OBJECTIVE: Smoking affects sensory gating, as assessed by the event related potential P50, which is evoked by auditory stimuli and is considered to be involved in the pathophysiology of schizophrenia (SCZ). However, few studies have compared sensory gating and cognitive performance between smoking and non-smoking SCZ patients in the Chinese Han population.
METHODS: We recruited two groups of Chinese subjects: 128 male chronic SCZ patients and 76 male healthy controls, measuring cognition with the MATRICS Consensus Cognitive Battery (MCCB) and sensory gating with the P50 EEG components. Based on their smoking status, they were further divided into 4 subgroups: smoking SCZ patients, non-smoking SCZ patients, smoking healthy controls, and non-smoking healthy controls. We assessed psychopathological symptoms of the patients using the Positive and Negative Syndrome Scale (PANSS).
RESULTS: Compared with healthy controls, SCZ patients had lower MCCB total score and scores of all 10 tests (all p < 0.05), while SCZ patients had higher S2 amplitudes and P50 ratios (both p < 0.05). When comparing smoking versus non-smoking SCZ patients, non-smokers had significantly better spatial span (p < 0.05). Furthermore, the S1 amplitude was negatively correlated with the Brief Visuospatial Memory Test (BVMT-R) in smoking patients (p < 0.05), while the S1 latency was negatively correlated with spatial span in non-smoking patients (p < 0.01).
CONCLUSIONS: Our finding shows a difference in the relationship between sensory gated P50 and cognition in smoking and non-smoking SCZ patients, suggesting that nicotine may improve cognitive and P50 deficits in SCZ patients.

OBJECTIVE: The purpose of this study was to comprehensively monitor the auditory skill development of children with hearing loss after hearing instrument fitting, and a battery of four assessments was proposed.
METHODS: This battery was designed to fill the gap in speech discrimination in clinically available evaluations. The battery includes both behavioral and neural assessments. On the other hand, both tests in structured settings (sound-treated booth) and daily life were included in the battery. The four assessments include visual reinforced infant speech discrimination (VRISD), cortical auditory evoked potentials (CAEP), Auditory Skills Checklist (ASC), and Parents\' Evaluation of Aural/Oral Performance of Children (PEACH).
RESULTS: Two cases were reported, and their clinical implications were discussed.
CONCLUSIONS: The proposed comprehensive assessment battery is suitable for evaluating children who are developmentally appropriate for visual reinforcement audiometry. More importantly, the VRISD assessment fills in the current gap, which is the discrimination stage, for the available clinical tests for assessing auditory developmental stages.

Objective: Auditory P50 gating changed might be a neurophysiological biomarker of the diagnosis of Mild Cognitive Impairment (MCI). We aimed to determine the impact of MCI in auditory P50 gating. Methods: All recruited participants completed structured questionnaires and finished auditory P50 gating measure. Results: A total of 20 MCI patients and 17 controls had been recruited. MCI patients had a significant higher reduction of P50 gating at Fz site, when compared to controls (1.21 ± .68 vs .66 ± .37, P = .00). Zero point five was the best cut off point to distinguish MCI and control of auditory P50 gating S2/S1 at Fz site. The P50 average amplitude at Pz site in MCI patients was significantly higher than controls (2.62 ± 1.20 vs 1.70 ± .74, P = .01). Conclusion: MCI patients might have impaired the ability of inhibiting the repeated stimulus.

SHANK3 encodes a scaffold protein involved in postsynaptic receptor density in glutamatergic synapses, including those in the parvalbumin (PV)+ inhibitory neurons-the key players in the generation of sensory gamma oscillations, such as 40-Hz auditory steady-state response (ASSR). However, 40-Hz ASSR was not studied in relation to SHANK3 functioning. Here, we present a 15-year-old girl (SH01) with previously unreported duplication of the first seven exons of the SHANK3 gene (22q13.33). SH01\'s electroencephalogram (EEG) during 40-Hz click trains of 500 ms duration binaurally presented with inter-trial intervals of 500-800 ms were compared with those from typically developing children (n = 32). SH01 was diagnosed with mild mental retardation and learning disabilities (F70.88), dysgraphia, dyslexia, and smaller vocabulary than typically developing (TD) peers. Her clinical phenotype resembled the phenotype of previously described patients with 22q13.33 microduplications (≈30 reported so far). SH01 had mild autistic symptoms but below the threshold for ASD diagnosis and microcephaly. No seizures or MRI abnormalities were reported. While SH01 had relatively preserved auditory event-related potential (ERP) with slightly attenuated P1, her 40-Hz ASSR was totally absent significantly deviating from TD\'s ASSR. The absence of 40-Hz ASSR in patients with microduplication, which affected the SHANK3 gene, indicates deficient temporal resolution of the auditory system, which might underlie language problems and represent a neurophysiological biomarker of SHANK3 abnormalities.

Frequency-following responses to musical notes spanning the octave 65-130 Hz were elicited in a person with auditory neuropathy, a disorder of subcortical neural synchrony, and a control subject. No phaselocked responses were observed in the person with auditory neuropathy. The control subject had robust responses synchronized to the fundamental frequency and its harmonics. Cortical onset responses to each note in the series were present in both subjects. These results support the hypothesis that subcortical neural synchrony is necessary to generate the frequency-following response-including for stimulus frequencies at which a cortical contribution has been noted. Although auditory cortex ensembles may synchronize to fundamental frequency cues in speech and music, subcortical neural synchrony appears to be a necessary antecedent.NEW & NOTEWORTHY A listener with auditory neuropathy, an absence of subcortical neural synchrony, did not have electrophysiological frequency-following responses synchronized to an octave of musical notes, with fundamental frequencies ranging from 65 to 130 Hz. A control subject had robust responses that phaselocked to each note. Although auditory cortex may contribute to the scalp-recorded frequency-following response in healthy listeners, our results suggest this phenomenon depends on subcortical neural synchrony.

To highlight the effectiveness of using PS testing in conjunction with electrically evoked auditory evoked potentials (eAEPs) to help guide treatment plans in patients with limited behavioural responses.
Case report on a 59-year-old male with traumatic brain injury. Electrophysiological measurements in conjunction with PS were performed.
eAEPs were obtained up to the thalamo-cortical region, supporting the viability of a CI in the non-implanted ear.
Use of PS in conjunction with electrically evoked auditory evoked potentials can provide valuable information to guide clinical decisions regarding implantation.

We report a case in which we quantified the maturation of the central auditory pathway in children with hearing loss and associated disabilities; the audiological intervention was performed using the BAHA softband. The hearing aid was applied according to the international clinical protocols. The presented case reveals the importance of using the P1 CAEP biomarker in clinical practice along with a neuropsychological evaluation to assess the maturation of the central auditory pathways and to objectively quantify the results of auditory rehabilitation in children with hearing loss and associated disabilities.

Nutritional deficiencies often precede the diagnosis of cystic fibrosis (CF) in infants, and occur at a stage where the rapidly developing brain is more vulnerable to insult. We aim to compare fat-soluble nutrient status of newly diagnosed non-screened infants with CF to that of healthy infants, and explore the association with neurodevelopment evaluated by electroencephalography (EEG). Our results show that CF infants had lower levels of all fat-soluble vitamins and docosahexaenoic acid (DHA) compared to controls. The auditory evoked potential responses were higher in CF compared to controls whereas the visual components did not differ between groups. DHA levels were correlated with auditory evoked potential responses. Although resting state frequency power was similar between groups, we observed a negative correlation between DHA levels and low frequencies. This study emphasizes the need for long-term neurodevelopmental follow-up of CF infants and pursuing intervention strategies in the future.

OBJECTIVE: A cochlear implant (CI) has the potential to improve the functioning of a deaf child in many aspects. Nevertheless, the dynamics of the general development, beyond the typically measured language abilities, directly after CI, is still unknown, especially if a child is implanted early. In this study we present a methodological framework for assessment of different domains of development, as well as the central auditory nervous system (CANS) maturation in infants and toddlers with a CI.
METHODS: Three children with bilateral congenital hearing loss and a unilateral CI, aged below 2.5 years, participated in a longitudinal study. Children were tested at three time points after cochlear implantation using the Polish Children Development Scale (CDS) consisting of a comprehensive battery of tests, as well as recordings of Cortical Auditory Evoked Potentials (CAEP).
RESULTS: All three children revealed gradual improvement in the overall CDS result as well as most of the CDS subscales. After 9 months of CI experience two younger children showed age-appropriate performance. In CAEP measurements a decrease of latency of the P1 component (an established biomarker of cortical auditory maturation) was observed in the same two children, with one achieving normal ranges of P1 latency after 9 months of CI use.
CONCLUSIONS: Our novel methodological framework can be successfully applied in small children with cochlear implants. It contributes to better understanding of the general development in early implanted children. The preliminary results indicate variability in children\'s performance in various developmental domains and thus the need to monitor the development of each child individually and holistically.

The auditory frequency-following response (FFR) reflects synchronized and phase-locked activity along the auditory pathway in response to sound. Although FFRs were historically thought to reflect subcortical activity, recent evidence suggests an auditory cortex contribution as well. Here we present electrophysiological evidence for the FFR\'s origins from two cases: a patient with bilateral auditory cortex lesions and a patient with auditory neuropathy, a condition of subcortical origin. The patient with auditory cortex lesions had robust and replicable FFRs, but no cortical responses. In contrast, the patient with auditory neuropathy had no FFR despite robust and replicable cortical responses. This double dissociation shows that subcortical synchrony is necessary and sufficient to generate an FFR.NEW & NOTEWORTHY The frequency-following response (FFR) reflects synchronized and phase-locked neural activity in response to sound.  The authors present a dual case study, comparing FFRs and cortical potentials between a patient with auditory neuropathy (a condition of subcortical origin) and a patient with bilateral auditory cortex lesions. They show that subcortical synchrony is necessary and sufficient to generate an FFR.