A family affected with oculopharygeal muscular dystrophy (OPMD) is reported. This is an uncommon progressive myopathy. The proband presented for evaluation of secretory otitis media with effusion, as a result of tubal dysfunction. CT examination of the father revealed prominent muscular atrophy, and widespread fatty degeneration of the psoas, paraspinal, gluteal and femoral muscles. (C) 2001 Published by Elsevier Science Ltd.