Otological disease in Turner's syndrome

1988 was the 50th anniversary of Henry Turner's original publication of the constellation of physical features which were subsequently recognized to be associated with a deprivation of X chromosomal material. The abnormalities of phenotype and karyotype are now eponymously associated with his name. Very many phenotype stigmata have now been recognized to be associated with Turner's syndrome. However, a survey of females with the syndrome revealed that they consider that a major disability associated with the abnormal karyotype results from otological disease and that this is poorly recognized as a significant feature of the syndrome. The incidence of otological disease in patients with this syndrome has been difficult to evaluate because of ascertainment bias and the apparent diversity of otological disorders previously reported. This report reviews the literature and details a study of otological disease and hearing impairment in a sample of Turner's syndrome females enrolled from a defined area.