Genetic factors affecting hearing development.
Both genetic background and single gene mutations may affect the development of the auditory system. A classification system is presented for those single gene mutations causing hearing impairment. The new feature of this classification is the inclusion of a category for hereditary deafness of central origin. The other categories involve peripheral abnormalities and are: morphogenetic defects, in which the overall structure of the labyrinth is deformed; neuroepithelial degeneration, in which the primary defect appears to occur in the organ of Corti; and cochleo-saccular degeneration, where the stria vascularis is abnormal and Reissner's membrane collapses, leading to further degeneration.
D000818:Animals; D001306:Auditory Pathways / Q000002:abnormalities*; D003051:Cochlea / Q000002:abnormalities; D004195:Disease Models, Animal; D007758:Ear, Inner / Q000002:abnormalities*; D004627:Embryonic Induction; D006311:Hearing Disorders / Q000145:classification / Q000235:genetics*; D006801:Humans; D051379:Mice; D008818:Mice, Neurologic Mutants; D009024:Morphogenesis; D009154:Mutation; D010542:Peromyscus; D051381:Rats; D011911:Rats, Gunn; D013216:Reflex, Startle; D012444:Saccule and Utricle / Q000002:abnormalities; D012683:Sensory Deprivation / Q000502:physiology