Ludwig van Beethoven

Abstract

Among the diseases of Beethoven, deafness was of particular importance. Numerous theories were established for the explanation of this illness. The possibility of chronic otitis interna developing as a result of otitis externa may be excluded on the basis of postmortem findings. Otitis externa was due to continuous local therapeutic attempts. The often mentioned congenital or acquired syphilitic aetiology of his deafness is improbable because of the lack of other symptoms of the disease. Until today otosclerosis has got the most supporters, though, according to the report of Naiken, the hypothesis of Paget's disease (osteitis deformans) seems to be the most probable. It is very difficult and in many cases even impossible to differentiate otosclerotic deafness from deafness caused by Paget's disease. Both may lead to conduction as well as perceptive deafness. Beethoven observed the first symptoms of hearing loss in 1796, when he still tried to conceal his disease. In 1802 his condition deteriorated and for this reason he gave up conducting. He became completely deaf in 1818 and from this time he could communicate with his visitors only by means of the famous conversation pads. At the beginning his progressive deafness was probably of the conduction type, since the use of wooden ear trumpets, one end of which he put into contact with the piano, improved temporarily his hearing. The diagnosis of Paget's disease starting in adulthood is based today on X-ray examination. The disease is of dominant inheritance. However, we do not know about the recurrence of the disease in Beethoven's family. As a consequence, either a new mutation may be supposed or the disease showing different clinical symptoms and developing at different periods in adulthood (especially deafness) could not be recognized in other members of the family. His mother died at the age of 41, his father at 53. The possibility of hyperostosis corticalis generalisata of recessive inheritance showing symptoms similar to Paget's disease, may be raised also. This condition is more consistent with the family tree (the parents are healthy, since they are only carriers of the mutant gene).