ABSTRACT
OBJECTIVES/HYPOTHESIS: To reexamine the cause of Beethoven's hearing loss because of significant recent articles. DATA SOURCES: Medical and musical literature online, in print, and personal communication. METHODS: Relevant literature review. RESULTS: Evidence of otosclerosis is lacking because close gross examination of Beethoven's middle ears at autopsy did not find any otosclerotic foci. His slowly progressive hearing loss over a period of years differs from reported cases of autoimmune hearing loss, which are rapidly progressive over a period of months. He also lacked bloody diarrhea that is invariably present with autoimmune inflammatory bowel disease. The absence of mercury in Beethoven's hair and bone samples leads us to conclude that his deafness was not due to syphilis because in that era syphilis was treated with mercury. High levels of lead deep in the bone suggest repeated exposure over a long period of time rather than limited exposure prior to the time of death. The finding of shrunken cochlear nerves at his autopsy is consistent with axonal degeneration due to heavy metals such as lead. Chronic low-level lead exposure causes a slowly progressive hearing loss with sensory and autonomic findings, rather than the classic wrist drop due to motor neuropathy from sub-acute poisoning. Beethoven's physicians thought that he had alcohol dependence. He particularly liked wine that happened to be tainted with lead. CONCLUSIONS: Beethoven's chronic consumption of wine tainted with lead is a better explanation of his hearing loss than other causes.
