The Mondini defect in Turner's syndrome. A temporal bone report

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Abstract
The histopathology of the right temporal bone of a 47 yr old deaf woman with Turner''s Syndrome (gonadal dysgenesis) is described. The appearances are of the Mondini deformity, the cochlea consisting of a single basal turn with a bulbous apical dilatation. The organ of Corti was absent. The remainder of the bony labyrinth save for the cochlear aqueduct was normal. The patient''s death was due to a cerebral astrocytoma. Previous reports concerning the co-existence of gonadal dysgenesis, congenital deafness and non-gonadal neoplasia are discussed.

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