How about talking about Beethoven’s deafness again ?

Numerous studies have sought to understand and explain the deafness of this famous composer who ended up totally deaf about ten years before his death, managing to compose his most famous symphonies during this time. Some diagnoses are emerging, others are currently rejected. We will take up one by one the evoked diagnostic hypotheses by pointing out their weaknesses and their strong points in order to try to find the right diagnosis.

A brief review of his life seems essential to understanding.

Ludwig van Beethoven was born in Bonn in December 1770 and died in March 1827 at the age of 56. His grandfather Louis van Beethoven was a well-known musician who sang in the Bonn Court Chapel of the Elector of Cologne. The father of our composer, Johann van Beethoven, was a court musician, without any notable talent. He was violent and alcoholic, and died at the age of 52, worn out and in poverty. Very early on, he noticed the talent of his son (the eldest of a family of seven children of whom Ludwig was the only musical genius) and tried to take advantage of it. Of these seven children, only the three boys will survive. There were no musical descendants.

Ludwig van’s childhood was divided between an alcoholic, violent and undiplomatic father and a loving and discreet mother who died early (1787) when Beethoven was only 17 years old. His father died 5 years later, sinking into alcoholism and misery. From then on, Ludwig van was responsible for his two younger brothers. Not much has come to light about his youth. In particular, there is no mention of ear infections during his childhood. However, it is known for certain that he contracted typhoid at the age of 26 (1796), as the manuscript Fischhoff: Diaries testifies, and that he mentioned years later to his doctor the frequent occurrence of hearing pains.

In 1801, a few years after this date, Beethoven wrote to his friend, the doctor Franz Wegeler

For the last 3 years my hearing has been getting weaker. In the theater, I have to stand close to the orchestra, I can no longer hear the high-pitched sounds… I can hear the sounds, but cannot understand the words. In contrast, if someone shouts, I can’t stand it.

So Beethoven complains, almost at the same time as he contracts his infectious disease, of a notable hypoacusis and a painful hyperacusis to sounds that are too loud. He continues:

It is assumed that this is related to my digestive condition, which, as you know, was already impaired before I left Bonn and worsened in Vienna, where I suffered from diarrhea and, as a consequence, extraordinary weakness.

This decline in hearing evolved rapidly leading to a marked depressive state as he mentioned in his will of Heiligenstadt in October 1802 which, at times, prompted him to end his life. Only his desire to create held him back, even though he was only at the beginning of his life. By obligation, he restricts his social life, making him considered by his close relations as solitary and shady. But the master, as he himself will say, cannot reveal this terrible truth.

Will he fight against this blow of fate with different remedies and use the bottle more than he should? It is possible (which could explain the ascites that Dr. Andreas Ignaz Wawruch, cellist and unconditional admirer of his illustrious patient, will regularly puncture). The family history also remains a potential favourable factor. But the diagnosis of alcoholic ascites is far from being unequivocal.

Years go by. The deafness worsens inexorably. The digestive disorders made of abdominal pains, transit disorders like diarrhoea essentially, evolve by flare-ups sometimes relieved by lukewarm baths and various sometimes very crazy medications. Having become almost totally deaf, Beethoven stopped playing the organ and commissioned Conrad Graf, piano maker at the Imperial Court of Vienna, to build a quadruple-stringed instrument and to increase the sound amplitude by adding a resonator. He also used acoustic horns held by a headband in order to keep his hands free to play. He also used a wooden stick clamped between his teeth, the other end of which was pressed against the piano’s resonance box. In 1824, for the premiere of Symphony No. 9, he was completely deaf and at its first performance he remained with his back turned to the audience, discovering his triumph and the ovation it brought only after the singer Karoline Unger made him discover it by turning him towards her audience.

He died on March 26, 1827, carried away by a hepatic insufficiency with ascetic decompensation. He was 57 years old. The autopsy was performed the next day in the composer’s apartment by Dr. Wagner, Professor Wawruch, Beethoven’s personal physician, and Carl von Rokitanski. Let us quote the essential points.

“The body of the deceased presents significant emaciation and black and scattered petechiae especially on the extremities. The abdomen is distended and filled with fluid. The skin is distended, (…). The external auditory canal is filled with shiny skin scales up to the tympanic membrane which is masked (…). The mastoid process is large but without any particularities (…). The mastoid cells are lined with a blood-tinged mucosa. The whole petrous part of the temporal bone is traversed by a visible vascular network and also presents a large quantity of substance evoking blood, in particular in the region of the cochlea (inner ear), of which the spiral “membrane” (spiral blade now dividing the cochlea into 2 ramps: vestibular ramp at the top and tympanic ramp at the bottom, the whole being bathed in a liquid, the perilymph) appears a little reddened. (…) The acoustic nerves are wrinkled and devoid of myelin. The afferent auditory arteries are dilated (4 to 5 times the normal volume). (…) The left auditory nerve is by far the thinnest and presents three whitish and very tenuous roots; the right nerve presents a white and much larger root. The cerebral substance of the IVth ventricle region is much denser and more vascularized than the nerves emerging from it. The thorax and its contents are normal. The abdominal cavity contains four measures of a rust-colored fluid. The liver is reduced to half its normal size and looks like leather. It is hard and slightly bluish-green in color, and sometimes has nodules the size of beans (…). The gallbladder contains a dark colored fluid associated with a large amount of gravel-like sediment. The pancreas appears larger and firmer than normal (…). The stomach and intestines are very bloated. Both kidneys are pale red in color, and on sectioning there is a dark, cloudy fluid which interferes with the examination.”

The two rocks are taken for further study but Professor Rokitanski will find no trace of them when he takes up his post as pathologist in 1834.

All the elements are there that should allow us to advance in the diagnosis of this affection. One will notice the presence in many places of blood evoking bleeding due to vasculitis, which we will discuss later.

The main question that arises is the following: Should we associate the digestive disorders evoked throughout his life by the master with his rapidly evolving bilateral deafness? Let’s take the side of associating them as most of the doctors did.

Second question : Was the punctured ascites that of a decompensated alcoholic cirrhosis or not ? And if not, of what origin could it be. Most doctors attribute it to his “alcoholism” which he would have inherited from his father and grandfather. However, Beethoven’s ascites occurred 6 years before his death and required repeated punctures by his doctor, Dr. Wawruch, who applied a poultice soaked in lead after each one to limit the risk of infection at the puncture site. It is difficult to believe that a decompensated alcoholic cirrhosis lasted 6 years in our illustrious patient. We will come back to lead poultices.

Etiological diagnosis :

Let’s eliminate from the outset 2 very unlikely diagnoses evoked :

• Otosclerosis and particularly labyrinthine otosclerosis: this is a conductive deafness, of progressive evolution which results in an evolutionary attack of the deafness (blockage of the ossicular chain at the level of the stapes). The use of a vibrator (with the stick on the piano to hear in bone conduction) evoked a conductive deafness. The labyrinthisation corresponds to the attack of the inner ear in addition to the middle ear which gives a mixed deafness. However, the conductive deafness initially affects the bass and not the treble as mentioned by the teacher. Moreover, even if the damage can be severe, there is no total cophosis. Finally, the histology of the middle ear is normal even if one can argue that the blockage of the ossicular chain could have gone completely unnoticed especially in this period.

• Paget’s disease : it is a deforming bone pathology which can be of genetic origin (absent here), benign in general with a destruction and a typical bone reshaping. The shape of Beethoven’s face would have made the medical profession think of it. This disease can be complicated by a deafness due to the reshaping of the bones of the inner ear. The study of a part of the skull bones refuted this diagnosis.

As we have seen, these two conditions do not agree with the initial postulate, which took into account associated digestive disorders.

Let us now study the serious hypotheses:

• Typhoid : this is undeniable and Beethoven contracted this infectious disease in 1796. Due to the Salmonella typhi bacterium which is found in contaminated water and food, its clinical symptomatology is made of fever, intense fatigue, abdominal pains with diarrhea or constipation. It may be associated with an initial rash of pinkish spots that fade on pressure, and sometimes a red angina that is not very painful at first. Palpation almost always reveals a large spleen. Typhoid here would have taken on the symptoms of a chronic form lasting several years, which is not found in the literature, even if one can remain a healthy carrier for years. The hearing impairment in the acute phase can consist of acute otitis media with often tympanic perforations which retrograde to the healing. It is therefore a middle ear disease which, moreover, gives a transient conductive deafness of little importance. Inner ear damage is extremely rare, not to say exceptional. Moreover, it does not worsen with time but is the direct consequence of the disease (again, extremely rare). Cinchona, an excellent antipyretic, was prescribed to the famous patient. However, its ototoxic effects on the inner ear are known if the concentration reaches a certain threshold. I believe that we can therefore, without much risk of being wrong, eliminate this diagnosis.

• Lead intoxication aggravating a hepatic insufficiency of alcoholic origin : Beethoven was a wine lover, and because of this, many doctors have evoked alcoholism with decompensated ascites. What is known is that the master had ascites 6 years before his death. This is a very unusual length of life once alcoholic ascites is present. The punctured abdomen (Anatomopathology), brings back four measures of a rust colored fluid (the ascites fluid of an alcoholic liver failure is usually lemon yellow). Here, there is blood in the abdominal fluid. The spleen, liver and pancreas are enlarged, which is still consistent with alcoholic cirrhosis. Weight loss and petechiae on the abdomen are consistent with this. The kidneys are filled with blood. The liver is greatly reduced in size and looks like leather. Of hard consistency and bluish-green coloring, it presents on its surface nodules of the size of a bean. All this is totally compatible with terminal liver failure, the etiology of which has yet to be determined. Chronic lead intoxication is certain. An assay of lead in the hair found a high concentration of lead, which indicates recent intoxication. A subsequent determination on the skull bones (Paget’s test) also found large quantities of lead, indicating chronic intoxication dating back several years.

Where does it come from ?

Essentially from the poultice applied by his doctor after the ascites punctures carried out in order to avoid a superinfection at the puncture point, and consequently in the ascites liquid. It has also been mentioned that he was attracted to Hungarian wines, which were highly charged with lead and which he could have clarified with litharge (lead salts). In addition, the water in the thermal baths was highly charged with lead and was recommended by his doctors to treat his diarrhea and hearing problems (?). Lead intoxication causes symptoms of asthenia, abdominal pain, constipation (lead colic) more than diarrhea, frequent damage to the central nervous system with encephalopathy (affecting intelligence), damage to the peripheral nervous system and in particular damage to the peripheral nerves (lower limbs, upper limbs).

Ascites probably preceded the lead intoxication if one privileges the effect of the poultices and his hepatic insufficiency is not linked to lead. The deafness related to lead is more than debatable (this one having started well before) although lead on the inner ear can prove to be ototoxic. Again, this cause can probably be disproved.

What is left ?

Let us recall that the digestive disorders preceded the deafness (first the high-pitched ones) without vestibular damage (no vertigo) and that these two pathologies (digestive and auditory) have enamelled the whole life of the master. Let us specify again, if necessary, that it is not a question of knowing if the master had a decompensated hepatic insufficiency of alcoholic origin or not (much later than the first signs of deafness) increased by a lead intoxication, but rather what cause to attribute to his evolving inner ear deafness which will become total with the years. None of the previous causes can clearly explain it.

• Autoimmune disease : this is an abnormality of the immune system leading it to attack the normal components of the body. There are organ-specific autoimmune diseases that attack a single organ, such as thyroiditis, and systemic autoimmune diseases that can affect multiple organs. Their causes are still very poorly understood and several factors are certainly involved in their genesis:

• Genetic predisposition,
• Dysregulation of the organism by inhaled or ingested toxic substances,
• Infectious or allergic sequelae.

We have seen that Beethoven’s hearing problems were not associated with vestibular damage (no vertigo) and that this deafness followed his digestive problems. Any digestive disease catalogued as autoimmune can explain the symptomatology described (haemorrhagic rectocolitis, Crohn’s ileitis, primary biliary cholangitis (which can notably be complicated by cirrhosis).

These autoimmune diseases, and particularly ulcerative colitis, are characterized by digestive disorders that evolve in flare-ups (sometimes bloody diarrhea, abdominal pain, bloating). In 1979, Mc Cabe studied a series of 18 cases of patients with ulcerative colitis. Three of them had bilateral and symmetrical hypoacusis, which progressed more or less rapidly. The symmetry of the vestibular involvement in these patients explained the absence of vertigo which has led many authors to refute the diagnosis of autoimmune deafness. All the patients studied responded favorably to corticosteroid treatment instituted before the onset of irreversible sensorineural hearing loss.

An animal model of autoimmune sensorineural hearing loss was induced by vasculitis (present in UC) and circulating immune complexes were detected. This explains the atrophy of the auditory nerves with disappearance of the myelin sheath and certainly for a part (if not in totality) the hepatic insufficiency by vasculitis of the liver but also of the gall bladder and the mesenteric arteries.

Two centuries after his death, we can only deplore the premature death and the definitive loss of the sensory organ of one of the greatest composers of all times, who, today, could certainly have been cured of his condition. In spite of this extraordinary handicap, Beethoven managed, by his courage, his work and his will, to realize his greatest symphonies even though he was totally deaf.

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Bibliographical references :

1. Permanent deafness after typhoid fever (an observation in Dakar) – Faucher B., Ziegler O., Niang A., Bafall V., Mbaye PS. – Méd. Trop. 2009; 69: 73-74 ;
2. Contribution to the study of typhoid fever – Henri Leymarie – Thesis 1887 ;
3. Beethoven, victim of a medical treatment with lead, Catherine Petitnicolas – 30/08/2007 Article Le Figaro ;
4. Ludwig van Beethoven: an auto-immune deafness? Peter J. Davies – History of medical sciences. Volume XXIX n° 3-1995 ;
5. Beethoven’s deafness, new perspectives – 26/03/2020 – Patrice Imbaud. res musica ;
6. Ludwig van Beethoven : Carnets intimes – Manuscrit Fischhoff, Buchet / Chastel, 2005, 117p ;
7. Jean and Brigitte Massin – Ludwig van Beethoven, Fayard- 1967,845p ;
8. Beethoven, une vie plombée par l’alcool – Sandrine Cabut – Le Monde – July 2013.
9. A historical, scientific and legal analysis – NCBI /M. Guerriaux – 2020

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