Electrophysiological Screening for Children With Suspected Auditory Processing Disorder: A Systematic Review

Objective: This research aimed to provide evidence for the early identification and intervention of children at risk for auditory processing disorder (APD). Electrophysiological studies on children with suspected APDs were systematically reviewed to understand the different electrophysiological characteristics of children with suspected APDs.Methods: Computerized databases such as PubMed, Cochrane, MEDLINE, Web of Science, and EMBASE were searched for retrieval of articles since the establishment of the database through May 18, 2020. Cohort, case-control, and cross-sectional studies that evaluated the literature for the electrophysiological assessment of children with suspected APD were independently reviewed by two researchers for literature screening, literature quality assessment, and data extraction. The Newcastle–Ottawa Scale and 11 entries recommended by the Agency for Healthcare Research and Quality were used to evaluate the quality of the literature.Results: In accordance with the inclusion criteria, 14 articles were included. These articles involved 7 electrophysiological testing techniques: click-evoked auditory brainstem responses, frequency-following responses, the binaural interaction component of the auditory brainstem responses, the middle-latency response, cortical auditory evoked potential, mismatch negativity, and P300. The literature quality was considered moderate.Conclusions: Auditory electrophysiological testing can be used for the characteristic identification of children with suspected APD; however, the value of various electrophysiological testing methods for screening children with suspected APD requires further study.

What can visual electrophysiology tell about possible visual-field defects in paediatric patients

Recognising a potential visual-field (VF) defect in paediatric patients might be challenging, especially in children before the age of 5 years and those with developmental delay or intellectual disability. Visual electrophysiological testing is an objective and non-invasive technique for evaluation of visual function in paediatric patients, which can characterise the location of dysfunction and differentiate between disorders of the retina, optic nerve and visual pathway. The recording of electroretinography (ERG) and visual-evoked potentials (VEP) is possible from early days of life and requires no subjective input from the patient. As the origins of ERG and VEP tests are known, the pattern of electrophysiological changes can provide information about the VF of a child unable to perform accurate perimetry. This review summarises previously published electrophysiological findings in several common types of VF defects that can be found in paediatric patients (generalised VF defect, peripheral VF loss, central scotoma, bi-temporal hemianopia, altitudinal VF defect, quadrantanopia and homonymous hemianopia). It also shares experience on using electrophysiological testing as additional functional evidence to other tests in the clinical challenge of diagnosing or excluding VF defects in complex paediatric patients. Each type of VF defect is illustrated with one or two clinical cases.

Clinical diagnosis guidelines and neurorestorative treatment for chronic disorders of consciousness (2021 China version)

Chronic disorders of consciousness (DOC) include the vegetative state and the minimally consciousness state. The DOC diagnosis mainly relies on the evaluation of clinical behavioral scales, electrophysiological testing, and neuroimaging examinations. No specifically effective neurorestorative methods for chronic DOC currently exist. Any valuable exploration therapies of being able to repair functions and/or structures in the consciousness loop (e.g., drugs, hyperbaric medicines, noninvasive neurostimulation, sensory and environmental stimulation, invasive neuromodulation therapy, and cell transplantation) may become effective neurorestorative strategies for chronic DOC. In the viewpoint of Neurorestoratology, this guideline proposes the diagnostic and neurorestorative therapeutic suggestions and future exploration direction for this disease following the review of the existing treatment exploration achievements for chronic DOC.

Identification of a Novel de Novo Variant in the SYT2 Gene Causing a Rare Type of Distal Hereditary Motor Neuropathy

Objective: To report the first de novo missense mutation in the SYT2 gene causing distal hereditary motor neuropathy. Methods: Genetic testing was carried out, including clinical exome sequencing for the proband and Sanger sequencing for the proband and his parents. We described the clinical and electrophysiological features found in the patient. Results: We reported a proband with a new de novo missense mutation, c.917C>T (p.Ser306Leu), in the C2B domain of SYT2. The clinical presentation was similar to that of phenotypes described in previous studies. A notable feature in our study was normal electrophysiological testing results of the patient. Conclusions: In this study we reinforced the association between SYT2 mutations and distal hereditary motor neuropathy. We also described the clinical presentation of the patient carrying this pathogenic variant and provided unusual results of electrophysiological testing. The results showed that a diagnosis of SYT2-associated neuropathy should be based on the similarity of clinical manifestations, rather than the results of electrophysiological testing.

B-scan ultrasound, visual electrophysiology and perioperative videoendoscopy for predicting functional results in keratoprosthesis candidates

Background/AimsWe analysed the ability of B-scan ultrasound, ocular electrophysiology testing and videoendoscopic examination for predicting visual prognosis in Boston Type 1 keratoprosthesis (KPro-1) candidates. Indirect anatomical and electrophysiological findings and results from direct endoscopic evaluations were correlated with postoperative functional data.MethodsIn this prospective and interventional study, we included 13 individuals who had previously been indicated for Kpro-1 surgery. All subjects underwent preoperative screening, including ophthalmic evaluation, B-scan ultrasound, electrophysiological testing, and perioperative intraocular videoendoscopic evaluation (VE). B-scan ultrasound, electrophysiological testing, and VE evaluation results were categorised as favourable or unfavourable predictors of postoperative functional results according to predefined criteria. The predictability values of B-scan ultrasound, electrophysiological testing, and VE prognostication were calculated based on the visual acuity level achieved.ResultsAll surgeries and perioperative VEs were uneventful. Preoperative best-corrected visual acuity (BCVA) ranged from light perception to counting fingers. The 1-year postoperative BCVA was better than 20/200 (satisfactory visual acuity result) in 10 eyes (76.9%) and 20/40 or better in 5 eyes (38.5%). B-scan ultrasound presented a positive predictive value (PPV) of 85.7% for satisfactory postoperative visual acuity, electroretinography showed a PPV of 66.7%, and visual evoked potential presented a PPV of 66.7%. The perioperative VE PPV of a negative finding for satisfactory visual acuity was 100%.ConclusionsFundoscopic visualisation by intraocular VE is a minimally invasive procedure that can be used to predict functional outcomes in keratoprosthesis candidates. This technique demonstrated better prognostication in keratoprosthesis candidates than B-scan ultrasound and electrophysiological testing.