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Hearing impairment is characterized by the sudden and unexplained hearing loss. It can be transient or permanent in nature and can occur in both children and adults. A loss of hearing has been observed in about one in a thousand of all children, whereas in adults, approximately 1.5 percent of the general population is affected (Moskowitz, S. A. et al. (1999) The Lancet 354(9181):982-988). For the development of the inner ear, several studies have shown that Hedgehog signaling is required (Shibamoto, K. et al. (1999) Genes Dev 13(7):945-956; Laplante, R. A. et al. (2001) Development 128(5):773-782; Laplante, R. A. et al. (2001) Development 128(5):763-773). In these studies, an alteration of the morphogenesis of the organ of Corti in mice has been described. In addition, there is some evidence that demonstrates that Hedgehog signaling is involved in the development of the inner ear in humans (Burns, W. C. et al. (2002) Am J Med Genet 108(4):517-526). Furthermore, various eea19f52d2

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