ABRs and Electrically Evoked ABRs in Children

264 Pages·2022·20.44 MB·English

by  Kimitaka Kaga

Modern Otology and Neurotology
Modern Otology and Neurotology
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For more than 40 years, Auditory Brainstem Responses (ABRs) have been used as a
diagnostic tool for hearing disorders and brainstem disorders in the pediatric audiology
and neurotology and child neurology. While there are many publications in this field,
this new volume will review hearing problems and neurological disorders in babies,
infants and children, and discuss important new advances such as ABR figure and
threshold changes with infant development.
Kimitaka Kaga  Editor
The book will also cover auditory neuropathy, its numerous and new sub-classifications.
Readers will be thoroughly briefed on all the new perspectives in interpreting data.
Researchers and clinicians will find the text to be a valuable tool in the correct diagnosis
of hearing problems and neurological diseases among babies and infants.
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ISBN 978-4-431-54188-2
9 784431 541882
Modern Otology and Neurotology
Series Editor
Kimitaka Kaga, National Tokyo Medical Center
National Institute of Sensory Organ
Meguro-ku, Tokyo, Japan
This series plays a role as a clinical reference in the rapidly evolving subspecialty of
modern otology and neurotology. Written by prominent academic authorities, this
series integrates contents from all fields of medicine and covers every aspect of the
field, including surgical issues in pediatric audiology, neurotology and neurology,
genetic testing, oncological study in auditory and vestibular organs, geriatric
audiology and neurotology, and new clinical application of bone conduction hearing
etc. Historical developments and unsolved problems of each field will also be
described in detail to help readers’ understanding. The editors and contributors hope
that this book series will contribute to medical residents and experts of otolaryngology
and related clinical medicines in the evaluation of patients with otological and
neurotological disorders.
Kimitaka Kaga
Editor
ABRs and Electrically
Evoked ABRs in Children
Editor
Kimitaka Kaga
National Institute of Sensory Organs
National Hospital Organization
Tokyo Medical Center
Audiology Clinic
Kamio Memorial Hospital
Tokyo, Japan
ISSN 2567-2169 ISSN 2567-2525 (electronic)
Modern Otology and Neurotology
ISBN 978-4-431-54188-2 ISBN 978-4-431-54189-9 (eBook)
https://doi.org/10.1007/978-4-431-54189-9
© Springer Japan KK, part of Springer Nature 2022
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Preface
Ever since Dr. D. L. Jewett discovered the auditory brainstem response (ABR) in
cats and humans in 1970, ABRs and electrically evoked ABRs (EABRs) have been
successfully used as a necessary element of the audiologic and neurotologic diag-
nostic armamentarium worldwide.
This book is a compendium of our basic and clinical research studies of the ABR
and EABR over a 40-year time span and provides up-to-date information of the his-
tory, recording techniques, and the neurological substrate underlying these
responses. On the diagnostic side, we present the manifestation of changes in these
responses in patients with peripheral hearing loss, brainstem and cortical lesions,
auditory neuropathy (AN), and inner ear malformations.
In the last 25 years, hearing screening of newborns by ABR has been adopted by
many countries worldwide. Because of an increase in a combination of findings,
including the ABR, the syndrome of AN, as first reported by Kaga K, et al. and Starr
A, et al. in 1996, has gained the attention of many clinicians. Also, cochlear implants
for young infants with congenital deafness are performed worldwide. EABRs have
become a very valuable tool both in determining the integrity of the implant and in
evaluating cochlear nerve function. We herein discuss a few difficult cases of infants
with complicated inner ear malformations and/or cochlear nerve deficiencies to
illustrate the effectiveness of ABRs and EABRs as a diagnostic imperative.
I trust that with this book readers can gain an insight into auditory brainstem
physiology and its various pathologies, as well as hearing disorders, as revealed by
the ABR and the EABR. In addition, the EABR is essential in the programming of
cochlear implant electrodes.
I thank Ms. Kayoko Sekiguchi, B.S., for her indefatigable contributions for her
secretarial work and Dominic W. Hughes, Ph.D. for his scientific acumen and for
editing this manuscript in order for us to publish this book within 2 years.
Tokyo, Japan Kimitaka Kaga
January, 2022
v
Contents
Part I Introduction
1 History of ABR and EABR. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
Kimitaka Kaga
Part II ABRs
2 Origins of ABR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23
Kimitaka Kaga and Dominic W. Hughes
3 Gestational Development of the Human Auditory System
Including the Cochlea and the Central Auditory Pathways . . . . . . . . . 39
Kimitaka Kaga
4 ABR Recording Technique and the Evaluation
of Peripheral Hearing Loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51
Kimitaka Kaga and Dominic W. Hughes
5 Auditory Neuropathy Spectrum Disorders . . . . . . . . . . . . . . . . . . . . . . . 67
Kimitaka Kaga
6 Normalization and Deterioration of Auditory
Brainstem Response (ABR) in Child Neurology . . . . . . . . . . . . . . . . . . 77
Makiko Kaga
7 Hypoxic and Anoxic Brain Damage . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169
Kimitaka Kaga
8 Only Wave I, II of the ABR with Residual Hearing Acuity . . . . . . . . . . 177
Kimitaka Kaga
9 Auditory Agnosia and Later Cortical Deafness
in a Child over 29 Years Follow-Up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187
Kimitaka Kaga and Mitsuko Shindo
vii
viii Contents
Part III Electrically Evoked ABRs (EABRs)
10 Electrically Evoked Auditory Brainstem Responses (EABRs),
Recording Techniques, Normal (Control)
and Abnormal Waveforms of the EABR . . . . . . . . . . . . . . . . . . . . . . . . . 197
Kimitaka Kaga and Chieko Enomoto
11 Inner Ear Malformation and Cochlear Nerve Deficiency . . . . . . . . . . . 217
Shujiro B. Minami
12 Auditory Neuropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 229
Makoto Hosoya, Shujiro B. Minami, and Kimitaka Kaga
Part IV Particular Topics
13 Common Cavity Deformity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 247
Kimitaka Kaga
14 Galvanic VEMP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 257
Kimitaka Kaga
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 265
Abbreviations
ABR Auditory brainstem response
AABR Automated auditory brainstem response
ACS Air conduction stimulation
ACTH Adrenocorticotropic hormone
AE Anoxic encephalopathy
AEP Auditory evoked potential
AFD Appropriate for date
ALD Adrenoleukodystrophy
AN Auditory neuropathy
ANSD Auditory neuropathy spectrum disorder
AP Action potential
APGAR Appearance, pulse, grimace, activity, and respiration named from Dr.
Virginia Apgar
AR Autosomal recessive
ASD Atrial septal defect
ASSR Auditory steady-state response
AXD Alexander’s disease
BCV Bone conducted vibration
BMT Bone marrow transplantation
BOA Behavioral observation audiometry
CAG Cerebral arterial angiography
CC Chief complaint
CHD Congenital heart disease
CI Cochlear implant
CM Cochlear microphonic
CMTD Charcot-Marie-Tooth disease
CMV Cytomegalovirus
CN Cochlear nucleus
CN Cochlear nerve
CND Cochlear nerve deformity
CNV Contingent negative variation
ix
x Abbreviations
COR Conditioned orientation response audiometry
CP Cerebral palsy
CPA Cardiopulmonary arrest
CSF Cerebral spinal fluid
CT Computerized tomography
CVN Cochlear vestibular nerve
DCN Dorsal cochlear nucleus
DIC Disseminated intravascular coagulation
DPOAE Distortion product otoacoustic emission
DQ Developmental quotient
DRPLA Dentato-rubro-Pallido-luysian Atrophy
EABR Electrically evoked auditory brainstem response
ECMO Extracorporeal membranous oxygenation
EEG Electroencephalography
ECoG Electrocochleogram
EMG Electromyogram
EP Epilepsy
ERG Electroretinogram
ERT Enzyme replacement therapy
F-VEP Flash-Evoked visual evoked potential
GA Gestational age
GLD Globoid Cell leukodystrophy
GVS Galvanic vestibular stimulation
HSAN Hereditary sensory-autonomic neuropathy
HSCT Hematopoietic stem cell transplantation
HSMN Hereditary sensory-motor neuropathy
IA Infantile autism
IAC Internal auditory canal
IAR Interaural asymmetry ratio
IC Inferior colliculus
ICH Intracerebral brain hemorrhage
ICU Intensive care unit
ID Intellectual disability (= mental retardation)
IPL Inter peak latencies
LL Lateral lemniscus
LLR Long latency response
MAP Mean arterial pressure
MAS Massive aspiration syndrome
MELAS Mitochondrial encephalomyopathy with lactic acidosis
MERRF Mitochondrial encephalomyopathy with ragged red fibers
MGB Medial geniculate body
MLC Middle latency component
MLD Metachromatic leukodystrophy
MLR Middle latency response
MMN Mismatch negativity