Sensory neural deafness associated with hereditary nephritis is seen in

Clinically patients with Alpos syndrome develop hematuria, thinning and splitting of GBM's,mild proteinuria (<1-2g/24hrs) which appears late in the course followed by chronic glomerulosclerosis leading to renal failure associated with sensorineural deafness. There r four forms of alpos syndrome Type 1 classic As -x linked disorder with Hematuria,sensorineural deafness ,lenticonus Type 2-x linked form associated with diffuse leiomymomatosis T3 -autosomal recessive ,T4autosomal dominant -both forms can cause renal disease without deafness or lenticonus. Ref:Harrison 20 th edition pg no 2146,2977