Encyclopedia of Audiology and Hearing Research (4 Volume Set)

Contents
Preface
Volume 1
Chapter 1
Inner Ear Endothelial Dysfunction Due to Oxidative Stress: A Possible Role in the Pathogenesis of Sensorineural Hearing Loss
	Abstract
	Introduction
	Endothelial Dysfunction and Inner Ear
		Experimental Data
		Clinical Evidences
	Conclusion
	References
Chapter 2
Hearing Screening for School Children
	Abstract
	Development of School Hearing Screening
	Goals of School Hearing Screening
	Pure Tone Screening Audiometry
	Periodicity of Screening
	Program Management
		Test Environment
		Accountability and Other Concerns
	Alternative Screening Methods
		Tympanometry
		Otoacoustic Emissions
		Questionnaires
		Modifications to Traditional Pure Tone Screening
		Other Methods
	Follow-Up Actions
	Future Considerations
		Screening for Central Auditory Processing Disorder
			Defining CAPD
			Screening for CAPD
			How to Screen for CAPD
			Tools Used to Screen for CAPD
			Conclusion
		Noise-Induced Hearing Loss
		Telehealth
		Genetic Screening
	Conclusion
	References
Chapter 3
Working with Learners with Hearing
Loss in STEM
	Abstract
	Introduction
	Supporting Families to Provide STEM Development Success
	Effective Early Intervention Programs Supporting  STEM Knowledge and Skills Development
	Fostering STEM Knowledge and Skills Using Information Communication Technology and Software
	Effective Teaching Strategies and Study Habits on  STEM Skills in Learners with Hearing Loss
	Conclusion
	References
Chapter 4
Hearing and Cognitive Outcomes  of Cochlear Implantation in the Elderly
	Abstract
	List of Abbreviations
	Introduction
	Methods
	Results
	Conclusion
		Competing Interests
	References
Chapter 5
Effects of Impulse Noise on Hearing  in Members of the Police Special
Operations Battalion
	Abstract
	Introduction
	Study on Hearing of the Military Police
	Research Process
		1) Noise Level for Firearms
		2) Knowledge about Hearing Health by Police Officers
		3) Auditory Profile of Police Officers
	HearingPreservation for Officers Exposed to Firearms Noise
	Conclusion
	References
Chapter 6
Hearing Health and Stress for  Military Police
	Universidade Tuiuti do Paraná, Brazil
	Abstract
	Introduction
	Occupational Stress
	Studies on Stress in the Military Police in Brazil
	Final Considerations
	References
Chapter 7
Effectiveness of Hearing Protection Devices (HPD) in Activities with Firearms
	Abstract
	Introduction
	Types of HPD for Exposure to Firearms Noise
	Parameters of Comfort for Military HPD
	Evaluation of Attenuation Earmuff Type Protector  Used by Military
	Conclusion
	Appendices
		Questionnaire 1. Evaluation of Comfort Parameters
		Questionnaire 2.- Evaluation of Personal Hearing Protector
	References
Chapter 8
Hearing Impairment after Perinatal Asphyxia
	Abstract
	Introduction
	Part I. BAER Studies
		BAER Threshold and Perinatal Hypoxia or Hypoxia-Ischemia
		Change in BAER Threshold during the Neonatal Period
		Prevalence of Hearing Impairment during the Neonatal Period
		Postnatal Change in BAER Threshold
		Persistent or Permanent Hearing Impairment
	Part II. DOAEs Studies
		DPOAE Changes and Cochlear Impairment
		DPOAE Changes after Perinatal Asphyxia
	Part III. Auditory Neuropathy
	References
Chapter 9
“I Will Make a Difference”: Using the 5As Model to Improve Issues for Adults with Learning Disabilities and Hearing Loss
	Abstract
	1. Introduction
		1.1. Coexistence
		1.2. Hearing Loss with Learning Disabilities; Double Jeopardy
		1.3. Conceptualising the Issue; the 3As
			1.3.1. Understanding Access
			1.3.2. Understanding Assessment
			1.3.3. Understanding Aftercare
	2. Methodology
	3. Cycle 1 – Defining the Problem (See McShea et al., 2015)
		3.1. Recruitment of Caregivers
		3.2. Motivation and Expectations
		3.3. Knowledge of Hearing, Audiology and Hearing Aids
		3.4. Communication
		3.5. Training and Future Needs
		3.6. Perception of Audiology
		3.7. Symbolic Interactionism; A Theory
	4. Cycle 2 – Designing and Piloting a Solution  (See McShea, 2015)
		4.1. Traditional Approaches to Learning
		4.2. Experiential Learning; an Alternative Approach
		4.3. The Kolb Cycle of Experiential Learning (Kolb, 1984)
		4.4. Experiential Learning in Practice
		4.5. How Training Took Place
			4.5.1. Aim 1 – Increase Knowledge
			4.5.2. Aim 2 – Change Attitudes
				Key Element of Aim 2 - Stories and Storytelling
			4.5.3. Aim 3 – Long Term Change to Practice
				Key Element of Aim 3 - Pledges
	5. Cycle 3 – Evaluating the Solution (See McShea, 2015)
		5.1. Focus Group Discussions
		5.2. Knowledge Creation
		5.3. Translation to Practice
		5.4. Empowerment and Confidence
		5.5. Experience of Primary Care
	6. Cycle 4 – Investigated an Unexpected Issue
	(See McShea, 2015b)
		6.1. Requirements
	7. Discussion
		7.1. Contextualising the Findings
			Cycle 1
			Cycle 2
			Cycle 3
			Cycle 4
		7.2. What were the Key Elements in Ensuring the Research Aims were Met?
		7.3. A Multidisciplinary Team (MDT)
		7.4. Audiology’s Visibility within a Multidisciplinary Framework
		7.5. Creating an MDT for Audiology and People with Learning Disabilities
			Benefits to Caregivers
			Benefits to Primary Care
			Benefits to Audiology
		7.6. A Theoretical Model for the MDT
			Assembly
			Awareness
		7.7. Generalisability/Transferability of the Model
	Conclusion
	References
Chapter 10
Hearing Loss and Intellectual Disabilities
	Abstract
	1. Introduction
	2. Hearing Loss
		2.1. Cause of Hearing Loss
			2.1.1. Conductive Hearing Loss
			2.1.2. Sensori-Neural Hearing Loss
		2.2. Hearing Loss and Down Syndrome
		2.3. Hearing Loss and Other Causes of Intellectual Disabilities
	3. Accessing Audiology Services
		3.1. Referral Pathways
		3.2. Screening
		3.3. Suitability of Audiology Services
		3.4. Impact on Other Services
		3.5. Considerations for a Specialist Service Model
	4. Hearing Assessment
		4.1. Getting to know the Patient’s Auditory History
		4.2. Ear Health
		4.3. Behavioural Assessment
			4.3.1. Observation
			4.3.2. Audiometry
				Responses to No Sound
				No Responses to Any Sound
				Starting and Not Finishing
				Dislike of Headphones
			4.3.3. Visual Reinforcement Audiometry (VRA)
			4.3.4. Speech Discrimination Testing
		4.4. Electrophysiological Assessment
			4.4.1. Otoacoustic Emissions (OAEs)
			4.4.2. Auditory Brainstem Response Audiometry
			4.4.3. Cortical Evoked Response Audiometry
		4.5. Assessment Recommendations
	5. Rehabilitation
		5.1. Hearing Aids
			5.1.1. Setting Hearing Aids
			5.1.2. Adapting to Hearing Aids
		5.2. Alternative Rehabilitation
			5.2.1. Hearing Tactics
			5.2.2. Assistive Listening Devices
		5.3. Rehabilitation Recommendations
	Conclusion
	References
Chapter 11
Looking with Ears, Hearing with Eyes: Visual and Aural Interaction  in Cervantes and Shakespeare
	Abstract
	References
	About the Author
Chapter 12
Universal Newborn Hearing Screening in  the United States
	Abstract
	Introduction
	Epidemiology
	The history of Universal Newborn Hearing Screens (UNHS) in the United States
	Cost Benefit
	Conclusion
	Acknowledgments
	References
Chapter 13
Hearing Loss in Neonatal Intensive Care Units (NICUs): Follow-Up Surveillance
	Abstract
	Introduction
	Cytomegalovirus
	Bacterial Meningitis
	Head Injury
	Ototoxic Drugs
	Follow-Up Surveillance
	Conclusion
	References
Chapter 14
Endothelial Dysfunction, Microvascular Disease and Sensorineural Hearing Loss
	Abstract
	Introduction
	Sensorineural Hearing Loss and Endothelial Dysfunction
	Sudden Sensorineural Hearing Loss  and Endothelial Dysfunction
	Conclusion
	References
Chapter 15
Superoxide Dismutase  and Sensorineural Hearing Loss
	Abstract
	Introduction
	Superoxide Dismutase and the Inner Ear
	Superoxide Dismutase  and Noise-Induced Hearing Loss
	Superoxide Dismutase and Presbycusis
	Superoxide Dismutase and Sudden Sensorineural
	Hearing Loss
	Conclusion
	References
Chapter 16
Cardiovascular Risk Factors and Sensorineural Hearing Loss
	Abstract
	Introduction
	Sensorineural Hearing Loss and Cardiovascular
	Risk Factors
	Sudden Sensorineural Hearing Loss and Cardiovascular Risk Factors
	Conclusion
	References
Chapter 17
Audiology, Hearing Aids  and Cochlear Implants
	Hearing
	Audiological Evaluation: Air and Bone Conduction
	Audiogram/Speech Perception/Inter-Test Checks
	Nature of Hearing Loss
	Audiometric Findings for Different Ages/Pathologies
	Immittance Battery
	Special Tests
	Health Conditions and Hearing Loss
	Hearing Aids
	Terminology
	Hearing aid Components/Styles
	CROS/BICROS
	Osseointegrated Devices (OID)/ aka Bone Anchored Hearing Aids (BAHA)
	Middle Ear Implantable Hearing Aids
	Cochlear Implants (CI)
	Candidacy
	Assistive Listening Devices (ALD’s)
	References
Chapter 18
Hearing Loss: Conductive and Sensorineural
	Department of Otolaryngology, Saint Louis University School of Medicine,  Saint Louis, MO, US
	Sensorineural Hearing Loss
	Conductive Hearing Loss
	References
Chapter 19
Sign Acquisition and Development by Hearing Children with Autism Spectrum Disorders
	Introduction
	Communication Training Interventions
		Behaviour Modification Programmes
		Sign Communication Programmes
	Advantages of the Sign Modality
	Problems in Sign Acquisition and Use
		Delays in Implementing Intervention
		Deficits in Motor Skills and Imitation
		Relationships between Motor Skills and Language and  Communication Development
	Intervention Approaches
		The Simplified Sign System
		Aided Communication
	Conclusion
	Acknowledgments
	References
Chapter 20
Hyperbaric Oxygen Therapy in Sudden Sensorineural Hearing Loss
	Abstract
	Introduction
	Indications
	Side Effects and Complications
	Contraindications
	Related Articles
	References
Chapter 21
Aminoglycoside Mediated Ototoxicity and Hearing Loss in Cystic Fibrosis Patients:  An Unmet Medical Need
	Abstract
	Introduction
	Molecular Mechanisms Underlying Ototoxicity
	Alternative Approaches to Avoid  Aminoglycoside Ototoxicity
	Conclusion
	Acknowledgments
	References
Chapter 22
Low-Level Laser Therapy: Progress and Future Trends in Hearing Loss and Vestibular Dysfunction
	Abstract
	Introduction
	Role of LLLT in Hearing Loss (Animal Models)
	Role of LLLT in Hearing Loss in Human
	Treatment of Tinnitus
	Role of LLLT in Gene Therapy for Hearing Loss
	Role of LLLT in Vestibular Dysfunction
	Conclusion
	References
Volume 2
Chapter 23
Novel Deafness Genes and Mutations Identified by Next Generation Sequencing
	Abstract
	Introduction
	Whole Genome Sequencing
	Whole Exome Sequencing
	Targeted Deafness Gene Capture and NGS
	Challenge and Future
	References
Chapter 24
The Molecular Pathogenesis of Dominant Deafness-Onychodystrophy (DDOD) Syndrome
	1Department of Otolaryngology, Chinese PLA General Hospital,  Beijing, P.R.C.
	2Department of Otolaryngology, Emory University School of Medicine,  Atlanta, GA, US
	Abstract
	Introduction
		Pedigrees and Clinical Evaluations
		Evidence Support that a De Novo Mutation in ATP6V1B2 Causes the  DDOD Syndrome
		Atp6v1b2 Cochlea Knockdown Mouse Shows Hearing Loss
		Western Blot Analysis of Atp6v1b2 Shows Reduced Expression of Atp6v1b2  in the Spiral Ganglion Neurons and the Organ of Corti
		ATP6V1B2 C.1516 C>T is a Dominant Loss-of-Function Mutation, Causing Abnormal Acidification in Lysosomes
	Discussion
	References
Chapter 25
Association between Sensorineural Hearing Loss and Sleep-Disordered Breathing: Literature Review
	Abstract
	Introduction
	What Are the Mechanisms that Determine  the Audiological Damage in Patients with SDB?
	Literature Review
	Conclusion
	References
Chapter 26
Occupational Exposure to  Ototoxic Chemicals
	Abstract
	Introduction
	Organic Solvents
		Styrene
		Toluene
		Xylenes
		Dichloromethane
		Trichloroethylene
		Carbon Disulphide
		Solvent Mixtures
	Metals
		Lead
		Mercury (Methyl Mercury Chloride, Mercuric Sulfide)
		Cadmium
		Chromium
		Tin
		Arsenic
	Pesticides
		Organophosphates (OPs)
		Pyrethroids
	Other Chemicals
		Polychlorinated Biphenyls (PCBs)
		Carbon Monoxide (CO)
	Conclusion
	References
Chapter 27
Conduct Disorder in Children and
Youth with Hearing Impairment
	Abstract
	Conduct Disorder in Children and Youth  with Hearing Impairment
	Method
		Participants
		Instrument
			Procedure
	Results
		Discussion
		Limitations
		Praxis Implications
	Conclusion
	References
Chapter 28
Sudden Sensorineural Hearing Loss and Polymorphisms in Iron Homeostasis Genes
	Abstract
	Introduction
	Methods
	Iron Metabolism and Oxidative Stress
	Iron Metabolism and the Inner Ear
	Iron metabolism and Sudden Sensorineural Hearing Loss
	Conclusion
	References
Chapter 29
Chronic Tinnitus: Pith, Loudness, and Discomfort in Adults and Elderly Patients
	Abstract
	Introduction
	Methodology
	Results
		Discussion
	Conclusion
	References
Chapter 30
Effect of Hearing Loss on Traffic
Safety and Mobility
	Abstract
	List of Papers
	List of Abbreviations
	Concepts and Definitions
	Introduction
	Hearing Loss
		Assessment of Hearing Ability
	Cognition
		Working Memory
		Long-Term Memory
		Executive Functions
		Assessment of Cognitive Ability
			WM Capacity
			Processing Speed, Divided Attention, Selective Attention
		Hearing Loss, Cognition and Aging
		Cognitive Consequences of Aging
		Cognitive Consequences of HL
		Cognition and Traffic Safety
			Driver Behavior Models
			Motivational Models
	Traffic Safety
		Mobility and Quality of Life
		Effects of Aging on Driving Behavior
		Assessment of Driver Behavior
		Advanced Driver Assistance Systems
	General Aim and Research Questions
	Methods
		Ethical Considerations
		Methodological Challenges
			HL Population and Recruitment of Participants
			Driving Simulator Versus Real Driving
			Challenges with Cognitive Assessments
		Procedures and Validity
			Questionnaire Study
			Experimental Studies
			Pretests
			Secondary Task
			Performance Indicators
		Participants and Data Collection
		Design and Statistical Analyses
			Questionnaire Survey
			Driving Simulator Study
			Field Study
	Summary of Studies and Papers
		Study 1: A Questionnaire Survey
			Paper I: The Influence of Hearing Loss on Transport Safety and Mobility
				Purpose
				Method
				Results
				Conclusion
		Study 2: A Driving Simulator Study
			Purpose
			Method
			Paper II: Cognitive Workload and Driving Behavior in Persons with Hearing Loss
				Method
				Results
				Conclusion
			Paper III: Cognitive Workload and Visual Behavior in Elderly Drivers with  Hearing Loss
				Method
				Results
				Conclusion
		Study 3: A Field Study in Real Traffic
			Paper IV: Hearing Loss and a Supportive Tactile Signal in a Navigation System: Effects on Driving Behavior and Eye Movements
				Purpose
				Method
				Results
				Conclusion
	General Discussion
		Summary of Results
		Choice of Transportation
		Driving Behavior
		Visual Behavior
		Driver Assistance Systems
		Methodological Discussion
	Conclusion
	Suggestions for Future Research
	Acknowledgments
	References
Chapter 31
Genetics of Hearing Loss: Testing Methodologies and Counseling of Audiology Patients and Their Families
	Abstract
	1. Introduction
	2. DNA Provides the Genetic Code
		2.1. Structure of DNA
		2.2. DNA Is Packaged into Chromosomes
		2.3. Chromosomes Come in Pairs
	3. Patterns of Inheritance
		3.1. Autosomal Dominant
		3.2. Autosomal Recessive
		3.3. X-Linked
		3.4. Mitochondrial
	4. Syndromic Hearing Loss
		4.1. Syndromes with Autosomal Dominant Inheritance
			4.1.1. Stickler Syndrome
			4.1.2. CHARGE Syndrome
			4.1.3. Cornelia de Lange Syndrome
			4.1.4. Neurofibromatosis Type 2
			4.1.5. Branchio-oto-Renal Syndrome
			4.1.6. Waardenburg Syndrome
			4.1.7. Treacher Collins Syndrome
			4.1.8. Crouzon Syndrome
			4.1.9. Apert Syndrome
		4.2. Syndromes with Autosomal Recessive Inheritance
			4.2.1. Pendred Syndrome
			4.2.2. Usher Syndrome
			4.2.3. Jervell and Lange-Nielsen Syndrome
		4.3. Syndrome with X-Linked Inheritance: Alport Syndrome
		4.4. Syndromes with Mitochondrial Inheritance: MELAS and MERRF
		4.5. Down Syndrome: The Most Common Genetic Syndrome
	5. Nonsyndromic Hearing Loss
		5.1. Nonsyndromic Hearing Loss: Autosomal Recessive Inheritance
			5.1.1. Connexin 26 Hearing Loss Is Caused by the GJB2 Gene
			5.1.2. Other Genes
				5.1.2.1. SLC26A4
				5.1.2.2. MYO15A
				5.1.2.3. OTOF
				5.1.2.4. CDH23
				5.1.2.5. TMC1
				5.1.2.6. TMPRSS3
				5.1.2.7. TECTA
		5.2. Nonsyndromic Hearing Loss: Autosomal Dominant inheritance
			5.2.1. WFS1
			5.2.2. KCNQ4
			5.2.3. COCH
			5.2.4. GJB2
		5.3. Nonsyndromic Hearing Loss: X-Linked Inheritance
		5.4. Nonsyndromic Hearing Loss: Mitochondrial Inheritance
	6. Technologies in Genetic Testing
		6.1. Cytogenetics
			6.1.1. Classical Cytogenetics: Creation of a Karyotype
			6.1.2. FISH: A Molecular Cytogenetic Technique
			6.1.3. Array CGH: Molecular Cytogenetics of the Entire Genome
		6.2. Polymerase Chain Reaction and Gel Electrophoresis
		6.3. DNA Sequencing
			6.3.1. Genome Sequencing
			6.3.2. Exome Sequencing
	7. Making a Genetics Referral
		7.1. When Should a Genetics Referral Be Made?
		7.2. Benefits of Genetic Testing
		7.3. Impacts of a Positive Genetic Test Result
		7.4. Limitations of Genetic Testing
		7.5. Testing Recommendations
	Conclusion
	References
Chapter 32
Audiological and Surgical Outcome after Cochlear Implant Revision Surgery
	Abstract
	Introduction
	Causes of Reimplantation
		Device Failure
		Scalp Infection
		Electrode Extrusion
		Cochlear Implant Electrode Misplacement
		Magnet Displacement
	Surgical Steps
	Surgical Outcome
	Audiological Outcome
		Impedance
		Speech Recognition
	Conclusion
	References
Chapter 33
Posturology: The Scientific Investigation of Postural Disorders
	Abstract
	Introduction
	Human Posture Evaluation
	Patient History
	Visual Postural Analysis
	Postural Clinical Tests
	Posturography
	Intervention Programs
	References
Chapter 34
The Influence of Otovestibular System  on Body Posture
	Abstract
	The Otovestibular System
	Otovestibular Sensory Informations
	Otovestibular Disorders and Body Balance
	Posturography: A Quantitative Assessment
	of Body Balance
	Influence of Otovestibular System on Body Balance
	The Role of Physical Activity on Body Balance
	References
Chapter 35
Auditory Brainstem Response and  Frequency Following Response in Patients with Sickle Cell Disease
	Abstract
	Introduction
	Methods
	Results
	Discussion
	References
Chapter 36
The Relationship between Self-Reported Restriction in Social Participation, Self-Reported Satisfaction/Benefit and the Time of Use of Hearing Aids
	Abstract
	Introduction
	Methods
	Result
	Discussion
	Conclusion
	References
Volume 3
Chapter 37
Telecommunications Relay Service:  FCC Should Strengthen Its Management of Program to Assist Persons with Hearing or Speech Disabilities*
	Why GAO Did This Study
	What GAO Recommends
	What GAO Found
	Abbreviations
	Background
	Total TRS Minutes and Costs Have Grown Significantly since 2002 due to Internet-Based TRS and Increased Usage
	FCC Has Not Established Performance Goals and Related Performance Measures for the TRS Program
	FCC Has Designed Some Internal Controls but Lacks  a Comprehensive Internal Control System to Manage Program Risks
		An Internal Control System Helps Assure That Program Goals Are Met
		FCC Has Designed Some TRS Internal Controls That Address Compliance and Reporting
		FCC Lacks a Comprehensive Internal Control System to Manage TRS Program Risks
	Stakeholders Cited Several Challenges to TRS Service Quality and Competition
		Stakeholders Identified Challenges to Providing High Quality Services
			Skill-Based Routing
			Interpreter Accuracy
			TRS Reimbursement Rates
		Stakeholders Identified Challenges to Encouraging Competition  and Technological Innovation
			TRS Rate Reductions
			Lack of Compensation for Marketing and Outreach and Research and Development
			Lack of Interoperability among VRS Providers
	Conclusion
	Recommendations for Executive Action
	Agency and Third-Party Comments
	Appendix I: Objectives, Scope, and Methodology
	Appendix II: Survey of Telecommunications Relay  Service Providers
	Appendix III: GAO Analysis of Provider Concentration in TRS Product Markets, 2008–2014 Rate Years
		End Notes
		End Note for Appendix I
Chapter 38
Video Relay Service:
Program Funding and Reform(
	Summary
	Introduction: How Video Relay Service Works
	Program Overview
		Management
		VRS Provider Service Standards
		Funding
		Provider Compensation/Reimbursement
	June 2013 Report and Order and Further Notice  of Proposed Rulemaking
		Further Notice of Proposed Rulemaking
	Policy Considerations
		Congressional Considerations
	Appendix. History of Proposed Changes to the  VRS Program, 2010-2013
		October 2012 FCC Request for Additional Comment
			Proposed Changes to VRS Access Technology
			Proposed Enhancements to the iTRS Database
			Proposed Rate Changes
		Opposition to the October 2012 Proposed Reform Options
			Proposed Changes to VRS Access Technology
		Proposed Enhancements to the iTRS Database
			Proposed Rate Changes
		End Notes
Chapter 39
Sensorineural Hearing Loss Secondary  to Otitis Media
	Abstract
	Introduction
	Epidemiology
	Bacteriology
	Diagnosis
	Physiopathology
	Conclusion
	References
Chapter 40
Sudden Sensorineural Hearing Loss: Pathophysiology, Diagnosis, Treatment Options, and Prognostic Factors
	Abstract
	Introduction
	Pathogenesis
		Viral Theory
		Vascular Theory
		Autoimmunity Theory
		Membrane Rupture Theory
		Toxicity Theory
	Diagnosis
	Treatment
		Anti-Inflammatory Treatment
		Therapy to Increase Cochlear Blood Flow (CBF)
		Anti-Viral Therapy
		Shotgun Therapy
		Other Treatment Modalities
	Prognosis
	Conclusion
	Acknowledgments
	References
Chapter 41
Up-to-Date in Auditory Neuropathy Spectrum Disorder: Clinical, Diagnostic and Therapeutic Features
	Abstract
	Introduction
	Epidemiology
	Diagnostic
	Rehabilitation
	Conclusion
	References
Chapter 42
Genetic Kidney Diseases with  Sensorineural Hearing Loss
	Abstract
	1. Introduction
	2. Hearing Loss and Alport Syndrome
		Introduction
		Physiopathology
		Clinic
		Treatment
		Conclusion
	3. Hearing Loss and MYH9-Related Disorders
		Introduction
		Physiopathology
		Clinic
		Treatment
		Conclusion
	4. Hearing Loss and Chronic Kidney Disease
	References
Chapter 43
Stepwise Approach to the Diagnosis of Hearing Loss in Children
	Abstract
	Introduction
	Epidemiology and Etiopathogenesis
	Diagnosis
		Universal Neonatal Hearing Screening
		Audiological Evaluation
		Infectious Disease Assessment
		Neuroimaging
		Genetic Assessment
		Other Specific Evaluations
	Conclusion
	References
Chapter 44
Hearing Loss After Traumatic Conditions: Histopathology and Clinical Features
	Abstract
	Introduction
	The Eustachian Tube
	Barotrauma
		Middle Ear Barotrauma
		Inner Ear Barotrauma
	Head Trauma and Temporal Bone Fracture
	Conclusion
	References
Chapter 45
Idiopathic Sudden Sensorineural Hearing Loss and Cardiovascular Risk Factors
	Abstract
	Introduction
	Idiopathic Sudden Sensorineural Hearing Loss (ISSNHL) and Cardiovascular Risk Factors
	ISSNHL Cardiovascular Risk Factors and Oxidative Stress: Physiopathology of the Damage
	Conclusion
	References
Chapter 46
Hearing Loss of Volga-Ural Region in Russia
	Abstract
	References
Chapter 47
Sudden Sensorineural Hearing Loss,  an Invisible Male: State of the Art
	Etiology
	Clinical Diagnosis
	Pathophisiology of the Sudden Hearing Loss
	Pathophisiology of the Sudden Hearing Loss:  Vascular Theory
	Pathophisiology of the Sudden Hearing Loss: Viral Theory
	Pathophisiology of the Sudden Hearing Loss:  Autoimmune Theory
	Treatment
	Prognosis
	Conclusion
	References
Chapter 48
The Influence of Sounds in Postural Control
	Abstract
	Introduction
	Anatomical Elements of Sound and Postural Control
	Sound and Inner Ear
	The Influence of Sound on Postural Control
	References
Chapter 49
Chronic Otitis Media and Hearing Loss
	Abstract
	1. Introduction
	2. Otitis Media with Effusion
	3. Chronic Otitis Media
	4. Chronic Otitis Media with Cholesteatoma
	5. Chronic Otits Media Treatment
		5.1. Tympanoplasty and Ossicular Reconstruction
		5.2. Bone – Anchored Hearing Aid and Coclhear Implant
	6. Impact of Conductional Hearing Loss  in Language Development
	References
Chapter 50
Binaural, Sequential or Simultaneous Cochlear Implants in Children: A Review
	Abstract
	Introduction
	Cochlear Implants (CI)
	CI Indications in Children
	CIs in Children with Inner Ear Malformations
	CI in Children and other Disabilities
	Bilateral CIs in Children
	Conclusion
	References
Chapter 51
Virtual Reality for Cochlear  Implant Surgery
	Abstract
	Introduction
	What Is the Virtual Reality Simulation?
	Challenges in Surgical Training
	Virtual Reality Surgical Training
	Training in Temporal Bone Surgery
	Virtual Reality Temporal Bone Simulators
		Current Systems
		Validity of Simulator Systems
		Efficacy of Simulators on Skills Improvement
	Cochlear Implantation
	Current Technology for Cochlear Implant Surgery
		Virtual Guidance for Cochlear Implantation
		Case-Specific Virtual Reality Surgery
		Real-Time Modeling Electrode Insertion
		Who Can Get the Benefit of Virtual Reality System for  Cochlear Implant Surgery?
	Conclusion
	References
Chapter 52
Cross-Modal Plasticity in Deaf Children with Visual-Impairment: Electrophysiological Results after  Long-Term Use of Cochlear Implants
	Abstract
	Introduction
	Methods
	Results
	Discussion
	References
Volume 4
Chapter 53
Anatomy and Physiology of the Peripheral and Central Auditory System
	Abstract
	Introduction
		1. Anatomical Bases of Hearing: An Overview
		1.1.  The Ear
			External Ear
			Middle Ear
			The Inner Ear
		1.2. The Vestibulocochlear Nerve
		1.3 The Cochlear Nuclei
		1.4.The Auditory Cortex
	2.Physiology of the Auditory System
		2.1 Outer and Middle Ear Actions: Funneling and Conduction of Sound
		2.2 Inner Ear Function: Transduction of Sound
		2.3 Hair Cells Functions
	3. Role of Nervous System
		3.1. Auditory Nerve
		3.2. Descending Control on Inner Ear
		3.3. Central Auditory Pathways
		3.4. Auditory Cortex
	References
Chapter 54
Genetics in Sensorineural Hearing Loss
	Abstract
	Introduction
	Hearing Loss and Its Genetics
		Non-Syndromic Hearing Loss (Approximately 65% of All Genetic Causes  of Hearing Loss): Audioprofiles of Dominant and Recessive Patterns
		Syndromic Hearing Loss, without Congenital Craniofacial Findings  and Recessive Inheritance Pattern
		Pendred Syndrome (Prevalence 7,5:100000, approximately 5% of Cases  of Congenital Hearing Loss), otherwise the FOXI1-SLC26A4/ KCNJ10 Genetic Variants Responsible for Ions Disorders in the Inner Ear)
		Usher Syndrome (Ciliopathies Reflecting the Potential Effects of  Variations in the Genes Encoding Actin-Based Structures and  Tip Links in Inner Ear Cells)
		Jervell and Lange-Nielsen Syndrome (Prevalence 0.3: 100,000), or Genetic Variants of KCN1/KCNE1 Genes Responsible for Ions Disorders in the Inner Ear
		Syndromic Hearing Loss with Congenital Craniofacial Findings  and Dominant Inheritance Pattern
		BOR Syndrome and EYA1 Related Disorders (or Branchial Defects Potentially Resulting from Genetic Variants in EYA1, SIX5, and SIX5, Genes on the Axis of the Tbx1-Six1/Eya1-Fgf8 Genetic Pathway)
		CHARGE Association (or Overlapping Features with DiGeorge Syndrome and Other Branchial Defects Resulting from Genetic Variations in the SMAD1/CHD7-FGF8/BMP Family/WNT1-OTX2-FOXA2-TBX1  Genetic Pathways)
		Mutations in the MITF Pathway (Responsible for Waardenburg Syndrome)
	Conclusion: Perform Simple Tasks  with the Highest Attention
	References
Chapter 55
Congenital Sensorineural Hearing Loss
	Abstract
	Introduction
	Non Syndromic CHL
		Non Syndromic CHL_Autosomal Recessive Hearing Loss
		GJB2 (Connexin 26) – DFNB1A
		GJB6 (Connexin 30) – DFNB1B
		MYO7A (Myosin VIIA) – DFNB2
		MYO15A (Myosin XV) – DFNB3
		SLC26A4 (Pendrin) – DFNB4
		OTOF (Otoferlin) – DFNB9
		CDH23 (Otocadherin) – DFNB12
		USH1C (Harmonin) – DFNB18
		TECTA (α-Tectorin) – DFNB21
		COL11A2 (Collagen 11α2) – DFNB53
			Non Syndromic CHL_Autosomal Dominant Hearing Loss
		DIAPH1 (Diaphanous) – DFNA1
		KCNQ4 – DFNA2
		GJB2 (Connexin 26) – DFNA3
		TECTA (a-Tectorin) – DFNA8/DFNA12
		EYA4 – DFNA10
		WFS1 – DFNA 6/14/38
	Syndromic CHL
		Syndromic CHL_Autosomal Recesive Hearing Loss
			Usher Syndrome
			Pendred Syndrome
			Jervell and Lange-Nielsen Syndrome
			Wolfram Syndrome
			Pompe Disease
		Syndromic CHL_Autosomal Dominant Hearing Loss
			Waardenburg Syndrome
			Brachio-Oto-Renal Syndrome
			Stickler Syndrome
			Treacher Collins Syndrome
		Syndromic CHL_X-Linked Hearing Loss
			Alport Syndrome
	Conclusion
	References
Chapter 56
Neuroplasticity and Sensorineural  Hearing Loss
	Abstract
	Introduction to Neuroplasticity
	Neuroplasticity in the Auditory System
	Unimodal Plasticity
	Cross-Modal Plasticity
	Neuroplasticity and Cochlear Implants
	Conclusion
	References
Chapter 57
Neuroradiology of the Hearing System
	Abstract
	1. Introduction to the Role of Imaging
		1.1. Computerized Tomography (CT) Study Protocol
		1.2. Magnetic Resonance Imaging (MRI) Study Protocol
	2. External Ear
	3. Middle Ear and Mastoid
		3.1. Congenital Anomalies of the Middle Ear
		3.2. Acute Infections of Middle Ear
		3.3. Chronic Otitis Media
			3.3.1. Chronic Otitis Media without Cholesteatoma
			3.3.2. Chronic Otitis Media with Cholesteatoma
		3.4. Post-Operative Imaging
		3.5. Otosclerosis
	4. Inner Ear
		4.1. Malformations
			4.1.1. Complete Labyrinthine Aplasia (Michel deformity)
			4.1.2. Cochlear Aplasia
			4.1.3. Common Cavity Deformity
			4.1.4. Cochlear Hypoplasia (CH)
			4.1.5. Incomplete Partitions
			4.1.6. Enlarged Vestibular Aqueduct (EVA)
			4.1.7. Malformations of the Vestibule and Semicircular Canals
			4.1.8. Abnormalities of the Vestibulo-Cochlear Nerve
		4.2. Infections
		4.3. Autoimmune Labyrinthitis
		4.4. Imaging in Cochlear Implantation
	5. Imaging of Cerebellopontine Angle and Internal Auditory Canal Lesions
		5.1. Vestibular Schwannoma
		5.2. Meningioma
		5.3. Aneurysms
		5.4. Epidermoid Cyst
		5.5. Arachnoid Cyst
		5.6. Lipochoristomas (Lipomatous Tumors)
		5.7. Dermoid Cyst
		5.8. Chordoma
		5.9. Intra-Axial Tumors with CPA Involvement
	6. Traumatic Lesions of the Temporal Bone
		6.1. Temporal Bone Fractures
		6.2. Ossicular Injuries
		6.3. Contusio Labyrinthi (Labyrinthine Concussion)
		6.4. Post Traumatic Hearing Loss
		6.5. Facial Nerve Injury
		6.6. Vascular Injuries (Carotid Canal and Jugular Gulf Impairment)
	7. Petrous Apex Lesions and Other Lesions of the  Temporal Bone
		7.1. Leave-Me-Alone Lesions
		7.2. Infections and Inflammatory Diseases
		7.3. Neoplasms
		7.4. Non-Neoplastic Lesions
		7.5. Other Lesions of the Temporal Bone
			7.5.1. Tumor-Like Lesions
			7.5.2. Tumors
	8. Facial Nerve
		8.1. Facial Paralysis
			8.1.1. Central Facial Palsy
			8.1.2. Peripheral Facial Palsy
			8.1.3. Facial Nerve Tumors
			8.1.4. Congenital Malformations
		8.2. Facial Dystonias
	References
	Atlas
		Imaging Based Atlas of the Hearing System
Chapter 58
Age-Related Hearing Loss
	Abstract
	Definition
	Epidemiology
	Causes and Risk Factors
		1. Genetics
		2. Environmental Factors
		3. Health Co-Morbidities
		4. Ageing of the Auditory System
			Peripheral ARHL
			Central ARHL
	ARHL and Frailty
	Diagnosis
		Pure Tone Audiometry
		Speech Audiometry
		Redundancy and Sensitized Speech Measures (SSM)
		Tympanometry
		Otoacoustic Emissions
	Prevention and Rehabilitation
	References
Chapter 59
Traumatic Sensorineural Hearing Loss
	Abstract
	Introduction
	Pathophysiology
		TBI with Temporal Bone Fracture
		TBI without Temporal Bone Fracture
		Perilymphatic Fistula with Pneumolabyrinth
	Diagnosis
		Clinical Evaluation (Table 2)
		Radiological Evaluation (Table 2)
		Temporal Bone Fractures and Ossicular Injuries
		Labyrinthine Concussion
		Perilymphatic Fistula
		Endolymphatic Hydrops
		Injury to the Central Auditory Pathways
		Labyrinthitis Ossificans
	Treatment
	References
Chapter 60
Advanced Otosclerosis
	Abstract
	Introduction
	Definition
	Pathogenesis
	Radiologic Diagnosis and Classification
	Treatment Strategies
		Hearing Aids and Follow-Up
		Stapes Surgery and Hearing Aids
		Cochlear Implantation
		Direct Acoustic Cochlear Implant
		Treatment Strategy and Counselling
	Conclusion
	References
Chapter 61
Sudden Sensorineural Hearing Loss
	Abstract
	Etiology
	Diagnostic Evaluation
	Pathophysiology of Sudden Hearing Loss
	Vascular Theory
	Viral Theory
	Autoimmune Theory
	Treatment
	Prognosis
	Conclusion
	References
Chapter 62
Cause, Pathogenesis, Clinical Manifestations and Treatment of Meniere’s Disease and Endolymphatic Hydrops
	Abstract
	Introduction
	Cause of Meniere’s Disease
	Pathogenesis of Meniere’s Syndrome
	Treatment of Meniere’s Disease and  Endolymphatic Hydrops
	Conclusion
	References
Chapter 63
Autoimmune Inner Ear Disease
	Abstract
	Introduction
		Environmental Factors
		Genetic Factors
		Diagnosis
		Cogan’s Syndrome
		Vogt-Koyanagi-Harada Syndrome
		Susac’s Syndrome
		Rheumatoid Arthritis
		Primary Sjögren Syndrome
		Polyarteritis Nodosa
		Systemic Lupus Erythematosus
	Specific Tests of the Inner Ear
		Lymphoblastic Transformation Test Concerning Cellular Immunity
		Western Blot
		Indirect Immunofluorescence Test
	Other Useful Tests for Diagnosis
	Therapy
		Corticosteroids
		Immunosuppressive
		Plasmapheresis
		Gammaglobuline
		Intratympanic Injections
		Cochlear Implants
	Conclusion
	References
Chapter 64
Occupational Hearing Loss
	Abstract
	Introduction
	Pathophysiology of Occupational Hearing Loss
	Clinical and Audiometric Characteristics of NIHL
	Non Auditory Effects of Noise Exposure
	Inner Ear Protection from Noise
	References
Chapter 65
Single Side Deafness in Children
	Abstract
	Introduction
	How to Test Auditory and Memory
	Studies Results
	Discussion
	Conclusion
	References
Chapter 66
Pharmacological Treatment  of Sensorineural Hearing Loss
	Abstract
	Introduction
	Sudden Sensorineural Hearing Loss:  Into the Medical History
	Symptoms: From the Mechanistic Damage to  the Distorted Perception
	Diagnostic Measures: The State of the Multiple Evidences
	Pharmacological Management:  Glucocorticoids Mechanisms of Action
	Pharmacological Management: Oral and Intratympanic Corticosteroid Therapy
	Pharmacological Management: Vasodilators Agents
	Conclusion
	References
Chapter 67
Management of Sensorineural Hearing  Loss with Hearing Aids
	Abstract
	Introduction
	Functional Principles and Essential Components of Hearing Aids
	Classification of Hearing Aids
	Types of Amplification
	Fitting of Hearing Aids
	Technological Advancements and Future Developments
	Hearing Aids for Unilateral Hearing Loss in Children
	Hearing Aids for Unilateral Hearing Loss in Adults
	References
Chapter 68
Cochlear Implant of SNHL Patients
	Abstract
	Introduction
		Effects of Hearing Loss
	Candidacy Criteria for Cochlear Implantation
		FDA Guidelines
		Medical Evaluation
		Etiology
		History of Hearing Loss
		Radiological Examination
		Genetic Diagnostics
		Audiologic Evaluation
		Performance Measures in Children
		Psychological/Rehabilitation Evaluation
		Patient Counseling and Expectations
	The CI Team
		Otologist/Otosurgeon
		Aural Rehabilitation Specialists
		Psychologist
		Neuropsychologist
		Educational Specialists
		Social Worker
	CI Components
	The CI Surgery
		Standard Surgical Technique
	CI Complications
		Device Failure (Technical Complications)
		Medical Complications
	Setting the Cochlear Implant Speech Processor
		Postoperative Fitting and Hearing-Speech Training
		The Use of Objective Measures in Speech Processor Programming
		Electrically Evoked Auditory Brainstem Response (EABR)
		Electrically Evoked Compound Action Potential (ECAP)
		Electrically Evoked Acoustic Reflex Threshold (EART)
	Results
		Comparison of Sensory Aids in Children
		Bilateral Implantation
		Cochlear Implants and Cognitive Effort
	References
Chapter 69
Presbyastasis: From Diagnosis  to Management
	Abstract
	Introduction
	Patient’s Evaluation
	Tinetti Performance Oriented Mobility  Assessment (POMA)
	Balance Section
	Gait Section
	Risk Indicators
		 normal mobility: patients who are autonomous for balance and for prehension tasks perform it in less than 10 seconds
		 normal limits for weak, elderly and disabled people: patients who are indipendent for transfers only perform them in less than 20 seconds
		 a range higher than 20 seconds means the person needs assistance outside and indicates the necessity of further examinations and interventions [15]
		 a score of 30 seconds or more suggests that the person has an severe risk to fall
	Berg’s Balance Scale
	Rehabilitation Strategy
	Proprioceptive Training
	Proprioceptive Self-Analysis
	Learing to Get up after a Fall
	Conclusion
	References
Index
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