Systemic sclerosis is a multisystem disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. Cranial nerve involvement is an uncommon feature of this connective tissue disorder, and when it occurs it is the trigeminal nerve that is primarily affected. We report an elderly woman who presented with sensorineural hearing loss and was then diagnosed with the CREST syndrome of progressive systemic sclerosis (calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia). Involvement of the eighth cranial nerve with scleroderma and CREST syndrome is rare, but appears to be the cause of sensorineural hearing loss in our patient.