The diagnosis of classic AS is based on X-linked inheritance of hematuria, sensorineural deafness, and lenticonus. Because of the X-linked transmission, women are generally underdiagnosed and are usually less severely affected than men. The hematuria progresses to nephritis and may cause renal failure in late adolescence in affected males and at older ages in some women. The sensorineural deafnes is primarily in the high-tone range. It can frequently be detected only by an audiogram and is usually not progressive. The lenticonus can occur without nephritis but is generally considered to be pathognomonic of classic ASR. Renal transplantation is usually successful.">