A comprehensive program is proposed to facilitate recruitment of human temporal bone donors, accrual and distribution of donated specimens to active laboratories, brokering of special needs of research investigators using human otologic material, educational efforts including short-term training opportunities in Otopathology for the profession and providing educational outreach to the public and interested professionals including scientists and organ procurement specialists and undertakers. This will be accomplished through the development and maintenance of a coordinated Temporal Bone Registry Office. The proposed Registry function will be overseen by Joseph B. Nadol, Jr., M.D. and M. Charles Liberman, Ph.D., (Co-Principal Investigators). Co- Investigators who will help to supervise the activities of the Registry will include a list of individuals with experience and professional interest in furtherance of human temporal bone histopathology. These will include Michael J. McKenna, MD, Steven D. Rauch, MD, Joe C. Adams, Ph.D., Alicia Quesnel, MD, and Felipe Santos, MD. Additional oversight will 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.

Public Health Relevance

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 are incompletely understood in part due to deficient pathologic information. The National Temporal Bone Hearing and Balance Pathology Resource Registry is proposed in order 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 conventional microscopy or more modern techniques such as the application of molecular genetics and proteomic methodology.

National Institute of Health (NIH)
National Institute on Deafness and Other Communication Disorders (NIDCD)
Resource-Related Research Projects--Cooperative Agreements (U24)
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Special Emphasis Panel (ZDC1)
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Cyr, Janet
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Massachusetts Eye and Ear Infirmary
United States
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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
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
Seyyedi, Mohammad; Nadol Jr, Joseph B (2014) Intracochlear inflammatory response to cochlear implant electrodes in humans. Otol Neurotol 35:1545-51
Seyyedi, Mohammad; Viana, Lucas M; Nadol Jr, Joseph B (2014) Within-subject comparison of word recognition and spiral ganglion cell count in bilateral cochlear implant recipients. Otol Neurotol 35:1446-50
Nadol Jr, Joseph B; O'Malley, Jennifer T; Burgess, Barbara J et al. (2014) Cellular immunologic responses to cochlear implantation in the human. Hear Res 318:11-7