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Table of Contents
Preface; Contents; Chapter 1: Overview; 1.1 Inner Ear Malformation; 1.1.1 The Eighteenth Century: "Dissection ofßtheßTemporal Bone byßMondini (Figs. 1.1 andß1.2)"; 1.1.2 The Nineteenth Century: "Temporal Bone Histology"; 1.1.3 The Twentieth Century: "X-ray andßCT ofßTemporal Bone"; 1.1.4 The Twenty-First Century: "High-Resolution CT andßMRI ofßtheßTemporal Bone"; 1.2 Cochlear Nerve Deficiency (CND); References; Chapter 2: Embryology ofßInner Ear andßIts Malformation; 2.1 Anatomical Development ofßtheßCochlea andßAuditory System.
2.1.1 Development ofßtheßCochlea andßMyelination ofßtheßAuditory System2.1.2 Inner Ear Malformation andßArrest ofßDevelopment ofßtheßInner Ear; 2.1.3 Development ofßtheßCentral Auditory Pathway; 2.1.4 As Developmental Arrest ofßtheßInner Ear, Common Cavity, andßCochlear Nerve Deficiency May Occur; 2.1.5 Conduction Speed ofßNerve Impulse BeforeßandßAfter Myelination; References; Chapter 3: Embryology ofßCochlear Nerve andßIts Deficiency; 3.1 Introduction; 3.2 Embryology; 3.2.1 First Trimester; 3.2.2 Second Trimester; 3.2.3 Third Trimester; 3.2.4 Summary; 3.3 Cochlear Nerve Deficiency.
3.4 ConclusionReferences; Chapter 4: Morphology, Development, andßNeurotrophic Regulation ofßCochlear Afferent Innervation; 4.1 Introduction; 4.2 Morphology ofßSGNs andßTheir Neural Connection; 4.3 Development ofßSGNs; 4.3.1 Neurogenesis; 4.3.2 Cell Death inßSGNs; 4.3.3 Postnatal Rearrangement ofßCochlear Afferent Innervation; 4.4 Neurotrophic Factors andßSGNs; 4.4.1 Expression ofßNeurotrophins andßTheir Receptors inßtheßCochlea; 4.4.2 Neurotrophic Support forßSGN Survival inßEmbryonic Development; 4.4.3 Other Neurotrophic Factors Involved inßtheßDevelopment ofßCochlear Innervation.
4.4.4 Neurotrophic Factors Promote Survival andßNeuritogenesis ofßSGNs InßVitro4.4.5 Damage ofßtheßOrgan ofßCorti andßSecondary Degeneration ofßSGNs; 4.4.6 Therapeutic Potential ofßNeurotrophic Factors inßCochlear Implant Therapy; References; Chapter 5: Genetics ofßInner Ear Malformation andßCochlear Nerve Deficiency; 5.1 Introduction; 5.2 Nonsyndromic Hearing Loss; 5.2.1 DFNB4/Pendred Syndrome; 5.2.2 DFN3; 5.3 Syndromic Hearing Loss; 5.3.1 Waardenburg Syndrome; 5.3.2 BOR Syndrome; 5.3.3 CHARGE Syndrome; 5.3.4 Okihiro Syndrome; 5.3.5 Distal Renal Tubular Acidosis (DRTA).
5.4 Chromosomal Disorders Associated withßHearing Loss5.4.1 Trisomy 21 (Down Syndrome); 5.4.2 Trisomy 18 (Edwards Syndrome); 5.4.3 Trisomy 13 (Patau Syndrome); 5.4.4 22q11.2 Deletion Syndrome (DiGeorge Syndrome); 5.5 Perspective; References; Chapter 6: Classification ofßInner Ear Malformations; 6.1 Introduction; 6.1.1 Complete Labyrinthine Aplasia (CLA, Michel Deformity); 6.1.1.1 Definition andßRadiology; 6.1.1.2 Audiological Findings; 6.1.1.3 Management; 6.1.2 Rudimentary Otocyst; 6.1.2.1 Definition andßRadiology; 6.1.2.2 Management; 6.1.3 Cochlear Aplasia; 6.1.3.1 Definition andßRadiology.
2.1.1 Development ofßtheßCochlea andßMyelination ofßtheßAuditory System2.1.2 Inner Ear Malformation andßArrest ofßDevelopment ofßtheßInner Ear; 2.1.3 Development ofßtheßCentral Auditory Pathway; 2.1.4 As Developmental Arrest ofßtheßInner Ear, Common Cavity, andßCochlear Nerve Deficiency May Occur; 2.1.5 Conduction Speed ofßNerve Impulse BeforeßandßAfter Myelination; References; Chapter 3: Embryology ofßCochlear Nerve andßIts Deficiency; 3.1 Introduction; 3.2 Embryology; 3.2.1 First Trimester; 3.2.2 Second Trimester; 3.2.3 Third Trimester; 3.2.4 Summary; 3.3 Cochlear Nerve Deficiency.
3.4 ConclusionReferences; Chapter 4: Morphology, Development, andßNeurotrophic Regulation ofßCochlear Afferent Innervation; 4.1 Introduction; 4.2 Morphology ofßSGNs andßTheir Neural Connection; 4.3 Development ofßSGNs; 4.3.1 Neurogenesis; 4.3.2 Cell Death inßSGNs; 4.3.3 Postnatal Rearrangement ofßCochlear Afferent Innervation; 4.4 Neurotrophic Factors andßSGNs; 4.4.1 Expression ofßNeurotrophins andßTheir Receptors inßtheßCochlea; 4.4.2 Neurotrophic Support forßSGN Survival inßEmbryonic Development; 4.4.3 Other Neurotrophic Factors Involved inßtheßDevelopment ofßCochlear Innervation.
4.4.4 Neurotrophic Factors Promote Survival andßNeuritogenesis ofßSGNs InßVitro4.4.5 Damage ofßtheßOrgan ofßCorti andßSecondary Degeneration ofßSGNs; 4.4.6 Therapeutic Potential ofßNeurotrophic Factors inßCochlear Implant Therapy; References; Chapter 5: Genetics ofßInner Ear Malformation andßCochlear Nerve Deficiency; 5.1 Introduction; 5.2 Nonsyndromic Hearing Loss; 5.2.1 DFNB4/Pendred Syndrome; 5.2.2 DFN3; 5.3 Syndromic Hearing Loss; 5.3.1 Waardenburg Syndrome; 5.3.2 BOR Syndrome; 5.3.3 CHARGE Syndrome; 5.3.4 Okihiro Syndrome; 5.3.5 Distal Renal Tubular Acidosis (DRTA).
5.4 Chromosomal Disorders Associated withßHearing Loss5.4.1 Trisomy 21 (Down Syndrome); 5.4.2 Trisomy 18 (Edwards Syndrome); 5.4.3 Trisomy 13 (Patau Syndrome); 5.4.4 22q11.2 Deletion Syndrome (DiGeorge Syndrome); 5.5 Perspective; References; Chapter 6: Classification ofßInner Ear Malformations; 6.1 Introduction; 6.1.1 Complete Labyrinthine Aplasia (CLA, Michel Deformity); 6.1.1.1 Definition andßRadiology; 6.1.1.2 Audiological Findings; 6.1.1.3 Management; 6.1.2 Rudimentary Otocyst; 6.1.2.1 Definition andßRadiology; 6.1.2.2 Management; 6.1.3 Cochlear Aplasia; 6.1.3.1 Definition andßRadiology.