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 RFA-DC-11-003 that is meant to establish the NIDCD Otopathology Research Collaboration Network. Our goals include the following: 1. Investigate pathology of hearing loss caused by cochleovestibular schwannoma. 2. Investigate radiologic-histologic correlations in otosclerosis, and the pathogenetic mechanism of sensorineural hearing loss when otosclerosis involves the cochlea. 3. Investigate dynamics of sensory and non-sensory cell turnover in the human inner ear. 4. Establish a program for the training of future temporal bone researchers. 5. Cooperate with the NIDCD Otopathology Research Collaboration Network. The proposed research is designed to provide new knowledge and insight into the pathology and pathophysiology of otosclerosis, acoustic tumors and cell turnover in the inner ear, which, in turn, should lead to better methods of diagnosis and therapy.

Public Health Relevance

We are proposing to use light microscopy and tools of molecular medicine to conduct research into the pathology and pathogenesis of common disorders of the ear (otosclerosis, acoustic tumors and cell turnover in the inner ear), which, in turn, 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 #
5U24DC011943-03
Application #
8519980
Study Section
Special Emphasis Panel (ZDC1-SRB-R (32))
Program Officer
Cyr, Janet
Project Start
2011-09-01
Project End
2016-08-31
Budget Start
2013-09-01
Budget End
2014-08-31
Support Year
3
Fiscal Year
2013
Total Cost
$522,025
Indirect Cost
$189,525
Name
Massachusetts Eye and Ear Infirmary
Department
Type
DUNS #
073825945
City
Boston
State
MA
Country
United States
Zip Code
02114
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Totonchy, Mariam B; Tamura, Deborah; Pantell, Matthew S et al. (2013) Auditory analysis of xeroderma pigmentosum 1971-2012: hearing function, sun sensitivity and DNA repair predict neurological degeneration. Brain 136:194-208
Quesnel, Alicia M; Moonis, Gul; Appel, Jason et al. (2013) Response to letter to the editor by Dr. Tamas Karosi Re: "Correlation of computed tomography with histopathology in otosclerosis", Quesnel et al. Otol Neurotol 2013; 34(1):22-28. Otol Neurotol 34:1363-4
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Salviz, Mehti; Montoya, Jose G; Nadol, Joseph B et al. (2013) Otopathology in congenital toxoplasmosis. Otol Neurotol 34:1165-9
Viana, Lucas M; Seyyedi, Mohammad; Brewer, Carmen C et al. (2013) Histopathology of the inner ear in patients with xeroderma pigmentosum and neurologic degeneration. Otol Neurotol 34:1230-6
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
Friedmann, David R; Eubig, Jan; Winata, Leon S et al. (2012) A clinical and histopathologic study of jugular bulb abnormalities. Arch Otolaryngol Head Neck Surg 138:66-71
Roosli, Christof; Linthicum Jr, Fred H; Cureoglu, Sebahattin et al. (2012) What is the site of origin of cochleovestibular schwannomas? Audiol Neurootol 17:121-5
Roosli, Christof; Linthicum Jr, Fred H; Cureoglu, Sebahattin et al. (2012) Dysfunction of the cochlea contributing to hearing loss in acoustic neuromas: an underappreciated entity. Otol Neurotol 33:473-80

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