A case study of amnion rupture sequence with acalvaria, blindness, and clefting: clinical and psychological profiles.

The purpose of this article is to report the case of a 10-year-old girl born with anophthalmia, bilateral oblique facial clefts, and missing scalp and bones over the temporal and parietal areas of the cranial vault bilaterally. Early amnion rupture seems to be the most probable cause of this rare combination of anomalies. Because no similar case has been reported in the literature so far, we describe here the clinical and psychosocial history of this unusual patient, who has been able to live the intellectually and socially normal life of a blind child in spite of the major craniofacial deformities. The already completed and possible future therapeutic strategies are discussed.

Maturation of the auditory change detection response in infants: a longitudinal ERP study.

Mismatch negativity (MMN) is a negative component of auditory event-related potential (ERP), reflecting the brain's automatic change detection process. In the present study we investigated the development of the pitch change detection, as indexed by the MMN, in the same infants from birth until 12 months of age. The MMN was identified in approximately 75% of infants at each age, being relatively stable in latency and amplitude at the group level across the ages studied. However, within the same subjects the MMN substantially varied from age to age. The inspection of individual data revealed a possible source of this variability: in a portion of 3- to 9-month-old infants, a large-amplitude positive component commenced at the latency of the MMN and thus might have masked it. The results of the additional experiment, employing distracting novel sounds in 2-year-old infants and newborns, suggested that the observed positive component could represent an infant analogue of the adult P3a response, indexing an involuntary orienting of attention. Therefore, the variability from age to age might be, at least partially, caused by the differences in degree of infants' orienting, resulting in the reduction of the scalp recorded mismatch negativity in recordings when the orienting P3a positivity was elicited.

Auditory ERPs reveal brain dysfunction in infants with plagiocephaly.

It is suspected that the developmental delay in school-aged children diagnosed as infants suffering from plagiocephaly is caused by the modification of the skull form. To detect possible cognitive impairment in these children, we examined auditory ERPs to tones in infant patients. The infants with plagiocephaly exhibited smaller amplitudes of the P150 and the N250 responses to tones than healthy controls. Differences between the patients and control subjects indicate that already at this early age the presence of the plagiocephalic skull signals compromise of brain functioning. The present data suggest that most of the plagiocephalic infants have an elevated risk of auditory processing disorders. In the current study we demonstrated, for the first time, that the central sound processing, as reflected by ERPs, is affected in children with plagiocephaly.

Maturation of the auditory event-related potentials during the first year of life.

This study examined the maturation of cortical auditory event-related potentials (ERPs) from birth until 12 months of age. In the 15 infants studied, all ERP peaks observable at 12 months of age, the P150, N250, P350, and N450 were identifiable already at birth, As in previous studies, the amplitudes of the ERP peaks increased and latencies shortened with increasing age. In addition, the time courses of the amplitude growth of these peaks differed from each other. It was concluded, that the generators of all the infantile ERP peaks are functional already at birth, and that the maturational changes in the waveform morphology can mostly be accounted for by the changing relative strengths of the different generators.