Adam K Kubba & Madeleine Young. The Lancet. Volume 347. January, 1996.
The controversy surrounding Ludwig van Beethoven’s personal tragedy is mountainous. A supreme moulder of mighty harmonies, his compositions in a short life include: nine symphonies, five piano concertos, one violin concerto, 17 string quartets, one opera, and 32 piano sonatas.
Ironically, he struggled for many years against the imprisonment of a world devoid of all sounds. Therefore, it is not surprising that much is written about Beethoven’s deafness. However, his systemic illnesses remain largely unilluminated. In this article, we aim to put the historical records and previous medical biographies of this musical genius under the knife of a gastrointestinal surgeon. The main purpose of this review, therefore, is to reinterpret the facts and the opinions of previous medical biographers, in the light of modern clinical knowledge. We hope to widen discussion beyond his extensively reported and much analysed deafness and to illuminate the composer’s more obscure medical difficulties.
Beethoven was born in Bonn on Dec 15, 1770. His father was an alcoholic and died of alcohol misuse in 1792, his mother having died 5 years earlier of tuberculosis. Beethoven had two younger brothers. Casper Carl (1774-1815) died of tuberculosis, leaving a son Karl, and Nikolaus Johann (1776-1852) died of atherosclerotic heart disease.
Beethoven remained in Bonn for the first 22 years of his life, but after his father’s death he moved with his family to Vienna. During the first 20 years of his life, little is known about his medical history other than that he was thought to be asthmatic since the age of 5 and continued to have a weak chest. However, it is not known whether he had tuberculosis. He also had smallpox, which resulted in facial scarring.
Beethoven’s abdominal disorders started in 1792 with recurrent abdominal pains and diarrhoea. During the summer of 1797 he had severe diarrhoea which is thought to have been related to typhoid fever. In 1801, he again complained of recurrent central abdominal pains and alternating bouts of diarrhoea and constipation, the latter occasionally leading to obstipation. In isolation, the gastrointestinal symptoms suggest two common diagnoses-namely, inflammatory bowel disease and irritable bowel syndrome.
Between 1812 and 1820, he repeatedly complained of diarrhoea, dehydration, prostration, anorexia, and abdominal colic. The colic was so severe that he was said to have had to “increase his alcohol intake to kill the pain.” However, towards the end of his life, he found that alcohol exacerbated the abdominal pain and diarrhoea. In 1821, he had a prolonged attack of jaundice, abdominal pain, and vomiting. The icterus remitted after several months. At this stage, one might consider viral or alcoholic hepatitis and chronic or recurrent pancreatitis as possible diagnoses.
Hearing and Psychological Difficulties
The information about Beethoven’s deafness is based on his letters to close friends, his conversation, books, and the findings of the post-mortem examination. At the age of 28, Beethoven started to complain of mild fluctuating deafness (left ear before right), which was associated with tinnitus and was preceded by a febrile illness. 3 years later he was still able to hide his deafness. Thereafter the problem became relentlessly progressive. He wrote in 1801,
“My hearing [has] grown steadily worse for three years … My ears whistle and buzz continually, day and night. In any other profession this might be more tolerable, but in mine such a condition is truelly frightful … To give you some idea of my extraordinary deafness, I must tell you that in the theatre I am obliged to lean close up against the orchestra in or to understand the actors, and when a little way off I hear none of the high notes of instruments or singers, and if I be a little farther away I do not hear at all. Frequently, I can hear the tones of low conversation but not the words and as soon as anyone shouts it is intolerable.”
By 1814, at the age of 44, he became stone deaf, and conversation became impossible. He struggled to use an eartrumpet (made by Johann Malzel), which he put on with a headband, leaving his hands free for conducting, but in 1822 he stopped conducting when he realised that he had become a serious hindrance to the performances. His speech remained intact but he was heavily dependent on the famous conversation pads. The possible causes of deafness are discussed in the panel.
In the last few years of life, his deafness was so profound that in 1824, when he was the honorary conductor at the premier performance of his ninth symphony, one of the soloists approached and kindly turned him towards the ovation of the cheering audience when the performance had finished. This condition led to fear, lack of self-esteem, emotional disarray, increasing isolation, and self-neglect.
By 1822, Beethoven began to neglect himself grossly, and he drank excessive amounts of “punch & wine,” especially the Hungarian variety. He was often seen walking the streets with no hat, an old coat, and rough appearance. His behaviour became odd, and he had frequent outbursts of temper. His relationship with his doctors (most of whom were at the top of their profession) was worse than ever; he called them bumbling doctors and medical asses. At the same time he started to complain of severe longlasting headaches, increased gastrointestinal problems and recurrent rheumatic attacks. His letters state that he was hardly ever free from these gastrointestinal and rheumatic complaints.
Deafness had an important influence on Beethoven’s compositions, which are divided into three general periods by musical biographers. The first, thought to extend to 1800 and the onset of his deafness, shows the influence of previous composers such as Mozart and Haydn. The middle period corresponds to the start and progression of his deafness (1800-15) during which he wrote the beautiful Moonlight Sonata and the third, fourth, fifth, and sixth symphony. The third period coincides with when he was stone deaf and had recurrent severe ill health and mental deteriorations. It was in these silent years that the tremendous ninth symphony and Missa Solemnis were composed. Much of the greatest music that ever influenced millions of people flowed from the mind of a man who never heard any of it.
Beethoven often referred to his rheumatic problems in conjunction with his abdominal complaints. In 1804, he had severe fever and an abscess that almost caused the loss of a finger, followed by, a year later, another abscess on his jaw. In 1822 he had “thoracic gout,” and, although the occurrence of this illness is well documented, no details have been available about the symptoms and signs associated with it. Between August, 1823, and June, 1824, the several references to a painful eye, signal a likely case of uveitis.
The Final Illness
In 1825, Beethoven again had jaundice, which was associated with mental and physical deterioration, including pyoderma (especially of the feet), recurrent nose bleeds, and recurrent haemoptysis. In November, 1826, he left Vienna for the countryside to work on quartets Opus 130 and 135. However, he soon had to return to Vienna on Dec 2 because of poor health. On his arrival, he was hyperpyrexial and thoroughly exhausted from chills and chest pain. His doctors detected ankle and pretibial swelling, cough, fever, anorexia, hepatomegaly, and ascitis. Hepatic failure and pneumonia were diagnosed.
By January, 1827, his abdomen was grossly distended with ascitic fluid, which was repeatedly tapped (paracentesis). He was given alcohol by doctors to ease the severe pain and prevent delirium tremens. In early March, 1827, Beethoven was attended by Dr Andreas Wawruch, who now reported shortness of breath, haemoptysis, and right-sided chest pain. Wawruch diagnosed pneumonia and ordered complete bed rest. A week later, he recorded again that Beethoven had diarrhoea, jaundice, vomiting, distended abdomen, gross ankle swelling, and eventually anuria.
In the following week, there were increasing signs of cardiorespiratory, hepatic, and renal failures. In mid-March, Wawruch used paracentesis twice, draining as much as 11 L and 22 L of fluid on the two occasions, on one of which the site of paracentesis became infected. Thereafter, ascitic fluid continuously leaked from the abdomen. By March 24, Beethoven was comatosed. 2 days later, at 1800 h, he died.
Necropsy was done by Dr Johann Wagner and Dr Karl Rokitansky. The examination was the first of at least three, but the findings of the first examination are probably the most authoritative. In the abdomen they reported liver cirrhosis of the macronodular variety (“shrunken liver”); this finding does not support alcoholic cirrhosis, which is usually of the micronodular type. They also found portal hypertension and splenomegaly (“the spleen three times the normal size”), observations that are consistent with severe and probably chronic liver disease. 9 L of infected ascitic fluid was present in the peritoneal cavity. Ascites is not uncommonly associated with hepatic failure, and the infection is almost certainly secondary to repeated paracentesis.
Chronic pancreatitis (“very firm abnormal pancreas”) and cholilithiasis (“gall stones”) were also reported.
In the head and neck, they commented that the vault of the skull was substantially and uniformly dense (estimated at 0.5 inches thick). The auditory nerves were very thin (the left thinner than the right), and the auditory arteries were atherosclerotic. However, there was no evidence of endarteritis obliterans, which is often associated with chronic inflammatory conditions such as syphilis. The ossicles and petrous temporal bone were saved for examination but were lost or stolen.
Even though the body was exhumed twice, in 1863 and 1888, no significant further knowledge was obtained.
Some authors have attempted to explain Beethoven’s illnesses as separate clinical entities, while others have sought to find a syndrome by which most, if not all, of these disorders could be explained. Several diseases have been suggested.
Systemic Lupus Erythematosus (SLE)
Larkin suggested that Beethoven had SLE. This diagnosis explains the rheumatic joint pains (SLE arthropathy), and also accounts for the eye problems (SLE-related uveitis) and recurrent nose bleeds (thrombocytopenia). However, we believe this is an unlikely diagnosis because, firstly, SLE is rare among men of the composer’s age group at the start of his illness; secondly, it is very rarely associated with liver cirrhosis (a major cause of Beethoven’s death); and, thirdly, many of the characteristics that we associate with SLE, such as alopecia, pericarditis, lymphadenopathy, and significant renal disease, are absent from the composer’s medical history.
Paget’s Disease of Bone
Naiken suggested Paget’s disease of bone as an explanation. He based his theory on unsubstantiated evidence that Beethoven, who was short (around 1.65 m), had an asymmetrical head, large forehead, over-hanging brows, protruding lower mandible, large hands, and thick fingers. All these observations might have been true to an extent. However, there is nothing to suggest that Beethoven’s appearance changed in this way. It has also been suggested that Paget’s disease would explain the composer’s recurrent headaches and progressive deafness which some argue was due to a mixture of nerve deafness (caused by compression from overgrowth of surrounding bony structures) and bony deafness (caused by Paget’s induced otosclerosis). It has also been argued that his recurring abdominal pains were caused by recurrent renal colic secondary to nephrolithiasis induced by Paget’s related hypercalcaemia. Furthermore, the post-mortem findings of a narrowed eustachian tube, especially at the bony part, and a large external auditory meatus was cited by some as further evidence suggestive of Paget’s disease.
We do not however believe that Beethoven had Paget’s disease that was severe enough to cause his deafness, abdominal problems, and death. Firstly, the thickening of the skull was uniform (unlikely in Paget’s disease); secondly, Paget’s disease does not cause severe diarrhoea and does not explain Beethoven’s early gastrointestinal symptoms; and thirdly, Paget’s disease is very rare below the age of 50 years.
Tuberculosis (which killed at least two members of Beethoven’s family) has been put forward as a cause of Beethoven’s illness and death. However, this is most unlikely since he would have to have had miliary disease to explain his rheumatic, gastrointestinal, and neurological disorders. No-one is expected to survive 30 years of this rapidly lethal disease. We believe that Beethoven probably had tuberculosis early in his life (which may account for his early respiratory difficulties), but it is very doubtful whether this disease had a major role in his life-long afflictions and death.
Sarcoidosis has also been advocated as a possible explanation of Beethoven’s illness. This granulomatous disease is often associated with tuberculosis, and it affects the lungs, heart, eyes, the central nervous system, and other organs. Though a good explanation for his eye conditions, sarcoidosis could not account for Beethoven’s deafness. He would have had to have developed neurosarcoidosis with all its associated neurological deficits for it to have caused loss of hearing. Others’ wondered whether Beethoven’s facial scarring was due to “lupus pernio” (known to be associated with sarcoidosis). However, one cannot dismiss the most common cause of facial scarring at the time–smallpox.
As far as the abdominal symptoms are concerned, sarcoidosis causes nephrolithiasis (found at necropsy) but gastrointestinal sarcoid is not only rare but also mostly symptomless.
Inflammatory Bowel Disease
Abdominal pains and chronic diarrhoea are the hallmarks of inflammatory bowel disease. Arthropathy, iritis, uveitis, and chronic liver disease (chronic active hepatitis and Crohn’s-related sclerosing cholangitis) have all been identified with inflammatory bowel disease.
However, adhesions, strictures, or perforations were not mentioned in the necropsy report. Furthermore, there is no strong history of rectal bleeding, which is commonly associated with ulcerative colitis.
Beethoven’s association of many of his problems with an illness he had around 1792 direct us towards the possibility of dysentery leading to enteritis which can cause seronegative reactive arthritis, sacroileitis ankylosing spondylitis, and recurrent uveitis.
This rare systemic disorder, caused by Tropheryma whippelli, may have an autoimmune aetiology which predisposes the patient to infection with this organism. The disease is most often seen in the fourth and fifth decades of life, is nine times more common in men than women, and predominantly effects whites (85%). One main abnormality found in this disease is gastrointestinal features leading to malabsorption and chronic diarrhoea which Beethoven had. The extraintestinal features include arthritis and arthralgia, hepatosplenomegaly (infrequent), thrombocytopenia (in 15% of cases), pancreatitis, renal impairment, personality changes, changes in the central nervous system, and respiratory disorders (especially recurrent haemoptysis).
Beethoven exhibited most of the above abnormalities. Lastly, this disease is associated with valvular heart disease in 25% of cases, although there is no indication that Beethoven had this condition.
Many medical biographers have sought the exotic and allencompassing diagnosis. Our interpretation of the historical records and the published literature lead us to suggest that the cause of Beethoven’s deafness is that encountered most commonly in adult ear, nose, and throat practice namely, a mixture of nerve deafness and a degree of otosclerotic bony deafness, both of which are unlikely to be related to his other medical conditions.
There is enough evidence to suspect that Beethoven’s gastrointestinal complaints were secondary to inflammatory bowel disease, Crohn’s disease being more likely than ulcerative colitis. Crohn’s disease-related arthropathy and uveitis would account for his rheumatological complaints. Liver cirrhosis could be associated with inflammatory bowel disease, and in Beethoven must have been accelerated by alcohol misuse which caused his chronic pancreatitis. Hepatic failure was undoubtedly responsible for Beethoven’s ultimate death.