A knowledge of the pathologic basis of disease is central to the study of medicine, including disorders affecting the auditory and vestibular systems. Otology is unique in that the inner ear is inaccessible during life and so, conventional techniques of pathologic study such as biopsy and surgical excision are not feasible. Consequently, insight into the pathologic basis of ear disease can only be obtained by postmortem study of temporal bones and by developing credible animal models. An improved understanding of the pathology and pathogenesis of disorders affecting the auditory and vestibular systems should lead to more rational diagnosis and clinical management of these diseases. The procurement, processing and study of human temporal bones is a time-consuming and costly endeavor which lies outside the purview of clinical departments of pathology, and is therefore a research endeavor that is performed in temporal bone laboratories. This proposal was developed in keeping with the guidelines announced in the RFA (DC-06-001) that is meant to establish the Human Temporal Bone Consortium for Research Resource Enhancement. Our goals include the following: 1. To develop customized protocols for assessment of prospectively acquired temporal bones. 2. To archive in digital format the histopathology reports and images for all temporal bones in order to enhance the research and educational value of archival and newly acquired specimens. 3. To develop improved protocols for applying molecular biologic assays to temporal bone research, especially protocols that will optimize the applicability of immunostaining and mass spectrometry- based proteomics while preserving the morphology of the processed tissues. 4. To collect comprehensive clinical information from registered temporal bone donors. 5. To establish a program for the training of future temporal bone researchers. 6. To cooperate with the Temporal Bone Research Consortium. This research will provide new knowledge about the causes of disorders of hearing and balance, which should lead to better methods of diagnosis and therapy. ? ? ?

Agency
National Institute of Health (NIH)
Institute
National Institute on Deafness and Other Communication Disorders (NIDCD)
Type
Resource-Related Research Projects--Cooperative Agreements (U24)
Project #
5U24DC008559-03
Application #
7477914
Study Section
Special Emphasis Panel (ZDC1-SRB-R (39))
Program Officer
Platt, Christopher
Project Start
2006-09-21
Project End
2011-08-31
Budget Start
2008-09-01
Budget End
2009-08-31
Support Year
3
Fiscal Year
2008
Total Cost
$593,188
Indirect Cost
Name
Massachusetts Eye and Ear Infirmary
Department
Type
DUNS #
073825945
City
Boston
State
MA
Country
United States
Zip Code
02114
Richard, CĂ©line; Doherty, Joni K; Fayad, Jose N et al. (2015) Identification of target proteins involved in cochlear otosclerosis. Otol Neurotol 36:923-31
Santos, Felipe; McCall, Andrew A; Chien, Wade et al. (2012) Otopathology in Osteogenesis Imperfecta. Otol Neurotol 33:1562-6
de Heer, Anne-Martine R; Merchant, Saumil N; Kammeraad, Janneke A E et al. (2012) Clinical follow-up and histopathology of the temporal bones in Nathalie syndrome. Audiol Neurootol 17:219-27
Teixido, Michael; Kung, Brian; Rosowski, John J et al. (2012) Histopathology of the temporal bone in a case of superior canal dehiscence syndrome. Ann Otol Rhinol Laryngol 121:7-12
Yildirim-Baylan, Muzeyyen; Cureoglu, Sebahattin; Paparella, Michael M (2012) Langerhans' cell histiocytosis of the temporal bone. Otol Neurotol 33:e15-6
Lysaght, Andrew C; Kao, Shyan-Yuan; Paulo, Joao A et al. (2011) Proteome of human perilymph. J Proteome Res 10:3845-51
Nam, Sung-Il; Linthicum Jr, Fred H; Merchant, Saumil N (2011) Temporal bone histopathology in neurofibromatosis type 2. Laryngoscope 121:1548-54
McCall, Andrew A; Linthicum Jr, Fred H; O'Malley, Jennifer T et al. (2011) Extralabyrinthine manifestations of DFNA9. J Assoc Res Otolaryngol 12:141-9
Yoda, Shigetoshi; Cureoglu, Sebahattin; Shimizu, Shigetaka et al. (2011) Round window membrane in Meniere's disease: a human temporal bone study. Otol Neurotol 32:147-51
Chung, Jong Woo; Fayad, Jose; Linthicum, Fred et al. (2011) Histopathology after endolymphatic sac surgery for Meniere's syndrome. Otol Neurotol 32:660-4

Showing the most recent 10 out of 40 publications