Health technology assessment in Ireland.

Ireland's health system is primarily funded from general taxation and is publicly provided, although private health care retains a considerable role. It is a unique structure, a mixture of universal health service free at the point of consumption and a fee-based private system where individuals subscribe to private health insurance that covers some of their medical expenses. The recent history of the Irish health services saw consolidation of existing services and an expansion into new areas to adapt to changing practices and needs. There has also been a drive to extract maximum efficiency so as to maintain the volume and quality of patient services at a time of very tight financial constraints. Introduction of new health technologies continued to accelerate. New technologies tended to spread rapidly before systematic appraisal of their costs and benefits. When the state is involved in funding the public hospital system, acceptance of new technology is a matter for discussion between agencies and the Department of Health and Children. Decisions about spending annual "development funding" have generally not been based on careful assessment of proposals for new technology. In 1995, a healthcare reform put new Public Health Departments in Health Boards in a prime position in Ireland's health services organization. These departments now emphasize evidence-based medicine. While Ireland does not have a national health technology assessment (HTA) program, there are plans to form an advisory group on HTA in 1998. HTA is seen as a significant element of future health policy in Ireland.

The ermine phenotype: pigmentary-hearing loss heterogeneity.

The term ermine phenotype has been chosen to describe patients with white hair with black tufts. The patients also have sensorineural hearing loss. This rare phenotype may come about either by failure of migration of melanocytes or by an autoimmune mechanism. Examples of each are cited. The authors describe a possible third type. Comparison with other pigment loss-sensorineural hearing loss syndromes is made.

Proteus syndrome: ocular complications.

Proteus syndrome is a recently recognized hamartoneoplastic malformation syndrome of uncertain etiology and variable expression, whose cardinal manifestations are pigmented nevi, hemihypertrophy, macrodactly, lipomata, and cerebroid-gyriform configuration of the skin on the soles of the feet. The characteristic features may be present at birth but become more apparent with time. In the past this syndrome has been confused with other overgrowth disorders such as neurofibromatosis, Klippel-Trenaunay-Weber syndrome, Maffucci syndrome, and Bannayan syndrome. The ophthalmic features of the proteus syndrome require clarification. We review the ocular findings in 16 previously described cases and describe the findings unique to our patient, in particular, unilateral epibulbar and suspected posterior segment hamartomas.

A new hearing test applicable to the crying newborn infant.

Hearing tests in newborn infants hitherto described have been designed to elicit a response to a sound when the infants are quiet or asleep. It has been accepted that there was no response when they were crying. The present observation describes the effect of a sound stimulus on the crying newborn infant. I have observed that if the infant is not extremely distressed it is possible to get a response regularly. What I did was to make a continuous sound at a distance of 15 to 30 cms. and the babies regularly stopped their cry sequence. The vocal intensity required was less than that of the baby's cry. This quieting response suggests auditory attention or a reflex mediated at a cortical level. The response to this vocal sound is analysed in terms of its effect on heart rate and respiration. It is compared with the effect of a pure tone stimulus of different pitch. It seems to be elicited with notes of low pitch and occurs independent of age or birth weight. The implications and possible long-term significance of the response will be discusses.