Abstract

1. Gene therapy for genetic hearing loss: we are working with a mouse model of genetic hearing and delivering functional copies of the gene of interest to the cochlea to see if the hearing can be restored. 2. Genome editing: we are investigating whether genome editing techonologies (ZFNs, TALENs, CRISPR/Cas) can be applied to the inner ear as a way of gene therapy to correct the underlying genetic mutation. 3. Inner gene delivery: we are in the process of examining ways of minimizing trauma to the inner ear during gene delivery surgery. We are examining different surgical approaches to access the inner ear and investigating the consequences of surgical manipulation to the inner ear.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute on Deafness and Other Communication Disorders (NIDCD)
Type
Investigator-Initiated Intramural Research Projects (ZIA)
Project #
1ZIADC000082-04
Application #
9147438
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
4
Fiscal Year
2015
Total Cost
Indirect Cost

  Institution

Name
Deafness & Other Communication Disorders
Department
Type
DUNS #
City
State
Country
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  Publications

Chien, Wade W (2018) A CRISPR Way to Restore Hearing. N Engl J Med 378:1255-1256
Isgrig, Kevin; Chien, Wade W (2018) Posterior Semicircular Canal Approach for Inner Ear Gene Delivery in Neonatal Mouse. J Vis Exp :
Rose, Jane; Muskett, Julie A; King, Kelly A et al. (2017) Hearing loss associated with enlarged vestibular aqueduct and zero or one mutant allele of SLC26A4. Laryngoscope 127:E238-E243
Isgrig, Kevin; Shteamer, Jack W; Belyantseva, Inna A et al. (2017) Gene Therapy Restores Balance and Auditory Functions in a Mouse Model of Usher Syndrome. Mol Ther 25:780-791
Muskett, Julie A; Chattaraj, Parna; Heneghan, John F et al. (2016) Atypical patterns of segregation of familial enlargement of the vestibular aqueduct. Laryngoscope 126:E240-7
Nieman, Carrie L; Weinreich, Heather M; Sharon, Jeffrey D et al. (2016) Use of Pericranial Flap Coverage in Cochlear Implantation of the Radical Cavity: Rationale, Technique, and Experience. Otolaryngol Head Neck Surg 155:533-7
Lawrence, Monica G; Leiding, Jennifer W; Lyons, Jonathan J et al. (2016) GATA3 haploinsufficiency does not block allergic sensitization or atopic disease. J Allergy Clin Immunol 137:627-629.e2
Chien, Wade W; McDougald, Devin S; Roy, Soumen et al. (2015) Cochlear gene transfer mediated by adeno-associated virus: Comparison of two surgical approaches. Laryngoscope :
Zalewski, Chris K; Chien, Wade W; King, Kelly A et al. (2015) Vestibular Dysfunction in Patients with Enlarged Vestibular Aqueduct. Otolaryngol Head Neck Surg 153:257-62
Ravicz, Michael E; Chien, Wade W; Rosowski, John J (2015) Restoration of middle-ear input in fluid-filled middle ears by controlled introduction of air or a novel air-filled implant. Hear Res 328:8-23

Showing the most recent 10 out of 27 publications