Disease mechanisms and gene therapy for Usher syndrome

GGS Géléoc, A El-Amraoui - Hearing research, 2020 - Elsevier
Usher syndrome (USH) is a major cause of deaf-blindness in humans, affecting∼ 400 000
patients worldwide. Three clinical subtypes, USH1-3, have been defined, with 10 USH …

Partisipasi Guru Musik Berbasis Potensi Siswa Pendidikan Musik: Kajian Studi di Sekolah Musik

N Seruyanti, MO Sihombing, S Hanriani… - Jurnal Pengajaran …, 2023 - journals.eduped.org
Penelitian ini dilatarbelakangi oleh pelaku sekaligus pengguna pembelajaran yakni guru
musik bagi anak musik. Penggunaan tersebut penulis temukan melalui piano klasik …

Can haptic stimulation enhance music perception in hearing-impaired listeners?

MD Fletcher - Frontiers in Neuroscience, 2021 - frontiersin.org
Cochlear implants (CIs) have been remarkably successful at restoring hearing in severely-to-
profoundly hearing-impaired individuals. However, users often struggle to deconstruct …

Preclinical evaluation of the efficacy and safety of AAV1-hOTOF in mice and nonhuman primates

L Zhang, H Wang, M Xun, H Tang, J Wang, J Lv… - … Therapy-Methods & …, 2023 - cell.com
Pathogenic mutations in the OTOF gene cause autosomal recessive hearing loss (DFNB9),
one of the most common forms of auditory neuropathy. There is no biological treatment for …

Frequency Selectivity via Inner Boundary Conditions for A Self‐Powered Multiresonant Acoustic Sensing Array with Broad Bandwidth

S Lee, W Kim, NC Park, JW Park - Advanced Functional …, 2023 - Wiley Online Library
This study investigates and proposes innovative approaches to achieve frequency selectivity
within a limited space. Traditional multiresonant acoustic devices use individual sensing …

[HTML][HTML] Hair cell-specific Myo15 promoter-mediated gene therapy rescues hearing in DFNB9 mouse model

H Wang, MZ Xun, H Tang, J Zhao, S Hu… - … Therapy-Nucleic Acids, 2024 - cell.com
Adeno-associated viral (AAV) vectors are increasingly used as vehicles for gene delivery to
treat hearing loss. However, lack of specificity of the transgene expression may lead to …

Combined AAV-mediated gene replacement therapy improves auditory function in a mouse model of human DFNB42 deafness

K Isgrig, AX Cartagena-Rivera, HJ Wang, M Grati… - Molecular Therapy, 2023 - cell.com
Hearing loss is a common disorder affecting nearly 20% of the world's population. Recently,
studies have shown that inner ear gene therapy can improve auditory function in several …

Association between flat-panel computed tomographic imaging–guided place-pitch mapping and speech and pitch perception in Cochlear implant users

NT Jiam, M Gilbert, D Cooke… - … –Head & Neck …, 2019 - jamanetwork.com
Importance Cochlear implant users generally display poor pitch perception. Flat-panel
computed tomography (FPCT) has recently emerged as a modality capable of localizing …

Adeno-associated virus gene replacement for recessive inner ear dysfunction: Progress and challenges

C Askew, WW Chien - Hearing research, 2020 - Elsevier
Approximately 3 in 1000 children in the US under 4 years of age are affected by hearing
loss. Currently, cochlear implants represent the only line of treatment for patients with severe …

Music perception and training for pediatric cochlear implant users

NT Jiam, C Limb - Expert review of medical devices, 2020 - Taylor & Francis
ABSTRACT Introduction Cochlear implants (CIs) are biomedical devices that restore sound
perception for people with severe-to-profound sensorineural hearing loss. Most postlingually …