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Beethoven may not be the only composer who was deaf but he was a composer and he did go deaf later in his life.

Ludwig van Beethoven, the German composer and pianist, was born in 1770. Somewhere around 1796, Beethoven began to lose his hearing, suffering from severe tinnitus (ringing in the ears) which made it difficult for him to hear the music. By 1814, he was almost completely deaf. There has been much speculation as to what caused his deafness, but the true reason remains unknown.

Beethoven continued to compose music, despite the fact that his hearing had deteriorated to the point that he used "conversation books". He became sick in December 1826, becoming mostly bedridden until his death on 26 March, 1827.

ADDITION: Other fairly composers have gone deaf, including William Boyce (prior to Beethoven) and Bedřich Smetana. Both of these continued to work after their hearing loss, as Beethoven.

ADDITION: Gabriel Urbain Fauré also went deaf towards the end of his life and continued to compose. Also the lesser known German composer Felix Draeseke.

ADDITION: A living composer who actually began her music career with profound deafness (from age 12) is Dame Evelyn Glennie, who has composed/performed some of the most fascinating percussion music I've ever heard.

Ludwig Van Beethoven

ADDITION: Bedrich Smetana has already lost much of his hearing when he composed the famous piece, "Die Moldau"

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