About The Book

The second edition of Disorders of the Auditory System reflects the combined efforts of renowned audiologists and otologists to provide the reader with both the audiologic and medical aspects of auditory dysfunction associated with disorders of the peripheral and central auditory system. This book includes numerous insightful case studies covering both classic and unique clinical presentations that will provide informative reading for students and professionals in the fields of audiology, otology, and neurology. The book also includes color images of video otoscopy.

New to the Second Edition

  • Coverage of additional auditory disorders, including meningitis, cytomegalovirus, enlarged vestibular aqueduct syndrome, and barotrauma
  • New case studies
  • Updated references and resources
  • Access to a PluralPlus companion website for instructors that includes PowerPoint lecture slides and the color images from the text

From the Foreword

“Writing a book on disorders of the auditory system is a daunting task. Few individuals have the depth and breadth of knowledge to accomplish it effectively. But the authors of this volume, Drs. Frank E. Musiek, Jennifer B. Shinn, Jane A. Baran, and Raleigh O. Jones, are among a very small number of teams truly able to meet the challenge. This team has dealt with the issues and problems surrounding the evaluation of auditory disorders for many years, has devised a number of the tools in current use for clinical evaluation, and has applied them successfully in a variety of settings. They have been in the trenches. This team also reflects a fine working relationship between audiology, otology, and neurology critical to understanding the complex issues related to auditory disorders. …In this new edition, they bring us very nicely up to date on the present status of what has become, over the last five decades, a broad array of auditory disorders. This second edition especially emphasizes more case studies, coverage of additional disorders, and some very sophisticated new illustrations. In addition, the all-important reference list has been updated. I continue to recommend this book, especially this new second edition, as an excellent text for an advanced undergraduate or graduate course on auditory disorders and their evaluation.”
James Jerger, PhD

About The Authors

Frank E. Musiek, PhD, CCC-A, is Professor and Director of the NeuroAudiology Lab, Dept. of Speech Language and Hearing Sciences University of Arizona. He is Professor Emeritus at the University of Connecticut and former Professor and Director of Audiology at the Dartmouth Hitchcock Medical Center.

Jennifer B. Shinn, PhD, CCC-A, FAAA is board certified in Audiology through both the American Speech-Language Hearing Association and the American Academy of Audiology. She currently holds an appointment as Assistant Professor at the University of Kentucky in the Division of Otolaryngology, Department of Surgery. Additionally, she is the Chief of Audiology and Resident Research Director. She has a broad experience in a variety of areas of audiology, including advanced diagnostics and neuroaudiology, with particular emphasis on diagnosis of auditory processing disorders and subsequent (re)habilitation. She has published both peer reviewed articles as well as book chapters in the above named areas. Her presentation record in these areas includes state, national, and international levels.

Jane A. Baran, PhD is Professor Emerita in the Department of Communication Disorders at the University of Massachusetts Amherst. Her primary research and clinical interests are in the areas of (central) auditory processing disorders, auditory evoked potentials, and neuroanatomy and neurophysiology of the auditory system. She is coauthor of three books, Neuroaudiology: Case Studies, The Auditory System: Anatomy, Physiology and Clinical Correlates, and Disorders of the Auditory System. In addition, she has authored or co-authored a number of journal articles and book chapters in the areas of normal and disordered auditory processing, language processing, auditory evoked potentials, and auditory neuroanatomy and neurophysiology. She has presented numerous research papers and educational workshops at regional, national, and internationals meetings. Dr. Baran is a Fellow of the American Speech-Language-Hearing Association (ASHA), the 2001 Recipient of the Clinical Educators Award from the American Academy of Audiology, a 2005 Recipient of a Recognition Award for Special Contributions to Higher Education from ASHA, and the 2013 Recipient of the Journal of the American Academy of Audiology Editor’s Award. She also is one of a select group of scholars to receive the Carnegie National Scholar Fellowship Award from the Carnegie Foundation for the Advancement of Teaching and Learning.

Raleigh O. Jones, MD is Associate Professor of Surgery and Chief of Otolaryngology-Head and Neck Surgery at the University of Kentucky Medical Center. He is a graduate of the University of Kentucky College of Medicine and the Otolaryngology Residency Program at the University of North Carolina at Chapel Hill Hospitals. He completed a fellowship in Neurotology at the Ear Research Foundation of Florida and has specialized in the medical and surgical treatment of patients with disorders of hearing and balance. He has authored over 30 articles and 8 book chapters and has been awarded several teaching awards.

Table Of Contents

Chapter 1. Introduction
Rationale For This Book
Overview
Summary
References

Chapter 2. Structure and Function of the Auditory and Vestibular Systems
Introduction
Anatomic Terminology
The Peripheral Auditory System
The Temporal Bone
The Outer Ear
The Middle Ear
The Cochlea
The Auditory Nerve
The Central Auditory System
The Cochlear Nucleus
The Superior Olivary Complex
The Lateral Lemniscus
Inferior Colliculus
The Medial Geniculate Body
Auditory Cortex and Subcortex
The Corpus Callosum
The Efferent System
Structure
Function
Vascular Supply for the Auditory System
The Peripheral System
The Central System
The Vestibular System
Structure
Function
Vascular Supply for the Vestibular System
Summary
References

Chapter 3. Audiologic, Vestibular, and Radiologic Procedures
Introduction
Audiologic Assessment
Examination of Hearing Sensitivity and Speech Recognition Ability
Immittance Audiometry
Electroacoustic Measures
Auditory Processing Tests
Electrophysiologic Assessment
Vestibular Assessment
Electronystagmography and Videonystagmography
Vestibular Evoked Myogenic Potentials
Rotational Chair
Basics of Computed Tomography and Magnetic Resonance Imaging
Computed Tomography
Magnetic Resonance Imaging
Normal Anatomy and Clinical Applications of CT and MRI in the Evaluation of the Auditory                       System
Summary
References

Chapter 4. Outer and Middle Ear Disorders
Introduction
Aural Atresia
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 4–1: Canal Atresia and Stenosis
Eustachian Tube Dysfunction
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 4–2: Eustachian Tube Dysfunction
Otitis Media
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 4–3: Otitis Media
Case 4–4: Otitis Media
Cholesteatoma
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 4–5: Cholesteatoma
Case 4–6: Cholesteatoma
Paraganglioma Tumors
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 4–7: Paraganglioma Tumor
Otosclerosis
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 4–8: Otosclerosis
Temporal Bone Trauma
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 4–9: Ossicular Disarticulation
Case 4–10: Temporal Bone Fracture
Other Disorders Affecting the Outer and Middle Ears
External Otitis
Exostoses and Osteomas
Tympanic Membrane Perforation
Tympanosclerosis
Ossicular Chain Discontinuity
Summary
References

Chapter 5. Inner Ear Disorders
Introduction
Temporal Bone Trauma
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 5–1: Temporal Bone Trauma
Noise-Induced Hearing Loss
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 5–2: Noise-Induced Hearing Loss
Case 5–3: Noise-Induced Hearing Loss
Ototoxicity
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 5–4: Ototoxicity
Ménière’s Disease (Endolymphatic Hydrops)
Introduction
Symptoms
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 5–5: Bilateral Ménière’s Disease
Case 5–6: Unilateral Ménière’s Disease
Autoimmune Inner Ear Disease
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 5–7: Autoimmune Inner Ear Disease
Age-Related Hearing Loss (Presbycusis)
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 5–8: Age-Related Hearing Loss
Superior Semicircular Canal Dehiscence Syndrome
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 5–9: Superior Semicircular Canal Dehiscence
Case 5–10: Superior Semicircular Canal Dehiscence
Sudden Idiopathic Sensorineural Hearing Loss
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Case 5–11: Sudden Sensorineural Hearing Loss
Case 5–12: Sudden Sensorineural Hearing Loss
Other Disorders Affecting The Cochlea
Enlarged Vestibular Aqueduct Syndrome
Barotrauma
Meningitis
Cytomegalovirus
Diabetes Mellitus
Perilymph Fistulas
Summary
References

Chapter 6. Auditory Nerve Disorders
Introduction
Acoustic Neuroma
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Audiologic Management
Medical Management
Additional Information
Case 6–1: Acoustic Neuroma
Case 6–2: Acoustic Neuroma
Case 6–3: Acoustic Neuroma
Auditory Neuropathy Spectrum Disorder
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Medical and Audiologic Management
Case 6–4: Auditory Neuropathy Spectrum Disorder
Case 6–5: Auditory Neuropathy Spectrum Disorder
Vascular Loop Syndrome
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Audiology
Medical Examination
Medical Treatment
Case 6–6: Vascular Loop Syndrome
Summary
References

Chapter 7. Disorders of the Central Auditory Nervous System
Introduction
Audiology
Anatomic Factors
Types of Tests
Brain Plasticity
Mass Lesions
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Medical Diagnosis
Medical Management
Audiology
Vascular Disorders
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Medical Management
Audiology: Mass and Vascular Lesions
Behavioral Test Procedures
Electrophysiologic Tests
Case 7–1: Temporal Lobe Tumor
Case 7–2: Temporoparietal Stroke
Degenerative Disorders
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Medical Diagnosis
Medical Management
Audiology
Case 7–3: Multiple Sclerosis
Case 7–4: Alzheimer’s Disease
Neurotoxicity
Introduction
Heavy Metals and Solvents
Hyperbilirubinemia
Audiology and Neurotoxins
Audiology: Heavy Metals and Solvents
Case 7–5: Mercury Poisoning
Audiology: Hyperbilirubinemia
Trauma, Head Injury (Traumatic Brain Injury)
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Medical Diagnosis
Medical Management
Audiology
Case 7–6: Head Injury
Case 7–7: Head Injury
Temporal Lobe Epilepsy
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Medical Diagnosis
Medical Management
Audiology
Case 7–8: Temporal Lobe Epilepsy
Case 7–9: Temporal Lobe Epilepsy
Surgical Interventions That Alter Central Auditory Function
Temporal Lobectomy
Commissurotomy
Case 7–10: Commissurotomy
Central Auditory Disorder Associated with Learning Difficulties
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Medical Diagnosis
Audiology
Audiologic Management
Case 7–11: CAPD Associated with Learning Difficulties
Summary

Chapter 8. Tinnitus, Hyperacusis, and Auditory Hallucinations
Introduction
Tinnitus
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Medical and Audiologic Evaluation
Medical and Audiologic Treatment
Hyperacusis
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Medical and Audiologic Evaluation
Medical and Audiologic Treatment
Auditory Hallucinations
Introduction
Symptoms
Incidence and Prevalence
Etiology and Pathology
Site of Lesion
Medical and Audiologic Evaluation
Models for the Generation of Auditory Hallucinations
Medical and Audiologic Treatment
Summary
References

Chapter 9. Hereditary and Congenital Hearing Loss
Introduction
Genetics Overview
Autosomal Recessive Inheritance Pattern
Autosomal Dominant Inheritance Pattern
X-Linked Inheritance Pattern
Mitochondrial Inheritance
Multifactorial Traits
The Genetics of Hearing Loss
Syndromic and Nonsyndromic Hearing Loss
Congenital Malformations
External and Middle Ear Malformations
Inner Ear Malformations
Auditory Nerve and Internal Auditory Meatus
Auditory Nervous System Malformations
Genetic Evaluation and Counseling for Hearing Loss
Genetic Evaluation
Genetic Counseling

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