Results 51 to 60 of about 35,576 (284)

Histidine Supplementation Stabilizes Hearing and Vision and Improves Growth in HARS1‐Related Autosomal Recessive Disorder Associated With Usher‐Like Symptoms

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Autosomal recessive HARS1‐related disorder (originally described as Usher syndrome type 3B) caused by a homozygous Y454S variant in the histidyl‐tRNA synthetase gene (HARS1) is characterized by progressive sensorineural hearing and vision loss and respiratory deterioration with risk for sudden death following febrile illnesses.
Victoria Mok Siu, Rosan Kenana, Rana Chakrabarti, Sarah D. P. Wilhelm, Joseph Andrews, Susan J. Leat, Christina Parker, Michael Miller, Leslie A. Nangle, Wendy McCaul, Ashfia Chowdhury, Natalie Hutchings, Ryan A. Adams, Lauren Guy, Mandy Rhody, Verena Juncal, Marisa I. Mendes, Desiree E. C. Smith, Gajja S. Salomons, Angelica A. Moresco, Daphne L. McCulloch, D. Holmes Morton, Ilka U. Heinemann, C. Anthony Rupar   +23 more
wiley   +1 more source

[The auditory brainstem response].

No to hattatsu = Brain and development, 1989
In 1967, Sohmer and Feinmesser recorded four negative peaks from extracochlear surface electrodes and they stated that the later components may be due to repetitive firing of auditory nerve fibers or may be due to the discharge of neurons in brain stem auditory nuclei.
openaire   +2 more sources

Partial hearing recovery after cochlear nerve surface transplantation of hair follicle‐derived neural crest stem cells in neural hearing loss rat

Animal Models and Experimental Medicine, EarlyView.
This study comprises three experimental parts. Part 1. Time‐course of ouabain‐induced hearing loss. Rats received ouabain (10 mM) via round window application. ABR/DPOAE tests and histological analyses were performed at 2, 7, and 30 days post‐treatment to characterize SGN and glial cell degeneration. Part 2.
Huidong Chen, Yunlong Zhang, Huanzhi Wan, Bingqian Yang, Tianyi Liu, Jie Wang, Hua Liao, Yuanyuan Zhang, Zhijian Zhang, Qingquan Hua   +9 more
wiley   +1 more source

Show Me the Brain!!: A modern approach to neuroanatomy education

Anatomical Sciences Education, EarlyView.
Abstract Show Me the Brain!! (SMtB) is a digital system for interactive graphics that is designed to support instruction in neuroanatomy and neuroscience. It will soon be made open‐source and freely available. SMtB bridges medical and traditional neuroanatomy instruction with the computational systems and representational conventions common in ...
Nicholas C. Hindy, Anthony J. Bishara, Ellie Lovellette, John R. Pani   +3 more
wiley   +1 more source

Atypical Predictive Processing Is Associated With Sensory Over‐Responsivity in Autism

Autism Research, EarlyView.
ABSTRACT Autism is characterized by sensory processing atypicalities including sensory over‐responsivity (SOR), a heightened negative response to sensory stimuli. Although the neural mechanisms underlying SOR are not fully understood, SOR has been associated with sensory‐limbic hyper‐reactivity during sensory stimulation.
Bar Yosef, Valerie Burgess, Megan Banchik, Joshua Ceballos, Mirella Dapretto, Shulamite Green   +5 more
wiley   +1 more source

Transforming Brain Health With Neurotechnology Convergence (Part II): Intelligent Neurointervention Systems for Neurological Disorders

Brain Health, EarlyView.
ABSTRACT Neurological disorders represent a critical domain within global health, necessitating advanced interventions to address complex pathologies such as tumors, functional disorders, and cerebrovascular diseases. Despite the proven benefits of early intervention, current treatment paradigms face significant challenges: (1) limited precision in ...
Qing Ye, Fei Peng, Chao Pan, Ping Zhang, Xuewei Xie, Zixiao Li, Yingxin Tang, Danyang Chen, Yuanwei Li, Jiawen Wu, Zao Han, Qing Dai, Quansheng Gu, Jizong Zhao, Zhouping Tang   +14 more
wiley   +1 more source

The segregation of Calb1, Calb2, and Prph neurons reveals distinct and mixed neuronal populations and projections to hair cells in the inner ear and central nuclei

Developmental Dynamics, EarlyView.
Three populations of hair cells have a distinct expression of Calb1 and Calb2. (A, A′D) The central is highly positive for Calb1 while surrounding HC are positive for Calb2. Later, a calyx forms primarily with Calb1. (B, B′, D′, D″) Saccule and utricle start out positive for Calb2 but will upregulate the Calb1 in the striola that is primarily forming ...
Jeong Han Lee, Ebenezer N. Yamoah, Jennifer Kersigo, Karen Elliott, Niya LaRoda, Gabriela Pavlinkova, Bernd Fritzsch   +6 more
wiley   +1 more source

Inherited metabolic epilepsies–established diseases, new approaches

Epilepsia Open, EarlyView.
Abstract Inherited metabolic epilepsies (IMEs) represent the inherited metabolic disorders (IMDs) in which epilepsy is a prevailing component, often determining other neurodevelopmental outcomes associated with the disorder. The different metabolic pathways affected by individual IMEs are the basis of their rarity and heterogeneity.
Itay Tokatly Latzer, Phillip L. Pearl
wiley   +1 more source

Prognostic value of auditory brainstem response for neurologic outcome in patients with neonatal indirect hyperbilirubinemia

, 2001
To investigate the value of the auditory brainstem response as a reliable test for the neurologic prognosis. of infants. with neonatal indirect hyperbilirubinemia, auditory brainstem response studies were performed in 22 infants.
ÇİPRUT, AYŞE AYÇA
core   +2 more sources

Auditory evoked potentials: predicting speech therapy outcomes in children with phonological disorders

Clinics, 2014
OBJECTIVES: This study investigated whether neurophysiologic responses (auditory evoked potentials) differ between typically developed children and children with phonological disorders and whether these responses are modified in children with ...
Renata Aparecida Leite, Haydée Fiszbein Wertzner, Isabela Crivellaro Gonçalves, Fernanda Cristina Leite Magliaro, Carla Gentile Matas   +4 more
doaj   +1 more source