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Auditory Development and Restoration Program
Michael Hoa, M.D., Otolaryngology Surgeon-Scientist
The Auditory Development and Restoration Program investigates the development and function of adult cochlear cell types and the pathophysiological processes that result in their structural and functional degeneration. The long-term goal of the laboratory is to establish a basis for hearing restoration in the adult inner ear.
The endocochlear potential (EP) is essential for proper hair cell function and hearing. The structure and function of the lateral wall of the cochlea is central to the generation of this endocochlear potential. With this in mind, the lab seeks to understand (1) the genetic mechanisms responsible for generation of the endocochlear potential and the necessary cell-type specific context of these genetic mechanisms in the stria vascularis; and (2) the mechanisms responsible for EP degeneration. Pathophysiology of the lateral wall has been implicated in age-related hearing loss (presbycusis), sudden sensorineural hearing loss, Ménière's disease, DFN3, and a diverse spectrum of inherited disorders, including Jervell and Lange-Nielson syndrome, Bartter syndrome, SeSAME syndrome, and Waardenburg syndrome. Ultimately, the lab’s research seeks to improve our understanding of the development and function of the stria vascularis. Investigations may provide insight into hearing loss related to pathology in the cochlear lateral wall, as well as potential avenues for therapeutic targeting of this pathology.
The model system used to study the stria vascularis in the cochlear lateral wall is the mouse. The stria vascularis is composed of 3 cell layers consisting of marginal cells on the luminal surface, intermediate cells in central layer, and basal cells in the basolateral layer. The intermediate cell is fundamental to the generation of the EP, but it is the close interaction between the 3 cell types that results in a fully functional organ. To investigate these interactions, the lab uses a combination of single-cell mRNA-sequencing, bioinformatics, fluorescent-activated cell sorting (FACS), adult and perinatal immunohistochemistry, confocal microscopy, animal auditory function testing, pharmacology, and molecular biology techniques.
- Nakanishi N, Kawashima Y, Kurima K, Chae JJ, Ross AM, Pinto-Patarroyo G, Patel SK, Muskett JA, Ratay JS, Chattaraj P, Park YH, Grevich S, Brewer CC, Hoa M, Kim HJ, Butman JA, Broderick L, Hoffman HM, Aksentijevich I, Kastner DL, Goldbach-Mansky R, Griffith AJ. NLRP3 mutation and cochlear autoinflammation cause syndromic and nonsyndromic hearing loss DFNA34 responsive to anakinra therapy. Proc Natl Acad Sci USA 2017;114(37):E7766-E7775.
- Hoa M, Friedman RA, Fisher LM, Derebery MJ. Prognostic implications of and audiometric evidence for hearing fluctuation in Meniere’s disease. Laryngoscope 2015 Sep 7. doi: 10.1002/lary.25579.[Epub ahead of print]
- Burns JC, Kelly MC, Hoa M, Morell RJ, Kelley MW. Single-cell RNA-Seq resolves cellular complexity in sensory organs from the neonatal inner ear. Nat Commun 2015;6:8557.
- Dhillon VK, Hoa M, Winter M, Wilkinson EP. Extrusion of a cochlear implant positioner through the tympanic membrane in a pediatric patient: Management of a delayed complication. Ann Otol Rhinol Laryngol 2014;123(8):537-540.
- Hoa M, Wilkinson EP, Slattery WH 3rd. Delayed recovery of speech discrimination after fractionated stereotactic radiotherapy for vestibular schwannoma in neurofibromatosis 2. Ear Nose Throat J 2014;93(2):E20-2.
- Hoa M, Linthicum FH Jr. Spiral ganglion deficiency in adult-onset deafness-dystonia syndrome. Otol Neurotol 2013;34(9):e130-1. doi: 10.1097/MAO.0b013e3182a09b3b
- Hoa M, House JW, Linthicum FH Jr., Go JL. Petrous apex cholesterol granuloma: Pictorial review of radiologic considerations in diagnosis and surgical histopathology. J Laryngol Otol 2013; 127(4):339-48.
- Friedman RA, Hoa M, Brackmann DE. Surgical management of endolymphatic sac tumors. J Neurol Surg B 2013; 74(01):012-019 DOI: 10.1055/s-0032-1329622
- Hoa M, Drazin D, Hanna G, Schwartz M, Lekovic GP. The approach to the patient with incidentally diagnosed vestibular schwannoma. Neurosurg Focus 2012; 33(3):E2.
- Hoa M, House JW, Linthicum FH Jr. Petrous apex cholesterol granuloma: Maintenance of the drainage pathway, the histopathology of surgical management, and histopathologic evidence for the exposed marrow theory. Otol Neurotol 2012; 33(6):1059-65.
- Hoa M, Slattery WH 3rd. Update on neurofibromatosis 2. Otolaryngol Clin N Am 2012; 45(2):315-32, viii.
- Wilkinson EP, Hoa M, Slattery WH 3rd, Fayad JN, Friedman RA, Brackmann DE. Evolution in the management of facial nerve schwannoma. Laryngoscope 2011; 121(10):2065-74.
- Slattery WH, Hoa M, Bonne N, Friedman RA, Schwartz MS, Fisher L, Brackman DE. Middle fossa decompression for hearing preservation: A review of institutional results and indications. Otol Neurotol 2011; 32(6):1017-24.
- Hoa M, Syamal M, Schaeffer MA, Sachdeva L, Berk R, Coticchia J. Biofilms and chronic otitis media: An initial exploration into the role of biofilms in the pathogenesis of chronic otitis media. Am J Otolaryngol 2010; 31(4):241-5.