We report a 53-year-old male having Kennedy disease who complained of severe bilateral tinnitus and autophony. He wasdiagnosed as patulous Eustachian tube based on the observation of fluttering motion of the tympanic membrane associatedwith respiration, which is presumed to occur secondary to bulbar weakness. The patulous Eustachian tube affected the managementof his respiratory problem by impairing his ability to tolerate BiPAP. We report this rare condition with a literaturereview.