Menière’s Disease: Etiopathogenesis

This chapter will discuss idiopathic Menière’s syndrome. That is to say—Menière’s disease. We will start with a brief recall on the History of Menière’s disease beginning with the description of the syndrome by Prosper Menière in 1861, the description of endolymphatic hydrops in temporal bone studies by Hallpike and Cairns in 1938 and by Yamakaua in the same year. Endolymphatic hydrops became a pathologic correlate for Menière’s syndrome. Theories that considered endolymphatic hydrops as the cause of the syndrome will be discussed. More recent studies ques-tioning the old theories and thinking of endolymphatic hydrops as an epiphenomenon in the course of the syndrome rather than the cause of the symptoms will be discussed. Temporal bone studies were the basis of these new theories too. Familial Menière’s disease will be discussed and several families will be described in detail. Because the phenotype of siblings on each family studied was variable and migraine was present in many affected members of these families a spectrum was postulated going from migraine alone to full blown Menière’s disease. Some siblings had what has been described recently as vertiginous migraine and a detailed description of this syndrome will be provided and the differences between this syndrome and Menière’s disease will be made clear. About 20% of Menière’s disease patients have a familial history. Sporadic Meniere’s disease might have a genetic predisposition and other environmental and behavioral factors contribute for the surfacing of the disease (multifactorial etiology). Because migraine is a central phenomenon and the vertiginous episodes and auditory symptoms are peripheral a hypothesis is presented for the pathophysiology of Menière’s disease. Recent research comparing vestibular migraine and Manière’s disease reinforcing the concept of these syndromes repre-senting a continuum process with similar etiology are discussed at the end.