Deafness

The material presented here is broadly divided into subsections following Fraser's arrangements. The bulk of it is files on cases of deaf children from surveys carried out by him. Most of these cases (2,355) were from a survey of special schools in the United Kingdom and Ireland, with further cases from a survey in Oxfordshire and Berkshire (adults and children) and a later survey in South Australia. In addition Fraser had cases from a small survey of people with severe visual handicap or mentally subnormality; a survey of 72 women in a home connected with a school for deaf children in Dublin; and 176 cases referred specially as having clinical features of particular interest.

The case files include questionnaires, medical notes, correspondence with parents of affected children, family and hospital doctors, teachers and education authority officials (some of the children were in special schools), audiograms, electrocardiograms and photographs. See also PP/GRF/E.30.

In addition to the case files presented in the surveys of British and Irish schools for the deaf, Oxfordshire and Berkshire deaf adults and children and South Australia deaf adults and children, there are many case files kept by Fraser with the material for specific chapters of the book, The Causes of Profound Deafness in Childhood. These cases are presented by chapter and were selected by Fraser as illustrative of various auditory impediments. Other material here includes correspondence with colleagues, drafts of chapters, tables and figures, and background material, chiefly reference material in the form of offprints or photocopies thereof but including unpublished typescripts and notes.

1861-2007

35 boxes

In 1957 George Fraser was awarded a Medical Research Council (MRC) scholarship to study human genetics at the Galton Laboratory at University College London, working under Professor L. S. Penrose as a Ph.D. student. His thesis was on 'Deafness with Goitre (Syndrome of Pendred) and some related aspects of thyroid disease' (see PP/GRF/B.1). In October 1959 Fraser moved to Oxford to join A.C. Stevenson's Medical Research Council Population Genetics Research Unit. This Unit was gathering research data in population genetics and Fraser began his study of profound childhood deafness, studying 2,330 children in special schools for the deaf in the UK and Ireland. For the studies in Belfast and Dublin he was accompanied by his long-term collaborator in studies of deafness with abnormal ECG (Jervell and Lange-Nielsen syndrome), P. Froggatt of Queen's University, Belfast. The methodology was based on Penrose’s famous Colchester survey of mental defect ('A clinical and genetic survey of 1280 cases of mental defect', Medical Research Council Special Report 229, 1938). In this period Fraser made a study of the causes of deafness: clinico-genetical studies of inherited conditions, the importance of various factors in the causation of pre- and peri-natally acquired deafness and a statistical and genetical analysis of the data to uncover the relative importance of dominant, recessive and sex-linked types of deafness.

In the mid 1960s Fraser again worked extensively with Froggatt on the association between electrocardiographic (ECG) abnormalities and deafness. The work is described in 'The cardio-auditory syndrome of Jervell and Lange-Nielsen: the anatomy of a population study' by Froggatt in the Festschrift to Fraser (copy is at PP/GRF/B.293). The syndrome could cause sudden or 'cot' death in young children. On his move to Adelaide 1966-1967, Fraser carried out a further survey in South Australia (adults and children).

By 1968 Fraser had documentation of over 3,500 cases of individuals who had been profoundly deaf from childhood - that is, sufficiently serious and of sufficiently early onset to necessitate the use of special educational methods for the acquisition of speech or alternative methods of communication. These cases formed the data for Fraser's book, The Causes of Profound Deafness in Childhood. A study of 3,535 individuals with severe hearing loss present at birth or of childhood onset, with a foreword by V.A. McKusick (Johns Hopkins University Press, Baltimore and London, 1976). Fraser also authored and co-authored a significant number of papers on aspects of deafness.

See also PP/GRF/E.32-33, G.13, Section H, and K.8.

Reference material in the form of offprints or photocopies thereof has been discarded unless annotated or of particular importance. The book The Causes of Profound Deafness in Childhood contains a full bibliography.

Additional material related to Fraser's study of Pendred Syndrome was acquired from Dr Fraser in 2015. This material is currently uncatalogued and primarily comprises patient data, which is closed under the Data Protection Act.