Dr. Ron’s Research Review – September 13, 2017

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This week’s research review focuses on Ludwig van Beethoven and lead poisoning.

Beethoven’s deafness began when he was 28 years of age. The left ear was involved first, and initial symptoms included tinnitus and high-tone hearing loss, which was associated with poor discrimination and recruitment. The symptoms were intermittent but after 1 year, they became persistent. (Hui and Wong, 2000)
Beethoven was not an alcoholic, but he consumed relatively large amounts of alcohol every day, mainly wine and dessert wine (Hungarian Tokayer wine) taken with the daily meals, and sometimes beer, as described by friends and visitors and by his physician. (Schwarz, 1993)
Based on his autopsy, Ludwig van Beethoven was diagnosed with renal papillary necrosis that was most probably the consequence of analgesic abuse together with decompensated liver cirrhosis. The autopsy report of Beethoven is the first case of papillary necrosis recorded in the literature. (Schwarz, 1993)
Analgesic abuse because of headaches, back pain, and attacks of rheumatism or gout may be presumed on the basis of Beethoven's uncontrolled way of taking medication. Salicin, a commonly used analgesic substance of that time (dried and powdered willow bark), is able to cause papillary necrosis. (Schwarz, 1993)
Recent research, however, disproves this theory and points to lead poisoning with repeated low level exposure over a period of many years, based on recent analysis of his hair and bone.
At that time lead was added illegally to inexpensive wine to improve the flavor. Beethoven was particularly fond of the adulterated or fortified Hungarian wine. When he was 30 years of age, Beethoven began to consume a lot of wine with meals to stimulate his appetite and ease his abdominal pain. This coincides with when he confessed his hearing loss to others.
Evidence of otosclerosis is lacking because close gross examination of Beethoven's middle ears at autopsy did not find any otosclerotic foci. High levels of lead deep in the bone suggest repeated exposure over a long period. Shrunken cochlear nerves are 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 chronic consumption of wine tainted with lead is a better explanation of his hearing loss than other causes. (Stevens et al., 2013)

Dr. Ron


 

Articles

Deafness and liver disease in a 57-year-old man: a medical history of Beethoven.
            (Hui and Wong, 2000) Download
Ludwig van Beethoven had a number of medical conditions, including deafness and chronic liver disease, for which there are contemporary descriptions. An autopsy was performed on the day after his death. Physicians and historians have tried to reinterpret original sources to determine the causes of his deafness and systemic illnesses. We have reviewed the differential diagnoses that have been proposed by otologists and physicians. Clinical and post-mortem findings point to renal papillary necrosis and liver cirrhosis of unknown aetiology. In the absence of further histological examination, there is no definitive answer to the cause of his deafness and gastro-intestinal symptoms.

Beethoven's renal disease based on his autopsy: a case of papillary necrosis.
            (Schwarz, 1993) Download
The autopsy report of Ludwig van Beethoven written by Dr Johann Wagner in 1827 reveals that he had renal calculi that had not been diagnosed during his lifetime, together with perirenal fibrosis. The most comprehensive interpretation of this autopsy finding is that the regular calcareous deposits in every one of his renal calices represented calcified necrotic papillae. Severe urinary obstruction or diabetes as possible causes of papillary necrosis were not present. Analgesic abuse because of headaches, back pain, and attacks of rheumatism or gout may be presumed on the basis of Beethoven's uncontrolled way of taking medication. Salicin, a commonly used analgesic substance of that time (dried and powdered willow bark), is able to cause papillary necrosis. Perirenal fibrosis may be due to chronic infection or drug intake. Beethoven's other well-known diseases are deafness caused by otosclerosis of the inner ear, relapsing attacks of diarrhea as the symptoms of irritable bowel syndrome, and liver cirrhosis following viral hepatitis and chronic alcohol consumption. Liver cirrhosis also may cause papillary necrosis. In Beethoven's case, renal papillary necrosis was most probably the consequence of analgesic abuse together with decompensated liver cirrhosis. The autopsy report of Beethoven is the first case of papillary necrosis recorded in the literature.


 

Lead and the deafness of Ludwig van Beethoven.
            (Stevens et al., 2013) Download
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.

 

References

Hui, AC and SM Wong (2000), ‘Deafness and liver disease in a 57-year-old man: a medical history of Beethoven.’, Hong Kong Med J, 6 (4), 433-38. PubMed: 11177170
Schwarz, A (1993), ‘Beethoven’s renal disease based on his autopsy: a case of papillary necrosis.’, Am J Kidney Dis, 21 (6), 643-52. PubMed: 8503419
Stevens, MH, T Jacobsen, and AK Crofts (2013), ‘Lead and the deafness of Ludwig van Beethoven.’, Laryngoscope, 123 (11), 2854-58. PubMed: 23686526