Results 31 to 40 of about 931,628 (294)

TECTB Variants Reveal Tectorial Membrane Vulnerability in Dominant Non‐Syndromic Hearing Loss

Advanced Science, EarlyView.
TECTB is a non‐collagenous protein of the tectorial membrane – an extracellular matrix of the cochlea. This study identifies dominant missense variants in TECTB linked to human hereditary deafness in two unrelated families. Genetically engineered mice homozygous for one of the variants are profoundly deaf, whereas heterozygous mice have normal hearing ...
Evan B. Hale, Barbara Vona, Richard J. Goodyear, Richard T. Osgood, Jun Shen, Sami S. Amr, Karen Mojica, Michaela A. H. Hofrichter, Jörg Schröder, Sophie Flandin, Paul Gratias, Ricardo Vera‐Monroy, Katherine Callahan, Kerry L. Gudlewski, Rolen Quadros, Masato Ohtsuka, JoAnn McGee, Edward J. Walsh, Cynthia C. Morton, Thomas Haaf, Channabasavaiah Gurumurthy, James E. Saunders, Guy P. Richardson, Artur A. Indzhykulian   +23 more
wiley   +1 more source

Automating Speech Audiometry in Quiet and in Noise Using a Deep Neural Network

Biology
In addition to pure-tone audiometry tests and electrophysiological tests, a comprehensive hearing evaluation includes assessing a subject’s ability to understand speech in quiet and in noise. In fact, speech audiometry tests are commonly used in clinical
Hadrien Jean, Nicolas Wallaert, Antoine Dreumont, Gwenaelle Creff, Benoit Godey, Nihaad Paraouty   +5 more
doaj   +1 more source

Effect of face masks on speech understanding: A clinical perspective during speech audiometry

Journal of Otology, 2022
The objective was to measure the effect of various face masks on speech recognition threshold and the word recognition score in the presence of varying background noise levels. 20 normal-hearing adult subjects (a total of 40 ears) participated. Pure tone
Rawish Kumar, Sanjay Kumar Munjal, Anuradha Sharma, Md Noorain Alam, Naresh K. Panda   +4 more
doaj   +1 more source

Transient Sleep Deprivation Induces Persistent Auditory Neuropathy via ROS‐Initiated Neuroinflammation and BK Channel Suppression

Advanced Science, EarlyView.
Sleep disturbance severity closely tracks hearing loss in a clinical cohort, yet the mechanistic link remains unclear. Acute sleep deprivation is shown to trigger transient cochlear oxidative stress that switches into a self‐sustaining neuroinflammatory state, suppressing BK channels and causing irreversible synaptopathy.
Dan Chen, Ying Ma, Lixuan Huang, Xin Chen, Fanliang Kong, Zhenzhou Qian, Mingyue Guan, Xinlin Wang, Yanyan Yang, Shasha Zhang, Peng Li, Renjie Chai   +11 more
wiley   +1 more source

Brain Network Dynamics of Local and Global Predictive Processing in Aging

Advanced Science, EarlyView.
Separation of concurrent whole‐brain networks in source‐reconstructed magnetoencephalography (MEG) data suggests that healthy aging reorganizes, rather than uniformly attenuates, neural responses elicited from hierarchical auditory violations. Enhanced early sensory deviance processing alongside reduced higher‐order cognitive responses suggests a large‐
Mathias Houe Andersen, Gemma Fernández‐Rubio, David R. Quiroga‐Martinez, Mattia Rosso, Mathias Klarlund, Kit Melissa Larsen, Hartwig Roman Siebner, Morten L. Kringelbach, Peter Vuust, Leonardo Bonetti   +9 more
wiley   +1 more source

wav2vec2-based Speech Rating System for Children with Speech Sound Disorder

, 2022
The computational resources were provided by Aalto ScienceIT. This work was supported by NordForsk through the funding to Technology-enhanced foreign and second-language learning of Nordic languages, project number 103893.Speaking is a fundamental way of
Getman, Yaroslav, Grósz, Tamás, Al-Ghezi, Ragheb, Svendsen, Torbjørn, Kurimo, Mikko, Voskoboinik, Ekaterina, Strömbergsson, Sofia, Voskoboinik, Katja, Salvi, Giampiero   +8 more
core   +1 more source

ADNP‐Related Helsmoortel–Van der Aa Syndrome: A Review of the Literature and Clinical Recommendations for Assessment and Monitoring

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT ADNP‐related Helsmoortel–Van der Aa syndrome (ADNP‐related HVDAS) is a single gene form of autism spectrum disorder (ASD) caused by pathogenic sequence variants in the activity‐dependent neuroprotective protein (ADNP) gene. In addition to ASD, ADNP‐related HVDAS is associated with a wide range of cognitive, behavioral, and physical health ...
Jarrett Fastman, Ana Kolevzon, Paige Siper, Tess Levy, Jeffrey M. Saland, Yitzchak Frank, Robert Rapaport, Kayla Kupietzky, William E. Bennett Jr, Bruce Gelb, Sandra Bedrosian Sermone, Alexander Kolevzon   +11 more
wiley   +1 more source

Chinese Emotional Speech Audiometry Project (CESAP): Establishment and validation of a new material set with emotionally neutral disyllabic words

, 2023
Speech audiometry is a clinical tool to assess the ability to recognize and distinguish speech sounds. Since traditional speech audiometry tests have scantly considered the indispensable emotional components of everyday speech, our Chinese Emotional ...
Jessie Gong
core   +5 more sources

First Report of Uniparental Isodisomy of Chromosome 15 Revealing Angelman Syndrome and Microphthalmia Associated With a Novel Homozygous ALDH1A3 Variant

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Paternal isodisomy of chromosome 15 (iUPD15) is a recognized cause of Angelman syndrome (AS), accounting for approximately 2%–5% of cases. Additionally, another recognized consequence of iUPD is the unmasking of autosomal recessive disorders. However, reports of recessive disorders resulting from iUPD15 remain scarce in the literature.
Gabriela Roldão Correia‐Costa, Joana Rosa Marques Prota, Antonia Paula Marques‐de‐Faria, Carlos Eduardo Steiner, Társis Paiva Vieira   +4 more
wiley   +1 more source

Changes in the audiometric patterns in presbycusis with ongoing medical upgradation

Apollo Medicine, 2022
Background: Presbycusis or age-related hearing loss. Nowadays, age-related hearing impairment is recognized as a disorder with environmental and genetic factors, use of ototoxic medication, hypertension with increasing age, i.e., longevity of life the ...
Sugandha Sharma, Girja Thakur, Arvinpreet Kour, Ajaydeep Singh   +3 more
doaj   +1 more source