UNASSIGNED: This study aims to assess the potential efficacy of cochlear implantation as a treatment for patients with Waardenburg syndrome (WS) and to guide clinical work by comparing the effect of auditory and speech recovery after cochlear implantation in patients with WS and non-WS.
UNASSIGNED: PubMed, the Cochrane Library, CNKI, and Wanfang Data were sources for retrieving literature on cochlear implantation in WS, and clinical data meeting the inclusion criteria were meta-analyzed using RevMan5.41.
UNASSIGNED: A total of nine articles were included in this study, including 132 patients with WS and 815 patients in the control group. Meta-analysis showed that there are no significant differences in the scores for categories of audit performance (CAP), speech intelligibility rating (SIR), and parents\' evaluation of aural/oral performance of children (PEACH) between the WS group and the control group.
UNASSIGNED: Cochlear implantation demonstrates comparable auditory and speech recovery outcomes for WS patients and non-WS patients.

Neurexins play diverse functions as presynaptic organizers in various glutamatergic and GABAergic synapses. However, it remains unknown whether and how neurexins are involved in shaping functional properties of the glycinergic synapses, which mediate prominent inhibition in the brainstem and spinal cord. To address these issues, we examined the role of neurexins in a model glycinergic synapse between the principal neuron in the medial nucleus of the trapezoid body (MNTB) and the principal neuron in the lateral superior olive (LSO) in the auditory brainstem. Combining RNAscope with stereotactic injection of AAV-Cre in the MNTB of neurexin1/2/3 conditional triple knockout mice, we showed that MNTB neurons highly express all isoforms of neurexins although their expression levels vary remarkably. Selective ablation of all neurexins in MNTB neurons not only reduced the amplitude but also altered the kinetics of the glycinergic synaptic transmission at LSO neurons. The synaptic dysfunctions primarily resulted from an impaired Ca2+ sensitivity of release and a loosened coupling between voltage-gated Ca2+ channels and synaptic vesicles. Together, our current findings demonstrate that neurexins are essential in controlling the strength and temporal precision of the glycinergic synapse, which therefore corroborates the role of neurexins as key presynaptic organizers in all major types of fast chemical synapses.

Parvalbumin (PV) interneurons in the auditory cortex (AC) play a crucial role in shaping auditory processing, including receptive field formation, temporal precision enhancement, and gain regulation. PV interneurons are also the primary inhibitory neurons in the tail of the striatum (TS), which is one of the major descending brain regions in the auditory nervous system. However, the specific roles of TS-PV interneurons in auditory processing remain elusive. In this study, morphological and slice recording experiments in both male and female mice revealed that TS-PV interneurons, compared with AC-PV interneurons, were present in fewer numbers but exhibited longer projection distances, which enabled them to provide sufficient inhibitory inputs to spiny projection neurons (SPNs). Furthermore, TS-PV interneurons received dense auditory input from both the AC and medial geniculate body (MGB), particularly from the MGB, which rendered their auditory responses comparable to those of AC-PV interneurons. Optogenetic manipulation experiments demonstrated that TS-PV interneurons were capable of bidirectionally regulating the auditory responses of SPNs. Our findings suggest that PV interneurons can effectively modulate auditory processing in the TS and may play a critical role in auditory-related behaviors.

BACKGROUND: Previous studies have shown that children with intermittent exotropia (IXT) have a higher rate of psychiatric abnormalities as they grow up, such as attention deficit. This study explored visual and hearing attention among children with IXT, and evaluated its association with clinical characteristics and cognitive development.
METHODS: Forty-nine children with a diagnosis of IXT and 29 children with traditional development were recruited. The Integrated Visual and Auditory Continuous Performance Test (IVA-CPT) was used to measure the subjects\' full-scale response control quotient (FSRCQ), full-scale attention quotient (FSAQ), auditory response control quotient (ARCQ), auditory attention quotient (AAQ), visual response control quotient (VRCQ), and visual attention quotient (VAQ). The Wechsler Intelligence Scale for Children-Fourth Edition (WISC-IV) was used to assess their cognitive function. The differences between the scores of children with IXT and normal controls were analyzed.
RESULTS: The results showed that the FSRCQ, FSAQ, ARCQ, AAQ, VRCQ, and VAQ of children with IXT were all lower than those of normal controls with the same age (P < 0.05). The level of attention was significantly correlated with the age of strabismus onset (P < 0.05), but not with the degree of strabismus, stereopsis, or fusion control score. In addition, audiovisual attention was correlated significantly with their cognitive development level. The random forest classifier prediction model showed that age of strabismus onset was an important predictor of attention.
CONCLUSIONS: Children with IXT have lower visual and auditory attention and control than their peers, and the age of onset of strabismus may be a major factor.

The Yangtze finless porpoises (Neophocaena asiaeorientalis asiaeorientalis) living in different environments display significant differences in behavior and physiology. To compare and analyze gene expression differences between an ex situ population and a controlled environment population of the Yangtze finless porpoise, we sequenced the transcriptome of blood tissues living in a semi-natural reserve and an artificial facility, respectively. We identified 6860 differentially expressed genes (DEGs), of which 6603 were up-regulated and 257 were down-regulated in the controlled environment vs ex situ comparison. GO and KEGG enrichment analysis showed that the up-regulated genes in the controlled environment population were significantly associated with glucose metabolism, amino acid metabolism, and the nervous system, while those up-regulated in the ex situ population were significantly associated with energy supply and biosynthesis. Further analysis showed that metabolic and hearing-related genes were significantly affected by changes in the environment, and key metabolic genes such as HK, PFK, IDH, and GLS and key hearing-related genes such as OTOA, OTOF, SLC38A1, and GABBR2 were identified. These results suggest that the controlled environment population may have enhanced glucose metabolic ability via activation of glycolysis/gluconeogenesis, the TCA cycle, and inositol phosphate metabolism, while the ex situ population may meet higher energy requirements via enhancement of the amino acid metabolism of the liver and muscle and oxidative phosphorylation. Additionally, the acoustic behavior and auditory-related genes of Yangtze finless porpoise may show responsive changes and differential expression under different environment conditions, and thus the auditory sensitivity may also show corresponding adaptive characteristics. This study provides a new perspective for further exploration of the responsive changes of the two populations to various environments and provides a theoretical reference for further improvements in conservation practices for the Yangtze finless porpoise.

Direct neural recordings from human auditory cortex have demonstrated encoding for acoustic-phonetic features of consonants and vowels. Neural responses also encode distinct acoustic amplitude cues related to timing, such as those that occur at the onset of a sentence after a silent period or the onset of the vowel in each syllable. Here, we used a group reduced rank regression model to show that distributed cortical responses support a low-dimensional latent state representation of temporal context in speech. The timing cues each capture more unique variance than all other phonetic features and exhibit rotational or cyclical dynamics in latent space from activity that is widespread over the superior temporal gyrus. We propose that these spatially distributed timing signals could serve to provide temporal context for, and possibly bind across time, the concurrent processing of individual phonetic features, to compose higher-order phonological (e.g. word-level) representations.

The mammalian cochlear epithelium undergoes substantial remodeling and maturation before the onset of hearing. However, very little is known about the transcriptional network governing cochlear late-stage maturation and particularly the differentiation of its lateral nonsensory region. Here, we establish ZBTB20 as an essential transcription factor required for cochlear terminal differentiation and maturation and hearing. ZBTB20 is abundantly expressed in the developing and mature cochlear nonsensory epithelial cells, with transient expression in immature hair cells and spiral ganglion neurons. Otocyst-specific deletion of Zbtb20 causes profound deafness with reduced endolymph potential in mice. The subtypes of cochlear epithelial cells are normally generated, but their postnatal development is arrested in the absence of ZBTB20, as manifested by an immature appearance of the organ of Corti, malformation of tectorial membrane (TM), a flattened spiral prominence (SP), and a lack of identifiable Boettcher cells. Furthermore, these defects are related with a failure in the terminal differentiation of the nonsensory epithelium covering the outer border Claudius cells, outer sulcus root cells, and SP epithelial cells. Transcriptome analysis shows that ZBTB20 regulates genes encoding for TM proteins in the greater epithelial ridge, and those preferentially expressed in root cells and SP epithelium. Our results point to ZBTB20 as an essential regulator for postnatal cochlear maturation and particularly for the terminal differentiation of cochlear lateral nonsensory domain.

Internal forward models hypothesize functional links between motor and sensory systems for predicting the consequences of actions. Recently, the cascaded theory proposes that somatosensory estimation in the inferior parietal lobe (IPL) can be a relay computational structure, converting motor signals into predictions of auditory consequences in a serial processing manner during speech production. The study used fMRI with functional connectivity (FC) analyses to investigate the proposed cascaded processes using three speech tasks: overt articulation (OA), silent articulation (SA) and imagined articulation (IA). The FC results showed that connectivity between aIPL and STG was increased in OA compared with SA, suggesting that the relationship between somatosensory and auditory estimations can be modulated by speech tasks. Moreover, stronger connectivity between IFGoper and pIPL, and between pIPL and STG were observed in SA and IA compared with OA. These results are consistent with a cascaded process in the internal forward models.

Multiple object tracking (MOT) is generally regarded as a pure attention-consuming task that draws heavily on attention resources. In the present study, we adopted a cross-channel visual-audio dual-task paradigm, i.e., the MOT task combined with the concurrent auditory N-back working memory task, to test whether working memory indeed plays a necessary role in the process of multiple tracking, as well as to further identify the specific types of working memory components involved in this process. Experiments 1a and 1b examined the relationship between the MOT task and nonspatial object working memory (OWM) processing by manipulating the tracking load and working memory load, respectively. Results in both experiments indicated that the concurrent nonspatial OWM task did not have a significant effect on the tracking capacity of the MOT task. In contrast, Experiments 2a and 2b examined the relationship between the MOT task and spatial working memory (SWM) processing by a similar approach. Results in both experiments indicated that the concurrent SWM task significantly impaired the tracking capacity of the MOT task, showing a gradual decrease with increasing SWM load. Overall, our study provides empirical evidence that multiple object tracking does involve working memory, primarily related to spatial working memory rather than nonspatial object working memory, which sheds more light on the mechanisms of multiple object tracking.

Cochlear implants (CIs) allow patients with severe to profound hearing loss to gain or regain their sense of hearing. However, the objective assessment of auditory rehabilitation in CI users remains a challenge. In particular, the utility of phase-amplitude coupling (PAC) for evaluating postoperative rehabilitation of CI users remains unknown. In the present study, we conducted an oddball paradigm with stimuli varying in sample speech syllables and collected electroencephalography (EEG) signals for 10 CI users at the time the implant was activated and 180 days after activation. Twelve normal-hearing subjects served as controls. We explored the oscillatory properties of the neural response to syllable incongruence and the cross-frequency coupling between multiple frequencies in CI users. We found that beta-gamma coupling appeared to be enhanced in CI users compared with normal controls and this difference gradually disappeared with increasing implantation time. The present results suggest that predictively encoded auditory pathways are gradually restored in CI users. In addition, the PAC feature in unilateral CI users was found to be lateralized in the auditory cortex, which was consistent with previous studies of auditory-evoked cortical activity. Therefore, PAC may be a reference biomarker for the rehabilitation of speech discrimination in CI users.