Neurodevelopmental Aspects and Cortical Auditory Maturation in Children with Cochlear Implants.

Background and objectives: The cochlear implant is not only meant to restore auditory function, but it also has a series of benefits on the psychomotor development and on the maturation of central auditory pathways. In this study, with the help of neuropsychological tests and cortical auditory potentials (CAEPs), we intend to identify a series of instruments that allow us to monitor children with a cochlear implant, and later on, to admit them into an individualized rehabilitation program. Materials and methods: This is a longitudinal study containing 17 subjects (6 boys and 11 girls) diagnosed with congenital sensorineural hearing loss. The average age for cochlear implantation in our cohort is 22 months old. Each child was tested before the cochlear implantation, tested again 3 months after the implant, and then 6 months after the implant. To test the general development, we used the Denver Developmental Screening Test (DDST II). CAEPs were recorded to assess the maturation of central auditory pathways. Results: The results showed there was progress in both general development and language development, with a significant statistical difference between the overall DQ (developmental quotient) and language DQ before the cochlear implantation and three and six months later, respectively. Similarly, CAEP measurements revealed a decrease of positive-going component (P1) latency after cochlear implantation. Conclusion: CAEPs and neuropsychological tests prove to be useful instruments for monitoring the progress in patients with cochlear implants during the rehabilitation process.

Hemophagocytic lymphohistiocytosis in a child with human immunodeficiency virus infection - a case report.

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition and furthermore human immunodeficiency virus (HIV)-associated HLH is rarely reported in the literature. The most frequent presentation of secondary HLH is in association with infections, malignancies or drugs. In HIV-positive patients, the viral infection itself or the antiretroviral therapy (ART) could trigger HLH. CASE PRESENTATION: A 14-year-old boy was admitted for persistent diarrhea, severe weight loss and chest burns. Laboratory tests revealed important neutropenia, which led to HIV infection diagnosis. ART in combination was started associated with granulocyte-colony stimulating factor. Viral copies declined after six weeks of treatment, but the cluster of differentiation 4 (CD4) T-lymphocytes and neutrophil counts remained very low. Infections and malignancies were ruled out. The bone marrow aspirate revealed hemophagocytosis which in association with fever, bicytopenia, hypofibrinogenemia and hypertriglyceridemia established HLH diagnosis. Cortisone therapy and intravenous immunoglobulins were added. Due to lack of response, HLH-2004 protocol was initiated in collaboration with pediatric hematologist. In the first six months of treatment, CD4 T-lymphocytes and neutrophil count remained low and then they showed significant increase simultaneously. During treatment, the patient developed spontaneous severe back pain. Magnetic resonance imaging showed vertebral compression, osteomalacia and a thoracic vertebral fracture. CONCLUSIONS: Having ruled out the usual associated infections and malignancies, this is a case of HIV-associated with HLH. In this case, only the ART and immunomodulatory therapy were not enough and failed to work. Systemic immunosuppression also worked only after a prolonged course which led to an unfortunate complication: a vertebral fracture.

Added Value of QEEG for the Differential Diagnosis of Common Forms of Dementia.

INTRODUCTION: Quantitative electroencephalography (QEEG) has been documented as a helpful tool in the differential diagnosis of Alzheimer's disease (AD) with common forms of dementia. The main objective of the study was to assess the role of QEEG in AD differential diagnosis with other forms of dementia: Lewy body dementia (LBD), Parkinson's disease dementia (PDD), frontotemporal dementia (FTD), and vascular dementia (VaD). METHODS: We searched PubMed, Embase, and PsycNET, for articles in English published in peer-reviewed journals from January 1, 1980 to April 23, 2019 using adapted search strategies containing keywords quantitative EEG and Alzheimer. The risk of bias was assessed by applying the QUADAS tool. The systematic review was conducted in line with the PRISMA methodology. RESULTS: We identified 10 articles showcasing QEEG features used in diagnosing dementia, EEG slowing phenomena in AD and PDD, coherence changes in AD and VaD, the role of LORETA in dementia, and the controversial QEEG pattern in FTD. Results vary significantly in terms of sociodemographic features of the studied population, neuropsychological assessment, signal acquisition and processing, and methods of analysis. DISCUSSION: This article provides a comparative synthesis of existing evidence on the role of QEEG in diagnosing dementia, highlighting some specific features for different types of dementia (eg, the slow-wave activity has been remarked in both AD and PDD, but more pronounced in PDD patients, a diminution in anterior and posterior alpha coherence was noticed in AD, and a lower alpha coherence in the left temporal-parietal-occipital regions was observed in VaD). CONCLUSION: QEEG may be a useful investigation for settling the diagnosis of common forms of dementia. Further research of quantitative analyses is warranted, particularly on the association between QEEG, neuropsychological, and imaging features. In conjunction, these methods may provide superior diagnostic accuracy in the diagnosis of dementia.

The Role of Quantitative EEG in the Diagnosis of Neuropsychiatric Disorders.

Quantitative electroencephalography (QEEG) is a modern type of electroencephalography (EEG) analysis that involves recording digital EEG signals which are processed, transformed, and analyzed using complex mathematical algorithms. QEEG has brought new techniques of EEG signals feature extraction: analysis of specific frequency band and signal complexity, analysis of connectivity, and network analysis. The clinical application of QEEG is extensive, including neuropsychiatric disorders, epilepsy, stroke, dementia, traumatic brain injury, mental health disorders, and many others. In this review, we talk through existing evidence on the practical applications of this clinical tool. We conclude that to date, the role of QEEG is not necessarily to pinpoint an immediate diagnosis but to provide additional insight in conjunction with other diagnostic evaluations in order to objective information necessary for obtaining a precise diagnosis, correct disease severity assessment, and specific treatment response evaluation.

Assessment of Cortical Auditory Function Using Electrophysiological and Neuropsychological Measurements in Children with Bone-Anchored Hearing Aids.

Children suffering from conductive or mixed hearing loss may benefit from a bone-anchored hearing aid system (BAHA Attract implantable prosthesis). After audiological rehabilitation, different aspects of development are improving. The objective of this case report is to propose a comprehensive framework for monitoring cortical auditory function after implantation of a bone-anchored hearing aid system by using electrophysiological and neuropsychological measurements. We present the case of a seven-year-old boy with a congenital hearing loss due to a plurimalformative syndrome, including outer and middle ear malformation. After the diagnosis of hearing loss and the audiological rehabilitation with a BAHA Attract implantable prosthesis, the cortical auditory evoked potentials were recorded. We performed a neuropsychological evaluation using the Wechsler Intelligence Scale for Children - Fourth Edition, which was applied according to a standard procedure. The P1 latency was delayed according to the age (an objective biomarker for quantifying cortical auditory function). The neuropsychological evaluation revealed that the child's working memory and verbal reasoning abilities were in the borderline range comparing with his nonverbal reasoning abilities and processing abilities, which were in the average and below-average range, respectively. Cortical auditory evoked potentials, along with neuropsychological evaluation, could be an essential tool for monitoring cortical auditory function in children with hearing loss after a bone-anchored hearing aid implantation.

The Potential Use of P1 CAEP as a Biomarker for Assessing Central Auditory Pathway Maturation in Hearing loss and Associated Disabilities: a case report.

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.