50 research outputs found

    Tire-road noise: an experimental study of tire and road design parameters

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    It is widely known that road traffic noise has negative influences on human health. Hence, as tire-road noise is considered to be the most dominant cause of road traffic noise above 30-50 km/h, a lot of research is performed by the two involving industries: road authorities/manufacturers and tire manufacturers. Usually, the parameters influencing exterior tire-road noise are often examined separately, whereas it is the tire-road interaction which obviously causes the actual noise. An integral approach, i.e. assessing possible measures to reduce tire-road noise from both the road and the tire point of view, is needed to further reduce traffic noise. In a project Silent Safe Traffic, this tire-road interaction is studied in more detail without focusing on either tire or road but looking at the tire-road system. In this publication we present experimental results of tire and road design parameters influencing tire-road noise from a fixed reference tire-road configuration. The influence of tire tread pattern, compound and construction as well as the influence of road roughness, acoustic absorption and driving speed on the exterior tire-road noise, measured by a CPX-set up, is reported. Keywords: Tire, Road, Measuremen

    Genotype–Phenotype Correlation in DFNB8/10 Families with TMPRSS3 Mutations

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    In the present study, genotype–phenotype correlations in eight Dutch DFNB8/10 families with compound heterozygous mutations in TMPRSS3 were addressed. We compared the phenotypes of the families by focusing on the mutation data. The compound heterozygous variants in the TMPRSS3 gene in the present families included one novel variant, p.Val199Met, and four previously described pathogenic variants, p.Ala306Thr, p.Thr70fs, p.Ala138Glu, and p.Cys107Xfs. In addition, the p.Ala426Thr variant, which had previously been reported as a possible polymorphism, was found in one family. All affected family members reported progressive bilateral hearing impairment, with variable onset ages and progression rates. In general, the hearing impairment affected the high frequencies first, and sooner or later, depending on the mutation, the low frequencies started to deteriorate, which eventually resulted in a flat audiogram configuration. The ski-slope audiogram configuration is suggestive for the involvement of TMPRSS3. Our data suggest that not only the protein truncating mutation p.T70fs has a severe effect but also the amino acid substitutions p.Ala306Thr and p.Val199Met. A combination of two of these three mutations causes prelingual profound hearing impairment. However, in combination with the p.Ala426Thr or p.Ala138Glu mutations, a milder phenotype with postlingual onset of the hearing impairment is seen. Therefore, the latter mutations are likely to be less detrimental for protein function. Further studies are needed to distinguish possible phenotypic differences between different TMPRSS3 mutations. Evaluation of performance of patients with a cochlear implant indicated that this is a good treatment option for patients with TMPRSS3 mutations as satisfactory speech reception was reached after implantation

    Diagnosis and treatment of urticaria and angioedema: a worldwide perspective

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    Urticaria and angioedema are common clinical conditions representing a major concern for physicians and patients alike. The World Allergy Organization (WAO), recognizing the importance of these diseases, has contributed to previous guidelines for the diagnosis and management of urticaria. The Scientific and Clinical Issues Council of WAO proposed the development of this global Position Paper to further enhance the clinical management of these disorders through the participation of renowned experts from all WAO regions of the world. Sections on definition and classification, prevalence, etiology and pathogenesis, diagnosis, treatment, and prognosis are based on the best scientific evidence presently available. Additional sections devoted to urticaria and angioedema in children and pregnant women, quality of life and patient-reported outcomes, and physical urticarias have been incorporated into this document. It is expected that this article will supplement recent international guidelines with the contribution of an expert panel designated by the WAO, increasing awareness of the importance of urticaria and angioedema in medical practice and will become a useful source of information for optimum patient management worldwide

    Hereditary Hearing Impairment. Clinical and genetic aspects of DFNA3, DFNB8/10, DFNX4, Muckle-Wells syndrome and otosclerosis

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    Contains fulltext : 105822.pdf (publisher's version ) (Open Access)Radboud Universiteit Nijmegen, 12 maart 2013Promotores : Kremer, J.M.J., Cremers, C.W.R.J. Co-promotor : Kunst, H.P.M

    Phenotype of a Belgian family with 6p25 deletion syndrome

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    Background: The 6p25 deletion syndrome is one of the many syndromes with both hearing impairment as well as vision impairment. However, the audiometric characteristics and radiological findings of patients with 6p25 deletions are only scarcely described in literature. This study focused on characterizing the audiometric and radiological features of a Belgian family with a chromosome 6p25 deletion. Objective: To evaluate the hearing impairment, audiometric testing and radiological examination of the temporal bones in 3 family members with a 3.4 Mb deletion in chromosome band 6p25. Results: All 3 family members demonstrated slowly progressive sensorineural or mixed hearing impairment. Radiologic examination revealed thickened and sclerotic stapes in all patients and a minor internal partition type II of the cochlea in 2 patients. Conclusion: There is a significant phenotypic variability within and among families with the 6p25 deletion syndrome. A thorough genotype-phenotype correlation is difficult because of the small number of affected patients and the limited clinical data available. More clinical data of families with 6p25 deletions need to be published in order to create a reliable and precise phenotypic characterization. However, our findings can facilitate counseling of hearing impairment caused by 6p25 deletions
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