Genetic therapies for hearing loss: Accomplishments and remaining challenges

S Taiber, KB Avraham - Neuroscience letters, 2019 - Elsevier
More than 15 years have passed since the official completion of the Human Genome
Project. Predominantly due to this project, over one hundred genes have now been linked to …

[HTML][HTML] Selection of viral capsids and promoters affects the efficacy of rescue of Tmprss3-deficient cochlea

KA Aaron, K Pekrun, PJ Atkinson, SE Billings… - … Therapy-Methods & …, 2023 - cell.com
Adeno-associated virus (AAV)-mediated gene transfer has shown promise in rescuing
mouse models of genetic hearing loss, but how viral capsid and promoter selection affects …

[HTML][HTML] Current AAV-mediated gene therapy in sensorineural hearing loss

J Qi, X Fu, L Zhang, F Tan, N Li, Q Sun, X Hu, Z He… - Fundamental …, 2022 - Elsevier
The number of patients with hearing loss is on the rise due to congenital abnormalities,
degenerative changes in old age, and acquired injuries such as virus or ototoxic drug …

Auditory brainstem response (ABR) measurements in small mammals

YH Kim, KM Schrode, AM Lauer - … and functional neurobiology of the inner …, 2022 - Springer
Auditory brainstem responses (ABRs) are evoked potentials that are measured from the
scalp and reflect the activity of the auditory neurons in the auditory pathway in response to …

[HTML][HTML] Gene therapy in mouse models of deafness and balance dysfunction

L Wang, JB Kempton, JV Brigande - Frontiers in molecular …, 2018 - frontiersin.org
Therapeutic strategies to restore hearing and balance in mouse models of inner ear disease
aim to rescue sensory function by gene replacement, augmentation, knock down or knock …

Early phase trials of novel hearing therapeutics: Avenues and opportunities

AGM Schilder, MP Su, R Mandavia, CR Anderson… - Hearing Research, 2019 - Elsevier
Novel hearing therapeutics are rapidly progressing along the innovation pathway and into
the clinical trial domain. Because these trials are new to the hearing community, they come …

Genetics of vestibular syndromes

P Roman-Naranjo, A Gallego-Martinez… - Current opinion in …, 2018 - journals.lww.com
Most of episodic or progressive syndromes show familial clustering. A detailed phenotyping
with a complete familial history of vestibular symptoms is required to conduct a genetic …

Posterior semicircular canal approach for inner ear gene delivery in neonatal mouse

K Isgrig, WW Chien - JoVE (Journal of Visualized Experiments), 2018 - jove.com
Inner ear gene therapy offers great promise as a potential treatment for hearing loss and
dizziness. One of the critical determinants of the success of inner ear gene therapy is to find …

Cochlear gene therapy

L Lustig, O Akil - Cold Spring Harbor perspectives …, 2019 - perspectivesinmedicine.cshlp.org
Over 450 million people worldwide suffer from hearing loss, leading to an estimated
economic burden of∼ $750 billion. The past decade has seen significant advances in the …

[HTML][HTML] Fetal gene therapy and pharmacotherapy to treat congenital hearing loss and vestibular dysfunction

ML Hastings, JV Brigande - Hearing research, 2020 - Elsevier
Disabling hearing loss is expected to affect over 900 million people worldwide by 2050. The
World Health Organization estimates that the annual economic impact of hearing loss …