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Diagnosing Giants: Solving the Medical Mysteries of Thirteen Patients Who Changed the World

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BY PHILIP A. MACKOWIAK MARCH 26TH 2015
Since Beethoven’s death on this day 188 years ago, debate has raged as to the cause of his deafness, generating scores of diagnoses ranging from measles to Paget’s disease. If deafness had been his only problem, diagnosing the disorder might have been easier, although his ear problem was of a strange character no longer seen. It began ever so surreptitiously and took over two decades to complete its destruction of Beethoven’s hearing.

Deafness, however, was but one of many health problems that plagued Beethoven throughout his lifetime. He suffered with intermittent episodes of abdominal pain and diarrhea from early adulthood until his death at age 56. Beginning in his 30s, he had recurrent attacks of “feverish catarrhs” (bronchitis), “rheumatism” of his hands and back, tormenting headaches, and incapacitating inflammation of his eyes. During his final year, he developed massive ascites accompanied by bloody vomitus indicative of cirrhosis of the liver. Post-mortem examination revealed vestigial auditory nerves, atrophy of the brain, an abnormally thick and dense skull, a fibrotic liver riddled with nodules the size of beans, an enlarged, hard pancreas, and abnormal kidneys. A recent analysis of a lock of hair purported to be Beethoven’s detected high levels of lead but no mercury.

If, as some have suggested, all of these abnormalities were the work of several different disorders gathered together in one poor soul, irritable bowel syndrome, alcoholic cirrhosis, and chronic lead intoxication would be three of the most likely culprits. Beethoven’s recurrent episodes of abdominal pain and diarrhea are certainly consistent with a diagnosis of irritable bowel syndrome. However, this is a diagnosis in name only since its cause is unknown and it has no effective treatment. Alcoholic cirrhosis is a reasonable explanation for Beethoven’s fibrotic liver, although cirrhosis due to alcohol does not typically exhibit nodules of the size described in Beethoven’s post-mortem report, nor would it explain the destruction of Beethoven’s auditory nerves. Chronic lead intoxication does damage nerves, and given the high levels of lead detected in the sample of Beethoven’s hair as well as the possibility that the wine he consumed was adulterated with lead, chronic lead intoxication is a diagnosis that can’t be ignored. However, although lead is toxic to nerves and also causes abdominal distress, it characteristically impairs the function of motor nerves (producing palsies), not sensory nerves such as the auditory nerves. Moreover, the abdominal complaints most commonly associated with chronic lead intoxication are recurrent pain, which Beethoven had, and nausea, vomiting, loss of appetite, and constipation (not diarrhea), which he did not have.

If Beethoven’s various disabilities were the result of a single disease rather than a multitude of diseases congregated in one unfortunate person, only syphilis could explain the character and course of his illness, in addition to virtually all of the post-mortem findings. Syphilis, the “great imitator,” has clinical manifestations so protean that Sir William Osler was moved to remark: “He who knows syphilis, knows medicine.” In its advanced stage, which may take decades to reach climax, syphilis can inflict heavy damage on all of the organs affected by Beethoven’s illness. The full spectrum of disabilities orchestrated by the infection has largely been forgotten thanks to the advent of penicillin, which is spectacularly effective in eradicating the infection. However, if one examines the extensive literature devoted to syphilis prior to the antibiotic era, the disorder emerges as a highly satisfactory explanation for virtually all of Beethoven’s ailments.

Beethoven’s deafness, for example, had a course along with associated abnormalities of the acoustic nerves found on post-mortem examination that are unlike any disorder encountered today. In over three decades of practice as an infectious diseases consultant, I have encountered no such patient, nor have several prominent neurologists and ear specialists, with whom I’ve discussed Beethoven’s case. Those who practiced medicine in the pre-antibiotic era saw many such cases of slowly progressive, bilateral destruction of the acoustic nerves. More often than not, the cause was syphilis.

Beethoven’s episodes of eye inflammation, which today we would call “interstitial keratitis,” his thick cranium, non-deforming rheumatism, macro-nodular cirrhosis, irritable bowel, tormenting headaches (“migraines”), and abnormal pancreas were likewise manifestations of advanced syphilis encountered during the pre-antibiotic era.

Unlike more exotic disorders offered over the years as Beethoven’s diagnosis, syphilis is a disease some would consider too banal and unbecoming to have extinguished the life of such a remarkable patient. The very thought that syphilis might have been the disease that silenced the source of some of the most sublime sounds ever conceived offends our sense of cosmic harmony. Nevertheless, whereas Beethoven was an artist, he was also a man. It made no difference to the disease that his nine symphonies, five piano concertos, violin concerto, seventeen string quartets, opera, and thirty-two piano sonatas were some of the most brilliant ever composed. Nor did it pause to consider whether the deafness it induced might impair the composer’s creativity, or to the contrary, enhance it by allowing Beethoven to perceive the new brand of polyphonic music that was to be his greatest gift to mankind.

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