Cochlear vascular pathology and hydrops in otosclerosis

Three ears with otosclerosis were found incidentally in a series of human temporal bones examined to evaluate cochlear sensorineural degeneration. Otosclerosis was identified with microdissection, surface preparation technique and transmission electron microscopy. Vascular abnormalities were present in all ears, and otosclerosis involved the cochlear endosteum extensively, mainly in the scala tympani of the basal turn. In the scala tympani of the lower half of the basal turn, shunts had formed so that venules deviated abruptly from their normal radiating course towards the spiral vein, left the scala and entered into otosclerotic foci. There was a marked loss of radiating venules in areas where otosclerosis affected the endosteum of the scala. In the pair of bones capillaries in the stria vascularis were extremely dilated, the widest being 80 microns in diameter. The third single bone from a patient with Meniere's disease had severe cochleo-saccular hydrops. Ten serially sectioned temporal bones with known otosclerosis were reviewed. Two of the bones, one of which had cochleo-saccular hydrops, displayed vascular shunts in the scala tympani and enormously dilated strial capillaries with a maximum diameter of 139 microns.