What caused Beethoven’s hearing loss?
In Episode 54, A Lincoln Theory, we discussed an original theory about Abraham Lincoln’s health and whether he might have had Loeys-Dietz syndrome. The health of another famous historical figure, composer Ludwig van Beethoven, has been in the news lately. In this episode, we investigate what may have caused Beethoven’s infamous deafness, along with a myriad of other chronic ailments that he suffered from.
Beethoven is a fascinating historical figure: a titan of classical music, easily a top three composer of all time, with some considering him to be the greatest in terms of the scope and power of his compositions. He had an enigmatic persona, which created a mystique about him. But his story is a medical one too, as he famously began to lose his hearing early in his career and wrote many of the pieces that he’s best known for while partially or, at the end of his life, even completely deaf. And he had a number of other physical symptoms throughout his life that may tie into potential unifying diagnoses to explain his chronic hearing loss.
Beethoven was born in Bonn, Germany in 1770. He was recognized to have considerable musical talent as a child and came from a family of musicians. His father, Johann, tried to promote Beethoven as a child prodigy in the fashion of Mozart, and as he began to carve a career as a composer in Vienna in his early 20s he wrote music with a Mozartian syle. But over time, he found his own unique voice as a composer, and musicologists categorize his career into three main periods. A first, conventional period in which he wrote music that sounded like other popular classic music at the time, such as Mozart and Haydn; a middle period that has been called Beethoven’s heroic period, as he often wrote music on grand scales; his final, late period was characterized by the some of the most complex, innovative, and personal music of his career.
Beginning in his late 20s, he began to slowly but steadily lose his ability to hear. At the same time, he also developed significant tinnitus, which added to his difficulty performing as a pianist or conducting his own music. Over time he became more isolated and depressed and even contemplated suicide. But he found a way to persevere and continue to compose, even though it became increasingly hard to hear the music he wrote. He apparently could continue to hear very loud or low sounds but was otherwise completely deaf for the latter portion of his career.
Beethoven consulted a number of top physicians in Vienna to try to find a solution for his hearing loss. One was Johann Frank, who tried stuffing cotton balls soaked in almond oil into his ears. But Beethoven wrote in a letter that Frank’s treatment had no effect, and that his deafness became even worse. Gerhard Von Vering, a surgeon who was director of the Viennese Health Institute, prescribed bathing in water from the Danube River and drinking herbal tonic. When these didn’t work, Von Vering somewhat desperately tried the application of bark from the toxic mezereum plant to Beethoven’s arms using belts. This led to debilitating hypersensitivity reactions and painful blistering, which precluded Beethoven from playing or composing for days at a time after each application. He tried living in the country to rest his ears from the bustle of city life and may have used leeches and galvanic therapy involving focused electric shocks to different parts of the body. It is hard to know exactly what he attempted since we don’t have a medical record to reference. Most of what we know comes from his correspondence and references he made in conversations with others.
Beethoven used more traditional adaptive aids including ear trumpets, and had a special piano made with extra strings and a resonator to increase the volume of sound it produced. But much of his later music, including some of the most important classical music ever written, was composed in silence.
In addition to hearing loss, Beethoven suffered from a number of other symptoms throughout his life, which he wrote in correspondence and letters. In 1823 he had a red and painful eye that lasted for the better part of a year, which may have represented an inflammatory eye process such as uveitis or keratitis. He had chronic abdominal pain, diarrhea, and migratory joint pains, which have led some to whether he had inflammatory bowel disease. He also drank alcohol heavily throughout his life. His final illness in 1827 was characterized by a global decline with anasarca, renal failure, peritonitis, and confusion, all consistent with decompensated cirrhosis. Apparently, one paracentesis drained a whopping 22 liters – nearly 50 pounds – of ascitic fluid. And his autopsy by Johann Wagner and Karl Rokitansky was consistent with decompensated cirrhosis, noting a nodular, shrunken liver, splenomegaly, and 9 liters of infected ascitic fluid in his abdomen. They also found chronic pancreatitis, evidenced by what they called a “firm abnormal pancreas.”
Beethoven requested the autopsy to attempt to solve the mystery of his hearing loss. In their report, Wagner and Rokitansky note that his skull was very dense and thick. The auditory nerves were thin and atrophied, with the left more atrophied than the right. And perhaps most dramatically, the auditory arteries were hard and sclerotic. As Wagner noted, Beethoven’s auditory arteries were “thickened and cartilaginous as though stretched over a raven’s quill.”
There have been many diagnostic theories offered to explain his deafness and his other, apparently systemic, symptoms. Some have wondered whether he had syphilis, which could have caused the vascular sclerosis seen on his autopsy. Others have suggested Paget’s disease because of the thickened skull. Still others have theorized sarcoidosis or lupus or even chronic lead poisoning based on an analysis of a purported lock of Beethoven’s hair. Most have seen his cirrhosis as a separate problem related to chronic alcohol use.
Most recently, in April 2023, an article was published in Current Biology, an open-access journal from Cell. The authors analyzed 8 locks of hair attributed to Beethoven. Each lock of hair has a name (e.g., the Stumpff lock, the Bermann lock, and the Halm-Thayer lock) based on who was originally intended to possess them or did for an extended period of time. Ironically the authors found that only five of the hairs were authentic (i.e., having come from the same male of European ancestry). One of the inauthentic hair, the Hiller lock, was the source of the theory that Beethoven was chronically lead poisoned. But the Hiller lock originated from a woman of mixed North African and Middle Eastern ancestry, undercutting the key piece of evidence for this theory.
The authors used the five authentic locks to sequence Beethoven’s genome. In doing so, they identified a genetic predisposition to liver disease, with homozygosity for a gene variant (PNPLA3) associated with an increased risk. They also found that he was compound heterozygous for HFE gene mutations, which can cause hemochromatosis. The authors noted that because of the low penetrance of HFE we can’t know if he was clinically iron overloaded, but these results suggest that he was at increased risk for liver disease, especially in combination with his alcohol use. If that wasn’t enough, they also found he was chronically infected with hepatitis B, which probably further predisposed him to cirrhosis and ultimate liver failure. And finally, they discovered that based on Y chromosome analysis there was an extra-pair paternity event somewhere in his paternal line between 1572-1770 (Beethoven’s year of birth). This means there was a source of Y chromosomes outside of the Beethoven lineage (i.e., there was very likely an affair somewhere in that timespan).
Several years ago, Avi, Joe Tremaglio, and Sunil Nair discussed a unifying theory that could explain a lot of Beethoven’s symptoms, including his deafness: Cogan Syndrome.
Cogan Syndrome was first described by an ophthalmologist named David Cogan in a case series published in what’s now JAMA Opthalmology in 1945. Cogan reported 4 patients with inflammatory eye disease from keratitis, tinnitus, and profound deafness. It subsequently was found to be caused by a medium vessel vasculitis that can affect many parts of the body, but classically as Cogan described, causes deafness and inflammatory eye disease. Both of these appear to have occurred in Beethoven, recalling his red and painful eye that lasted for about a year. Similarly, Cogan Syndrome can associate with things like inflammatory bowel disease, which it’s been theorized Beethoven may have had given his chronic gastrointestinal symptoms, as well as the types of migratory arthralgias that Beethoven suffered from.
Beethoven’s autopsy could be consistent with a diagnosis of Cogan’s Syndrome. Wagner noted that Beethoven’s auditory arteries were “thickened and cartilaginous as though stretched over a raven’s quill.” In Cogan Syndrome, deafness develops from fibrotic hypertrophy of the auditory arteries that leads to sensorineural hearing loss. Unfortunately, we don’t have access to histology, but this could be consistent with Cogan syndrome. Other forms of focal vasculopathy could explain this finding, including syphilis. Because advanced syphilis can cause protean systemic symptoms and vasculopathy, one of those great mimickers, and can lead to inflammatory eye disease and sensorineural hearing loss, it would make sense that syphilis and Cogan syndrome would be on a differential diagnosis list together. David Cogan’s original 1945 description of this syndrome focused on ruling out syphilis in those cases. If one seeks parsimony and a unifying diagnosis, Cogan syndrome and syphilis appear to be the most likely possibilities.
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Credits & Citation
◾️Episode written by Avi Cooper
◾️Show notes written by Avi Cooper and Tony Breu
◾️Audio edited by Clair Morgan of nodderly.com
Cooper AZ, Abrams HR, Breu AC. Bringing up Beethoven. The Curious Clinicians Podcast. June 14, 2023.
Image credit: https://medium.com/@seattlesymphony/five-facts-you-probably-dont-know-about-beethoven-2d01b41b2d1b
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