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Meniere's Disease

edited by: J.P. Harris

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Publication details: Book. 1999. xiii and 419 pages. Publication date: 1999-04-19. 132 figures and 44 tables. Hardbound.

ISBN: 978-90-6299-162-4 (ISBN 10: 90-6299-162-9; Kugler Publications)

Also available as ebook


This book provides up-to-date, authoritative presentations on the historical aspects, epidemiology and natural history, histopathology, pathogenesis, pathophysiology, diagnosis, clinical aspects and treatment of Meniere's disease written by the worlds most leading experts in the field today. By providing provocative discussions and complete references on state-of-the-art research and clinical aspects of Meniere's disease this text will take its place among the definitive reference texts in the field of Otology and Neurotology.



In many ways we are at a new pinnacle of discovery in Biology and Medicine. Never before has there been such rapid discovery of the fundamental issues involving cellular mechanisms, immunology, genetics, molecular biology and neuroscience. Each day we read about diseases for which the cause or a genetic basis has been identified, or for which a successful new treatment has been developed. With some of the best and the brightest students from our medical schools now turning to otolaryngology as their chosen profession, and with the attention of basic scientists newly focused on the inner ear, it is conceivable that many diseases that cause terrible afflictions to our patients will soon be cured. Unfortunately, we are not yet there in Meniere's disease. Reading through this textbook, written by the leading experts in this field, I see pieces of the puzzle but not the solution. The etiology of this disease remains 'idiopathic'. However, this is only because of our ignorance, and there are significant clues to its cause. There are derange­ments in serological factors seen in too many patients to suggest that it is all coincidental or anecdotal. Patients have acute exacerbations of the disease and then long periods of remission. Yet there is an inexorable loss of inner ear function despite relative quiescence in the disease over time. There is no doubt that this is a disease that has a great propensity to become bilateral, does that suggest that it is autoimmune, genetic or infectious? We spend an inordinate amount of time trying to restore function to the endolymphatic sac - but is this the correct approach, or does Meniere's disease really represent an 'overfunctioning' sac rather than an underfunctioning one? In the provocative chapter by Gibson, we see evidence suggesting that the sac's destruction may alleviate symptoms rather than creating worsening endolymphatic hydrops. In medical school we are taught to attempt to classify diseases into one of several categories: metabolic, developmental, neoplastic, genetic, autoimmune and infectious. In Meniere's disease we can only with certainty rule out one of these categories, and that is neoplastic. There is evidence cited in the literature that supports each one of the other categories. If the disease is multifactorial, all this evidence may be valid. It seems, therefore, that we have not yet unlocked the mechanism underlying the development of Meniere's disease. Until that occurs we will continue to search for effective therapy, though it will not be curative. I look forward to the next decade of research on this disorder, as we find more precise means of determining the specific etiology for the development of Meniere's disease in a single individual, and begin to apply new ways of treating the inner ear with drug delivery systems, gene therapy and methods not yet invented. I suspect and hope that by the time the Second Edition of this textbook is written the etiology(ies) will be determined, and that rational therapies based on these etiologies will be readily available.

Jeffrey P. Harris, M.D., Ph.D.
La Jolla, California

Table of Contents

Table of Contents

O.J. Harris, 1914-1994

In memoriam
Harold F. Schuknecht, 1917-1996

J.P. Harris

History of Meniere's Disease

Meniere's role in the recognition of the ear as a source of vertigo. Historical perspective
R.J. Ruben and J.P. Harris

Epidemiology and Natural History

The epidemiology of Meniere's disease
U. Friberg and J. Stahle

The natural history of Meniere's disease
M.M Paparella and H. Sajjadi


Histopathology of Meniere's disease
H.F. Schuknecht

Histopathology of Meniere's- like conditions
F.H. Linthicum

Nerve fibers in the endolymphatic sac
M. Barbara


Pathogenesis of Meniere's syndrome
J.B. Nadol Jr

Possible role of the endolymphatic sac in the pathogenesis of Meniere's disease
D. Bagger-Sjöbäck

Fluid homeostasis in the inner ear
A.N. Salt

Pathogenesis of Meniere's disease: endolymph ion regulation
E. Ferrary, O. Sterkers and P. Tran Ba Huy

Experimental hydrops
R.S. Kimura

Immunology/virology of Meniere's disease
J.P. Harris and S. Tomiyama

Viral etiology of Meniere's disease
D.B. Welling and R.L. Daniels

The molecular mechanisms and genetics of Meniere's disease
R.A. Friedman and A.F. Ryan

Models for Meniere's disease
S.F.L. Klis, J.E. Veldman and G.F. Smoorenburg


The pathophysiology of Meniere's disease
A.F. Ryan

Ultrastructure of the endolymphatic duct and sac in normal and Meniere's disease
D.J. Lim

Cochlear pathophysiology in Meniere's disease. A critical appraisal
M.J. Ruckenstein and R.V. Harrison

Pathophysiology: Transduction and motor disturbances of hair cells by endolymphatic hydrops and transitory endolymph leakage
H.-P. Zenner, JP Ruppersberg, H. Löwenheim and A.W. Gummer

Electrocochleographic pathophysiology in Meniere's disease
J.-M. Aran

Pathophysiology of Meniere's disease. Vestibular system
V. Honrubia

Diagnosis and Clinical Aspects

Clinical manifestations of Meniere's disease
M.J. Ruckenstein and J.J. Shea Jr

Differential diagnosis of Meniere's disease
B.J. Gantz and P.W. Gidley

Migraine and Meniere's disease
R.W. Baloh and J.C. Andrews

Diagnostic and laboratory evaluation of Meniere's disease
A.W. Morrison

MR imaging in Meniere's disease
D.C. Fitzgerald and A.S. Mark

Staging and outcomes for Meniere's disease
S.P. Cass       


Meniere's disease: medical therapy
G.A. Gates

Medical management of Meniere's syndrome
J.B. Nadol Jr

How I do it: Medical management of Meniere's disease
H. Sajjadi and M.M. Paparella

Medical management of Meniere's disease
A.W. Morrison

Internal and external endolymphatic shunts for Meniere's disease
I.S. Storper and M.E. Glasscock III

Endolymphatic sac surgery
B.D. Ress and JP Harris

Removal of the extraosseous portion of the endolymphatic sac
W.P.R. Gibson

Vestibular neurectomy
H. Silverstein, J. Arruda and S. Rosenberg

Selective chemical ablation as treatment for Meniere's disease
S.W. Hone and J.M. Nedzelski

Treatments of Meniere's disease applied to the semicircular canals
E.M. Monsell

Dexamethasone perfusion of the inner ear through the round window membrane for Meniere's disease
J.J. Shea Jr

Meniere's disease: an overview
W.F. House    

Unsolved mysteries of Meniere's disease
D.E. Mattox   

Index of authors


Book reviews

    By: Prof. John Birchall, MD, FRCS, Otolaryngologist, Nottingham, UK

We all like a good mystery and judging by the amount of work expended on trying to understand it, and the amount of debate surrounding it, Menieres Disease fills this role for the otologist. 
The editor of this volume has assembled an impressive group of authors to produce this comprehensive volume. It consists of 40 chapters covering the history of the disease, epidemiology, histopathology, pathogenesis, pathophysiology, diagnosis and treatment in its 419 pages. It is a reference book and therefore, may be forgiven for repeated vignettes of Menieres Disease at the beginning of many chapters. It is generally well written, well referenced and has a pleasing layout. 
The first chapter is a detailed scholarly review of the history of the disease. The chapter on epidemiology is an excellent source of information on which to base advice to patients on the likely progression and outcome of the disease. The chapter on inner ear and fluid home­ostasis is excellent, coupled with the pathophysiology section which debunks the classical view of the formation of endolymphatic hydrops and the role of the endolymphatic sac. 
The section on diagnosis would benefit clinicians outside Otology and reduce Menieres status as the most wrongly overdiagnosed cause of dizziness. 
The section on treatment is what will interest most readers; even the surgically orientated authors emphasise the importance of medical treatment as the prima­ry modality but what do we do next when medical treatment fails? The beauty of this book is that it gives the reader the information on which to develop a management plan and justify it. 
The final chapter is entitled the Unsolved Mysteries of Menieres Disease; which deals with 13 mysteries as yet unsolved. The Menieres saga is still going to run for quite some time to come. I can unreservedly recommend this book for those who are interested in keeping up with the plot.

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