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Norrie disease is caused by mutation of the NDP gene, presenting as congenital blindness followed by later onset of hearing loss. Protecting patients from hearing loss is critical for maintaining their quality of life. This study aimed to understand the onset of pathology in cochlear structure and function. By investigating patients and juvenile Ndp-mutant mice, we elucidated the sequence of onset of physiological changes (in auditory brainstem responses, distortion product otoacoustic emissions, endocochlear potential, blood-labyrinth barrier integrity) and determined the cellular, histological, and ultrastructural events leading to hearing loss. We found that cochlear vascular pathology occurs earlier than previously reported and precedes sensorineural hearing loss. The work defines a disease mechanism whereby early malformation of the cochlear microvasculature precedes loss of vessel integrity and decline of endocochlear potential, leading to hearing loss and hair cell death while sparing spiral ganglion cells. This provides essential information on events defining the optimal therapeutic window and indicates that early intervention is needed. In an era of advancing gene therapy and small-molecule technologies, this study establishes Ndp-mutant mice as a platform to test such interventions and has important implications for understanding the progression of hearing loss in Norrie disease.

Each chapter in Volume I is devoted to a primary activity in the procurement process. The chapters are organized in the same sequence as the activities which occur in the building procurement process: Chapter 1: Feasibility, provides guidelines on whether a project should be executed through conventional means or by using industrialized building methods; Chapter 2: Programming, describes how to define and schedule the work; Chapter 3: Design and Documentation, directs specification writing and the defining of interfaces between building and site; Chapter 4: Proposal and Evaluation, provides methods for selecting the best response from industry and for awarding the contract; and Chapter 5: Construction Administration, defines the construction documents which are required of the contractor, as well as the quality control.

INTERACTIVE COMPUTER GRAPHICS can reduce the time and cost of any design process by at least one-third. To realize this great reduction, interactive computer graphics programs will have to be developed at every design level. SEARCH is an automated architectural criteria maintenance and design evaluation system. SEARCH is used in two phases of Corps design work. CORPS, the Conversationally Oriented Real-Time Program-Generating System, provides computational support for engineering planners, designers, and managers who require a responsive computer resource, but do not choose to be computer programmers. This paper gives (1) guidelines for programs incorporated in CORPS, (2) the run sequence for using CORPS, (3) an example problem showing the use of one program, (4) a list of the programs presently in CORPS at WES, (5) a list of the programs being added to the CORPS library, and (6) excerpts from an article by Robert L. Renner outlining an early conception of CORPS., Proceedings of Conference held 22-26 Sep 75, New Orleans, La. See also Volume 1, AD-A031 243.

This report presents results of the monitorship of the construction and design of a demonstration project of industrialized building applied to Department of the Army construction requirements. The project was a 250-man bachelor officers' quarters (BOQ) at Fort Knox, KY, constructed by FCE-Dillon, Akron, OH, using their system of precast concrete panels and three-dimensional kitchen/bath modules. The experiences in design and production, one-step procurement, construction, and contract administration at Fort Knox are analyzed, and the cost per man housed is compared for BOQs in the FY 73 MCA program. (Author)