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Published on: April 11, 2025
Functional vision and cognition in infants with congenital disorders of the peripheral visual system
Naomi Dale1,2, Elena Sakkalou2, Michelle O'Reilly2
1Neurodisability Service, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Insights
Vision impairment in infants with congenital disorders is linked to cognitive delays. Early
Area of Science:
- Pediatric ophthalmology
- Developmental neuroscience
- Clinical audiology
Background:
- Congenital disorders of the peripheral visual system are a significant cause of visual impairment in infants.
- Understanding the relationship between vision and cognitive development is crucial for early intervention strategies.
Purpose of the Study:
- To investigate the relationship between vision and cognition in a national cohort of 1-year-old infants with congenital disorders of the peripheral visual system and visual impairment.
- To assess vision levels and non-verbal cognitive abilities in infants with varying degrees of visual impairment.
Main Methods:
- Cross-sectional observational study of a nationally recruited cohort of infants (8-16 months).
- Vision assessed using the Near Detection Scale; non-verbal cognition evaluated with the Reynell Zinkin Scales (sensorimotor understanding).
- Demographic data collected via parental questionnaires.
Main Results:
- 90 infants included: 28% with profound visual impairment, 72% with severe visual impairment.
- Significant correlation found between Near Detection Scale scores and sensorimotor understanding developmental quotients (p<0.001).
- Vision and age accounted for 48% of the variance in sensorimotor understanding; profound visual impairment, especially in complex cases, was associated with greater cognitive delay.
Conclusions:
- Lack of vision is associated with delayed early object manipulative abilities and concepts.
- 'Form' vision appears to support early developmental progress.
- This study provides essential baseline data for ongoing longitudinal investigations into vision and cognitive development.
Aim:
To investigate how vision relates to early development by studying vision and cognition in a national cohort of 1-year-old infants with congenital disorders of the peripheral visual system and visual impairment.
Method:
This was a cross-sectional observational investigation of a nationally recruited cohort of infants with 'simple' and 'complex' congenital disorders of the peripheral visual system. Entry age was 8 to 16 months. Vision level (Near Detection Scale) and non-verbal cognition (sensorimotor understanding, Reynell Zinkin Scales) were assessed. Parents completed demographic questionnaires.
Results:
Of 90 infants (49 males, 41 females; mean 13mo, standard deviation [SD] 2.5mo; range 7-17mo); 25 (28%) had profound visual impairment (light perception at best) and 65 (72%) had severe visual impairment (basic 'form' vision). The Near Detection Scale correlated significantly with sensorimotor understanding developmental quotients in the 'total', 'simple', and 'complex' groups (all p<0.001). Age and vision accounted for 48% of sensorimotor understanding variance. Infants with profound visual impairment, especially in the 'complex' group with congenital disorders of the peripheral visual system with known brain involvement, showed the greatest cognitive delay.
Interpretation:
Lack of vision is associated with delayed early-object manipulative abilities and concepts; 'form' vision appeared to support early developmental advance. This paper provides baseline characteristics for cross-sectional and longitudinal follow-up investigations in progress. A methodological strength of the study was the representativeness of the cohort according to national epidemiological and population census data.
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