Ear Anomalies Associated with Renal Dysplasia and Immunodeficiency Disease

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Abstract
The histopathologic study of the temporal bones of a case with low-set rudimentary auricles, without preauricular pits or cervical fistula is presented. Abnormalities of the middle and inner ear, fusion of the kidneys, hydrocephalus, short-limbed dwarfism and immunodeficiency are described. An abnormally low position of the middle cranial fossa in relation to the petrous pyramid was observed. The cochlea was of normal length. The modiolus was poorly developed with apparently normal population of ganglion cells and moderate diffuse hydrops of the cochlear duct and saccule. The stria vascularis was partially degenerated in the upper apical coil. Vestibular abnormalities included bilateral absence of common crus of the vertical canals and unusually high origin of endolymphatic aqueduct with no medial dilation present, the convoluted portion of the sac located beneath the dura.