostic criteria, and completeness of data.
For analysis, patients were stratified into two main groups: those with bilateral Ménière’s disease and those with unilateral Ménière’s disease. Within the unilateral Ménière’s disease group, a subset of patients with follow-up data from disease onset was identified. In these patients, vertigo progression enabled classification into three evolutionary models, based on the pattern of vertigo episodes in the first 10 years of disease (clinical evolutionary models).Reference Pérez-Carbonell, Orts-Alborch, Pérez-Guillén, Tenías-Burillo, Pla-Gil and Marco-Algarra17, Reference Pérez-Carbonell, Orts-Alborch, Pla-Gil, Pérez-Guilén, Tenías-Burrillo and Marco-Algarra21
The statistical analysis focused on describing the collected variables through univariate analysis, estimating appropriate descriptive statistics based on the nature of each variable. For quantitative variables, measures of central tendency (mean or median, depending on distribution) and dispersion (standard deviation or interquartile range) were calculated. Qualitative variables were analysed by absolute and relative frequencies. In the bivariate analysis, relationships between variables were examined using chi-square tests for categorical variables, correlation tests for quantitative variables, and mean comparison tests (Student’s t-test, ANOVA, or non-parametric equivalents: Mann–Whitney U test and Kruskal–Wallis H test). Statistical analysis was conducted using SPSS version 22.
Results
Patients with Unilateral Ménière’s Disease (n = 327)
A total of 19.6 per cent of vertigo episodes reported by patients with unilateral Ménière’s disease were classified as moderate or severe in terms of functional disability throughout the disease course. The relative frequency of episodes with moderate or severe disability showed a marked decline during the first five years of disease evolution (Figure 1). Similarly, the vertigo index exhibited a trend towards stabilisation over time (Figure 2).
Evolution of the relative frequency of moderate/severe disability vertigo episodes over years of disease progression.
Evolution of the relative vertigo index over the years of disease progression.
At the end of the follow up, half of the patients reported either no disability or only mild disability. Specifically, 11.2 per cent indicated that vertigo did not interfere with their daily activities, while 48.3 per cent reported that, although vertigo episodes required them to pause their activities temporarily, they were able to resume them without major difficulties. A total of 18.2 per cent stated that vertigo episodes forced them to modify their plans, while another 18.2 per cent indicated that they had to make additional efforts to maintain their normal life. Finally, 3.5 per cent reported being unable to work or drive, and only 0.7 per cent were on long-term sick leave.
Patients with unilateral Ménière’s disease followed from disease onset (n = 87)
The degree of functional disability varied according to the clinical evolutionary model of the disease. Patients classified under evolutionary model 3 exhibited a higher percentage of episodes classified as severe and a higher average vertigo index throughout the disease course. Specifically, the proportion of severe episodes was 11.15 per cent in model 1, 7.55 per cent in model 2, and 17.45 per cent in model 3. Similarly, the mean vertigo index was highest in model 3 (Figures 3 and 4).
Evolution of the vertigo index over the first 10 years of disease progression in the identified patterns (from left to right: model 1, 2 and 3).
Evolution of the frequency of vertigo episodes classified as moderate/severe disability over the first 10 years of disease progression in the identified patterns (from left to right: model 1, 2, and 3).
Patients with bilateral Ménière’s disease (n = 59)
Patients with bilateral Ménière’s disease exhibited greater functional impairment compared to those with unilateral Ménière’s disease. Throughout disease evolution, 31.4 per cent of vertigo episodes were classified as moderate or severe. During the early years, most vertigo episodes were perceived as highly disabling. However, over time, the perceived severity progressively decreased, reaching values below 20 per cent after 10 years of disease evolution (Figure 5).
Average percentage of moderate/severe vertigo episodes reported by patients with bilateral Ménière’s disease according to years of disease progression.
The vertigo index showed a progressive reduction as the disease advanced. The highest average values were recorded during the first 10 years, remaining below 5 throughout the rest of the follow-up period, except for specific years of increased disease activity (Figure 6).
Average vertigo index of vertigo episodes in patients with bilateral Ménière’s disease according to years of disease progression.
Discussion
Degree of Disability and Evolution of Vertigo
Disability caused by Ménière’s disease has been assessed using various methodologies, as reported in the literature.Reference Kentala, Levo, Pyykko and Pyykkő22, Reference Yardley, Dibb and Osborne23 In this study, we used two primary variables: the vertigo index, which combines episode frequency and duration,Reference Pérez-Garrigues18 and the AAO-HNS disability classification scale. Both tools demonstrated a direct correlation, indicating that patients with longer and more frequent episodes experience greater functional impairment.
Patients with unilateral Ménière’s disease reported that 11 per cent of their vertigo episodes were moderate or severe, which is a lower percentage than that described in other studies reporting greater functional effect.Reference Anderson and Harris6, Reference Hägnebo, Melin, Larsen, Lindberg, Lyttkens and Scott24 This discrepancy may be partially explained by the temporal distribution of vertigo episodes. Previous research has shown that vertigo episodes are more frequent in the early years of the disease, leading to more significant functional impairment during this initial phase.Reference Söderman, Bagger-Sjöbäck, Bergenius and Langius25 However, in our study, disability was assessed either at the end of follow up or as an average across the entire disease course, which may have masked the initial effects observed in other reports.
When analysing the evolution of disability in unilateral Ménière’s disease patients, a significant increase in the proportion of moderate or severe vertigo episodes was observed after 20 years of disease progression (Figure 1). This finding aligns with previous studiesReference Havia and Kentala12, Reference Huppert, Strupp and Brandt26 suggesting that, although the total number of episodes decreases over time, the remaining episodes tend to be longer and more intense, increasing the perception of disability.
Conversely, bilateral Ménière’s disease patients exhibited a higher proportion of moderate or severe vertigo episodes (31.4 per cent) than unilateral Ménière’s disease patients. This difference could explain the higher incidence of psycho-affective disorders, such as anxiety and depression, observed in bilateral Ménière’s disease compared to unilateral Ménière’s disease, as previously reported by Sakagami et al.Reference Sakagami, Kitahara, Okayasu, Yamashita, Hasukawa and Ota27 Additionally, this greater functional impairment may justify the poorer outcomes in quality-of-life assessments, such as the Health-Related Quality of Life (HRQL) and the Short Form-16 (SF-16).Reference Lopez-Escamez, Viciana and Garrido-Fernandez7
Comparison with Previous Literature
Several studies have examined vertigo evolution in Ménière’s disease, providing data that reinforce our findings. Havia and KentalaReference Havia and Kentala12 conducted a retrospective study in 243 Ménière’s disease patients, reporting that 63 per cent experienced a vertigo episode once a month, while 5 per cent had continuous vertigo. Furthermore, episode intensity and duration increased with disease progression.Reference Havia and Kentala12 These results are consistent with our findings, where patients with longer follow-up periods reported more disabling episodes despite a decrease in frequency.
Pérez-Garrigues et al.Reference Pérez-Garrigues, Lopez-Escamez, Perez, Sanz, Orts and Marco13 conducted a prospective multicentre study with a seven-year follow up of 588 patients receiving conservative treatment. They demonstrated a progressive decline in the number of annual episodes during the first eight years, followed by a plateau and a slight further reduction over the next decade. This pattern of initial decline and later stabilisation is consistent with our results regarding vertigo index evolution and disability perception over time.
Tokumasu et al.Reference Tokumasu, Fujino, Yoshio and Hoshino14, Reference Tokumasu, Fujino, Naganuma, Hoshino and Arai28 also reported a decrease in vertigo episode frequency as the disease progressed. However, in patients with more than 15 years of disease evolution, they observed a resurgence in the number of annual episodes, suggesting a late-phase vestibular decompensation. Similar findings were reported by Green et al.Reference Green, Blum and Harner15 who found that, after nine years of follow up, 54 per cent of patients no longer experienced vertigo episodes, while 30 per cent reported a substantial reduction in frequency. However, 17 per cent described their episodes as more intense, which aligns with the increased perception of disability observed in our study.
Other studies, including those by Friberg et al.Reference Friberg, Stahle and Svedberg2 and Charachon et al.Reference Charachon, Gratacap, Barthez and Benoît-Gonin16 have also documented a progressive reduction in vertigo episodes over time, although some patients continued to experience recurrent vertigo even after 20 years of disease progression. Regarding inter-critical periods, Lee et al.Reference Lee, Paparella, Margolis and Le29 and OosterveldReference Oosterveld30 reported that some patients experienced vertigo-free intervals of up to five years, followed by periods of heightened disease activity. These findings suggest that Ménière’s disease may exhibit unpredictable fluctuations, even in advanced stages.
Clinical Implications
The results of this study highlight the importance of long-term patient monitoring in Ménière’s disease, given that vertigo evolution and functional effects vary over time. The greater disability observed in bilateral Ménière’s disease patients underscores the need for more intensive management strategies, including vestibular rehabilitation and psychological support. Furthermore, the increase in episode severity in later disease stages reinforces the necessity of prolonged follow up to adapt treatment according to individual disease progression.
• The effect of Ménière’s disease on quality of life is closely related to frequency, duration, and perceived severity of vertigo episodes
• Bilateral Ménière’s disease shows a higher proportion of moderate/severe vertigo episodes compared to unilateral Ménière’s disease, leading to greater functional disability
• In unilateral Ménière’s disease, vertigo frequency decreases over time; however, after 20 years of disease progression, remaining episodes tend to be longer and more disabling
• Long-term follow-up is crucial to adapting treatment strategies, particularly in bilateral Ménière’s disease, where vestibular and psycho-affective impairment is more pronounced
• Classifying unilateral Ménière’s disease based on vertigo burden in the first 10 years of disease evolution helps identify patients at higher risk of long-term disability
Conclusions
Patients with unilateral Ménière’s disease exhibited a progressive reduction in episode frequency over time; however, in later stages, the remaining episodes were perceived as longer and more disabling. In bilateral Ménière’s disease patients, the proportion of moderate or severe vertigo episodes was more than double that of unilateral Ménière’s disease patients, suggesting greater functional and psycho-affective effects.
These findings underscore the need to tailor Ménière’s disease management according to disease evolution. In early stages, the focus should be on controlling episode frequency, while in later stages, it is essential to address residual disability and associated psycho-affective disorders.
Further studies should investigate the factors driving increased disability perception in later disease stages and assess the efficacy of specific interventions for these patients. Additionally, future research could explore long-term quality-of-life outcomes and the identification of subgroups at risk of severe functional deterioration.
Competing interests
None declared.
Funding
This research received no specific grant from any funding agency, commercial or not-for-profit sectors.
