Universal newborn hearing screening in the Lazio region, Italy

Background: The introduction of Universal Newborn Hearing Screening (UNHS) programs has drastically contributed to the early diagnosis of hearing loss in children, allowing prompt intervention with significant results on speech and language development in affected children. UNHS in the Lazio region has been initially deliberated in 2012; however, the program has been performed on a universal basis only from 2015. The aim of this retrospective study is to present and discuss the preliminary results of the UNHS program in the Lazio region for the year 2016, highlighting the strengths and weaknesses of the program. Methods: Data from screening facilities in the Lazio region for year 2016 were retrospectively analyzed. Data for Level I centers were supplied by the Lazio regional offices; data for Level II and III centers were provided by units that participated to the study. Results: During 2016, a total of 44,805 babies were born in the Lazio region. First stage screening was performed on 41,821 children in 37 different birth centers, with a coverage rate of 93.3%. Of these, 38.977 (93.2%) obtained a "pass" response; children with a "refer" result in at least one ear were 2844 (6.8%). Data from Level II facilities are incomplete due to missing reporting, one of the key issues in Lazio UNHS. Third stage evaluation was performed on 365 children in the three level III centers of the region, allowing identification of 70 children with unilateral (40%) or bilateral (60%) hearing loss, with a prevalence of 1.6/1000. Conclusions: The analysis of 2016 UNHS in the Lazio region allowed identification of several strengths and weaknesses of the initial phase of the program. The strengths include a correct spread and monitoring of UNHS among Level I facilities, with an adequate coverage rate, and the proper execution of audiological monitoring and diagnosis among Level III facilities. Weakness, instead, mainly consisted in lack of an efficient and automated central process for collecting, monitoring and reporting of data and information

EEG activity as an objective measure of cognitive load during effortful listening: A study on pediatric subjects with bilateral, asymmetric sensorineural hearing loss

Objectives: Deaf subjects with hearing aids or cochlear implants generally find it challenging to understand speech in noisy environments where a great deal of listening effort and cognitive load are invested. In prelingually deaf children, such difficulties may have detrimental consequences on the learning process and, later in life, on academic performance. Despite the importance of such a topic, currently, there is no validated test for the assessment of cognitive load during audiological tasks. Recently, alpha and theta EEG rhythm variations in the parietal and frontal areas, respectively, have been used as indicators of cognitive load in adult subjects. The aim of the present study was to investigate, by means of EEG, the cognitive load of pediatric subjects affected by asymmetric sensorineural hearing loss as they were engaged in a speech-in-noise identifi- cation task. Methods: Seven children (4F and 3M, age range ¼ 8e16 years) affected by asymmetric sensorineural hearing loss (i.e. profound degree on one side, mild-to-severe degree on the other side) and using a hearing aid only in their better ear, were included in the study. All of them underwent EEG recording during a speech-in-noise identification task: the experimental conditions were quiet, binaural noise, noise to the better hearing ear and noise to the poorer hearing ear. The subjects' Speech Recognition Thresholds (SRT) were also measured in each test condition. The primary outcome measures were: frontal EEG Power Spectral Density (PSD) in the theta band and parietal EEG PSD in the alpha band, as assessed before stimulus (word) onset. Results: No statistically significant differences were noted among frontal theta power levels in the four test conditions. However, parietal alpha power levels were significantly higher in the “binaural noise” and in the “noise to worse hearing ear” conditions than in the “quiet” and “noise to better hearing ear” conditions (p < 0.001). SRT scores were consistent with task difficulty, but did not correlate with alpha and theta power level variations. Conclusion: This is the first time that EEG has been applied to children with sensorineural hearing loss with the purpose of studying the cognitive load during effortful listening. Significantly higher parietal alpha power levels in two of three noisy conditions, compared to the quiet condition, are consistent with increased cognitive load. Specifically, considering the time window of the analysis (pre-stimulus), parietal alpha power levels may be a measure of cognitive functions such as sustained attention and selective inhibition. In this respect, the significantly lower parietal alpha power levels in the most challenging listening condition (i.e. noise to the better ear) may be attributed to loss of attention and to the subsequent fatigue and “withdrawal” from the task at hand

Neurophysiological characterization of normal hearing and unilateral hearing loss children: a comparison among EEG-based indices for information processing and decision-making levels.

The identification of measurable indices of cerebral functions to be applied in clinical settings is ever more felt as necessary for a more thorough and objective evaluation of patients cognitive performance. In the present paper, the electroencephalographic-based indices of mental workload (WL = frontal θ/parietal α) and of mental engagement (ME = β/(α+θ)), calculated along the brain midline, have been employed to characterize the eventual specific patterns of cerebral activations during a speech in noise perception task in normal hearing (NH) and unilateral hearing loss (UHL) children. Results showed no differences between the groups for the frontal bilateral noise condition (in which both signal and noise were emitted by two loudspeakers placed +45° and -45° in relation to the participant), while in lateralized noise conditions the UHL group showed higher parietal ME values for the Noise to the Deaf Ear condition). Finally, the NH group showed a different distribution of ME values among frontal, central and parietal electrodes, with higher ME values in the central and parietal ones in correspondence of the Noise to the Left Ear condition. The WL index analysis did not provide any significant differences. Results suggest the relevance of including the analysis of the beta rhythm in the neurophysiological assessment of the neural processing of speech in noise stimuli in normal hearing and hearing impaired participants

Secondary cytomegalovirus infections: How much do we still not know? Comparison of children with symptomatic congenital cytomegalovirus born to mothers with primary and secondary infection

Congenital cytomegalovirus (cCMV) infection can follow primary and secondary maternal infection. Growing evidence indicate that secondary maternal infections contribute to a much greater proportion of symptomatic cCMV than was previously thought. We performed a monocentric retrospective study of babies with cCMV evaluated from August 2004 to February 2021; we compared data of symptomatic children born to mothers with primary or secondary infection, both at birth and during follow up. Among the 145 babies with available data about maternal infection, 53 were classified as having symptomatic cCMV and were included in the study: 40 babies were born to mothers with primary infection and 13 babies were born to mothers with secondary infection. Analyzing data at birth, we found no statistical differences in the rate of clinical findings in the two groups, except for unilateral sensorineural hearing loss (SNHL) which was significantly more frequent in patients born to mother with secondary infection than in those born to mother with primary infection (46.2 vs. 17.5%, P = 0.037). During follow up, we found a higher rate of many sequelae (tetraparesis, epilepsy, motor and speech delay, and unilateral SNHL) in the group of children born to mothers with secondary infection, with a statistical difference for tetraparesis and unilateral SNHL. Otherwise, only children born to mothers with primary infection presented bilateral SNHL both at birth and follow up. Our data suggest that the risk of symptomatic cCMV and long-term sequelae is similar in children born to mother with primary and secondary CMV infection; it is important to pay appropriate attention to seropositive mothers in order to prevent reinfection and to detect and possibly treat infected babies

Multi-messenger observations of a binary neutron star merger

On 2017 August 17 a binary neutron star coalescence candidate (later designated GW170817) with merger time 12:41:04 UTC was observed through gravitational waves by the Advanced LIGO and Advanced Virgo detectors. The Fermi Gamma-ray Burst Monitor independently detected a gamma-ray burst (GRB 170817A) with a time delay of ~1.7 s with respect to the merger time. From the gravitational-wave signal, the source was initially localized to a sky region of 31 deg2 at a luminosity distance of 40+8-8 Mpc and with component masses consistent with neutron stars. The component masses were later measured to be in the range 0.86 to 2.26 Mo. An extensive observing campaign was launched across the electromagnetic spectrum leading to the discovery of a bright optical transient (SSS17a, now with the IAU identification of AT 2017gfo) in NGC 4993 (at ~40 Mpc) less than 11 hours after the merger by the One- Meter, Two Hemisphere (1M2H) team using the 1 m Swope Telescope. The optical transient was independently detected by multiple teams within an hour. Subsequent observations targeted the object and its environment. Early ultraviolet observations revealed a blue transient that faded within 48 hours. Optical and infrared observations showed a redward evolution over ~10 days. Following early non-detections, X-ray and radio emission were discovered at the transient’s position ~9 and ~16 days, respectively, after the merger. Both the X-ray and radio emission likely arise from a physical process that is distinct from the one that generates the UV/optical/near-infrared emission. No ultra-high-energy gamma-rays and no neutrino candidates consistent with the source were found in follow-up searches. These observations support the hypothesis that GW170817 was produced by the merger of two neutron stars in NGC4993 followed by a short gamma-ray burst (GRB 170817A) and a kilonova/macronova powered by the radioactive decay of r-process nuclei synthesized in the ejecta

Genetic prothrombotic factors in children with otogenic lateral sinus thrombosis: five case reports

Lateral sinus thrombosis (LST) is an uncommon, but life-threatening complication of both acute and chronic otitis media. There is some evidence that acquired or hereditary prothrombotic disorders are risk factors for LST. The aim of this work was to evaluate the role of thrombotic screening, anticoagulant therapy or prophylaxis in patients with either acute or chronic otitis media and LST. The medical records of five children hospitalized at Pediatric Hospital Bambino Gesu of Rome because of acute or chronic otitis media complicated by mastoiditis and LST were reviewed. All children underwent laboratory workup for hypercoagulability. All the five children were found to be heterozygote for the C677T MTHFR mutation and a child presented also heterozygosity for factor V Leiden mutation. They have been successfully treated with anticoagulant therapy without sequels. Children with acute or chronic otitis media may have a prothrombotic tendency that becomes clinically evident because of the inflammatory state. Patients with a family and/or personal history of thrombosis and/or thrombophilic conditions need anticoagulant prophylaxis also in the absence of clear signs of LST. Treatment with low molecular weight is successful in patients with LST. Blood Coagul Fibrinolysis 23:158-163 (C) 2012 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins

Neuroelectrical imaging study of music perception by children with unilateral and bilateral cochlear implants

Objective: To investigate by means of non-invasive neuroelectrical imaging the differences in the perceived pleasantness of music between children with cochlear implants (CI) and normal-hearing (NH) children. Methods: 5 NH children and 5 children who received a sequential bilateral CI were assessed by means of High-Resolution EEG with Source Reconstruction as they watched a musical cartoon. Implanted children were tested before and after the second implant. For each subject the scalp Power Spectral Density was calculated in order to investigate the EEG alpha asymmetry. Results: The scalp topographic distribution of the EEG power spectrum in the alpha band was different in children using one CI as compared to NH children (see figure). With two CIs the cortical activation pattern changed significantly, becoming more similar to the one observed in NH children. Conclusions: The findings support the hypothesis that bilateral CI users have a closer-to-normal perception of the pleasantness of music than unilaterally implanted children. © W. S. Maney & Son Ltd 2014

Neuroelectrical imaging investigation of cortical activity during listening to music in prelingually deaf children with cochlear implants

Objective: To date, no objective measure of the pleasantness of music perception by children with cochlear implants has been reported. The EEG alpha asymmetries of pre-frontal cortex activation are known to relate to emotional/affective engagement in a perceived stimulus. More specifically, according to the "withdrawal/approach" model, an unbalanced de-synchronization of the alpha activity in the left prefrontal cortex has been associated with a positive affective state/approach toward a stimulus, and an unbalanced de-synchronization of the same activity in the right prefrontal cortex with a negative affective state/withdrawal from a stimulus. In the present study, High-Resolution EEG with Source Reconstruction was used to compare the music-induced alpha asymmetries of the prefrontal cortex in a group of prelingually deaf implanted children and in a control group of normal-hearing children. Methods: Six normal-hearing and six age-matched deaf children using a unilateral cochlear implants underwent High-Resolution EEG recordings as they were listening to a musical cartoon. Musical stimuli were delivered in three versions: Normal, Distort (reverse audio flow) and Mute. The EEG alpha rhythm asymmetry was analyzed: Power Spectral Density was calculated for each Region of Interest, together with a right-left imbalance index. A map of cortical activation was then reconstructed on a realistic cortical model. Results: Asymmetries of EEG alpha rhythm in the prefrontal cortices were observed in both groups. In the normal-hearing children, the asymmetries were consistent with the withdrawal/approach model, whereas in cochlear implant users they were not. Moreover, in implanted children a different pattern of alpha asymmetries in extrafrontal cortical areas was noticed as compared to normal-hearing subjects. Conclusions: The peculiar pattern of alpha asymmetries in implanted children's prefrontal cortex in response to musical stimuli suggests an inability by these subjects to discriminate normal from dissonant music and to appreciate the pleasantness of normal music. High-Resolution EEG may prove to be a promising tool for objectively measuring prefrontal cortex alpha asymmetries in child cochlear implant users. (C) 2014 Elsevier Ireland Ltd. All rights reserved