Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies

Background. Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. Methods. The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. Results. Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). Conclusions. Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies represent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity and stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent

Risk of Guillain-Barré syndrome after 2010–2011 influenza vaccination

Influenza vaccination has been implicated in Guillain Barré Syndrome (GBS) although the evidence for this link is controversial. A case–control study was conducted between October 2010 and May 2011 in seven Italian Regions to explore the relation between influenza vaccination and GBS. The study included 176 GBS incident cases aged ≥18 years from 86 neurological centers. Controls were selected among patients admitted for acute conditions to the Emergency Department of the same hospital as cases. Each control was matched to a case by sex, age, Region and admission date. Two different analyses were conducted: a matched case–control analysis and a self-controlled case series analysis (SCCS). Case–control analysis included 140 cases matched to 308 controls. The adjusted matched odds ratio (OR) for GBS occurrence within 6 weeks after influenza vaccination was 3.8 (95 % CI: 1.3, 10.5). A much stronger association with gastrointestinal infections (OR = 23.8; 95 % CI 7.3, 77.6) and influenza-like illness or upper respiratory tract infections (OR = 11.5; 95 % CI 5.6, 23.5) was highlighted. The SCCS analysis included all 176 GBS cases. Influenza vaccination was associated with GBS, with a relative risk of 2.1 (95 % CI 1.1, 3.9). According to these results the attributable risk in adults ranges from two to five GBS cases per 1,000,000 vaccinations

Conceptual design of the enhanced coolant purification systems for the European HCLL and HCPB test blanket modules

The Coolant Purification Systems (CPSs) is one of the most relevant ancillary systems of European Helium Cooled Lead Lithium (HCLL) and Helium Cooled Pebble Bed (HCPB) Test Blanket Modules (TBMs) which are currently in the preliminary design phase in view of their installation and operation in ITER. The CPS implements mainly two functions: the extraction and concentration of the tritium permeated from the TBM modules into the primary cooling circuit and the chemistry control of helium primary coolant. During the HCLL and HCPB-TBSs (Test Blanket Systems) Conceptual Design Review (CDR) in 2015 it was recognized the need of reducing the tritium permeation into the Port Cell #16 of ITER. To achieve this and, then, to lower the tritium partial pressure in the Helium Cooling Systems in normal operation, the helium flow-rate treated by each CPS has been increased of almost one order of magnitude. In 2017, to satisfy the CDR outcomes and the new design requirements requested by Fusion for Energy (F4E, the European Domestic Agency for ITER), ENEA performed a preliminary design of the “enhanced” CPSs. This paper presents the current design of the “enhanced” CPSs, focusing on design requirements, assumptions, selection of technologies and preliminary components sizing

Quality of Life and Personality Traits in Patients with Malignant Pleural Mesothelioma and Their First-Degree Caregivers.

Asbestos exposure causes significant pleural diseases, including malignant pleural mesothelioma (MPM). Taking into account the impact of MPM on emotional functioning and wellbeing, this study aimed to evaluate the quality of life and personality traits in patients with MPM and their first-degree caregivers through the World Health Organization Quality of Life–BREF (WHOQOL-BREF) and the Minnesota Multiphasic Personality Inventory-2 Restructured Form (MMPI-2-RF). The sample was composed of 27 MPM patients, 55 first-degree relatives enrolled in Casale Monferrato and Monfalcone (Italy), and 40 healthy controls (HC). Patients and relatives reported poorer physical health than the HC. Patients had a higher overall sense of physical debilitation and poorer health than relatives and the HC, more numerous complaints of memory problems and difficulties in concentrating, and a greater belief that goals cannot be reached or problems solved, while often claiming that they were more indecisive and inefficacious than the HC. First-degree relatives reported lower opinions of others, a greater belief that goals cannot be reached or problems solved, support for the notion that they are indecisive and inefficacious, and were more likely to suffer from fear that significantly inhibited normal activities than were HC. In multinomial regression analyses, partial models indicated that sex, physical comorbidities, and the True Response Inconsistency (TRIN-r), Malaise (MLS), and Behavior-Restricting Fears (BRF) dimensions of the MMPI-2-RF had significant effects on group differences. In conclusion, health care providers should assess the ongoing adjustment and emotional wellbeing of people with MPM and their relatives, and provide support to reduce emotional distress

Long term variations at Campi Flegrei (Italy) volcanic system highlighted by the monitoring of hydrothermal activity

Long time-series of chemical composition of fumaroles and of soil CO2 flux reveal that important variations in the activity of Solfatara fumarolic field, the most important hydrothermal site of Campi Flegrei, occurred in the 2000- 2008 period. A continuous increase of the CO2 concentration and a general decrease of the CH4 concentration are interpreted as the consequence of the increment of the relative amount of magmatic fluids, rich in CO2 and poor in CH4, hosted by the hydrothermal system. Contemporaneously the H2O-CO2-He-N2 gas system shows remarkable compositional variations in the samples collected after July 2000 with respect to the previous ones, indicating the progressive arrival at the surface of a magmatic component different from that involved in the 1983-84 bradyseism. The change starts in 2000 concurrently with the occurrence of relatively deep long periods seismic events which, in our interpretation, were the indicator of the opening of an easy pathway for the transfer of magmatic fluids towards the shallower, brittle domain hosting the hydrothermal system. Since 2000 this magmatic gas source is active and causes ground deformations, seismicity as well as the expansion of the area interested by diffuse soil degassing of deeply derived CO2

Carbon Dioxide degassing at Latera caldera (Italy): evidence of geothermal reservoir and evaluation of its potential energy

In order to test the potentiality of soil CO2 diffuse degassing measurements for the study of underground mass and heat transfer in geothermal systems detailed surveys were performed at Latera Caldera which is an excellent test site, due to the abundant available subsurface data. Over 2500 measurements of soil CO2 flux revealed that endogenous CO2 at Latera Caldera concentrates on a NE-SW band coinciding with a structural high of fractured Mesozoic limestones hosting a water-dominated high-enthalpy geothermal reservoir. The total hydrothermal CO2 degassing from the structural high has been evaluated at 350 t d-1 from an area of 3.1 km2. It has been estimated that such a CO2 release would imply a geothermal liquid flux of 263 kg s-1, with a heat release of 239 MW. The chemical and isotopic composition of the gas indicates a provenance from the geothermal reservoir and that CO2 is partly originated by thermal metamorphic decarbonation in the hottest deepest parts of the system and partly has a likely mantle origin. The ratios of CO2, H2, CH2 and CO to Ar, were used to estimate the T-P conditions of the reservoir. Results cluster at T ~ 200-300°C and PCO2 ~ 100-200 bars, close to the actual well measurements. Finally the approach proved to be an excellent tool to investigate the presence of an active geothermal reservoir at depth and that the H2-CO2-CH4-CO-Ar gas composition is a useful T-P geochemical indicator for such CO2 rich geothermal systems

Models and experimental results from the wide aperture Nb-Ti magnets for the LHC upgrade

MQXC is a Nb-Ti quadrupole designed to meet the accelerator quality requirements needed for the phase-1 LHC upgrade, now superseded by the high luminosity upgrade foreseen in 2021. The 2-m-long model magnet was tested at room temperature and 1.9 K. The technology developed for this magnet is relevant for other magnets currently under development for the high-luminosity upgrade, namely D1 (at KEK) and the large aperture twin quadrupole Q4 (at CEA). In this paper we present MQXC test results, some of the specialized heat extraction features, spot heaters, temperature sensor mounting and voltage tap development for the special open cable insulation. We look at some problem solving with noisy signals, give an overview of electrical testing, look at how we calculate the coil resistance during at quench and show that the heaters are not working We describe the quench signals and its timing, the development of the quench heaters and give an explanation of an Excel quench calculation and its comparison including the good agreement with the MQXC test results. We propose an improvement to the magnet circuit design to reduce voltage to ground values by factor 2. The program is then used to predict quench Hot-Spot and Voltages values for the D1 dipole and the Q4 quadrupole.Comment: 8 pages, Contribution to WAMSDO 2013: Workshop on Accelerator Magnet, Superconductor, Design and Optimization; 15 - 16 Jan 2013, CERN, Geneva, Switzerlan

Level of carbon dioxide diffuse degassing from the ground of Vesuvio: comparison between extensive surveys and inferences on the gas source

An extensive campaign of diffuse CO2 soil flux was carried out at the cone of Vesuvio in October 2006 with two main objectives: 1) to provide an estimation of CO2 diffusely discharged through the soils in the summit area and 2) to evidence those sectors of the volcano where structural and morphological conditions could favour the gas output. The survey consisted of 502 measurements of soil CO2 flux homogenously distributed over an area of about 1.8 km2. Results of this survey were compared with those obtained during a similar campaign carried out by Frondini et al. in 2000, from which we have taken and reinterpreted a subset of data belonging to the common investigated area. Graphical statistical analysis showed three overlapping populations in both surveys, evidencing the contribution of three different sources feeding the soil CO2 degassing process. The overall CO2 emission pattern of 2006 is coherent with that observed in 2000 and suggests that a value between 120 and 140 t/day of CO2 is representative of the total CO2 discharged by diffuse degassing from the summit area of Vesuvio. The preferential exhaling area lies in the inner crater, whose contribution resulted in 45.3% of the total CO2 emission in 2006 (with 62.8 t/day) and in 57.4% (with 70.3 t/day) in 2000, although its extension is only 13% of the investigated area. This highly emissive area correlated closely with the structural discontinuities of Vesuvio cone, mainly suggesting that the NW-SE trending tectonic line is actually an active fault leaking deep gas to the bottom of the crater. The drainage action of the fault could be enhanced by the “aspiration” effect of the volcanic conduit

Tomograms and other transforms. A unified view

A general framework is presented which unifies the treatment of wavelet-like, quasidistribution, and tomographic transforms. Explicit formulas relating the three types of transforms are obtained. The case of transforms associated to the symplectic and affine groups is treated in some detail. Special emphasis is given to the properties of the scale-time and scale-frequency tomograms. Tomograms are interpreted as a tool to sample the signal space by a family of curves or as the matrix element of a projector.Comment: 19 pages latex, submitted to J. Phys. A: Math and Ge