PubMed · 9918185
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A J Gulya. 1999. Clarifications requested re: cochlear modiolar defects.. https://pubmed.ncbi.nlm.nih.gov/9918185/
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The inner ear is located within the petrous portion of the temporal bone and consists of the membranous, perilymphatic, and the outer osseous labyrinths. Together, these structures form the end organs for mediating hearing and balance. This article provides a detailed review of the neonatal anatomy and development of these structures, knowledge of which derives in great part from advances in CT and sophisticated MR imaging. Current research should soon enable clinicians to identify a wide variety of subtle lesions of the inner ear that heretofore have been inaccessible to imaging diagnosis.
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Using dot-blot hybridization and Southern blotting, the authors detected a point mutation at nucleotide pair (np) 3243 in mitochondrial DNA from temporal bone sections of a woman with diabetes and deafness. The mutation could not be detected with agarose gel electrophoresis, suggesting that the degree of heteroplasmy is low. Histologically, there was marked degeneration of the stria vascularis and outer hair cells throughout the cochlea, as well as a reduction of spiral ganglion cells in the base. These findings suggest that the mutation affects these inner ear structures preferentially and that deafness can occur even when the proportion of np 3243 mutation is low.