Alport's syndrome

摘要: Five patients representing four kindreds of Alport's syndrome (approximately 5% the reported world literature) are presented with emphasis on audiological, electronystagmographic, and temporal bone findings. This is characterized by two basic hereditary defects sensorineural hearing loss nephritis. The cause site lesion unknown. audiological findings bilateral symmetrical loss, high short increment sensitivity index scores, type II Bekesy tracings, tone decay within normal limits point to cochlea as lesions. Thermal vestibulometry reveals a consistent decreased response successive increases in thermic stimuli, same pattern for established cases endorgan hair-cell disease. changes involve vestibular cochlear neuroepithelium.