Kimitaka Kaga · Arnold Starr (Eds.) Neuropathies of the Auditory and Vestibular Eighth Cranial Nerves Kimitaka Kaga · Arnold Starr Editors Neuropathies of the Auditory and Vestibular Eighth Cranial Nerves Kimitaka Kaga, M.D., Ph.D. Emeritus Professor, The University of Tokyo Director, National Institute of Sensory Organs National Tokyo Medical Center 2-5-1 Higashigaoka, Meguro-ku, Tokyo 152-8902, Japan Arnold Starr, M.D. Professor Department of Neurology and Neurobiology University of California Irvine Irvine, CA 92717, USA Library of Congress Control Number: 2008930929 ISBN 978-4-431-09432-6 Springer Tokyo Berlin Heidelberg New York e-ISBN 978-4-431-09433-3 This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifi cally the rights of translation, reprinting, reuse of illustrations, recitation, broadcast- ing, reproduction on microfi lms or in other ways, and storage in databanks. The use of registered names, trademarks, etc. in this publication does not imply, even in the absence of a specifi c statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Product liability: The publisher can give no guarantee for information about drug dosage and application thereof contained in this book. In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature. Springer is a part of Springer Science+Business Media springer.com © Springer 2009 Printed in Japan Typesetting: SNP Best-set Typesetter Ltd., Hong Kong Printing and Binding: Hicom, Japan Printed on acid-free paper Preface The International Mini-Symposium on Auditory and Vestibular Neuropathy (Audi- tory Nerve Disease) was held on March 3, 2007, at the University of Tokyo, Japan. The symposium was planned to commemorate my new departure from the Univer- sity of Tokyo to the National Institute of Sensory Organs. Guest speakers from the United States, Australia, Korea, and Japan presented papers that focused on history, gene, pathophysiology, analysis, perception, sound localization, cochlear implants, and balance for auditory and vestibular neuropathy, with up-to-date information and knowledge. Altogether, there were many important presentations about this new disease, which was fi rst reported by Kaga K et al and Starr A et al in 1996. There are many unsolved issues such as gene location, site of pathology, and the effect of cochlear implants. In the last 10 years, hearing screening of newborns has been introduced in many countries, and auditory neuropathy is paid much more attention because of the increase in new fi ndings. In this international mini- symposium, in addition to auditory neuropathy, vestibular neuropathy in particular was discussed because of the simultaneous existence of both auditory and vestibu- lar issues. This book provides up-to-date information and knowledge on the neuropathy of auditory and vestibular eighth nerves VIII. The title for the book was suggested by Prof. Arnold Starr as one that would cover the entire range of topics. I am hopeful that, with this book, readers can gain insight into the new disease entity, auditory and vestibular neuropathy. I would like to acknowledge, with gratitude, that this publication was fi nancially supported in part by the alumni association of the Department of Otolaryngology, Faculty of Medicine, University of Tokyo, and by the Society for the Promotion of Oto-Rhino-Laryngology. Kimitaka Kaga, M.D., Ph.D. Director, National Institute of Sensory Organs Tokyo Medical Center Emeritus Professor, The University of Tokyo V Contents Preface . V Contributors . IX Part I Overview . 1 “Hearing” and Auditory Neuropathy: Lessons from Patients, Physiology, and Genetics Arnold Starr . 3 Part II Pathophysiology and Genetics . 11 Auditory Nerve Disease, New Classifi cation: Auditory and Vestibular Neuropathy Kimitaka Kaga . 13 Identifi cation of Different Subtypes of Auditory Neuropathy Using Electrocochleography Catherine M. McMahon, Robert B. Patuzzi, William P.R. Gibson, and Halit Sanli . 21 Sound Localization and Lateralization of Patients with Auditory Neuropathy Kenji Itoh, Sozo Kuroki, Sotaro Sekimoto, and Kimitaka Kaga . 37 Trends in Genetic Research on Auditory Neuropathy Tatsuo Matsunaga . 43 Part III Cochlear Implants . 51 Environmental Sound Perception in Patients with Cochlear Implants Compared with That in Patients with Auditory Nerve Diseases (Auditory Neuropathy) and Cortical Deafness Kimitaka Kaga and Yusuke Akamatsu . 53 Pediatric Cochlear Implantation in Auditory Neuropathy Lee-Suk Kim and Sung-Wook Jeong . 61 VII VIII Contents Cochlear Implantation for Children with Auditory Neuropathy Among Japanese Language Users Kunihiro Fukushima, Yuko Kataoka, Yukihide Maeda, Shin Kariya, Susumu Tominaga, Rie Nagayasu, Akihiro Kawasaki, Shouichiro Fukuda, Naomi Toida, and Kazunori Nishizaki . 71 Cochlear Implantation for a Child with Auditory Nerve Disease: a Case Report Yukiko Shinjo, Yulian Jin, and Kimitaka Kaga . 77 Part IV Vestibular Neuropathy . 83 Vestibular Neuropathy and Vestibular Evoked Myogenic Potential Toshihisa Murofushi . 85 Impulsive Testing of Semicircular Canal Function G. Michael Halmagyi, Konrad P. Weber, Swee T. Aw, Michael J. Todd, and Ian S. Curthoys . 93 Part V Neurological Cases . 111 Similarities and Differences Between Auditory Neuropathy and Acoustic Neuroma Tatsuya Yamasoba . 113 Diagnosis of Auditory Neuropathy (AN) in Child Neurology Makiko Kaga, Masumi Inagaki, Kaori Kon, Akira Uno, and Tatsuro Nobutoki . 123 A Case of Unilateral Auditory Neuropathy Yuki Saito, Mitsuya Suzuki, and Tunemasa Sato . 135 Part VI Historical Issues . 143 Prehistory of Auditory Neuropathy in Japan Toshihiro Tsuzuku . 145 Is Auditory Neuropathy an Appropriate Diagnosis if There Is No Neuropathy? Roger R. Marsh and Ken Kazahaya . 149 Subject Index . 157 Contributors Yusuke Akamatsu Department of Otolaryngology, and Head and Neck Surgery, The University of Tokyo, Tokyo, Japan Swee T. Aw Neurology Department, Royal Prince Alfred Hospital, Sydney, NSW, Australia Ian S. Curthoys Neurology Department, Royal Prince Alfred Hospital, Sydney, NSW, Australia Shouichiro Fukuda Okayama Kanaria Gakuen, Okayama, Japan Kunihiro Fukushima Department of Otolaryngology Head and Neck Surgery, Okayama University Postgraduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama, Japan William P.R. Gibson Department of Surgery, University of Sydney, Sydney, NSW, Australia G. Michael Halmagyi Neurology Department, Royal Prince Alfred Hospital, Sydney, NSW, Australia Masumi Inagaki Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan Kenji Itoh Department of Sensory and Motor Neuroscience, The University of Tokyo School of Medicine, Tokyo, Japan Sung-Wook Jeong Department of Otolaryngology—Head and Neck Surgery, College of Medicine, Dong-A University, Busan, Korea Yulian Jin National Institute of Sensory Organs, National Tokyo Medical Center, Tokyo, Japan IX X Contributors Kimitaka Kaga National Institute of Sensory Organs, National Tokyo Medical Center, Tokyo, Japan Makiko Kaga Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan Shin Kariya Department of Otolaryngology Head and Neck Surgery, Okayama University Postgraduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama, Japan Yuko Kataoka Department of Otolaryngology Head and Neck Surgery, Okayama University Postgraduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama, Japan Akihiro Kawasaki Department of Otolaryngology Head and Neck Surgery, Okayama University Postgraduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama, Japan Ken Kazahaya Division of Pediatric Otolaryngology, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA Department of Otolaryngology: Head and Neck Surgery, University of Pennsylvania School of Medicine, Philadelphia, PA, USA Lee-Suk Kim Department of Otolaryngology—Head and Neck Surgery, College of Medicine, Dong-A University, Busan, Korea Kaori Kon Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan Shozo Kuroki Department of Sensory and Motor Neuroscience, The University of Tokyo School of Medicine, Tokyo, Japan Yukihide Maeda Department of Otolaryngology Head and Neck Surgery, Okayama University Postgraduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama, Japan Roger R. Marsh Center for Childhood Communication, and Division of Pediatric Otolaryngology, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA Contributors XI Tatsuo Matsunaga Laboratory of Auditory Disorders, National Institute of Sensory Organs, National Tokyo Medical Center, Tokyo, Japan Catherine M. McMahon Center for Language Sciences, Macquarie University, Sydney, NSW, Australia Toshihisa Murofushi Department of Otolaryngology, Teikyo University School of Medicine Mizonokuchi Hospital, Kawasaki, Japan Rie Nagayasu Department of Otolaryngology Head and Neck Surgery, Okayama University Postgraduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama, Japan Kazunori Nishizaki Department of Otolaryngology Head and Neck Surgery, Okayama University Postgraduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama, Japan Tatsuro Nobutoki