INTRODUCTION

The funerals of Ludwig van Beethoven took place at the end of March 29 1827 in the church of street Alserstrasse in Vienna, Austria.

Vienna offered him all honors he was denied in life. The schools were closed and on that day the Viennese were sure they had lost something truly great; however the large and emotional participation did not compensate before the eyes of those who loved him deeply the long indifference to the musician in the last years of his sad existence.

A few days after, his devoted companion, Nikolaus Zmeskal, wrote to Therese von Brunsvik, who was more than a friend to the conductor: "His death roused an emotion of which one has no memory. From twenty to thirty thousand people followed the funeral. The most honorable composers, amongst whom Franz Schubert (who died aged 31 years 1 year after Beethoven and buried beside him), were beside his coffins.

Beethoven received the extreme unction in the morning of March 24, his brother Johann's wife and a young musician of Graz, called Anselm Huettenbrenner, were with him and he was deemed to be dead on the 26th at 5h45 p.m. The young sculptor Danhauser molded his mortuary mask in the next morning.

In spite of his artistic success, this is how the life of the conductor finished as a sad and lonely life, tormented in the last 30 years by a poor health and above all by a progressive and distressing deafness early begun with tinnitus and persecutory auditory intrusions, which gradually and inevitably excluded him from life in society.

At the age of 31 years, he wrote to a friend and doctor Franz Gerhard Wegeler, on June 21 1801:

"Have you heard of my situation? In the last 3 years, my ears have been weaker and weaker; Frank, the director of the Hospital of Vienna tried to restrengthen my organism with tonics and my ears with Mandorle oil. There was no effect, the deafness became even worse. Then a stupid doctor suggested cold baths, which led me to strong pains. Another doctor advised me to take quick baths in the Danube, however, the deafness persists, and the ears remain snarling and cracking day and night. I confess I have been living a very miserable life. For almost 2 years I got away from all social activities, mainly because it is impossible for me to say to the people: I am deaf!... If I had another profession, I would maybe adapt to my disease, but in my case deafness represents a terrible obstacle. What if my enemies learn about it? What will they say? To have you get the picture of this strange deafness, in the theater I need to be very close to the orchestra in order to understand the words of the actors and at a given distance I cannot hear the acute sounds from the instruments and the chant. Surprisingly, in talk with people, many do not realize my deafness, they think I am absent-minded. Many times I can hear the sound of the voice but I don't understand the words, and if someone screams I can't stand it! Doctor Vering told me my ear will certainly improve, and if this is not possible there are moments I think I am the poorest creature of God.

There is no data enough to establish exactly the beginning of his deafness. According to some Beethoven's manuscripts, it seems the symptoms manifested when he was 26 years old (in 1796), the year in which he made his first tour in Berlin, Dresda, Prague, Lipsia, Nuremberg and Budapest. This imprecision is due to the fact he probably did not realize or did not consider it to be worth thinking about because it was not sudden and as he was young and conscious of his large artistic and musical capacity, he did not believe he would become deaf.

In spite of being rational in his writings about the inefficacy of medical treatments, he always relied on new treatments.

In November 16 1801, the composer wrote again to his friend Wegeler: "do you want to know how I am? And what I need? Doctor Vering* puts tourniquets in my arms. This treatment is very unpleasing; besides the pain, I get deprived from using my arm for 2 or 3 days. I must confess the noise in my ears get lower, specially in the left ear where the disease began, but hearing remains the same. I don't like changing doctors, but I believe Vering is a little empirical. What do you think of doctor Schmidt**? He seems to be another man.

I hear of wonders from him. What do you think?

A doctor told me he saw a deaf-mute boy in Berlin to begin to hear and a deaf man to be totally cured seven years ago. But even Dr. Schmidt did not do more than advise him to live in the country side to be protected from the city's nervousness. Beethoven took his advices and at the end of April 1802, he moved to Heiligenstadt, a little and peaceful town around the woods of Vienna. This made him concentrate for six months and achieve the fullness of his musical thoughts. And it was where they found years after his death a manuscript inside a writing desk his will in which among many things, he said: For my friends and for those who thought I was antisocial, absent-minded and hermit, they judged me bad. You don't know the secret reason of it all. I was tormented by a hopeless evil, which worsened due to insensate doctors. For years I was deceived with hopes of improvement and I ended up constrained to accept the reality of an incurable disease. Born with an excited and active mood and sensitive to the society's attractions, I needed to isolate very early and live my life in loneliness. If sometimes I tried to forget, my ear would bring me back to reality. I couldn't even ask the people: Please, speak loud, shout, because I'm deaf! How could I admit a disease that affected the sense that for me more than for anybody should be perfect? I had to live alone and if I am with someone I become very anxious afraid of running the risk of having my condition realized. I endured this humiliation when a student that was beside me heard the sound of a flute and I didn't, he heard the chant of a preacher and I heard nothing. I almost put an end to my life sometimes. Music was what entertained me. It seemed to me impossible to abandon this world before creating all the operas I felt an imperious need to compose. This was my life, a distressing life. When you read these lines you will know that those who spoke of me committed a great injustice. Ask Dr. Schmidt to describe my disease so that the world may reconcile with me, at least after my death (1).

This document witnesses perfectly the psychological drama of the great composer and fully justifies his nature involution. However, the deafness did not interfere whatsoever with the creative vein that ended up to express in a sublime manner all his inside world, all feelings, all passions, emotions and each perception of his soul and nature.

The conductor's introspective nature was part of his disease's reflex, but it is evident that his formation was also influenced by his childhood and adolescence (2).

Beethoven was born on December 16 1770, in Bonn, his father was a mediocre tenor with alcohol addiction, his mother, Maria Madalena Keverich, was 19 years old when he was born, she was the daughter of a cook and already widow of a court's chamberlain. His childhood was rigid and sad. As he early manifested his talent for music, he was not even 8 years old, and his father, unable to preview the genius in formation and trying to obtain personal profits, presented him as a six-year-old prodigy in the Music Academy of Cologne. At the age of 11 he was part of the orchestra of Bonn and of 13 he was organist. It is no doubt his father bothered the beginning of his career, by obliging him to make money. Aged 22 years he left Bonn and moved to Vienna, the music capital. It was in Vienna that he quickly achieved notoriousness and success as a concertist and composer. In 1814 there came Beethoven's musical life apogee, when in the Congress of Vienna, during the Europe's restructuring, after Napoleon, he was claimed as the greatest living musician. The Austrian Emperor Francis Joseph I (brother of the Brazilian Princess Leopoldina) placed at his disposal two salons in his palace in Vienna and granted him Viennese honorary citizenship. But it was also then that the aggravation of his deafness made him abandon the career of concertist.


TREATMENT OF DEAFNESS

In 1814 Beethoven found Dr. Weissenbach, who became interested in his case. The doctor was composer and poet and was also affected by hearing loss. The treatments imposed to him were the silliest and the most bizarre and curious treatments, obviously empirical and helpless. Sweating, tourniquets, washings, smoke steaming, diuretics, field seasons, instillation of several chemicals into the auditory meatus, diets, thermal cold and hot baths, continuous current electric stimulations. The acoustic horns built up by the court's mechanic Joahann Maelzel were not either much helpful. He used his acoustic apparatus in the left ear, because the right ear was totally deaf. He always said the sound did not enter through the channel but throughout the cranium. He also used a wooden drumstick between the teeth and supported it on the piano's resonance box to feel the vibrations.

He used pieces of cotton in the ears because by modifying the resonance of the tubular system of the external auditory meatus these assured favorable sensations, and filtered filtering some frequencies and relieved part of the tinnitus.

Between 1821 and 1826, his health worsened with other manifestations such as diarrhea, epistaxis and other evident signals of hepatic manifestations. He became an undisciplined patient, by drinking too much wine and strong coffee. With pneumonia, his general state got worse with ascites with several punctures to relieve and from then on he never recovered.

Historians, biographers, doctors, scholars always attempted to come to definitive conclusions of the causes that led to Beethoven's deafness and the etiopathological mechanisms of his evolutionary nature in order to verify the possibilities of the disease's interference with his character, his life and his musical production.


DISCUSSION

Many were the pathogenic hypothesis and interpretations proposed, but the true nature of the disease and the pathological conditions that determined the deafness are still unknown. The most important reference we have to justify his deafness followed by tinnitus is the autopsy carried out on the day after his death by Prof. Johann Wagner and his disciple Karl Rokitansi (who became one of the greatest authors of his time). The part of the ears was (briefly) described as follows:

-"The pavilion cartilage is large and irregular. The external acoustic meatus close to the tympanum shows epithelial desquamations. The Eustachian tube has a thick mucosa and a narrow osseous part. The mastoid cells and the temporal bone petrous part, mainly close to the cochlea, is hyperemic. The acoustic nerves are atrophic and demyelinated. The auditory arteries are dilated and sclerotic".

The presence of sclerosis in the auditory vessels may lead to the hypothesis of vascular insufficiency of the inner ear. We could know with precision if it was possible to find the temporal bones conserved in formaline, which Wagner kept for further studies. In 1863, Beethoven's sepulture was open, but Adam Politzer only found some fragments of his cranium. The middle ear autopsy data could only supply information that excludes chronic inflammatory pathology.

Most scholars as from BARATOUX AND NATTIER (1905) maintain the thesis of progressive otospongiosis of the labyrinthic capsule and ankylosis of the stirrup. Which is equivalent to a classical otosclerosis. In a large study published in 1921, called Beethoven's deafness, Guglielmo Bilancioni states: "The middle ear could have a lesion, although obscure in its pathogenesis, the auricular sclerosis", otosclerosis, which determines a reduction of the progressive hearing, without evaluable cause and relevant symptoms, except for a very high degree of deafness and tinnitus, and is eminently familiar and hereditary.

In another edition, SCHARTER recalls a particular form of otosclerosis called cochlear and described in 1912 by MANASSE and later by CARHART, in 1963.

In 1970, STEVENS AND HEMENWAY published that otosclerosis explains suitably the progressive loss and squeaking type tinnitus commented by Beethoven, however, to the extent of a differential diagnosis, we must take into account the hypothesis that the inner ear could have been affected by a toxic, infectious or luetic neuritis

In 2004 SALA (1984), MOTTA AND GRISSANTI published in favor of a mixed type deafness due to its early beginning, of bilateral, slightly symmetric deafness of different evolution at each side, but some anamnetic descriptions by Beethoven himself in letter of 1801 and 1802 could put in doubt the hypothesis of otosclerosis. There is no description of deafness in Beethoven's family. He did not present with symptoms of Willis' paracusis nor Weber's phenomenon symptoms. The conductor himself mentions that in the beginning the loss was in the acute frequencies, which is less common in otosclerosis. In spite the tinnitus has a common symptom in otosclerosis, the patients with this disease do not mention distress with high intensity sounds, as he does, and it is a classical phenomenon of recruitment. Other authors even mention the possibility of a auto-immune disease, obviously very difficult to confirm at that time.

Nowadays, Beethoven would probably have a better sound quality, and consequently a better life quality, mainly for the possibilities created by acoustic engineering and more recently electric (implants) and computing stimulation. Even though, many cases of neurosensorial deafness cases present recruitment and alterations in the auditory discrimination that makes it difficult and sometimes impossible to use hearing aid. As for the affection on the stirrup caused by an otosclerosis, there is the possibility of a surgical procedure, but a few things evolved in the medical treatment of otospongiosis of the otic capsule, the precise determination of the inner ear diseases etiology today regarding the sophisticated imaging, laboratorial and audiology exams we have present at hand. Many times we specialists cannot determine the cause of a neurosensorial deafness or tinnitus and we keep on using empirical treatments, certainly less aggressive than those used in Beethoven, but even though still empirical treatments! Would we actually be able to have done anything for the improvement of his hearing?

Many things still has to be studied and developed in the knowledge of our area so that we may help our patients more and more, and it will remain a constant challenge for the young people who begin in the specialty. This segment of otorhinolaryngology will be one of those which will mostly develop in the coming years.

In this case we should think, by extrapolating into our days, of what we could have done to Beethoven as our patient.

At the time in which he lived the auditory deprivation was more important than sensorial deprivation. In her book "The Story of my Life", published in 1900, HELLEN KELLER writes "The problems of deafness are deeper and more complex, more important than those of blindness. Deafness is a greater misfortune. It represents the loss of the most vital stimulus, the sound of the voice, that carries language, moves thinking and keeps us in the intellectual companionship of mankind." Hellen Keller was the unfortunate to have an USHER'S syndrome (progressive deafness and blindness) and became deprived of such senses along her life. She, more than anyone else, could witness and compare the fact of losing these sensorial stimuli. It was only after the discovery of graphic arts and printing, and then, along the centuries, the cheapening of books and publications and the possibility of almost people having access to writing and imaging. After the creation of photography, television, computer and all visual stimuli and specially the discovery and universalization of electric light (the weak light that existed at night made the vision difficult at night), the vision began to play an important role in civilization. At the time of Beethoven, the auditory stimulus was essential, without it the psychosomatic alterations and depressions were much higher in the deaf individual, mainly a composer!

In the other hand, the terrible deprivation for a musician no longer hearing the sounds of life and the world around him did not impair him of translating into melodic images and musical figures both the delicate sensations and the powerful interpretations found in his work. And his concentration was more facial than in a normal hearing person.

Thinking this way, we may paradoxally deduce that his born ingeniousness for music may have been exacerbated by deafness, which favored a purification of the melody found in his symphonies that was not conditioned by the fashion, the rigid system and the mannerism of his time. Only symphony number 1 is previous to 1796, which was the time of evolution of his deafness, therefore, almost all his musical production was conceived during his important period of deafness.

His auditory loss made him abandon his career of concertist and musical director, but did not influence his work, and his greatest creation was the ninth symphony, created between 1822 and 1824, at his fully deaf phase.

In short, indirectly and almost paradoxally, one may state that isolation and getting around from confrontations with the external world has somehow favored his musical talent and ingeniousness. While the artist lived internally his ideal world made of sound images that led him to a pure thought level we may infer, by listening to his works.

As a conclusion, all leads us to believe that thanks to this inevitable loneliness, Beethoven gradually achieved a
musical language full of emotion, abstracted from his isolation that he would probably never have achieved under normal physical conditions. His deafness ended up as one of the main contributors of his genial work. Would Beethoven have been the greatest composer of all ages without it?


BIBLIOGRAPHICAL REFERENCES

1.* Verning foi diretor do Instituto de Saúde de Viena de 1797 a 1809, médico conselheiro do Imperador José II.
** Foi Professor de Anatomia, conselheiro real, médico renomado particularmente em oftalmologia. Publicou vários artigos médicos. Beethoven confiou muito nele, seguiu seus conselhos de 1801 até sua morte. No seu testamento, Beethoven convidou a Schmidt a descrever sua doença. Beethoven dedicou a ele o Trio para piano, violino e violoncelo em b-maior op. 38.

2. Ascherson, N. Beethoven's deafness and saga of the stapes. Trans Hunterian Soc. 1965-1966; 24:7-34.

3. Baratoux G, Natier M. A propos de la surditè de Beethoven. Cron. Med. 1905, 12:492-496.

4. Keller H. O Romance da Vida 1900.

5. Liston SL. Beethoven's Deafness. Laryngoscope. 1989, 99:1301-1304.

6. McCabe,BF. Beethoven Deafness. Ann. Otol. Rhinol. Laringol. 1958, 67:192-206.

7. Mallardi V. Beethoven, la sorditá di un genio tra vita e mito - Acta Oto Rhinolayngol Ital.1999, 19:166-177.

8. Felisati D, Sperati G. Pazienti Celebri- Malati ORL nella storia e nell'arte. Societá Italiana de Otorrinolaringologia e Chirurgia Cervico-Facciale. 2008, 127-147.

9. Stevens HM, Hemenway WG. Beethoven's Deafness JAMA, 1970, 213: 434-437.









1. Head Professor of the Otorhinolaryngology of the Medicine College of USP. President of the Brazilian Association of Otorhinolaryngology and Cervico-Facial Surgery.

Institution: Faculdade de Medicina da Universidade de São Paulo. São Paulo / SP - Brazil. Mail Address: Ricardo Ferreira Bento - Rua Major Paladino, 464 - São Paulo - SP - Brazil - Zip code: 05307-000 - E-mail: rbento@gmail.com

Article received on August 27 2008. Accepted on August 10 2009.