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Hearing Loss in Children, An Issue of Otolaryngologic Clinics of North America, E-Book

Hearing Loss in Children, An Issue of Otolaryngologic Clinics of North America, E-Book

Bradley W. Kesser

(2016)

Additional Information

Abstract

This essential subject to Otolaryngologists on Hearing Loss in Children is edited by leading physicians and academicians Dr. Bradley Kesser and Dr. Margaret Kenna. Topics in this issue on the state of art of diagnosing and managing pediatric hearing loss include: Audiometric evaluation of children with hearing loss; Taking the history and physical exam of the child with hearing loss; Radiographic evaluation of children with hearing loss; Acquired Hearing Loss in Children and Laboratory evaluation of children with hearing loss; Management of children with non-atretic conductive hearing loss; Management of children with congenital aural atresia; Diagnostic evaluation of children with sensorineural hearing loss; Management of children with mild, moderate, and moderate-severe SNHL; Management of children with severe, severe-profound, and profound SNHL; Management of children with unilateral hearing loss; Auditory Neuropathy/Dyssynchrony Disorder; Genetics of Hearing Loss – Syndromic; Genetics of Hearing Loss – Nonsyndromic; Psychosocial aspects of hearing loss in children; Speech and Auditory-Verbal Therapy; On the horizon - cochlear implant technology; Auditory brainstem implants; On the distant horizon - medical therapy for SNHL; Early Practice Considerations for Pediatric Hearing Loss. Each article presents clinically focused diagnosis and management.

Table of Contents

Section Title Page Action Price
Front Cover Cover
Hearing Loss in Children\r i
Copyright\r ii
PROGRAM OBJECTIVE iii
LEARNING OBJECTIVES iii
ACCREDITATION iii
DISCLOSURE OF CONFLICTS OF INTEREST iii
UNAPPROVED/OFF-LABEL USE DISCLOSURE iii
TO ENROLL iv
METHOD OF PARTICIPATION iv
CME INQUIRIES/SPECIAL NEEDS iv
Contributors v
EDITORS v
AUTHORS v
Contents ix
Preface: The Child with Hearing Loss \r ix
Audiometric Evaluation of Children with Hearing Loss\r ix
Taking the History and Performing the Physical Examination in a Child with Hearing Loss\r ix
Radiographic Evaluation of Children with Hearing Loss\r ix
Acquired Hearing Loss in Children\r ix
Management of Conductive Hearing Loss in Children\r x
Diagnostic Evaluation of Children with Sensorineural Hearing Loss\r x
Management of Children with Mild, Moderate, and Moderately Severe Sensorineural Hearing Loss\r x
Management of Children with Severe, Severe-profound, and Profound Sensorineural Hearing Loss\r x
Management of Children with Unilateral Hearing Loss\r xi
Auditory Neuropathy/Dys-Synchrony Disorder: Diagnosis and Management\r xi
Genetics of Hearing Loss: Syndromic\r xi
Genetics of Hearing Loss: Nonsyndromic\r xi
Psychosocial Aspects of Hearing Loss in Children\r xii
Communication Assessment and Intervention: Implications for Pediatric Hearing Loss\r xii
On the Horizon: Cochlear Implant Technology\r xii
Pediatric Auditory Brainstem Implant Surgery\r xiii
On the Distant Horizon: Medical Therapy for Sensorineural Hearing Loss\r xiii
OTOLARYNGOLOGIC CLINICS\rOF NORTH AMERICA\r xiv
FORTHCOMING ISSUES xiv
February 2016 xiv
April 2016 xiv
June 2016 xiv
RECENT ISSUES xiv
October 2015 xiv
August 2015 xiv
June 2015 xiv
Preface: The Child with Hearing Loss \r xv
Audiometric Evaluation of Children with Hearing Loss 891
Key points 891
INTRODUCTION 891
SCREENING FOR HEARING LOSS 891
Otoacoustic Emissions 892
Auditory Brainstem Response 892
BEHAVIORAL AUDIOMETRY 893
Conditioned Play Audiometry 894
SPEECH AUDIOMETRY 894
Speech Perception Testing 895
OBJECTIVE MEASURES 897
Acoustic Immittance Measures 897
Tympanometry 897
Acoustic Reflex Threshold 897
Otoacoustic Emissions 897
Auditory Brainstem Response 898
FUNCTIONAL AUDITORY ASSESSMENT TOOLS 899
SUMMARY 899
REFERENCES 899
Taking the History and Performing the Physical Examination in a Child with Hearing Loss 903
Key points 903
INTRODUCTION 903
PREVALENCE AND IMPACT HEARING LOSS 905
SYMPTOM CRITERIA 905
CLINICAL FINDINGS 905
Review of Risk Factors for Hearing Loss 906
Perinatal history 907
Family history 907
Delays of motor milestones 907
Infection and immunization 907
Noise exposure 908
PHYSICAL EXAMINATION 908
Balance and Vestibular Dysfunction 908
DIAGNOSTIC MODALITIES 910
COLLABORATIVE APPROACH 910
SUMMARY 910
REFERENCES 911
Radiographic Evaluation of Children with Hearing Loss 913
Key points 913
INTRODUCTION 913
Choice of Imaging 914
SENSORINEURAL HEARING LOSS 915
Imaging Protocols 915
Bilateral sensorineural hearing loss 915
Unilateral sensorineural hearing loss 916
MRI versus computed tomography 916
Proposed algorithm 917
Common Findings 917
Enlarged vestibular aqueduct 917
Congenital cochlear abnormalities 919
Cochleovestibular nerve deficiency 920
Special Patient Populations 920
Syndromic children 920
Waardenburg syndrome 920
Pendred syndrome 921
Branchiootorenal syndrome 921
CHARGE syndrome 922
Infectious etiologies 922
Sudden sensorineural hearing loss 923
MIXED HEARING LOSS 924
CONDUCTIVE HEARING LOSS 925
Ossicular Malformations 925
Temporal Bone Fractures 925
Imaging in Cholesteatoma 926
Syndromic Conductive Hearing Loss 927
SUMMARY/CLINICAL PEARLS 927
REFERENCES 929
Acquired Hearing Loss in Children 933
Key points 933
INTRODUCTION 933
NEWBORN HEARING SCREENING 934
OVERVIEW OF CAUSES OF HEARING LOSS IN INFANTS AND CHILDREN 934
Genetic Causes of Hearing Loss 935
Infectious Causes 935
Toxoplasmosis 935
Rubella 937
Cytomegalovirus 937
Herpes simplex 938
Syphilis 939
Other Infectious Diseases Associated with Hearing Loss 939
Human immunodeficiency virus 939
Measles (rubeola) 939
Lyme disease 940
Mumps 940
Other viruses 941
Bacterial meningitis 941
Otitis media 942
HEARING LOSS SECONDARY TO TRAUMA 942
Noise-Related Hearing Loss 942
Ototoxicity 942
Head Trauma 943
Sudden Sensorineural Hearing Loss 943
Hearing loss in babies who require newborn intensive care unit management 944
Middle and inner ear structural anomalies 946
The overall evaluation of acquired neonatal and childhood-onset hearing loss 947
SUMMARY 948
REFERENCES 948
Management of Conductive Hearing Loss in Children 955
Key points 955
INTRODUCTION 956
ACQUIRED CONDUCTIVE HEARING LOSS 956
Otitis Media with Effusion 956
Prevalence 956
Effect of otitis media with effusion on speech and language development 957
Management of otitis media with effusion—American Academy of Otolaryngology guidelines 957
Conductive Hearing Loss Associated with Chronic Otitis Media 957
Tympanic membrane perforation 957
Tympanosclerosis 959
Cholesteatoma 959
Effect of chronic otitis media on hearing and speech/language development 960
CONGENITAL CONDUCTIVE HEARING LOSS 961
Overview 961
Development and Embryology of the Middle Ear 961
Patient Evaluation 962
Minor Malformations 964
Congenital stapes ankylosis 964
Malleoincudal fixation and malleus bar 965
Congenital incudostapedial discontinuity 966
Congenital absence of the oval window 966
Persistent stapedial artery 967
Congenital Aural Atresia 967
SUMMARY 969
REFERENCES 969
Diagnostic Evaluation of Children with Sensorineural Hearing Loss 975
Key points 975
INTRODUCTION 975
IDENTIFYING PATIENTS WITH SENSORINEURAL HEARING LOSS 976
CLINICAL HISTORY AND PHYSICAL EXAMINATION 976
DIAGNOSTIC TESTING 977
Laboratory Tests 977
Genetic Tests 978
Imaging 978
Cytomegalovirus Testing 979
CONSULTATION WITH SPECIALISTS 980
TESTING GUIDELINES 981
REFERENCES 981
Management of Children with Mild, Moderate, and Moderately Severe Sensorineural Hearing Loss 983
Key points 983
OVERVIEW: NATURE OF THE PROBLEM 983
IDENTIFICATION OF CHILDHOOD HEARING LOSS 984
MANAGEMENT GOALS 985
Communication Mode 986
Hearing Assistive Technology 986
Hearing aids 986
Remote microphone technology 988
Cochlear implants 988
Interprofessional Collaborative Approach 989
Monitoring 990
Transient conductive hearing loss 990
Progressive and late-onset hearing loss 990
Ongoing surveillance 990
Information for the General Otolaryngologist or Pediatrician 991
Hearing screening beyond the newborn period 991
SUMMARY 992
REFERENCES 992
Management of Children with Severe, Severe-profound, and Profound Sensorineural Hearing Loss 995
Key points 995
OVERVIEW: NATURE OF THE PROBLEM 996
PATIENT EVALUATION OVERVIEW 996
Medical Assessment 997
Audiologic Assessment 997
Imaging 998
Other Common Testing 999
MANAGEMENT GOALS 999
Communication Options and Intervention 1000
COORDINATION OF CARE 1001
COCHLEAR IMPLANTATION 1002
Treatment Goals 1002
Preoperative Planning and Preparation 1002
Patient Positioning 1003
Procedural Approach 1003
Potential Complications and Management 1003
Postprocedural Care 1003
Rehabilitation and Recovery 1004
Outcomes 1004
Evaluation of Outcome, Adjustment of Treatment, and Long-Term Recommendations 1006
AUDITORY BRAINSTEM IMPLANTS 1006
SUMMARY 1006
REFERENCES 1007
Management of Children with Unilateral Hearing Loss 1011
Key points 1011
INTRODUCTION AND BACKGROUND 1011
Epidemiology 1011
Etiology and Evaluation 1012
Advantages of Binaural Hearing 1013
CONSEQUENCES OF UNILATERAL HEARING LOSS 1014
Educational Impact 1014
Speech and Language Consequences 1014
Cognition and Executive Functions 1015
Impact on Quality of Life 1016
MANAGEMENT GOALS 1016
TREATMENT OPTIONS 1018
TREATMENT RESISTANCE OR COMPLICATIONS 1020
Hearing Rehabilitation 1020
Reluctance to Acknowledge Unilateral Hearing Loss as a Problem for Children 1020
EVALUATION OF OUTCOME 1021
SUMMARY 1022
REFERENCES 1022
Auditory Neuropathy/Dys-Synchrony Disorder 1027
Key points 1027
OVERVIEW OF CHARACTERISTICS 1028
INCIDENCE 1028
CLINICAL FINDINGS 1029
Hair Cell Responses: Otoacoustic Emissions and Cochlear Microphonics 1029
Neural Responses: Auditory Brainstem Response 1029
Neural Responses: Efferent Acoustic Reflexes 1030
Behavioral Findings: Pure-Tone Thresholds and Speech Recognition 1031
UNDERLYING MECHANISMS 1031
DIFFERENTIAL DIAGNOSIS 1032
Association with Other Neurologic Abnormalities 1032
Cochlear Nerve Deficiency 1032
Enlarged Vestibular Aqueduct 1032
DIAGNOSTIC AND MANAGEMENT DILEMMAS 1032
Variation Among Patients with Auditory Neuropathy Spectrum Disorder 1032
Auditory Neuropathy Spectrum Disorder in Infants 1033
Unilateral Auditory Neuropathy Spectrum Disorder 1033
Central Auditory Processing Disorder 1033
MANAGEMENT APPROACHES AND OUTCOMES 1034
Amplification 1034
Frequency Modulation Systems 1034
Cochlear Implants 1034
Sound Detection Versus Discrimination 1035
Two Patients Highlighting Detection Versus Discrimination 1035
A Team Approach 1036
SUMMARY 1037
ACKNOWLEDGMENTS 1037
REFERENCES 1037
Genetics of Hearing Loss 1041
Key points 1041
INTRODUCTION 1041
HEARING LOSS 1042
SYNDROMIC HEARING LOSS 1044
FORMS OF SYNDROMIC HEARING LOSS 1045
Usher Syndrome 1045
Waardenburg Syndrome 1046
Pendred Syndrome 1047
Additional Syndromes 1047
GENETICS OF HEARING LOSS 1049
DIAGNOSIS OF HEARING LOSS 1050
Identifying Disease Genes Through Linkage Mapping with Genetic Markers 1052
Identifying Disease Genes Through High-Throughput Sequencing 1053
The Importance of Genetic Research in Understanding the Processes Involved in Hearing and Deafness 1054
PROSPECTS OF FUTURE TREATMENT MODALITIES FOR HEARING LOSS 1054
SUMMARY 1055
REFERENCES 1055
Genetics of Hearing Loss—Nonsyndromic 1063
Key points 1063
AUTOSOMAL-RECESSIVE GENES 1064
GJB2 (DFNB1A) 1064
SLC26A4 (DFNB4) 1067
MYO15A (DFNB3) 1067
OTOF (DFNB9) 1067
CDH23 (DFNB12) 1067
TMC1 (DFNB7/11) 1068
AUTOSOMAL-DOMINANT GENES 1068
WFS1 (DFNA6/14/38) 1068
TECTA (DFNA8/12) 1068
COCH (DFNA9) 1068
KNCQ4 (DFNA2A) 1068
X-LINKED GENES 1068
POU3F4 (DFNX2) 1068
MITOCHONDRIAL INHERITANCE 1069
A1555G 1069
C1494T 1069
NEXT-GENERATION OR MASSIVELY PARALLEL SEQUENCING 1069
REFERENCES 1069
Psychosocial Aspects of Hearing Loss in Children 1073
Key points 1073
EARLY IDENTIFICATION AND INTERVENTION: A NEW PARADIGM 1073
THE WHOLE CHILD 1075
SOCIAL COMPETENCY 1075
RECENT ADVANCES IN DEVELOPING SOCIAL COMPETENCY 1076
Integrating Social-Emotional Learning Curricula 1077
THE RIVER SCHOOL: CHILD OUTCOMES IN THE SOCIAL-EMOTIONAL REALM 1078
SUMMARY 1078
REFERENCES 1079
Communication Assessment and Intervention 1081
Key points 1081
DEMOGRAPHICS OF PEDIATRIC HEARING LOSS 1081
EARLY INTERVENTION 1082
COMMUNICATION OPTIONS 1083
INTERVENTION AND ASSESSMENT 1085
AUDIOLOGIC MANAGEMENT 1087
LISTENING AND SPOKEN LANGUAGE AS AN OPTION 1088
SUMMARY 1090
RESOURCES 1090
Alexander Graham Bell Association for the Deaf and Hard of Hearing (www.agbell.org) 1090
American Academy of Audiology (AAA) (www.audiology.org) 1091
American Speech-Language-Hearing Association (ASHA) (www.asha.org/public/hearing/testing/) 1091
Centers for Disease Control and Prevention; National Center on Birth Defects and Developmental Disabilities; Early Hearing ... 1091
National Center for Hearing Assessment and Management (NCHAM) (www.infanthearing.org/screening/index.html) 1091
National Institutes of Health; National Institute on Deafness and Other Communication Disorders (www.nidcd.nih.gov/[Federal ... 1091
Office of Special Education and Rehabilitation Services (OSERS), US Department of Education (www.ed.gov/about/offices/list/ ... 1092
ZERO TO THREE: National Center for Infants, Toddlers, and Families (www.zerotothree.org) 1092
REFERENCES 1092
On the Horizon 1097
Key points 1097
INTRODUCTION 1097
HISTORY OF COCHLEAR IMPLANT DEVELOPMENT 1098
CONTEMPORARY COCHLEAR IMPLANTS AND TARGETS FOR INNOVATION 1099
TOTALLY IMPLANTABLE COCHLEAR IMPLANTS 1100
TELEMEDICINE AND REMOTE PROGRAMMING OF COCHLEAR IMPLANTS 1101
OPTICAL NEURAL STIMULATION AND OPTICAL COCHLEAR IMPLANTS 1101
INTRANEURAL COCHLEAR IMPLANTATION 1104
HEARING PRESERVATION AND ELECTROACOUSTICAL STIMULATION 1105
NEUROPROTECTION 1107
IMPROVING THE ELECTRODE AND COCHLEAR NERVE INTERACTIONS 1109
SUMMARY 1110
REFERENCES 1111
Pediatric Auditory Brainstem Implant Surgery 1117
Key points 1117
INTRODUCTION 1118
TREATMENT GOALS AND OUTCOMES 1121
PREOPERATIVE PLANNING AND PREPARATION 1121
Implant Systems 1121
Evaluation 1123
Radiographic Assessment 1123
Selection Criteria and Candidacy 1123
SURGICAL APPROACH 1127
Translabyrinthine Craniotomy 1128
Retrosigmoid Craniotomy 1130
Intraoperative EABRs 1130
Cranial Nerve Monitoring: Cranial Nerve VII and Cranial Nerve X 1133
POSTOPERATIVE MANAGEMENT 1134
COMPLICATIONS ASSOCIATED WITH PEDIATRIC ABI SURGERY 1134
RECOVERY AND AUDITORY HABILITATION 1135
OUTCOMES AND CLINICAL RESULTS IN THE LITERATURE 1137
FUTURE OF AUDITORY BRAINSTEM IMPLANT TECHNOLOGY 1140
SUMMARY 1145
ACKNOWLEDGMENTS 1145
SUPPLEMENTARY DATA 1145
REFERENCES 1145
On the Distant Horizon—Medical Therapy for Sensorineural Hearing Loss 1149
Key points 1149
INTRODUCTION 1149
GENE THERAPY FOR SENSORINEURAL HEARING LOSS 1151
Spiral Ganglion Neuron Survival 1153
Hair Cell Survival 1154
Genetic Hearing Loss 1154
Hair Cell Regeneration and Supporting Cell Transformation 1155
RNA-BASED THERAPY FOR SENSORINEURAL HEARING LOSS 1156
STEM CELL THERAPY FOR SENSORINEURAL HEARING LOSS 1156
Stem Cell Therapy for Generation of Hair Cells 1157
Stem Cell Therapy for Generation of Auditory Neurons 1159
FUTURE DIRECTIONS 1159
REFERENCES 1160
Index 1167