Alport syndrome. Report of two cases Alport syndrome is an inherited progressive form of glomerular disease that is often associated with sensorineural hearing loss and ocular abnormalities. We report two men with Alport syndrome. Both had chronic kidney disease and consulted for long-term loss of visual acuity. One had auditory abnormalities. On the ophthalmological examination, both had anterior lenticonus and one had dot or fleck retinopathy. Those findings are described in up to 50% and 70% of men with X-linked Alport syndrome, respectively. Both patients had a family history of Alport syndrome or suggestive signs and symptoms.
Aside from the clinical picture, the frequent occurrence of vascular risk factors favors a vascular pathogenesis of acute sensorineural hearing loss. However, the data on the association of vascular risk factors with sensorineural hearing loss are controversial. Achieving a sufficient number of patients and establishing a suitable control group is difficult. We analyzed the data of 393 patients suffering from acute sensorineural hearing loss. Risk factors, general, audiological, and laboratory parameters were investigated. Mean hearing loss and remission during 10 days of hospital treatment were calculated. The patient group was divided into patients with a certain risk factor and those without this risk factor, and mean remission and hearing loss were compared. Patients suffering from hyperlipidemia or hypotension had substantial and significantly higher mean hearing losses than patients not suffering from these diseases. Thrombosis, embolism, and hypertension were associated with a significantly worse remission of hearing loss. Repeated episodes of sensorineural hearing loss showed a significant worse remission, but less mean hearing loss before therapy. Smoking, diabetes mellitus, infections of the upper airways, and allergies had no influence on remission and mean hearing loss.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.