Deafness: From genetic architecture to gene therapy

C Petit, C Bonnet, S Safieddine - Nature Reviews Genetics, 2023 - nature.com
Progress in deciphering the genetic architecture of human sensorineural hearing
impairment (SNHI) or loss, and multidisciplinary studies of mouse models, have led to the …

[HTML][HTML] Advances in gene therapy hold promise for treating hereditary hearing loss

L Jiang, D Wang, Y He, Y Shu - Molecular Therapy, 2023 - cell.com
Gene therapy focuses on genetic modification to produce therapeutic effects or treat
diseases by repairing or reconstructing genetic material, thus being expected to be the most …

[HTML][HTML] Inner ear gene therapies take off: current promises and future challenges

S Delmaghani, A El-Amraoui - Journal of clinical medicine, 2020 - mdpi.com
Hearing impairment is the most frequent sensory deficit in humans of all age groups, from
children (1/500) to the elderly (more than 50% of the over-75 s). Over 50% of congenital …

The genomics of auditory function and disease

S Taiber, K Gwilliam, R Hertzano… - Annual review of …, 2022 - annualreviews.org
Current estimates suggest that nearly half a billion people worldwide are affected by hearing
loss. Because of the major psychological, social, economic, and health ramifications …

[HTML][HTML] Advances and challenges in adeno-associated viral inner-ear gene therapy for sensorineural hearing loss

K Bankoti, C Generotti, T Hwa, L Wang… - … Therapy-Methods & …, 2021 - cell.com
There is growing attention and effort focused on treating the root cause of sensorineural
hearing loss rather than managing associated secondary characteristic features. With recent …

Gene therapy restores auditory functions in an adult Vglut3 knockout mouse model

X Zhao, H Liu, H Liu, R Cai, H Wu - Human Gene Therapy, 2022 - liebertpub.com
Adeno-associated virus (AAV)-based gene therapy has been demonstrated to be extremely
effective for treating genetic hearing loss over the past several years. However, successful …

[HTML][HTML] Vestibular deficits in deafness: Clinical presentation, animal modeling, and treatment solutions

A Maudoux, S Vitry, A El-Amraoui - Frontiers in neurology, 2022 - frontiersin.org
The inner ear is responsible for both hearing and balance. These functions are dependent
on the correct functioning of mechanosensitive hair cells, which convert sound-and motion …

Advances in cochlear gene therapies

MJ Klimara, RJH Smith - Current opinion in pediatrics, 2023 - journals.lww.com
Gene therapies for hearing loss are expected to enter clinical trials in the immediate future.
To provide referral for appropriate trials and counseling regarding benefits of genetic …

Advances in genome editing for genetic hearing loss

N Ding, S Lee, M Lieber-Kotz, J Yang, X Gao - Advanced drug delivery …, 2021 - Elsevier
Abstract According to the World Health Organization, hearing loss affects over 466 million
people worldwide and is the most common human sensory impairment. It is estimated that …

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 …