Familial Ménière's disease: clinical and genetic aspects

ABSTRACT Background and purpose:Mre's disease is not uncommon, with an incidence in Caucasians of about one in 2000. The incidence peaks in the fifth decade. Cases are usually isolated or sporadic, but in perhaps five per cent other family members are affected. We report here the clinical and genetic characteristics of a comprehensive set of familial Mre's disease cases from the UK.Methods:Forty-six affected families were studied. All cases were diagnosed using the American Academy of Otolaryngolog 8211;Head and Neck Surgery committee on hearing and equilibrium 1995, or more stringent, criteria.Outcomes and results:Autosomal dominant inheritance with reduced penetrance was the most likely mode of inheritance overall. Apparent genetic anticipation was observed, but may also be a result of ascertainment bias given the collection strategy. There was also a slight tendency for cases to result from maternal transmission within the families in this set. The family pedigrees are presented, and the authors have also set up a website at which all the pedigrees may be viewed in greater detai

On Krein-like theorems for noncanonical Hamiltonian systems with continuous spectra: application to Vlasov-Poisson

The notions of spectral stability and the spectrum for the Vlasov-Poisson system linearized about homogeneous equilibria, f_0(v), are reviewed. Structural stability is reviewed and applied to perturbations of the linearized Vlasov operator through perturbations of f_0. We prove that for each f_0 there is an arbitrarily small delta f_0' in W^{1,1}(R) such that f_0+delta f_0$is unstable. When$f_0$ is perturbed by an area preserving rearrangement, f_0 will always be stable if the continuous spectrum is only of positive signature, where the signature of the continuous spectrum is defined as in previous work. If there is a signature change, then there is a rearrangement of f_0 that is unstable and arbitrarily close to f_0 with f_0' in W^{1,1}. This result is analogous to Krein's theorem for the continuous spectrum. We prove that if a discrete mode embedded in the continuous spectrum is surrounded by the opposite signature there is an infinitesimal perturbation in C^n norm that makes f_0 unstable. If f_0 is stable we prove that the signature of every discrete mode is the opposite of the continuum surrounding it.Comment: Submitted to the journal Transport Theory and Statistical Physics. 36 pages, 12 figure

Post-metamorphic fluid infiltration into granulites from the Adirondack Mountains, USA

Post-metamorphic effects in the anorthosites of the Adirondacks, New York were described. Calcite-chlorite-sericite assemblages occur as veins, in disseminated form and as clots, and document retrograde fluid infiltration. These features are associated with late-state CO2-rich fluid inclusions. Stable isotope analyses of calcites indicates that the retrograde fluids interacted with meta-igneous and supracrustal lithologies, but the precise timing of the retrogression is as yet unknown

Definition, analysis and development of an optical data distribution network for integrated avionics and control systems. Part 2: Component development and system integration

Fiber optic transmission is emerging as an attractive concept in data distribution onboard civil aircraft. Development of an Optical Data Distribution Network for Integrated Avionics and Control Systems for commercial aircraft will provide a data distribution network that gives freedom from EMI-RFI and ground loop problems, eliminates crosstalk and short circuits, provides protection and immunity from lightning induced transients and give a large bandwidth data transmission capability. In addition there is a potential for significantly reducing the weight and increasing the reliability over conventional data distribution networks. Wavelength Division Multiplexing (WDM) is a candidate method for data communication between the various avionic subsystems. With WDM all systems could conceptually communicate with each other without time sharing and requiring complicated coding schemes for each computer and subsystem to recognize a message. However, the state of the art of optical technology limits the application of fiber optics in advanced integrated avionics and control systems. Therefore, it is necessary to address the architecture for a fiber optics data distribution system for integrated avionics and control systems as well as develop prototype components and systems

Reynolds number dependence of streamwise velocity spectra in turbulent pipe flow

Spectra of the streamwise velocity component in fully developed turbulent pipe flow are presented for Reynolds numbers up to 5.7×10^6. Even at the highest Reynolds number, streamwise velocity spectra exhibit incomplete similarity only: while spectra collapse with both classical inner and outer scaling for limited ranges of wave number, these ranges do not overlap. Thus similarity may not be described as complete, and a region varying with the inverse of the streamwise wave number, k1, is not expected, and any apparent k1-1 range does not attract any special significance and does not involve a universal constant. Reasons for this are suggested

Scaling of the streamwise velocity component in turbulent pipe flow

Statistics of the streamwise velocity component in fully developed pipe flow are examined for Reynolds numbers in the range 5.5 x 10^4 ≤ ReD ≤ 5.7 x 10^6. Probability density functions and their moments (up to sixth order) are presented and their scaling with Reynolds number is assessed. The second moment exhibits two maxima: the one in the viscous sublayer is Reynolds-number dependent while the other, near the lower edge of the log region, follows approximately the peak in Reynolds shear stress. Its locus has an approximate (R^+)^{0.5} dependence. This peak shows no sign of ‘saturation’, increasing indefinitely with Reynolds number. Scalings of the moments with wall friction velocity and $(U_{cl}-\overline{U})$ are examined and the latter is shown to be a better velocity scale for the outer region, y/R > 0.35, but in two distinct Reynolds-number ranges, one when ReD 7 x 10^4. Probability density functions do not show any universal behaviour, their higher moments showing small variations with distance from the wall outside the viscous sublayer. They are most nearly Gaussian in the overlap region. Their departures from Gaussian are assessed by examining the behaviour of the higher moments as functions of the lower ones. Spectra and the second moment are compared with empirical and theoretical scaling laws and some anomalies are apparent. In particular, even at the highest Reynolds number, the spectrum does not show a self-similar range of wavenumbers in which the spectral density is proportional to the inverse streamwise wavenumber. Thus such a range does not attract any special significance and does not involve a universal constant

Aquatic Invertebrate Community Structure, Biological Condition, Habitat, and Water Quality at Ozark National Scenic Riverways, Missouri, 2005-2014

Ozark National Scenic Riverways (OZAR) was established to protect the corridor of the Current River and its major tributary, the Jacks Fork. The Current River is one of the few remaining free-flowing rivers in the U.S., with much of its base flow coming from several large springs. To assess the biological condition of these rivers, aquatic invertebrate community structure was monitored from 2005 to 2014. Benthic invertebrate samples and associated habitat and water quality data were collected from each of nine sampling sites using a Slack-Surber sampler. The Stream Condition Index (SCI), a multimetric index that incorporates taxa richness, EPT (Ephemeroptera, Plecoptera, Trichoptera) richness, Shannon’s diversity index, and Hilsenhoff Biotic Index (HBI), was calculated. The benthic invertebrate fauna was diverse with 155 distinct taxa identified from all sites. Mean taxa richness was high, ranging from 22 to 30 among sites. The invertebrate taxa of the Current River and Jacks Fork are largely intolerant across all taxa represented (mean tolerance value= ~4.25). Mean HBI did not exceed 3.9 in the Current River or 4.4 for the Jacks Fork. Mean SCI scores across sampling sites generally were well above 16, indicating they are not impaired. Habitat and water quality data were summarized, but they were poorly correlated with individual invertebrate metrics. Sørenson’s similarity index was used to assess community similarity among sites, and similarity scores were then analyzed using ascendant hierarchical cluster analysis. Similarity among sites was 72% or greater. Cluster analysis showed that Current River and Jacks Fork sites clustered separately and in a downstream progression. The uppermost collection site on the Current River was most unlike the other sites, which probably relates to the distinct physical features of that site compared to the others. Nonmetric Multidimensional Scaling (NMDS) was used to evaluate the relationship of invertebrate metrics to habitat and water quality. The NMDS model was found to be a good fit (stress=0.04) and specific conductance, temperature, discharge, filamentous algae and aquatic vegetation were among the most important habitat variables in defining the relationship among sampling sites. The three lower Current River and Jacks Fork sites each were closely grouped in ordination space, but the three upper Current River sites were farther apart from each other. The influence of several large volume springs near those sites is suspected of producing such disparity through press type disturbances. Although the invertebrate communities and water quality in the Current River and Jacks Fork are largely sound and have high biological condition, ongoing and projected threats to these resources remain, and those threats largely originate outside park jurisdictional boundaries. Inherent variability of invertebrate community diversity across sites and years highlights the importance of using multi-metric assessments and multiyear monitoring to support management decisions

Further observations on the mean velocity distribution in fully developed pipe flow

The measurements by Zagarola & Smits (1998) of mean velocity profiles in fully developed turbulent pipe flow are repeated using a smaller Pitot probe to reduce the uncertainties due to velocity gradient corrections. A new static pressure correction (McKeon & Smits 2002) is used in analysing all data and leads to significant differences from the Zagarola & Smits conclusions. The results confirm the presence of a power-law region near the wall and, for Reynolds numbers greater than 230×10^3 (R+ >5×10^3), a logarithmic region further out, but the limits of these regions and some of the constants differ from those reported by Zagarola & Smits. In particular, the log law is found for 600<y+ <0.12R+ (instead of 600<y+ <0.07R+), and the von Kármán constant κ, the additive constant B for the log law using inner flow scaling, and the additive constant B∗ for the log law using outer scaling are found to be 0.421 ± 0.002, 5.60 ± 0.08 and 1.20 ± 0.10, respectively, with 95% confidence level (compared with 0.436±0.002, 6.15±0.08, and 1.51±0.03 found by Zagarola & Smits). The data also confirm that the pipe flow data for ReD ≤ 13.6×10^6 (as a minimum) are not affected by surface roughness

A study of poultry processing plant noise control techniques

A number of techniques can be used to reduce noise in poultry processing plants. In general, covering the ceiling with a noise-absorbing medium is a practical first step. Once the reflected noise levels are abated, treatment of specific identifiable noise courses can take place. The development, flammability, and mechanical properties of acoustic panels to be vertically suspended from the ceiling are discussed as well as the covers need to comply with USDA cleanability requirements. The isolation of drive motors and pumps from large expansive areas, the muffling of pneumatic devices, and the insulation of ice chutes are methods of source quieting. Proper maintenance of machinery and vibration monitoring are also needed to reduce hearing damage risk and to improve worker productivity and employee/supervisor relations