Mutations in MYH9 cause DFNA17 and can also result in a series of syndromes associated with hearing loss in up to 50% (e.g., Alport syndrome, Epstein syndrome, Fechtner syndrome and macrothrombocytopenia with progressive sensorineural deafness). This evidence concerns the gene MYH9 and macrothrombocytopenia and granulocyte inclusions with or without nephritis or sensorineural hearing loss.