Ménière and Endolymphatic Hydrops

In 1861, Prosper Ménière first describes the unknown pathogenic pressure syndrome that is dealt with in this section.

Hydrops is a swelling of the membranous labyrinth due to an imbalance between the secretion system and the excretion of endolymph.
During crises, which appear at random in patients with Ménière-like syndromes, there is a disruption at some point of this membranous labyrinth. The pressure, which up to then is too high, suddenly falls and gives the following clinical panorama:


Upon examination ... we often find "Nothing" at the early stages (recently diagnosed with Ménière’s); and therefore, diagnosis is based on clinical signs.
During pre-crisis, the pressure is high and often there is hypovalency with homolateral ocular drift.
Conversely, during and especially after the crisis, a spontaneous nystagmus appears beating to the side of the lesion.
Between crises, the search for a spontaneous nystagmus using VNS can give us an indicator of the state of the pressure of the offending labyrinth.

How it evolves is completely random with regard to the number and the severity of the dizzy episodes, and auditory dysfunction becomes more severe and longer lasting. However, crises are less frequent and the syndrome includes an almost permanent sense of imbalance.
Hydrops does the equivalent of the pressure syndrome without interruption of the membranous vestibule, and is therefore crisis free. We move from subnormal to above normal pressure and vice-versa, and occasionally this can happen several times a day!

Treatment is through medication, comprising Betahistine as a basic treatment together with sedatives, anti-vertigo medication such as Tanganil via I.V., as well as antiemetics if there are major vertigo attacks.
Often patients do not respond to, or respond badly to this first line of medication. They must therefore turn to vestibular rehabilitation, which should only be carried out by vestibular therapist with a great deal of experience in this field.
Nothing of course can be done about the endolymphatic pressure: the success of the therapy is based on the impression of only having slight discomfort in stead of major vertigo attacks experienced in the past.
Treatment is long term and often there are no less than fifty sessions over the course of the first year in order to ensure results. The alternative is surgery, which justifies the initial choice of VR.

Three surgeries are advocated:

It is often carried out using the retrosygmoid approach.
This major surgery has reached such a level of expertise that generally speaking, both hearing and the facial nerve are unaffected (through electrical and pressure monitoring during surgery).

Vestibular rehabilitation should therefore always be carried out post-surgery.

Ménière pressure syndrome: diagnosis and treatment