1. health and fitness
2. disease
3. headaches and migraines

Meniere’s Disease or
Migraine Associated Dizziness
That is the Question?
Thomas G. Brammeier, M.D., F.A.C.S.
Hearing & Balance Center
Clinical Case
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42 year old female
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Complaint of dizziness for 6 months:
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Last attack was 72 hours ago.
Lightheadedness, and imbalance for years, worse with head
movement, or riding in the car or on a boat.
Five attacks of severe vertigo lasting 4-5 hours in durations which
cause nausea and vomiting.
Not sure if the hearing changed during the vertigo attack since she was
vomiting and very sick.
Intermittent tinnitus that fluctuates in pitch and loudness in both ears.
She felt a humming sensation in the left ear prior to the attack and
phonophobia.
Severe headache prior to the first attack and a mild headache after the
last attack.
Headaches are intermittent with severe pain behind the left eye and
side of head. History of migraines for the past 15 years. She has 2-3
migraine headaches per year with photophobia and phonophobia.
Clinical Case
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Past medical history
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Medication
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Excedrin for migraines.
Family history
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Unremarkable.
Mother, sister, aunt, and brother all have migraines.
Father has hearing loss and vertigo.
Physical Examination
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Neurological exam is normal.
ENT exam is normal except head-shaking test demonstrates
a right beating nystagmus.
Clinical Case
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Audiogram
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ENG
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Left low frequency sensorineural hearing
loss at 250, and 500 Hertz at 30 decibels
18% left unilateral weakness
Abnormal saccades and tracking
What is your diagnosis?
History of Meniere disease
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In 1861, before the Paris Academy of
Medicine, Prosper Meniere first described a
group of patients with symptoms of vertigo,
hearing loss, nausea, and vomiting.
He pointed out that the symptoms come from
the inner ear and not the brain
(apoplectiform cerebral congestion).
Meniere’s triad: 1. Tinnitus, 2. Fluctuating
hearing loss, 3. Repeated attacks of vertigo
Prosper Meniere, in his original article, noted
an association to migraines.
Meniere P. Pathologie auriculaire: memoirs sur une lesion de
de l’oreille interne donnant lieu a des symptoms de
congestion cerebrale apoplectiforme. Gaz Med Paris 1861;16:
597-601.
History of migraines
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Headaches first described as early as
3000 B.C.
Migraines first described as early as
100 A.D.
Recently, increasing reports in the
literature of association of migraines
and vertigo.
Diagnostic dilemma
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Vertigo, dizziness, light-headedness, disequilibrium
are common symptoms.
Headaches and migraines are common symptoms.
There are no specific diagnostic tests for migraines or
Meniere’s disease.
Diagnosis for Meniere’s disease and migraines is
made mostly on medical history.
Migraines associated dizziness (MAD) patients may
have dizziness independent of migraine headaches.
There is a high prevalence of migraine headaches
with patients suffering from Meniere’s disease.1
1. Radthke A. et al, Migraine and Meniere’s disease: Is
there a link? Neurology 2002 59:1700-1704.
Meniere’s disease
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A clinical disorder defined as the idiopathic syndrome of
endolyphatic hydrops. A disease is a pathophysiologic
state.1
Etiology is unknown.
Symptoms of early stage Meniere’s disease are episodic
rotational vertigo, transient low frequency sensorineural
hearing loss, low pitch tinnitus, and aural fullness.
Symptoms of late stage Meniere’s disease are persistent
disequilibrium, permanent sensorineural hearing loss,
permanent low pitch tinnitus and aural fullness.
Usually affects one ear.
1. 1995 AAO-HNS Committee on Hearing and Equilibrium Guidelines for
the Diagnosis and Evaluation of Therapy in Meniere’s disease
Meniere’s disease
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NIH estimates 545,000 people in the
U.S. and that 38,250 are diagnosed
each year.1
No gender preponderance.
Age: widely variable with a peak age
range around 40-60 year age group.
Affect only one ear 70-80%.
Familial occurrence: around 10-20%.
1. Haybach, P. Meniere’s Disease What you need to Know,
2002
1995 Guidelines Meniere’s disease
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Possible Meniere’s disease
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Probable Meniere’s disease
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One definitive episode of vertigo, audiometrically documented
hearing loss on at least one occasion, tinnitus or aural fullness in
the treated ear, and other causes excluded.
Definite Meniere’s disease
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Episodic vertigo without documented hearing loss, or sensorineural
hearing loss, fluctuating or fixed, with disequilibrium but without
definitive episodes, and other causes excluded.
Two or more definitive spontaneous episodes of vertigo 20 minutes
or longer, audiometrically documented hearing loss on at least one
occasion, tinnitus or aural fullness in the treated ear, and other
causes excluded.
Certain Meniere’s disease
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Definite Meniere’s disease, plus histopathologic confirmation.
1995 AAO-HNS Committee on Hearing and Equilibrium Guidelines for
the Diagnosis and Evaluation of Therapy in Meniere’s disease.
Meniere’s disease
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Primary types
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Classic Meniere’s disease
Cochlear Meniere’s disease
Vestibular Meniere’s disease
Crisis of Tumarkin
Secondary types
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Infectious:
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Trauma:
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Viral, bacterial, and syphilitic
Closed head injury, noised induce-hearing loss
Autoimmune:
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Allergy, autoimmune inner ear disease, Cogan’s syndrome
Migraines
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A primary headache disorder that is intermittent,
varies in pain intensity, duration, pattern associated
features, and frequency of occurrence.
Etiology is uncertain.
Approximately 18-25% of the general population.1
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Migraine in men 4.0-6.5%.2
Migraine in women 11.2-18.2%.2
Onset in the first and second decade of life.
Peak prevalence occurred in third and fourth decade
of life.
Declines slowly with aging.
1. Radthke A. et al, Migraine and Meniere’s disease:
Is there a link? Neurology 2002; 59:1700-1704.
2. Henry P.,et.alPrevalence and clinical characteristics of
migraines in France. Neurology 2000; 59:232-237.
Migraines
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High incidence of family history of migraines.
Migraines without aura is a multi-factorial disorder, caused by
combination of genetic and environmental factors.
Migraine attacks are associated with a large number of hereditary
diseases which may help in chromosomal linkage studies.
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Familial hemiplegic migraine
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Usher syndrome, Childhood epilepsy, Hereditary essential tremor,
Tourette’s syndrome, Hereditary hemorrhagic telangiectasia
Rare migraine that causes cerebellar ataxia and nystagmus.
Chromosome 19p13.
Mutation in the CACNA1A gene coding for the alpha 1A subunit of
neuronal calcium channel, which is heavily expressed in the cerebellum.2
Benign paroxysmal vertigo of childhood
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Strong family history of migraines.
1. Neuhauser H., Vertigo and dizziness related to migraine: a
diagnostic challenge Cephalalgia 2004; 24:83-91.
IHS 2004 Classification of Migraines
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Migraine without aura
Migraine with aura:
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Typical aura with migraine headache
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Typical aura with non-migraine headaches
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Familial hemiplegic migraine
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Sporadic hemiplegic migraine
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Basilar-type migraine
Childhood periodic syndromes that are commonly precursors of migraine:
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Cyclical vomiting
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Abdominal migraine
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Benign paroxysmal vertigo of childhood
Retinal migraine
Complications of migraine:
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Chronic migraine
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Status migrainosus
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Persistent aura without infarction
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Migrainous infarction
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Migraine-triggered seizure
Probable migraine:
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Probable migraine without aura
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Probable migraine with aura
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Probable chronic migraine
IHS 2004 Classification of Migraines
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IHS classification of migraine headaches requires five attacks which fulfill the following
criteria:
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Headaches lasting between 4-72 hours
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Headaches having at least two of the following four features:
 Unilateral
 Pulsating quality
 Moderate or severe intensity
 Aggravated with physical activity
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Headaches associated with nausea, vomiting, photophobia, or phonophobia.
IHS classification of migraines with aura is based on aura symptoms and not headache
with the following criteria:
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One or more fully reversible aura symptoms, indicating focal cerebrocortical or brainstem dysfunction.
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At least one of the aura symptoms develops gradually for over more than four
minutes, or two or more aura symptoms occur in succession.
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Aura lasts no longer than 60 minutes.
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Headaches usually follow the aura symptoms with a symptom-free interval of less
than 60 minutes. Headaches may also begin before or simultaneously with the aura.
Migraines and Vertigo
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Benign paroxysmal vertigo of childhood:
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Vertigo in very young children ages 3-5.
No symptoms of headaches.
Basilar-type migraine:
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Posterior fossa symptoms of vertigo, ataxia, dysarthria, and
tinnitus along with visual phenomena consistent with
ischemia in the distribution of the posterior cerebral arteries.
Vertigo lasting 5 to 60 minutes.
Headaches range from mild to severe. Occur in the occipital
region. In children the headaches can occur any region of
the head
Migraine in Meniere’s disease
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Radtke Study
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Higher lifetime prevalence of migraine in patients
with Meniere’s disease (56%) as compared to age
and sex matched controls (25%).
Migraine and Meniere’s attacks occurred at same
time in 28% of patients.
45% of patients experienced at least one migraine
symptom with vertigo.
Study suggests common etiology and
pathophysiology between Migraines and Meniere’s
disease.
Radtke, Lempert, et al. Migraine and Meniere’s disease: Is
there a link? Neurology 2002; 59:1700-1704
Migraines in Meniere’s disease
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Kayan and Hood Study
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Identified vestibular symptoms in 54.5% of
patients with migraines as opposed to 30.2% of
patients with tension-type headaches.1
Vertigo was present in 26.5% of migraine patients
and 7.8% of tension type headache patients.1
Bayazit Study
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Reported 30% dizziness and 25% vertigo in
migraine patients.2
1. Kayan A, Hood JD, Neuro-otological manifestion of
Migraines. Brain 1984:107;1123-1142.
2. Bayazit Y, et al. Assessment of migraine-related cochleovestibular
symptoms. Rev Laryngol Otol Rhinol (Bord) 2001; 122:85-88.
Migraine associated dizziness
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Clinical Manifestation
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Occurs with a peak around the third and fourth decades.
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Vestibular manifestation can range from light-headiness to severe
vertigo.
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Vertigo last few seconds to several hours.
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Patients are easily subject to motion sensitivity and movement
disequilibrium outside of migraine attacks.
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May be associated with mild and diffuse headaches to severe unilateral
headaches.
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Headaches do not always occur with vestibular symptoms.
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Aura does not always occur with vestibular symptoms.
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Aura such as photophobia and phonophobia may be associated with
dizziness.
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Fluctuating low frequency sensorineural hearing loss is more common
with Meniere’s disease.
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Tinnitus is usually present which can be unilateral or bilateral.
Migraine associated dizziness
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Not included in the International
Headache Society or American Academy
of Otolaryngology classification.
Neuhauser proposed criteria for
migrainous vertigo
Neuhauser Criteria
Definite migrainous vertigo:
A.
Recurrent episodic vestibular symptoms of at least
moderate severity.
B.
Current or previous history of migraine according to
the criteria of the International Headache Society.
C.
One of the following migrainous symptoms during
at least two vertiginous attacks: migraine
headache, photophobia, phonophobia, visual or
other auras.
D.
Other causes ruled out by appropriate
investigations.
Neuhauser Criteria
Probable migrainous vertigo:
A.
Recurrent episodic vestibular symptoms of at least moderate
severity.
B.
One of the following:
1.
Current or previous history of migraine according to the
criteria of the International Headache Society.
2.
Migrainous symptoms during greater than 2 attacks of
vertigo
3.
Migraine-precipitants before vertigo in more than 50% of
attacks: food triggers, sleep irregularities, hormonal
changes.
4.
Response to migraine medication in more than 50% of
attacks.
C.
Other causes ruled out by appropriate investigations.
Audiometric testing
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Battista Study
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Compared audiometrics with Meniere’s disease
and Migraine associated dizziness patients.
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Migraine associated dizziness rarely demonstrates
progressive sensorineural hearing loss.
80% of basilar migraine patients will demonstrate
fluctuating low frequency sensorineural hearing loss
similar to Meniere’s disease patients.
Meniere disease will over time demonstrate fluctuating
and progressive sensorieneural hearing loss.
Battista R. Audiometric findings of patients with migraineassociated dizziness. Otology & Neurotology 2004; 25:987-992.
Vestibular testing
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Cass Study
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Compared vestibular testing with Meniere’s disease and
Migraine associated dizziness patients.
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Spontaneous and positional nystagmus are not diagnostic for
Meniere’s disease or migraine associated dizziness.
Ocular motor abnormalities with pursuit tracking or optokinetic
testing abnormalities have slightly increased prevalence in
migraine associated dizziness. This indicates central vestibular
involvement with the cerebellum and brainstem.
Caloric testing demonstrates reduced vestibular response in
approximately 18-20% of migraine associated dizziness
patients. Findings for Meniere’s patients will vary depending on
the early or late stages of their disease.
Rotational chair asymmetry is the most frequent finding for
migraine associated dizziness.
Cass et al. Migraine-related vestibulopathy. Ann Otol
Rhinol Laryngol. 1997; 106:182-189.
Meniere’s disease
Pathophysiology
Endolymphatic hydrops
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There is distension of the membranous labyrinth due to excessive
endolymphatic fluid.
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Due to impaired resorption of endolymph in the endolymphatic duct and
sac.
Histopathologic evidence of reduced vascularization and fibrosis in the
perisaccular tissue which shows the reduction of resorptive capacity of the
endolymphatic sac. Additionally there has been accumulation of
proteinaceous debris in the lumen.1
Possible obstruction of the endolymphatic duct.2
Possible overproduction of endolymph by stria vascularis in the cochlea or
dark cells in the labyrinth.3
Reisner’s membrane
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Schuknecht discovered through histopathologic examination that there
was bowing and rupture of Reisner’s membrane in the pars inferior
portion of the inner ear.4
2. Franklin et al. Meniere’s symptoms resullting from
1. Fukuda et al., The development of
bilateral otosclerosis occlusion of the endolymphatic
endolymphatic hydrops following CMV
inoculation of the endolymphatic sac. 1988 duct: an analysis of the causual relationship between
otosclerosis and Meniere’s disease. 1990 J Otol.
Laryngoscope 98:439.
11(2):135.
3. Henriksson et al. Pathophysiology of
Meniere’s disease. 1986.Controversial aspects
Meniere’s disease. Georg. Thieme. Verlag.
4. Schuknecht, Pathology of the ear,
Boston, 1974 Harvard University
Migraine Pathophysiology
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Vascular theory (Wolff Hypothesis 1963)
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Initially there is vasoconstriction during the prodromal phase of migraine
followed by vasodilatation in the headache phase.
Neurotransmitter release
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Serotonin, histamine, catecholamine, and prostaglandins have been implicated
as part of the cause of migraines.
Medication such as calcium channel blockers may reduce migraine symptoms.
Autonomic nervous system
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Cerebral vessels are richly innervated with norepinephrine-containing neurons
whose cell bodies are located in the brainstem, superior cervical ganglia, the
locus coeruleus; these have connection to the hypothalamus.
Activation of alpha-adrenergic receptors results in vasoconstriction and
decreases cerebral blood flow, while beta-adrenergic receptor stimulation leads
to vasodilatation and increases cerebral blood flow.
Appenzeller postulated that migraine patients demonstrate abnormal vascular
responsiveness manifested as vasoconstriction, as a result of an abnormal
adrenergic drive , abnormal hypersensitivity of adreno-receptors, or some other
sympathetic system abnormalities.1
Medications such as beta-blockers may reduce migraine symptoms.
1. Appenzeller, et al., Clinical and experimental studies in
migraines. Res. Clin Stud. Headaache, 1978; 6:160-166.
Migraine Pathophysiology
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Vascular theory
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Prostaglandins produced by the cyclo-oxygenase
pathway
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Can cause vasoconstriction or vasodilatation associated
with aura and headache.
Increases the effects of bradykinin and other chemically
induced pain transmitters by sensitizing pain receptors.
Mediates inflammatory response
Modulates neurotransmitters release from nerve
terminals.
Medication such as NSAIDS may reduce symptoms of
migraines.
Migraine Pathophysiology
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Neurological theory
 Primary dysfunction of the brain that leads to secondary vascular abnormalities.
 Described by Leao: First a wave of short increased cortical or brainstem
neuronal activity followed by a wave of depression of the cortical neuronal
activity.
 Potassium ions and the neurotransmitter glutamate enhance neuronal
excitation, coupled with firing in a localized region of the cortex which results in
the accumulation of potassium ions in the extracellular space. The increased
extracellular potassium results in depolarization of the adjacent inactive neurons
concentration which plays a crucial role in creating and maintaining central
nervous system depression.
 The potassium ion concentration cannot be rebalanced by reuptake, diffusion or
transportation away from the active site by the glial potassium buffering system.
 Glutamate is released by the depolarization which also increases the intracellular
calcium during the spreading wave of depression.
 Neural theory suggests that the instability created by the ionic and
neurotransmitter disturbance causes the release of vasoactive amines,
serotonin, bradykinin, histamine and substance P that have a role in the
vasodilation.
Migraine Pathophysiology
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Intracranial vascular vessels, venous sinuses
and dura mater derive innervations from the
ophthalmic division of the trigeminal
ganglion.
Contributions also occur from upper cervical
dorsal roots.
Involves of the caudal brainstem and high
cervical spinal cord extending into the
thalamus.
Migraine and Meniere’s disease
A Common Pathophysiology
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Vestibulocortical system includes the vestibular nuclei, the
thalamus, and the cerebral cortex.
Vestibulothalamic tracts originate from neurons in the superior
and lateral vestibular nuclei and ascends to the thalamus
through a dominant anterior tract and smaller posterior tract.
Vestibulocortical tracts proceed to the parieto-insular vestibular
cortex.
Thalamic and cortical regions that involve the
vestibulothalamocortical tracts integrate proprioceptive,
vestibular, and visual stimulations to provide a conscious
awareness or “body oriented map” of orientation.
Neurotransmitters (calcitonin-gene related peptide, serotonin,
noradrenaline, dopamine) are know to modulate the activity of
vestibular neural pathways.
Migraine associated dizziness may be considered both a
peripheral vestibular and central nervous system disorder.
Migraine associated dizziness
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Treatment:
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Determine if patient has Meniere’s disease or
migraine associated dizziness.
Differentiation between Meniere’s disease and
migraine associated dizziness is important because
the treatments for each condition are different.
Follow-up with additional history, audiograms and
effectiveness of treatment is essential.
Meniere’s disease
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Medical treatment:
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Diet: 2000mg low sodium diet, caffeine reduction to 5075mg per day, alcohol and nicotine restriction.
Weather changes
Meniere’s log
Diuretics: Maxzide, HCTZ, Lasix, Diamox
Vasodilators: Histamine (Serc), Papaverine (Pavabid)
Calcium Channel Blocker: Verapamil
Steroids: Prednisone
Emergency vertigo kit
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Valium and Phenergan
Meniere’s disease
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Surgical treatment:
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Intratympanic gentamicin injections
Intratympanic dexamethasone infusions
Endolymphatic sac shunt
Vestibular nerve section
Labyrinthectomy
Migraine
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Diet:
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Avoid aged, canned, cured or processed meats, including bologna, game ham,
herring, hot dogs, pepperoni, and sausage, aged cheese, meat tenderizer,
monosodium glutamate, aspartame, chocolate, coffee, nuts, peanut butter, red
wine, avocados, beans, brewer’s yeast, raisins, pickles, figs, lentils, canned soups,
sauerkraut, seasoned salts, snow peas, red plums, papaya, pickled, preserved or
marinated foods i.e. olives and cultured dairy products i.e. buttermilk and sour
cream.
Weather changes, menstrual cycle, pregnancy, eating disorders fasting or
binges, sleep disorders
Migraine log
Symptomatic treatment:
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Over the counter medication (OTC) analgesics such as: acetaminophen, aspirin,
ibuprofen, and naproxen.
Antimetic: Phenergan
Sympathomimetic vasoconstrictor
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Isometheptene, dichloraphenazone, and acetaminophen (Midrin)
Aspirin, caffeine, and butalbital (Fiorinal)
Migraine
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Abortive treatment:
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Serotoninergic vasoconstrictors
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Cafergot
Dihydroergotamine
Triptans
Preventive treatment:
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Tricyclics: Amitriptyline, Doxepin, Nortriptyline
Beta blockers: Atenolol, Propanolol
Calcium channel blockers: Verapamil
Anticonvulsants: Tegretol, Neurontin, Topamax
Migraine associated dizziness
versus Meniere’s disease
Symptoms Meniere’s
Migraine (MAD)
Gender
equal
mostly females
Hearing loss
fluctuate, progressive,
low frequency
fluctuate, low frequency
Family Hx
10-20%
60-70%
Age onset
variable
teenage to 20 years
Headaches
variable
5 or more attacks
Episodic
always
always
Diet triggers
salt, caffeine, MSG
preservatives, MSG, red
wine, cheese, etc.
Vertigo
almost always
dizzy to vertigo
THE END
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