During the last century, hundreds of studies have been performed to identify factors that are involved in the aetiology of otosclerosis. These studies include a variety of aetiological factors and, although many theories have been postulated, the process of the development of the disease remains unclear. A historical overview and analysis of the literature dealing with the aetiology of otosclerosis is presented. The role of collagen disorders, genetic linkage studies, associations with the HLA system and measles virus infection as causal factors is discussed. From an epidemiological point of view, the disease has an autosomal dominant mode of inheritance. Therefore, since the introduction of new genetic research techniques over the last decades, more studies have been performed to find evidence of a genetic factor that initiates the development of otosclerosis. In this review, we tried to categorize the most prominent studies in sections according to their subjects within the diversity of aetiological factors that have been studied.
To standardize the reporting of hearing results after middle ear surgery, the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology-Head and Neck Surgery proposed 2 levels of guidelines: level 1 for reporting summary data and level 2 for reporting raw data. The Committee encourages the reporting of raw data from each individual case. However, in studies in which the examined population is too large, this can yield difficulties. With respect to this point, we designed a method for a simple visual presentation of hearing results in an attempt to provide data from each individually operated ear in a patient group. In this method the relation between the preoperative and postoperative bone-conduction levels is evaluated to assess overclosure and iatrogenic cochlear damage, and the relation between postoperative gain in air conduction and the preoperative air-bone gap is evaluated as a measure of technical success rate. This results in 2 plots, which we called the Amsterdam Hearing Evaluation Plots. Audiometric data from 451 stapes operations were used to demonstrate the use of the Amsterdam Hearing Evaluation Plots.
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