x-linked sensorineural hearing: Topics by WorldWideScience.org

X-linked nephrogenic diabetes insipidus mutations in North America and the Hopewell hypothesis.

Purpose: To determine the rankings of 6 input-output functions for understanding low-level, conversational, and high-level speech in multitalker babble without manipulating volume control for listeners with normal hearing, flat sensorineural hearing loss, and mildly sloping sensorineural hearing loss. Method: Peak clipping, compression limiting, and 4 wide dynamic range compression (WDRC) input-output functions were compared in a repeated-measure design. Interactions among the compression characteristics were minimized. Speech and babble were processed and recorded at 3 input levels: 45, 65, and 90 dB sound pressure level. Speech recognition of 3 groups of listeners (n = 6/group) was tested for speech processed by each input-output function and at each input level. Results: Input-output functions that made low-level speech audible and high-level speech less distorted by avoiding ...

2007-04-01

RISK-XLR: A Microcomputer-Based Genetic Risk Program for X-Linked Recessive Traits

In X-linked nephrogenic diabetes insipidus (NDI) the urine of male patients is not concentrated after the administration of the antidiuretic hormone arginine-vasopressin. This disease is due to mutations...Full Text Available

1993-09-01

Evaluating the benefit of hearing aids in solving the cocktail party problem

A computer program, RISK-XLR, which calculates genetic risk for carrier status of a Mendelian X-linked recessive condition has been written for the Macintosh series of microcomputers. The program,...Full Text Available

1987-11-04

Prestin and high frequency hearing in mammals

The benefit of wearing hearing aids in multitalker, reverberant listening environments was evaluated in a study of speech-on-speech masking with two groups of listeners with hearing loss (younger/older)....Full Text Available

2008-12-01

Digital Hearing Aids: Current "State-of-the-Art"

Recent evidence indicates that the evolution of ultrasonic hearing in echolocating bats and cetaceans has involved adaptive amino acid replacements in the cochlear gene prestin. A substantial...Full Text Available

2011-03-01

Analgesic Use and the Risk of Hearing Loss in Men

... promise of highly advanced signal processing, ensured that digital hearing aid technology had come of age. So how far have ...

Bone Anchored Hearing Treatment Procedure

BackgroundHearing loss is a common sensory disorder, yet prospective data on potentially modifiable risk factors are limited. Regularly used analgesics, the most...Full Text Available

2010-03-01

Medline Plus

... the top of the screen, acting as a hinge. Beneath that the soft tissue consisting of fat ...

The prevalence and correlates of self-reported hearing impairment in the Ibadan Study of Ageing

Mutations in TPRN Cause a Progressive Form of Autosomal-Recessive Nonsyndromic Hearing Loss

AbstractThis cohort study of 1302 persons aged ≥65 years, conducted in the Yoruba-speaking regions of Nigeria, determines the prevalence and correlates of hearing impairment (HI)...Full Text Available

2010-08-01

Audiometric notch as a sign of noise induced hearing loss

We performed genome-wide homozygosity mapping in a large consanguineous family from Morocco and mapped the autosomal-recessive nonsyndromic hearing loss (ARNSHL) in this family to the DFNB79...Full Text Available

2010-03-12

Hearing pathways and directional sensitivity of the beluga whale, Delphinapterus leucas

OBJECTIVES—To investigate the relation between different types of exposure to noise and a classic sign of noise induced hearing loss (NIHL), the audiometric notch. METHODS—The...Full Text Available

2001-01-01

British Library Electronic Table of Contents (United Kingdom)

Odontocetes are believed to receive sounds primarily through the pan bone region of the lower jaw although much variation in jaw morphology exists among species. In order to further examine this jaw hearing hypothesis we tested the head receiving sensitivity and directional hearing of a beluga whale, Delphinapterus leucas. Hearing thresholds were measured using auditory evoked potentials (AEPs). The subject proved to have highly directional hearing for far-field click stimuli similar to that of bottlenose dolphins and more directional than the harbor porpoise. For near-field jawphone stimulation, the beluga's lowest thresholds were found when click stimuli were presented at the rostrum tip (76?dB re: 1??Pa) although thresholds from the pan bone region stimulation were only 2?3?dB higher. S...

2008-01-01

Hearing loss in Turner syndrome

British Library Electronic Table of Contents (United Kingdom)

ObjectiveTo address the characteristics of hearing loss in patients with Turner syndrome (TS), we evaluated hearing levels of patients with TS and analyzed causative factors.Study designThirty-three patients with TS (8 to 40 years of age) were studied through the use of audiological measurements, and causative factors were explored.ResultsTwenty cases (35 of 66 ears tested) showed high-frequency (8 kHz) sensory neural hearing loss (HFQ-SNHL). Fifteen cases (26 ears) and 15 cases (24 ears) of the impaired 20 cases were unresponsive to distortion-product otoacoustic emissions and transient-evoked otoacoustic emissions, respectively. HFQ-SNHL showed little relation to the history of middle ear infection and puberty, although middle ear infections were seen in 11 of the 20 cases. The hearing t...

2006-01-01

Education qualification levels and school careers of unilateral versus bilateral hearing aid users

British Library Electronic Table of Contents (United Kingdom)

Objective: Analyse the difference in school careers and secondary school qualification levels between unilateral hearing aid users and bilateral hearing aid users. Study design: Retrospective questionnaire study. Setting: Postal-based questionnaire. Participants: Names of adults known to have been fitted with unilateral or bilateral hearing aids during childhood were retrieved. This resulted in 292 names. Participants were selected using the following criteria: availability of the medical record, presence of bilateral hearing loss, completed secondary school education, normal IQ and a minimum aided word-recognition score of 70% at 10 years of age. The questionnaire was sent to 50 potential participants of whom 40 responded, resulting in two groups comprising 19 unilateral and 21 bilateral ...

2007-01-01

The red-green visual pigment gene region in adrenoleukodystrophy.

Ichthyosis follicularis, alopecia, and photophobia (IFAP) syndrome

Although recent data established that a specific very-long-chain fatty acyl-CoA synthetase is defective in X-linked adrenoleukodystrophy (ALD), the ALD gene is still unidentified. The ALD locus has...Full Text Available

1990-03-01

Occurrence of hearing loss in a cohort of civilians employed at a US Navy industrial facility. Interim report

The IFAP syndrome is a rare X-linked genetic disorder reported in nearly 40 patients. It is characterized by the triad of Ichthyosis Follicularis, Alopecia, and Photophobia from birth. Other features...Full Text Available

Nonlinear time-domain modeling of balanced-armature receivers

Although hearing loss has been the focus of national surveys in the civilian population, these surveys typically do not include occupational exposure information. Furthermore, very few studies have addressed this problem in the military, particularly in industrial settings. Audiometric data, including hearing loss information, recorded and stored in the prototype application of the Navy's Occupational Health Information Management System (NOHIMS) has not been systematically evaluated to identify military and civilian populations that are at high risk for hearing loss. The objective of this study was to determine the prevalence of hearing loss in a cohort of Navy civilian workers employed at an industrialized facility. It is both appropriate and timely to look at hearing loss among civilian workers, as well as among the military, especially in relation to the recent ...

1986-12-18

DEFF Research Database (Denmark)

Nonlinear distortion added by the loudspeaker in a hearing aid lowers the signal-to-noise ratio and may degrade the hearing aid user's ability to understand speech. The balancedarmature- type loudspeakers, predominantly used in hearing aids, are inherently nonlinear devices, as any displacement of the loudspeaker diaphragm inevitably changes the magnetic and electrical characteristics of the loudspeaker. A numerical time-domain model capable of describing these nonlinearities is presented. By simulation it is demonstrated how the output distortion could potentially be reduced significantly through careful design of the mechanical properties of the armature.

2011-01-01

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notes4.htm - NASA's History Office

Technical Evaluation Report

Subcommittee of the Joint Committee on Atomic Energy of the United States, Outer Space Propulsion by Nuclear Energy, hearings, 85th Cong., 2d sess., 22, ...

NASA partners with teacher institute NASA strives to improve computers

... and/or hearing mechanisms. Seven papers dealt with 3D audio technology and/or its application. Eight papers presented ...

2005-04-01

NASA Project Inducted Into Space Technology Hall of

of the digital hearing aid technology that led to the cochlear implant. Former. Marshall Space Flight Center engineers. John Richardson and Joseph Howard ...

Apr 9, 2003 ... Adam Kissiah Jr., a retired KSC engineer, will be honored for his development of the digital hearing aid technology that led to the cochlear ...

Goddard Bible Club

BIBLIOGRAPHY OF TECHNICAL PUBLICATIONS AND ...

"Then Isaiah said, "Hear now, you house of David! Is it not enough to try the patience of men? Will you try the patience of my God also? ...

Extent and significance of the impact on reactor licensing of recent court decisions. Hearing before the Joint Committee on Atomic Energy, Congress of the United States, Ninety-Fourth Congress, Second Session, August 27, 1976

... (l0)6o). 316. Statf,'ent. In Hearings before the Subcommittee on Research, Do'v,-Iopment, and Radiation of the Joint Committee on Atomic Energy. ...

1967-07-01

Serum Globotriaosylceramide Assay as a Screening Test for Fabry Disease in Patients with ESRD on Maintenance Dialysis in Korea

A report is presented of a hearing conducted before the Joint Committee on Atomic Energy on August 27, 1976, to discuss the legal implications for reactor licensing resulting from court challenges to procedures for assessing the environmental impact of radioactive waste disposal. (DG)

1976-01-01

Disruption of the ATP-binding Cassette B7 (ABTM-1/ABCB7) Induces Oxidative Stress and Premature Cell Death in Caenorhabditis elegans*

Background/AimsFabry disease is an X-linked recessive and progressive disease caused by α-galactosidase A (α-GaL A) deficiency. We sought to assess the prevalence...Full Text Available

2010-12-01

Comparative analysis of the ATRX promoter and 5' regulatory region reveals conserved regulatory elements which are linked to roles in neurodevelopment, alpha-globin regulation and testicular function

X-linked sideroblastic anemia with ataxia (XLSA/A) is a rare inherited disorder characterized by mild anemia and ataxia. XLSA/A is caused by mutations in the ABCB7 gene, which encodes...Full Text Available

2011-06-17

The McGurk phenomenon in Italian listeners

BackgroundATRX is a tightly-regulated multifunctional protein with crucial roles in mammalian development. Mutations in the ATRX gene cause ATR-X syndrome, an X-linked...Full Text Available

Sequence variants of the DFNB31 gene among Usher syndrome patients of diverse origin

SummaryIn the classic example of the McGurk effect, when subjects see a speaker say /ga/ and hear a simultaneous /ba/, they typically perceive /da/, a syllable that was not presented...Full Text Available

2009-08-01

Role-Reversal Exercise with Deaf Strong Hospital to Teach Communication Competency and Cultural Awareness

PurposeIt has been demonstrated that mutations in deafness, autosomal recessive 31 (DFNB31), the gene encoding whirlin, is responsible for nonsyndromic hearing loss...Full Text Available

Gamma Knife radiosurgery for vestibular schwannoma: case report and review of the literature

ObjectiveTo implement a role-reversal exercise to increase first-year pharmacy students' awareness of communication barriers in the health care setting, especially for deaf and hard-of-hearing...Full Text Available

2011-04-11

Effects of Semantic Context and Feedback on Perceptual Learning of Speech Processed through an Acoustic Simulation of a Cochlear Implant

Vestibular schwannomas, also called acoustic neuromas, are benign tumors of the vestibulocochlear nerve. Patients with these tumours almost always present with signs of hearing loss, and many also experience...Full Text Available

Discrimination of Schroeder-Phase Harmonic Complexes by Normal-Hearing and Cochlear-Implant Listeners

The effect of feedback and materials on perceptual learning was examined in normal hearing listeners exposed to cochlear implant simulations. Generalization was most robust when feedback paired...Full Text Available

2010-02-01

Discrete molecular states in the brain accompany changing responses to a vocal signal

The temporal fine structure (TFS) of sound contributes significantly to the perception of music and speech in noise. The evaluation of new strategies to improve TFS delivery in cochlear implants (CIs)...Full Text Available

2008-03-01

New experiences can trigger changes in gene expression in the brain. To understand this phenomenon better, we studied zebra finches hearing playbacks of birdsong. Earlier research had shown that initial...Full Text Available

2009-07-07

At-Risk Populations

Short term energy shortages. Hearings before the subcommittee on energy of the Committee on Science and Astronautics, U.S. House of Representatives, Ninety-Third Congress, First Session, May 3, 8, 17, 1973

... hearing protection since you play, sit or stand near loud instruments and speakers. Here are a few ... piper's calling you to join him" Led Zeppelin (Robert Plant), "Stairway to Heaven" "My ears are ringin' ...

mtDNA mutation C1494T, haplogroup A, and hearing loss in Chinese

>The causes and implications of the impeading shortages of gasoline, heating oil, diesel fuel, jet fuel, and electricity were investigated at the May 3, 1973 hearings. Discussions and related data were presented on short-term fuel shortages and their effects on the electric utilfties and the present situation in the regulated energy industries and its effects on energy R&D. The statement of the national oil lobbers council is presented in an appendix. (MCW)

1973-01-01

British Library Electronic Table of Contents (United Kingdom)

Mutation C1494T in mitochondrial 12S rRNA gene was recently reported in two large Chinese families with aminoglycoside-induced and nonsyndromic hearing loss (AINHL) and was claimed to be pathogenic. This mutation, however, was first reported in a sample from central China in our previous study that was aimed to reconstruct East Asian mtDNA phylogeny. All these three mtDNAs formed a subclade defined by mutation C1494T in mtDNA haplogroup A. It thus seems that mutation C1494T is a haplogroup A-associated mutation and this matrilineal background may contribute a high risk for the penetrance of mutation C1494T in Chinese with AINHL. To test this hypothesis, we first genotyped mutation C1494T in 553 unrelated individuals from three regional Chinese populations and performed an extensive search ...

2006-01-01

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Wetlands: Community and individual rights v. unchecked government power. Hearing before the Subcommittee on National Economic Growth, Natural Resources, and Regulatory Affairs of the Committee on Government Reform and Oversight, House of Representatives, One Hundred Fifth Congress, First Session, June 16, 1997

Titles V and X of the Water Resources Development Act of 1983. Hearings before the Subcommittee on Water Resources of the Committee on Environment and Public Works, United States Senate, Ninety-Eighth Congress, Second Session, January 24 and 25, 1984

The purpose of this hearing was to bring to the public`s attention a better understanding of both the shortcomings as well as the benefits of the existing wetlands regulations programs, policies and regulations. In addition an attempt was made to highlight those areas where there have been problems with restrictive or confusing regulations, time delays, and costs involved. Ultimately, the purpose is to identify areas in the federal regulations, and if necessary in the federal wetlands related laws, that can be amended, so these work better.

1997-12-31

The DFNA5 gene, responsible for hearing loss and involved in cancer, encodes a novel apoptosis-inducing protein

Two days of hearings on Titles V and X of the Water Resources Development Act of 1983 brought comments from representatives of several states, farm and transport industries, environmental groups, and concerned taxpayers. Title V requires the collection of new funds from users beyond a ceiling set for annual spending on water projects, while Title X proposes a cost-sharing partnership for certain navigation projects. Witnesses were requested to confine their comments to constructive alternatives to the proposed legislation. Additional material on the deep river study and statements submitted for the record follow the testimony of 28 witnesses.

1984-01-01

British Library Electronic Table of Contents (United Kingdom)

DFNA5 was first identified as a gene causing autosomal dominant hearing loss (HL). Different mutations have been found, all exerting a highly specific gain-of-function effect, in which skipping of exon 8 causes the HL. Later reports revealed the involvement of the gene in different types of cancer. Epigenetic silencing of DFNA5 in a large percentage of gastric, colorectal and breast tumors and p53-dependent transcriptional activity have been reported, concluding that DFNA5 acts as a tumor suppressor gene in different frequent types of cancer. Despite these data, the molecular function of DFNA5 has not been investigated properly. Previous transfection studies with mutant DFNA5 in yeast and in mammalian cells showed a toxic effect of the mutant protein, which was not seen after transfection ...

2011-01-01

Mode of Communication, Perceived Level of Understanding, and Perceived Quality of Life in Youth Who Are Deaf or Hard of Hearing

British Library Electronic Table of Contents (United Kingdom)

Given the important role of parent-youth communication in adolescent well-being and quality of life, we sought to examine the relationship between specific communication variables and youth perceived quality of life in general and as a deaf or hard-of-hearing (DHH) individual. A convenience sample of 230 youth (mean age = 14.1, standard deviation = 2.2; 24% used sign only, 40% speech only, and 36% sign + speech) was surveyed on communication-related issues, generic and DHH-specific quality of life, and depression symptoms. Higher youth perception of their ability to understand parents' communication was significantly correlated with perceived quality of life as well as lower reported depressive symptoms and lower perceived stigma. Youth who use speech as their single mode of communication ...

2011-01-01

Crop diversification and trade liberalization: Linking global trade and local management through a regional case study

British Library Electronic Table of Contents (United Kingdom)

Some models anticipate that liberalized agricultural trade will lead to increased crop diversity, while other models make the opposite claim. These positions were explored in southwestern British Columbia, Canada where, between 1992 and 1998, government subsidies and other measures designed to protect horticultural farmers were lifted, exposing these farmers to foreign competition. Public hearings on the future of agriculture provided an opportunity to tap the knowledge and experience of people affected by this transition. Analysis of transcripts from these hearings, which was confirmed by industry data, shows that trade liberalization has led to the loss of the local fruit and vegetable processing industry. Stakeholders saw the loss as a major factor affecting the choice of crops grown lo...

2006-01-01

Comparison of auditory sense organs in parasitoid Tachinidae (Diptera) hosted by Tettigoniidae (Orthoptera) and homologous structures in a non-hearing Phoridae (Diptera)

British Library Electronic Table of Contents (United Kingdom)

The dipteran parasitoids Therobia leonidei and Homotrixa alleni (Tachinidae) use acoustic cues to locate their calling tettigoniid (Ensifera, Orthoptera) hosts. The sexually dimorphic tympanal organs of both fly species are located at the prosternum. For comparison a homologous chordotonal organ in the non-hearing fly Phormia regina, Meigen (Phoridae) is also described. The scolopidial sense organs of the ears have approximately 180 sensory cells in Th. leonidei and 250 cells in H. alleni. Interspecific analysis indicates that the cell number and arrangement might be genus specific in Tachinidae. The mononematic scolopidia, each with one sensory cell, are of different sizes and insert at the tympanal membrane. Large scolopidial units (diameter of sensory cells up to 50??m) extend longitudi...

2007-01-01

Coastal Zone Management Act amendments of 1980. Hearing before the Committee on Commerce, Science, and Transportation, United States Senate, Ninety-Sixth Congress, Second Session on S. 2622, April 30, 1980

Magnetic Fusion Energy Engineering Act of 1980. Hearings before the Subcommittee on Energy Research and Development of the Committee on Energy and Natural Resources, United States Senate, Ninety-Sixth Congress, Second Session, July 28 and August 5, 1980

Testimony at an April 30, 1980 hearing addressed whether to reauthorize or amend the Coastal Zone Management Act of 1978, specifically section 306, to further implement state programs and institutionalize state management of coastal zones. The text of the reauthorization bill (S. 2622) is followed by the statements of eleven witnesses and additional articles, letters, and statements submitted for the record. Witnesses are present for the fishing and oil industries, state and national governments, and public interest groups. Among the points brought out in testimony were that uniform standards are unwise - given the variety of coastal regions; and that there is a need to balance protection and development policies. (DCK)

1980-01-01

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Wastenet

... There are common woodland birds in spring and turtle doves in spring and summer. Havergate Island This small island in the River ...for its breeding avocets and terns, which can be seen throughout the spring and summer. Access is by boat only and the trip ... Visit in spring to hear a symphony of birdsong, especially the nightingales which breed here, and to see bluebells and primroses ... Spring is also an excellent time to visit. Loch na Muilne Loch na Muilne is a fantastic place to see a variety ...

Mary Lyon and the hypothesis of random X chromosome inactivation

The statements given by the following people are included: Steven Dean, Edward Frieman, Zalman Shapiro, Lee Berry, Ronald Davidson, Kenneth Fowler, Melvin Gottlieb, and Tihiro Ohkawa. (MOW)

1981-01-01

British Library Electronic Table of Contents (United Kingdom)

The 50th anniversary of Mary Lyon?s 1961 Nature paper, proposing random inactivation in early embryonic life of one of the two X chromosomes in the cells of mammalian females, provides an opportunity to remember and celebrate the work of those involved. While the hypothesis was initially put forward by Lyon based on findings in the mouse, it was founded on earlier studies, notably the work of Susumu Ohno; it was also suggested independently by Beutler and colleagues using experimental evidence from a human X-linked disorder, glucose-6-phosphate dehydrogenase deficiency, and has proved to be of as great importance for human and medical genetics as it has for general mammalian genetics. Alongside the hypothesis itself, previous cytological studies of mouse and human chromosomes, and the obse...

2011-01-01

Neural-network-based voice-tracking algorithm

Negotiated settlements with a cost of service backstop: The consequences for depreciation

A voice-tracking algorithm was developed and tested for the purposes of electronically separating the voice signals of simultaneous talkers. Many individuals suffer from hearing disorders that often inhibit their ability to focus on a single speaker in a multiple speaker environment (the cocktail party effect). Digital hearing aid technology makes it possible to implement complex algorithms for speech processing in both the time and frequency domains. In this work, an average magnitude difference function (AMDF) was performed on mixed voice signals in order to determine the fundamental frequencies present in the signals. A time prediction neural network was trained to recognize normal human voice inflection patterns, including rising, falling, rising-falling, and falling-rising patterns. The neural network was designed to track the fundamental frequency of a single talker based on the training procedure. The output of the neural network can be ...

2002-11-01

A Blueprint for Telerehabilitation Guidelines?October 2010

The movement from traditional regulatory hearings to negotiated settlements represents both a departure from cost of service regulation and a relaxation of regulatory oversight. Under negotiation parties are able to renegotiate inclusions in their cost of service while simultaneously creating a profit margin for the regulated firm where none existed under the cost of service outcome of a traditional hearing. This paper constructs a model to illustrate the existence of positive gains to pipeline and shipper from the re-allocation of expenses through time in the regulated pipeline services market in Canada. Behaviour consistent with the model is observable in anecdotal and econometric evidence gathered from the library of the National Energy Board of Canada, responsible for pipeline toll regulation in Canada. Empirical investigation by into settlement procedures in the Florida electricity market reveals similar findings; however, this analysis ...

2011-03-01

British Library Electronic Table of Contents (United Kingdom)

Committee Members Co-Chairs David Brennan, M.B.E., Senior Research Engineer, National Rehabilitation Hospital, Washington, District of Columbia. Lyn Tindall, Ph.D., Speech-Language Pathologist, Department of Veterans Affairs Medical Center, Lexington, Kentucky. Writing Committee Janet Brown, M.A., CCC-SLP, Director, Health Care Services, American Speech-Language-Hearing Association, Rockville, Maryland. Mike Campbell, M.S., M.B.A., CCC-SLP, Director of the Speech and Hearing Program, The University of North Carolina at Greensboro, Browns Summit, North Carolina. Jana Cason, D.H.S., OTR/L, Assistant Professor, Auerbach School of Occupational Therapy, Louisville, Kentucky. Diana Christiana, M.A.T., CCC-SLP, President/CEO, Clinical Communications, Sugar Land, TX. Alan Lee, Ph.D., P.T., D.P.T.,...

2011-01-01

A review and update of refining practice in Canada

Process for repairing a cryogenic heat exchanger

This paper first reviews the history of the Port Hope uranium refinery of Eldorado Nuclear Limited. The current processes used for the production of UO_3, UO_2 and UF_6 from yellow cake are then discussed. Recent process changes and developments in the areas of solvent extraction and UO_3 production together with new processes for the production of ceramic UO_2 and UF_4, are described. Environmental and waste management problems, progress and solutions are next considered. Finally, some experience in siting a new UF_6 refinery, with respect to Environmental Impact Statements and public hearings, conclude the paper. (author).

1979-06-08

Positive safety features of US nuclear reactors: technical lessons confirmed at Chernobyl. Hearing before the Subcommittee on Energy Research and Production of the Committee on Science and Technology, US House of Representatives, Ninety-Ninth Congress, Second Session, May 14, 1986, No. 138

The patent describes a method for repairing leakage-causing cracks and fissures in a cryogenic heat exchanger. It comprises: reducing the interior pressure of the heat exchanger to a level which does not exceed the external pressure upon the hear exchanger while maintaining the temperature of the heat exchanger at a low level relative to the ambient external temperature; applying a curable liquid filler composition to the surface of the heat exchanger proximal the leakage site for seepage into the cracks and fissures located at the leakage. The composition upon a relatively short period of cure at low temperature forming a solid material which fills the cracks and fissures; curing the filler composition; and, applying a sealant composition to the surface of the heat exchanger at the filled leakage site. The sealant composition having long-term sealing performance under cyrogenic conditions.

1987-10-13

Dr. Rudolf Schulten of West Germany and expert witnesses from national laboratories, utilities, and the nuclear industry testified on reactor safety issues as they relate to the Chernobyl accident and public concern that modern technology has not paid enough attention to public safety. Each of the witnesses contributed safety-related information based on what has been learned from the Soviet incident. Particular focus went to similarities and differences between the Chernobyl and US reactors in safety design and engineering and to the environmental effects of the accident. The N reactor near Richland and a commercial reactor at Fort St. Vrain, Colorado are the only two operating graphite reactors, but neither is a boiling water reactor.

1986-01-01

New models needed to manage gas supply, APPrO conference hears

National technical report (Matsushita Electric Industrial Company), Vol. 40, No. 4, August 1994. Special issue on batteries

Details of a 1 day conference designed to create more efficient interactions between the gas supply and electricity systems in Ontario and new models for gas supply and the electricity system were presented. Market efficiency, generators' perspectives on critical gas issues, an overview of the North American gas and an outline of issues concerning Ontario were discussed. A natural gas industry forecast and issues concerning marketers and producers were presented. The terms on which gas is bought and sold were discussed. Regulatory tools, cost allocation and issues relating to unbundling were also presented. Infrastructure needs were reviewed, as well as basic market structures. It was suggested that appropriate commercial service options need to be developed and approved, including cross-utility services.

2005-08-01

Indexes to Nuclear Regulatory Commission issuances, July--December 1996. Volume 44, Index 2

Contents: recent trend of battery technologies; alkaline manganese battery with no mercury addition; high capacity zinc-air battery series for hearing aids; characteristics of vanadium-niobium-lithium rechargeable battery; high-energy density lithium-ion rechargeable battery; lithium polymer rechargeable battery: polymer gel electrolyte and electrode; high-capacity ni-cd battery sm120; high-capacity nickel-metal hydride battery; sealed-type batteries for electric vehicles; high-temperature-durable calcium-type battery for automobiles; sealed lead acid batteries for upss; solar cell power unit; solid oxide fuel cell (1); battery management system.

1994-08-01

Design and evaluation of coils for a 50 mm diameter induction coilgun launcher

Digests and indexes for issuances of the Commission (CLI), the Atomic Safety and Licensing Board Panel (LBP), the Administrative Law Judges (ALJ), the Directors` Decisions (DD), and the Decision on Petitions for Rulemaking (DPRM) are presented in this document. These digests and indexes are intended to serve as a guide to the issuances. Information elements common to the cases heard and ruled upon are: case name (owner(s) of facility); full text reference (volume and pagination); issuance number; issues raised by apellants; legal citations (cases, regulations, and statutes); name of facility, docket number; subject matter of issues and/or rulings; type of hearing (operating license, operating license amendment, etc.); type of issuance (memorandum, order, decision, etc.).

1997-04-01

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Computed tomography of middle ear cholesteatomas without tympanic membrane perforation

Coilguns have the ability to provide magnetic pressure to projectiles which results in near constant acceleration. However, to achieve this performance and control projectile hearing, significant constraints are placed on the design of the coils. We are developing coils to produce an effective projectile base pressure of 100 MPa (1kbar) as a step toward reaching base pressures of 200 MPa. The design uses a scalable technology applicable to the entire range of breech to muzzle coils of a multi-stage launcher. This paper presents the design of capacitor-driven coils for launching nominal 50 mm, 350 gram projectiles. Design criteria, constraints, mechanical stress analysis, launcher performance, and test results are discussed.

1993-12-31

Block Talk: Spatial Language During Block Play

The growth of a middle ear cholesteatoma behind a normal tympanic membrane is a rate though possible event. In such cases, CT may provide useful information for diagnosis. The results are presented of a CT study carried out on 14 patients affected with unilateral conductive hearing loss and with normal tympanic membrane. CT allowed the diagnosis of meddle ear cholesteatoma to be made in all cases. All patients were treated with surgery: 8 of them underwent tympanoplasty and 6 explorative tympanotomy. While the diagnosis of cholesteatoma was confirmed in 13 patients, in 1 case tympanosclerosis was diagnosed. CT diagnosis of middle ear cholesteatoma is based on the demonstration of a low-density soft-tissue mass, in association with bone erosion or ossicular dislocation. The author emphasizes the difficulty of a CT diagnosis of cholesteatoma in the patients with middle ear soft-tissue masses in the absence of bone alterations.

1991-01-01

British Library Electronic Table of Contents (United Kingdom)

Spatial skills are a central component of intellect and show marked individual differences. There is evidence that variations in the spatial language young children hear, which directs their attention to important aspects of the spatial environment, may be one of the mechanisms that contributes to these differences. To investigate how play affects variations in language, parents and children were assigned to 1 of 3 conditions: free play with blocks, guided play, or play with preassembled structures (Study 1). Parents in the guided play condition produced significantly higher proportions of spatial talk than parents in the other two conditions, and children in the guided play condition produced significantly more spatial talk than those in the free play condition. Study 2 established baseli...

2011-01-01

Birth Weight and Health and Developmental Outcomes in US Children, 1997?2005

British Library Electronic Table of Contents (United Kingdom)

The primary goal of this study was to assess the association between the full birth weight distribution and prevalence of specific developmental disabilities and related measures of health and special education services utilization in US children. Using data from the 1997?2005 National Health Interview Survey (NHIS) Sample Child Core, we identified 87,578 children 3?17?years of age with parent-reported information on birth weight. We estimated the prevalences of DDs (attention-deficit/hyperactivity disorder [ADHD], autism, cerebral palsy, hearing impairment, learning disability without mental retardation, mental retardation, seizures, stuttering/stammering, and other developmental delay) and several indicators of health services utilization within a range of birth weight categories. We cal...

2011-01-01

Assessment of noise levels generated by music shops in an urban city in Nigeria

British Library Electronic Table of Contents (United Kingdom)

SummaryObjectiveTo assess the level of noise generated by music shops in an urban city in Nigeria. Study designCross-sectional, descriptive study. MethodologyThe study involved music shops in three out of eight identified clusters of market areas in Benin City. A semi-structured, researcher-administered questionnaire was also used to collect data from music shop owners. Noise levels generated by speakers in the music shops were measured using a sound level meter, and blood pressure measurements were taken with a mercury sphygmomanometer. ResultsOf the 250 music shops studied, more than 90.0% generated noise levels >85 dB, and 54.8% had a continuous pattern of noise. Longer duration of working years was significantly associated with decreased hearing (P = 0.01), shouting when talking (P = 0...

2011-01-01

Reasons for decision in the matter of TransCanada PipeLines Limited. Motifs de decision (relative a) TransCanada Pipelines Limited; Volume 3. Installations, exportations de gaz et demandes deposees en vertu de l'article 71

Reasons for decision in the matter of TransCanada PipeLines Limited

TransCanada Pipelines Limited submitted an application to the National Energy Board for new facilities to increase natural gas deliveries to its domestic and export markets. The proposed facilities consist of 1,590 km of pipeline, 21 new compressor units and two new compressor stations. The total cost of the facilities was estimated at $2,408 million. The Board considered at the hearing 15 applications made pursuant to Part IV of the National Energy Board Act for gas exports at existing delivery points at Emerson and Niagra Falls as well as at proposed new delivery points at Chippawa and Iroquois, Ontario. Twelve of these applications were filed in support of TransCanada's facilities application. Five applications were also filed pursuant to section 71 of the National Energy Board Act for orders requiring TransCanada to receive, transport and deliver natural gas offered by the applicants and to provide adequate and suitable facilities to do so. A public ...

1991-04-01

Aarskog-Scott syndrome: Clinical update and report of nine novel mutations of the FGD1 gene

1991-04-01

DEFF Research Database (Denmark)

Mutations in the FGD1 gene have been shown to cause Aarskog-Scott syndrome (AAS), or facio-digito-genital dysplasia (OMIM#305400), an X-linked disorder characterized by distinctive genital and skeletal developmental abnormalities with a broad spectrum of clinical phenotypes. To date, 20 distinct mutations have been reported, but little phenotypic data are available on patients with molecularly confirmed AAS. In the present study, we report on our experience of screening for mutations in the FGD1 gene in a cohort of 60 European patients with a clinically suspected diagnosis of AAS. We identified nine novel mutations in 11 patients (detection rate of 18.33%), including three missense mutations (p.R402Q; p.S558W; p.K748E), four truncating mutations (p.Y530X; p.R656X; c.806delC; c.1620delC), one in-frame deletion (c.2020_2022delGAG) and the first reported splice site mutation (c.1935+3A>C). A recurrent mutation (p.R656X) was detected in three independent families. ...

2010-01-01

ATRX ADD domain links an atypical histone methylation recognition mechanism to human mental-retardation syndrome

Surgery for acoustic neurinoma treated by gamma-knife radiosurgery. A case report

ATR-X (alpha-thalassemia/mental retardation, X-linked) syndrome is a human congenital disorder that causes severe intellectual disabilities. Mutations in the ATRX gene, which encodes an ATP-dependent chromatin-remodeler, are responsible for the syndrome. Approximately 50% of the missense mutations in affected persons are clustered in a cysteine-rich domain termed ADD (ATRX-DNMT3-DNMT3L, ADD{sub ATRX}), whose function has remained elusive. Here we identify ADD{sub ATRX} as a previously unknown histone H3-binding module, whose binding is promoted by lysine 9 trimethylation (H3K9me3) but inhibited by lysine 4 trimethylation (H3K4me3). The cocrystal structure of ADD{sub ATRX} bound to H3{sub 1-15}K9me3 peptide reveals an atypical composite H3K9me3-binding pocket, which is distinct from the conventional trimethyllysine-binding aromatic cage. Notably, H3K9me3-pocket mutants and ATR-X syndrome mutants are defective in both H3K9me3 binding and localization at ...

2011-07-19

Participation of the public in licensing procedures under the Atomic Energy Act and the Federal Emission Control Act. Oeffentlichkeitsbeteiligung bei atom- und immissionsschutzrechtlichen Genehmigungsverfahren

A 52-year-old woman had a history of left hearing loss for 5 years. An acoustic neurinoma with 3.2 cm in diameter was diagnosed and treated with gamma-knife radiosurgery (19 Gy of marginal dose) 1 year and 4 months ago. She developed headache, nausea, and visual disturbance 1 month prior to admission. Slight left facial palsy appeared after radiosurgery. Magnetic resonance imaging demonstrated the tumor with central necrosis in the left cerebellopontine angle cistern, increasing in size to 3.5 cm in diameter, and hydrocephalus. Tumor removal was performed incompletely, because of the fibrous appearance of the tumor and severe adherence with the surrounding cerebellar tissue. Facial palsy did not worsen after surgery. Since the hydrocephalus was not resolved, a right ventriculo-peritoneal shunt was inserted. The clinical course in this case suggests that tumor removal followed by radiosurgery was an approximately effective treatment for large acoustic neurinoma. ...

2001-08-01

Identification, characterization, and chromosomal localization of the human homolog (hES) of ES/130

Section 7 of the Atomic Energy Act (AtG), section 4 of the Federal Emission Control Act (BImSchG), the Nuclear Installations Licensing Ordinance (AtVfV), and the Ninth Ordinance on the Implementation of the BImSchG (Principles of the licensing procedure) require participation of the public in the procedure before administrative provisions or decisions are issued. The book presents the legally prescribed steps at which participation of the public is mandatory, for the simple case (only one license on the agenda), and for the multi-stage licensing procedure: preliminary negotiations / filling of applications for a license and filing of documents / public announcement of projects / access to files / objections / preclusion of delayed objections / public hearing and other expert discussions / termination of procedure, decision-making by the authorities / decisions on subdivision of procedure into defined stages / modification of the procedure. The analysis of the ...

1994-01-01

From Light to Consciousness Journey to the End of Science or Fin de Siecle Malaise

The chicken extracellular matrix glycoprotein ES/130 is necessary for epithelial-mesenchymal transformation in the developing hear and is also expressed in noncardiac chicken tissues such as limb and notochord. We have identified hES, the human homology of chicken ES/130. Fluorescence in situ hybridization analysis (FISH) localizes hES to human chromosome 20p11.2-p12. FISH analyses of individuals with 20p12 deletions and affected by Alagille syndrome exclude hES as a candidate gene for this disorder. Reverse transcriptase-polymerase chain reaction studies reveal that hES is expressed in both fetal and adult human tissues and that hES expression in the left ventricle is increased in the failing adult heart. Further studies will evaluate how hES mutations may relate to congenital human cardiac and skeletal anomalies as well as cardiac remodeling in the adult. 16 refs., 2 figs.

1996-08-01

CERN Document Server

As the twentieth century gropes toward its uncertain conclusion, the pontification quotient increases. We hear that the end of history and science are near; that the questions we face today (in contrast with the end of the 19th century) are no longer soluble or present theories verifiable. These arguments will be examined using two examples. The first, familiar to all physicists, already resides securely in the museum of intellectual achievement. The second is a work in progress : the physiological and/or molecular basis for brain functions such as learning and memory storage. How do these lead to various higher level system properties - in particular, consciousness ? About consciousness, much has been said and written - not all of it illuminating. We discuss some of this, in particular a recent argument of Penrose. We consider whether the nature of consciousness is a (possibly) soluble scientific question. If so, how should it be posed ? Finally, if the end is not ...

1996-01-01

Challenges in environmental monitoring and reporting

A study on brain CT of neurological endemic cretinism

The Canadian Association of Petroleum Producers (CAPP) represents about 170 crude oil and natural gas exploration, development and production companies in regulatory hearings and legal proceedings. Its principal role is to enhance the economic well-being and sustainability of the Canadian upstream petroleum industry. This presentation explores the role that information technology (IT) plays in knowledge and information management and how this role relates to meeting Canada`s target for greenhouse gas emissions reductions set by the Kyoto Protocol. Actions such as the Voluntary Challenge Registry (VCR) and emissions reductions trading are re-visited and the information requirements associated with these actions are highlighted. It is shown that among others, emissions databases between jurisdictions (provincial, national, international) have to be integrated, reduction claims must be tracked, verified and cross-referenced to eliminate double counting, comprehensive ...

1998-12-31

Video equipment of tele dosimetry and audio

A study on brain CT was made in 20 cases of typical endemic cretinism and 15 cases of normal persons. These patients from severely iodine deficient area were diagnosed as neurological endemic cretinism characterized by severe mental retardation with impairment of hearing, speech, neuromotor, etc. to varying degrees. The brain CT showed that there were many and deep depressions in cerebral cortex, especially in frontal and parietal lobes of some cretins, the lateral ventricle system was dilated, particularly in posterior part of it, and the interhemisphere fissure, lateral fissure and subarachnoid cisterns were also expanded. The CT value of cortex was higher and white matter was lower than that of normal persons. In some severe cases, the two hemispheres of brain were not the same in size. These findings above indicated that these endemic cretins had a severe retardation of brain development including cerebral cortex and white matter. In addition, some abnormal ...

Public hearings on water management in Quebec: First Nations and water resources -- Supporting documentation for the Commission Workshop at Mashteuiatsh, Aug 18, 1999; Consultation publique sur la gestion de l'eau au Quebec: Les premieres nations et la ressource eau -- Document de soutien a atelier de travail de la Commission du 18 aout 1999 a Masteuiatsh

To develop a work in an area with high radiation, it requires of a detailed knowledge of the surroundings work, a communication and effective vision, a near dosimetric control. In a work where the spaces variables and reduced accesses exist, noise that hinders the communication, defendant operative condition, radiation field and taking of decision, it is necessary to have tools that allow a total control of the environment to make opportune and effective decisions, there where the task is developed. Under this elementary concept, it was developed in the Laguna Verde Central a project that it allowed a mechanism, interactive of control in spaces complex; to see, to hear, to speak, to measure. This concept takes to the creation of an equipped system with closed circuit of television, wireless communication systems, tele dosimetry wireless systems, VHS and DVD recording equipment, uninterrupted energy units. The system requires of an electric power socket, and the ...

2007-07-01

Recent environment, energy and resources cases and issues in Alberta

Since mid-March the Commission on Water Management, with the assistance of the appropriate ministries, visited all regions of the province in order to acquaint themselves first hand with regional water management problems. During the month of June, the Commission also organized a series of ten workshops, attended by a limited number of experts, to explore in depth certain specific questions in the area of water management. During these public sessions the Commission hoped to discuss specifically the specific questions relating to water management problems of First Nations communities, bearing in mind the essential nature of water in maintaining traditional activities. The workshop took place on the Montanard reserve of Mashteuiatsh (Pointe-Bleu). The morning session was devoted to presentations by invited experts, followed by an exchange of views between the experts and the Commissioners, with opportunity for participants to address question to the experts. This document was compiled ...

1999-08-13

Monthly report of activities: APRIL 1, 1969

Significant environmental issues and regulatory proceedings in Alberta were discussed. The Mackenzie Valley Pipeline Project was reviewed in relation to the Deh Cho First Nation's advanced actions in the federal court to enjoin the review panel from proceeding with the review process. The Deh Cho First Nation is seeking a declaration that the plan violates their rights under the Canadian Charter of Rights and Freedoms and the Constitution Act. Various regulatory and government agencies have now developed a plan to coordinate their different regulatory processes and approvals in the Cooperation Plan for the Environmental Impact Assessment and Regulatory Review of the Northern Gas Pipeline project. The Deh Cho allege that they were not afforded the opportunity to participate in the development and implementation of the plan. A review panel plans to proceed once the environmental and social impacts are determined, and the consortiums plan to minimize harm has been submitted. ...

2005-07-01