Menière  Syndrome

Compiled By;  Dr. Hafiz Shahid Amin (MBBS, DCA, DLO)  ENT Surgeon,  drshahee@brain.net.pk   Gujranwala Pakistan

The concept of endolymphatic hydrops was proposed by Gruber in Vienna (1895).  He studied 100 temporal bones and found that the anatomy of the endolymphatic sac varied a lot. He postulated that obliteration of the endolymphatic duct could possibly impede the drainage of endolymph, and that increased pressure in the endolymphatic system could possibly result in tearing of the membranous labyrinth, which would, like a stroke, produce the symptoms of Menière’s disease.

      Quoted by Feldman H:  Laryngo-Rhino-Otologie 72,1-8, 1993 (in German)   +

In 1926 Georges Portman developed the endolymphatic sac operation.

In 1938 Hallpike and Cairns in England, and Yamakawa in Japan, independently discovered distended endolymphatic ducts in the cochleas of deceased patient with Menière’s disease, and concluded that the distention was probably caused by raised endolymph pressure.

This was followed by innumerous studies and theories (e.g., Guild’s theory of longitudinal flow of endolymph), on which followed innovation surgery, diets, as well as money spinning medicines – all of them with a quoted 70% success rate (statement by Schuknecht in 1965 on a visit to Pretoria). 

In 1952 Furstenberg noted: “

When vertigo dominates the clinical picture but is associated with burning sensation in the pharynx, numbness on one side of the face and ear, or a typical tic doloreux, it is impossible to reconcile these findings with an isolated lesion in the internal ear”.

 Lempert et al (1952) concluded that

“Menière’s disease is a chronic Herpetic Neuritis of  the vestibular labyrinth of either toxic or trophic origin”.

In 1972, at the Collegium meeting in Basel, the author discussed an 18 year old patient with Prof Schuknecht of Harvard University.  This patient, who had a deaf ear caused by mumps, developed incapacitating Menière symptoms which was cured by labyrinthectomy.  Schuknecht remarked that this was already a well known phenomenon that Menière’s disease could develop in a deaf ear.

In 1974 Schuknecht postulated the concept of secondary and delayed endolymphatic hydrops which could develop following previous pathology in an inner ear.  “Assuming that viral labyrinthitis can occur in infants as a subclinicl disease that results in delayed endolymphatic hydrops, we may have an explanation for the cause of Menière’s disease. Viewed in this context, the disease entity known as delayed endolymphatic hydrops becomes the missing link in understanding the pathogenesis of Menière’s disease.

In 1980 Kedar Adour, Frederick Byl, Raymond Hilsinger and Robert Wilcox published  an article: Menière’s disease as a form of cranial polyganglionitis”, and postulated:

“Although endolymphatic hydrops is generally considered to be the most prominent factor in the etiology and pathology of Menière’s disease, we have concluded that this condition more probably represents a polyganglionitis caused by the herpes simplex virus with secondary hydrops changes.

             Laryngoscope 90:392-398, 1980

In 1997 Arnold and Niedermeyer of München discovered Herpes Simplex Virus in the perilymph of patients with Menière’s disease .

     Arch Otolaryngol 123:53-56, 1997

In 1997 Adour and Hamersma suggested a new name for the cranial polyganglionitis of Adour et al of 1980, i.e.  Polyganglionitis Episodica (PGE),  which explained the etiology and symptomatology of Bell’s palsy as well as Menières syndrome.

In 1998 Pyykkö et all (Stockholm) confirmed the presence of circulating auto-antibodies , and concluded: “It is hypothesized that a viral infection breaks the labyrinthine barrier and triggers an auto-immune reaction.  Specially the herpes group of viruses are primary candidates for causing Menière disease”.

In 1998 Adour supported a concept about HSV-1 virus infection which was forwarded by Biswell (ophthalmologist), and hypothesized that, what happens in the inner ear in Menière syndrome may very well be regarded as

‘Antigen-Antibody-Complement (AAC) Immune Complex

Disease’.

In July 2001 Drs Richard and Mark Gacek from Mobile, Alabama, USA, published an article: “Menière’s Disease as a manifestation of Vestibular Ganglionitis”, and concluded:

“Morphologic changes in temporal bones of patients with Menière disease, and clinical observations in patients with recurrent vestibulopathy, support the concept that the pathologic mechanism responsible for auditory and vestibular symptoms in Menière disease may be reactivation of a latent viral vestibular ganglionitis”

 They found focal neuronal degeneration in both vestibular and cochlear nerves, sometimes even in ganglion cells inside the so-called meatal facial nerve ganglion, as well as inflammatory fibrosis in the perilymphatic spaces.  There was marked distention of the saccular wall and Reissner’s membrane, with angulation resembling that found in endolymphatic hydrops.  These changes may be produced by traction on the distensible membranous walls of the pars inferior.

H.H.: Therefore the apparent endolymphatic hydrops was not due to  increased endolymphatic pressure, but distortion of the membranous labyrinth occurring in the advanced stage of a diseased inner ear.

 American Journal of Otolaryngology, Vol 22, No. 4 (July-August), 2001: pp 241-250

Richard R Gacek and Mark R Gacek:

Menière’s Disease as a Manifestation of Vestibular Ganglionitis.  

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