Hereditary deafness with XXXX and anomalous calcium phosphate deposits.

@entity1 @entity13267 @entity66 @entity4 @entity38106 @entity4494 @entity2319 @entity528
The temporal bones from a 58-year-old white @entity1 who had had hereditary @entity2319 were examined with the techniques of microdissection and surface preparations followed by sectioning of the modiolus. There was bilateral, almost total @entity528 , which also involved the saccule. The degeneration of the distal processes of the cochlear neurons in the osseous spiral lamina was almost complete, whereas @entity4494 cells and proximal processes remained in the modiolus and the internal auditory canal. Severe cochleo-saccular @entity4 was present in the left ear with Reissner's membrane bulging into the horizontal canal. X-ray diffraction and electron probe analysis were used to study the @entity66 in both ears. On the left side the saccular otoconia were composed of calcite, but the utricular macula was covered by a crust of @entity13267 spherulites. More @entity13267 occurred around the maculae and in the scala media. The cupulae were composed of @entity13267 and @entity38106 . On the right side the utricular otoconia were of normal calcite, but there was a deposit of @entity13267 on the macula sacculi. The upper part of the scala media was completely filled by a deposit of @entity13267 and @entity38106 .
