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MUSC Otolaryngology - Head & Neck Surgery E-Update                 June 2016 
It's June already?

In our June article, Habib G. Rizk, M.D., MSc, describes recent criteria for identifying Ménière's disease and Vestibular Migraines and discusses the association between the two pathologies.
    
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Professor and Department Chair

Is Ménière's Disease a Migraine of the Inner Ear?
Habib G. Rizk, M.D., MSc

Prosper Meniere
  Prosper Ménière 

Over 150 years ago, Prosper Ménière described the disease bearing his name. He also observed that many of these patients have migraines. This association between Ménière's Disease (MD) and Migraines became more credible with more recent publications, which estimated that lifetime prevalence of migraine in men with MD is around 40%. This prevalence is up to 75% in women with MD.
The Barany society recently published criteria to diagnose a vestibular migraine (VM). The patient should have at least five episodes of vestibular symptoms (spontaneous, positional, visual vertigo, motion-induced), with a current or previous history of migraine with or without aura according to the International Classification of Headache Disorders, along with one or more migraine features. At least 50% of the vestibular episodes should include at least one of the following characteristics: 1) a headache that is one sided, pulsing, moderate to severe in intensity and aggravated by exertion, 2) a visual aura or 3) photophobia and/or phonophobia. Probable VM is defined when we have at least five episodes with vestibular symptoms of moderate or severe intensity lasting 5 minutes to 72 hours with either a migraine history or migrainous features during the episode.
 
The recent criteria for classification of Ménière's Disease (MD) from the Barany Society, published in 2015, also distinguish probable MD and definite MD. The former is defined as two or more episodes of vertigo or dizziness, each lasting 20 minutes to 24 hours with fluctuating aural symptoms (hearing, tinnitus or fullness). Definite MD is diagnosed in the setting of two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours with an audiometrically documented low to medium frequency sensorineural hearing loss in the affected ear on at least one occasion, before, during or after one of the episodes of vertigo, as well as fluctuating aural symptoms in the affected ear.
It is evident that the lines are blurry at times between MD and VM and that some patients may present with features of both diseases. In deed, MD patients can present with some migrainous features, notably photosensitivity and motion sickness but can also have headaches. These headaches do not always fulfill the criteria for migraines according to ICHD.

One frequent confounder is sinus headaches. It is estimated that over 75% of self-reported sinus headaches (sinus pain, sinus fullness-with normal CT scan of the sinuses), are actually migrainous in origin. In addition, the vast majority of patients with ICHD migraine headaches report nasal and sinus symptoms. The activation of the trigeminal pathways in migraineurs is postulated to be the cause for these "mimickers" of sinus disease. On the other hand, it is sometimes difficult, especially early in the disease process to document a low frequency sensorineural hearing loss if the patient does not present to the clinic close to the "spell". If a patient fulfills criteria for both probable MD and probable VM, does he have Ménière's disease or does he have Migraines? Is there an overlap syndrome? Are these two pathologies on a different spectrum of the same disease?
 
The confusion goes even further when we look at the modalities of treatment of both pathologies. Low salt diet can prevent hypovolemia by decreasing diuresis which will mean it will prevent dehydration. Dehydration is a common trigger for migraineurs. Allergies and low barometric pressure are also major triggers for both pathologies, with the caveat that these so-called allergies may actually be migraine-related sinonasal symptoms. Finally, migraine is currently considered to be a channelopathy with alteration of ion channel functions, which disturbs the electrolyte distribution within the cell and in the interior milieu. The medications used for prevention are all ion channel modifiers, whether it is sodium-channel blockers such as nortriptyline and propranolol, calcium-channel blockers such as verapamil or GABAergic receptors activators such as topiramate. When we look at the endolymphatic space physiology, all of these ion channels are present in abundance within the lining cells. A channelopathy expressed in the inner ear could very well be the pathophysiological primer of MD...Of course, this is still speculation at this point.
 
What about endolymphatic hydrops? Over 75 years ago, the enlargement of the endolymphatic space was considered to be the primary pathology related to MD. This caused a lot of ink to flow, and novel surgical techniques were developed. Some of these techniques are not used any more (Cody's tack, sacculotomy...), whereas others are still part of the clinicians armamentarium to treat debilitating attacks of vertigo (endolymphatic sac decompression/shunt). In 1995 the American Academy of Otolaryngology-Head and Neck Surgery Equilibrium Committe defined Certain MD in patients with the characteristic symptom constellation and with endolymphatic hydrops (EH). Given that EH could only be confirmed on autopsy, it was extremely difficult to reach a diagnosis of certain MD. However, with the advent of high resolution MRI, this obstacle seems to be easier to get around. Intratympanic injection of gadolinium and more recently delayed (four hours) heavily weighted T2 and FLAIR sequences after intravenous injection of gadolinium, seem to provide a clear view of the endolymphatic spaces, and even objectively put in evidence an endolymphatic hydrops.
Is Ménière's disease a migraine of the inner ear?
The observations compiled over the course of a century and a half do seem compelling. The evidence is not there yet. One thing is certain, both pathologies take a significant toll on the patient's quality of life with increased absenteeism and disability. Patients presenting with atypical features of MD or VM will at some point develop more characteristic features that will guide us toward the most appropriate diagnosis and treatment modalities.
 
Time always tells! "The real voyage of discovery consists not in seeking new lands but seeing with new eyes" (dixit Marcel Proust).
References
  1. Ménière P. Pathologie auriculaire: memoire sur des lesions de l'oreille interne donnant lieu a des symptomes de congestion cerebrale apoplectiforme. Gaz Med Paris 1861;16:597-601.
  2. Radtke A, Lempert T, Gresty MA, Brookes GB, Bronstein AM and Neuhauser H, Migraine and Meniere's disease Is there a link?, Neurology, 2002, 59: 1700-1704
  3. Ghavami Y, Mahboubi H, Yau AY, Maducdoc M and Djalilian HR, Migraine features in patients with Ménière's disease, Laryngoscope, 2016, 126: 163-168
  4. Lopez-Escamez JA, Carey J, Chung WH et al. Diagnostic criteria for Ménière's disease Consensus Document of the Bárány Society, the Japan Society for Equilibrium Research, the European Acadmy of Otology and Neurotology (EAONO), the American Academy of Otolaryngology-Head and Neck Surgery and the Korean Balance Society, J Vest Res, 2015, 25: 1-7
  5. Lempert T, Olesen J, Furman J et al Vestibular Migraine: Diagnostic criteria Consensus document of the Bárány Society and the International Headache Society, J Vest Res, 2012, 22: 167-172
  6. Schreiber CP, Hutchinson S, Webster CJ, Ames M, Richardson MS and Powers C, Prevalence of Migraine in Patients with a History of Self-Reported or Physician-Diagnosed "Sinus" Headache, Arch Intern Med, 2004, 164: 1769-1772
  7. Gates P, Hypothesis: could Ménière's disease be a channelopathy, Intern Med J, 2005, 35: 488-489
  8. Gürkov R, Kantner C, Strupp M, Flatz W, Krause E and Ertl-Wagner B, Endolymphatic hydrops in patients with vestibular migraine and auditory symptoms, Eur Arch Otorhinolaryngol, 2014, 271: 2661-2667
Habib G. Rizk, M.D., MSc

Assistant Professor, Otology & Neurotology
Director, Vestibular Program 
M.D.: Saint Joseph University, Beirut, Lebanon
Residency: Saint Joseph University and Hotel-Dieu de France Hospital,  Beirut, Lebanon
Fellowship: Medical University of South Carolina
Special Interests: Medical and surgical management of vestibular disorders
Email: [email protected]

E-Update Articles 
Look for these articles in upcoming issues!

July:
Contemporary Management of Recurrent Respiratory Papilloma

August:
The Effect of USP 797 Compounding Guidelines and The Compounding Bill on the Administration of Allergen Immunotherapy
   
September: Endoscopic Sinus Surgery: When and how much?


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