scholarly journals Dual-vector gene therapy restores cochlear amplification and auditory sensitivity in a mouse model of DFNB16 hearing loss

2021 ◽  
Vol 7 (51) ◽  
Author(s):  
Olga Shubina-Oleinik ◽  
Carl Nist-Lund ◽  
Courtney French ◽  
Shira Rockowitz ◽  
A. Eliot Shearer ◽  
...  
Keyword(s):  
Gene Therapy ◽  
Hearing Loss ◽  
Mouse Model ◽  
Auditory Sensitivity ◽  
Dual Vector ◽  
Cochlear Amplification

scholarly journals Methionine Sulfoxide Reductase B3-Targeted In Utero Gene Therapy Rescues Hearing Function in a Mouse Model of Congenital Sensorineural Hearing Loss

2016 ◽  
Vol 24 (11) ◽  
pp. 590-602 ◽  
Author(s):  
Min-A Kim ◽  
Hyun-Ju Cho ◽  
Seung-Hyun Bae ◽  
Byeonghyeon Lee ◽  
Se-Kyung Oh ◽  
...  
Keyword(s):  
Gene Therapy ◽  
Hearing Loss ◽  
Mouse Model ◽  
Sensorineural Hearing Loss ◽  
Methionine Sulfoxide ◽  
In Utero ◽  
Sensorineural Hearing ◽  
Methionine Sulfoxide Reductase ◽  
Hearing Function

Treating Combat Hearing Loss with Atoh1 Gene Therapy

2011 ◽  
Author(s):  
Andrew K. Groves
Keyword(s):  
Gene Therapy ◽  
Hearing Loss

Vascular Endothelial Growth Factor Electro-Gene Therapy Improves Functional Outcome in a Mouse Model of ALS

2013 ◽  
Vol 13 (2) ◽  
pp. 107-111
Author(s):  
Tatsufumi Murakami ◽  
Yuki Shimada ◽  
Yoshimi Imada ◽  
Akihiro Nakamura ◽  
Yoshihide Sunada
Keyword(s):  
Gene Therapy ◽  
Vascular Endothelial Growth Factor ◽  
Growth Factor ◽  
Mouse Model ◽  
Functional Outcome ◽  
Vascular Endothelial ◽  
Endothelial Growth Factor

scholarly journals Gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 2

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Xuewen Wu ◽  
Li Zhang ◽  
Yihui Li ◽  
Wenjuan Zhang ◽  
Jianjun Wang ◽  
...  
Keyword(s):  
Gene Therapy ◽  
Mouse Model ◽  
Inner Ear ◽  
Viral Vectors ◽  
Survival Rates ◽  
Semicircular Canals ◽  
Dependent Manner ◽  
Syndrome Type ◽  
Dose Dependent

AbstractMutations in voltage-gated potassium channel KCNE1 cause Jervell and Lange-Nielsen syndrome type 2 (JLNS2), resulting in congenital deafness and vestibular dysfunction. We conducted gene therapy by injecting viral vectors using the canalostomy approach in Kcne1−/− mice to treat both the hearing and vestibular symptoms. Results showed early treatment prevented collapse of the Reissner’s membrane and vestibular wall, retained the normal size of the semicircular canals, and prevented the degeneration of inner ear cells. In a dose-dependent manner, the treatment preserved auditory (16 out of 20 mice) and vestibular (20/20) functions in mice treated with the high-dosage for at least five months. In the low-dosage group, a subgroup of mice (13/20) showed improvements only in the vestibular functions. Results supported that highly efficient transduction is one of the key factors for achieving the efficacy and maintaining the long-term therapeutic effect. Secondary outcomes of treatment included improved birth and litter survival rates. Our results demonstrated that gene therapy via the canalostomy approach, which has been considered to be one of the more feasible delivery methods for human inner ear gene therapy, preserved auditory and vestibular functions in a dose-dependent manner in a mouse model of JLNS2.

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