Alport's Syndrome Most common form of hereditary nephritidis Patients develop glomerulonephritis that progresses to renal failure Some patients develop hearing loss 10% have eye abnormalities such as cataracts Leopard Syndrome Autosomal dominant Presenation lentigines EKG abnormalities ocular hypertelorism pulmonary stenosis abnormal genitalia growth retardation sensorineural deafness Pendred's Syndrome Autosomal recessive Presentation deafness and goiter the goiter appears at puberty and most patients are euthyroid Waardenburg's Syndrome Autosomal dominant Presentation white forelock pathches of hypopigmentation sensorineural hearing loss lateral displacement of the inner canthi sometimes concurrent with Hirschsprung disease Usher's Syndrome Presentation deafness mental retardation seizures retinitis pigmentosa cataracts