1. Gene therapy for genetic hearing loss: we have been working on methods of delivering gene therapy to the cochlea. This includes the round window and cochleostomy approaches. In addition, 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. Stem cell therapy: we are working on differentiating human embryonic stem cells into hair cell-like cells and spiral ganglion-like cells in vitro. We are transplanting these cells to mouse cochlear explants to study stem cell integration. We will be transplanting human stem cell-derived otic precursors to mouse models of hearing loss to see if hearing can be restored.

Project Start
Project End
Budget Start
Budget End
Support Year
2
Fiscal Year
2013
Total Cost
$449,685
Indirect Cost
Name
National Institute on Deafness and Other Communication Disorders
Department
Type
DUNS #
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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; Isgrig, Kevin; Roy, Soumen et al. (2015) Gene therapy restores hair cell stereocilia morphology in inner ears of deaf whirler mice. Mol Ther :
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
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 :
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
Chien, Wade W; Monzack, Elyssa L; McDougald, Devin S et al. (2014) Gene Therapy for Sensorineural Hearing Loss. Ear Hear :
Gurbani, Saumya S; Wilkening, Paul; Zhao, Mingtao et al. (2014) Robot-assisted three-dimensional registration for cochlear implant surgery using a common-path swept-source optical coherence tomography probe. J Biomed Opt 19:057004
Chien, Wade Wei-De; Leiding, Jennifer W; Hsu, Amy P et al. (2014) Auditory and vestibular phenotypes associated with GATA3 mutation. Otol Neurotol 35:577-81

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