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

Principles for effective impact assessment: Examples from Western Australia

What makes an impact assessment process effective with respect to underlying legal and other principles? Prompted by a local review of administrative processes for environmental impact assessment (EIA), I identify 10 key aspects of IA legislation and practice in Western Australia along with corresponding principles. The EIA system in Western Australia (WA) has established an international reputation as a strong model for successful practice, and draws on more than 30 years of operation. Recent government reviews pose some threat and uncertainty regarding the future. In this context I reflected on the key ingredients of the legal and operating framework and realised that each conformed with important principles for good practice. Examples include a significance test at the screening step based on an environment-centred approach; public involvement that upholds natural justice expectations, transparency and accountability; the application of rational-scientific principles in the pursuit of adaptive environmental management; as well as upholding the polluter pays principle by ensuring that the proponent is responsible for all major EIA tasks and outcomes which in turn are legally binding and enforced. I outline each of the 10 principles using extracts from the legal arrangements for EIA in WA practice to illustrate each. I argue that the simultaneous attainment of all principles is necessary to deliver an effective impact assessment practice. The WA arrangements may have relevance to practice elsewhere in the world. I conclude with some observations on the implications of recent EIA review for the situation in Western Australia

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Review of Environmental Impact Assessment in Australia: Theory and Practice by I Thomas and M Elliot

Utility of Environmental Impact Assessment Processes in Western Australia: submission to inquiry into the environmental effects statement process in Victoria, Environment and Natural Resources Committee of the Parliament of Victoria

I have been asked to discuss a number of issues relating to the inquiry, including: •the key strengths of environmental impact assessment (EIA) processes in Western Australia (WA), including objectives, project referrals, levels of assessment, appeal rights for third parties, and the role of the Environment Protection Authority; •proposed reforms to the WA EIA Framework; •your experiences in environmental impact assessment processes in other jurisdictions, including examples of EIA best practice in Australia and overseas; •the role of strategic environmental assessment; •the most suitable body/agency to carry out EIA; and •post-EIA monitoring and enforcement. A brief report addressing these points is provided following an account of the EIA context in WA

Investigating shellfish deposition and landscape history at the Natia Beach site, Fiji

The relationship between environmental variation and subsistence practices is a central point of discussion in much Oceanic archaeology. While human predation can significantly reduce prey populations, environmental variation also contributes to reductions in prey abundance, possibly leading to increased human competition and resource scarcity. At the Natia Beach site, Nacula Island, Fiji, geoarchaeological evidence suggests that coastal progradation began soon after initial occupation of the coastal plain. Additionally, at approximately 650 BP a marked increase in clay and silt deposition occurred. Changes in coastal geomorphology may be explained by landscape response to regional Mid-Holocene sea level fall combined with human induced soil erosion due to upland settlement. Smaller scale environmental changes associated with climate variability may have also played a role. Additionally, landscape change appears to have had a measurable impact on local nearshore mollusks that are sensitive to high levels of water turbidity. Minor evidence of human exploitation is observable in this shellfish assemblage, although changes in predation pressure may have allowed shellfish populations to recover. Increased ceramic diversity and fortified settlements also appear at approximately 650 BP on Nacula and other parts of Fiji. The suite of changes at Natia may be explained by processes of regional and local environmental changes, and human adaptation in terms of subsistence, spatial organization, and competition