menieres disesse
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NOT NEUROLOGY FOR NEUROLOGISTSPract Neurol 2009; 9: 157–162
Meniere’sdiseaseMohamed A Hamid
Meniere’s disease affects about 1% of the population. Typically it presents asepisodic vertigo, fluctuating hearing loss, tinnitus and aural fullness. Thehistory and physical examination are critical in making the diagnosis.Laboratory tests are useful in tailoring and following up patients. Treatmentoptions are limited and usually targeted towards reducing endolymphatichydrops to stabilise the hearing loss and reduce the vertiginous episodes.
Although there are several different
disorders of the inner ear, and many
are very common, they all present
with hearing loss, vertigo or both.
The prevalence of hearing loss in industrial
populations is about 10% and of vestibular
and balance disorders about 20–30%. The
common peripheral vestibular disorders
amenable to medical and rehabilitative treat-
ments are Meniere’s disease, vestibular neu-
ronitis, vestibular migraine, benign
paroxysmal positional vertigo (BPPV), motion
intolerance and visual induced vertigo
(motion sickness), and age-related balance
deterioration (presbystasis). Here I will focus
on the aetiology, histopathophysiology, clin-
ical presentation and management of
Meniere’s disease which is a common otolo-
gical problem characterised by episodic
vertiginous spells, hearing loss, tinnitus and
aural fullness (there is a rather arcane debate
whether these symptoms should be grouped
under Meniere’s disease or Meniere’s syn-
drome).
EPIDEMIOLOGYThe prevalence of Meniere’s disease is about
1% and it is more common in industrialised
countries and in adult white populations.1
There is a slight female preponderance and
the typical age of onset is 30–50 years.
Bilateral disease is not uncommon (15–20%),
but both sides are seldom affected simulta-
neously except in patients with autoimmune
disease.1–3 Genetic predisposition has been
reported in families with Meniere’s disease.4, 5
Factors such as diet,6 weather changes (low
pressure fronts),7 emotional and physical
stress,8 and urban living can precipitate
attacks and make any symptoms worse.
AETIOLOGY ANDHISTOPATHOLOGYProsper Meniere was the first to realise that
vertigo and hearing loss could be attributable
to a disorder of the inner ear as opposed to
‘‘brain congestion’’ which was the accepted
cause prior to Meniere’s published case
report.9 Seventy seven years later, the first
M A HamidClinical Professor of
Otolaryngology and Audiology,
Founder and Director, The
Cleveland Hearing & Balance
Center, 29001 Cedar Road,
Cleveland, OH 44122, USA;
hamidma@clevelandhearingbalance.
com
157Hamid
www.practical-neurology.com
histopathological description of ‘‘endolym-
phatic hydrops’’ (fig 1) was simultaneously
and independently provided by Yamakawa,10
and Hallpike and Cairns.11 Since then the
histological entity of ‘‘endolymphatic
hydrops’’ has been associated with Meniere’s
disease, although not all patients with the
histopathology have the typical symp-
toms.12, 13 The conventional view is that
hydrops is caused by either increased produc-
tion of endolymph by the stria vascularis, or
decreased absorption by the endolymphatic
sac. Recently, however, it has been suggested
that hydrops is the result of dysfunction of
the spiral ligament fibrocytes, which inter-
feres with the recycling of K+ ions and results
in osmotic imbalance and expansion of the
endolymphatic compartment.14 Many factors
such as genetic abnormalities, infections,
allergies, autoimmune disorders, dietary,
endocrine and vascular problems could be
responsible for the development of hydrops
but the exact underlying cause is unclear.
NATURAL HISTORYThe natural history of Meniere’s disease is
poorly understood. Few studies1, 2, 15–19 have
had adequate length of follow-up (14 years19
and 21 years1) and sufficient numbers of
patients (53,16 11919 and 1612) to allow
reasonable conclusions about the natural
history of the syndrome. These studies also
underscore the difficulty in making the initial
diagnosis if the presenting symptom is just
hearing loss, tinnitus or vertigo, but not all of
the symptoms which make up the typical
presentation of ‘‘Meniere’s disease’’. What we
do know is:
N Hearing deteriorates over time to about50 db loss in 60–80% of patients.
N Speech scores (understanding/clarity)drop with time to below 50% which isthe minimal level at which a hearingdevice can be used.
N Audiometry (pure tone audiograms) showvariable configurations such as lowfrequency rising or high frequency fallingpatterns.
N Vertiginous episodes vary between 6 and11 per year (average 4),1 and decline withtime.
Vestibular testing (mostly electronystag-
mography) shows progressive decline of the
caloric response, relative to the normal ear,
over five years, and various types of sponta-
neous and positional nystagmus during or
between episodes.
The treatments used in the studies of
prognosis did not appear to alter the natural
history of the disease. An often quoted paper
in otology circles which reviewed treatment
results over 25 years15 reported that most
studies described ‘‘improvement’’ in 20–30%,
‘‘failure’’ in 10–25% and ‘‘recovery ’’ in 60%.
Recovery meant reduction of vertiginous
episodes, and this was independent of any
treatment in most studies. However, this
report, and other studies, did not address
hearing recovery (it is important to note that
most studies of Meniere’s disease have
focused on vertigo despite the fact that the
final disability is hearing loss1, 20).
CLINICAL PRESENTATION ANDDIAGNOSISThe typical Meniere’s patient is a middle aged
adult presenting with rotational vertigo (with
nausea and vomiting) lasting 30 minutes to
hours, unilateral fluctuating hearing loss and
tinnitus. The hearing loss frequently affects
the low frequencies, especially early in the
disease. Tinnitus is often described as a harsh
Figure 1Cochlear hydrops: the endolymphatic
space (EL) volume increases and
compresses Reissner’s membrane (RM)
so reducing the vestibular
perilymphatic space (PL) which
ultimately leads to the aural pressure
and vertiginous episodes of Meniere’s
disease.
Not all patientswith thehistopathologyhave the typicalsymptoms
158 Practical Neurology
10.1136/jnnp.2009.176602
roaring machine-like sound, especially during
vertigo attacks. Patients also may have
hyperacusis (hypersensitivity to sounds) and
duplacusis (distortion of sound) in the
affected ear. In atypical forms, the initial
symptom can be tinnitus or hearing loss. The
time between the initial symptom and the
subsequent typical constellation of symptoms
varies from months to years. Therefore, in
taking the history, the physician should focus
carefully on the patient’s symptoms, any
otological history (for example, ear disease or
surgery), the general medical history, allergies
and medications in order to tailor appropriate
laboratory tests and narrow the differential
diagnosis (see below).
The diagnosis of Meniere’s disease is
mainly clinical, based on the history and
audiometry. Therefore, it is important that
clinicians use standardised diagnostic criteria.
Laboratory tests are obtained to support the
diagnosis and to tailor and monitor treat-
ments. Guidelines have been proposed by the
American Academy of Otolaryngology - Head
and Neck Surgery (AAO-HNS) for the diag-
nosis as possible, probable, definite and
certain according to the criteria shown in
the box.21 However, physicians continue to
use the terms cochlear hydrops, when vertigo
is absent, and vestibular hydrops for cases
without hearing loss, which are considered
variants of the disease. This is important,
especially in the differential diagnosis. For
example, patients with only cochlear symptoms
may have genetic or immune mediated hearing
loss while patients with only vestibular symp-
toms may have vestibular neuronitis, BPPV or
vestibular migraine. Other atypical presenta-
tions include the otolith crisis of Tumarkin
(drop attacks usually of late Meniere’s disease
with sudden falls to one side with no warning
at all, and not necessarily in the middle of a
typical Meniere’s episode) and the Lermoyez
phenomenon (improvement of hearing after
vertigo episodes).
EXAMINATIONPatients presenting with dizziness or vertigo
should have a general medical, neurotological
and neurological examination, including using
either Frenzel’s glasses or video goggles to
pick up eye movement abnormalities that
would otherwise be missed on routine
examination.22 The important components of
the neurotological examination are the ear
itself, neck movements, eye movements,
vestibular responses (vestibulo-ocular (VOR)
and vestibulo-spinal (VSR)), cranial nerves,
motor and sensory signs, and cerebellum.
N Ear examination is generally normal withno evidence of middle ear effusion orvascular lesions.
N Hearing loss and hyperacusis are oftenpresent in Meniere’s ears, especiallyduring, or around, the time of an attack.
N Neck movements should be normalexcept in the acute or subacute stagesof a Meniere’s episode when patientsself-restrict movements to reduce dizzi-ness or vertigo.
N Saccadic, pursuit and optokinetic eyemovements are usually normal with nodysconjugate movements or gaze-evokednystagmus.
N Spontaneous nystagmus observed afterremoving visual fixation by Frenzel glassor video goggles, post head shakenystagmus and positional nystagmusare all frequently present in the acuteor subacute stages of an episode. Most, ifnot all, types of central nystagmus are
Classification of Meniere’s disease based on the AAO-HNScriteria
Certain Meniere’s diseasel Definite Meniere’s disease, plus histopathological confirmation
Definite Meniere’s diseasel Two or more definite spontaneous episodes of rotational vertigo for
20 minutes or longerl Audiometrically documented hearing loss (uni- or bilateral) on at least one
occasionl Tinnitus or aural fullness in the affected earl Other causes excluded such as vestibular schwannoma
Probable Meniere’s diseasel One definite episode of rotational vertigol Audiometrically documented hearing loss (uni- or bilateral) on at least one
occasionl Tinnitus or aural fullness in the affected earl Other causes excluded
Possible Meniere’s diseasel Episodic vertigo of the Meniere’s type without documented hearing loss, orl Sensorineural hearing loss (uni- or bilateral), fluctuating or fixed, with
disequilibrium but without definite episodes of vertigol Other causes excluded
159Hamid
www.practical-neurology.com
enhanced with visual fixation and there-fore can be readily seen with the nakedeye. Frenzel’s glasses or video-electro-nystagmography remove visual fixationand enhance the detection of peripheralnystagmus which is commonly sup-pressed by visual fixation.
N The vestibulo-spinal response is examinedby asking patients to stand up unassistedand walk for several steps watching forany gross gait abnormalities or sidestepping (abnormalities are more evidentduring an acute episode and less soduring subacute and inactive stages ofthe disease).
N Romberg’s sign is conducted with eyesopen and eyes closed with the armsoutstretched and observing postural swayand post stepping (Unterberger-Fukuda)rotation with eyes closed.
N The vestibulo-cerebellar examination isconducted by observing the vestibulo-ocular visual suppression response.
N The remainder of the neurological exam-ination is usually normal in Meniere’spatients. Positive neurological findings onexamination should direct the physician’sattention to the presence of centralnervous system disease.
DIFFERENTIAL DIAGNOSIS
N Migraine vertigo is more common thanMeniere’s and often poses a diagnosticchallenge.23 Of course, significant hearingloss or fluctuation are common inMeniere’s but nonetheless migraine maybe associated with mild low frequencyunilateral or bilateral hearing loss.However, migraine vertigo is oftendescribed as a chronic non-paroxysmalmotion sensitivity, lightheadedness ordisequilibrium rather than the rotatoryvertigo experienced in Meniere’s. Previoushistory and family history of migraine,associated nausea, photosensitivity, sen-sitivity to sounds and visual changessuggest migraine.
N Retrocochlear lesion and disorders of theotic capsule should be ruled out withimaging when clinically indicated. MRI isthe test of choice for vestibular schwan-nomas and demyelination plaques, andCT for cochlear otosclerosis and superiorcanal dehiscence.
N Bilateral Meniere’s disease can be idio-pathic or associated with syphilis orautoimmune diseases such as systemiclupus erythematosus and rheumatoidarthritis. Appropriate laboratory work upfor such disorders should be consideredalthough their yield is usually low.
DIAGNOSTIC TESTSIn the course of evaluating patients with
hearing and balance disorders, the tests that
are commonly considered include audiometry,
vestibular tests, blood tests and imaging (CT
of the temporal bone and MRI of the brain).
These need to be tailored according to the
history and physical findings.
Figure 2Early and late audiometric profile of
Meniere’s disease. In the early stage, a
low frequency rising pattern is evident
in the right ear (red circles). The peak
pattern (red dots) is evident later in the
disease. Also note that the left ear
exhibits a peak pattern (black X) in the
late stage suggesting bilateral rather
than just unilateral disease.
Figure 3(A) Audiogram of a patient presenting
with right hearing loss and episodic
vertigo. (B) Although the clinical history
was in favour of Meniere’s disease, the
MRI (T2-weighted) showed a well
defined demeyelinating plaque (arrow)
in the right cochlear/vestibular nuclei
of the brainstem.
160 Practical Neurology
10.1136/jnnp.2009.176602
In Meniere’s patients the most valuable test
is audiometry to document the type and
degree of hearing loss. Pure tone and speech
audiometry should be obtained in all patients.
Typically it shows a low frequency rising
pattern in the early stage of the disease but
patients can also present with peak or high
frequency profiles (fig 2). If asymmetrical
hearing loss is present, a contrast MR scan of
the brain and internal auditory canals is
needed to rule out a vestibular schwannoma
or brain stem lesion such as a demyelinating
plaque (fig 3).
Other tests such as electrocochleography
and electro- or video-nystagmography are
not usually needed in the early phase of
management. Electronystagmography should
however be considered in patients undergoing
intratympanic gentamicin treatment, labyr-
inthectomy or vestibular nerve section to rule
out subclinical disease (vestibular weakness)
in the contralateral inner ear.
Laboratory tests are seldom needed.
However, thyroid function, syphilis serology,
autoimmune and routine chemistry should be
done in patients presenting with bilateral
Meniere’s.
MANAGEMENTMost Meniere’s patients can be managed as
an outpatient; however, elderly dehydrated
patients may need to be admitted.
The most distressing symptom of an acute
attack is the vertigo which should be treated
symptomatically; 5 mg diazepam (Valium)
with 2 mg glycopyrolate (Robinul) is very
effective. Sublingual administration of 2 mg
lorazepam (Ativan) is also very effective in
controlling and shortening the duration of an
attack.
The long-term treatments for Meniere’s
disease vary considerably worldwide, the main
aim being to reduce the frequency of the
vertiginous episodes and the development of
increasing hearing loss:24, 25
N Diuretics and a low-salt diet (if patientsare salt sensitive based on their diaries)may decrease the natural progression ofsensorineural hearing loss.2, 25
N Systemic and intratympanic corticoster-oids have the potential for controllingvertigo and improve hearing, and offer anew treatment leading to significant
speech discrimination recovery, allowingfor hearing aid use in ears that wouldotherwise have become deaf.26
N The prevalence of allergy appears to behigher in Meniere’s patients than in thegeneral population, or the population ofpatients visiting an otological clinic forother symptoms; allergy therapy mayimprove vertigo and tinnitus.27
N Betahistine (B-Serc) is commonly used inEurope, Canada and many other coun-tries.24 It is believed that it exerts its effectby increasing the blood circulation to theinner ear and it also acts as a pre-synaptic agonist to histamine receptors. Arecent meta-analysis28 showed its efficacyin treating non- Meniere’s vertigo.
N Other treatments include pressure equal-ising tubes, pressure machines, laser,acupressure, acupuncture, hydrotherapy,oxygen therapy and herbal medicationsbut there are no randomised trials.
N Inner ear gentamicin perfusion is effec-tive in controlling vertigo and Tumarkindrop attacks.29–31
N A recent study comparing vestibularnerve section to intratympanic gentami-cin treatment concluded that both inter-ventions are acceptable for Meniere’sdisease patients; however, gentamicincaused more hearing loss, and vestibularnerve section controlled vertigo better.32
Meniere’s disease can be very disabling,
physically and emotionally, to patients and
their families. It is critical to address the
emotional impact of the disease and aggres-
sively treat secondary anxiety and depression.
It is also important to assess the impact of
the disease on the functional capacity of the
patient at home and work. Certain jobs can be
PRACTICE POINTS
l Meniere’s disease is an infrequent cause of hearing loss, tinnitus andepisodic vertigo.
l The cause is unknown, it is probably multifactorial, and the final commonhistopathology is endolymphatic hydrops of the stria vascularis.
l Meniere’s disease and vestibular migraine often overlap and must bedifferentiated for proper management.
l Meniere’s is a lifelong disease with good prognosis with early detectionand targeted treatments. It has significant morbidity if not adequatelytreated.
l In resistant cases, emotional and physical disabilities are prominent andmust be aggressively managed.
161Hamid
www.practical-neurology.com
hazardous to Meniere’s patients such as flying,
policing and firefighting, construction jobs,
working with heavy machinery, and most jobs
requiring critical control of balance. Physicians
should have low thresholds for approving
permanent or temporary disability to a
Meniere’s patient, particularly bilateral cases.
ACKNOWLEDGEMENTSI am grateful to Dr Marc Friedman,
Neurotology Consultant, for his invaluable
review of this manuscript.
REFERENCES1. Friberg U, Stahle J, Svedberg A. The natural course
of Meniere’s disease. Acta Otolaryngol Suppl1984;406:72–7.
2. Stahle J, Friberg U, Svedberg A. Long-term
progression of Meniere’s disease. Acta OtolaryngolSuppl 1991;485:78–83.
3. Hughes GB, Barna BP, Kinney SE, et al. Autoimmune
endolymphatic hydrops: five-year review.
Otolaryngol Head Neck Surg 1988;98:221–5.
4. Frykholm C, Larsen HC, Dahl N, et al. Familial
Meniere’s disease in five generations. Otol Neurotol2006;27:681–6.
5. Klar J, Frykholm C, Friberg U, et al. A Meniere’s
disease gene linked to chromosome 12p12.3.
Am J Med Genet B Neuropsychiatr Genet2006;141B:463–7.
6. Stephens D, Kentala E, Varpa K, et al. Positive
experiences associated with Meniere’s disorder.
Otol Neurotol 2007;28:982–7.
7. Watanabe Y, Mizukoshi K, Shojaku H, et al.Epidemiological and clinical characteristics of
Meniere’s disease in Japan. Acta Otolaryngol Suppl1995;519:206–10.
8. Hinchcliffe R. Emotion as a precipitating factor in
Meniere’s disease. J Laryngol Otol 1967;81:471–5.
9. Meniere’s P. Pathologie auricularie: memoire sur
des lesions de l’oreille interne donnant lieu a des
symptomes de congestion cerebrate
apoplectiforme. [abstract]. Gaz Med Paris1861;16:597–601.
10. Yamakawa K. Uber die pathologische Veranderung
bei einem Meniere-Kranken [abstract].
L Otorhinolaryngol Spc Jpn 1938;44:2310–12.
11. Hallpike CS, Cairns H. Observations on the
pathology of Meniere’s syndrome [abstract].
J Laryngol Otol 1938;53:625–55.
12. Schuknecht HF, Gulya AJ. Endolymphatic hydrops.
An overview and classification. Ann Otol RhinolLaryngol Suppl 1983;106:1–20.
13. Rauch SD, Merchant SN, Thedinger BA. Meniere’s
syndrome and endolymphatic hydrops. Double-
blind temporal bone study. Ann Otol RhinolLaryngol 1989;98:873–83.
14. Nin F, Hibino H, Doi K, et al. The endocochlear
potential depends on two K+ diffusion potentials
and an electrical barrier in the stria vascularis of
the inner ear. Proc Natl Acad Sci USA2008;105:1751–6.
15. Torok N. Old and new in Meniere disease.
Laryngoscope 1977;87:1870–7.
16. Schmidt PH, Brunsting RC, Antvelink JB. Meniere’s
disease: etiology and natural history. ActaOtolaryngol 1979;87:410–12.
17. Charachon R, Gratacap B, Barthez M, et al. [Natural
history of Meniere’s disease concerning 92 cases
from 1971 to 1980]. Rev Laryngol Otol Rhinol(Bord) 1989;110:453–6.
18. Silverstein H, Smouha E, Jones R. Natural history vs.
surgery for Meniere’s disease. Otolaryngol HeadNeck Surg 1989;100:6–16.
19. Green JD Jr, Blum DJ, Harner SG. Longitudinal
follow up of patients with Meniere’s disease.
Otolaryngol Head Neck Surg 1991;104:783–8.
20. Hamid M, Trune D. Issues, indications, and
controversies regarding intratympanic steroid
perfusion. Curr Opin Otolaryngol Head Neck Surg2008;16:434–40.
21. Monsell EM, Balkany TA, Gates GA, et al. Committee
on Hearing and Equilibrium guidelines for the
diagnosis and evaluation of therapy in Meniere’s
disease [abstract]. Otolaryngol Head Neck Surg1995;113:181–5
22. Hamid M, Sismanis A. Clinical Approach to Patients
with Auditory and Vestibular Disorders. In: Hamid
M, Sismanis A, eds. Medical otology andneurotology. A clinical guide to auditory andvestibular disorders. New York: Thieme, 2006:43–
63.
23. Neuhauser HK, Lempert T. Diagnostic criteria for
migrainous vertigo. Acta Otolaryngol2005;125:1247–8.
24. Smith WK, Sankar V, Pfleiderer AG. A national
survey amongst UK otolaryngologists regarding the
treatment of Meniere’s disease. J Laryngol Otol2005;119:102–5.
25. Hamid M. Recent advances in medical otology-
neurotology. Mediterr J Otol 2005:145–53.
26. Trune DR, Kempton JB, Gross ND. Mineralocorticoid
receptor mediates glucocorticoid treatment effects
in the autoimmune mouse ear. Hear Res2006;212:22–32.
27. Keles E, Godekmerdan A, Kalidag T, et al. Meniere’s
disease and allergy: allergens and cytokines.
J Laryngol Otol 2004;118:688–93.
28. Della PC, Guidetti G, Eandi M. Betahistine in the
treatment of vertiginous syndromes: a meta-
analysis. Acta Otorhinolaryngol Ital 2006;26:208–
15.
29. Bodmer D, Morong S, Stewart C, et al. Long-term
vertigo control in patients after intratympanic
gentamicin instillation for Meniere’s disease. OtolNeurotol 2007;28:1140–4.
30. Silverstein H, Lewis WB, Jackson LE, et al. Changing
trends in the surgical treatment of Meniere’s
disease: results of a 10-year survey. Ear NoseThroat J 2003;82:185–4.
31. Chia SH, Gamst AC, Anderson JP, et al.Intratympanic gentamicin therapy for Meniere’s
disease: a meta-analysis. Otol Neurotol2004;25:544–52.
32. Hillman TA, Chen DA, Arriaga MA. Vestibular nerve
section versus intratympanic gentamicin for
Meniere’s disease. Laryngoscope 2004;114:216–22.
162 Practical Neurology
10.1136/jnnp.2009.176602
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