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Series Preface 8 Preface 1992 9 Volume Preface 10 Contents 12 Contributors 14 To Hear or Not to Hear: Neuroscience of Deafness 16 1 The Sense of Hearing 16 2 Development and Hearing Loss 18 3 Therapy for Deafness: ``The War of Methods ́ ́ 19 4 Outline of the Book 23 5 The Future 26 References 28 Molecular Etiology of Deafness and Cochlear Consequences 31 1 Introduction 31 2 Complexity of the Auditory Apparatus 33 3 Old and New Technologies to Identify Genes and Mutations 36 4 Genes, Mutations, and Consequences on the Inner Ear 39 5 Mouse Models for Human Deafness 44 6 Summary 47 References 48 Effects of Early-Onset Deafness in the Developing Auditory System 54 1 Introduction 54 2 Histopathological Studies of the Human Cochlea 56 2.1 Histopathology of the Human Cochlea in Profound Sensorineural Hearing Loss 56 2.2 Histopathology of Cochlear Implantation 58 3 Animal Studies of Factors that Determine Cochlear SG Survival 59 3.1 SG Degeneration After Early-Onset Deafness Exhibits Two Phases 59 3.2 Effects of Electrical Stimulation on SG Neural Survival 61 3.3 Neurotrophic Agents to Promote Auditory Nerve Survival 65 3.4 The Role of Age at Onset of Deafness in Determining SG Survival Af

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