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Publication : Pigment anomaly-associated inner ear deafness.

First Author  Schrott A Year  1987
Journal  Acta Otolaryngol Volume  103
Issue  5-6 Pages  451-7
PubMed ID  3618172 Mgi Jnum  J:8813
Mgi Id  MGI:57278 Citation  Schrott A, et al. (1987) Pigment anomaly-associated inner ear deafness. Acta Otolaryngol 103(5-6):451-7
abstractText  In order to investigate the pathogenesis of pigment anomaly-associated hereditary deafness, we studied black-eyed white mutant mice, which become severely deaf in early life and lacked neural crest-derived melanocytes. In the inner ear, the primary alteration appears to be located in the stria, which remains much thinner than normal and lacks intermediate cells. Melanocytes are identified with the histochemical Dopa reaction. This reaction is positive in intermediate stria cells in many animals of different ages, proving that they are derived from melanocytes. No tyrosinase-positive reactions were found in the mutant mice. This clearly indicates that the lack of intermediate stria cells is the crucial factor in the pathogenesis of pigment anomaly-associated inner ear deafness.
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