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Prevalence of Menière’s Disease in General Population of Southern Finland
Otolaryngology–Head and Neck Surgery (2005) 133, 762-768
ORIGINAL RESEARCH
Prevalence of Menière’s Disease in General Population of Southern Finland Mari Havia, MD, PhD, Erna Kentala, MD, PhD, and Ilmari Pyykkö, MD, PhD, Helsinki and Tampere, Finland
M
OBJECTIVE: To study the prevalence of Menière’s disease (MD) in the general population of Southern Finland. STUDY DESIGN AND SETTING: Prospective study based on population register data. A questionnaire was sent to 5000 randomly selected persons aged 12 years or more living in the Helsinki University Hospital area. We enquired about whether recipients had experienced vertigo associated with a moving sensation, hearing loss, or tinnitus. For exclusion purposes, we also asked about general illnesses, ear infections, former head and ear traumas, noise exposure, medication, and use of tobacco and alcohol. To assess the validity of the population-based study, we randomly selected a sample of 100 people among those reporting vertigo. They were clinically examined at our vestibular unit. The clinical examination was supplemented by audiologic and otoneurologic tests. RESULTS: The response rate was 63%. In the final study sample of 3116 people, 216 reported the triad of vertigo, hearing loss, and tinnitus. By using the most recent criteria of the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology–Head and Neck Surgery, we were able to identify 16 definite MD patients from the total sample, yielding a prevalence of 513 of 100,000 persons. Among the 16 MD subjects, 9 patients had already been diagnosed with MD and 1 patient was diagnosed during the clinical examination. A peak prevalence of 1709 of 100,000 was seen in the age group 61 to 70 years. CONCLUSIONS: Our population-based estimate of MD prevalence is much higher than in previous reports. EBM RATING: B-3. © 2005 American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. All rights reserved.
eniére’s disease (MD) has been a target of active otoneurologic research for decades. Although the diagnostic criteria have been specified, the prevalence of the disease is still unknown.1 Estimations of prevalence have been based on hospital register data extrapolated to the general population. In Great Britain in 1954, Cawthorne and Hewlett2 reported 43 patients in 1 year in a population of 27,365 people, yielding an incidence of 157 per 100,000. Their study has been criticized by Wladislavosky-Waserman et al,3 who suspected that the result might be a combination of incidence and prevalence because it was unclear whether the study included patients with onset of the symptoms in preceding years. Wladislavosky-Waserman et al themselves reported a prevalence of 218 per 100 000. Stahle et al4 described an incidence of 46 per 100,000 in Sweden in 1978. According to Wladislavosky-Waserman et al,3 however, the statistics in the Swedish study were a combination of incidence and prevalence and did not include data from private practices, which comprised approximately 23% of Swedish medical care at that time. In Japan, Watanabe et al5 reported a prevalence of 16 or 17 per 100,000 but their study was not population-based either. Celestino and Ralli6 found the incidence of MD to be 8 per 100,000 in Italy and the figure to be 3.4 times greater among hospital personnel. The greater availability of medical facilities apparently increased the number of diagnoses. Kotimäki et al7 assessed the prevalence of MD in Finland in 1999. Their study was based on the re-evaluation of hospital records of 306 MD patients treated in 7 different hospitals in Finland. They reported a prevalence of 43 per 100,000 but came to the conclusion, that the prevalence is
From the Department of Otorhinolaryngology (Drs Havia and Kentala), Helsinki University Hospital, and the Department of Otorhinolaryngology (Dr Pyykkö), Tampere University Hospital, Tampere, Finland. Presented as a poster at the Annual Meeting of American Academy of
Otolaryngology–Head and Neck Surgery, New York, NY, September 1922, 2004. Reprint requests: Mari Havia, PO Box 220, 00029 HUS, Finland. E-mail address: [email protected].
0194-5998/$30.00 © 2005 American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. All rights reserved. doi:10.1016/j.otohns.2005.06.015
Havia et al
Prevalence of Menière’s Disease in General . . .
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Table 1 Criteria for diagnosis of MD (AAO-HNS 1995) Certain MD
Definite MD, plus histopathologic confirmation
Definite MD
Two or more definitive spontaneous episodes of vertigo lasting 20 minutes or longer Audiometrically documented hearing loss on at least one occasion Tinnitis or aural fullness in the treated ear Other causes excluded
Probable MD
One definitive episode of vertigo Audiometrically documented hearing loss on at least one occasion Tinnitis or aural fullness in the treated ear Other causes excluded.
Possible MD
Episodic vertigo of the Menière type without documented hearing loss, or Sensorineural hearing loss, fluctuating or fixed, with dysequilibrium but without episodes Other causes excluded.
probably underestimated when data are retrieved retrospectively from patient documents. MD among the elderly has been reported by Ballester et al8 to be quite common. In their opinion, drop attacks are also more frequent in patients with MD than previously reported, and these can mimic a stroke of the brainstem. Meyerhoff et al9 found approximately 3% of all the MD patients to belong to the pediatric age group. The American Academy of Otolaryngology–Head and Neck Surgery (AAO–HNS) has updated its criteria twice since 1972. The current AAO–HNS criteria for definite MD require 2 or more episodes of vertigo lasting 20 minutes or more in addition to hearing loss and tinnitus (Table 1). Tinnitus can also be replaced by aural fullness. Vertigo is often accompanied by nausea. MD is not associated with a loss of consciousness. Horizontal or horizontal-rotatory nystagmus is present during the acute attack.10,11 The purpose of this survey was to study the prevalence of MD in the general population of Southern Finland.
MATERIAL AND METHODS A questionnaire was sent to 5000 persons aged 12 years or more living in the Helsinki University Hospital area. The people were randomly selected from the population register data. Forty-seven percent (n ⫽ 2359) of the recipients were male and 53% (n ⫽ 2641) were female. Their mean age was 44 (range, 12 to 99) years. The mean age of men was 43 years and of women 46 years. In the questionnaire (Appendix 1), we asked whether the recipient had experienced vertigo associated with a moving sensation. The questionnaire included specific questions on hearing loss, tinnitus, medication, general illnesses, former head and ear traumas, ear operations, ear infections, exposure to noise, headache, and use of tobacco and alcohol. We also enquired about socioeconomic sta-
tus and education. Patients reporting vertigo attacks were further asked about the frequency, intensity, and duration of the attacks. We also asked their age at onset of vertigo symptoms and whether concomitant nausea was present during the attacks. Finally, we asked how much time had elapsed since the last vertigo attack and whether vertigo has an influence on working capacity. The questionnaire was structured based on a “dizzy” questionnaire designed for otoneurology expert system ONE.12 The questionnaire reached 4732 people. A group of 241 people was not reached because of a change in postal address, 25 people had died before getting the questionnaire, and 2 were staying abroad. The questionnaire was returned by 3138 subjects after 1 postal reminder, yielding a response rate of 63%. Twenty people returned the questionnaire unanswered being unwilling or unable to answer the questions. Among the unanswered questionnaires was one addressed to a 12-year-old girl whose mother had returned it, declaring it unsuitable for someone her daughter’s age. Two women, employed in the health care field, answered the questionnaires on behalf of their husbands. One reported doing so because her husband was too busy to respond himself, and the other because she had vertigo symptoms, not her husband. After excluding the unanswered questionnaires and these 2 women, the final study group consisted of 3116 people, which was 63% of the initial group of 5000 when the dead and individuals living abroad were excluded. To evaluate the validity of the population study, we randomly selected a sample of 100 people among those reporting vertigo. The sample was clinically examined by the first author (M.H.) in the vestibular unit of Helsinki University Hospital. The examinations consisted of otorhinolaryngologic and neurologic investigations supplemented with a hearing test and otoneurotologic tests (electronystagmography, posturography, saccadic and pursuit eye movements, otoacustic emissions).
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Table 2 Age distribution Total sample 5000
Final sample 3116
Age (years)
Total
Male
Female
Total
Male
Female
12–30 31–40 41–50 51–60 61–70 ⬎70
1308 941 908 856 473 514
657 454 433 409 226 180
651 487 475 447 247 334
732 545 606 592 351 282
285 238 265 285 175 100
444 306 340 304 176 181
concomitant nausea significantly increased the possibility of previous examinations in this group. Cochlear symptoms preceded vertigo in 61% of subjects. In 29%, the first cochlear symptom was hearing loss, and in 32% tinnitus. Using the 1995 AA0 –HNS criteria and excluding subjects who may have had another reason such as former head and ear traumas, previous ear operations, brain and heart ischemia, exposure to noise without ear protection, epilepsy, provocing factors for vertigo (eg, getting up rapidly from a sitting position or bending the head backwards), we found 16 definite MD patients. In addition, there were 31 probable and 28 possible MD patients. A flow chart describing subject selection is presented in Fig 1.
In the statistical analysis, we calculated frequencies, means, and ranges for the study variables. Differences between the groups were assessed by cross-tabulation and tested by chi-square test. Correlation coefficients (Pearson) were calculated to show the degree of relation. Statistical significance was defined as P ⬍ 0.05. All analyses were performed using the SPSS 9.0 statistical program. The study protocol was approved by the Ethics Committee of the Helsinki University Hospital.
RESULTS The final study group comprised 1350 (43%) men and 1751 (56 %) women. Fifteen people (⬍ 1%) did not reveal their gender and 8 did not mention their age, as can be seen in Table 2. The mean age of respondents was 45 (range, 12 to 99) years. Six percent were aged 18 years or under, and 6 12-year-old children returned their carefully filled in questionnaires. Of the 3116 subjects in the study, 908 (29%) reported vertigo, 1245 (40%) reported tinnitus, and 627 (20%) reported hearing loss. The mean age of those reporting vertigo (48 years) was higher than the mean age of the entire study group. Two hundred sixteen subjects reported the triad of vertigo, hearing loss, and tinnitus. There were 91 (42%) men and 125 (58%) women in this group, and 61% of them had not been examined for their vertigo. An increase in intensity, frequency, and duration of vertigo attacks as well as
Definite MD Patients After excluding other possible reasons for symptoms, 16 patients remained who were considered to have definite MD. Among this group were 9 people who had previously been given a MD diagnosis. Their medical records confirmed their diagnosis of definite MD. One male patient reporting MD was excluded from this group because his medical records revealed that vertigo attacks lasted only for 2 minutes. One of the 9 patients with a previously known diagnosis was included in the randomly selected sample of vertigo patients, and thus, was clinically examined. She was not, however, included in the triad group because she failed to report tinnitus in the questionnaire; but when asked, she did report tinnitus, and this symptom was also mentioned in her medical records. One new MD patient was diagnosed in
3116 subjects in final study sample
216 subjects with triad of symptoms
100 vertigo patients
8 patients with previous MD diagnose
2 MD patients (one new patient)
59 possible/probable MD patients 6 patients without previous MD diagnose
3041 no MD
28 possible MD 31 probable MD
Figure 1
16 definite MD
Flow chart of the subject selection.
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Prevalence of Menière’s Disease in General . . .
Figure 2
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Prevalence of the definite MD patients in different age groups.
the clinical examination of the sample. She had been previously examined for hearing loss symptoms but had been misdiagnosed as having hereditary cochlear degeneration. Altogether, there were 10 people for whom a definite MD diagnosis could be confirmed; 9 by evaluating their medical records and one by clinical examination. Six other people in this group of 16 definite MD patients did not have a previous MD diagnosis. Four of these patients had not been examined for their symptoms, and 2 had been examined but had not been diagnosed as having MD. Of the definite MD patients, 13 were women and 3 were men. The mean duration of their symptoms was 19 (range, 2 to 41) years, and the mean age at onset was 44 (range,19 to 77) years. Fourteen of 16 definite MD patients had had symptoms for more than 10 years. Six had fluctuating hearing loss, 8 had fixed hearing loss, and 2 did not report the nature of their hearing loss. One of the patients with fixed hearing loss had had vertigo symptoms for 4 years, but in all other patients the symptoms began 19 to 41 years earlier. All of the patients with fluctuating hearing loss had had symptoms for less than 23 years. Thirteen of the16 definite MD patients answered the question on initial symptom of the triad; and 7 had started the disease with cochlear symptoms, 5 with hearing loss, and 2 with tinnitus. Ten of the 16 patients reported sudden drop attacks, and in 90% of these the vertigo symptoms had started more than 10 years ago. No one had a history of brain infarction. Three people reported excessive noise at work, and 2 of these belonged to the group of 10 whose medical records confirmed definite MD. One of the 16 patients had been operated on because of MD (2 operations: saccus operation and vestibular neurectomy) and another patient had received gentamicin treatment. Based on the 16 definite MD patients found here the population prevalence of definite MD was estimated to be 513 of 100 000 persons. If we are extremely conservative and include only the 10 patients for whom MD diagnosis could be confirmed (9 by medical records and 1 by clinical examination), the rate would be 321 of 100 000. To assess the prevalence of definite MD by age, we divided the data
into 6 age groups. The detailed information is displayed in Table 2. A peak prevalence of 1709 of 100 000 was seen in the age group 61 to 70 years. Prevalence of the definite MD patients in different age groups is presented in Fig 2.
DISCUSSION We found the prevalence of MD in a general Finnish population to be 513 of 100 000 persons. This figure is much higher than in earlier reports, and the actual prevalence can be assumed to be even higher for several reasons.2-7 Only 3 men were in the final group of 16 definite MD patients and no children; the youngest patient was 39 years old. In previous reports, MD has been shown to affect both genders equally.13 However, men tend to be less eager to participate in these kinds of questionnaires, as can be seen in our response ratio: women (56%) vs men (43%). Interestingly, in the age group 51 to 70 years, which was particularly affected by MD, the relative response rate of men was found higher than that of women. In addition, men, more so than women, are exposed to noise, especially in the workplace. Their reported noise-induced hearing loss with or without audiologic measurements forced us to exclude many of them from the definite MD group. Sixty-two percent of those reporting hearing loss and history of noise at work of the entire subject pool of 3116 people were men. Meyerhoff et al9 found 3% of all patients with MD to belong to the pediatric age group. When prevalence rate is assessed by questionnaire, children may be unable to answer the questions, and thus being excluded from the study, as the letter from a mother of a 12-year-old confirmed. The actual prevalence rate may be higher than reported here also because only 2 of the 16 definite MD patients had had symptoms less than 10 years. The subjects with recent onset of disease seemed to be missing. The mean age of 44 years at the onset of symptoms is in line with Watanabe et al,5 who reported the mean age at onset for women to be 41 and for men to be 42. The mean
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age in the final study group was 45 years. Because the mean age of the random sample of 5000 people was 44 years, our final study group can be considered to be quite representative of the general population. The definition of MD has changed over the years, which makes it difficult to compare prevalence rates. The then high prevalence estimation of 218 of 100 000 by WladislavoskyWaserman et al3 was published at the time of the first AAO–HNS recommendations. Kotimäki et al7 speculated that the reason for this high figure could be that both cochlear and vestibular forms of the disease were included in the prevalence calculations. We present, however, a prevalence rate more than 2-fold higher than WladislavoskyWaserman’s rate using the same criteria from 1995 as Kotimäki et al. Tokomasu et al14 reported cochlear signs to start before onset of a vertigo attack in 17 (60%) of 28 patients. We found cochlear symptoms to precede vertigo in 61% of subjects in the triad group; in 29%, the first symptom was hearing loss, and in 32% tinnitus. Over half of the definite MD patients whose initial symptom was reported to us had also started their disease with cochlear symptoms; in their cases, 5 patients had hearing loss and 2 tinnitus. When the initial symptoms are cochlear there is an increased risk that they will be missattributed to another cause or presumed to be due to aging. After the vestibular component becomes apparent, the long periods of remission may mask the episodic nature of vertigo, and the triad of symptoms may go such that only clinically moderate to severe cases are taken into prevalence estimations.15 We noted also that the longer, the more intense, and the more frequent the vertigo attacks and the more severe the concomitant nausea, the more probable it was that previous examinations had been performed to investigate vertigo symptoms. Earlier studies on the prevalence of MD have been based on hospital registers, which have their weaknesses. Data collected during routine hospital visits can vary in accuracy and quality since people acquiring the information change.1 In the clinical examination of the subjects with vertigo, we found a patient with definite MD who had been examined by an ENT specialist, but when evaluating her medical records, no mention of vertigo attack could be found. Twenty-five years ago she had been set a diagnosis of cochlear degeneration; she had received a hearing aid but no treatment for vertigo. The 9 MD patients whose medical records confirmed their diagnosis and the 1 discovered in the sample of vertigo patients, all came from the group who had been previously examined for vertigo. We found that only 39% of subjects with the triad of symptoms had previous examinations for vertigo. Thus, many possible MD patients can be assumed to be contained within the group without examinations. Creating a questionnaire that is easy to understand and complete for all recipients can be challenging. Some people had to be excluded from the prevalence estimations because of inadequate answers to the questions that were most important to us concerning the frequency and duration of
vertigo attacks and the presence of tinnitus. By doing so, we may have excluded some definite MD patients. One definite MD patient was not included in the triad group because she failed to report tinnitus on the questionnaire, although she had tinnitus when examined in the vertigo sample. Apparently tinnitus no longer bothered her. The changes in the clinical picture of MD over time also influence the answers. The symptoms resolve to varying degrees with disease progression.16 Some definite MD patients described shorter, less nausea-associated, and less frequent vertigo attacks than before. Retired individuals have been found to be more likely to report “no disability” despite a significant number of vertigo attacks.17 We observed a peak prevalence in the age group 61 to 70 years. The possibility of MD should be considered in this group affected by presbyacusis and whose vertigo might easily be diagnosed as being of central origin. From our group of definite MD patients, we had excluded those reporting a history of brain ischemia. By doing so, we may have also excluded some patients who initially had MD but who now also had brain ischemia.
CONCLUSIONS The MD prevalence rate of 513 of 100,000 persons in Southern Finland is much higher than the prevalence estimations based on hospital registers around the world have suggested. The peak prevalence was seen in elderly people aged 61 to 70 years. The reason for the decrease in the rate in the oldest age group (⬎70 years) may be that these individuals, with their numerous other diseases and symptoms, are often excluded using this method. We thank Professor Hilkka Riihimäki, Head of Department of Epidemiology and Statistics, Finnish Institute of Occupational Health for her valuable comments.
REFERENCES 1. Costa SS, Sousa LC, Piza MR. Menière’s disease: overview, epidemiology, and natural history. Otolaryngol Clin N Am 2002;35:455–95. 2. Cawthorne T, Hewlett AB. Menière’s disease. Proc R Soc Med 1954; 47:633–70. 3. Wladislavosky P, Facer GW, Mokri B, et al. Menière’s disease: a 30-year epidemiologic and clinical study in Rochester, MN, 19511980. Laryngoscope 1984;94:1098 –102. 4. Stahle J, Stahle C, Arenberg K. Incidence of Menière’s disease. Arch Otolaryngol 1978;104:99 –102. 5. Watanabe Y, Mizukoshi K, Shojaku H, et al. Epidemiological and clinical characteristics of Meniere’s disease in Japan. Acta Otolaryngol 1995;519:206 –10. 6. Celestino D, Ralli G. Incidence of Meniere’s disease in Italy. Am J Otol 1991;12:135– 8. 7. Kotimäki J, Sorri M, Aantaa E, et al. Prevalence of Meniere’s disease in Finland. Laryngoscope 1999;109:748 –53. 8. Ballester M, Liard P, Vibert D, et al. Meniere’s disease in the elderly. Otol Neurotol 2002;23:73– 8.
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9. Meyerhoff WL, Paparella MM, Shea D. Meniere’s disease in children. Laryngoscope 1978;88:1504 –11. 10. Pearson BW, Brackmann DE. Committee on Hearing and Equilibrium guidelines for reporting treatment results in Meniere’s disease. Otolaryngol Head Neck Surg 1985;93:579 – 81. 11. Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere’s disease. Otolaryngol Head Neck Surg 1995;113:181–5. 12. Kentala E. Characteristics of six otologic diseases involving vertigo. Am J Otol 1996;17:883–92. 13. Tokumasu K, Fujino A, Yoshio S, et al. Prognosis of Meniere’s disease by conservative treatment: retrospective study on the
14.
15.
16. 17.
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time course of the disease. Acta Otolaryngol Suppl 1995;519: 216 – 8. Tokumasu K, Fujino A, Naganuma H, et al. Initial symptoms and retrospective evaluation of prognosis in Meniere’s disease. Acta Otolaryngol Suppl 1996;524:43–9. Arenberg IK, Balkaby TJ, Goldman G, et al. The incidence and prevalence of Meniere’s disease: a statistical analysis of limits. Otolaryngol Clin N Am 1980;13:597– 601. Stahle J, Friberg U, Svedberg A. Long-term progression of Meniere’s disease. Acta Otolaryngol Suppl 1991;485:78 – 83. Silverstein H, Smouha E, Jones R. Natural history vs surgery for Meniere’s disease. Otolaryngol Head Neck Surg 1989;100:6 –16.
APPENDIX POPULATION QUESTIONNAIRE Please answer the following questions and circle the most appropriate alternative. Surname: First name: Social security number: Sex: Woman Man Age: Height: Weight: Place of residence (e.g. town, countryside): Smoking: Yes No Does your skin get easily burned by the sun?: Always Often Sometimes never Occupation (also the previous one): Education: a) elementary school b) trade school c) college d) secondary school graduate e) university Have you been exposed to noise in your work? Yes No Has the use of hearing protection been recommended or is the noise level in your work over 85 dB? Yes No How long have you been exposed to noise in your work? Noise level at work? Have you had a 1) head trauma Yes No 2) sudden acoustic trauma Yes No Have you had recurrent (more than 3) middle ear infections? Yes No Do you consider your hearing to be normal for a person of your age? Yes No If your hearing is impaired, please answer the following questions: Is there a specific reason for your hearing loss? Is your hearing impaired: in the Right Left Both ears? Does your hearing fluctuate? Yes No Do you suffer from your Not at all A little Quite a Very impaired hearing? lot much Has your hearing level been Yes No Do you know your hearing measured? level? Do you suffer from ringing or humming in your ears? Not at all A little Quite a lot Very much If ringing or humming occurs, is it in your Right Left Both ears? Do you suffer from headaches? Not at all A little Quite a lot Very much Do you ever faint? Never Seldom Sometimes Very often Are you on medication on a regular basis? What medication? Yes No
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Do you have any of the following diseases? Hypertonia Diabetes Coronary artery disease Brain circulation disorder Thyroid disorder Another disease: Is your cholesterol level above normal? What is your cholesterol level? Yes No Have you experienced sudden falls or staggered without loss of consciousness? Yes No Have you experienced vertigo together with a moving sensation? Yes No If you answered “yes” to the previous question, please answer the following questions. If you answered “no,” we thank you for your participation and for returning the questionnaire. How long have you experienced vertigo? How old were you when your vertigo symptoms began? How much do you suffer from your vertigo? Not at all A little Quite a lot Very much How often does vertigo occur? a) only one time b) less than once a year c) 1-2 times a year d) 3-12 times a year e) 1-4 times a month f) 2-7 times a week g) several times a day h) constantly How long does the sensation of vertigo last? a) less than 15 seconds b) 15 seconds-5 minutes c) more than 5 minutes-4 hours d) more than 4 hours-24 hours e) several days Does nausea occur concomitantly with vertigo? a) never b) seldom c) often d) always How intense is your attack? a) mild (can continue working) b) moderate (have to stop working) c) severe (have to rest) d) very severe (have trouble even in rest) Have you noticed anything that might provoke vertigo? If yes, what? When did you have your last episode of vertigo? Have you been examined by a doctor for vertigo? Yes No Does vertigo affect your work ability? Yes No If you have more than one of the above symptoms (vertigo, hearing loss, tinnitus), which was the first to appear?
ORIGINAL RESEARCH
Prevalence of Menière’s Disease in General Population of Southern Finland Mari Havia, MD, PhD, Erna Kentala, MD, PhD, and Ilmari Pyykkö, MD, PhD, Helsinki and Tampere, Finland
M
OBJECTIVE: To study the prevalence of Menière’s disease (MD) in the general population of Southern Finland. STUDY DESIGN AND SETTING: Prospective study based on population register data. A questionnaire was sent to 5000 randomly selected persons aged 12 years or more living in the Helsinki University Hospital area. We enquired about whether recipients had experienced vertigo associated with a moving sensation, hearing loss, or tinnitus. For exclusion purposes, we also asked about general illnesses, ear infections, former head and ear traumas, noise exposure, medication, and use of tobacco and alcohol. To assess the validity of the population-based study, we randomly selected a sample of 100 people among those reporting vertigo. They were clinically examined at our vestibular unit. The clinical examination was supplemented by audiologic and otoneurologic tests. RESULTS: The response rate was 63%. In the final study sample of 3116 people, 216 reported the triad of vertigo, hearing loss, and tinnitus. By using the most recent criteria of the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology–Head and Neck Surgery, we were able to identify 16 definite MD patients from the total sample, yielding a prevalence of 513 of 100,000 persons. Among the 16 MD subjects, 9 patients had already been diagnosed with MD and 1 patient was diagnosed during the clinical examination. A peak prevalence of 1709 of 100,000 was seen in the age group 61 to 70 years. CONCLUSIONS: Our population-based estimate of MD prevalence is much higher than in previous reports. EBM RATING: B-3. © 2005 American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. All rights reserved.
eniére’s disease (MD) has been a target of active otoneurologic research for decades. Although the diagnostic criteria have been specified, the prevalence of the disease is still unknown.1 Estimations of prevalence have been based on hospital register data extrapolated to the general population. In Great Britain in 1954, Cawthorne and Hewlett2 reported 43 patients in 1 year in a population of 27,365 people, yielding an incidence of 157 per 100,000. Their study has been criticized by Wladislavosky-Waserman et al,3 who suspected that the result might be a combination of incidence and prevalence because it was unclear whether the study included patients with onset of the symptoms in preceding years. Wladislavosky-Waserman et al themselves reported a prevalence of 218 per 100 000. Stahle et al4 described an incidence of 46 per 100,000 in Sweden in 1978. According to Wladislavosky-Waserman et al,3 however, the statistics in the Swedish study were a combination of incidence and prevalence and did not include data from private practices, which comprised approximately 23% of Swedish medical care at that time. In Japan, Watanabe et al5 reported a prevalence of 16 or 17 per 100,000 but their study was not population-based either. Celestino and Ralli6 found the incidence of MD to be 8 per 100,000 in Italy and the figure to be 3.4 times greater among hospital personnel. The greater availability of medical facilities apparently increased the number of diagnoses. Kotimäki et al7 assessed the prevalence of MD in Finland in 1999. Their study was based on the re-evaluation of hospital records of 306 MD patients treated in 7 different hospitals in Finland. They reported a prevalence of 43 per 100,000 but came to the conclusion, that the prevalence is
From the Department of Otorhinolaryngology (Drs Havia and Kentala), Helsinki University Hospital, and the Department of Otorhinolaryngology (Dr Pyykkö), Tampere University Hospital, Tampere, Finland. Presented as a poster at the Annual Meeting of American Academy of
Otolaryngology–Head and Neck Surgery, New York, NY, September 1922, 2004. Reprint requests: Mari Havia, PO Box 220, 00029 HUS, Finland. E-mail address: [email protected].
0194-5998/$30.00 © 2005 American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. All rights reserved. doi:10.1016/j.otohns.2005.06.015
Havia et al
Prevalence of Menière’s Disease in General . . .
763
Table 1 Criteria for diagnosis of MD (AAO-HNS 1995) Certain MD
Definite MD, plus histopathologic confirmation
Definite MD
Two or more definitive spontaneous episodes of vertigo lasting 20 minutes or longer Audiometrically documented hearing loss on at least one occasion Tinnitis or aural fullness in the treated ear Other causes excluded
Probable MD
One definitive episode of vertigo Audiometrically documented hearing loss on at least one occasion Tinnitis or aural fullness in the treated ear Other causes excluded.
Possible MD
Episodic vertigo of the Menière type without documented hearing loss, or Sensorineural hearing loss, fluctuating or fixed, with dysequilibrium but without episodes Other causes excluded.
probably underestimated when data are retrieved retrospectively from patient documents. MD among the elderly has been reported by Ballester et al8 to be quite common. In their opinion, drop attacks are also more frequent in patients with MD than previously reported, and these can mimic a stroke of the brainstem. Meyerhoff et al9 found approximately 3% of all the MD patients to belong to the pediatric age group. The American Academy of Otolaryngology–Head and Neck Surgery (AAO–HNS) has updated its criteria twice since 1972. The current AAO–HNS criteria for definite MD require 2 or more episodes of vertigo lasting 20 minutes or more in addition to hearing loss and tinnitus (Table 1). Tinnitus can also be replaced by aural fullness. Vertigo is often accompanied by nausea. MD is not associated with a loss of consciousness. Horizontal or horizontal-rotatory nystagmus is present during the acute attack.10,11 The purpose of this survey was to study the prevalence of MD in the general population of Southern Finland.
MATERIAL AND METHODS A questionnaire was sent to 5000 persons aged 12 years or more living in the Helsinki University Hospital area. The people were randomly selected from the population register data. Forty-seven percent (n ⫽ 2359) of the recipients were male and 53% (n ⫽ 2641) were female. Their mean age was 44 (range, 12 to 99) years. The mean age of men was 43 years and of women 46 years. In the questionnaire (Appendix 1), we asked whether the recipient had experienced vertigo associated with a moving sensation. The questionnaire included specific questions on hearing loss, tinnitus, medication, general illnesses, former head and ear traumas, ear operations, ear infections, exposure to noise, headache, and use of tobacco and alcohol. We also enquired about socioeconomic sta-
tus and education. Patients reporting vertigo attacks were further asked about the frequency, intensity, and duration of the attacks. We also asked their age at onset of vertigo symptoms and whether concomitant nausea was present during the attacks. Finally, we asked how much time had elapsed since the last vertigo attack and whether vertigo has an influence on working capacity. The questionnaire was structured based on a “dizzy” questionnaire designed for otoneurology expert system ONE.12 The questionnaire reached 4732 people. A group of 241 people was not reached because of a change in postal address, 25 people had died before getting the questionnaire, and 2 were staying abroad. The questionnaire was returned by 3138 subjects after 1 postal reminder, yielding a response rate of 63%. Twenty people returned the questionnaire unanswered being unwilling or unable to answer the questions. Among the unanswered questionnaires was one addressed to a 12-year-old girl whose mother had returned it, declaring it unsuitable for someone her daughter’s age. Two women, employed in the health care field, answered the questionnaires on behalf of their husbands. One reported doing so because her husband was too busy to respond himself, and the other because she had vertigo symptoms, not her husband. After excluding the unanswered questionnaires and these 2 women, the final study group consisted of 3116 people, which was 63% of the initial group of 5000 when the dead and individuals living abroad were excluded. To evaluate the validity of the population study, we randomly selected a sample of 100 people among those reporting vertigo. The sample was clinically examined by the first author (M.H.) in the vestibular unit of Helsinki University Hospital. The examinations consisted of otorhinolaryngologic and neurologic investigations supplemented with a hearing test and otoneurotologic tests (electronystagmography, posturography, saccadic and pursuit eye movements, otoacustic emissions).
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Table 2 Age distribution Total sample 5000
Final sample 3116
Age (years)
Total
Male
Female
Total
Male
Female
12–30 31–40 41–50 51–60 61–70 ⬎70
1308 941 908 856 473 514
657 454 433 409 226 180
651 487 475 447 247 334
732 545 606 592 351 282
285 238 265 285 175 100
444 306 340 304 176 181
concomitant nausea significantly increased the possibility of previous examinations in this group. Cochlear symptoms preceded vertigo in 61% of subjects. In 29%, the first cochlear symptom was hearing loss, and in 32% tinnitus. Using the 1995 AA0 –HNS criteria and excluding subjects who may have had another reason such as former head and ear traumas, previous ear operations, brain and heart ischemia, exposure to noise without ear protection, epilepsy, provocing factors for vertigo (eg, getting up rapidly from a sitting position or bending the head backwards), we found 16 definite MD patients. In addition, there were 31 probable and 28 possible MD patients. A flow chart describing subject selection is presented in Fig 1.
In the statistical analysis, we calculated frequencies, means, and ranges for the study variables. Differences between the groups were assessed by cross-tabulation and tested by chi-square test. Correlation coefficients (Pearson) were calculated to show the degree of relation. Statistical significance was defined as P ⬍ 0.05. All analyses were performed using the SPSS 9.0 statistical program. The study protocol was approved by the Ethics Committee of the Helsinki University Hospital.
RESULTS The final study group comprised 1350 (43%) men and 1751 (56 %) women. Fifteen people (⬍ 1%) did not reveal their gender and 8 did not mention their age, as can be seen in Table 2. The mean age of respondents was 45 (range, 12 to 99) years. Six percent were aged 18 years or under, and 6 12-year-old children returned their carefully filled in questionnaires. Of the 3116 subjects in the study, 908 (29%) reported vertigo, 1245 (40%) reported tinnitus, and 627 (20%) reported hearing loss. The mean age of those reporting vertigo (48 years) was higher than the mean age of the entire study group. Two hundred sixteen subjects reported the triad of vertigo, hearing loss, and tinnitus. There were 91 (42%) men and 125 (58%) women in this group, and 61% of them had not been examined for their vertigo. An increase in intensity, frequency, and duration of vertigo attacks as well as
Definite MD Patients After excluding other possible reasons for symptoms, 16 patients remained who were considered to have definite MD. Among this group were 9 people who had previously been given a MD diagnosis. Their medical records confirmed their diagnosis of definite MD. One male patient reporting MD was excluded from this group because his medical records revealed that vertigo attacks lasted only for 2 minutes. One of the 9 patients with a previously known diagnosis was included in the randomly selected sample of vertigo patients, and thus, was clinically examined. She was not, however, included in the triad group because she failed to report tinnitus in the questionnaire; but when asked, she did report tinnitus, and this symptom was also mentioned in her medical records. One new MD patient was diagnosed in
3116 subjects in final study sample
216 subjects with triad of symptoms
100 vertigo patients
8 patients with previous MD diagnose
2 MD patients (one new patient)
59 possible/probable MD patients 6 patients without previous MD diagnose
3041 no MD
28 possible MD 31 probable MD
Figure 1
16 definite MD
Flow chart of the subject selection.
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Prevalence of Menière’s Disease in General . . .
Figure 2
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Prevalence of the definite MD patients in different age groups.
the clinical examination of the sample. She had been previously examined for hearing loss symptoms but had been misdiagnosed as having hereditary cochlear degeneration. Altogether, there were 10 people for whom a definite MD diagnosis could be confirmed; 9 by evaluating their medical records and one by clinical examination. Six other people in this group of 16 definite MD patients did not have a previous MD diagnosis. Four of these patients had not been examined for their symptoms, and 2 had been examined but had not been diagnosed as having MD. Of the definite MD patients, 13 were women and 3 were men. The mean duration of their symptoms was 19 (range, 2 to 41) years, and the mean age at onset was 44 (range,19 to 77) years. Fourteen of 16 definite MD patients had had symptoms for more than 10 years. Six had fluctuating hearing loss, 8 had fixed hearing loss, and 2 did not report the nature of their hearing loss. One of the patients with fixed hearing loss had had vertigo symptoms for 4 years, but in all other patients the symptoms began 19 to 41 years earlier. All of the patients with fluctuating hearing loss had had symptoms for less than 23 years. Thirteen of the16 definite MD patients answered the question on initial symptom of the triad; and 7 had started the disease with cochlear symptoms, 5 with hearing loss, and 2 with tinnitus. Ten of the 16 patients reported sudden drop attacks, and in 90% of these the vertigo symptoms had started more than 10 years ago. No one had a history of brain infarction. Three people reported excessive noise at work, and 2 of these belonged to the group of 10 whose medical records confirmed definite MD. One of the 16 patients had been operated on because of MD (2 operations: saccus operation and vestibular neurectomy) and another patient had received gentamicin treatment. Based on the 16 definite MD patients found here the population prevalence of definite MD was estimated to be 513 of 100 000 persons. If we are extremely conservative and include only the 10 patients for whom MD diagnosis could be confirmed (9 by medical records and 1 by clinical examination), the rate would be 321 of 100 000. To assess the prevalence of definite MD by age, we divided the data
into 6 age groups. The detailed information is displayed in Table 2. A peak prevalence of 1709 of 100 000 was seen in the age group 61 to 70 years. Prevalence of the definite MD patients in different age groups is presented in Fig 2.
DISCUSSION We found the prevalence of MD in a general Finnish population to be 513 of 100 000 persons. This figure is much higher than in earlier reports, and the actual prevalence can be assumed to be even higher for several reasons.2-7 Only 3 men were in the final group of 16 definite MD patients and no children; the youngest patient was 39 years old. In previous reports, MD has been shown to affect both genders equally.13 However, men tend to be less eager to participate in these kinds of questionnaires, as can be seen in our response ratio: women (56%) vs men (43%). Interestingly, in the age group 51 to 70 years, which was particularly affected by MD, the relative response rate of men was found higher than that of women. In addition, men, more so than women, are exposed to noise, especially in the workplace. Their reported noise-induced hearing loss with or without audiologic measurements forced us to exclude many of them from the definite MD group. Sixty-two percent of those reporting hearing loss and history of noise at work of the entire subject pool of 3116 people were men. Meyerhoff et al9 found 3% of all patients with MD to belong to the pediatric age group. When prevalence rate is assessed by questionnaire, children may be unable to answer the questions, and thus being excluded from the study, as the letter from a mother of a 12-year-old confirmed. The actual prevalence rate may be higher than reported here also because only 2 of the 16 definite MD patients had had symptoms less than 10 years. The subjects with recent onset of disease seemed to be missing. The mean age of 44 years at the onset of symptoms is in line with Watanabe et al,5 who reported the mean age at onset for women to be 41 and for men to be 42. The mean
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age in the final study group was 45 years. Because the mean age of the random sample of 5000 people was 44 years, our final study group can be considered to be quite representative of the general population. The definition of MD has changed over the years, which makes it difficult to compare prevalence rates. The then high prevalence estimation of 218 of 100 000 by WladislavoskyWaserman et al3 was published at the time of the first AAO–HNS recommendations. Kotimäki et al7 speculated that the reason for this high figure could be that both cochlear and vestibular forms of the disease were included in the prevalence calculations. We present, however, a prevalence rate more than 2-fold higher than WladislavoskyWaserman’s rate using the same criteria from 1995 as Kotimäki et al. Tokomasu et al14 reported cochlear signs to start before onset of a vertigo attack in 17 (60%) of 28 patients. We found cochlear symptoms to precede vertigo in 61% of subjects in the triad group; in 29%, the first symptom was hearing loss, and in 32% tinnitus. Over half of the definite MD patients whose initial symptom was reported to us had also started their disease with cochlear symptoms; in their cases, 5 patients had hearing loss and 2 tinnitus. When the initial symptoms are cochlear there is an increased risk that they will be missattributed to another cause or presumed to be due to aging. After the vestibular component becomes apparent, the long periods of remission may mask the episodic nature of vertigo, and the triad of symptoms may go such that only clinically moderate to severe cases are taken into prevalence estimations.15 We noted also that the longer, the more intense, and the more frequent the vertigo attacks and the more severe the concomitant nausea, the more probable it was that previous examinations had been performed to investigate vertigo symptoms. Earlier studies on the prevalence of MD have been based on hospital registers, which have their weaknesses. Data collected during routine hospital visits can vary in accuracy and quality since people acquiring the information change.1 In the clinical examination of the subjects with vertigo, we found a patient with definite MD who had been examined by an ENT specialist, but when evaluating her medical records, no mention of vertigo attack could be found. Twenty-five years ago she had been set a diagnosis of cochlear degeneration; she had received a hearing aid but no treatment for vertigo. The 9 MD patients whose medical records confirmed their diagnosis and the 1 discovered in the sample of vertigo patients, all came from the group who had been previously examined for vertigo. We found that only 39% of subjects with the triad of symptoms had previous examinations for vertigo. Thus, many possible MD patients can be assumed to be contained within the group without examinations. Creating a questionnaire that is easy to understand and complete for all recipients can be challenging. Some people had to be excluded from the prevalence estimations because of inadequate answers to the questions that were most important to us concerning the frequency and duration of
vertigo attacks and the presence of tinnitus. By doing so, we may have excluded some definite MD patients. One definite MD patient was not included in the triad group because she failed to report tinnitus on the questionnaire, although she had tinnitus when examined in the vertigo sample. Apparently tinnitus no longer bothered her. The changes in the clinical picture of MD over time also influence the answers. The symptoms resolve to varying degrees with disease progression.16 Some definite MD patients described shorter, less nausea-associated, and less frequent vertigo attacks than before. Retired individuals have been found to be more likely to report “no disability” despite a significant number of vertigo attacks.17 We observed a peak prevalence in the age group 61 to 70 years. The possibility of MD should be considered in this group affected by presbyacusis and whose vertigo might easily be diagnosed as being of central origin. From our group of definite MD patients, we had excluded those reporting a history of brain ischemia. By doing so, we may have also excluded some patients who initially had MD but who now also had brain ischemia.
CONCLUSIONS The MD prevalence rate of 513 of 100,000 persons in Southern Finland is much higher than the prevalence estimations based on hospital registers around the world have suggested. The peak prevalence was seen in elderly people aged 61 to 70 years. The reason for the decrease in the rate in the oldest age group (⬎70 years) may be that these individuals, with their numerous other diseases and symptoms, are often excluded using this method. We thank Professor Hilkka Riihimäki, Head of Department of Epidemiology and Statistics, Finnish Institute of Occupational Health for her valuable comments.
REFERENCES 1. Costa SS, Sousa LC, Piza MR. Menière’s disease: overview, epidemiology, and natural history. Otolaryngol Clin N Am 2002;35:455–95. 2. Cawthorne T, Hewlett AB. Menière’s disease. Proc R Soc Med 1954; 47:633–70. 3. Wladislavosky P, Facer GW, Mokri B, et al. Menière’s disease: a 30-year epidemiologic and clinical study in Rochester, MN, 19511980. Laryngoscope 1984;94:1098 –102. 4. Stahle J, Stahle C, Arenberg K. Incidence of Menière’s disease. Arch Otolaryngol 1978;104:99 –102. 5. Watanabe Y, Mizukoshi K, Shojaku H, et al. Epidemiological and clinical characteristics of Meniere’s disease in Japan. Acta Otolaryngol 1995;519:206 –10. 6. Celestino D, Ralli G. Incidence of Meniere’s disease in Italy. Am J Otol 1991;12:135– 8. 7. Kotimäki J, Sorri M, Aantaa E, et al. Prevalence of Meniere’s disease in Finland. Laryngoscope 1999;109:748 –53. 8. Ballester M, Liard P, Vibert D, et al. Meniere’s disease in the elderly. Otol Neurotol 2002;23:73– 8.
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9. Meyerhoff WL, Paparella MM, Shea D. Meniere’s disease in children. Laryngoscope 1978;88:1504 –11. 10. Pearson BW, Brackmann DE. Committee on Hearing and Equilibrium guidelines for reporting treatment results in Meniere’s disease. Otolaryngol Head Neck Surg 1985;93:579 – 81. 11. Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere’s disease. Otolaryngol Head Neck Surg 1995;113:181–5. 12. Kentala E. Characteristics of six otologic diseases involving vertigo. Am J Otol 1996;17:883–92. 13. Tokumasu K, Fujino A, Yoshio S, et al. Prognosis of Meniere’s disease by conservative treatment: retrospective study on the
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time course of the disease. Acta Otolaryngol Suppl 1995;519: 216 – 8. Tokumasu K, Fujino A, Naganuma H, et al. Initial symptoms and retrospective evaluation of prognosis in Meniere’s disease. Acta Otolaryngol Suppl 1996;524:43–9. Arenberg IK, Balkaby TJ, Goldman G, et al. The incidence and prevalence of Meniere’s disease: a statistical analysis of limits. Otolaryngol Clin N Am 1980;13:597– 601. Stahle J, Friberg U, Svedberg A. Long-term progression of Meniere’s disease. Acta Otolaryngol Suppl 1991;485:78 – 83. Silverstein H, Smouha E, Jones R. Natural history vs surgery for Meniere’s disease. Otolaryngol Head Neck Surg 1989;100:6 –16.
APPENDIX POPULATION QUESTIONNAIRE Please answer the following questions and circle the most appropriate alternative. Surname: First name: Social security number: Sex: Woman Man Age: Height: Weight: Place of residence (e.g. town, countryside): Smoking: Yes No Does your skin get easily burned by the sun?: Always Often Sometimes never Occupation (also the previous one): Education: a) elementary school b) trade school c) college d) secondary school graduate e) university Have you been exposed to noise in your work? Yes No Has the use of hearing protection been recommended or is the noise level in your work over 85 dB? Yes No How long have you been exposed to noise in your work? Noise level at work? Have you had a 1) head trauma Yes No 2) sudden acoustic trauma Yes No Have you had recurrent (more than 3) middle ear infections? Yes No Do you consider your hearing to be normal for a person of your age? Yes No If your hearing is impaired, please answer the following questions: Is there a specific reason for your hearing loss? Is your hearing impaired: in the Right Left Both ears? Does your hearing fluctuate? Yes No Do you suffer from your Not at all A little Quite a Very impaired hearing? lot much Has your hearing level been Yes No Do you know your hearing measured? level? Do you suffer from ringing or humming in your ears? Not at all A little Quite a lot Very much If ringing or humming occurs, is it in your Right Left Both ears? Do you suffer from headaches? Not at all A little Quite a lot Very much Do you ever faint? Never Seldom Sometimes Very often Are you on medication on a regular basis? What medication? Yes No
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Do you have any of the following diseases? Hypertonia Diabetes Coronary artery disease Brain circulation disorder Thyroid disorder Another disease: Is your cholesterol level above normal? What is your cholesterol level? Yes No Have you experienced sudden falls or staggered without loss of consciousness? Yes No Have you experienced vertigo together with a moving sensation? Yes No If you answered “yes” to the previous question, please answer the following questions. If you answered “no,” we thank you for your participation and for returning the questionnaire. How long have you experienced vertigo? How old were you when your vertigo symptoms began? How much do you suffer from your vertigo? Not at all A little Quite a lot Very much How often does vertigo occur? a) only one time b) less than once a year c) 1-2 times a year d) 3-12 times a year e) 1-4 times a month f) 2-7 times a week g) several times a day h) constantly How long does the sensation of vertigo last? a) less than 15 seconds b) 15 seconds-5 minutes c) more than 5 minutes-4 hours d) more than 4 hours-24 hours e) several days Does nausea occur concomitantly with vertigo? a) never b) seldom c) often d) always How intense is your attack? a) mild (can continue working) b) moderate (have to stop working) c) severe (have to rest) d) very severe (have trouble even in rest) Have you noticed anything that might provoke vertigo? If yes, what? When did you have your last episode of vertigo? Have you been examined by a doctor for vertigo? Yes No Does vertigo affect your work ability? Yes No If you have more than one of the above symptoms (vertigo, hearing loss, tinnitus), which was the first to appear?