Sensory neural deafness associated with hereditary nephritis is seen in
Fanconi syndrome
Berger's disease
Albright syndrome
Alpo's syndrome
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
Kidney