Autosomal recessive Alport syndrome-3B (ATS3B) is a progressive hematuric glomerulonephritis characterized by glomerular basement membrane abnormalities. Sensorineural hearing loss and ocular manifestations may be present (summary by Boye et al., 1998). For a general phenotypic description of Alport syndrome, see the X-linked dominant form (ATS1; 301050). [from OMIM]