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Grant funding is helping Boys Town National Research Hospital research scientists study the causes of hearing loss and other communication disorders and develop treatment and intervention methods in 20 state-of-the-art laboratories.

Current External Support

INDIVIDUAL GRANTS
Sudhakar Akulapalli, PhD Flight Attendant Medical Research Institute (FAMRI)/062558_YCSA; Regulation of Tumor Angiogenesis by Combostatin; This proposal is specifically aimed at identifying the signaling mechanisms and associated effector pathways for combostatin, which is derived from the active regions of alpha1, alpha3(IV)NC1 and endostatin. Successful completion of the proposed work will provide valuable insights that may facilitate the development of new anti-tumor therapies by a targeted approach based on the hypoxia/COX-2 induced signaling mechanisms.
Catherine C. Carotta, PhD NE Department of Education/99-2814-248-1D6-08; Interpreter, Educator and Parent Training; Training designed to meet the needs of interpreters, educators and parents of children with hearing loss.
Dominic E. Cosgrove, PhD Novartis; Follow-up Efficacy Study of Novartis' Compound LBR645 in Alport's Disease Mouse; The goal of this set of experiments is to further the investigation of LBR645 in a mouse model of Alport Disease.

NIH-NIDDK/5 R01 DK055000-08; Molecular Aspects of Alport Renal Disease Progression; This proposal is a focused approach aimed at defining specific aspects that may contribute towards mechanisms underlying glomerular pathogenesis.

NIH-NIDCD/2 R01 DC006442-04 (Subcontract with the University of Pennsylvania, Gratton, PI); Matrix Otopathology; The proposed work clarifies the relationship of basement membrane to strial function and normal hearing.

NIH-NIDCD/2 R01 DC004844-06; Usherin: Structural and Functional Analysis; The goal of this proposal is to define the common functional role of the Usher protein complex in cochlear hair cells and retinal photoreceptors. Emphasis is aimed at the possible functional mechanisms underlying defects in regulated vesicle trafficking of protein cargo at the apical aspect of these cells.
Michael P. Gorga, PhD NIH-NIDCD/5 R01 DC02251-13; Cochlear Nonlinearity and Auditory Function in Humans; The goal of this research program is to use noninvasive techniques to gain a better understanding of cochlear nonlinearity in humans with normal hearing and with hearing loss.

NIH-NIDCD/2 R13 DC006616-05; Building the Next Generation of Clinical Researchers-AAS; This NIH conference-grant support has enabled the American Auditory Society (AAS) to encourage the next generation of clinical researchers by providing a forum for students and resident physicians to present their research, and to receive feedback and constructive criticism from senior members of both the basic-science and clinical-research communities.
 
Walt Jesteadt, PhD NIH-NIDCD/5 R01 DC006648-04; Decision Processes in Detection and Discrimination; The goal of this research program is to develop a better understanding of fundamental aspects of human hearing by characterizing differences in decision processes for three tasks that use the same basic stimuli in different temporal configurations: intensity discrimination, increment detection, and forward masking.

NIH-NIDCD/5 T32 DC000013-28; Research in Human Communication and Its Disorders; This grant provides institutional training support for three postdoctoral fellows.

NIH-NIDCD/1 T35 DC008757-02; Short-Term Research Training for AuD Students; The goals of the Short-Term Training Program for AuD students at BTNRH is to give 4 AuD students per year the opportunity to pursue a full-time hands-on clinical or translational research experience in the hearing sciences over a 3-month period.
Douglas H. Keefe, PhD NIH-NIDCD/5 R01 DC003784-10; Acoustic Responses of the Human Cochlea and Middle Ear; The primary goal of this application is to use a combination of acoustic and behavioral responses to test theories of auditory processing at the mechanical and behavioral levels. A subordinate goal is to relate acoustic responses measured non-invasively in the ear canal to cochlear and middle-ear function.
William J. Kimberling, PhD FFB/BRGE06060343-BTNRH; Medical and Population Genetic Studies of Usher Syndrome; It is the objective of this proposal to build the infrastructure for genetics-epidemiologic studies and for clinical trials by improving diagnosis, establishing an Usher syndrome working group, and developing an Usher syndrome database for mutations and cases that would facilitate future studies.

Morris J. & Betty Kaplun Foundation, Inc.; Usher Syndrome; The specific aim of this project is to search for the Usher III gene.

Hear See Hope; Feasibility for Early Diagnosis of Usher Syndrome; The aim of the project is to conduct a pilot study to screen 150-200 deaf and hard of hearing students in the State of Oregon .  The ultimate objective is to develop a national screening program that will detect 50-100 new cases of Usher syndrome each year.  In addition to providing families and school systems with vital information regarding a child's health and educational needs, the program will also identify children as potential candidates for clinical treatment trials.

HRSA/MCHB U22MC03962 (Subcontract with the University of Oklahoma Health Science Center); Usher Syndrome in Children with Cochlear Implants; This small project program is part of the Heartland Genetics and Newborn Screening Collaborative.  This project proposes a pilot study to determine the frequency of Usher syndrome in 100 implant recipients from Nebraska and Iowa .

NIH-NICDC/2 R01 DC003544 (Subcontract with the University of Iowa, Smith, PI); Autosomal Dominant Non-Sydromic Hearing Loss; The aims of this study are 1) to continue to localize and clone genes that cause ADNSHL; 2) to develop audioprofiling as a method to prioritize genes for mutation screening in families segregating ADNSHL but with insufficient number of informative meioses for linkage analysis; 3) to test the efficacy of RNA interference as a potential therapy in modifying the hearing loss phenotype in a ADNSHL caused by dominant-negative mechanism of action.
Dawna Lewis,
PhD		 NIH-NIDCD/1 R03 DC009675-01; The Impact of Minimal Hearing Loss on Functional Auditory Skills in Children; This research will enhance our understanding of factors that affect auditory perception, speech, language, and learning in children with minimal hearing loss. At present there is no consensus on the difficulties experienced by this population, and, as a group, they are probably underserved. The results of studies will lead to greater clarity about the auditory and educational needs of this population.

Yesha Lundberg, PhD NIH-NIDCD/1 R01 DC008603-02; Otoconia Development and Maintenance; This proposal is designed to perform in-depth quantitative expression studies and protein biochemical analyses to address the roles of Oc90 and otolin in providing optimal otoconia calcification and begin to identify factors critical for the spatial specific development of otoconia.

NIH-NIAMS/1 P50 AR055081-01 (Subcontract U of Mo, Kanas City), Genomic Convergence for Female Osteoporosis Risk Genes, The goal of this project is to identify genes that are differentially expressed in females low vs. high bone density with menopausal status changes.
Mary Pat Moeller, PhD NIH-NIDCD/5 R01 DC006681-04; Word Learning in Infants with Hearing Loss; The overall goal of this project is to gain an understanding of the early word-learning processes of children with hearing loss, and to delineate factors that influence language development.
Barbara J.
Morley, PhD		 NIH-NIDCD/5 R01 DC006907-03; Transient Expression of Cholinergic Markers in the PVCN; The goal of the proposed studies is to elucidate the cholinergic molecular mechanisms related to the maturation of synapses and regulation of NMDA receptor gene expression in the rodent PVCN.
Stephen T. Neely, DSc NIH-NIDCD/1 R01 DC008318-02; Cochlear Mechanics, Wave Propagation, and Compression; This research program focuses on theoretical and empirical studies that address current issues in cochlear mechanics relevant to noninvasive evaluation of cochlear function. The goal of the proposed research is the development of a computational model of cochlear mechanics that will facilitate the interpretation of noninvasive measures of peripheral auditory status in humans.
Donna L. Neff, PhD NOHR, Predicting Listening Strategies with Cochlear Implants, This protocol examines whether the performance of adult CI users on a level-discrimination task using multi-tonal complexes is similar to that for NH listeners and can be predicted by the relative level and information presented to particular frequency regions.
Kanae Nishi, PhD NIH-NIDCD/1 R03 DC009334-01A1; Auditory Training for Persons with Difficulty Understanding Speech in Noise; The ultimate goal of this research project is to develop a protocol for the assessment and training of speech perception for individuals who experience marked difficulty understanding speech in noise. The efficacy of a novel auditory training protocol will be evaluated in two groups of individuals who are know to have difficulty understanding speech in noise: English speaking children with auditory processing disorders and/or language-based learning disabilities and adult L2 speakers of English.
You-Wei Peng, PhD NIH-NCRR/5 P20 RR018788-05 (Subcontract with the University of Nebraska Medical Center (UNMC), Smith, PI); COBRE Project - The Molecular Biology of Neurosensory Systems; The central hypothesis is that usherin-mediated signaling though alpha1beta1 integrin on RPE cells is required to regulate normal basement membrane metabolism, and that absence of this interaction is the underlying cause for USH2a retinal pathogenesis. We will test this hypothesis by characterizing usherin mediated signaling and downstream modulation of genes involved in modulating basement membrane metabolism using RPE cell cultures from wild type and integrin alpha1 knockout mice.
Patricia G. Stelmachowicz, PhD NIH-NIDCD/5 R01 DC004300-09; Optimizing Amplification for Infants and Young Children; The overall goal of this project is to explore ways in which to enhance auditory access and auditory experiences in young children with hearing loss.
Edward J. Walsh, PhD NIH-NEI/5 R01 EY016247-03 (Subcontract with the University of Texas SW , White, PI) Functions of Very Large G-Protein Coupled Receptor-1; This project investigates structural abnormalities in the cochleae of mice lacking the gene for the very large G-protein coupled receptor.

NIH-NCRR/5 P20 RR018788-05 (Subcontract with the University of Nebraska Medical Center (UNMC), Smith, PI) The Molecular Biology of Neurosensory Systems--Core A: Administration; The goal is to coordinate administration of the COBRE grant by UNMC, BTNRH and Creighton University .

NOHR, Age-Related Hearing Loss and Hypothyroidism, The long term goal of the proposed plan of study is to determine if and how ARHL is influenced by hypothyroidism and to identify interactions between hypothyroidism and noise exposure as factors that affect the disposition of the condition, if such an interaction exists.
CORE CENTER GRANT
Walt Jesteadt, PhD NIH-NIDCD/2 P30 DC04662-07; Core Center --Administration; The Core Center meets current and anticipated needs in the areas:  (1) Laboratory Computing; and (2) Recruitment of Human Research Subjects.
Stephen T. Neely, DSc NIH-NIDCD/2 P30 DC04662-07; Core Center --Core A: Laboratory Computing; The purpose of this core is to accelerate ongoing research at BTNRH by providing troubleshooting, development, maintenance, and consulting services related to laboratory hardware and software. This core allows us to maintain a higher level of laboratory hardware and software expertise than is possible in individual laboratories. The immediate availability of this expertise allows investigators to focus more on scientific issues and pursue their research projects more efficiently.
Michael P. Gorga, PhD NIH-NIDCD/2 P30 DC04662-07; Core Center--Core C: Human Subject Recruitment; The goals for the Human Research Subjects Core (HRSC) are to support the research process by providing access to a large pool of individuals who are interested in participating in research.