A comprehensive program is proposed to facilitate 1) recruitment of human temporal bone donors, 2) accrual and distribution of donated specimens to active laboratories, 3) development and maintenance of searchable de-identified databases including diagnoses, histopathological summaries, audiometric data and complete sets of digitized images, 4) brokering of special needs of research investigators using human otologic material, and 5) educational efforts including educational outreach to the public and interested professionals including scientists and organ procurement specialists and undertakers. As has been the case for the last 26 years, this will be accomplished through the maintenance and enhancement of a coordinated Temporal Bone Registry Office. The proposed Registry function will be overseen by Felipe Santos, M.D. (Principal Investigator) with assistance from Alicia Quesnel, M.D. (Co-Prinicpal Investigator) and M. Charles Liberman, Ph.D. (Co- Principal Investigator). Additional input will be solicited from other Departmental members with experience and professional interest in furtherance of human temporal bone histopathology, including D. Bradley Welling, M.D., Ph.D and Joseph B. Nadol, M.D. Additional oversight will be provided by the NIDCD Program Coordinator for the Registry who will help in the selection of a Steering Committee including the PI of the Registry, the Project Coordinator and 2 to 3 external scientists with an understanding and appreciation of the value of otopathology and basic otologic research.
Pathologic study of disease processes and evaluation of treatment modalities are critical aspects of the scientific basis of medicine. Many disorders of hearing, balance and facial nerve function are incompletely understood in part due to deficient pathologic information. The ongoing mission of the National Temporal Bone Hearing and Balance Pathology Resource Registry is to facilitate information sharing, solicitation of human temporal bone donors during life, the procurement of specimens as soon as possible after death and coordination of the study of these specimens either by 1) histological methods such as conventional microscopy or more modern techniques such as multi-label immunofluorescence confocal microscopy, 2) gene expression approaches such as molecular genetic and proteomic methodologies, or 3) imaging approaches such as advanced microstructural MRI or synchrotron radiation imaging.
|Okayasu, Tadao; O'Malley, Jennifer T; Nadol Jr, Joseph B (2018) Focal Degeneration of Vestibular Neuroepithelium in the Cristae Ampullares of Three Human Subjects. Otol Neurotol 39:e1100-e1110|
|Handzel, Ophir; Nadol Jr, Joseph B (2018) Temporal Bone Histopathology in Cockayne Syndrome. Otol Neurotol 39:e387-e391|
|Trakimas, Danielle R; Kozin, Elliott D; Ghanad, Iman et al. (2018) Human Otopathologic Findings in Cases of Folded Cochlear Implant Electrodes. Otol Neurotol 39:970-978|
|Kamakura, Takefumi; O'Malley, Jennifer T; Nadol Jr, Joseph B (2018) Preservation of Cells of the Organ of Corti and Innervating Dendritic Processes Following Cochlear Implantation in the Human: An Immunohistochemical Study. Otol Neurotol 39:284-293|
|Sagers, Jessica E; Landegger, Lukas D; Worthington, Steven et al. (2017) Human Cochlear Histopathology Reflects Clinical Signatures of Primary Neural Degeneration. Sci Rep 7:4884|
|O'Malley, Jennifer T; Burgess, Barbara J; Galler, Donald et al. (2017) Foreign Body Response to Silicone in Cochlear Implant Electrodes in the Human. Otol Neurotol 38:970-977|
|Ishai, Reuven; Herrmann, Barbara S; Nadol Jr, Joseph B et al. (2017) The pattern and degree of capsular fibrous sheaths surrounding cochlear electrode arrays. Hear Res 348:44-53|
|Nadol Jr, Joseph B (2016) Focal Endolymphatic Hydrops as Seen in the Pars Inferior of the Human Inner Ear. Otol Neurotol 37:859-64|
|Quesnel, Alicia M; Nakajima, Hideko Heidi; Rosowski, John J et al. (2016) Delayed loss of hearing after hearing preservation cochlear implantation: Human temporal bone pathology and implications for etiology. Hear Res 333:225-234|
|Jung, David H; Nadol Jr, Joseph B; Folkerth, Rebecca D et al. (2016) Histopathology of the Inner Ear in a Case With Recent Onset of Cogan's Syndrome: Evidence for Vasculitis. Ann Otol Rhinol Laryngol 125:20-4|
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