Endolymphatic hydrops: pathophysiology and experimental models

Abstract

It is well established that endolymphatic hydrops plays a role in Ménière disease, even though the precise role is not fully understood and the presence of hydrops in the ear does not always result in symptoms of the disease. It nevertheless follows that a scientific understanding of how hydrops arises, how it affects the function of the ear, and how it can be manipulated or reversed could contribute to the development of effective treatments for the disease. Measurements in animal models in which endolymphatic hydrops has been induced have given numerous insights into the relationships between hydrops and other pathologic and electrophysiological changes, and how these changes influence the function of the ear. The prominent role of the endolymphatic sac in endolymph volume regulation, and the cascade of histopathological and electrophysiological changes that are associated with chronic endolymphatic hydrops, have now been established. An increasing number of models are now available that allow specific aspects of the interrelationships to be studied. The yclical nature of Ménière symptoms gives hope that treatments can be developed to maintain the ear in permanent state of remission, possibly by controlling endolymphatic hydrops, thereby avoiding the rogressive damage and secondary pathologic changes that may also contribute to the patient's symptoms.

Figure 1

Mid-modiolar sections through the normal guinea pig cochlea (left) and from a cochlea in which endolymphatic hydrops was induced by surgical ablation of the endolymphatic duct and sac (right). The endolymphatic space has been colored blue to highlight the endolymph enlargement into the perilymphatic space of scala vestibuli resulting from the distension of Reissner's membrane. Histology of these specimens was performed in the laboratory of Dr. R. Kimura as part of a collaborative study (5).