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This is a summary of a report which was the result of an epidemiological investigation, carried out under the sponsorship of the Commission for the European Communities (CEC) and its Committee on Medical and Public Health Research (CRM) in the nine countries of the European Community, in order to determine the prevalence of childhood deafness. The design of the study specified that all children born during the year 1969 would be included if their hearing loss averaged 50 dB or worse in the better ear. Most of the children were enumerated in 1977 when they were eight years old.The prevalence of the degree of deafness in the CEC was 0.9/1000 live births in 1969. There were more deaf children born in the winter than in the summer months. Perceptive hearing loss accounts for 92% of all the cases of deafness and Rubella was the largest single cause of the deafness where the aetiology was known. If perceptive deafness is considered as a whole, in 42% of the children no cause was reported. 29% of the children were reported as having associated handicaps. In 33% of the children there was a mean hearing loss of 100 Db or more. Approximately two-thirds of the children were suspected of being deaf by the time they reached their 3rd birthday and almost 50% had their deafness confirmed by the same age. For 33% of the children there was a delay of up to 12 months between confirmation of their hearing loss and the issue of hearing aids. There were major differences in hearing aid arrangements adopted by the different countries and in aid usage both 'in' and 'out of school'. 10% of the children had no evidence of hearing even when using hearing aids. Approximately two-thirds of the children attend special schools for the deaf and the remainder attend ordinary schools; however there are marked differences between countries in the types of schools attended. Speech that was at least intelligible to strangers, was only reported in 47% of the children and only 32% were stated as being able to read like a normal 8-year-old. There is a possible relationship between hearing aid usage 'out of school and the intelligibility of speech'.

The clinical, pathological, and genetic findings in two closely related families in which a number of cases of lipoid proteinosis occurred are described. The necropsy findings, particularly the neuropathological aspects, in a patient who died from a coincidental pancreatic carcinoma are detailed. The genetic aspects are reviewed.