Facioscapulohumeral dystrophy associated with hearing loss and coats syndrome

Abstract Two unrelated cases of infantile facioscapulohumeral dystrophy (FSHD) with Coats syndrome of the retina and sensorineural hearing loss are described. Children with otherwise unexplained visual or hearing deficit should be examined for somatic muscle weakness, which may present only as facial weakness. Early recognition of Coats syndrome in children with FSHD may lead to preservation of vision with appropriate therapy.