The hearing loss of Ludwig von Beethoven has fascinated physicians, audiologists, and musicologists for almost 200 years. In this review of the literature, the features, treatment, and differential diagnosis of the composer's malady are discussed. An original series of hypothetical audiograms and actual photomicrographs depicting cochlear otosclerosis and Paget's disease are also provided. The author concludes with the majority of otologists that cochlear otosclerosis is the most likely diagnosis for the hearing loss. In addition, a summary of Beethoven's complicated medical history and results of the autopsy that confirmed chronic liver disease are included.