1. Transcriptional targeting of adult mammalian cochlear supporting cells: We have validated new adult cochlear supporting cell-specific genes in both adult mouse and human temporal bone tissue. We have developed new targeting vectors driven by adult supporting cell promoters and are assaying their effectiveness in vivo for targeting adult cochlear supporting cells. 2. Systems approach to understanding the development, structure and function of the stria vascularis: Our initial goal is to generate transcriptome profiles for isolated strial cells at specific ages relevant to the endocochlear potential. We have accumulated single cell transcriptomes from adult stria vascularis and are adapting nucleus isolation methods for single cell RNA-sequencing for the stria vascularis. We are in the process of accumulating transcriptomes from the stria vascularis and lateral wall during the period of endocochlear potential development. We are investigating the potential of novel imaging modalities to characterize the cell surface interactions between marginal and intermediate cells in the stria vascularis. We are in the process of validating new transgenic cell type-specific fluorescent reporter lines for stria vascularis cell types.

Project Start
Project End
Budget Start
Budget End
Support Year
3
Fiscal Year
2017
Total Cost
Indirect Cost
Name
Deafness & Other Communication Disorders
Department
Type
DUNS #
City
State
Country
Zip Code
Sharlin, David S; Ng, Lily; Verrey, François et al. (2018) Deafness and loss of cochlear hair cells in the absence of thyroid hormone transporters Slc16a2 (Mct8) and Slc16a10 (Mct10). Sci Rep 8:4403
Imtiaz, Ayesha; Belyantseva, Inna A; Beirl, Alisha J et al. (2018) CDC14A phosphatase is essential for hearing and male fertility in mouse and human. Hum Mol Genet 27:780-798
Honda, Keiji; Kim, Sung Huhn; Kelly, Michael C et al. (2017) Molecular architecture underlying fluid absorption by the developing inner ear. Elife 6:
Nakanishi, Hiroshi; Kawashima, Yoshiyuki; Kurima, Kiyoto et al. (2017) NLRP3 mutation and cochlear autoinflammation cause syndromic and nonsyndromic hearing loss DFNA34 responsive to anakinra therapy. Proc Natl Acad Sci U S A 114:E7766-E7775
Burns, Joseph C; Kelly, Michael C; Hoa, Michael et al. (2015) Single-cell RNA-Seq resolves cellular complexity in sensory organs from the neonatal inner ear. Nat Commun 6:8557
Hoa, Michael; Friedman, Rick A; Fisher, Laurel M et al. (2015) Prognostic implications of and audiometric evidence for hearing fluctuation in Meniere's disease. Laryngoscope :
Dhillon, Vaninder K; Hoa, Michael; Winter, Margaret et al. (2014) Extrusion of a cochlear implant positioner through the tympanic membrane in a pediatric patient: management of a delayed complication. Ann Otol Rhinol Laryngol 123:537-40
Hoa, Michael; Wilkinson, Eric P; Slattery 3rd, William H (2014) Delayed recovery of speech discrimination after fractionated stereotactic radiotherapy for vestibular schwannoma in neurofibromatosis 2. Ear Nose Throat J 93:E20-2