Round window atresia can be seen in association with syndromal anomalies such as mandibulofacial dysostosis, Mondini type anomalies or cretinism, or with extensive otosclerosis. Non-syndromal round window atresia is extremely rare and until today there is no proof whether non-syndromal round window atresia can be inherited. We present two family members with non-syndromal round window atresia. Based on this case and on an extensive literature review, we discuss the possible ways of sound transmission with round window occlusion as well as the possibility of an autosomal dominant inheritance pattern of these two non-syndromal cases. Evaluating the hearing test results, the effect of round window atresia in non-syndromal cases cannot be thoroughly explained by current theories of sound transmission. In general, full occlusion of the round window should result in a complete air-bone-gap and a surgical procedure to open the window, e.g. a cochlear fenestration which creates a new pressure outlet for the inner ear fluids, should result in normalizing the hearing levels. Astonishingly, patients with non-syndromal round window atresia, show hearing tests similar to those in patients with otosclerosis. In contrast to otosclerosis, complete closure of the air-bone-gap by surgery in patients with round window atresia does not seem to be possible according to currently reported cases. Therefore, routine examination of the round window in stapes surgery should be part of every stapes surgery and a high resolution CT scan should be considered in particular prior to revision of unsuccessful stapes surgery. As a genetic inheritance pattern can be assumed, specific emphasis should also be laid on the patient's family history.

Automated progress monitoring becomes more and more common in the construction industry. Recent approaches often use new methods like Unmanned aerial vehicles (UAVs) for a capturing large construction sites. However, the used methods often lack accuracy due to occluded elements and/or reconstruction inaccuracies from using photogrammetric methods. This paper presents a novel approach for further improvement of element detection rates. 4D BIM semantic information is used, to generate precise "as-planned" geometric models. These models are used to render a building from all points of view during the monitoring phase. Based on this information, a more accurate and reliable estimation of all detected elements can be achieved.

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Abstract. The problem of georeferencing building information modelling (BIM) models is complex and in need of a comprehensive solution. We focus on the open BIM data format Industry Foundation Classes (IFC) and its georeferencing implementation. The requirements voiced by the domain experts during recent years have been collected and analysed. While IFC already covers some of the concepts, we propose an extension to the IFC schema to handle the inadequacy. Our proposal composes of two new entities: one supports geographic coordinate reference systems (CRSs) and the other enables a rigid transformation of BIM geometries. We showcase the possibilities with three examples, one for each of the required scenarios. The improvements assure much-needed semantically clear definitions of the georeferencing concept within the IFC data model. As such, the interpretation of IFC data content is unambiguous for stakeholders and software implementers.