Direct and indirect pupil responses were present. Orienting responses could be reliably elicited with a large light stimulus (a halogen lamp with a diameter of 10 cm at a distance of 80 cm). OKN was positive, when elicited with black and white stripes of at least 10 cm in width. Visual field, form, colour and object vision could not be assessed. The visual acuity was not measurable.

Lisa exhibited profound developmental impairment in all modalities. Her visual capacity was limited to detection of bright light stimuli at a distance of 80 cm, indicating a severe form of CVI (corresponding to legal blindness).

The first phase of intervention comprised measures aimed at enhancing her overall mental and physical condition. Given the severity and multiplicity of functional impairments, it was clear to all experts that systematic intervention would need to take place over a considerable time before Lisa could gain any significant improvement in activities of daily life. A tailor-made intervention programme, appropriate for her developmental stage, was developed with the main aim of improving her mental and visual capacities for everyday life activities. Intervention measures were carried out for 1–2 h daily 5 days a week, where possible.

For the initial intervention, presentation of visual stimuli was accompanied by acoustic cues (noises) to indicate the location of the visual stimulus. Lisa was placed in her most comfortable head and body positions such that she was able to direct her attention and fixate straight ahead towards the screen, where large, highly saturated colour surfaces (red, green, yellow, blue, etc.) were shown to her in alternating order at a distance of 50–100 cm, until she directed her gaze towards the stimulus. By using, this procedure of stimulus presentation, Lisa could maintain her attention for at least 15 min; moving visual stimuli enhanced her attention and her attempts to fixate. In addition to this visual ‘stimulation’, Lisa was afforded perceptual experiences with auditory stimuli (e.g. rhythmical tunes), tactile vibrations, and gustatory and olfactory stimuli. The balanced order of presentation of stimuli in the different sensory modalities also improved Lisa’s overall interest and attention span. In the next phase, Lisa was motivated to acquire her own perceptual experiences in a small room that was particularly adapted for her actual visual capacities. This room contained coloured and glittering foils and mobiles at various spatial positions and allowed diverse visual and tactile and body experiences in a spatially limited environment. Once Lisa could manage spatial orientation in this little room (and it was no longer of sufficient interest), she was given similar visual experiences in larger rooms, including her own nursery. However, for distances greater than 2 m she still relied exclusively on acoustic cues. Intervention times varied between 60 and 120 min daily (session duration: 20–30 min), depending on Lisa’s daily condition.

Once Lisa had gained basic visual and other perceptual experiences and had shown longer periods of attention and interest, the next step in the intervention programme was systematic practice with visual fixation and visual discrimination in a room with normal daylight. For the practice with visual fixation, small light stimuli (diameter: 1 cm at a distance of 20 cm) were shown to Lisa in primary (straight ahead) position, then the position was changed to the left or right, with varying distances from the midline. Practice periods usually lasted 15 min. For practice with visual discrimination, checkerboard patterns, gratings and dot patterns on dark background were shown on slides at a distance of 20–50 cm, either singly to one side, or as a pair on either side. Intervention times varied between 60 and 120 min daily (session duration: 20–30 min), depending on Lisa’s daily interest and attention level. Lisa showed slow but consistent improvements in orienting responses, fixation stability and in differential responses to stimulus patterns. In addition to the practice in the visual modality, speech therapy and physiotherapy continued to improve her language capacities and her motor functions.

After 16 months of intervention, Lisa was integrated in a kindergarten for children with special needs (by which time she was 3 years old), but the visual intervention programme was continued in addition. Lisa now preferred structured visual materials like patterns, and was very interested in differences between patterns. She could now also sit upright and control her head position, and was able to reach out and grasp for objects (ball, luminescent ring) with either hand and manipulate them with both hands together. Visual objects were always positioned in the central position in front of her to enhance integration of body and visual midline perception.

Most of the visual and oculomotor functions were unchanged, except for orienting responses that had become prompt and reliable, to weaker and smaller light stimuli (normal light with a diameter of 1 cm) at a distance of 80 cm. Fixation attempts were sometimes successful with her normal head posture. Pursuit eye movements to large (diameter: 1–2 cm) and bright light stimuli were improved, but could not always be reliably elicited. OKN could now be reliably elicited with 5 cm wide stripes.

Lisa was a child with normal development until the age of 4 months when she suffered chronic brain hypoxia and consequently severe multiple functional handicaps. After systematic and regular interdisciplinary intervention for nearly 24 months Lisa showed differential improvement in several domains, but visual perceptual capacity remained low. Whether an earlier start of intervention measures would have resulted in a better outcome remains open to question. Lisa did not however possess the prerequisites that are crucial for an earlier systematic visual training, in particular concerning the control of head position and controlled gaze shifts. Furthermore, atrophy of both optic nerves may have been the principal limiting factor for the recovery of visual acuity as well as fixation accuracy. Considering these circumstances, the intervention programme can nevertheless be deemed successful, at least in part, because it facilitated Lisa to now collect visual experiences in her familiar surroundings, to manifest a degree of visual orientation in familiar rooms, to play with her favourite toys, and to interact socially with familiar people, as well as other children and adults.

Visual and oculomotor functions were largely negative/absent, except for intact pupil responses and light–dark responses. Barbara’s fixation was impaired by jerky horizontal nystagmus, and her head position was permanently shifted to the left side.

Barbara showed profound functional impairments in all modalities. Initially her visual capacity seemed completely lost, but the apparent absence of responses even to light stimuli may also have been explained in part by her oculomotor difficulties and her severely reduced mental functions. In the context of her intact pupil responses and normal optic nerve examination her visual condition can be described as cerebral blindness. However, she subsequently showed good recovery particularly in the domains of visual curiosity, attention and motor functions.

Barbara showed improvements with respect to visual and oculomotor functions. Orienting responses to large moving and stationary visual stimuli (e.g. hand movements) could reliably be observed. A low level of visual acuity was now present; Barbara responded reliably to balls of 5 mm in diameter at a distance of 20 cm, and showed pursuit eye movements to such stimuli, provided that sufficient concentration was available. She also showed differential responses to coloured stimuli of high contrast and high saturation, and to coloured forms and objects with a diameter of 1 cm at a distance of 20 cm. Accurate fixation was present for very brief periods (1–2 s), however, this was impaired by intermittent pendular nystagmus, and by her still unstable head position, whereby the head was now mostly directed rightwards. Saccadic eye movements to moving stimuli in the periphery were consistently prompt and accurate in all directions.

Barbara was a child with normal development until the age of 7 months when she was hit by a car and suffered severe closed head trauma with chronic hypoxia and severe multiple functional handicaps. After systematic and regular interdisciplinary intervention for nearly 6 years Barbara showed good functional recovery including in the visual domain, with improved visual orienting responses, oculomotor behaviour, visual acuity, spatial orientation and object recognition. However, language and social development were still considerably impaired. It seems that brain injury had affected, in particular, her functional system in the prefrontal and limbic structures involved in regulation and control of attention and affect. This may explain her low level of frustration tolerance and her auto-aggressive behaviour, and may also have impeded better visual and cognitive development. Furthermore, atrophy of both optic nerves may have limited further improvement in visual acuity beyond about 6/36. Nonetheless, the intervention effects can be evaluated as positive because Barbara ultimately became able to collect visual experiences of her surroundings, possessed visual orientation in familiar rooms, could play with her favourite toys, and liked social interactions with familiar people, as well as other children and adults.

Anna showed a large homonymous central scotoma, with a diameter of ~40° (see Fig. 9.1a). Visual acuity, colour vision and object vision could not be tested, although Anna reported coloured and black–white impressions when confronted with large colour or form stimuli or with large objects. Her fixation was highly unstable and inaccurate, although she tried hard to find each stimulus and keep her eyes on it. Eye alignment was slightly divergent (~10°), but no nystagmus was present. Anna tried to scan the visual environment spontaneously, but without great success.

Anna showed normal development until the age of 6 years. Because of her well-developed mental capacities, she had already attended elementary school and could read and write. There was no history of visual abnormalities. After her anoxic brain injury, which had mainly affected posterior brain structures, she exhibited severe visual deficits due to the loss of the central visual field, as well as left-sided hemiparesis. As her cognitive and language abilities had fully recovered 6 weeks after brain injury, systematic, regular intervention was initiated 2 weeks later. During the periods of intervention, Anna was an in-patient in a nearby hospital for children, but was transported to the Max Planck Institute of Psychiatry on a daily basis for visual training.

In the first phase of practice with localisation, Anna was instructed to find a light target in front of her, fixate it and point at it but only after she could fixate it. Once she could do this more accurately, she was asked to reach for and grasp the light target. The light target was always presented at grasping distance, i.e. at 30 cm, but in different directions (midline, and in various directions to the left and right of the midline); the presentation time was about 8–10 s in the initial sessions, and 2–5 s once Anna could point at and grasp the target more accurately.

In the second phase of practice with localisation, Anna was asked to reach out for and grasp objects positioned on a desk either in front of her or at varying distances to the left or right of her midline. She was asked to look straight-ahead at first, and then to search for the object and fixate it, and eventually to grasp it. The order of presentation initially followed an order known to Anna, i.e. mid position, left, right, mid position, right, left, etc. Later, the order of positions was made unpredictable.