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Language development builds upon a complex network of interacting subservient systems. It therefore follows that variations in, and subclinical disruptions of, these systems may have secondary effects on emergent language. In this paper, we consider the relationship between genetic variants, hearing, auditory processing and language development. We employ whole genome sequencing in a discovery family to target association and gene x environment interaction analyses in two large population cohorts; the Avon Longitudinal Study of Parents and Children (ALSPAC) and UK10K. These investigations indicate that USH2A variants are associated with altered low-frequency sound perception which, in turn, increases the risk of developmental language disorder. We further show that Ush2a heterozygote mice have low-level hearing impairments, persistent higher-order acoustic processing deficits and altered vocalizations. These findings provide new insights into the complexity of genetic mechanisms serving language development and disorders and the relationships between developmental auditory and neural systems., Peter Perrino et al. identify variants in USH2A linked to a dominant language disorder in a human family and find that these variants are also associated with low-frequency hearing at the population level. They investigate the function of USH2A variants in mice, showing that mutations in this gene are responsible for hearing loss.

Autism Spectrum Disorder (ASD) is a neurodevelopmental disorder characterized by a core set of atypical behaviors in social-communicative and repetitive-motor domains. Individual profiles are widely heterogeneous and include language skills ranging from nonverbal to hyperlexic. The causal mechanisms underlying ASD remain poorly understood but appear to include a complex combination of polygenic and environmental risk factors. SHANK3 (SH3 and multiple ankyrin repeat domains 3) is one of a subset of well-replicated ASD-risk genes (i.e., genes demonstrating ASD associations in multiple studies), with haploinsufficiency of SHANK3 following deletion or de novo mutation seen in about 1% of non-syndromic ASD. SHANK3 is a synaptic scaffolding protein enriched in the postsynaptic density of excitatory synapses. In order to more closely evaluate the contribution of SHANK3 to neurodevelopmental expression of ASD, a knockout mouse model with a mutation in the PDZ domain was developed. Initial research showed compulsive/repetitive behaviors and impaired social interactions in these mice, replicating two core ASD features. The current study was designed to further examine Shank3B heterozygous and homozygous knockout mice for behaviors that might map onto atypical language in ASD (e.g., auditory processing, and learning/memory). We report findings of repetitive and atypical aggressive social behaviors (replicating prior reports), novel evidence that Shank3B KO mice have atypical auditory processing (low-level enhancements that might have a direct relationship with heightened pitch discrimination seen in ASD), as well as robust learning impairments.

We present the design, fabrication, and testing of a microscale positioning system. The system was designed in the SUMMiT V process to produce in-plane, bidirectional, micrometer-scale linear motion of a shuttle using a ratcheting mechanism and multilayered chevron actuators. A single latching system with oppositely faced ratchet teeth on either side of the shuttle is used for achieving the bidirectional actuation. The device has a smaller footprint and fewer electrical connections compared to similar devices, without compromising performance. Moreover, this device is capable of generating more force compared to previous devices. A LabVIEW-based optical characterization setup was developed for automated testing of the device. The device produced a maximum displacement of ~180 μm with a step size of 9 μm.

We present the design, fabrication, and testing of a micro-scale positioning system. The SUMMiT V™ processed design allows an in-plane, bi-directional, micron-scale linear motion of a shuttle using a ratcheting mechanism and multilayered chevron actuators. A single latching system with oppositely faced ratchet teeth on either side of the shuttle is used for achieving the actuation. The design is intended to reduce the footprint and number of electrical connections needed, compared to similar devices. A LabVIEW based optical characterization setup was developed for automated testing of the device. The device produced a maximum displacement of ~180μm.

The objectives of study of the Benoud Trough northwest Algeria were two fold. The first objective was to decipher the geologic history and basin architecture of the Benoud Trough. With that accomplished, the assessment of the hydrocarbon potential of the Benoud was to be conducted. Both of these objectives were addressed and are discussed in this communication.