How exactly did Beethoven's ears become deaf?

Who is the hottest musician in the world? It is difficult to conclude on this issue.

But if you ask, who is the hottest musician in the medical world? The answer is Beethoven.

Or to be more precise, it is the ears of music masters.

This "Le Saint" who suffered from deafness prayed in the "Heiligenstadt Testament" that one day the cause of his deafness could be revealed to the world.

In this regard, the medical scientists answered very sincerely: nearly a hundred research documents, and the causal inferences that are always the same.

On the 250th anniversary of Beethoven’s birth, we collected relevant documents in order to restore an answer that is closest to the truth.

The sickly saint

In the "Biography of Beethoven" by Romain Roland, there is a sentence that basically everyone can recite:

"I want to hold the throat of fate, I will never admit defeat!"

But it is not so much that Beethoven is struggling with fate, it is better to say that he is fighting with him for a lifetime of illness. If this great artist had his own medical record, his past medical history would be densely written.

Before the age of 17, Beethoven's body was still healthy, but since a respiratory infection began, the illness has clung to him.

In 1792, the 22-year-old Beethoven had abdominal pain and diarrhea for the first time. At the same time, arthritis also began to attack. Beethoven, who suffered from abdominal pain for many years, contracted jaundice in his later years and was accompanied by vomiting blood. His liver problems are also getting worse.

A year before his death, he had begun to have yellowish face and oliguria and abdominal edema. Before he died, he had taken four times for the accumulation of water in his abdomen, each time he only got short relief.

According to modern medicine, abdominal fluid accumulation and acid-base imbalance cause breathing difficulties. The final cause of coma, liver and kidney failure should be the direct cause of Beethoven's death.

Timeline of Beethoven's medical history (source: made by myself)

Talking to his friends about his growing belly, the music master is occasionally optimistic and even self-deprecating, but when it comes to the gradually malfunctioning ears, his words are almost full of despair and pain.

In 1801, the 26-year-old Beethoven first talked about his ear disease in his correspondence. Since then, his progressive illness has repeatedly appeared in the letter. Of course, these complaints with his friends also inadvertently enriched his illness for future generations.

To sum up, Beethoven's initial symptoms are sudden inability to recognize high-frequency sounds in the left ear, followed by progressive tinnitus and hearing loss, but the progress is slow bilaterally. The 56-year-old is determined to be deaf in the left ear, and the 57-year-old is determined to be both ears. deaf.

The tortured Le Sheng wrote in his will before his death that he must undergo an autopsy after death, so that later generations of doctors can clarify the truth of deafness.

But the ridiculous thing is that before the anatomy doctor's report was issued, the rumors had begun to spread everywhere. Beethoven's deafness was caused by syphilis, even now this assertion still appears.

But as long as it is more truthful, this hypothesis is actually the least reliable of all the inferences.

As far as Ye Youshi is concerned, he really has no chance of being exposed to syphilis. Although Beethoven is a gifted man, he is not romantic. Because of his ugly appearance, Beethoven has not been married in his life. The talented composer has a conservative view of men and women:

When Beethoven learned that his friend was tainting his wife, he chose to resolutely break with him. Moreover, he once spit out Mozart in the letter because of "Don Juan"-he actually used this kind of promiscuous sex ghost as the protagonist of the opera.

"Don Juan" source: Wikipedia

From a clinical point of view, syphilis theory is also difficult to stand up. Although the medicine in the Renaissance era was very obscure, it is not difficult to diagnose a highly apparent disease such as syphilis. In Beethoven's information, we could not find any medical advice or prescriptions related to syphilis. In the autopsy report of the body after death, there is no description of syphilis.

The demise of the syphilis viewpoint is also reflected in the paper, and the literature on Beethoven and syphilis has almost disappeared in recent years.

However, the opinions put forward by medical scientists based on Beethoven's clinical symptoms and anatomical reports have become more and more.

Differential diagnosis

Up to now, there are many possible causes of Beethoven's deafness on PubMed, and there are many industry leaders, which can be called fairy fights.

A rough distinction can be divided into two gangs: conductive deafness and neurological deafness.

The cause of conductive hearing loss is otosclerosis, which is an unexplained bony disease of the ear capsule that involves overgrowth of bone in the stapes foot.

Reference 6

The pathological mechanism is the accumulation of abnormal new bone. With the development of overgrowth, the stapes can no longer have the function of a piston, but shake back and forth, and finally become completely fixed. The conductivity gradually deteriorates until it reaches the maximum conductive hearing loss of 60dB.

Progressive hearing loss in both ears or one ear is the main symptom, and it can also be accompanied by tinnitus and dizziness. These seem to overlap with Beethoven's symptoms. At the same time, the incidence of otosclerosis is the highest among Caucasians, and most of the patients are young and middle-aged, which is also in line with Beethoven's race and time of onset.

But this theory also has flaws. First of all, the actual basis for conductive deafness comes from a widely circulated physics story: Beethoven used bone conduction hearing aids for a long time after hearing problems with his teeth-biting one end of a wooden stick with his teeth. Put the other end on the piano to listen to the sound of your own playing.

In fact, this story has always lacked written evidence. Beethoven used many hearing aids, and he has been complaining about ineffectiveness.

The hearing aid used by Beethoven. Source: Wikipedia

Second, Beethoven's autopsy did not support this theory. During the dissection, the doctor carefully examined the temporal bone, including descriptions of the opening of the Eustachian tube, the middle ear space and the mastoid process. However, the anatomy report did not mention any abnormality of the oval window. Instead, it wrote the abnormality of the ear nerve: "The auditory nerve is atrophy, and there is no medulla."

Out of the interpretation of the clinical symptoms, the neurological deafness group made a lot more judgments. According to the composer’s strong abdominal symptoms, some doctors suggested that Beethoven may be a patient with IBD. His deafness and liver cirrhosis are immunoregulatory problems outside the intestine.

On this basis, in 2016, three Harvard Medical School professors published a document that Beethoven suffered from a rare autoimmune disease "Cogan syndrome", which is also known as interstitial keratitis-vertigo-neuropathic Deafness syndrome.

Reference 2

From the perspective of clinical symptoms alone, Cogan syndrome is almost the correct answer: respiratory infections and joint pain above the disease are prodromal symptoms, which are consistent with Beethoven's medical history in youth. Cogan syndrome can cause inflammation of the vascular system and affect multiple organs. The prominent manifestations are keratitis, auditory vestibular dysfunction (hearing loss, dizziness), manifestations of vascular involvement in other organs, arthritis, hepatomegaly, and possibly accompanying IBD.

These performances can be one-to-one correspondence with Beethoven.

However, this document is also more substantial. It clearly points out that Cogan syndrome lacks persuasiveness in deafness: autoimmune hearing loss is usually bilateral hearing loss, which is characterized by sudden, bilateral, fluctuating and progressive . Judging from the case report, the hearing of patients with Cogan syndrome usually disappears quickly within a few weeks or months, but Beethoven's deafness started from a single ear, and it has tortured him for 30 years.

In addition to these two major gangs, there are many other things in the medical arena: Paget's bone disease, typhus, lupus erythematosus, etc., have different opinions. Beethoven may not have thought that he would be able to blossom in both the music industry and the medical world.

However, with the release of a peculiar case report this year, the truth about Beethoven's deafness may be revealed to the world.

Beethoven Deaf

In June last year, Sotheby's auction house successfully auctioned a strand of Beethoven's hair for 300,000 yuan. A few years ago, Beethoven's hair was even carbonized and made into blue diamonds. Of course, this thing is more expensive.

Hair worth 300,000 (YouTube video screenshot)

Before achieving extremely high collection value, these Beethoven hairs first realized the medical mission.

In 2000, a document on the Lancet reported that researchers conducted metal detection on Beethoven's hair and found that the lead element was 100 times higher than the normal value. The article suggested that Beethoven may be suffering from lead poisoning.

References 7

In this regard, the doctor’s attitude is very cautious, because the doctors in the Beethoven era liked to use lead and mercury to treat patients. The lead-rich hair may also be caused by some drugs he took before his death, which is not enough to explain him. Lead poisoning.

But in 2005, doctors analyzed the skull of Beethoven's parietal lobe that was verified by DNA, and the results showed that the lead content was significantly higher. The lead found in bones is very important, which shows that Beethoven has been repeatedly exposed for many years and has been chronic lead poisoning.

Lead toxicity also successfully explained why the atrophied auditory nerves lacked medullary lines during Beethoven’s cadaveric dissolution: damage to the central nerves suggests axonal degeneration, which is common in lead poisoned people.

However, the effect of chronic lead poisoning on hearing has not been the main symptom, which is also a point that is often questioned. However, a case report in January this year provided a strong evidence for this.

The report stated that in 18 years, a woman in Italy experienced progressive hearing loss, accompanied by fatigue, upper limb paresthesia and abdominal pain. Her hearing assessment also showed impairments in perceiving sound frequency and discriminating ability. Researchers believe that the patient’s auditory history almost overlaps with Beethoven’s clinical course.

The patient’s hearing assessment form (Reference 4)

As for why doctors think of that music master? Because this lady is also a poor person poisoned by lead, the blood lead in the test result is much higher than normal.

After investigation, the culprit of the disease was also found: a pot with broken ceramics and exposed lead layers, the lady used it for breakfast every day. After treatment, the patient's abdominal pain and other symptoms have been fully relieved. Although the ear damage is irreversible, there is still hope for treatment.

In the literature, the author also sighs: This modern "Beethoven" no longer has to experience the pain and torture of more than two hundred years ago.

end

The question of why Beethoven was exposed to lead poison is difficult.

Combining the background of the times, later people speculate that it may be a contaminated diet: Beethoven especially loves a poor-quality Hungarian wine whose aroma and sweetness depend on the addition of lead. Or maybe it's just a problem with cups: adding lead to glass is the code of wealth more than two hundred years ago.

Although lead poisoning is currently the answer that is closest to the truth, it may not be long before more reasonable and detailed cases appear to overturn it. Since Beethoven's death two hundred years ago, the medical profession has not forgotten his last words, and must thoroughly investigate the cause of his deafness.

As for why there are always medical scientists so persistent and enthusiastic about studying Beethoven? An interview with a doctor involved in skull analysis may give the answer:

"In fact, Beethoven in medical research is more cruel and worthy of admiration than the biography written. It is still hard for me to imagine how he completed his peak work "Symphony Ninth" under the condition of total deafness. This is not just music. The miracle in the world is also a miracle in medicine.”