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Objectives Gradually progressive sensorineural hearing loss (SNHL) is a prevalent sensory defect. It is generally untreatable, making rehabilitation by hearing aid or cochlear implantation the only option. However, SNHL as one of the symptoms of the hereditary autoinflammatory systemic disease cryopyrin-associated periodic syndrome, or as the only symptom of the cochlea-specific form (DFNA34), was suggested to respond to IL-1 antagonist (anakinra) therapy, which ameliorates NLRP3 variants-induced over-secretion of IL-1β. We analysed genotypic and phenotypic spectrum of cryopyrin-associated periodic syndrome or DFNA34, specifically focusing on the responsiveness of SNHL to anakinra. Methods Seventeen families diagnosed with either cryopyrin-associated periodic syndrome or DFNA34 were recruited. Genotyping and phenotyping including audiogram, MRI findings, and in vitro IL-1β assay were performed. Results Our cohort had an etiologic homogeneity of 94.1% to NLRP3 variants and a high de novo occurrence (84.6%). We identified the second DNFA34 pedigree worldwide with a novel NLRP3 variant supported by in vitro analysis. Significant improvement of hearing status against the natural course, showing response to anakinra, was identified in three probands, one of whom used to have severe SNHL. Hearing threshold worse than 60 dB at the start of anakinra and cochlear enhancement on brain MRI seemed to be related with poor audiologic prognosis and responsiveness to anakinra therapy despite stabilized systemic symptoms and inflammatory markers. Conclusion We propose a constellation of biomarkers comprising NLRP3 genotypes, hearing status at diagnosis, and cochlear radiological findings as prognostic factors of hearing status after anakinra treatment and possibly as sensitive parameters for treatment dosage adjustment. [ABSTRACT FROM AUTHOR]

This paper explores ways in which Roland Barthes' discussion of the encyclopaedia provides us with resources for thinking about education and research practice today. What Barthes addresses in his essay 'The Plates of the Encyclopedia' is a particular encyclopaedia, the Encyclopédie produced by Denis Diderot and Jean le Rond d'Alembert, which was published in France between 1751 and 1772. This is commonly referred as the first of a form that we recognise as the encyclopaedia today. I begin with Barthes' analysis and critique of the Encyclopédie. Barthes, writing in 1964, engages with the Encyclopédie as an iconic product of its time, seeing it as conditioned by and, in effect, reinforcing a particular way of experiencing the world. Next, I consider ways in which a parallel critique is pertinent today. I explore some current examples of encyclopaedic form in relation to education and educational research. The purpose of this is to examine the interplay between particular cultural products and their society, in which not only certain types of knowledge but also a certain conception of knowledge are produced and reinforced. So, it will not be the purpose of this paper to provide direct analysis or critique of the Enlightenment, or to provide a historical account of knowledge. Rather, what I am interested in is problematising a particular understanding of language and knowledge that arises through these cultural products, particularly with regard to educational inquiry. This lays the way for thoughts expressed by Gilles Deleuze and Félix Guattari in What is Philosophy? which, as I shall try to show, are of help in furthering the analysis of the dominant research culture's use of such products and in imagining the task of education and research differently. [ABSTRACT FROM AUTHOR]

This paper explores the concept of Gelassenheit as developed in the later thought of Martin Heidegger. It seeks to show the relevance of this to aspects of education, especially to the ways that teaching can be enhanced in order to do better justice both to the learners and to what is studied. Thinking in this way helps to overcome the dominance of representational thought, a kind of thinking that imposes barriers on the understanding and restrictions on how the world can come to be. In the end the thinking illustrated by Gelassenheit constitutes a more responsible and more responsive relationship to the truth. My paper provides examples of ways that this can enhance education. In the end, with a caveat, I revisit and re‐examine aspects of Heidegger's work about which I think there is reason to be cautious. This is to encourage a degree of vigilance in relation to Heidegger's thought, but in a way that does not deny its powerful insights. [ABSTRACT FROM AUTHOR]

The importance of Levinas's philosophy for education has been widely discussed. In 'Levinas: Ethics or Mystification?' however, Alistair Miller questions whether Levinas's work is of value at all, raising doubts about whether his philosophy can, in any way, be helpful when it comes to practical ethical decision‐making, especially within education. Levinas's ethics, he argues, in the light of its lack of rational argument, amounts only to poetic imagery and mystical incantation. The purpose of this paper is to read Levinas in relation to education against this indictment by Miller. This paper proceeds by investigating the basis of Miller's arguments, in the light of exegesis of relevant aspects of Levinas's work. My purpose is not the positing of a new system or the formulation of systematic ideas of ethics but rather achieving an understanding of human experience, especially in contexts of teaching and learning, under a different, more accurate description. In doing so I attempt to show that Levinas's philosophy indeed provides a ground for ethics—that is, a means of understanding the practicality of ethics differently, beyond systematic approaches. In the last section, where education is discussed more directly, ethics will be seen as its very condition. [ABSTRACT FROM AUTHOR]