Acta Otolaryngol (Stockh) 1995; Suppl 520: 25 1-255

Clinical Survey of Menieres Disease: 574 cases

C.T. HAID, D. WATERMEIER, S.R. WOLF and M. BERG

From the ENT-Deportinent tmd Skull Bcise Center, Euro-Med-Clmic, Fuerth, Germany. and ENT-Deppcirinwnt. Uiiiwrsily of
Erlangen - Nuremberg. Germany

Haid CT, Watermeier SR, Wolf SR, Berg M. Clinical survey of Menieres clisease: 574 ccises. Acta Otolaryngol (Stockh)
1995; Suppl 520: 251-255.
Only 27% out of 574 patients suffering from Menieres disease initially started with the typical Menieres triad. A great
advantage in order to diagnose Menieres disease can be established with the telemetric-ENG. With this equipment it is
possible to verify a nystagmus outside the clinic ( a t home, at work) at any time in patients suffering from vertigo attacks.
Most of our patients showed an omnifrequent sensorineural hearing loss on the affected side. Many of our patients with
Menieres disease suffered from internal diseases (e.g. hypotonia, hyperlipidemia. diabetes mellitus), allergy or an affection
of the paranasal sinuses, which may constitute co-factors triggering Menieres disease. Key ivords: Menieres clisecisr.
vertigo artacks, sensorineural hearing loss, tinnitus, k h n e t r i c - ENG.
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INTRODUCTION paranasal sinuses and petrous bone ( Stenvers projec-

Menieres disease, which was first described by Pros-
per Meniere in 186 I , consists of three classical symp-
toms: vertigo attacks, fluctuating hearing loss, and
tinnitus. Usually the patients are strongly annoyed by
these symptoms. The pathophysiology and pathogen-
For personal use only.
tion). In many cases a cranial C T or MRJ were
performed. The audiometry consisted of pure tone
threshold, speech audiogramm, supraliminal audio-
metric test and mostly a brain stem evoked response
audiometry ( BERA). In the vestibular examination

esis of Menieres disease seems to be an en-
dolymphatic hydrops ( 1) resulting in ruptures and
fistulas of the membranous labyrinth leading to sud-
den potassium contamination from the endolymph to
the perilymph of the tissue fluid environment of the
auditory and vestibular end-organs and peripheral
nerves. Furthermore it seems that Menieres disease
can be triggered by co-factors (2) like internal dis-
eases, affection of the paranasal sinuses or vessel loop
in the region of the cerebello-pontine angle. In this
paper a clinical survey of patients suffering from
Menieres disease will be presented.
MATERIAL AND METHODS
Our retrospective study is based on examinations of
574 patients suffering from classical Menieres dis-
ease (triad of symptoms). The examination period
was from 1974 until February 1994. In this publica-
tion the results of the first investigation are discussed.
A total of 299 patents were male (52%) with an
average age of 41 years (youngest person 10 years
and oldest 80 years); 275 were female (48%) with an
average age of 40 years (youngest patient 9 years and
oldest 73 years). The age distribution peaked between
40-50 years (164 patients = 29%). There was no side
preference. The diseased side was located on the right
side in 245 cases (43%) and on the left side in 262
(45Y~). Sixty-seven patients were suffering from a
bilateral Menieres disease ( 12%).
After establishing the patients history, ENT status,
routine X-ray examinations were made of the
(Frenzels glasses, ENG) the spontaneous nystagmus,
gaze nystagmus, postional/positioning test, vestibu-
lar-spinal reflex, neck reflex ( 3 ) , ocular motor func-
tion test, examination of the cranial nerves and
caloric test were carried out routinely, in many cases
also a rotatory test. Since 1991 it is possible to
substantiate a spontaneous nystagmus outside the
clinic (at home, at work) at any time with the tele-
metric-ENG (4) in patients suffering e.g. from vertigo
attacks like in Menieres disease.
RESULTS
The incidence of Menieres disease in our department
is 2,9% From 1974 until February 1994 approxi-
mately 20 000 thorough vestibular examinations were
carried out on patients suffering from vestibular dis-
orders, of which 574 were diagnosed as Menieres
disease.
At the beginning of the disease only 27% com-
plained of Menieres triad (Table I), whereas its
incidence was 100% at the end of the first year after
onset of the symptoms. Fifty-six percent of these
patients felt furthermore a fullness in the diseased ear.
Another initial symptom started with vertigo attacks
( 18%) or fluctuating hearing loss ( 14%) or tinnitus
(13%) or sudden hearing loss (12%) or fluctuating
hearing loss combined with tinnitus ( 11%).
The duration of vertigo attacks (Table 11) varied
widely, in 74% of our patients between half an hour
and some hours (irrigation stage). The number of
attacks (Table 111) showed great variation during the

( 1995 Scandinavian University Press ISSN 0365-5237

 252 C.T. Haid et al.

Table I. Initial symptoms of patients sufering from Table IV, Pathological vestibular results of putients
Menieress disease (285 cases) with Menieres disease (571 cases)

Cases %I Pathlcases %,

Meniere triad 761285 Spontaneous nystagmus 1421571 25%

Vertigo attacks 5212x5 Gaze nystagmus 01538 0Y
Fluctuating hearing loss 4112x5 Positional/positioning nystagmus 303/568 53Yu
Tinnitus 3712x5 Vestibular spinal reflexes 1471285 52%
Sudden hearing loss 3412x5 Ocular motor function test 2912x5 1 0%
Fluctuating hearing loss + tinnitus 321285 Caloric reaction 2881538 54%)
Sudden hearing loss + tinnitus 71285 Cranial nerves 5/285 2%)
Vertigo attacks and hearing loss 31285
Vertigo attacks and tinnitus 212x5
Fullness in the ear 11285 Table V. Results of the caloric reaction of patients
with Menieres disease (538 cases)
Table 11. Duration of vertigo attacks of patients
suffering from Menieress disease (126 cases) Pathlcases %I
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Hypofunction (unilateral) 671285 24%

Duration of vertigo attacks Cases o/U Hypofunction (bilateral) 121285 4%)
No caloric response 201538 4%
Seconds-30 minutes 18 14% Preponderance (directional) 37/285 13%)
112 h-12 h 93 74% Hyperreflexia (bilateral) 1 11285 4%
1 day-2 days 15 12%1 Hyperreflexia (diseased side) 14/285 5%

Normal reaction (2501538) 46%

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Table 111. Frequency of vertigo attacks of patients

sufering from Menieres diseuse (126 cases)
Frequency of vertigo attacks Cases %I
1-10 per day 25 20%
1-4 per week 58 46%
I--4 per month 30 24%)
1-8 per year 13 IOU/;,
course of the disease. Forty-six percent of our patients
suffered from vertigo attacks 1-4 times a week. The
time course of Menieres disease over years showed
mostly a constant tendency. Most of our patients
visited our department during the interval stage.
Spontaneous nystagmus occurred in only 25% of the
patients suffering from Menieres disease (Table IV).
In 55% of the cases it was directed toward the healthy
ear. With the aid of telemetric-ENG it was possible to
recognize the direction of the spontaneous nystagmus
outside the clinic during the irrigation stage. Even here
the spontaneous nystagmus was either directed toward
the healthy or diseased side. Sometimes it changed
directions, at the beginning of the irrigation stage first
beating to the diseased side and at the end toward the
healthy side and thereafter it disappeared. In a few
cases, a vertical beating spontaneous nystagmus could
be observed, perhaps caused by an affection of the
otoliths. In about half of our patients a weak position-
ing nystagmus, a disturbance of the vestibular spinal
reflexes and pathlogical results in the caloric test could
be observed (Table IV). The caloric response was
reduced on the diseased side in only 28% (24% unilat-
eral and 4% bilateral) of the cases and completely
abolished in only 4% (Table V). At the beginning of
the disease the caloric reaction was often normal.
Within years a hypofunction on the diseased side
usually occurred in one third of our cases. A direc-
tional preponderance was seen in 13%).A hyperreflexia
on the diseased side started in 5%) of our patients
suffering from Menieres disease. This was probably
due to irrigations of the labyrinth. In about half of our
examinations a peripheral vestibular lesion could be
verified on the diseased side. Using telemetric-ENG in
persons suffering from vertigo attacks like Menieres
disease a vestibular lesion could be established in
nearly 100% of our examined cases, if attacks occur
during the period when patients have this telemetric-
ENG on loan.
Normal hearing function was a rare finding in our
patients (6%). Most of them showed (Table VI) an
omnifrequent hearing loss (55%) on the diseased side
in the pure tone audiogram. A low frequency hearing
loss and a combination of high and low frequency
hearing loss occurred in 5% respectively 3%. Only 4
patients complained of total deafness on the affected
side (1%). A high frequency hearing loss, which usu-
ally does not represent a typical curve of Menieres
disease, could be observed in 29% but always only
during the interval stage. The audiogram curves (sen-
 Clinical survey of Menieres Disease 253

Table VI. Character of pure tone threshold of patients Table VIII. Some aspects of patients suffering from
with Menieres disease (592 cases), including unilateral Menieres disease (547 cases)
and bilateral Menieres disease
Path/cases XI
pathlcases %I
Allergy 551207 27%
High frequency hearing loss 1711592 29% Affection of paranasal sinuses 1 lS/SOO 24%
Low frequency hearing loss 321592 5% Infection of tonsills 871547 16%
Middle frequency hearing loss 31592 1% Status after tonsillectomy 1621547 30%
Omnifrequent hearing loss 3281592 55% Smoker 531224 24%
Combination of high and low Alcohol (regular) 29/216 13%
frequency hearing loss 171.592 3%
Total deafness 41592 1%
Normal hearing function 371592 6%) lence of Menieres disease in Japan was estimated at
40 cases per 1 million inhabitants.
In Great Britain, Harrison & Naftalin (10) esti-
Table VII. Internal diseases of patients suffering from mated the condition to occur in 1 patient per 1000
Menieres disease (405 cases) and in Sweden Stahle et al. ( 1 1 ) in 1 of 2,163. Like
Stahle et al., (11) it must be pointed out that Me-
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Pathlcases %, Prevalence nieres disease does not usually occur in children (3).
Menieres disease occurs with equal coincidence in
Hypotonia 1691405 42% ?
H ypertonia 821405 20% 15%
male and female and age of onset is similar (peak
Hyperlipidemia 103/195 53% 15% between 40-50 years, (12, 13, 9, 8, 14, 15).
Hyperuricemia 391158 ~410/U/~1l1%
S20%/?3/, In general Menieres disease is a unilateral disease.
Diabetes mellitus 671262 26% 4% Twelve percent of our patients suffered from this
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illness on both sides. Bilateral involvement has been

sorineural hearing loss) mostly revealed fluctuations
depending on the stage (irrigation stage or interval
stage). The supraliminal audiometric test was pre-
sented as a cochlear lesion.
Interestingly, many of our patients with Menieres
disease suffered from internal diseases, which was
significantly increased to prevalance ( 5 ) , like hypoto-
nia (42%), hypertonia (20%1),hyperlipidemia (53%1),
hyperuricemia (25%) or diabetes mellitus (26%,, Table
VII). Twenty-seven percent suffered from an allergy
(pollinosis or food allergy) and 24% affection of the
paranasal sinuses (Table VIII). These observations
can be interpreted as co-factors triggering Menieres
disease.
Although an intensive treatment with drugs was
always given, 84 of our patients suffering from in-
tractable vertigo attacks needed surgical therapy
(15%). Usually a vestibular neurectomy combined
with a neuro-vascular decompression was done via
the enlarged middle cranial fossa approach (2, 6).
DISCUSSION
The incidence of Menieres disease in our ENT-
department of the University of Erlangen-Nuremberg
is 2,9%1.Colman (7) reported an incidence of more
than 5% in his ENT Department. Pfaltz & Matefi (8)
from Switzerland and Watanabe et al. (9) at their
university hospital in Japan diagnosed 0.2%- 1.O% of
the out patients having Menieres disease. The preva-
reported to be between 5% and 46%) (8, 14, 16).
The onset of Menieres disease is often monosymp-
tomatic. Only 27% of our patients initially (Table I)
started with the typical Menieres triad, Pfaltz &
Matefis cases in 25% and Stahles & Klockhoffs in
41%.
During attacks (irrigation stage) a spontaneous
nystagmus usually always appears, which can be
directed towards the healthy or diseased side. During
the interval stage a spontaneous nystagmus could
only be identified in one fourth of our cases (Table
11). These findings have been confirmed by Pfaltz &
Matefi (8) Stahle & Klockhoff (17) who did not find
a spontaneous nystagmus at all during a symptom-
free interval, and Wada et al. (18) only in I%,. A great
advantage in order to diagnose Menieres disease can
be established with the telemetric-ENG (4). With this
equipment it is possible to verify a nystagmus outside
the clinic (at home, at work) at any time in patients
suffering from vertigo attacks. Hereby not only a
horizontal beating spontaneous nystagmus can be
observed but also, in some cases, a vertical beating
nystagmus. This phenomenon has also been reported
by Aschan & Stahle J. (19). A positional or position-
ing nystagmus could be identified in 53% of our
cases. Wada et al. (18) could objectify a positional
nystagmus in 33% and a positioning nystagmus in
37% of his cases. The so-called benign paroxysmal
positioning nystagmus almost never occurred in our
Menieres patients. Considering the results in the
caloric test (Table 111) reduced excitability on the
 254 C.T. Haid et al.
Table IX. Conclusions of yutients suflering from Me-
nieres disease (538 cases)
History (vertigo attacks, fluctuating hearing loss, tinnitus.
fullness in the ear)
Combination of internal diseases
(e.g. hypotonia, hypertonia, hypolipidemia, diabetes melli-
tus)
Affection of paranasal sinuses possible (trigger factor)
Vestibular test often of limited value
(most of the examinations during interval stage)
Telemetric-ENG of important value
(chance to objectify the irrigation stage)
Audiology of important value
(pure tone threshold, electrocochleography, glycerol test)
Considerations of treatment
(drugs, diet, surgery)
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diseased side could be established in only 28% of our
cases (24% unilateral and 4% bilateral). Even Wada
et al. ( 18) could verify the same percentage of hypo-
function. Stahle & Bergman (20) and Meyerhoff et al.
(15) could show a reduced caloric function in 65%1,
Pfaltz & Matefi (8) in 62% and Oosterveld in 48%
(21). Only few patients with Menieres disease have a
For personal use only.
complete loss of caloric response on the affected side.
In some cases hyperreflexia on the diseased side oc-
curred in our patients.
Most of our patients showed a flat pure tone
threshold curve (Table VI) on the affected side (om-
nifrequent sensorineural hearing loss). This could also
be confirmed by other authors (22, 23).
A low frequency hearing loss seems to be more
common in the early stage of Menieres disease (8,
17). Total deafness occurred rarely on the diseased
side. Menieres triad consists of typical vertigo at-
tacks in combination with synchroneous fluctuating
hearing loss, tinnitus and even fullness in the diseased
ear. The supraliminal audiometric test always re-
vealed a cochlear lesion. With the aid of electrococh-
leography (24) and the glycerol test (25) the diagnosis
of Menieres disease can be substantiated.
Interestingly, many of our patients with Menieres
disease suffered from internal diseases (Table VII),
allergy o r an affection of the paranasal sinuses (Table
VIII), which may constitute co-factors triggering Me-
nieres disease. From our point of view it is very
important to consider these diseases for the treatment
(Table IX).
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 Clinical survev of Menieres Diseuse 255

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Address for correspondence:
C.T. Haid
ENT-Department and Skull Base Center
Euro-Med-Clinic
D-90763 Fuerth
Germany