UCL logo

UCL Discovery

UCL home » Library Services » Electronic resources » UCL Discovery

Deficient auditory interhemispheric transfer in patients with PAX6 mutations

Bamiou, D.-E.; Musiek, F.E.; Sisodiya, S.M.; Free, S.L.; Davies, R.A.; Moore, A.; ... Luxon, L.M.; + view all (2004) Deficient auditory interhemispheric transfer in patients with PAX6 mutations. Annals of Neurology , 56 (4) pp. 503-509. 10.1002/ana.20227.

Full text not available from this repository.

Abstract

PAX6 mutations are associated with absence/hypoplasia of the anterior commissure and reduction in the callosal area in humans. Both of these structures contain auditory interhemispheric fibers. The aim of this study was to characterize central auditory function in patients with a PAX6 mutation. We conducted central auditory tests (dichotic speech, pattern, and gaps in noise tests) on eight subjects with a PAX6 mutation and eight age- and sex-matched controls. Brain magnetic resonance imaging showed absent/hypoplastic anterior commissure in six and a hypoplastic corpus callosum in three PAX6 subjects. The control group gave normal central auditory tests results. All the PAX6 subjects gave abnormal results in at least two tests that require interhemispheric transfer, and all but one gave normal results in a test not requiring interhemispheric transfer. The left ear scores in the dichotic speech tests was significantly lower in the PAX6 than in the control group. These results are consistent with deficient auditory interhemispheric transfer in patients with a PAX6 mutation, which may be attributable to structural and/or functional abnormalities of the anterior commisure and corpus callosum, although the exact contribution of these two formations to our findings remains unclear. Our unique findings broaden the possible functions of PAX6 to include neurodevelopmental roles in higher order auditory processing.

Type:Article
Title:Deficient auditory interhemispheric transfer in patients with PAX6 mutations
DOI:10.1002/ana.20227
Publisher version:http://dx.doi.org/10.1002/ana.20227
Language:English
UCL classification:UCL > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Neurology > Clinical and Experimental Epilepsy
UCL > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Child Health > Department of Cardiorespiratory Sciences > ICH - Cardiac Unit
UCL > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
UCL > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Child Health > Department of Neurosciences and Mental Health > ICH - Audiological Medicine

Archive Staff Only: edit this record