Gene therapy for inherited hearing loss: updates and remaining challenges

R Hahn, KB Avraham - Audiology Research, 2023 - mdpi.com
Hearing loss stands as the most prevalent sensory deficit among humans, posing a
significant global health challenge. Projections indicate that by 2050, approximately 10% of …

[HTML][HTML] Gene therapy in pediatrics–Clinical studies and approved drugs (as of 2023)

TM Gol, F Zahedipour, P Trosien, G Ureña-Bailén… - Life Sciences, 2024 - Elsevier
Gene therapy in pediatrics represents a cutting-edge therapeutic strategy for treating a
range of genetic disorders that manifest in childhood. Gene therapy involves the …

[HTML][HTML] In vivo AAV9-Myo7a gene rescue restores hearing and cholinergic efferent innervation in inner hair cells

AP O'Connor, AE Amariutei, A Zanella, SA Hool… - JCI …, 2024 - pmc.ncbi.nlm.nih.gov
In the mammalian cochlea, sensory hair cells are crucial for the transduction of acoustic
stimuli into electrical signals, which are then relayed to the central auditory pathway via …

Ototoxicity: a high risk to auditory function that needs to be monitored in drug development

MP Pasdelou, L Byelyayeva, S Malmström… - Frontiers in Molecular …, 2024 - frontiersin.org
Hearing loss constitutes a major global health concern impacting approximately 1.5 billion
people worldwide. Its incidence is undergoing a substantial surge with some projecting that …

Low‐intensity noise exposure takes an essential part in the mechanism of late‐onset hereditary hearing loss caused by Abcc1 mutation

M Jiang, S Zhang, J Liu, B Pang, Y Bai… - Clinical …, 2024 - Wiley Online Library
With the development of the social economy, we are exposed to increasing noise in our
daily lives. Our previous work found an ABCC1 (NM_004996. 3: c. A1769G, NP_004987. 2 …

Hearing Loss: Genetic Testing, Current Advances and the Situation in Latin America

MA De Rosa, MT Bernardi, S Kleppe, K Walz - Genes, 2024 - mdpi.com
Congenital hearing loss is the most common birth defect, estimated to affect 2–3 in every
1000 births, with~ 50–60% of those related to genetic causes. Technological advances …

[HTML][HTML] Contemporary directions in the therapy of sensory hearing loss

A Rzepakowska, A Borowy, E Siedlecki… - Polish Journal of …, 2024 - otolaryngologypl.com
Introduction: More than 5% of the world's population experience hearing impairment. The
most common form is presbycusis (age-related hearing loss; ARHL). It affects almost one in …

Inhibition of Pi4kb activity causes malformation of vestibular apparatus in zebrafish by downregulating hey1

Y Feng, J Zhang, Y Li, G Chen, X Zhang, G Ning, S Wu - Gene, 2024 - Elsevier
Abstract Phosphatidylinositol 4 kinase-β (PI4KB) plays critical roles in human genetic
diseases. In zebrafish, Pi4kb is strongly expressed in hair cells (HCs), which are necessary …

Auditory Neuropathy: Auditory Processing Consequences and Management Options

G Rance, J Zanin - Auditory Processing Disorders: Assessment …, 2024 - books.google.com
170 AUDITORY PROCESSING DISORDERS of subjects typically presents with significantly
impaired sound detection thresholds—in addition to their processing difficulties. As such …

The Effects and Treatments for Usher Syndrome: A Review

DM Heerlie, F Margaretha, DR Fugaha… - Indonesian Journal of …, 2024 - journal.i3l.ac.id
Usher syndrome is defined as the rare genetic disorder that affects both vision and auditory.
Although the prevalence is really low, only about 4 to 17 per 100,000 people, it is noted to …