Morphological Aspects of Inner Ear Disease
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1 Morphological Aspects of Inner Ear Disease
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3 Yasuya Nomura Morphological Aspects of Inner Ear Disease
4 Yasuya Nomura President The Society for Promotion of International Oto-Rhino-Laryngology Tokyo, Japan Professor Emeritus The University of Tokyo Tokyo, Japan Visiting Professor Showa University Tokyo, Japan ISBN ISBN (ebook) DOI / Springer Tokyo Heidelberg New York Dordrecht London Library of Congress Control Number: Springer Japan 2014 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. Exempted from this legal reservation are brief excerpts in connection with reviews or scholarly analysis or material supplied specifically for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work. Duplication of this publication or parts thereof is permitted only under the provisions of the Copyright Law of the Publisher s location, in its current version, and permission for use must always be obtained from Springer. Permissions for use may be obtained through RightsLink at the Copyright Clearance Center. Violations are liable to prosecution under the respective Copyright Law. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. While the advice and information in this book are believed to be true and accurate at the date of publication, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper Springer is part of Springer Science+Business Media (
5 Preface The inner ear is a beautiful organ particularly when it is shown in color. Its sensory cells lie in a hard cavern (the temporal bone) of a deep sea (the inner ear fluids). Color illustrations bring the organ forth out of darkness into a brilliant world and enable us to see glittering original shapes suggesting functional significance. Alfonso Corti published a paper with colored figures in 1851 describing the hearing organ that later bore his name. He no doubt would have published his paper using color photographs, had they been available 160 years ago. Our knowledge of the pathophysiology of inner ear diseases is mainly based on histopathological studies of human temporal bones. However, the long gap in time between the onset of a disease and findings obtained from temporal bone specimens sometimes makes interpretation difficult. The specimens may appear before us years and years after the onset. During this time, diseased tissues will have been greatly altered in appearance due to cleansing and repairing processes. Aging also plays a part in this regard. Experimental studies may be useful for obtaining information about ongoing changes occurring within the inner ear. They may teach us how the organ reacts to artificial stimuli in the acute stage and how the organ shows morphological changes that provide us with information on the integrity or mysteries of the inner ear. At the same time, in the results obtained, there may be a discrepancy between clinical and experimental work. Taking these facts into account, when seeing patients one must extend the imagination regarding the pathophysiology of diseases. While revisiting old papers and photographs for the preparation of this book, I noticed several new findings that I had overlooked earlier. It was a pleasure for me to see old slides or photographs that glistened under new eyes. Advances in knowledge and experience will further unearth buried truths. The contents of this book mainly comprise earlier works. I am grateful for those who have collaborated with me and shared both pleasures and difficulties with me. My gratitude also extends to other researchers who were kind enough to contribute to this book with their valuable scientific photographs. I greatly appreciate their generosity. Tokyo, Japan Yasuya Nomura v
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7 Contents 1 Structure of the Inner Ear Phylogenesis of the Ear The Inner Ear Pars Superior Pars Inferior The Internal Auditory Canal The Middle Ear The External Ear References Inner Ear Fluid Inner Ear Fluid Perilymph and Endolymph Cortilymph The Cochlear Aqueduct Perilymph and the Limbus Spiralis Endolymph Perilymphatic Fistula Clinical Aspects Changes of CSF and Middle Ear Pressures in Daily Life Experimental Perilymphatic Fistula Early Hearing Change with Perilymphatic Fistula Vestibular Changes in Experimental Perilymphatic Fistula References Blood Vessels Anatomy Arteries and Veins Capillaries of the Membranous Cochlea Capillaries of the Vestibular Organs Diseases of the Blood and Blood Vessels Leukemia Buerger s Disease Cryoglobulinemia Takayasu s Arteritis Diabetes Mellitus References vii
8 viii Contents 4 Sudden Deafness Histopathology of Sudden Deafness The Tectorial Membrane Case Reports Case Case Sudden Deafness in a Patient with Relapsing Polychondritis Cochlear Neuronitis Sudden Deafness and Asymptomatic Mumps Vascular Disorders Criteria for Diagnosis of Sudden Deafness in Japan Postscript References Meniere s Disease Discovery of Endolymphatic Hydrops in Meniere s Disease Human Temporal Bone Study Diagnosis of Endolymphatic Hydrops Using Gadolinium MRI Experimentally Induced Endolymphatic Hydrops Observation of Experimental Cochlear Hydrops Using OCT Vestibular Evoked Myogenic Potentials Gentamicin References Viral Diseases Herpes Simplex Virus (HSV) Herpes Classification HSV and Sudden Deafness Morphological Study Cytomegalovirus Introduction Incidence of Congenital CMV Infection Among Newborns in Japan Histopathology of the Temporal Bone in a Case of CMV Experimental Cytomegalic Labyrinthitis Mumps Introduction Morphological Studies Criteria for Diagnosis of Mumps Deafness Mumps and Sudden Deafness Deafness in Mumps Patients Deafness in Mumps Meningoencephalitis References Laser Irradiation of the Inner Ear Introduction Animal Experiments The Cochlea The Otolithic Organs The Semicircular Canal Clinical Applications Irradiation of the Semicircular Canal Irradiation of the Otolithic Organ Surgical Procedure References
9 Contents ix 8 Presbycusis Nerve Fibers in the Osseous Spiral Lamina Nerve Fibers in the Organ of Corti Nerve Fibers Running Lateral to the Hair Cell Area Degeneration of Sensory Hairs on the Outer Hair Cells Strial Atrophy Lipidosis of the Basilar Membrane Prevention of Presbycusis References Anomalies Cochlear Hyperplasia Introduction Cochlear Hyperplasia Hypophosphatasia Introduction Case Report Alagille Syndrome Phylogenesis of the Semicircular Canal Anomalies of the Semicircular Canal Scheibe Dysplasia Introduction Dysplasia and Degeneration Case Report Comment Mondini Dysplasia Introduction Characteristics of Mondini Dysplasia Case Reports References Index
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