A Multicenter Clinical Evaluation of Data Logging in Cochlear Implant Recipients Using Automated Scene Classification Technologies

Currently, there are no studies assessing everyday use of cochlear implant (CI) processors by recipients by means of objective tools. The Nucleus 6 sound processor features a data logging system capable of real-time recording of CI use in different acoustic environments and under various categories of loudness levels. In this study, we report data logged for the different scenes and different loudness levels of 1,366 CI patients, as recorded by SCAN. Monitoring device use in cochlear implant recipients of all ages provides important information about the listening conditions encountered in recipients' daily lives that may support counseling and assist in the further management of their device settings. The findings for this large cohort of active CI users confirm differences between age groups concerning device use and exposure to various noise environments, especially between the youngest and oldest age groups, while similar levels of loudness were observed

Discovery of Therapeutic Approaches for Polyglutamine Diseases: A Summary of Recent Efforts

Polyglutamine (PolyQ) diseases are a group of neurodegenerative disorders caused by the expansion of cytosine-adenine-guanine (CAG) trinucleotide repeats in the coding region of specific genes. This leads to the production of pathogenic proteins containing critically expanded tracts of glutamines. Although polyQ diseases are individually rare, the fact that these nine diseases are irreversibly progressive over 10 to 30 years, severely impairing and ultimately fatal, usually implicating the full-time patient support by a caregiver for long time periods, makes their economic and social impact quite significant. This has led several researchers worldwide to investigate the pathogenic mechanism(s) and therapeutic strategies for polyQ diseases. Although research in the field has grown notably in the last decades, we are still far from having an effective treatment to offer patients, and the decision of which compounds should be translated to the clinics may be very challenging. In this review, we provide a comprehensive and critical overview of the most recent drug discovery efforts in the field of polyQ diseases, including the most relevant findings emerging from two different types of approaches-hypothesis-based candidate molecule testing and hypothesis-free unbiased drug screenings. We hereby summarize and reflect on the preclinical studies as well as all the clinical trials performed to date, aiming to provide a useful framework for increasingly successful future drug discovery and development efforts.Project ON.2 SR&TD Integrated Program (NORTE-07-0124-FEDER-000021), co-funded by North Portugal Regional Operational Program (ON.2-O Novo Norte), under the National Strategic Reference Framework, through the European Regional Development Fund (ERDF) and also supported by Fundação para a Ciência e Tecnologia through the project POCI-01-0145-FEDER-016818 (PTDC/NEU-NMC/3648/2014)info:eu-repo/semantics/publishedVersio

EEG variations as estimators of listening effort during recognition of words in noise in unilateral and bilateral sequential adult cochlear implant users.

The listening effort, defined as "the mental exertion required to attend to, and understand, an auditory message" [McGarrigle et al 2014] is a matter receiving wide interest in the scientific community. In particular the challenge is its evaluation beyond the self-report data obtained through questionnaires or individuals' verbal reports. Multiple evidences highlight a role for posterior Alpha and frontal Theta EEG rhythms variation for the estimation of the listening effort, implying an inhibitory activity toward irrelevant/distracting stimuli for Alpha, while working memory and lexico-semantic processing for Theta [Strauß et al 2014; Wisniewski et al 2015]. The evaluation of the listening effort appears extremely worthy in hearing impaired patients, since the presence of noise or distortions in a speech signal increases cognitive demand and listening effort [Stenfelt & Rönnberg 2009]. In addition, such evaluation it would be interesting to be investigated comparing the unilateral CI with the bilateral CI condition, since the availability of more auditory information in the second one. The balance between Theta and Alpha EEG rhythms seems essential for the word recognition. Therefore aim of the present study was the assessment of the listening effort during a word in noise recognition task in CI adult users before and after the implantation of the second CI. Present results evidenced an increase in the frontal Theta and parietal Alpha for a quite difficult noise condition, and for Theta in correspondence of the stimulus. Moreover, frontal Theta activity increased along the task for the same difficult noise condition during and after the stimulus. These considerations appears useful for the neurophysiological characterization of CI users in order of further applications toward the management of cognitive resources and tailor-made rehabilitations