Hematuria which causes an actual change in the color of the urine, generally occurs only at times of infection and fever. Microscopic hematuria which is detected only in the laboratory may be present from birth, and may be the only sign of kidney involvement in females. Medical management of the kidney disease includes a low protein diet, control of high blood pressure, and eventual dialysis or transplant. Hearing loss commonly occurs in childhood. Most subtypes of AS include a bilateral progressive sensorineural hearing loss of mild to moderate degree, but some individuals may have a severe to profound loss.
Females generally have a milder hearing loss than their male relatives if they have one at all.
Management of the hearing loss includes regular audiologic follow-up, use of hearing aides, and avoidance of certain drugs and excessive noise exposure that can cause further hearing loss. At this time a gene for autosomal dominant AS has not been identified. Second, the Registry collects information from individuals interested in supporting and participating in research projects. This information is used to support the third function of the Registry - matching families with collaborating research projects.