Etiology

MD was first classified as a syndrome by Prosper Ménière in 1861. In 1938, MD was associated with endolymphatic hydrops which is a condition of excess endolymphatic fluid. The etiology of hydrops is unknown, and its natural history is highly variable.³ 

Gulya and Schuknecht postulated that the disease is triggered by viral infection presumably affecting the endolymphatic fluid control mechanisms leading to distention of the scala media.⁴ Experience from morphological investigations⁵ and animal experiments6 suggest that the symptoms of Ménière's disease originate from a disturbance in the volume/pressure relationship of the endolymph. The increased volume of endolymph is likely due to fluid overproduction; however, deficient absorptive mechanisms have also been considered. In either event, the distended scala media (i.e., hydrops) is the histologic hallmark of Ménière's disease.⁷ Figure 2.

Figure 2A.  Cross section of a cochlea with endolymphatic hydrops.  Click on image to view larger.
Figure 2B. Cross section of a normal cochlea.  Click on image to view larger.

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