The Mondini defect in Turner's syndrome. A temporal bone report.
The histopathology of the right temporal bone of a 47 year 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.
D001254:Astrocytoma / Q000150:complications; D001932:Brain Neoplasms / Q000150:complications; D003051:Cochlea / Q000473:pathology; D003638:Deafness / Q000150:complications / Q000151:congenital / Q000473:pathology*; D007758:Ear, Inner / Q000002:abnormalities / Q000473:pathology*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009925:Organ of Corti / Q000002:abnormalities; D014424:Turner Syndrome / Q000150:complications*