Meniere s Disease or Migraine Associated Dizziness That is the Question? Thomas G. Brammeier, M.D., F.A.C.S. Hearing & Balance Center

Transcription

1 Meniere s Disease or Migraine Associated Dizziness That is the Question? Thomas G. Brammeier, M.D., F.A.C.S. Hearing & Balance Center

2 Clinical Case 42 year old female Complaint of dizziness for 6 months: 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.

3 Clinical Case Past medical history Unremarkable. Medication Excedrin for migraines. Family history Mother, sister, aunt, and brother all have migraines. Father has hearing loss and vertigo. Physical Examination Neurological exam is normal. ENT exam is normal except head-shaking test demonstrates a right beating nystagmus.

4 Clinical Case Audiogram Left low frequency sensorineural hearing loss at 250, and 500 Hertz at 30 decibels ENG 18% left unilateral weakness Abnormal saccades and tracking What is your diagnosis?

5 History of Meniere disease 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:

6 History of migraines 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.

7 Diagnostic dilemma 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 Radthke A. et al, Migraine and Meniere s disease: Is there a link? Neurology :

8 Meniere s disease 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 AAO-HNS Committee on Hearing and Equilibrium Guidelines for the Diagnosis and Evaluation of Therapy in Meniere s disease

9 Meniere s disease 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 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

10 1995 Guidelines Meniere s disease Possible Meniere s disease Episodic vertigo without documented hearing loss, or sensorineural hearing loss, fluctuating or fixed, with disequilibrium but without definitive episodes, and other causes excluded. Probable Meniere s disease 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 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 Definite Meniere s disease, plus histopathologic confirmation AAO-HNS Committee on Hearing and Equilibrium Guidelines for the Diagnosis and Evaluation of Therapy in Meniere s disease.

11 Meniere s disease Primary types Classic Meniere s disease Cochlear Meniere s disease Vestibular Meniere s disease Crisis of Tumarkin Secondary types Infectious: Viral, bacterial, and syphilitic Trauma: Closed head injury, noised induce-hearing loss Autoimmune: Allergy, autoimmune inner ear disease, Cogan s syndrome

12 Migraines 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 Migraine in men %. 2 Migraine in women %. 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: Henry P.,et.alPrevalence and clinical characteristics of migraines in France. Neurology 2000; 59:

13 Migraines 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. Usher syndrome, Childhood epilepsy, Hereditary essential tremor, Tourette s syndrome, Hereditary hemorrhagic telangiectasia Familial hemiplegic migraine 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 Strong family history of migraines. 1. Neuhauser H., Vertigo and dizziness related to migraine: a diagnostic challenge Cephalalgia 2004; 24:83-91.

14 IHS 2004 Classification of Migraines Migraine without aura Migraine with aura: Typical aura with migraine headache Typical aura with non-migraine headaches Familial hemiplegic migraine Sporadic hemiplegic migraine Basilar-type migraine Childhood periodic syndromes that are commonly precursors of migraine: Cyclical vomiting Abdominal migraine Benign paroxysmal vertigo of childhood Retinal migraine Complications of migraine: Chronic migraine Status migrainosus Persistent aura without infarction Migrainous infarction Migraine-triggered seizure Probable migraine: Probable migraine without aura Probable migraine with aura Probable chronic migraine

15 IHS 2004 Classification of Migraines IHS classification of migraine headaches requires five attacks which fulfill the following criteria: Headaches lasting between 4-72 hours Headaches having at least two of the following four features: Unilateral Pulsating quality Moderate or severe intensity Aggravated with physical activity 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: One or more fully reversible aura symptoms, indicating focal cerebrocortical or brainstem dysfunction. At least one of the aura symptoms develops gradually for over more than four minutes, or two or more aura symptoms occur in succession. Aura lasts no longer than 60 minutes. 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.

16 Migraines and Vertigo Benign paroxysmal vertigo of childhood: Vertigo in very young children ages 3-5. No symptoms of headaches. Basilar-type migraine: 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

17 Migraine in Meniere s disease Radtke Study 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:

18 Migraines in Meniere s disease Kayan and Hood Study 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 Reported 30% dizziness and 25% vertigo in migraine patients Kayan A, Hood JD, Neuro-otological manifestion of Migraines. Brain 1984:107; Bayazit Y, et al. Assessment of migraine-related cochleovestibular symptoms. Rev Laryngol Otol Rhinol (Bord) 2001; 122:85-88.

19 Migraine associated dizziness Clinical Manifestation Occurs with a peak around the third and fourth decades. Vestibular manifestation can range from light-headiness to severe vertigo. Vertigo last few seconds to several hours. Patients are easily subject to motion sensitivity and movement disequilibrium outside of migraine attacks. May be associated with mild and diffuse headaches to severe unilateral headaches. Headaches do not always occur with vestibular symptoms. Aura does not always occur with vestibular symptoms. Aura such as photophobia and phonophobia may be associated with dizziness. Fluctuating low frequency sensorineural hearing loss is more common with Meniere s disease. Tinnitus is usually present which can be unilateral or bilateral.

20 Migraine associated dizziness Not included in the International Headache Society or American Academy of Otolaryngology classification. Neuhauser proposed criteria for migrainous vertigo

21 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.

22 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.

23 Audiometric testing Battista Study Compared audiometrics with Meniere s disease and Migraine associated dizziness patients. 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:

24 Vestibular testing Cass Study Compared vestibular testing with Meniere s disease and Migraine associated dizziness patients. 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:

25 Meniere s disease Pathophysiology Endolymphatic hydrops There is distension of the membranous labyrinth due to excessive endolymphatic fluid. 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 Schuknecht discovered through histopathologic examination that there was bowing and rupture of Reisner s membrane in the pars inferior portion of the inner ear Fukuda et al., The development of endolymphatic hydrops following CMV inoculation of the endolymphatic sac Laryngoscope 98: Franklin et al. Meniere s symptoms resullting from bilateral otosclerosis occlusion of the endolymphatic duct: an analysis of the causual relationship between otosclerosis and Meniere s disease J Otol. 11(2): Henriksson et al. Pathophysiology of Meniere s disease Controversial aspects Meniere s disease. Georg. Thieme. Verlag. 4. Schuknecht, Pathology of the ear, Boston, 1974 Harvard University

26 1. Appenzeller, et al., Clinical and experimental studies in migraines. Res. Clin Stud. Headaache, 1978; 6: Migraine Pathophysiology Vascular theory (Wolff Hypothesis 1963) Initially there is vasoconstriction during the prodromal phase of migraine followed by vasodilatation in the headache phase. Neurotransmitter release 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 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.

27 Migraine Pathophysiology Vascular theory Prostaglandins produced by the cyclo-oxygenase pathway 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.

28 Migraine Pathophysiology 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.

29 Migraine Pathophysiology 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.

30 Migraine and Meniere s disease A Common Pathophysiology 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.

31 Migraine associated dizziness Treatment: 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.

32 Meniere s disease Medical treatment: Diet: 2000mg low sodium diet, caffeine reduction to 50-75mg 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 Valium and Phenergan

33 Meniere s disease Surgical treatment: Intratympanic gentamicin injections Intratympanic dexamethasone infusions Endolymphatic sac shunt Vestibular nerve section Labyrinthectomy

34 Migraine Diet: 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: Over the counter medication (OTC) analgesics such as: acetaminophen, aspirin, ibuprofen, and naproxen. Antimetic: Phenergan Sympathomimetic vasoconstrictor Isometheptene, dichloraphenazone, and acetaminophen (Midrin) Aspirin, caffeine, and butalbital (Fiorinal)

35 Migraine Abortive treatment: Serotoninergic vasoconstrictors Cafergot Dihydroergotamine Triptans Preventive treatment: Tricyclics: Amitriptyline, Doxepin, Nortriptyline Beta blockers: Atenolol, Propanolol Calcium channel blockers: Verapamil Anticonvulsants: Tegretol, Neurontin, Topamax

36 Migraine associated dizziness versus Meniere s disease Symptoms Meniere s Migraine (MAD) Gender equal mostly females Hearing loss fluctuate, progressive, low frequency Family Hx 10-20% 60-70% fluctuate, low frequency 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

37 THE END QUESTIONS?

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