Eur Arch Otorhinolaryngol DOI 10.1007/s00405-015-3638-1
OTOLOGY
Disability perception in Menie`re’s disease: when, how much and why? Andres Soto-Varela1,2 • Belen Huertas-Pardo3 • Pilar Gayoso-Diz4 Sofia Santos-Perez1,2 • Ines Sanchez-Sellero5
•
Received: 12 February 2015 / Accepted: 24 April 2015 Springer-Verlag Berlin Heidelberg 2015
Abstract The purpose of the study was to evaluate selfperceived handicap in patients with definite Menie`re’s disease (MD). A cross-sectional study was conducted. To examine the self-perception of disability, participants completed a DHI (Dizziness Handicap Inventory). Parameters compared with DHI scores: sex, age, unilateral/bilateral affectation, time elapsed since the onset of symptoms, pure-tone average (PTA), stages of MD, audiometric change (last 6 months), PTA in low frequencies (PTAl) and audiometric change in PTAl, subjective perception of fluctuating hearing threshold, tinnitus between attacks, number of vertiginous episodes (last 6 months), time elapsed since last attack, subjective perception of instability intercrises and Tumarkin attacks. 90 patients were included; they completed a total of 104 questionnaires. DHI scores ranged from 2 to 100 (average: 47.08, SD
24.45). In 29 cases (27.9 %) the disability perception was mild, in 43 (41.3 %) moderate, and in 32 (30.8 %) severe. Correlation between disability perception and some vestibular symptoms was found: number of typical attacks (last 6 months), time elapsed since last attack, instability intercrises and Tumarkin attacks. No relationship was found with the rest of variables. Disability perception in patients with MD depends primarily on vestibular symptoms (particularly, instability and frequency of attacks). So, we suggest to design a new staging system of MD taking into account both auditory criteria and also vestibular symptoms. Keywords Disability Menie`re’s disease DHI Instability Staging
Introduction & Andres Soto-Varela [email protected] 1
Division of Neurotology, Department of Otorhinolaryngology, Complexo Hospitalario Universitario de Santiago de Compostela, Travesı´a da Choupana, s/n, 15706 Santiago de Compostela, Spain
2
Department of Dermatology and Otorhinolaryngology, University of Santiago de Compostela, Santiago de Compostela, Spain
3
Department of Otorhinolaryngology, Complexo Hospitalario Universitario de Santiago de Compostela, Santiago de Compostela, Spain
4
Clinical Epidemiology Unit, Instituto de Investigacio´n Sanitaria de Santiago (IDIS), Complexo Hospitalario Universitario de Santiago de Compostela, Santiago de Compostela, Spain
5
Department of Pathology and Forensic Sciences, University of Santiago de Compostela, Santiago de Compostela, Spain
Menie`re’s disease is an inner ear disease characterised by intermittent episodes of vertigo, with progressive and fluctuating deterioration of auditory function (with hearing loss and tinnitus). The clinical course is highly variable between patients. In some of them, crises are sporadic and both auditory impairment and impact of disease on everyday life are limited. Other patients show a different clinical picture that includes frequent and unexpected vertigo attacks, instability in between attacks and severe hearing damage. In these cases, patient’s work duties and social activities become highly affected. Me´nie`re’s disease can seriously affect quality of life, but we hypothesised that the impairment of quality of life and self-perceived disability are associated with some clinical characteristics as bilateral affectation or Tumarkin attacks (sudden onset of vertigo with fall). Different symptoms of
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Menie`re’s disease are proposed as relevant factors correlated with the quality of life and self-perceived disability of patients. Which of the symptoms of Menie`re’s disease (vertigo, hearing loss, tinnitus, instability,…) causes greater impairment of the ability of patients to perform their daily activities remains unclear. The impact of disease on daily life activities could depend not only on the severity of those symptoms but also on demographic factors (age, sex, work duties, family responsibilities,…). Questionnaires designed to evaluate the handicapping effects imposed by Menie`re’s disease have been shown to be useful for quantifying the impact on lifestyle. The Dizziness Handicap Inventory (DHI) is the questionnaire most widely used to assess the effects due to vertigo and balance disorders. The DHI is a 25-item questionnaire that evaluates self-perceived handicap on everyday life. Items are subgrouped into three domains: physical (7 questions), emotional (9 questions) and functional (9 questions). The DHI has been widespread used and translated and adapted to several languages (included Spanish version) [1]. The variability in disease presentation can make it difficult to establish the clinical stage of the disease. After several attempts [2–5], in 1995, the American Academy of Otolaryngology—Head and Neck Surgery (AAO-HNS) established a specific set of criteria or guidelines for the diagnosis and evaluation of therapy in Menie`re’s disease [6]. The staging system, that includes four stages, is based only on average value of audiometric hearing threshold for pure tone at frequencies 500, 1000, 2000 and 3000 Hz. This is an useful but limited classification, because symptoms related to vestibular disorders are not considered. The objective of this study is to evaluate self-perceived handicap in patients with Menie`re’s disease in different clinical stages. So, clinical features [such as uni or bilateral affectation, presence or absence of Tumarkin attack, recent or old episodes of vertigo, presence or absence of disequilibrium in between crises, hearing loss (not necessarily fluctuating),…] are assessed in order to establish which of them causes a greater impairment of quality of life.
•
The exclusion criteria were: • • •
• •
Diagnosis made at least 6 months before. Pure-tone audiometry made 6 months before the study.
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Previous or actual history of other diseases affecting hearing and/or balance. Presence of severe system diseases that can modify the patient perception of disability. Intellectual disability (because of cultural, psychiatric or neurological conditions) to understand questionnaire.
All patients included in this study consented to take part in accordance with the Declaration of Helsinki. Informed consent was obtained from all individual participants included in the study. To examine the self-perception of disability, all participants completed a DHI (Dizziness Handicap Inventory), Spanish version validated [1]. The DHI questionnaire was developed to evaluate the handicapping effects of dizziness on physical, emotional and functional domains. The DHI contains 25 items, subgrouped into three subscales: DHIP, DHIF and DHIE, that comprise 7 physical, 9 functional and 9 emotional questions, respectively. Each question has three possible responses: ‘‘yes’’ (4 points), ‘‘sometimes’’ (2 points) and ‘‘no’’ (0 points). Thus, a total score (0–100 points) is obtained by summing ordinal scale responses. Higher scores indicate more severe handicap. Questions were not developed to explore a particular symptom. Items were designed to measure the handicapping effects of the disease, avoiding establishing a relation between the questions and the symptoms. The total DHI score obtained was analysed as a continuous quantitative variable and also as a discrete variable, with three groups [7]: • • •
Mild disability: 0–30 points. Moderate disability: 31–60 points. Severe disability: 61–100 points.
In order to establish the relationship with the perception of disability measured by DHI, the following demographic and clinical parameters were evaluated: • • •
Materials and methods This cross-sectional study evaluated patients with definite Menie`re’s disease (diagnosed according to the AAO-HNS criteria) [6]. Participants included in this study were recruited from patients with Menie`re’s disease consulted in an Otoneurology unit integrated in a service of Otorhinolaryngology of a tertiary hospital. Inclusion criteria were:
At least one vertigo attack in the last 2 years.
•
• •
Sex and age. Unilateral or bilateral affectation. Time elapsed since the onset of symptoms (months). This parameter was studied as a continuous quantitative variable and also as a discrete one (three groups: to 24 months, from 25 to 60 months, and more than 60 months). Pure-tone average (PTA): average of thresholds (in dB) for frequencies 500, 1000, 2000 and 3000 Hz. It was analysed as a quantitative variable. Stages of Menie`re’s disease established from PTA value, according to AAO-HNS classification [6]. Audiometric change in the last 6 months (PTA difference): PTA 6 months before—current PTA. It was
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analysed as a discrete variable, comprising three groups: • • • •
•
Pure-tone average in low frequencies (PTAl): average of thresholds (in dB) for frequencies 125, 250 and 500. It was analysed as a quantitative variable. Audiometric change observed in low frequencies in the last 6 months (PTAl difference): PTAl 6 months before—current PTAl. It was analysed as a discrete variable, comprising three groups: • • •
• • •
•
•
None. From one to six (with a maximum of one per month). More than six (more than one per month).
Time elapsed since the last attack. This was classified into three categories, according to the differences observed in posturographic scores depending on the time elapsed intercrises [8]: • • •
•
Impairment: PTAl difference \-10. Stabilization: PTAl difference between -10 and 10. Improvement: PTAl difference [10.
Subjective perception of fluctuating hearing threshold. Presence or absence of tinnitus between attacks. Number of definitive vertiginous episodes in the last 6 months. These were grouped into three categories: • •
•
Impairment: PTA difference \-10. Stabilization: PTA difference between -10 and 10. Improvement: PTA difference [10.
Short term (recent attack): to 7 days. Medium term: from 1 week to 2 months. Long term (old attack): more than 2 months.
Presence or absence of subjective perception of instability intercrises. Presence or absence of Tumarkin attack in the last 6 months.
With regard to the parameters in relation to cochlear symptoms (hearing loss, tinnitus, and aural pressure), in bilateral cases, values obtained in the most affected ear were included in this paper. Data were entered into a database created especially for the purpose. Statistical analysis of results was performed with the Statistical Package for the Social Sciences (SPSS) version 15.0 for Windows software program. In order to evaluate if quantitative variables showed a normal distribution, Kolmogorov–Smirnov was used. Once normality could be assumed, relationships between those and the discrete variables were checked by t Student test or ANOVA. On the other hand, in those cases in which the quantitative variables did not show a normal distribution,
Mann–Whitney and Kruskal–Wallis non-parametric tests were applied to study these relationships. Finally, the Chi square test was used to analyse the relationships between discrete variables. General linear model (GLM) was performed to analyse the effect of variables such as number of crises (0, 1–6, [6), time elapsed from the last attack, and instability intercrises (yes or not), on DHI score. Sex and age were entered as covariates in order to control their potential modifying effect. Time elapsed from the last attack was not observed statistically significant, so this variable was excluded from the final model. Interaction terms with age were included in ‘‘number of crises’’ and ‘‘instability intercrises’’. In both cases, the presence of interaction was rejected. Akaike Information Criterion (AIC) corrected for finite sample sizes was performed as goodness-of-fit test statistic. Wald’s Chi square test was used.
Results 90 patients were included in the study. All of them met the inclusion criteria. They completed a total of 104 questionnaires, with the following distribution: • •
•
80 patients: one questionnaire. 6 patients: two questionnaires (with an elapsed time between them of at least 6 months and with changes of clinical features). 4 patients: three questionnaires (with the same assumptions as described above).
Therefore, 104 records would be the sample size considered. Sex distribution was homogeneous (48.1 % women and 51.9 % men). Mean age of patients studied was 56 years (range 32–84 years, SD 12.42). Unilateral disease was observed in 78 cases (75 %) and the remaining 26 cases (25 %) showed bilateral affectation. The average of time elapsed since the onset of disease was 107 months, ranged from 7 to 408 months (SD 74.60). Figure 1 shows the distribution of time evolution of symptoms. This variable was subdivided in three groups, in 11 cases (10.6 %) time from onset of symptoms was less than 2 years, in 20 (19.2 %) it was between 2 and 5 years, and in the remaining 73 (70.2 %) it was more than 5 years. In relation to pure-tone average (PTA), it was 57.75 dB (SD 20.61). The distribution of audiometric stages was as follows: • • • •
Stage Stage Stage Stage
I: 8 cases (7.7 %). II: 10 cases (9.6 %). III: 62 cases (59.6 %). IV: 24 cases (23.1 %).
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(30.8 %) severe. When the three scales were analysed, the following mean values were obtained: • • •
Fig. 1 Distribution of time evolution of symptoms
Audiometric change in the last 6 months (PTA difference) showed an impairment in 22 cases (21.2 %), stabilization in 70 (67.3 %) and improvement in 12 (11.5 %). When hearing in low frequencies (PTAl) was analysed, mean threshold obtained was 60.51 dB (SD: 21.19). In this range of frequencies, compared with 6 months before, an impairment was detected in 26 cases (25 %), stabilization in 63 (60.6 %) and improvement in 15 (14.4 %). 61 patients (58.7 %) reported subjective perception of fluctuating hearing, and the remaining 43 patients (41.3 %) did not. In relation to presence or absence of tinnitus, 92 cases showed it (88.5 %), while only 12 patients did not experience that symptom (11.5 %). The number of definitive vertiginous episodes in the last 6 months ranged from 0 to 60 with an average of 4 (SD 8.62). When the value of this variable was divided by groups, it was noted that 29 patients (27.9 %) had no vertigo episodes, 58 patients (55.8 %) had from one to six episodes, and 17 patients (16.3 %) had more than six episodes. The mean time elapsed since the last attack was 172 days (range 1 day–15 months; SD 266.61). In 23 cases (22.1 %), time elapsed was less than 1 week, in 26 (25 %) it was from 1 week to 2 months, and in the remaining 55 cases (52.9 %) it was more than 2 months. Patients with continuous perception of instability intercrises were 80 (76.9 %), and those without this perception were 24 (23.1 %). 11 patients (10.6 %) reported having suffered Tumarkin attack in the last 6 months, while 93 patients (89.4 %) had not show them. DHI scores ranged from 2 to 100 with an average of 47.08 (SD 24.45). In 29 cases (27.9 %) the disability perception was mild, in 43 (41.3 %) moderate, and in 32
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Physical scale: 13.75 (SD 7.72) Emotional scale: 14.67 (SD 9.52) Functional scale: 18.63 (SD 9.83)
Then, the influence of the different clinical characteristics on the disability perception was assessed. No relationships were observed between disability perception and sex, age, unilateral or bilateral affectation, time elapsed since the onset of symptoms, PTA, audiometric stage, changes in PTA, hearing in low frequencies, hearing changes in low frequencies, subjective perception of fluctuating hearing threshold, and presence or absence of tinnitus. Tables 1 and 2 show statistical tests used to explore the relationships between these variables, with values of statistical significance. Correlation between disability perception and some vestibular symptoms was found: •
•
•
•
Number of typical attacks in the last 6 months: DHI score was substantially lower in patients without crises (mean 31; SD 19.69) than in those with them. The average of DHI in patients suffering from few episodes (from one to six) was 52 (SD 24.63) and in patients with many episodes (more than six) it was 58 (SD 148.81). The three scales of DHI (physical, emotional and functional) were proved to be affected by the number of recent attacks, as shown in Table 1. Time elapsed since the last attack: Disability perception was much lower in cases with old attacks (time elapsed of at least 2 months) (mean DHI 39; SD 23.59) than in patients with recent attacks. In patients with attacks in the last week, the mean DHI was 56 (SD 22.41), and in those with crises from 1 week to 2 months before the mean DHI was 56 (SD 22.89). Again, it was observed that the time elapsed since the last attack affected the three scales of DHI (physical, emotional and functional). Presence of instability intercrises: This factor was strongly associated with the disability perception. In those patients suffering from instability between attacks, mean DHI was 64 (SD 19.12), while the patients without this symptom showed a mean DHI of 42 (SD 23.59). The three scales of DHI were affected by the presence of instability between crises, specially the functional scale. Report of Tumarkin attack in the last 6 months. In this case, influence was detected only on emotional subdomain of DHI. The other subdomains and the total DHI score remained unchanged.
Table 3 shows the results from final general linear model. We can observe that the number of crises and the presence of instability intercrises modify DHI scores,
Eur Arch Otorhinolaryngol Table 1 Relation between clinical characteristics and DHI value (continuous variable)
Clinical indicators
p value
Test
Sex
0.257
t Student
Uni or bilateral affectation
0.619
t Student
Time elapsed since the onset of symptoms, in months (0–24; 25–60; \60)
0.674
Anova
Audiometric stage
0.351
Kruskal–Wallis
PTA difference (\-10; -10 to 10;[10)
0.883
Anova
PTA difference in low frequencies (\-10; -10 to 10; [10)
0.160
Anova
Hearing fluctuation
0.182
t Student
Tinnitus between attacks
0.160
t Student
DHI total score
8.06 e2005
Anova
DHIp
0.001
Anova
DHIe
0.0002
Kruskal–Wallis
DHIf
0.0003
Anova
Number of episodes in the last 6 months (0; 1–6; [6)
Time elapsed from the last attack (less than 1 week; from 1 week to 2 months; more than 2 months) DHI total score DHIp
0.002 0.004
Anova Kruskal–Wallis
DHIe
0.005
Kruskal–Wallis
DHIf
0.006
Kruskal–Wallis
Presence of continuous instability between attacks DHI total score
5.05 e2005
t Student
DHIp
0.0002
t Student
DHIe
0.003
Mann–Whitney
DHIf
1.72 e2005
t Student
Tumarkin attack DHI total score
0.060
t Student
DHIp
0.183
Mann–Whitney
DHIe
0.044
Mann–Whitney
DHIf
0.157
t Student
Values in bold show those relations with statistical significance DHIp physical subdomain of DHI, DHIe emotional subdomain of DHI, DHIf functional subdomain of DHI
without influence of sex and age of patients. The presence of instability intercrises causes an increase of 18.5 on DHI score (95 % CI 8.78, 28.28), compared to its absence, with sex, age and number of crises unchanged. An increase of 24.12 (95 % CI 11.79, 36.47) on DHI score is observed in patients suffering more than six episodes, and in patients with 1–6 episodes the increase of DHI score is 17.69 (95 % CI 8.23, 27.16), compared to the group of patients with no crises.
Discussion In order to establish a classification system in Menie`re’s disease, different attempts have been developed. These have showed certain limitations due to many factors. Perhaps, the most important ones are two of them. One of them is the variability in disease presentation: vertigo,
hearing loss, tinnitus, fullness, instability and/or Tumarkin attack. For each patient (and depending on the stage of disease), one or some of these symptoms can be the most prominent ones. In addition, each patient may have a different self-perception of disability, so that the symptoms suffered may be evaluated in varying degrees among patients. Therefore, a classification system of Menie`re’s disease must take into account at least some of these symptoms instead only one of them. The hallmark of the Menie`re’s disease is its clinical fluctuation. It is well known that this disease can present with a wide variety of symptom timings. In relation to the development and the appearance of symptoms, it is not necessarily a progressive disease, in such a way that periods with many severe symptoms (exacerbations) may be followed by others with few symptoms (remissions). Then, we propose that the stage classification of Menie`re’s disease must be flexible and allow to change the stage of the
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Eur Arch Otorhinolaryngol Table 2 Relation between clinical characteristics and disability perception (mild, moderate or severe) measured by DHI
Clinical variables
p value
Test
Sex
0.538
Chi square
Age
0.515
Kruskal–Wallis
Uni or bilateral affectation
0.713
Chi square
Time elapsed since the onset of symptoms, in months
0.778
Kruskal–Wallis
Time elapsed since the onset of symptoms, in months (0–24; 25–60; \60)
0.896
Chi square
Pure-tone average (PTA)
0.053
Kruskal–Wallis
Audiometric stage
0.135
Chi square
PTA difference (\-10; -10 a 10; [10)
0.335
Chi square
Pure-tone average in low frequencies (PTAl)
0.068
Kruskal–Wallis
PTA difference in low frequencies (\-10; -10 a 10; [10)
0.130
Chi square
Hearing fluctuation Tinnitus between attacks
0.357 0.401
Chi square Chi square
Number of episodes in the last 6 months (0; 1–6; [6)
0.001
Chi square
Time elapsed from the last attack (less than 1 week; from 1 week to 2 months; more than 2 months)
0.010
Chi square
Presence of continuous instability between attacks
0.001
Chi square
Tumarkin attack
0.266
Chi square
Values in bold show those relations with statistical significance
Table 3 Results from final general linear model
Variable
Coef. (95 % CI)
Wald´s Chi square
p value
Crises ([6)
24.13 (11.79, 36.47)
14.69
\0.0001
Crises (1–6)
17.69 (8.23, 27.16)
13.24
\0.0001
Instability intercrises (yes)
18.53 (8.78, 28.28)
13.88
\0.0001
Age
0.076 (-0.25, 0.40)
0.20
0.652
Sex (woman)
-6.33 (-14.23, 1.56)
2.47
0.116
Intercept
28.00 (7.06, 48.93)
6.87
0.009
Akaike Information Criterion (AIC) 938.3; N = 104 subjects
disease for each patient depending on the actual clinical presentation. All these factors have made difficult to construct a staging system of this disease. Various systems for Menie`re’s disease staging have been proposed: Kumagami et al. [2] designed a classification comprising three stages based on fluctuating hearing between attacks; Shea [3] proposed a complex classification with five stages, taking into account clinical, pathophysiological and electrocochleographical features; and Arenberg and Stahle [4] classified Meniere’s disease depending on audiometric stages, tinnitus, aural pressure, disequilibrium and disability. After all these attempts, in 1995, the AAO-HNS established a specific set of criteria for the diagnosis of Menie`re’s disease [6], that it is the most widely used nowadays. Stage was defined using the PTA in functional frequencies (500, 1000, 2000 and 3000 Hz) and categorises four stages (stage 1: B25 dB; stage 2: 26–40 dB; stage 3: 41–70 dB; stage 4: [70 dB). This classification has been useful and shows some advantages.
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The criterion used is fairly objective, since does not depend on the interpretation of the doctor (though PTA, as subjective test, depends on patient collaboration). In addition, the discrimination between stages is clear, so it is not doubtful to classify patients applying these guidelines. But this system also suffers from several disadvantages. Perhaps the most important one is that vestibular symptoms (vertigo, instability, Tumarkin attack,…) are not taken into account, when in many patients these are the most relevant clinical features. In relation to cochlear symptoms, there are some descriptors that are not considered and may become relevant: • • • •
Hearing in low frequencies (the most affected parameter in many patients). Presence or absence of tinnitus between attacks. Fluctuating hearing (hearing threshold can vary substantially even along a day). Spoken language understanding.
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Therefore, although the AAO-HNS classification is still widely used, new attempts to approach a staging system that takes into account these descriptors are being developed. In 1981 (before the AAO-HNF guidelines), Stahle et al. [5] had proposed a system similar to TNM classification of tumours, including both a cochlear affectation staging (based on hearing loss, tinnitus and aural pressure) and also a vestibular affectation staging (based on frequency and duration of vertigo attacks, instability and disability to work). This staging system was complete but complex, so it did not become very applied. In 1997, Filipo et al. [9] proposed a classification system based on cochlear symptoms and vertigo. This scale distinguished the following stages: • • • •
Ia: vertigo attack, tinnitus and fluctuating hearing loss. Ib: vertigo attack, tinnitus and not fluctuating hearing loss. II: stabilization of vertigo attacks, with progressive hearing impairment. III: bilateral affectation.
Perhaps the most comprehensive classification system of Menie`re’s disease (including all symptoms and exploratory data) might be that proposed by Montes-Jovellar et al. [10]. This system comprises descriptors as age, cochlear function, vestibular function, posturographic data and disability. This is, more than a classification system, a panel with four groups of patients, named as ‘‘mildly active elderly’’, ‘‘mildly active young’’, ‘‘active compensated’’, and ‘‘active uncompensated’’. Nevertheless, a definitive staging system including all above-mentioned factors must still be developed. The AAO had established a staging system of functional impairment [6], with six stages, as shown in Table 4. The aim of our study was to assess the value of self-perception of disability in order to know to what extent the different symptoms might affect patient functioning. DHI questionnaire, Spanish version validated [1], was used to measure the self-
perception of disability. The DHI is a disease specific and well-known questionnaire that has been widely applied in patients with Menie`re’s disease [11–16]. Although DHI was designed to quantify the impact of dizziness on everyday life, items are generic and evaluate the handicapping effects imposed by the ‘‘problem’’ suffered. For this reason, we assume its use appropriate. We avoided to put the focus on a particular symptom. So, patients were questioned about their ‘‘disease’’. The results of our study show that vestibular symptoms are the factors with the greatest impact on the perception of disability. DHI score is lower in those cases with no recent vertigo attack (in the last 6 months), representing less perception of disability. There is no difference between having few or many episodes. Data observed in the present study suggest that the most handicapping condition is the patient fear of having an attack, more than the fact of a current vertigo (except in some cases with many episodes, asymptomatic periods are longer than symptomatic ones). The handicapping effects derived from the fear of having an attack decrease (and the self-perception of disability improves) when the time elapsed from the previous attack increases. Thus, the time elapsed from the last attack is a key factor to be kept in mind to assess the disability perception. When the last attack is recent (less than 1 week), the fear of having other one is higher. As the time elapsed increases, this fear diminishes. This relation had been reported previously [15]: DHI scores in the same group of patients were significantly worse just after vertigo than those obtained later. The factor with a greatest impact on disability perception is the presence of continuous instability between attacks. Imbalance, in contrast to vertigo attacks, fluctuating hearing loss and tinnitus, is not one of the major symptoms of Meniere’s disease. However, this symptom might cause severe handicapping effects, because (unlike vertigo) it is continuous. It usually appears in advanced stages of disease
Table 4 AAO-HNF staging panel of functional affectation for Menie`re’s disease 1.
My dizziness has no effect on my activities at all
2.
When I am dizzy, I have to stop what I am doing for a while, but it soon passes and I can resume activities. I continue to work, drive and engage in any activity I choose without restriction. I have not changed any plans or activities to accommodate my dizziness
3.
When I am dizzy, I have to stop what I am doing for a while, but it does pass and I can resume activities. I continue to work, drive and engage in most activities I choose, but I have had to change some plans and make some allowance for my dizziness
4.
I am able to work, drive, travel, take care of a family or engage in most essential activities, but I must exert a great deal of effort to do so. I must constantly make adjustments in my activities and budget my energies. I am barely making it
5.
I am unable to work, drive or take care of a family. I am unable to do most of the active things that I used to. Even essential activities must be limited. I am disabled
6.
I have been disabled for 1 year or longer and/or I receive compensation (money) because of my dizziness or balance problem
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(associated with uncompensated unilateral damages), in bilateral cases or a side effect of destructive procedures (transtympanic gentamicin, neurectomy, labyrinthectomy,…). It was noted that the three subdomains of DHI were influenced by this symptom, in a special way the functional one. In our point of view, this finding is relevant, since we believe that instability in patients with Menie`re’s disease is not a minor symptom. The possibility of its appearance should be considered in the decision to undergo treatments that damage vestibular function. In these cases, we must offer effective therapies to improve it, such as vestibular rehabilitation. It is also relevant that the auditory parameters analysed had no influence on disability perception. Previous articles had reported the influence of bilateral disease on DHI scores [15]. These findings suggest that the impact of symptoms on disability perception of patients is not reflected into the staging system currently used (based only on audiometric measures). Consequently, we recommend to design a new system for classifying and reporting Menie`re’s disease. This classification should take into account both auditory criteria (PTA, understanding threshold, tinnitus,…) and also, in particular, vestibular symptoms (frequency of attacks and presence of instability). Conflict of interest of interest.
The authors declare that they have no conflict
References 1. Pe´rez N, Garmendia I, Martı´n E, Garcı´a Tapia R (2000) Adaptacio´n cultural de dos cuestionarios de medida de la salud en pacientes con ve´rtigo. Acta Otorrinolaringol Esp 51(7):572–580 2. Kumagami H, Nishida H, Baba M (1982) Electrocochleographic study of Me´nie`re’s disease. Arch Otolaryngol 108(5):284–288 3. Shea JJ (1993) Classification of Menie`re’s disease. Am J Otol 14(3):224–229 4. Arenberg IK, Stahle J (1980) Staging Meniere’s disease (or any inner ear dysfunction) and the use of the vertigogram. Otolaryngol Clin North Am 13(4):643–656
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5. Stahle J, Arenberg IK, Goldman G (1981) Staging Meniere’s disease: description of a vertigo-disability profile. Am J Otol 2(4):357–364 6. Committee on Hearing and Equilibrium (1995) Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Menie`re’s disease. Otolaryngol Head Neck Surg 113(3):181–185 7. Whitney SL, Wrisley DM, Brown KE, Furman JM (2004) Is perception of handicap related to functional performance in persons with vestibular dysfunction? Otol Neurotol 25(2):139–143 8. Soto A, Labella T, Santos S, Rı´o MD, Lirola A, Cabanas E, Elhendi W (2004) The usefulness of computerized dynamic posturography for the study of equilibrium in patients with Meniere’s disease: correlation with clinical and audiologic data. Hear Res 196(1–2):26–32 9. Filipo R, Barbara M (1997) Natural history of Menie`re’s disease: staging the patients or their symptoms? Acta Otolaryngol Suppl 526:10–13 10. Montes-Jovellar L, Guillen-Grima F, Perez-Fernandez N (2011) Cluster analysis of auditory and vestibular test results in definite Menie`re’s disease. Laryngoscope 121(8):1810–1817. doi:10. 1002/lary.21844 11. Barbara M, Lazzarino AI, Biagini M, Costa M, Monini S (2010) Influence of Meniett treatment on hearing. Acta Otolaryngol 130(11):1256–1259. doi:10.3109/00016481003782033 12. Zapata C, Lo´pez-Esca´mez JA (2011) A pilot study of sexual health in patients with Me´nie`re’s disease. Acta Otorrinolaringol Esp 62(2):119–125. doi:10.1016/j.otorri.2010.09.010 13. Garcia AP, Gananc¸a MM, Cusin FS, Tomaz A, Gananc¸a FF, Caovilla HH (2013) Vestibular rehabilitation with virtual reality in Me´nie`re’s disease. Braz J Otorhinolaryngol 79(3):366–374. doi:10.5935/1808-8694.20130064 14. Loader B, Beicht D, Hamzavi JS, Franz P (2013) Tenotomy of the stapedius and tensor tympani muscles reduces subjective dizziness handicap in definite Meniere’s disease. Acta Otolaryngol 133(4):368–372. doi:10.3109/00016489.2012.746470 15. Cunha F, Settanni FA, Gananc¸a FF (2005) What is the effect of dizziness on the quality of life for patients with Meniere’s disease? Rev Laryngol Otol Rhinol (Bord) 126(3):155–158 16. Perez-Fernandez N, Montes-Jovellar L, Cervera-Paz J, Domenech-Vadillo E (2010) Auditory and vestibular assessment of patients with Me´nie`re’s disease who suffer Tumarkin attacks. Audiol Neurootol 15(6):399–406. doi:10.1159/000310899
OTOLOGY
Disability perception in Menie`re’s disease: when, how much and why? Andres Soto-Varela1,2 • Belen Huertas-Pardo3 • Pilar Gayoso-Diz4 Sofia Santos-Perez1,2 • Ines Sanchez-Sellero5
•
Received: 12 February 2015 / Accepted: 24 April 2015 Springer-Verlag Berlin Heidelberg 2015
Abstract The purpose of the study was to evaluate selfperceived handicap in patients with definite Menie`re’s disease (MD). A cross-sectional study was conducted. To examine the self-perception of disability, participants completed a DHI (Dizziness Handicap Inventory). Parameters compared with DHI scores: sex, age, unilateral/bilateral affectation, time elapsed since the onset of symptoms, pure-tone average (PTA), stages of MD, audiometric change (last 6 months), PTA in low frequencies (PTAl) and audiometric change in PTAl, subjective perception of fluctuating hearing threshold, tinnitus between attacks, number of vertiginous episodes (last 6 months), time elapsed since last attack, subjective perception of instability intercrises and Tumarkin attacks. 90 patients were included; they completed a total of 104 questionnaires. DHI scores ranged from 2 to 100 (average: 47.08, SD
24.45). In 29 cases (27.9 %) the disability perception was mild, in 43 (41.3 %) moderate, and in 32 (30.8 %) severe. Correlation between disability perception and some vestibular symptoms was found: number of typical attacks (last 6 months), time elapsed since last attack, instability intercrises and Tumarkin attacks. No relationship was found with the rest of variables. Disability perception in patients with MD depends primarily on vestibular symptoms (particularly, instability and frequency of attacks). So, we suggest to design a new staging system of MD taking into account both auditory criteria and also vestibular symptoms. Keywords Disability Menie`re’s disease DHI Instability Staging
Introduction & Andres Soto-Varela [email protected] 1
Division of Neurotology, Department of Otorhinolaryngology, Complexo Hospitalario Universitario de Santiago de Compostela, Travesı´a da Choupana, s/n, 15706 Santiago de Compostela, Spain
2
Department of Dermatology and Otorhinolaryngology, University of Santiago de Compostela, Santiago de Compostela, Spain
3
Department of Otorhinolaryngology, Complexo Hospitalario Universitario de Santiago de Compostela, Santiago de Compostela, Spain
4
Clinical Epidemiology Unit, Instituto de Investigacio´n Sanitaria de Santiago (IDIS), Complexo Hospitalario Universitario de Santiago de Compostela, Santiago de Compostela, Spain
5
Department of Pathology and Forensic Sciences, University of Santiago de Compostela, Santiago de Compostela, Spain
Menie`re’s disease is an inner ear disease characterised by intermittent episodes of vertigo, with progressive and fluctuating deterioration of auditory function (with hearing loss and tinnitus). The clinical course is highly variable between patients. In some of them, crises are sporadic and both auditory impairment and impact of disease on everyday life are limited. Other patients show a different clinical picture that includes frequent and unexpected vertigo attacks, instability in between attacks and severe hearing damage. In these cases, patient’s work duties and social activities become highly affected. Me´nie`re’s disease can seriously affect quality of life, but we hypothesised that the impairment of quality of life and self-perceived disability are associated with some clinical characteristics as bilateral affectation or Tumarkin attacks (sudden onset of vertigo with fall). Different symptoms of
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Menie`re’s disease are proposed as relevant factors correlated with the quality of life and self-perceived disability of patients. Which of the symptoms of Menie`re’s disease (vertigo, hearing loss, tinnitus, instability,…) causes greater impairment of the ability of patients to perform their daily activities remains unclear. The impact of disease on daily life activities could depend not only on the severity of those symptoms but also on demographic factors (age, sex, work duties, family responsibilities,…). Questionnaires designed to evaluate the handicapping effects imposed by Menie`re’s disease have been shown to be useful for quantifying the impact on lifestyle. The Dizziness Handicap Inventory (DHI) is the questionnaire most widely used to assess the effects due to vertigo and balance disorders. The DHI is a 25-item questionnaire that evaluates self-perceived handicap on everyday life. Items are subgrouped into three domains: physical (7 questions), emotional (9 questions) and functional (9 questions). The DHI has been widespread used and translated and adapted to several languages (included Spanish version) [1]. The variability in disease presentation can make it difficult to establish the clinical stage of the disease. After several attempts [2–5], in 1995, the American Academy of Otolaryngology—Head and Neck Surgery (AAO-HNS) established a specific set of criteria or guidelines for the diagnosis and evaluation of therapy in Menie`re’s disease [6]. The staging system, that includes four stages, is based only on average value of audiometric hearing threshold for pure tone at frequencies 500, 1000, 2000 and 3000 Hz. This is an useful but limited classification, because symptoms related to vestibular disorders are not considered. The objective of this study is to evaluate self-perceived handicap in patients with Menie`re’s disease in different clinical stages. So, clinical features [such as uni or bilateral affectation, presence or absence of Tumarkin attack, recent or old episodes of vertigo, presence or absence of disequilibrium in between crises, hearing loss (not necessarily fluctuating),…] are assessed in order to establish which of them causes a greater impairment of quality of life.
•
The exclusion criteria were: • • •
• •
Diagnosis made at least 6 months before. Pure-tone audiometry made 6 months before the study.
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Previous or actual history of other diseases affecting hearing and/or balance. Presence of severe system diseases that can modify the patient perception of disability. Intellectual disability (because of cultural, psychiatric or neurological conditions) to understand questionnaire.
All patients included in this study consented to take part in accordance with the Declaration of Helsinki. Informed consent was obtained from all individual participants included in the study. To examine the self-perception of disability, all participants completed a DHI (Dizziness Handicap Inventory), Spanish version validated [1]. The DHI questionnaire was developed to evaluate the handicapping effects of dizziness on physical, emotional and functional domains. The DHI contains 25 items, subgrouped into three subscales: DHIP, DHIF and DHIE, that comprise 7 physical, 9 functional and 9 emotional questions, respectively. Each question has three possible responses: ‘‘yes’’ (4 points), ‘‘sometimes’’ (2 points) and ‘‘no’’ (0 points). Thus, a total score (0–100 points) is obtained by summing ordinal scale responses. Higher scores indicate more severe handicap. Questions were not developed to explore a particular symptom. Items were designed to measure the handicapping effects of the disease, avoiding establishing a relation between the questions and the symptoms. The total DHI score obtained was analysed as a continuous quantitative variable and also as a discrete variable, with three groups [7]: • • •
Mild disability: 0–30 points. Moderate disability: 31–60 points. Severe disability: 61–100 points.
In order to establish the relationship with the perception of disability measured by DHI, the following demographic and clinical parameters were evaluated: • • •
Materials and methods This cross-sectional study evaluated patients with definite Menie`re’s disease (diagnosed according to the AAO-HNS criteria) [6]. Participants included in this study were recruited from patients with Menie`re’s disease consulted in an Otoneurology unit integrated in a service of Otorhinolaryngology of a tertiary hospital. Inclusion criteria were:
At least one vertigo attack in the last 2 years.
•
• •
Sex and age. Unilateral or bilateral affectation. Time elapsed since the onset of symptoms (months). This parameter was studied as a continuous quantitative variable and also as a discrete one (three groups: to 24 months, from 25 to 60 months, and more than 60 months). Pure-tone average (PTA): average of thresholds (in dB) for frequencies 500, 1000, 2000 and 3000 Hz. It was analysed as a quantitative variable. Stages of Menie`re’s disease established from PTA value, according to AAO-HNS classification [6]. Audiometric change in the last 6 months (PTA difference): PTA 6 months before—current PTA. It was
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analysed as a discrete variable, comprising three groups: • • • •
•
Pure-tone average in low frequencies (PTAl): average of thresholds (in dB) for frequencies 125, 250 and 500. It was analysed as a quantitative variable. Audiometric change observed in low frequencies in the last 6 months (PTAl difference): PTAl 6 months before—current PTAl. It was analysed as a discrete variable, comprising three groups: • • •
• • •
•
•
None. From one to six (with a maximum of one per month). More than six (more than one per month).
Time elapsed since the last attack. This was classified into three categories, according to the differences observed in posturographic scores depending on the time elapsed intercrises [8]: • • •
•
Impairment: PTAl difference \-10. Stabilization: PTAl difference between -10 and 10. Improvement: PTAl difference [10.
Subjective perception of fluctuating hearing threshold. Presence or absence of tinnitus between attacks. Number of definitive vertiginous episodes in the last 6 months. These were grouped into three categories: • •
•
Impairment: PTA difference \-10. Stabilization: PTA difference between -10 and 10. Improvement: PTA difference [10.
Short term (recent attack): to 7 days. Medium term: from 1 week to 2 months. Long term (old attack): more than 2 months.
Presence or absence of subjective perception of instability intercrises. Presence or absence of Tumarkin attack in the last 6 months.
With regard to the parameters in relation to cochlear symptoms (hearing loss, tinnitus, and aural pressure), in bilateral cases, values obtained in the most affected ear were included in this paper. Data were entered into a database created especially for the purpose. Statistical analysis of results was performed with the Statistical Package for the Social Sciences (SPSS) version 15.0 for Windows software program. In order to evaluate if quantitative variables showed a normal distribution, Kolmogorov–Smirnov was used. Once normality could be assumed, relationships between those and the discrete variables were checked by t Student test or ANOVA. On the other hand, in those cases in which the quantitative variables did not show a normal distribution,
Mann–Whitney and Kruskal–Wallis non-parametric tests were applied to study these relationships. Finally, the Chi square test was used to analyse the relationships between discrete variables. General linear model (GLM) was performed to analyse the effect of variables such as number of crises (0, 1–6, [6), time elapsed from the last attack, and instability intercrises (yes or not), on DHI score. Sex and age were entered as covariates in order to control their potential modifying effect. Time elapsed from the last attack was not observed statistically significant, so this variable was excluded from the final model. Interaction terms with age were included in ‘‘number of crises’’ and ‘‘instability intercrises’’. In both cases, the presence of interaction was rejected. Akaike Information Criterion (AIC) corrected for finite sample sizes was performed as goodness-of-fit test statistic. Wald’s Chi square test was used.
Results 90 patients were included in the study. All of them met the inclusion criteria. They completed a total of 104 questionnaires, with the following distribution: • •
•
80 patients: one questionnaire. 6 patients: two questionnaires (with an elapsed time between them of at least 6 months and with changes of clinical features). 4 patients: three questionnaires (with the same assumptions as described above).
Therefore, 104 records would be the sample size considered. Sex distribution was homogeneous (48.1 % women and 51.9 % men). Mean age of patients studied was 56 years (range 32–84 years, SD 12.42). Unilateral disease was observed in 78 cases (75 %) and the remaining 26 cases (25 %) showed bilateral affectation. The average of time elapsed since the onset of disease was 107 months, ranged from 7 to 408 months (SD 74.60). Figure 1 shows the distribution of time evolution of symptoms. This variable was subdivided in three groups, in 11 cases (10.6 %) time from onset of symptoms was less than 2 years, in 20 (19.2 %) it was between 2 and 5 years, and in the remaining 73 (70.2 %) it was more than 5 years. In relation to pure-tone average (PTA), it was 57.75 dB (SD 20.61). The distribution of audiometric stages was as follows: • • • •
Stage Stage Stage Stage
I: 8 cases (7.7 %). II: 10 cases (9.6 %). III: 62 cases (59.6 %). IV: 24 cases (23.1 %).
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(30.8 %) severe. When the three scales were analysed, the following mean values were obtained: • • •
Fig. 1 Distribution of time evolution of symptoms
Audiometric change in the last 6 months (PTA difference) showed an impairment in 22 cases (21.2 %), stabilization in 70 (67.3 %) and improvement in 12 (11.5 %). When hearing in low frequencies (PTAl) was analysed, mean threshold obtained was 60.51 dB (SD: 21.19). In this range of frequencies, compared with 6 months before, an impairment was detected in 26 cases (25 %), stabilization in 63 (60.6 %) and improvement in 15 (14.4 %). 61 patients (58.7 %) reported subjective perception of fluctuating hearing, and the remaining 43 patients (41.3 %) did not. In relation to presence or absence of tinnitus, 92 cases showed it (88.5 %), while only 12 patients did not experience that symptom (11.5 %). The number of definitive vertiginous episodes in the last 6 months ranged from 0 to 60 with an average of 4 (SD 8.62). When the value of this variable was divided by groups, it was noted that 29 patients (27.9 %) had no vertigo episodes, 58 patients (55.8 %) had from one to six episodes, and 17 patients (16.3 %) had more than six episodes. The mean time elapsed since the last attack was 172 days (range 1 day–15 months; SD 266.61). In 23 cases (22.1 %), time elapsed was less than 1 week, in 26 (25 %) it was from 1 week to 2 months, and in the remaining 55 cases (52.9 %) it was more than 2 months. Patients with continuous perception of instability intercrises were 80 (76.9 %), and those without this perception were 24 (23.1 %). 11 patients (10.6 %) reported having suffered Tumarkin attack in the last 6 months, while 93 patients (89.4 %) had not show them. DHI scores ranged from 2 to 100 with an average of 47.08 (SD 24.45). In 29 cases (27.9 %) the disability perception was mild, in 43 (41.3 %) moderate, and in 32
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Physical scale: 13.75 (SD 7.72) Emotional scale: 14.67 (SD 9.52) Functional scale: 18.63 (SD 9.83)
Then, the influence of the different clinical characteristics on the disability perception was assessed. No relationships were observed between disability perception and sex, age, unilateral or bilateral affectation, time elapsed since the onset of symptoms, PTA, audiometric stage, changes in PTA, hearing in low frequencies, hearing changes in low frequencies, subjective perception of fluctuating hearing threshold, and presence or absence of tinnitus. Tables 1 and 2 show statistical tests used to explore the relationships between these variables, with values of statistical significance. Correlation between disability perception and some vestibular symptoms was found: •
•
•
•
Number of typical attacks in the last 6 months: DHI score was substantially lower in patients without crises (mean 31; SD 19.69) than in those with them. The average of DHI in patients suffering from few episodes (from one to six) was 52 (SD 24.63) and in patients with many episodes (more than six) it was 58 (SD 148.81). The three scales of DHI (physical, emotional and functional) were proved to be affected by the number of recent attacks, as shown in Table 1. Time elapsed since the last attack: Disability perception was much lower in cases with old attacks (time elapsed of at least 2 months) (mean DHI 39; SD 23.59) than in patients with recent attacks. In patients with attacks in the last week, the mean DHI was 56 (SD 22.41), and in those with crises from 1 week to 2 months before the mean DHI was 56 (SD 22.89). Again, it was observed that the time elapsed since the last attack affected the three scales of DHI (physical, emotional and functional). Presence of instability intercrises: This factor was strongly associated with the disability perception. In those patients suffering from instability between attacks, mean DHI was 64 (SD 19.12), while the patients without this symptom showed a mean DHI of 42 (SD 23.59). The three scales of DHI were affected by the presence of instability between crises, specially the functional scale. Report of Tumarkin attack in the last 6 months. In this case, influence was detected only on emotional subdomain of DHI. The other subdomains and the total DHI score remained unchanged.
Table 3 shows the results from final general linear model. We can observe that the number of crises and the presence of instability intercrises modify DHI scores,
Eur Arch Otorhinolaryngol Table 1 Relation between clinical characteristics and DHI value (continuous variable)
Clinical indicators
p value
Test
Sex
0.257
t Student
Uni or bilateral affectation
0.619
t Student
Time elapsed since the onset of symptoms, in months (0–24; 25–60; \60)
0.674
Anova
Audiometric stage
0.351
Kruskal–Wallis
PTA difference (\-10; -10 to 10;[10)
0.883
Anova
PTA difference in low frequencies (\-10; -10 to 10; [10)
0.160
Anova
Hearing fluctuation
0.182
t Student
Tinnitus between attacks
0.160
t Student
DHI total score
8.06 e2005
Anova
DHIp
0.001
Anova
DHIe
0.0002
Kruskal–Wallis
DHIf
0.0003
Anova
Number of episodes in the last 6 months (0; 1–6; [6)
Time elapsed from the last attack (less than 1 week; from 1 week to 2 months; more than 2 months) DHI total score DHIp
0.002 0.004
Anova Kruskal–Wallis
DHIe
0.005
Kruskal–Wallis
DHIf
0.006
Kruskal–Wallis
Presence of continuous instability between attacks DHI total score
5.05 e2005
t Student
DHIp
0.0002
t Student
DHIe
0.003
Mann–Whitney
DHIf
1.72 e2005
t Student
Tumarkin attack DHI total score
0.060
t Student
DHIp
0.183
Mann–Whitney
DHIe
0.044
Mann–Whitney
DHIf
0.157
t Student
Values in bold show those relations with statistical significance DHIp physical subdomain of DHI, DHIe emotional subdomain of DHI, DHIf functional subdomain of DHI
without influence of sex and age of patients. The presence of instability intercrises causes an increase of 18.5 on DHI score (95 % CI 8.78, 28.28), compared to its absence, with sex, age and number of crises unchanged. An increase of 24.12 (95 % CI 11.79, 36.47) on DHI score is observed in patients suffering more than six episodes, and in patients with 1–6 episodes the increase of DHI score is 17.69 (95 % CI 8.23, 27.16), compared to the group of patients with no crises.
Discussion In order to establish a classification system in Menie`re’s disease, different attempts have been developed. These have showed certain limitations due to many factors. Perhaps, the most important ones are two of them. One of them is the variability in disease presentation: vertigo,
hearing loss, tinnitus, fullness, instability and/or Tumarkin attack. For each patient (and depending on the stage of disease), one or some of these symptoms can be the most prominent ones. In addition, each patient may have a different self-perception of disability, so that the symptoms suffered may be evaluated in varying degrees among patients. Therefore, a classification system of Menie`re’s disease must take into account at least some of these symptoms instead only one of them. The hallmark of the Menie`re’s disease is its clinical fluctuation. It is well known that this disease can present with a wide variety of symptom timings. In relation to the development and the appearance of symptoms, it is not necessarily a progressive disease, in such a way that periods with many severe symptoms (exacerbations) may be followed by others with few symptoms (remissions). Then, we propose that the stage classification of Menie`re’s disease must be flexible and allow to change the stage of the
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Eur Arch Otorhinolaryngol Table 2 Relation between clinical characteristics and disability perception (mild, moderate or severe) measured by DHI
Clinical variables
p value
Test
Sex
0.538
Chi square
Age
0.515
Kruskal–Wallis
Uni or bilateral affectation
0.713
Chi square
Time elapsed since the onset of symptoms, in months
0.778
Kruskal–Wallis
Time elapsed since the onset of symptoms, in months (0–24; 25–60; \60)
0.896
Chi square
Pure-tone average (PTA)
0.053
Kruskal–Wallis
Audiometric stage
0.135
Chi square
PTA difference (\-10; -10 a 10; [10)
0.335
Chi square
Pure-tone average in low frequencies (PTAl)
0.068
Kruskal–Wallis
PTA difference in low frequencies (\-10; -10 a 10; [10)
0.130
Chi square
Hearing fluctuation Tinnitus between attacks
0.357 0.401
Chi square Chi square
Number of episodes in the last 6 months (0; 1–6; [6)
0.001
Chi square
Time elapsed from the last attack (less than 1 week; from 1 week to 2 months; more than 2 months)
0.010
Chi square
Presence of continuous instability between attacks
0.001
Chi square
Tumarkin attack
0.266
Chi square
Values in bold show those relations with statistical significance
Table 3 Results from final general linear model
Variable
Coef. (95 % CI)
Wald´s Chi square
p value
Crises ([6)
24.13 (11.79, 36.47)
14.69
\0.0001
Crises (1–6)
17.69 (8.23, 27.16)
13.24
\0.0001
Instability intercrises (yes)
18.53 (8.78, 28.28)
13.88
\0.0001
Age
0.076 (-0.25, 0.40)
0.20
0.652
Sex (woman)
-6.33 (-14.23, 1.56)
2.47
0.116
Intercept
28.00 (7.06, 48.93)
6.87
0.009
Akaike Information Criterion (AIC) 938.3; N = 104 subjects
disease for each patient depending on the actual clinical presentation. All these factors have made difficult to construct a staging system of this disease. Various systems for Menie`re’s disease staging have been proposed: Kumagami et al. [2] designed a classification comprising three stages based on fluctuating hearing between attacks; Shea [3] proposed a complex classification with five stages, taking into account clinical, pathophysiological and electrocochleographical features; and Arenberg and Stahle [4] classified Meniere’s disease depending on audiometric stages, tinnitus, aural pressure, disequilibrium and disability. After all these attempts, in 1995, the AAO-HNS established a specific set of criteria for the diagnosis of Menie`re’s disease [6], that it is the most widely used nowadays. Stage was defined using the PTA in functional frequencies (500, 1000, 2000 and 3000 Hz) and categorises four stages (stage 1: B25 dB; stage 2: 26–40 dB; stage 3: 41–70 dB; stage 4: [70 dB). This classification has been useful and shows some advantages.
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The criterion used is fairly objective, since does not depend on the interpretation of the doctor (though PTA, as subjective test, depends on patient collaboration). In addition, the discrimination between stages is clear, so it is not doubtful to classify patients applying these guidelines. But this system also suffers from several disadvantages. Perhaps the most important one is that vestibular symptoms (vertigo, instability, Tumarkin attack,…) are not taken into account, when in many patients these are the most relevant clinical features. In relation to cochlear symptoms, there are some descriptors that are not considered and may become relevant: • • • •
Hearing in low frequencies (the most affected parameter in many patients). Presence or absence of tinnitus between attacks. Fluctuating hearing (hearing threshold can vary substantially even along a day). Spoken language understanding.
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Therefore, although the AAO-HNS classification is still widely used, new attempts to approach a staging system that takes into account these descriptors are being developed. In 1981 (before the AAO-HNF guidelines), Stahle et al. [5] had proposed a system similar to TNM classification of tumours, including both a cochlear affectation staging (based on hearing loss, tinnitus and aural pressure) and also a vestibular affectation staging (based on frequency and duration of vertigo attacks, instability and disability to work). This staging system was complete but complex, so it did not become very applied. In 1997, Filipo et al. [9] proposed a classification system based on cochlear symptoms and vertigo. This scale distinguished the following stages: • • • •
Ia: vertigo attack, tinnitus and fluctuating hearing loss. Ib: vertigo attack, tinnitus and not fluctuating hearing loss. II: stabilization of vertigo attacks, with progressive hearing impairment. III: bilateral affectation.
Perhaps the most comprehensive classification system of Menie`re’s disease (including all symptoms and exploratory data) might be that proposed by Montes-Jovellar et al. [10]. This system comprises descriptors as age, cochlear function, vestibular function, posturographic data and disability. This is, more than a classification system, a panel with four groups of patients, named as ‘‘mildly active elderly’’, ‘‘mildly active young’’, ‘‘active compensated’’, and ‘‘active uncompensated’’. Nevertheless, a definitive staging system including all above-mentioned factors must still be developed. The AAO had established a staging system of functional impairment [6], with six stages, as shown in Table 4. The aim of our study was to assess the value of self-perception of disability in order to know to what extent the different symptoms might affect patient functioning. DHI questionnaire, Spanish version validated [1], was used to measure the self-
perception of disability. The DHI is a disease specific and well-known questionnaire that has been widely applied in patients with Menie`re’s disease [11–16]. Although DHI was designed to quantify the impact of dizziness on everyday life, items are generic and evaluate the handicapping effects imposed by the ‘‘problem’’ suffered. For this reason, we assume its use appropriate. We avoided to put the focus on a particular symptom. So, patients were questioned about their ‘‘disease’’. The results of our study show that vestibular symptoms are the factors with the greatest impact on the perception of disability. DHI score is lower in those cases with no recent vertigo attack (in the last 6 months), representing less perception of disability. There is no difference between having few or many episodes. Data observed in the present study suggest that the most handicapping condition is the patient fear of having an attack, more than the fact of a current vertigo (except in some cases with many episodes, asymptomatic periods are longer than symptomatic ones). The handicapping effects derived from the fear of having an attack decrease (and the self-perception of disability improves) when the time elapsed from the previous attack increases. Thus, the time elapsed from the last attack is a key factor to be kept in mind to assess the disability perception. When the last attack is recent (less than 1 week), the fear of having other one is higher. As the time elapsed increases, this fear diminishes. This relation had been reported previously [15]: DHI scores in the same group of patients were significantly worse just after vertigo than those obtained later. The factor with a greatest impact on disability perception is the presence of continuous instability between attacks. Imbalance, in contrast to vertigo attacks, fluctuating hearing loss and tinnitus, is not one of the major symptoms of Meniere’s disease. However, this symptom might cause severe handicapping effects, because (unlike vertigo) it is continuous. It usually appears in advanced stages of disease
Table 4 AAO-HNF staging panel of functional affectation for Menie`re’s disease 1.
My dizziness has no effect on my activities at all
2.
When I am dizzy, I have to stop what I am doing for a while, but it soon passes and I can resume activities. I continue to work, drive and engage in any activity I choose without restriction. I have not changed any plans or activities to accommodate my dizziness
3.
When I am dizzy, I have to stop what I am doing for a while, but it does pass and I can resume activities. I continue to work, drive and engage in most activities I choose, but I have had to change some plans and make some allowance for my dizziness
4.
I am able to work, drive, travel, take care of a family or engage in most essential activities, but I must exert a great deal of effort to do so. I must constantly make adjustments in my activities and budget my energies. I am barely making it
5.
I am unable to work, drive or take care of a family. I am unable to do most of the active things that I used to. Even essential activities must be limited. I am disabled
6.
I have been disabled for 1 year or longer and/or I receive compensation (money) because of my dizziness or balance problem
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(associated with uncompensated unilateral damages), in bilateral cases or a side effect of destructive procedures (transtympanic gentamicin, neurectomy, labyrinthectomy,…). It was noted that the three subdomains of DHI were influenced by this symptom, in a special way the functional one. In our point of view, this finding is relevant, since we believe that instability in patients with Menie`re’s disease is not a minor symptom. The possibility of its appearance should be considered in the decision to undergo treatments that damage vestibular function. In these cases, we must offer effective therapies to improve it, such as vestibular rehabilitation. It is also relevant that the auditory parameters analysed had no influence on disability perception. Previous articles had reported the influence of bilateral disease on DHI scores [15]. These findings suggest that the impact of symptoms on disability perception of patients is not reflected into the staging system currently used (based only on audiometric measures). Consequently, we recommend to design a new system for classifying and reporting Menie`re’s disease. This classification should take into account both auditory criteria (PTA, understanding threshold, tinnitus,…) and also, in particular, vestibular symptoms (frequency of attacks and presence of instability). Conflict of interest of interest.
The authors declare that they have no conflict
References 1. Pe´rez N, Garmendia I, Martı´n E, Garcı´a Tapia R (2000) Adaptacio´n cultural de dos cuestionarios de medida de la salud en pacientes con ve´rtigo. Acta Otorrinolaringol Esp 51(7):572–580 2. Kumagami H, Nishida H, Baba M (1982) Electrocochleographic study of Me´nie`re’s disease. Arch Otolaryngol 108(5):284–288 3. Shea JJ (1993) Classification of Menie`re’s disease. Am J Otol 14(3):224–229 4. Arenberg IK, Stahle J (1980) Staging Meniere’s disease (or any inner ear dysfunction) and the use of the vertigogram. Otolaryngol Clin North Am 13(4):643–656
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5. Stahle J, Arenberg IK, Goldman G (1981) Staging Meniere’s disease: description of a vertigo-disability profile. Am J Otol 2(4):357–364 6. Committee on Hearing and Equilibrium (1995) Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Menie`re’s disease. Otolaryngol Head Neck Surg 113(3):181–185 7. Whitney SL, Wrisley DM, Brown KE, Furman JM (2004) Is perception of handicap related to functional performance in persons with vestibular dysfunction? Otol Neurotol 25(2):139–143 8. Soto A, Labella T, Santos S, Rı´o MD, Lirola A, Cabanas E, Elhendi W (2004) The usefulness of computerized dynamic posturography for the study of equilibrium in patients with Meniere’s disease: correlation with clinical and audiologic data. Hear Res 196(1–2):26–32 9. Filipo R, Barbara M (1997) Natural history of Menie`re’s disease: staging the patients or their symptoms? Acta Otolaryngol Suppl 526:10–13 10. Montes-Jovellar L, Guillen-Grima F, Perez-Fernandez N (2011) Cluster analysis of auditory and vestibular test results in definite Menie`re’s disease. Laryngoscope 121(8):1810–1817. doi:10. 1002/lary.21844 11. Barbara M, Lazzarino AI, Biagini M, Costa M, Monini S (2010) Influence of Meniett treatment on hearing. Acta Otolaryngol 130(11):1256–1259. doi:10.3109/00016481003782033 12. Zapata C, Lo´pez-Esca´mez JA (2011) A pilot study of sexual health in patients with Me´nie`re’s disease. Acta Otorrinolaringol Esp 62(2):119–125. doi:10.1016/j.otorri.2010.09.010 13. Garcia AP, Gananc¸a MM, Cusin FS, Tomaz A, Gananc¸a FF, Caovilla HH (2013) Vestibular rehabilitation with virtual reality in Me´nie`re’s disease. Braz J Otorhinolaryngol 79(3):366–374. doi:10.5935/1808-8694.20130064 14. Loader B, Beicht D, Hamzavi JS, Franz P (2013) Tenotomy of the stapedius and tensor tympani muscles reduces subjective dizziness handicap in definite Meniere’s disease. Acta Otolaryngol 133(4):368–372. doi:10.3109/00016489.2012.746470 15. Cunha F, Settanni FA, Gananc¸a FF (2005) What is the effect of dizziness on the quality of life for patients with Meniere’s disease? Rev Laryngol Otol Rhinol (Bord) 126(3):155–158 16. Perez-Fernandez N, Montes-Jovellar L, Cervera-Paz J, Domenech-Vadillo E (2010) Auditory and vestibular assessment of patients with Me´nie`re’s disease who suffer Tumarkin attacks. Audiol Neurootol 15(6):399–406. doi:10.1159/000310899
