ABRs and Electrically Evoked ABRs in Children

Preface
Contents
Abbreviations
Part I: Introduction
	Chapter 1: History of ABR and EABR
		1.1 Auditory Evoked Potentials (AEPs)
		1.2 Electrically Auditory Brainstem Responses (EABRs)
		References
Part II: ABRs
	Chapter 2: Origins of ABR
		2.1 Origins of the ABR
			2.1.1 Experiment 1: Whole Brainstem Mapping Study in the Cat
			2.1.2 Experiment 2: Medial Geniculate Body Mapping Study in the Cat. ABR Wave P6
		2.2 Experiment 3: Origins of the Six Waves of the ABR Waveform in the Cat as Determined by Depth Recordings and Lesion Studies
			2.2.1 Cochlear Nerve (Eighth Cranial Nerve): ABR Wave P1
			2.2.2 Cochlear Nucleus: ABR Wave P2
			2.2.3 Superior Olivary Complex: ABR Wave P3
			2.2.4 Inferior colliculus in the Midbrain: ABR Wave P4 and P5
			2.2.5 Medial Geniculate Body: ABR Wave P6
			2.2.6 Comments
		References
	Chapter 3: Gestational Development of the Human Auditory System Including the Cochlea and the Central Auditory Pathways
		3.1 Gestational Development of Inner Ear
		3.2 Development of the Inner Ear Hair Cells and the Ganglion Cells
			3.2.1 Formation of the Otic Vesicle and the Development of the Inner Ear During Gestation
			3.2.2 Differentiation of Inner Ear Hair Cells within the Organ of Corti
			3.2.3 Differentiation of the Spiral Ganglion Cells within the Cochlea
			3.2.4 Innervation of the Inner Ear Hair Cells
		3.3 Development of the Central Ascending Auditory Pathway
			3.3.1 Gestational Development of Myelination of the Human Cochlear Nerve
			3.3.2 Development of the Rhombencephalon (Medulla Oblongata)
				3.3.2.1 The Developmental Origin of the Cochlear Nucleus and Its Neural Proliferation
				3.3.2.2 Migration of the Cochlear Nucleus During Fetal Development
				3.3.2.3 Differentiation Between the Development of the Primary and Secondary Cochlear Nuclei
				3.3.2.4 Myelination of the Auditory Neural Substrate
		3.4 Development of the Mesencephalon (Inferior Colliculus)
			3.4.1 Differentiation of the Inferior Colliculus in the Midbrain
			3.4.2 Myelination of the Inferior Colliculus
		3.5 Development of the Diencephalon (Medial Geniculate Body)
			3.5.1 Differentiation of the Medial Geniculate Body
			3.5.2 Myelination of the Medial Geniculate Body
			3.5.3 Development of the Telencephalon (the Auditory Cortex)
			3.5.4 Neuronal Differentiation within the Auditory Cortex
			3.5.5 Myelination of the Auditory Radiation
			3.5.6 Cytoarchitectural and Axonal Maturation of the Human Auditory Cortex
		References
	Chapter 4: ABR Recording Technique and the Evaluation of Peripheral Hearing Loss
		4.1 Requirements Necessary to Obtain ABR Recordings
			4.1.1 Location (Montage) and Application of ABR Electrodes
			4.1.2 Description of the Click Stimuli Employed to Provoke the ABR
		4.2 ABR Wave V Peak Latency-Intensity Curves
			4.2.1 ABRs from Normal Hearing Subjects
		4.3 Peripheral Hearing Loss
			4.3.1 Conductive Hearing Loss as it Effects the ABR
			4.3.2 Sensorineural Hearing Loss as Manifest by the ABR
		4.4 Audiogram Patterns Compared to ABR Findings
			4.4.1 ABR Findings of Patients with a Low-Frequency Hearing Loss but with Normal High-Frequency Hearing
			4.4.2 ABR Recordings from Each Ear of a Patient with a Typical Noise-Induced Hearing Loss (an Audiometric Concave Frequency Loss Pattern)
		4.5 ABR Recordings from Patients with High-Frequency Hearing Loss with Otherwise Normal Hearing Thresholds, Audiometrically, at the Low and Middle Frequencies
		4.6 Unusual ABR Findings
			4.6.1 A “Bump” in the ABR Appearing within the Expected Latency Range of Wave I (~ 1 Msec) with no Subsequently Evoked ABR Waves
			4.6.2 Unusual ABR Findings Wherein Only Wave I or Waves I and II Are Evoked
			4.6.3 The Influence of Incomplete Brainstem Maturation in Preterm Infants and the Progression of Their ABRs with Development
		References
	Chapter 5: Auditory Neuropathy Spectrum Disorders
		5.1 The First Report of Auditory Nerve Disease and Auditory Neuropathy in 1996
		5.2 Auditory Neuropathy Spectrum Disorders (ANSD) in Newborns
		5.3 Our Classification of ANSD in Newborns, Infants, and Children
		5.4 ANSD for Type III and Cochlear Implantation
		5.5 Genetic Mutation
		5.6 Adults AN and Newborns ANSD
		References
	Chapter 6: Normalization and Deterioration of Auditory Brainstem Response (ABR) in Child Neurology
		6.1 Normalization and Deterioration of ABR in Children
			6.1.1 What Constitutes the Difference Between Normal and Abnormal ABR?
			6.1.2 What Causes the Normalization and Deterioration of the ABR?
		6.2 Normalization
			6.2.1 Normal Development of the ABR
			6.2.2 Clinical Appreciation at NICU
			6.2.3 Improvement of Conductive Hearing Loss
			6.2.4 ABR Changes Pre- and Post-surgical Resection of Tumors on the Brainstem or Cerebellar
			6.2.5 Normalization of the ABR as a Result of Effective Medical Treatment
			6.2.6 Normalization of the ABR of Unknown Etiology
				6.2.6.1 Case Reports Showing Normalization of the ABR of Unknown Etiology
					Five Patients Without Chromosomal Abnormalities
					Nine Patients with Chromosomal Abnormalities Including Down Syndrome and ABR Improvement
				6.2.6.2 What Could Be the Underlying Cause/Causes of Normalization of the ABR in These Patients with Chromosomal Aberration?
			6.2.7 Recovery from Severe Anoxic Events with Normalization of the ABR
				6.2.7.1 ABRs in Anoxia
				6.2.7.2 Case Reports of Anoxic Patients with Unexpected ABR Recovery
				6.2.7.3 Discussion of Anoxic Patients with Unexpected ABR Recovery
		6.3 Deterioration of ABR in Child Neurology: Neurological Assessment of Childhood Deterioration of the ABR
			6.3.1 Loss or Decline of Hearing Acuity
				6.3.1.1 Intrauterine Infections
				6.3.1.2 Neurocutaneous Syndrome as a Category of Genetically Determined Systemic Diseases with Central Nervous System Involvement
				6.3.1.3 Progressive Hearing Loss in NICU Patients and the Graduates from NICU
					Patients with Hearing Loss Discovered in NICUs and in NICU Graduates
					Case Presentation of Patients With or Without PPHN and Delayed Hearing Impairment
					The Cause of the Later Deterioration of Hearing in Patients
			6.3.2 Deterioration of ABR in Degenerative Diseases
				6.3.2.1 Slow Virus Infection
				6.3.2.2 Subacute Sclerosing Panencephalitis
			6.3.3 Leukodystrophies
				6.3.3.1 Adrenoleukodystrophy (ALD)
				6.3.3.2 Krabbe Disease, Globoid Cell Leukodystrophy (GLD)
				6.3.3.3 Metachromatic Leukodystrophy (MLD)
				6.3.3.4 Alexander Disease (AXD)
				6.3.3.5 Pelizaeus-Merzbacher Disease (PMD)
				6.3.3.6 Leukodystrophy of Unknown Origin
			6.3.4 Lysosomal Diseases
				6.3.4.1 Lipidosis
					Tay-Sachs Disease
					Metachromatic Leukodystrophy (MLD)
					Globoid Cell Leukodystrophy (GLD), Krabbe Disease
					Mucopolysaccaridosis (MPS)
					Gaucher Disease
			6.3.5 Degenerative Diseases of Cerebral Gray Matter
				6.3.5.1 Neuronal Ceroid Lipofuscinosis
				6.3.5.2 Nieman-Pick Disease Type C (NPC)
				6.3.5.3 Mitochondrial Diseases
			6.3.6 Degenerative Diseases Mainly Effecting the Cerebellum and Spinal Cord (Spinocerebellar Degeneration, SCD)
				6.3.6.1 Dentate-Rubro-Pallido-Luysian Atrophy
				6.3.6.2 Joubert Syndrome
			6.3.7 Degenerative Disease of Peripheral Nervous System
				6.3.7.1 Charcot-Marie-Tooth Disease
				6.3.7.2 Giant Axonal Neuropathy
		References
	Chapter 7: Hypoxic and Anoxic Brain Damage
		7.1 Hypoxic and Anoxic Brain Damage in Infants and Children
		7.2 Hypoxic Brain Damage in Near-Suffocation (Hypoxic) Group
		7.3 Brain Damage in the Near-Drowning (Anoxic) Group
		7.4 Pathophysiology of Hypoxic and Anoxic Brain Damage Due to Near-Suffocation and Near-Drowning Accidents
		7.5 Effects of Deep Hypothermia and Circulatory Arrest on the ABR and the EEG Undergoing Cardiac Surgery
		7.6 ABRs from the Cat During Artificial Respiratory Arrest and Restoration
		References
	Chapter 8: Only Wave I, II of the ABR with Residual Hearing Acuity
		8.1 Introduction
		8.2 Pelizaeus–Merzbacher Disease
		8.3 Adrenoleukodystrophy
		8.4 Metabolic Disease
			8.4.1 Gaucher’s Disease
			8.4.2 Tay–Sachs Disease
				8.4.2.1 Case Report
		8.5 Brainstem Tumor
			8.5.1 Case Report
		References
	Chapter 9: Auditory Agnosia and Later Cortical Deafness in a Child over 29 Years Follow-Up
		9.1 Bilateral Auditory Cortex Lesions and Hearing
		9.2 Case Report: Long-Term Follow-Up of a Pediatric Patient over 29 Years Who Manifested Auditory Agnosia and Later Cortical Deafness Caused by Herpes Encephalitis
		9.3 Schema of the Auditory Cortex or the Auditory Radiation Lesions in Auditory Agnosia and Cortical Deafness
		9.4 Cortical Deafness Following Auditory Agnosia
		References
Part III: Electrically Evoked ABRs (EABRs)
	Chapter 10: Electrically Evoked Auditory Brainstem Responses (EABRs), Recording Techniques, Normal (Control) and Abnormal Waveforms of the EABR
		10.1 Two Types of EABRs Recordings for Patient
		10.2 EABR Recording Protocol
			10.2.1 Montage of Skin Needle Electrodes for Recording EABRs
			10.2.2 Electrical Stimulation from Intracochlear Electrodes
			10.2.3 Recording Conditions
		10.3 Measurement of the Amplitudes and Latencies of eIII and eV
		10.4 Typical Waveform of the EABR in Control (Normal) Subjects
			10.4.1 EABRs Waveforms Recorded from Each of the Twelve Intracochlear Electrodes of an Intracochlear Implant
			10.4.2 Amplitudes and Latencies of EABR Waves eIII and eV Evoked by Independent Stimulation of Each of the Twelve Nodes of the Intracochlear Electrode
		10.5 Comparison of the Thresholds of EABR Wave V Recorded from 6 Odd Numbered Nodes of an Implanted Electrode in Children and the Distribution of the Recorded Latencies of eII, eIII, and eV from the Apical Electrode in Adults
			10.5.1 Thresholds of EABR Waves eV Following Six Odd Numbered Stimulated CI Electrode Nodes of the Twelve Nodes Available
			10.5.2 Latencies of eII, eIII, and eV Following Stimulation of the Apical Electrode Node of the CI
		10.6 Developmental and Aging Influence on EABRs Recorded by Selected Three Stimulus Electrodes as Routine Examination
			10.6.1 Typical EABR of a One-Year-Old Female
			10.6.2 Comparison of EABR Wave eV Latencies from the Contralateral Mastoid (Channel 1) and Nape Recordings (Channel 2) from Five Different Age Groups: One-Year-Olds, Infants, Children, Adults, Elderly
		10.7 Comparison of EABR Waves Recorded from the Bionics and the Cochlear Companies’ CI
		10.8 Abnormal Waveform Classification of EABRs During Cochlear Implantation Under General Anesthesia
		References
	Chapter 11: Inner Ear Malformation and Cochlear Nerve Deficiency
		11.1 Introduction
		11.2 A Novel and Simple Grading
			11.2.1 Grading According to Observation of Modiolus Deficiency and/or Cochlear Nerve Deficiency
			11.2.2 Patient Characteristics of Each Different Grade
			11.2.3 EABR Responses and Categories of Auditory Performance (CAP) Scores
		11.3 EABR Waves of Patients in Each Grade
			11.3.1 EABR Waves of Patients in Grade I (Table 11.1)
			11.3.2 EABR Waves of Patients in Grade II (Table 11.2)
			11.3.3 EABR Waves of Patients in Grade III (Table 11.3)
			11.3.4 EABR Waves of Patients in Grade IV (Table 11.4)
		References
	Chapter 12: Auditory Neuropathy
		12.1 Introduction
		12.2 Auditory Neuropathy
			12.2.1 Pathophysiology of Auditory Neuropathy
			12.2.2 Genetics of Auditory Neuropathy
			12.2.3 Cochlear Implantation for Auditory Neuropathy
		12.3 Electrical Auditory Brainstem Responses and Auditory Neuropathy
			12.3.1 The Usefulness of Electrical Auditory Brainstem Responses in Auditory Neuropathy
			12.3.2 Electrical Auditory Brainstem Responses in Genetic Auditory Neuropathy
			12.3.3 Electrical Auditory Brainstem Responses in Patients with DIAPH3 Mutation
			12.3.4 Electrical Auditory Brainstem Responses in Patients with OTOF Mutations
			12.3.5 Electrical Auditory Brainstem Responses in Patients with OPA1 Mutations
			12.3.6 Electrical Auditory Brainstem Responses in Patients with Demyelinating Disease
			12.3.7 New Classification for Electrical Auditory Brainstem Responses in Auditory Neuropathy
		12.4 Conclusion
		References
Part IV: Particular Topics
	Chapter 13: Common Cavity Deformity
		13.1 Definition and Embryological View
		13.2 CT and MRI of Common Cavity Deformities
		13.3 EABRs of Common Cavity Deformities
		13.4 Latencies and Thresholds of EABRs
		13.5 Postoperative Hearing and Speech Development
		13.6 Developmental Changes in the Vestibular Ocular Reflex (VOR) and in the Acquisition of Postural Control in Infants with Common Cavity Deformities
		13.7 Conclusion
		References
	Chapter 14: Galvanic VEMP
		14.1 Auditory VEMPs and Galvanic (Electrically Stimulated) VEMP
		14.2 Stimulation and Recording of Galvanic VEMPs
			14.2.1 Recording
			14.2.2 Galvanic Stimulation
		14.3 Typical Response of Galvanic VEMP
		14.4 Galvanic VEMP in Auditory Neuropathy
		14.5 Galvanic VEMPs from Cochlear Implants
		References
Index