1/2. Evidence of central vestibulo-auditory dysfunction in atypical Cogan's syndrome: a case report.Auditory and vestibular functions were investigated in a patient with atypical Cogan's syndrome. The brainstem auditory evoked response revealed bilateral severe distortion of morphology and voltage diminution after potential I, greater in right ear recordings. Interwave latency I-III was increased bilaterally. Electronystagmographic studies showed nonfatigable direction changing positional nystagmus greater with right ear undermost and bilateral failure of optic suppression. Findings suggested bilateral pontocerebellar dysfunction. Correlation was found with the side of greater clinical manifestations. The patient had excellent recovery of auditory and vestibular functions with administration of corticosteroids within 2 weeks of the onset of hearing impairment. She was followed up with auditory and vestibular studies for 30 months.- - - - - - - - - - ranking = 1keywords = optic (Clic here for more details about this article) |
2/2. Histopathology of the ears, eyes, and brain in Norrie's disease (oculoacousticocerebral degeneration).Norrie's disease is an x-linked recessive disorder characterized by progressive oculoacousticocerebral degeneration. The light and electron microscopic changes in the temporal bones, eyes, and brain of an affected 77-year-old man who suffered from bilateral profound sensorineural hearing loss, blindness, and mental retardation are described. The inner ears showed marked atrophy of the stria vascularis, severe degeneration of hair cells and cochlear neurons, and connective tissue proliferation in the spiral ganglion, osseous spiral lamina, and walls of the membranous vestibular labyrinth. The eyes showed detached retinae, dense proliferation of fibrillary glial cells in the retina and vitreous, severe atrophy of the optic nerves, and degenerative hyalinization of blood vessels. This case is the first published report of the histopathology of the inner ear in Norrie's disease.- - - - - - - - - - ranking = 2.6696271727266keywords = optic, atrophy (Clic here for more details about this article) |