2016

Adaptive Learning Systems

WP1

This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Contents

1. Preamble...... 3 Terms ...... 3 Inclusivity ...... 4 Constraints ...... 5 Sensory Deficits ...... 5

2. Visual Deficits ...... 6 Overview ...... 6 Refractive Error ...... 8 Uncorrectable Low Visual Acuity (Visual Impairment)...... 9 Strabismus & Amblyopia ...... 10 Colour Vision Deficiency ...... 10 Nystagmus ...... 11 Saccade Initiation Failure ...... 13 Cerebral Visual Impairment ...... 14 Implications for Visual Displays ...... 16

3. Auditory Disabilities ...... 16 Overview ...... 16 General Hearing Loss and Consequences ...... 17 Measuring Hearing Loss ...... 19 Sensorineural hearing loss (SNHL) ...... 20 Conductive hearing loss ...... 20 Otitis media ...... 21 Auditory Neuropathy (Auditory Agnosia) ...... 21 Hyperacusis...... 22 Tinnitus ...... 22

4. Language delay or deviation: ...... 22 Overview ...... 22 Congenital language disorders ...... 23 Specific Language Impairments ...... 24 Children with multiple physical and sensorial (MDs) disabilities ...... 24 Bilingual children and ‘normal’ language delay ...... 25

5. Specific Learning Difficulties ...... 26 1 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Dyslexia ...... 26 Phonological Approach ...... 27 Programs that work (from http://www.dyslexia-reading-well.com/dyslexia-treatment.html): ...... 28 Auditory Aspects ...... 28 Visuo-Spatial Function ...... 30 Dyspraxia ...... 36 Effects ...... 37 Diagnostic ...... 37 Interventions ...... 37 Bottom-up Approaches ...... 38 Top-Down Approaches ...... 38 ...... 39 Neurobiology ...... 40 Genetic ...... 40 Environmental ...... 40 Interventions ...... 41 Learning styles ...... 42 Maths anxiety ...... 42 Language impairment ...... 43 Visuospatial impairment ...... 43 Memory impairment ...... 44 Dyscalculia guidance summary ...... 44 Benefits of digital technologies ...... 45 Examples of digital interventions which could be of help in dyscalculia ...... 46

6. References ...... 49

2 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

1. Preamble

Terms There is no agreed definition of learning problems encountered by children. Terms such as “learning difficulties”, “learning disabilities”, “intellectual disability”,“cognitive disability” , “mental retardation” are used variablyby stakeholders across countries. Here we will us the umbrella term “Learning Difficulties” (LDs) throughout to encompass all children who have difficulties in learning for any known or unknown reason, and regardless of IQ. LDs are often labelled as being “mild”, “moderate”, “severe”, and “profound”. There is no hard and fast definition of these terms and the borders between them are open to interpretation. But broadly they are understood to reflect increasing severity of LD ( from the British Institute of Learning Disabilties (http://www.bild.org.uk/))

 Mild: IQ 50-70. People (adults) are usually independent and able to hold a conversation and communicate most needs and wishes. Basic and writing is possible, but some support may be needed to understand complex ideas. Often mild LDs are ‘undiagnosed’.  Moderate: IQ 35-50. More severe than “mild”. Language skills are present enabling communication about day to day needs. Some support for caring for themselves may be needed.  Severe: IQ 20-35. Communication with basic words and gestures. High level support needed for everyday activities  Profound: IQ <20. May have other disabilities.

In addition there are people labelled with Specific Learning Difficulties, such as dyslexia, dyscalculia, and dyspraxia. IQ is claimed to be normal (70-130). An over emphasis on labelling LDs and measuring IQ tend to neglect the complex multi- dimensionality of LDs including other factors such as:

 Socioeconomic status  Ethnicity  Multilingualism  Medical History  Family history  Modality specific problems (sensory motor deficits, SMD)  Specific Learning Difficulties (SLD)

An important constraint is the degree of (lack of) knowledge and understanding of a child’s LDs by teachers and parents.Children who have migrated from within and from without the EU may have no medical or educational history. In any case, medical histories

3 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

are often not available to teachers.Poor learning can also result from poor education, and low family aspirations. Thus, our working assumption is that for any given child in mainstream school, LD may be present for any unidentified reason, and that a teacher cannot rely on a priori information about the child. Inclusivity For years, many EU countries have had some form of inclusion for children with special educational needs in mainstream schools. In 1996 The European Agency for Special Needs and Inclusive Education was formed, and by 1999 was funded collectively across the EU and in 2015 had 29 member countries (including the UK) [https://www.european-agency.org]. Despite the this commitment, there is still considerable variability across EU member countries [http://europa.eu/rapid/press-release_IP-12-761_en.htm] with varying percentage of pupils in segregated special needs schools. Thus, it is not possible to delineate the level of mental and/or physical disability that disqualifies/qualifies a child for entry into a mainstream school. However, children with severe and profound LDs would be unlikely to attend mainstream school. Statistics from the UK government provides an example of the prevalence of LDs schools. In the UK, children of all ages are identifiedwith special educational needs (SEN) and a proportion are given an individual education, health and care plan (EHC) (formerly a Statement of Needs). From 2010 to 2016 there has been aslight decline in the number of children with SEN from about 21% to 15% of all pupils in school. Those with a statement of EHC comprise 2.8%. The breakdown of LDs in UK state-funded mainstream and special schools is

4 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein. Figure 1: Breakdown of LD type in mainstream and special schools. From https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/539158/SFR29_2016 _Main_Text.pdf

Constraints The goal of this project is to develop an adaptive learning system to improve a child’s learning abilities via the use of dedicated device in the form of a “tablet” (i.e. a portable hand-held device that can be managed by a child), which we will call ALSTAB(Eric – I am not sure what you want to call it). It is envisioned that ALSTAB will be deployed in mainstream schools as decided specifically by teachers. This imposes constraints on the type of adaptive system and the recipient child. The ALSTABis a one-to-one device and requires focal attention to the device rather than to the teacher or classroom. It also depends on avoiding distraction from other sources (such as other pupils) and clearly may present problems for children with attentional issues. ALSTAB is also highly dependent on the visual and auditory modalities. This implicitly requires that the child can assess the presented visual and auditory information. Whilst this requirement also exists in a normal classroom, with ALSTAB there is no teacher continually monitoring a child’s behaviour.Thus, it becomes crucial to ensure that the child can engage with the presented material and not hindered by sensory deficits. Sensory Deficits A central problem in understanding LDs is distinguishing between cognitive impairments and sensory deficits (figure 1). ALSTAB provides a unique opportunity to ‘diagnose’ sensory deficits independently of LDs and to provide adaptations to reduce or avoid sensory deficit for the individual child, which is not possible in the classroom.

Figure 2: Venn diagram depicting overlap between sensory deficits and learning difficulties

5 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

For example, middle ear disease (otitis media with effusion (OME)) (conductive hearing loss) affects at least 75% of children at some time before the age of 6 years. There is considerable evidence that persistent OME with surgery is associated with delays in speech and language acquisition, cognition, and general education (Holm & Kunze 1969; Teele at al, 1984; Bess, 1985). There is some evidence that OME may be correlated with socioeconomic status (Smith & Boss, 2010) (outside the EU), so other factors may be at play as well. Children with mild bilateral and unilateral sensorineural loss also suffer from difficulties understand speech (Bess, 1985). Clearly, the auditory sound levels can be adjusted adaptively to suit the individual child, which is not possible in the normal classroom. Another revealing example is the relationship between colour vision defects (CVD) and LDs. Numerous studies have shown that children with CVD tend to achieve less than children with normal colour vision (Espinda, 1973; Litton 1979; Grassivaro Gallo et al., 1998), and children with LDs have higher than normal prevalence of CVD (Dwyer, 1991). The majority of CVDs are caused by mutations on two opsin genes on the X chromosome, and are not related to any other clinical condition (Deeb & Motulsky, 2005). Thus, the genetics of CVD cannot be causal in the genetics of LDs. It is possible that children with LDs are more difficult to test for CVD, hence leading to false positives for CVD, but Dwyer (1991) has argued against this. The most likely explanation is that the high reliance on coloured material (including computer displays) in teaching young children leads to poorer educational achievement in the colour blind child. It is conceivable that a child with very mild LDs may be disadvantaged by CVD, thereby pushing the child into a less mild LD category. In any case, since 8% of males have CVD (Deeb & Motulsky, 2005), it is important to test for CVD in any child with suspected LDs. It is also important to note that school children are not routinely screened for CVD, and in the UK, CVD is not even considered a clinical disorder. Thus, it likely that a child (and parents) will not be aware of their CVD.However, it is possible to adapt colour in the ALSTAB to eliminate any colour confusion. There are numerous specific sensory deficits, which we now identify separately as visual and auditory (although some children may have deficits from both).

2. Visual Deficits Overview It is well known that children with ‘delayed development’ have a much higher than average incidence of visual deficits, including visual impairment (VI) (i.e. uncorrectable low visual acuity; VA) (Copper & Schappert-Kimmijser, 1970; Warburg, 1975; Warburg et al., 1979; Tuppurainen 1983; Haussler 1996; Kwok et al. 1996; Chang et al., 2005; Nielsen et al, 2007a), and refractive errors and strabismus (Fletcher & Thompson 1961; Byron 1962; Bankes 1974; Woodruff, 1977; Tuppurainen 1983;Kwok et al. 1996; Chang et al., 2005;Nielsen et al, 2007b). The reason for this is varied and may reflect genetic causes, chromosomal abnormalities, brain disorders, prematurity, perinatal insults, and infectious 6 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

diseases. Nielsen et al (2007a) also reported ethnic differences with an over-representation of children originating from the middle- and far-east, and eastern Europe, which especially relevant to current human migration into the EU. In the UK, the impact of VI on learning has been extensively reported on by the Royal National Institute of the Blind (RNIB) (Chanfreau & Cebulla, 2009; Bassett 2010). The general findings for children with only VI are that educational attainment falls behind from reception (age 4 years) and this attainment gap increases until Key Stage 3 (ages 11- 14years), and remains the same thereafter. Reading, mathematics, and science are all affected.VI has other effects on school children including emotional well-being and social activities (Harris et al., 2014). When VI is associated with other disabilities, the effects of VI are considerably worse. It should be noted that the effects of visual deficits on cognitive processing is generally poorly understood. Low IQ (‘developmental delay’) is often used as a measure, but this not adequate for ‘special’ learning difficulties where IQ is defined (or assumed) to be normal. However, the causal relationships among IQ (especially how it is assessed), VI, and learning difficulties (LD) are poorly understood. For example, in dyslexia the emphasis in the most recent 10 years has been on poor phonological deficits (Castles & Coltheart, 2004), even though the primary input is visual orthographic text. As pointed out by Whitney & Cornelissen (2007), poor phonological awareness may be the symptom rather than the cause of dyslexia. Abnormalities of visual processing (and eye movement control) may be at the root of dyslexia, especially in the young early reader. We present below a range of known ‘clinical’ visual deficits that need to be considered as possibly contributory, or even causal in some instances, for learning difficulties. It is crucial to recognise that visual deficits in children in mainstream school may not be known to teachers for many reasons, including lack of medical assessment, socioeconomic causes, poor institutional communication between the educational and medical professions, different national systems in the EU, and statutory limitations. For example, in the UK, a colour vision deficit is not considered a disability, and is therefore not assessed at any age (in spite of 8% of boys being affected). Thus, it must be assumed that a child with LD could have one or more visual deficits. Most studies and reports on visual problems have focussed on either VI defined as low visual acuity (VA; poor resolution), refractive error (blur due to defocus), and strabismus (eye misalignment). These abnormalities can be quantified objectively and form the basis of any standard clinical assessment. There are many other visual deficits that also have impacts on vision, such as colour vision deficits, eye movement disorders and cerebral visual impairment (brain injury). These require specialist assessment and are not readily quantified by a single number (as in VA for example). Nevertheless, their impact may be more profound.

7 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

To understand this, it is important to recognise that much of visual processing (reading, looking at pictures, etc.) requires the control of a complex sequential process of foveation and processing visual content. Visual resolution is maximum at the retinal fovea (central vision), so that the image of a small feature must be imaged on the fovea of each eye. If the image is blurred due to poor optics (refractive error) or the retinal fovea is damaged (VI), the ‘visual’ image is poor and limits the ability for the child to detect or recognise the object. For example, the ability of children to recognise words decreases with smaller print size (Cornelissen et al. 1991) (with or without dyslexia). If, however, the image falls away from the fovea due to poor eye movement control, then a similar deficit will occur. Retinal signals are passed to the primary visual cortex (V1) where conscious seeing takes place, and are then processed by other parts of the brain where recognition and interpretation takes place (the ventral stream), and the guidance of movement takes place (the dorsal stream). Damage to different parts of the brain can lead to specific cognitive deficits (such as poor face recognition, poor letter recognition, poor reaching etc.). Once a specific feature has been processed, it is necessary to re-direct the fovea to the next visual feature (e.g. next word in a line of text), which requires saccadic eye movements. The whole procedure is integrated and usually subconscious, but any difficulty in any part of the process will interfere with cognitive vision and perception In practice, it is difficult to separate the cause and effect of cognitive deficits. For example, poor eye movement control can make reading difficult, but genuine dyslexia can lead to atypical eye movements. In general, a child labelled with a SLD may have a sensory-motor deficit instead of, or in addition to, any apparent cognitive deficit. Visual screening in early childhood is very limited in the range of tests carried out. For example, in the UK colour vision deficits are not considered a disability and not screened for. There is also no certainty that any specific child has undergone screening anyway. Our default position is that a child may have visual deficits until shown otherwise. We next describe some of the more common visual deficits that need to be examined for. Any single or combination of deficits can lead to poor visual function and slow learning in a young child.

Refractive Error Refractive error (RE) (sometimes called ‘ametropia’) is defocus of the image at the retina, which leads to blurry vision and reduced visual acuity (VA). VA is usually measured as the size of the smallest letter (or other optotype) that can be recognised on a chart. RE is measured with a retinoscope when accommodation is temporarily paralysed (cyclopegia). When the image of a distant object falls behind the retina, the RE is hyperopia (or ‘hypometropia’) (long-sightedness). When the image forms in front of the retina the RE is myopia (short-sightedness). If the image formed is in focus at the retina, no RE is present (emmetropia). The prevalence of RE varies widely depending on ethnicity (Rudnicka et al 2010).

8 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

In children, the shape of the lens can be controlled to change the optical power of the eye (called ‘accommodation’). Thus, making the lens more spherical increases optical power and allows the image of near objects to be brought to a focus on the retina. Conversely, making the lens flatter brings far objects into focus. Thus, to some extent RE can be overcome by accommodation, but this can be incomplete and effortful (tiring). RE can be corrected optically by spectacles or contact lenses and is therefore an avoidable cause of visual impairment. Nevertheless, uncorrected RE is the most common cause of poor visual acuity in school-age children. In a study of white children in Northern Ireland, Donoghue et al (2010) reported the prevalence of refractive error in two groups: 6-7year olds and 12-13 year olds. In the younger group, myopia was present in 2.8% and hyperopia in 26%, and in the older group myopia was present in 17.7% and hyperopia in 14.7%. However, even though spectacles had been prescribed, a quarter of them did not wear them at school. Thus, compliance is an important issue. Effects Uncorrected RE leads to loss of VA, blur, and low contrast sensitivity at high spatial frequencies. Myopia and hyperopia need to be considered separately. Myopic children are less affected for near work (such as a display) because the eye can accommodate, but even so, severe myopia can lead to very close work (bringing the display up very close). Hyperopic children may be able to accommodate near targets, but this can lead to eye strain and headache. Children with hyperopic RE tend to have poorer performance on visual cognitive tests (Atkinson et al., 2002), and poorer (Williams et al., 2005). Hyperopia is more prevalent in children with LD than children with no LD (Rosner & Rosner, 1987). Diagnostic RE is diagnosed by refraction by a specialist eye clinician

Uncorrectable Low Visual Acuity (Visual Impairment). Uncorrectable low visual acuity reflects intrinsic anomalies/damage to the visual pathways that cannot be corrected by lenses. It is often called ‘visual impairment’ (but this usage is variable). VI may be due to a retinal or brain disorder (or both). In children with delayed development (IQ<80), Nielsen at al. (2007a) found an incidence of 10.5% which correlated negatively with IQ, (reaching 22.4% for IQ<50). The causes of VI are manifold but most are pre-natal (with most being genetic) or peri-natal (with most due to prematurity). According to Nielsen et al, over half are secondary to brain damage, or cerebral visual impairment (CVI see below). There are very many specific disorders that cause VI, and for a given diagnosis, each child is affected to a different degree. Thus, medical diagnoses are only rough guides to the disability experienced by the child. The commonality is, however, poor contrast sensitivity and low visual acuity (VA) ranging from mild visual loss to total blindness (rare). Note that there may be associated visual deficits, such as nystagmus, amblyopia.

9 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Effect As with RE, the main effect is low VA, poor contrast sensitivity at high spatial frequencies. However, there is no treatment. RE often occurs in addition to VI. Diagnostic VI is diagnosed by an ophthalmologist.

Strabismus & Amblyopia Strabismus is a misalignment of the eyes (usually horizontal). In the UK it also known as “squint”. Strabismus occurs in about 5% of schoolchildren. The most common type (60%) is when one ‘turns in’ (esotropia), but one eye may ‘turn out’ (exotropia). The condition may start in infancy or later. Strabismus can usually be corrected by surgery. A common consequence of strabismus is amblyopia, where the vision of one eye fails to develop (cortically) normally. This is sometimes known as “lazy eye”. Amblyopia can be treated by occlusion therapy (patching) where the normal eye is covered to force the poorer eye to develop, but treatment must start early otherwise the poor vision becomes permanent. Amblyopia can also be caused by unequal RE in each eye or when one is deprived of vision (such as cataract). Effect Strabismus and amblyopia usually only affects vision in one eye. If vision is the unaffected eye is normal, then behavioural vision is near-normal. However, most children have poor or no stereopsis, thus affecting depth perception. For example, wearing 3-D glasses for a 3-D movie fails to give rise to the perception of depth. Diagnostic Diagnosed by a specialist.

Colour Vision Deficiency Normal human colour vision is trichromatic and requires normal functioning of three cone pigments referred to as ‘short’, ‘medium’, and ‘long’ wavelength pigments (or ‘blue’, ‘green’, ‘red’ pigments’). When a pigment is deficient, the terms tritan (short), deutan (medium) and protan (long) are used. The genes for the L and M pigments are on the X chromosome; the S pigment is on chromosome 7. Mutations of the L and M pigments give rise to anomalous colour vision for medium/long wavelengths (red-green colours for a normal trichromat). The vast majority of colour vision deficiencies are inherited, and occur independently of any other cognitive/brain problems. Most are X-linked recessive and affect about 8% of males and 0.5% females of European descent. Children with learning difficulties are just as likely to be affected as any other child.

10 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

The most common deficit is deuteranomaly, caused by an anomalous shifting of the medium wavelength to longer wavelengths. It affects 4.6% of males and 0.4% of females. Complete loss of medium wavelength function is called deuteranopia and affects 1.3% males and 0.01% females. In protanomaly, the L pigment is shifted to lower wavelength and affects 1.1% males, and 0.03% females, and loss of L pigment function is called protanopia affecting 1% males and 0.02% females. Abnormalities of the S pigment, called tritanopia, are much rarer (<0.02%) with autosomal dominant inheritance affecting males and females equally. Effects Colour vision is important for identifying objects and increasing contrast, especially in complex visual scenes. Colour vision is also used connotatively to convey a specific meaning or message (e.g. danger, traffic lights, etc.). The perception of colour depends on the relative absorption of the three pigments, so people with colour deficiency have a different perception of colours that can lead to difficulties in identifying and interpreting objects. For deutan and protan deficits, there is reduced (or absent) discriminability between long wavelengths, that is distinguishing between red – green colours. Diagnostics Difficult to diagnose, and not routine in the UK.

Nystagmus Nystagmus is an eye movement disorder characterised by involuntary oscillation of the eyes. Oscillations may be horizontal, vertical, or torsional (rotary) and may affect one eye or both eyes. There are many types of nystagmus, but they are usually classified as ‘developmental’ and ‘acquired’. Developmental nystagmus has an onset in early infancy and is usually associated with congenital visual deficits, whereas acquired nystagmus can have an onset from infancy (very rare) to adulthood and is associated with neurological disease. In school children, the vast majority of nystagmus is developmental and can be subdivided into two types: Latent nystagmus (LN) (also known as Fusion Maldevelopment Nystagmus Syndrome) and Infantile nystagmus (IN) (also known as infantile nystagmus syndrome, or congenital nystagmus. LN is strongly associated with early-onset strabismus; ~99% of cases of LN have strabismus, and ~50% of infants with early onset strabismus develop LN. Given that 1% of infants develop early-onset strabismus, the incidence of LN is 0.5% (1:200), but many cases go unreported (Harris 2014). A peculiarity of LN is that it becomes manifest or worse when one eye is occluded. LN is horizontal (sometimes with an additional torsional component). LN occurs with unequal vision from birth (see amblyopia), and most cases the nystagmus is always manifest to some degree since vision in one eye is worse (equivalent to partial occlusion).

11 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

IN occurs in ~0.3% of infants, and is associated in 70% of cases with bilateral visual deficits (e.g. albinism, cataracts, aniridia, and many more). In 30% no underlying visual defect can be identified, and the nystagmus is labelled as “idiopathic” IN (Harris 2014). IN is usually horizontal and conjugate (same in both eyes), but there are exceptions. Effects Even though the nystagmus causes the fovea to be constantly moving across the point of regard in a visual scene, children do NOT normally experience the visual world as oscillating (called “oscillopsia”). IN often increases in intensity when the child is under stress, and oscillopsia may then occur, but it is not a serious problem (unlike acquired nystagmus). In terms of VA, it is difficult to distinguish between the cause and effect of developmental nystagmus. Recent data suggests that, even in idiopathic IN, the moving eyes do not reduce VA. Rather, VA is already poor due either to an unknown retinal problem or amblyopia induced by the nystagmus (Dunn et al., 2014). VA is typically 0.3 logMar, but ranges widely. Thus, in this respect, developmental nystagmus should be viewed as an uncorrectable cause of poor VA. Nystagmus has other important and unique effects, however. The intensity of nystagmus in both LN and IN depends on the gaze direction (the direction of the eyes relative to the head), and typically there is a region of least nystagmus called a “null region”. For many the null region is not straight ahead, but to one side or up or down. The child will then adopt an abnormal head posture to compensate by bringing the null region to bear on the region of visual interest. For example, a null region in right gaze is compensated by turning the head to the left. Children with nystagmus often view objects very close (such TVs and computer screens), which restricts the field of view. Foveating a peripheral visual target is usually achieved by a saccadic eye movement, sometimes accompanied with a head movement. Thus, in normal viewing of a visual scene different visual targets are sequentially processed rapidly by an alternating sequence of fixations and saccades (called “visual scanning”). Nystagmus prevents precise foveation, so that viewing a peripheral target takes longer, which is colloquially known as the “time-to- see” phenomenon. It depends on the precise waveform of the oscillations, and is exacerbated by stress (Jones et al., 2013). Thus, nystagmus should be viewed as causing processing delays, although they may not have a cognitive component per se, but simply reflect delay in foveation. Diagnostics Nystagmus can usually be seen clinically, but eye movement recording is necessary to identify the type of nystagmus.

12 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Saccade Initiation Failure Saccade initiation failure (SIF) is a difficulty in triggering saccades (Harris et al., 1996). SIF is also known as “ocular motor apraxia”, or “congenital ocular motor apraxia”, but the condition is not a true apraxia. SIF is distinct from “dyspraxia”. SIF is often considered rare, but this reflects the difficulty in recognising the problem. SIF is associated with a very wide range of clinical conditions from brain malformations, perinatal disorders, neurodegenerative conditions, and a variety of syndromes (Cassidy et al, 2000). The most typical findings on brain imaging are cerebellar malformations and white matter disorders, but in many cases no underlying neurological cause can be found (Shawkat et al, 1995). Regardless of the underlying neurology, most children with SIF also have other issues. Developmental delay is common with late walking and poor motor coordination. Expressive (articulatory) speech delay is very common and may require speech therapy. Other eye movement abnormalities are also common including poor smooth pursuit, inaccurate saccades (saccade dysmetria), strabismus, and also nystagmus less commonly. To overcome the saccadic deficit, many children develop compensatory strategies such as a peculiar head-thrusting and/or blinking which helps to trigger saccades. However, this is not universal, and in any case, they are not easy to detect. Effects The main effect of SIF is the difficulty in shifting gaze from one visual object to another. For most children, this prolongs fixation on the current object and delays refixations. For the severely affected, refixations may be virtually impossible and the child appears not to look at or scan the visual scene. Indeed, infants with severe SIF appear blind. Reading and scanning pictures become difficult and can be misinterpreted by parents, teachers, and psychological assessors as indicating dyslexia, learning difficulties, and even low IQ (Jan et al. 1998). For example, performance on IQ tasks that require many saccades (such as comparing pictures) can be below normal but incorrectly interpreted as low visuo- spatial IQ, which when coupled with speech difficulties can indicate low general IQ, even though cognitive function may be normal. Currently, it is not known how many children with LD actually have SIF. Diagnostics SIF is difficult to test for, and diagnosis is usually made in a specialist paediatric ophthalmology centre. Eye movement recording will show long saccade latencies and prolonged fixations. Without eye movement recording, SIF appears similar to the time-to- see phenomenon in nystagmus. For cognitive visual tasks, it is important to gauge their demand for saccadic eye movements separately from their cognitive content (e.g. comparing look-alike pictures for differences).

13 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Reading may also be slow because of the difficulty in fixating the next word along the line, rather than a problem in comprehension. This is particularly important for left-right languages (rather than vertical languages), as SIF usually only affects horizontal saccades. Children with SIF will sometimes turn a book horizontally in order to read ‘down’ the line. Others will move the book jerkily to the left to reduce the need to make saccades (potentially a very useful diagnostic via hand-held tablets). There is a compelling overlap of SIF with the general condition of developmental coordination disorder (DCD), (viz: oral/speech dyspraxia and motor dyspraxia). Even the use of head movements instead of eye movements has been emphasised: see http://edbydesign.com/learning/learning-disabilities/dyspraxia.html.

Cerebral Visual Impairment Cerebral visual impairment (CVI) (sometime called “cortical visual impairment”) refers to children who have visual problems in processing visual information that is NOT due to problems with the eyes per se (although children with CVI may also have ocular problems). We consider eye movement disorders as separate from CVI. In schoolchildren, CVI usually originates from birth (pre-natal or peri-natal). In particular, there is a strong association of CVI with premature or traumatic birth (e.g. periventricular leukomalacia, perinatal asphyxiation). Postnatal head trauma (e.g. physical abuse, head injury) can also lead to CVI but is less common. CVI may also follow brain tumour, meningitis, hydrocephalus or encephalitis. Effects CVI can be due to damage to the visual cortex and associated cortices involved in processing visual information (as well as other areas of the brain). Damage to the visual cortex (V1) can result in a range of VI from complete cortical blindness to reduced visual acuity (with normal eyes), and various degrees of field defects in which various regions of the visual field are ‘blind’. When associated visual areas are affected, CVI can also lead to cognitive difficulties in:

1. Recognising objects, such as faces (prosopagnosia) 2. Spatial orientation and visually guided behaviour 3. Depth perception 4. Movement perception (akinetopsia) 5. Simultaneous perception (simultanagnosia), and figure-ground separation.

14 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Figure 3 From Dutton et al. (1996)

Simultanagnosia is the inability to see more than one object at the same time, and consequently patients have difficulty in seeing the ‘overall’ picture. According to Dutton et al. (1996), face recognition was more problematic for groups of well-known individuals rather than one-to-one, suggesting a simultanagnostic component as well. Diagnostics There is no simple way to identify CVI. It is usually based on poor behavioural vision with normal eyes. However, this is inadequate when there is an additional ocular problem, or when there are only cognitive deficits. It is also notoriously difficult to predict CVI from medical history or from MRI images. There is clearly an overlap with CVI and LDs, but the prevalence of some degree of CVI in children with LD is not known. Some important signs that suggest CVI are:

a) Not reaching for objects to one side, or looking away from an object/person in order to see them (hemianopia). b) Seeing only objects if they are still (akinetopsia), or for some children when objects do move. c) Poor responses to faces (prosopagnosia) d) Difficulty in responding to objects with a ‘busy’ background (simultanagnosia).

15 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

e) Looking for an object and then purposefully looking away before reaching for it (optic ataxia).

Implications for Visual Displays To maximise a child’s performance it is necessary to adapt a visual display to the child’s visual abilities. Assuming no eye movement recording ability, the main visual dimensions that need to be adapted and hence ‘diagnosed’ are:

1) Colour content 2) Minimum object size 3) Sensitivity to object density 4) Response time allowance 5) Pointing accuracy 6) Ability to recognise/discriminate faces

[ 7 Care with objects in depth (disparity) ] Interestingly, in a recent design of therapeutic visual games for children with CVI, Waddington et al. (2015) employed adapted control of (2), (3), and (4). They also recommended san-serif fonts (e.g. Arial), voiceover or audio feedback when the cursor or finger (touchscreen) is over a usable icon, and simplifying or removing visual backgrounds. It is important to recognise that these adaptations are not independent of each other. For example, a visual object with low visual contrast (from the child’s perspective) takes longer to respond to. Using red vs. green objects for diagnosing minimum object size would be counterproductive for a colour blind child. A child with simultanagnosia will not perform well on any task with a crowded display. A child with prosopagnosia will not perform well on a task that requires the discrimination of faces, and so on. Given that a child’s abilities are unknown at the outset, care is needed in the ‘diagnostic’ tests and their sequence of administration.

3. Auditory Disabilities Overview For humans the primary, though not the only, use of the sense of hearing is in social communications. For this reason, we focus primarily on the impact of hearing difficulties on language acquisition and development and social communications. Although a delay in language acquisition does not necessarily lead to grave consequences, age-appropriate verbal communications with adults and peers also promotes the development of general cognitive and social skills, so any language delays can impair other skills too, even those not directly linked to hearing. In addition, problems with language acquisition can be indicative 16 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

of more serious developmental issues such specific language impairment, dyslexia or autism spectrum disorder. Therefore, early diagnosis of hearing impairment is important and has given rise in many countries to neonate hearing screening programmes. In considering auditory disabilities we distinguish between afferent problems that relate primarily to mechanistic audibility; i.e. whether and to what extent the acoustic signal is received and processed within the cochlea and correctly transmitted to the rest of the auditory system via the auditory nerve, and integrative problems that apply to the perception of complex sounds in general. In the next section problems relating specifically to language impairment will be discussed. The auditory system is rather different to the visual system in that a great deal more processing occurs before the signal reaches the cortex. In addition, there are no good animal models of auditory cortical processing due to the huge expansion of communication sounds in humans. Consequently rather less is understood about the cortical processing and

representation of sounds than visual images. Figure 4 shows the key processing stages of the subcortical auditory system.

Figure 4. Key processing stages in the auditory system. Left: the anatomy of the hearing organ, the ear. Right: Processing stages in the subcortical auditory system, from the organ of Corti (lower right), located within the cochlea, to the primary auditory cortex (top right).

General Hearing Loss and Consequences

17 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Due to the huge dynamic range of sound intensities which we can hear, perceptual sensitivity is usually measured in terms of decibels (dB), the unit of relative sound pressure level (where the reference pressure in air is set to the minimum typical threshold of perception, 20 mPascals). Figure 5 shows the frequency sensitivity of normal hearing and

the sound levels of typical everyday sounds. Figure 5. Frequency dependence of hearing sensitivity in normal listeners and the typical sound levels of some exemplar sounds. Hearing loss is usually expressed in terms of the number of decibels (dB) needed to amplify a sound above normal hearing levels at a number of pure tone probe frequencies (typically 125, 250, 500, 1000, 2000, 4000 and 8000 Hz) before it is audible; the larger the amplification the worse the loss. Levels of impairment are classified as mild (26-40 dB), moderate (41-70 dB), severe (71-90 dB) and profound (> 90 dB) (Alzahrani, Tabet et al. 2015). People with severe or profound hearing loss are commonly referred to as deaf and those with mild or moderate hearing loss as hard of hearing. Hearing loss has a number of functional consequences for children, depending on the severity of loss and the frequencies affected; e,g, as consonants tend to be high frequency noisy sounds that are affected more by high frequency loss than vowels. For mild loss, nearly all speech can be heard in quiet, but some sounds, especially the relatively quieter consonants may be misheard in noisy environments unless the child is looking at the speaker. For moderate loss, there is some difficulty in hearing others even when they are close by. For this reason, children need visual cues (lip-reading) to guide hearing, and when speaking may miss some word endings and articles (e.g. a, the). Hearing aids and visual cues are essential for those with severe loss, and their speech is markedly affected too. For the purposes of this project, we assume that any hearing loss in the children using the proposed adaptive system will not exceed mild to moderate loss. Communication difficulties caused by childhood hearing impairment can have long term impact on social skills and academic achievement. Observable effects on social behaviour include difficulties in comprehending communicative intent, frequent requests for repetition of messages, abnormally intense focus on the face of lips of a speaker, failure to

18 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

focus attention on a speaker, a tendency to speak more loudly than appropriate, and ultimately social withdrawal. In studies of preschool hearing impaired children it has been found that they had fewer and shorter social interactions than normal hearing children (Brown, Rickards et al. 2001, Brown, Bortoli et al. 2008) and they often had fewer friends and felt rejected or neglected more often than their hearing peers, leading them to feel isolated and lonely ((Wauters and Knoors 2008). In addition, children with hearing loss often have associated vestibular and motor problems (e.g. balance deficits and clumsiness) (Rajendran and Roy 2011, Rajendran, Roy et al. 2012). Measuring Hearing Loss Hearing loss can be measured in a number of ways, and include both subjective and objective measures (Smith, Bale et al. 2005). Subjective tests included pure tone audiometry described above and behavioural tests, although need to be adjusted according to the maturational state of the child. Physiological (objective) measures include auditory evoked responses, otoacoustic emissions, and auditory steady-state response, and tympanometry; all of which assume normal middle ear function. Tympanometry is used to assess functioning of the middle ear. Otoacoustic emissions are sounds originating within the cochlea that are thought to activity of outer hair cells. Transient evoked otoacoustic emissions (elicited by short sound bursts) are generally absent when hearing loss is greater than 40–50 dB, except in the case of auditory neuropathy, which is characterised by the presence of otoacoustic emissions and the absence of a normal auditory brainstem response. The auditory brainstem response (see Figure 6) is a stimulus evoked electrophysiological measure of activity in the auditory nerve (VIIIth cranial nerve), and provide a clear indication of various processing stages in the subcortical auditory system. The auditory steady-state response is another electrophysiological measure which assesses the extent to which brain responses phase lock to the stimulus.

Figure 6. Auditory event related potentials, and the auditory nuclei thought to correspond each peak in the evoked response. 19 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Sensorineural hearing loss (SNHL) Sensorineural hearing loss generally arises from damage to, or abnormalities of, the hair cells of the organ of Corti, although occasionally the problem originates in the auditory nerve or more central areas. SNHL can be congenital, having a genetic basis, or acquired, caused by factors such as infectious diseases (e.g. rubella, bacterial meningitis) and noise trauma(Smith, Bale et al. 2005). Effects Hearing loss depending on severity can affect social skills, academic achievement, employment prospects, and even life expectancy (Carvill 2001). The consequences for children with mild hearing loss that remains stable and only affects a few frequencies is not well understood, as it may remain undetected for some time (Smith, Bale et al. 2005), but there may be societal and scholastic effects that are not properly appreciated. Recent studies have shown around 7% of 7 year olds may suffer from mild high frequency hearing loss which can affect perception of some speech sounds. Diagnosis Usually by a trained audiologist using one or more of the methods described in the section, 'Measuring hearing loss'. Conductive hearing loss Conductive hearing loss refers to any condition which impairs the transfer of sound energy from the world to the cochlea, including fluid in the middle ear, infections in the ear canal or middle ear, poor eustachian tube function, perforated eardrum, impacted earwax (cerumen)absence of malformation of occurs when there is a problem conducting sound waves anywhere along the route through the outer ear, tympanic membrane (eardrum), or middle ear (ossicles). Effects Conductive hearing loss increases hearing thresholds, i.e. the sound level necessary to detect a faint sound. Diagnosis Usually by a trained audiologist using one or more of the methods described in the section, 'Measuring hearing loss'. To distinguish sensorineural from conductive loss a differential test such as one or more of the ones in the table below is used.

Criteria Sensorineural hearing loss Conductive hearing loss Anatomical Inner ear, cranial nerve VIII, or more Middle ear, tympanic membrane, site central processing nuclei or external ear Weber test1 Sound localizes to normal ear Sound localizes to affected ear Rinne test2 Positive Rinne; air conduction is better Negative Rinne; bone conduction is

1The Weber test uses a tuning fork touched to the midline of the forehead. 20 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

than or bone conduction better than air conduction Sensorineural and conductive hearing loss comparisons Otitis media Otitis media refers to inflammatory disease of the middle ear which may be acute or chronic (otitis media with effusion (OME), chronic suppurative otitis media). The chronic forms of the disease seem to be related to viral respiratory infection or allergies, and worldwide affects about 80% of children at some point in time under the age of ten (Monasta, Ronfani et al. 2012, Minovi and Dazert 2014). Effects The chronic forms of the disease may result in hearing loss that affects the child's social communications (Minovi and Dazert 2014). Diagnosis Since chronic otitis media does not respnd to antibiotics, it is important to distinguish it from acute otitis media. Diagnosis is typically performed by general practitioners, otolaryngologists or paediatricians. Auditory Neuropathy (Auditory Agnosia) Auditory neuropathy is a general term denoting a failure in the transmission of sound- related signals from the inner ear to the brain. The condition may stem from a variety of causes relating to signal transmission in the afferent pathway, or, for the auditory agnosias, within the cortex. However, the outer hair cells, which are part of the efferent auditory system, appear to function as normal. Auditory neuropathy in children has a strong genetic basis (Manchaiah, Zhao et al. 2011) but has also been linked to perinatal problems (e.g. jaundice, premature birth, low birth weight, inadequate oxygen supply). There may also be genetic links with the disorders, Charcot-Marie-Tooth syndrome (a group of inherited diseases that damage the peripheral nerves) and Friedreich’s ataxia. Effects People with auditory neuropathy may even have normal hearing according to pure tone audiometry, but are unable to recognise or interpret sounds.Medical treatment of auditory neuropathy is not currently available. Some children benefit from teaching that focuses only on learning to listen and speak. However, sometimes a child with auditory neuropathy has great difficulty understanding what is heard and benefit more from visual communication approaches. Diagnosis The key diagnostic is a negligible or abnormal auditory brainstem response in combination with normal otoacoustic emissions.Diagnosis is typically performed by general practitioners, otolaryngologists or paediatricians.

2The Rinne testtests air conduction vs. bone conduction. 21 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Hyperacusis Hyperacusis refers to the over-sensitivity to sounds(Coelho, Sanchez et al. 2007), which may even be painful at normal levels. Certain groups of children, e.g. those with Autistic Spectrum Disorder or Down’s syndrome, are particularly prone to hyperacusis, which may also be accompanied by tinnitus. In some cases there hyperacusis may exhibit as an intense dislike or fear, termed phonophobia. Effects The main effect of hyperacusis is an emotional one, common signs include crying in noisy environments, clasping hands over the ears, fear of noise or noisy objects, or even self-harm when exposed to loud noise, and reluctance to participate in noisy or loud activities. Diagnosis As it is essentially subjective, hyperacusis is difficult to diagnose, but generally a trained audiologist or hearing specialist would be involved. Tinnitus Tinnitus is the perception of sounds, including buzzing, ringing or noise,in the absence of external sounds. It is not a disease per se but typically results from damage to the auditory system involving hearing loss, e.g. noise-induced hearing loss, neurological damage (multiple sclerosis), ear infections, oxidative stress, side effectsfrom certain medications(Levine and Oron 2015). Children with hearing loss have a high incidence of tinnitus, even though they seldom report it (Shetye and Kennedy 2010). Effects The effects of tinnitus range from slight to catastrophic according to the impact it has on day-to-day life, e.g. only perceptible in quiet through to sleep disturbance and interference with social communications (Baguley, McFerran et al. 2013). Among those children who do complain of tinnitus, there is an increased likelihood of associated problems such as migraine, juvenile Meniere’s disease or chronic otitis media(Shetye and Kennedy 2010). Diagnosis Tinnitus is generally also a subjective phenomenon which cannot be measured objectively. Generally a trained audiologist or hearing specialist would be involved in the diagnosis. It affects roughly 12% to 36% of normal hearing children and up to 66% of children with hearing loss(Shetye and Kennedy 2010)but there are no effective medications (Langguth, Kreuzer et al. 2013).

4. Language delay or deviation: Overview Children’s language ability develops continually into adolescence and beyond. The vocabulary size and the syntactic abilities also develop across the life span into later

22 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

adulthood. Therefore, the continuous language maturations have lasting effects on reading and writing skills. Continuous language development is based on a complex integration of three major aspects: sensory-motor (e.g. vocal and motor production), neuro-cognitive (e.g. audio-motor attention) and social-emotional (e.g. use of language in interacting and manipulating other people and environments). The typical average language development of a child serves as a base to consider how a language outcome is considered in an atypical development. This helps to identify the differences in term of language delay or language deviation from the normal pathway. There are two main categories of difference: in the first case all sub-areas (e.g. phonology, syntax, semantics and pragmatics) of language are delayed compared to a typical normal or ‘average’ child but the developmental patterns are similar to the language of typically developing children, in the second case, a or deviation, is noted when the pattern of impairments are different across sub-areas. Congenital language disorders In children with congenital developmental disorders the difficulties in language development are paralleled by a pervasive neuro-developmental disorder affecting both linguistic and non-linguistic processing. So, for these children their cognitive abilities are a limiting factor on any development of their language abilities. Children with learning difficulties typically show a later onset of language learning, and the overall level of language ability may be limited(Dick, Leech et al. 2008). Whilst language difficulties are easily identified at early stages, the developmental pathway diverges according to a disorder-specific profile, with its weaknesses and strengths, and is not constant throughout the development of the child. Language development during childhood is affected by a number of factors that include the mental ages (assessed through standard testing), severity of language impairment during the early stages of development, frequency of communicative acts, ability to understand the thought and the intentions of others, and the influence of additional non-linguistic problems. Effects Pervasive cognitive and/or social disorders impact on different areas of language to different degrees depending on the syndrome. For example, with the moderate cognitive impairment, children with Down's syndrome show better visuo-spatial abilities than verbal encoding compared with typically developing children that are matched with the same mental age(Varnhagen, Das et al. 1987, Jarrold and Baddeley 1997, Vicari, Bellucci et al. 2000).However, these strengths and weakmesses are not constant in the child’s life. Diagnostic & Implications For these children constant and regular assessment help to identify at specific moments in time the strengths and weaknesses of the linguistic, cognitive and social abilities of that child. The general rule is to simplify the text adjusting the difficulty to the child’s ability.

23 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Specific Language Impairments Specific language impairment (SLI) is defined as a delay in the understanding and production of language in the absence of known neurological impairment, intellectual disability, hearing loss, autistic spectrum disorders or severe social or emotional problems. The language delay is expressed in itself without any other apparent overt impairment in other areas. However, the delay is manifested in all sub-areas of language including reading and writing. Particularly strong weakness is evident in grammatical morphology (e.g. use of verbs inflections for example the use of the verb tense, and detecting grammatical violations). The problem for children with SLI is that they do not move through the optional infinitive stage as quickly as typically developing children. Evidence for this account comes from studies that have shown that English-speaking children with SLI use morphemes that are unrelated to tense (e.g., regular plural –s, -ing) with much higher accuracy than morphemes that are related to tense (e.g., third person singular –s) (Rice and Wexler 1996). The strict meaning of specific disorder has to be taken with caution as associated non- linguistic deficits such as working memory, motor skills and reaction speed may also be observed. In particular a non-linguistic factor identified is limited phonological working memory, assessed usually by the non-word repetition and sentence repetition task (Paul 2007). Effects Working memory capacity is reduced. Children with SLI have general difficulty in mastering language abilities in all sub-areas, and have great difficulty in understanding grammatical rules and figurative language. Diagnostic & Implications Best practice principles in the UK and USA are delineated by the findings of meta-analyses of early intervention and screening studies, but much work remains to be done(Law, Garrett et al. 2004, Nelson, Nygren et al. 2006). There is a need to regularly assess the language abilities and phonological working memory of the child using the non-word and sentence repetition tests to indicate in which level the child should be placed. Support should be given to assist in improving working memory abilities with particular attention to phonological working memory. For reading and writing, help given is to reduce working memory load using simple words and sentences. In addition appropriate simplification of texts to the level at which the child currently performs is helpful. Children with multiple physical and sensorial (MDs) disabilities For children with multiple physical and sensory disabilities (MDs) such as quadriplegic cerebral palsy, it is often not possible to communicate verbally; speech may be limited, hard to interpret, or extremely effortful. Children with MDs often develop their own basic form of communication using facial expressions, movement, gestures and sounds which may enable them to convey simple emotions, wants or needs to their family or close friends.

24 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Recently, various Augmented and Alternative Communication (AAC) devices have been developed to assist these children in communication; to build upon their existing vocabulary (whether verbal or non-verbal), to form new sentences and to facilitate interactions and social engagement (Sigafoos, W. et al. 2013). Effects Among the various AAC aids, Voice Output Communication Aids (VOCAs) are high technology communication systems that provide a ‘voice’ output for the individual that is easily understood by others. VOCAs allow young people with MDs to use varying methods (e.g., direct selection, pointers, scanning, etc.), depending on their abilities to select what they would like to communicate on their VOCA from an array of options programmed onto the machine. The VOCA then turns their input into a recognisable verbal output or sentence that almost everyone can understand and interpret. Diagnostic & Implications The linguistic, cognitive and social abilities of children with multiple disabilities are severely impaired so that social communication is almost absent. Non-verbal form of assessment tailored for these children should be used (Cattani and Carroll in preparation). Bilingual children and ‘normal’ language delay The bilingual population is increasing worldwide. According to the Office for National Statistics (ONS, 2011) the number of births to non-UK born mothers in England and Wales has seen a marked rise over the last decade. These births accounted for 25.1% of all live births in 2010 compared with 15.5% in 2000. This proportion has increased every year since 1990, when it was just under 12%. It is well-established that simultaneous bilingual children may have smaller vocabularies in each of their two languages when compared to monolinguals learning one of those languages in isolation (Bialystok 2009, De Houwer 2009). They also take a little longer to reach the same level as monolinguals on various grammatical tasks, cf. (Nicholls, Eadie et al. 2011). Nonetheless, amongst the general population the impression remains that one should expect bilingual children to be delayed – even quite dramatically delayed – in the early acquisition of language (Stow and Dodd 2003) and later development of grammar (Nicholls, Eadie et al. 2011). This is considered ‘normal’ delay. Limited expressive vocabulary skills in young children are considered to be the first warning signs of a potential Specific Language Impairment (SLI). However,since there is limited knowledge as to what bilingual vocabulary size should be considered as a risk factor for SLI, the effects of bilingualism and language-learning difficulties on early lexical production are often confounded. It has been found that the extent of English language mastery is strongly predicted by the amount of exposure to the host country (in this case English). Although there is a general consensus that it is usually invalid to compare bilingual children to monolingual norms, Cattani, Abbot-Smith et al. (2014)showed that at and above 60% of exposure to English, 25 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

measured using a language exposure questionnaire (see also (Gatt, O’Toole et al. 2015)), bilingual children are comparable to their monolingual peers and can be assessed using the monolingual norms so that a child with language disorder can be identified. For children with less than 60% of exposure to English, the findings are less clear but the suggestion is to test the child in the additional language. However, the general assessment does not give any indication of areas where the child has full knowledge competence in the vocabulary for that language (for example, at home the topics of conversation might not overlap in what happens at school, so the words within those topics might not be learnt in the host language). Increasing the amount of reading (in either language) on a vast array of topics will help the child to expand their vocabulary. Effects In bilingual language learning environments, the expressive vocabulary size in each of the child’s developing languages is usually limited compared to the number of words produced by their monolingual peers. The large variability depends primarily on the exposure to the language of the host country. Diagnostic & Implications It is difficult to disentangle the effect of bilingualism from the effect of specific language impairment. Vocabulary tests normed specifically for bilingual children do not currently exist. However, there is currently work on designing Cross-linguistic Lexical Tasksfor bilingual preschool children (Haman, Łuniewska et al. 2015).

5. Specific Learning Difficulties

Dyslexia Dyslexia (specific reading disability) is the most well-known SLD, and characterised by difficulties with accuracy and/or fluent word recognition and by poor spelling and phonological decoding ability (Lyon, Shaywitz, & Shaywitz, 2003). Dyslexia affects at least 10-15% of school age children (Vellutiono et al., 2004) with boys twice as likely to be affected as girls (Rutter et al., 2004). Dyslexia occurs even with normal levels of intelligence, education and socio-economic background (Ramus, 2003). Reading is not a natural behaviour, but must be learnt and presumably adapts cognitive functions that may have evolved for other purposes. It is without doubt a complex process that takes years to perfect, involving word identification and language comprehension. Word identification requires recognition of an array of alphabetic letters as a familiar word and retrieving the name of the word and its meaning from memory. Language comprehension is an integration of word meanings into a deeper understanding of ideas conveyed by sentences. Written language comprehension is dependent on word identification, but word identification also depends on comprehension as the ongoing

26 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

expectation of a sentence develops during reading. Nevertheless, in early readers, word identification is the limiting process, regardless of language comprehension, whereas language comprehension becomes the limiting process in advanced readers. There is ample evidence that (developmental) dyslexia is a problem in word identification (Vellutino et al., 2004). Word identification requires discriminating sequences of small visual symbols and translating them into phonemic sequences of sounds (Stein & Walsh, 1997). Over the years, different schools of thought have emerged. One school proposes that dyslexia is a linguistic deficit in which phonemes are poorly constructed from alphabetic sequences - the “phonological school”. There is also some recent evidence that auditory function may also be affected. However, it is difficult to explain the number of other deficits experienced by dyslexics, such as clumsiness, temporal sequencing problems, poor spatial localisation, spatial orientation, and directing auditory and visual attention (Stein & Walsh, 1997). The “visual school”, on the other hand, argues that the deficit lies in the visuomotor system, either as a deficit in visual perception (dorsal stream), visual attention, or in oculomotor control. Both schools claim experimental support, and the pendulum of popularity has swung back and forth between these two approaches for decades. With the advent of fMRI, however, there is now clear evidence that both auditory and visual cortices are modified by literacy training (Dahaene et al. 2010). Some have raised the issue of causality. Do visual deficits lead to linguistic problems, or do phonological deficits lead to visual disturbances? Based on fMRI, OLulade et al (2013) have shown that visual deficits cortical visual area V5 are affected by reading, implying that at least the dorsal stream deficit seen in dyslexics is an epiphenomenon of the dyslexia - not a cause. However, this is controversial, and it has been argued that attentional deficits could arise from poor dorsal stream function, and which could have top-down effects on reading (Gori & Facoetti, 2014). Thus, the debate continues. Phonological Approach Poor phonological processing is a core feature of dyslexia and is consistently related to poor reading (Vidyasagar & Pammer, 2009). Some studies show that up to 100% of sampled (dyslexic) participants (n=16) can be affected by phonological deficits (Ramus et al., 2003). Phonological theories argue that dyslexia is a direct result of cognitive deficits in the processing and representation of those speech sounds (for a review, see Beaton, 2004). However, some cases of dyslexia are not characterised by phonological deficits, but instead by failures in processing irregular words (Castles & Coltheart, 1993). Interventions

The best documented methods for teaching a student with phonological dyslexia are largely based on principles of the Orton-Gillingham approach to reading which was developed in the 1930's by Samuel Torrey Orton and Anna Gillingham. Their approach includes the following six elements:

27 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

1. Personalized: Teaching begins with recognizing the differing needs of learners. 2. Multisensory: Involve regular interaction between the teacher and the student and the simultaneous use of multiple senses including auditory, visual, and kinesthetic (touch). For example, a dyslexic learner is taught to see the letter A, say its name and sound and write it in the air – all at the same time. The use of multisensory input is thought to enhance memory storage and retrieval. 3. Structured, Systematic, Sequential, and Cumulative: Language elements and rules are introduced in a linguistically logical, understandable order. Students go back to the very beginning of their language learning, to lay a proper foundation. 4. Beginning by reading and writing sounds in isolation (phonemes), then blending sounds into syllables and words. Elements of language—consonants, vowels, digraph blends, and diphthongs are introduced in an orderly fashion. Only later, learners proceed to advanced structural elements such as syllable types, roots, prefixes and suffixes. 5. Cognitive: Students study the many generalizations and rules that govern the structure of language. 6. Flexible: Instructors ensure the learner is not simply recognising a pattern and applying it without understanding. When confusion of a previously taught rule is discovered, it is re-taught from the beginning. 7. Personal and Direct: Building a close teacher-student relationship with continuous feedback and positive reinforcement leading to success and self confidence.

Programs that work (from http://www.dyslexia-reading-well.com/dyslexia-treatment.html): • described in Sally Shaywitz's book "Overcoming dyslexia" • the Orton Gillingham approach (described here: http://www.dyslexia-reading- well.com/orton-gillingham.html) • the Lindamood Bell method (described here: http://www.dyslexia-reading- well.com/lindamood-bell.html); it can be said that it incorporates the OG method, plus something more; they have a focus on the articulation and perception of sounds.

Auditory Aspects Over the years, developmental dyslexia has been associated with various deficits, including poorer phonological awareness, speech in noise perception, working memory and attention. More recent work shows some specific temporal processing deficits which can be used as a basis for early screening and pre-literacy interventions. Firstly, those with poorer reading skills show greater variability in the auditory brainstem responses (ABRs) than normal readers (see figure 7) (Hornickel & Kraus, 2013). Variability in ABRs specifically in response to speech sounds, longer latency responses, and higher degradation in the presence of noise (Hornickel et al., 2011) are all factors which would make it harder for the developing brain to form stable representations of the sounds of their native language. These temporal deficits at the time scale of 10's of milliseconds, are interestingly not

28 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

evident in normal hearing tests of peripheral processing or in ABRs to click trains. Secondly, phonological awareness correlates strongly with temporal processing at the time scale of the syllable (~2Hz); a) the ability to entrain to an external temporal signal (beat) is predictive of phonological awareness (figure8) (Woodruff Carr et al., 2014), rhythmic production (remembering and reproducing short rhythmic patterns, ~8 notes) predicts phonological abilities (Flaugnacco et al., 2014), and deficits in neural encoding of the speech envelope is strongly linked to developmental dyslexia (Goswami et al., 2011). While early musical experience has often been suggested as an intervention, e.g. Patel(Patel, 2011), the lack of randomised trials and idiosyncratic training means that conclusive evidence for its efficacy is still lacking (Cogo-Moreira et al., 2012). Nevertheless, in its promotion of sensorimotor integration, engagement with the emotional and reward systems, and use of regular patterns which encourage development of predictive representations, musical training may have beneficial effects. However, these benefits may only be realised within a social context (e.g. improvements in beat synchronisation abilities of 2-3 year olds were only achieved within a social/imitative context (Kirschner & Tomasello, 2009)).

Figure 7. Auditory brainstem responses are more vairable in poor readers than in good readers (from(Hornickel & Kraus, 2013)).

29 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Figure 8. synchronisation to an external beat (tapping) is associated with phonological awareness, from(Woodruff Carr et al., 2014) It is possible that neither school is incorrect. Interaction among sub-networks is typical of biological development, and causality is elusive (Harris, 2011). From the ALS perspective it would not be prudent to back the wrong ‘horse’, and we propose that both phonological and visual attention interventions should be adopted. However, we recognise that visual manipulations are probably more easily implemented than phonological ones.

Visuo-Spatial Function Learning to read is a perceptually taxing task requiring fine auditory, visual and motor skill. Vidyasagar and Pammer (2009) argue that one of the most relevant neurocognitive functions employed during reading are the same top-down visual-spatial attentional mechanisms used for visual search. The visual system consists of two parallel processing streams specialised to perform particular tasks. The dorsal stream is associated with spatial localisation and visually guided action, and the ventral stream is associated with object and form recognition (for a review, see Grill-Spector & Malach, 2004). To read, the dorsal stream employs an attention spotlight which guides visual attention to extract the spatial sequences of letters in words. A gating function in the dorsal stream parses whole and parts

30 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

of words which are input to ventral visual pathway for detailed processing. A deficit in the dorsal stream may result in poorer allocation of spatial attention and thus reading deficits, both of which are characteristic of dyslexia (Vidyasagar & Pammer, 2009; Schneps, Thomson, Chen, Sonnert, & Pomplun, 2013a; Schneps et al., 2013b).

Evidence for visual spatial attention deficits - visual search Vidyasagar and Pammer (2009) argue that dorsal stream deficits in dyslexia should be made apparent using visual search paradigms. They argue that serial visual search requires the dorsal stream to select information in visual scenes for detailed ventral processing, as with reading. In visual search tasks, participants are required to detect a target item amongst a number of distractor items. When time to detect a target is not dependent on the number of distracters, the task is a parallel search task. If time to detect a target is dependent on distracter items then the task is a serial search task. In conjunction search tasks, targets differ from distractor items across two or more dimensions (e.g., orientation and colour) and are serial as time taken to find targets increases linearly with number of distractors. Serial search tasks are thought to tap parietal lobe functioning due to the requirement for shifting attention across space (Jones, Branigan, & Kelly, 2007). Vidyasagar and Pammer (1999) explored serial visual search using conjunction search tasks in reading impaired and age matched normal reading children. The authors report that dyslexic children show impaired performance compared with normal readers when number of distracter items was large. Similar findings are reported by Sireteanu et al. (2008) who found that dyslexic children were impaired on conjunction search tasks, where dyslexic children were faster but less accurate in detecting letter-like stimuli amongst distractors, but slower and less accurate when detecting non-letter-like distractors. However, the search deficits in dyslexic children reported were less apparent in groups of older children (between ages 8-13 years old). Associations between poor visual search in a letter cancellation task and poor reading performance have also been reported by Casco, Tressoldi, and Dellantonio (1998). Another study reports that high performing adult dyslexic readers show poorer performance on a visual search task than normal readers, but also that visual search performance was negatively correlated with phonological decoding tasks of non-word reading (r = -.416, p < .01) as well as rapid automized naming (r = -.275, p < .05). Further, regression analysis showed that visual search performance predicts non-word reading (Jones et al., 2007). These studies provide more direct evidence that visual-spatial attention is related to reading ability, and that deficit in this ability are associated with poorer reading skills observed in dyslexia.We now explore how paradigms have been used to understand differences in the distribution of visual spatial attention between dyslexic and normal readers

31 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Evidence for diffused and asymmetric visual-spatial attention in dyslexia Evidence suggests that people with dyslexia have a broader visual attention gradient, showing poorer inhibition of information from the periphery (Facoetti, Paganoni, & Lorusso, 2000; Facoetti et al., 2006; Geiger et al., 2008; Geiger & Lettvin, 1987; Geiger, Lettvin, & Fahle, 1994; Geiger, Lettvin, & Zegarra-Moran, 1992; Lorusso et al., 2004; Lorusso, Facoetti, Toraldo, & Molteni, 2005; Schneps, Rose, & Fischer, 2007). The peripheral bias in dyslexia was demonstrated by Geiger and colleagues in studies of lateral masking, the inhibition of background and flanking stimuli which enhances foveal information. It is argued that reading problems in dyslexia may result from a mislearned use of lateral masking (Geiger & Lettvin, 2000). To study this hypothesis, Geiger and Lettvin (1987) developed a visual perception task called the Form-Resolving Field (FRF), which measures accuracy of letter recognition across a horizontal axis. Geiger and Lettvin (1987) used a tachistoscope to briefly present adult dyslexic and normal readers with pairs of letters simultaneously to a central fixation point and to eccentricities of 2.5°, 5°, 7.5°, 10° and 12.5° of the right visual field. Successful performance in this task requires accurate identification of both letters and the FRF is found by plotting correct recognition rates at various eccentricities. The study found that ordinary readers show the greatest accuracy in letter recognition when letter pairs were presented within a more central visual angle < 5° with a sharp drop in accuracy as eccentricity increases. However, the surprising finding was the FRF of dyslexic groups differed from normal readers, as they displayed stable recognition rates up to peripheral eccentricities of 10° before dropping at 12.5°. So, the FRF in normal readers is narrow, and in dyslexia the FRF is wider as demonstrated by greaterperipheral accuracy. Similar findings were reported elsewhere (Geiger et al., 2008; Lorusso et al., 2004; Lorusso et al., 2005; Perry, Dember, Warm, & Sacks, 1989) but not all report performance differences between dyslexic and non-dyslexic readers (Bjaalid, Hoien, & Lundberg, 1993; Goolkasian & King, 1990; Klein, Berry, Briand, D'Entremont, & Farmer, 1990). The work by Geiger and colleagues argues that lateral masking (or lack of) is a learned perceptual strategy in reading. The concept of crowding is relevant here as it refers to how we process information in peripheral vision. Crowding is the difficulty recognising letters surrounded by other letters in the periphery characterised by excessive feature integration and inappropriate inclusion of surrounding features during target (e.g., word) recognition. When we read, we have an uncrowded central window through which we can read, and a crowded peripheral window through which we cannot read. Reading is thus limited to the visual span of the central window, which is simply the number of characters that are not crowded (Pelli et al., 2007). Crowding effects are increased in dyslexia, and increased crowing is associated with slower letter processing in children (Spinelli, De Luca, Judica, & Zoccolotti, 2002) and poorer reading and spelling scores in adults with dyslexia (Moores, Cassim, & Talcott, 2011). Enhanced crowding effects may be the result of visual-spatial attention deficits in dyslexia.

32 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Facoetti et al. (2000) present evidence using visual attention measures that visual attentional resources are more diffused over the visual fields in dyslexia. There is also evidence that attention is not distributed equally across visual fields in dyslexia. Spatial cueing paradigms (Posner, 1980) demonstrate dyslexic asymmetry in covert spatial orientation across visual fields. Using a spatial cueing paradigm Facoetti, Turatto, Lorusso and Mascetti (2001) showed that dyslexic children show lack of inhibition of information in the right visual field when they are attending to the left visual field and tend to neglect information in the left visual field when attending to the right visual field. Similar findings are reported using flanker tasks (Facoetti & Turatto, 2000). Facoetti et al. (2006) show that asymmetry in the spatial cueing paradigm predicts impaired non-word reading in dyslexic children, which specifically links deficits in visual-spatial attention to dyslexia. In addition, the FRF is asymmetric in dyslexia (Geiger & Lettvin, 2000; Geiger et al., 1992; Lorusso et al., 2004; Lorusso et al., 2005) where recognition accuracy is higher in the peripheral field which corresponds to reading direction (cf. Geiger et al., 2008). Furthermore, Lorusso et al. (2004) also show that the wider more diffused gradient of visual attention does not differ according to dyslexia subtypes and therefore may be general to the dyslexic population. Attention shifting also appears sluggish in people with dyslexia. For example, dyslexic groups are shown to be slower than normal readers when processing stimuli presented to the left vs. right hemifield (Facoetti & Molteni, 2001). Others report attentional capture is sluggish over both visual fields in dyslexia (Facoetti, Lorusso, Cattaneo, Galli, & Molteni, 2005; Hari et al., 2001). The attention bias and sluggish attention may be intensified during reading. If attention is slow to disengage from previously fixated words the effects of crowding are thus exaggerating (Schneps et al., 2013b). Visual-spatial interventions to improve reading in dyslexia Interventions reviewed here come in two forms. Some aim to train visual spatial attention mechanisms in dyslexic people to operate as those in normal readers. Others aim to modify text displays to provide an optimal fit the visual attention span of those with dyslexia. Some apply both methods as we shall see in the first example. Attention training and crowding interventions Gieger and Lettvin (2000)argue that the differences in FRF are learned task dependent perceptual strategies that can be modified through training. Geiger et al. (1992) shows that the FRF of severe adult dyslexics show little variation over time. However when adult dyslexics practice a new regime which emphasizes use of focussed attention, reading ability improves and the FRF narrows to that of normal readers (for a review, see Geiger & Lettvin, 2000; Geiger et al., 1994; Lorusso et al., 2005). The training regime by Geiger et al. (1992) aimed to teach dyslexic people to 1) mask text surrounding a word and to direct attention to the word to be read, 2) practice reading word by word, and 3) practice the recognition of word forms. The regime consisted of two complementary tasks. Participants were asked to complete small scale, hand-eye coordination tasks like drawing or painting for 1-2 hours a day. Following this, participants were asked to practice reading using a reading aid, a specially designed mask laid on text with a small window isolating single words to be 33 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

read,for 1 hour a day. Participants could shift the window along lines of text, masking surrounding text and thusreducing crowding and allowing greater focus on single word reading. Daily practice for 4-6 months narrowed the FRF and improved reading ability dramatically in severe adult dyslexics (Geiger et al., 1992). In a controlled examination of the new reading strategy in dyslexic children aged approximately 11 years old, Geiger et al. (1994) found that dyslexic children also demonstrate narrowing of the FRF and improved reading ability and that the new regime was more effective than general remedial interventions. The studies by Geiger and colleagues appear to offer an effective albeit time consuming remediation to reading difficulties experienced by people with dyslexia. However, the studies are now quite dated in regards to technology available today. More recent research has identified the value of e-readers in reading interventions, offering new ways of displaying texts better suited for the atypical neural functioning of those with dyslexia. Indeed, people with dyslexia are known to report how reading is easier on e-reading devices (Schneps et al., 2013a). Optimising text displays using e-readers Schneps, O'Keeffe, Heffner-Wong, and Sonnert (2010) argued that reformatting text into narrow columns in e-readers and presenting only a few words per line is better matched to the larger peripheral span of those with dyslexia. A study by Schneps et al. (2013b) looked at the role of e-readers in addressing problems associated with dyslexia, such as poor oculomotor control and deficits in visual-spatial attention span. They used eye tracking to monitor eye movements during reading in high school students with dyslexia whilst they used traditional texts or e-readers. More specifically, the authors were interested in the effects of attention on holding the device in the hand (HAND vs NO HAND), the effects of line width using devices of different size (IPOD vs IPAD), and effects of letter spacing in words (SPACED vs NORMAL). Overall, findings show that using a smaller device (IPOD) improved reading speed by 27%, reduced number of fixations by 11% and reduced regressive saccades by more than a factor of 2. All these improvements came at no cost to comprehension. However, the authors argue that the benefit of device size is entirely down to the device linewidth, since the IPOD displayed 2.19 words per line (12.7 characters) and the IPAD displayed 11.6 words per line (67.2 characters). Increased letter spacing improved comprehension in dyslexic readers, however dyslexic students with poor visual attention span (measured using a six letter global report task - number of letters correctly identified) incurred an oculomotor cost making more fixations which slowed their reading. Another study by Schneps et al. (2013a) compared the speed and comprehensionof reading in high school students with dyslexia (n=103) who used either paper or an IPOD e- reader with modified short line text. They found that reading speed and comprehension was significantly improved in subset of dyslexic readers who read from IPOD with shorter lines. More specifically, those with phonological decoding or sight word reading deficits read

34 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

faster on the IPOD, and those with visual attention span deficits as measured with using the global report task gained in comprehension. Overall, the findings by Schneps and colleagues show that effective interventions are available with modern e-reader technology. Dyslexia is associated with poor oculomotor control, but e-readers with shorter lines of text and larger fonts reduce demands of fixation accuracy. Furthermore, because text scrolling is operated manually, the oculomotor demands for tracking gaze are reduced. Shorter lines also reduce crowding of surrounding text and helps inhibit text previously read. Thisfacilitates reading in with dyslexic people sluggish attention shifting and crowding problems by guiding attention to the uncrowded attention span using a narrower window of text (Schneps et al., 2013a; Schneps et al., 2013b). Fun computer game interventions improve visual-spatial attention Playing action video games has a positive effect on visual-spatial attention. Green and Bavelier (2003) have found that people who play video games (VGP) outperform non video game players (NVGP), showing enhanced attentional resources and greater spatial distributions of attention. Other researchers have also demonstrated that VGP of all ages show enhanced visual spatial attention compared with NVGP (e.g., Dye, Green, & Bavelier, 2009). As video games may have positive effect on attention, Franceschini et al. (2013) explored the effects of video games on children with dyslexia, proposing that action game training should produce improvements in attention which manifest in improved reading abilities. Franceschini et al. (2013) measured pre-post attention and reading ability in Italian dyslexic children assigned to either action video game treatment condition (VGP; n=10) or non-action video game control condition (NVGP; n=10). Gaming took place for 12-hours over 9 days using mini-games in a commercial video game called "Rayman Raving Rabbids". The authors report that children assigned to the AVG treatment group had greatest improvements post-treatment showing faster general reading, pseudo-word reading and word test reading, without decrements in accuracy. In addition, scores in measures of focussed and distributed attention also increased in AVG groups post-treatment. Regression analysis revealed that the attentional enhancements accounted for ~50% of unique variance in reading improvements (R² = .48, p < .01). Franceschini et al. (2013) conclude that AVG training could thus be part of an effective low-resource-demanding early prevention program that could reduce the incidence of reading disorders. However, whilst speed of reading in the children improved following AVG treatment, comprehension was not assessed and so the there is no evidence that AVG actually helps dyslexic children comprehend and understand text (Bavelier, Green, & Seidenberg, 2013).

Visual Spatial Intervention Summary Interventions reviewed take form of attention training, modification of reading materials, or both. The work of Geiger and colleagues shows that a new reading strategy emphasising focused attention and modification of text to reduce crowding can improve reading ability in 35 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

adults and children, albeit at a heavy investment of time and resources. The promising research by Schneps et al. (2013a) and Schneps et al. (2013b) show that manipulating texts on e-readers has value for improving reading speed and comprehension in people with dyslexia. Action gaming also may also provide valuable intervention for training attention systems. Whilst the action mini-games used in the Franceschini et al. (2013) emphasized visuo-motor control, precision aiming and precise motor action, divided attention and planning, a greater understanding how AVG treatment can improve reading will be useful for designing games for fun intervention for children and adults with dyslexia (Bavelier et al., 2013).

Dyspraxia Dyspraxia is a somewhat enigmatic and poorly defined term for children with gross and/or fine motor coordination problems that cannot be explained by a known medical condition (although this does not mean that they have normal neurological function). The terminology used is highly variable, and confusing. Other or similar terms include “clumsy child syndrome”, “developmental dyspraxia”, ”minimal brain damage” (MBD), “developmental coordination disorder” (DCD), “deficits in attention, motor control, and perception“ (DAMP). There may be subtle differences among these terms, but in practice there is little to distinguish them (Gibbs et al., 2007). There is a move to use the term developmental coordination disorder (DCD) (American Psychiatric Association, 2013), but the term dyspraxia is still commonly used (which we will continue here). Dyspraxia is not rare; it probably affects about 6% of children (Gibbs et al, 2007), but estimates vary from 1.4 to 19.0% (Deng et al., 2014), and 4 time more likely in boys. Historically, the child with dyspraxia has been later diagnosed with a wide range of neurological conditions (Gibbs et al, 2007).Supposedly, dyspraxia is not due to a general medical condition (such as cerebral palsy) and is not Pervasive Developmental Disorder (Zwicker et al, 2012).There is also an association with other disorders including dyslexia, dyscalculia, , ADHD and also autism (Dziuk et al., 2007), which can make diagnosis difficult (Barnhart et al., 2003) . Depending on the main type of motor deficit, various subtypes have been identified, including:

 Ideomotor DyspraxiaDifficulty in completing single step motor tasks, such holding a fork or pen, ball throwing, jumping, climbing stairs, carrying books, etc.  Ideational DyspraxiaDifficulty in completing sequence of movements such as tying shoe laces, brushing teeth, getting dressed, writing, typing on a computer keyboard, playing sports.  Oromotor Dyspraxia (verbal apraxia, apraxia of speech) Difficulty in coordinating muscles to pronounce words that are difficulty to understand (even by parents)  Contructional DyspraxiaDifficulty in understanding spatial relationships, such as arranging items, copying/drawing geometric shapes, craft work.

36 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

The cause of dyspraxia is unknown, but prematurity (<37 weeks), low birth weight, a positive family history, and maternal alcohol/drugs during pregnancy are known associations. Periventricular leukomalacia (PVL), a focal brain damage that occurs in pre- term infants, is a known cause of visual and cognitive deficits. When the cortico-spinal tracts are also involved, motor deficits also occur, and interestingly, there is also a strong association with constructional dyspraxia (Koeda et al., 1997). The cerebellum, pre-frontal cortex, and the striatum have been implicated (see Deng et al., 2014). Thus, an underlying brain abnormality is likely. The possibility of some overlap, or confusion, with ocular motor apraxia should be kept in mind.

Effects Motor coordination in daily activities is substantially below normal - given the child’s age and measured intelligence. It includes delayed motor development (late walking, crawling and sitting), clumsiness, poor performance in sports and poor/slow handwriting. The condition is sufficiently severe as to interfere with academic achievement and daily living. Dyspaxia was traditionally thought to be a benign transitory phenomenon of early childhood. Today it is recognised that dyspraxia can have significant social and emotional difficulties, such as difficulty in joining in group activity (e.g. sports), lack of fitness, and awkwardness can invoke bullying and ostracism leading to social withdrawal, behavioural problems, low self-esteem and academic under performance.

Diagnostic Diagnosis can be made by a wide range of health professionals, including paediatricians, neurologists, clinical psychologists, and educational psychologists. A quarter of dyspraxic children will have been diagnosed in the pre-school years, typically instigated by concerned parents, but diagnosis is difficult before the age of 4 years. The remaining 75% emerge in primary school years, when clumsiness persists, and poor handwriting skills do not improve. Thus, it cannot be assumed that the parents or teachers know that a particular child is dyspraxic.

Interventions Numerous interventions have been proposed, and they have been reviewed a number of times (Pless & Carlsson, 2000; Mandich et al, 2001; Barnhart et al., 2003; Polatajko et al., 2006. Unfortunately, the reviews are quite disparate in how the different interventions have been classified, and there is a lack of cross-referencing, implying a lack of consensus. Nevertheless, there is increasing evidence that the more recent “top-down” approaches are superior to the more traditional “bottom-up” approaches.

37 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Bottom-up Approaches The idea behind bottom-up approaches is motivated by theoretical considerations of motor hierarchy. Complex movement are based on simpler motor and sensory components, so that practice/training on components should bring about an overall improvement in motor control at a higher level. However, supporting evidence is weak. Sensory Integration (SI) This approach assumes that motor skills (and other cognitive skills) depend on sensory integration, including proprioceptive, tactile and vestibular input. The key to SI is full body movements and training in specific perceptual and motor skills. This was originally proposed by Ayres (1989), but numerous comparisons have failed to show much if any long term improvement in dyspraxic children (see Mandich et al., 2001). Process Oriented Treatment It has been proposed that dyspraxia reflects a kinaesthetic deficit – the child has reduced sensitivity to her own movements (Laszlo & Bairstow, 1985). Intense practice in kinaesthetic tasks was proposed by Laszlo, as well as positive feedback and adapting to the child’s ability. Children show motor improvement when compared to controls with no intervention, but there is little evidence to support that it is due to the kinaesthetic training per se. Rather it is probably the positive feedback and raised motivation (Sims et al., 1996) Perceptual Motor Training This approach combines both sensory and motor training and claimed to be better than sensory integration and process oriented training, but meta-analysis of many studies does not support this, at least in dyspraxia (Kavale et al, 1983).

Top-Down Approaches The top down approach is to intervene at the task level, rather than at sensory and motor components. The goal is to learn the task. Task Specific Intervention In this approach as task is broken down into steps. Each step is trained, and the steps are then brought together to form the original task. Positive results have been shown (Mandich et al., 2001), although there is little information on the generalisability of this approach. Cognitive Approach Cognitive interventions introduce an active component via the so-called Cognitive Orientation to daily Occupational Performance (CO-OP) approach (Miller et al., 2001; Barnhart et al., 2003):

 Goal: What am I going to do:  Plan: How am I going to accomplish the skill  Do it: Go ahead and perform the skill:  Check: How well didmy plan work? 38 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

The role of the therapist is to guide the child to discover which aspect of the task are presenting problems and how to overcome these problems. This is done by a process of asking questions of the child, such as “what is going wrong?”, “How did you do that?”, What do you need to first?”, or “how might you fix that?”(Miller et al, 2001). Clearly, the key is to overcome the anosognosia of dyspraxia by guided self-discovery. However, it is important to maintain motivation. To our knowledge, tablets have not been exploited specifically for dyspraxic children. Nevertheless, here are many potential avenues to explore. Handwriting and typing skills could be recorded and tested. Pointing can be addressed. Even balance and stability could be recorded via accelerometers. However, a top-down approach (task specific and cognitive) would seem to be the most successful.

Dyscalculia Dyscalculia is a SLD affecting the learning and comprehension of arithmetic and mathematics, in which performance is below that expected for chronological age and IQ, and also not due to poor education. Other terms have been used, such as “mathematical learning difficulty”, “mathematical disability” , “mathematical disorder”, and “developmental dyscalculia”, but these refer to the same condition (Geary, 1993; Geary & Hoard, 2001), We will use the term “dyscalculia”, as this has the most common usage. Dyscalculia is a neurologically based mathematical characterised by a difficulty to understand simple number concepts (such as operations: + - x and /) and to acquire the numeracy skills necessary to understand and apply mathematics (for example multiplication tables and long divisions), all of which manifests in the absence of intellectual disability (Department for education and Skills (DfES, 2001).Some authors have attempted to differentiate between dyscalculia, classed as a severe difficulty with mathematics, and ‘arithmetical dysfluency’ characterised by a more general deficit of mathematical achievement. (Reigosa-Crespo et al., 2011). Whilst some have stated that arithmetic dysfluency represents a more general impairment of which dyscalculia is a subcomponent, so far studies have not found a clear differentiation between these two groups (Murphy, Mazzocco, Hanich & Early, 2007). The prevalence of dyscalculia in school-aged children is between 4% and 10% (Peterson & Pennington, 2012), as shown by school age cohort studies (Gross-Tsur, Manor & Shalev, 1996) and population-based, retrospective, birth cohort studies (Barbaresi et al., 2005). Although this is similar to the prevalence of dyslexia, dyscalculia has received much less attention in terms of research and public interest. Yet, the effects are as profound for individuals and society (Hanushek & Woessmann, 2010, Butterworth, Varma and Laurillard (2011).

39 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Neurobiology In adults, the neural correlates of mathematical and numerical have been well definedprocesses (Dehaene, Piazza, Pinel and Cohen, 2003). However, studies in children are scarce and evidence for the differences in brain function between children with dyscalculia and controls is even more rare. A small review of fMRI studies in children revealed that the frontal-parietal regions are consistently reported as being involved in simple calculation processes (Kaufmann, Wood, Rubinsten & Henik, 2011). Although this review included only a limited number of studies looking specifically at the comparison between dyscalculic children and controls, the authors concluded that the differences in neural activation patterns between these two groups are very distinct. Specifically, during number comparison tasks, children with dyscalculia display a reduced activation of the bilateral interparietal sulci (IPS) (Mussolin et al., 2010; Rykhlevskaia, Uddin, Kondos, & Menon, 2009). However, a study by Price et al. (2007) found that the abnormal activation pattern were inthe right IPS - not in the left. There is supporting evidence from TMS studies which have found that interrupting activity in the right parietal area in participants without any problems in numerical comprehension results in behaviours similar to those encountered in dyscalculia (Cohen et al 2007). Additionally, children with dyscalculia have been shown to recruit distributed brain regions possibly pointing towards a compensatory strategy and display a deficit recruitment of frontal brain regions (Kaufman et al. ;2011; Mussolin et al.,2010; Price et al., 2007). Genetic The role of heredity in the development of mathematical skill was proposed early on (Kosc, 1974). Support for this hypothesis comes from twin studies which show that in 58% of monozygotic and 39% of dizygotic twins, both siblings are diagnosed with dyscalculia (Alarcon, Defries, Gillis Light, & Pennington, 1997). Even non-twin siblings of children with dyscalculia are at an increased risk of mathematical disability, up to tenfold greater than in the general population (Shalev et al., 2001). Some chromosome disorders are also associated with dyscalculia, such as Williams syndrome (Paterson, Girelli, Butterworth, & Karmiloff-Smith, 2005) and Turner’s syndrome(Butterworth et al., 1999). Molko et al. (2003) have reported abnormalities in the right IPS in people with Turner syndrome, who have particular difficulties with subtraction and large numbers. They suggest that at least in the context of Turner syndrome, abnormal IPS structure may result from an X-linked gestational problem. Environmental Parenting environment has also been highlighted as contributory factor in the acquisition of numeracy and mathematics. Studies assessing children and parents attitudes to learning mathematics found that parent’s apprehension in relation to mathematical tasks influences their child's approach to such activities (Young-Loveridge, 1989); Aning and Edwards, 1999). In highlighting guidelines for the interventions in dyscalculia, Hannell (2005) states that parents can enable children’s learning by promoting a positive attitude towards

40 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

mathematics. Socio-economic status has also been linked to challenges in learning mathematics (Sammons et al., 2002). Interventions Since dyscalculia is a brain-based disorder, interventions trying to address these deficits should be derived from a very specific understanding of cognitive deficits and neuronal abnormalities. Such understanding should equally concern individuals that have to action interventions, such as administrators, parents and teachers (Goswami, 2006), as well as researchers that hope to develop them. It has already been highlighted that one of the most pertinent ways to drive research forward in this field is through interdisciplinary collaborations (Kroeger, Brown & O'Brien, 2012). Butterward and Laurillard (2010) present a broad interdisciplinary strategy for the development of such interventions (Figure 1), this emphasises the cyclical nature of interaction between the different disciplines that call equally contribute to development of targeted interventions.

In the following section we will review strategies known to benefit children with dyscalculia whilst also highlighting the cognitive factors they relate to. Both individual factors such as learning styles and maths anxiety as well generic impairments such as language, visuospatial and memory deficits will be mentioned.

41 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Learning styles Whilst children with dyscalculia will display a range of learning styles, Kay and Yeo (2003) suggest that dyscalculic learners are more likely to display a more sequential learning style (i.e. “inchworm” approach). When analysing a problem, inchworms will tend to focus on the parts rather than on the whole, they might constrain focus by using a single method, work in serially forward ordered steps and writing down information rather than using mental calculations. Inchworms will also tend to work mechanically and not understand procedures or values of numbers ( Marolda & Davidson,2000). This suggests that children with dyscalculia will have a preferred way of working. An awareness of learning styles can help structure and personalise interventions, but teaching strategies should not be solely focused on one style. Chin (2001) suggests that at first a child should be taught using their preferred style in order to avoid failure, but once confidence is achieved it is advisable that teaching should make use of complementary strategies. In this particular case, children should be taught to expand their focus when solving problems by being introduced to different methods in order to enhance theunderstanding of numbers and relationships. Children with dyscalculia are comfortable with the familiar, so any such strategies should be introduced gradually.

Maths anxiety Dyscalculia can have detrimental impact on children's emotional attitude towards mathematics. The difficulties children encounter might affect their confidence and hence their motivation to participate successfully in activities that involve mathematics. Children with dyscalculia have been shown to display anxiety when confronted with mathematical tasks(Rubinsten & Tannock, 2010). Maths anxiety is defined broadly as a negative affective response to mathematics experienced when required to solve a mathematical problem (Tobias & Weissbrod, 1980). In order to help learners overcome maths anxiety, effective interventions need to foster self-confidence. Hannell (2005) recommends ample opportunities to successfully practice a skill, by ensuring the level of difficulty of a problem matches the student's ability, and allowing children to select the level of difficulty they feel comfortable with. Additionally, in order to motivate students, the purpose and usefulness of mathematics should be emphasised by including activities that connects mathematics to everyday life such as counting change and going groceryshopping (Geist, 2010; Sun & Pyzdrowski, 2009; Jackson, 2008). However these must be appropriate to students life in order to be effective. Wadlington and Wadlington (2008) suggest that in order to combat maths anxiety, it is equally important to encourage students to celebrate success and allow them to map their own progress by providing chat and graphs. Individual differencesin learning and emotional styles as highlighted above, accentuate the need to target interventions toward a child’s particular difficulties (Dowker, 2009).

42 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Individualised interventions have indeed been found to be highly effective; however a meta- analysis of single-subject design interventions in participants with learning difficulties, including dyscalculia, found that the particular method of intervention largely determines the degree of effectiveness (Swanson & Sachse-Lee, 2000). For example, interventions that explicitly conveyed how and why a particular strategy was being used were more successful than interventions in which the strategies being used were passively communicated. Furthermore, the effectiveness of individualised interventions have also been found to dependent on the availability of specialised training available to teaching staff (Dowker, 2009). Another downside of individualised interventions might be that they have the potential to be lengthy, although, Dowker (2009) suggests that they do not need to be very time-consuming or intensive to be effective. Languageimpairment Mathematical thinking is greatly intertwined with language ability. This hypothesis is supported in equal measures by neuroimaging studies which have shown that numerical tasks also activate language areas (Cipolotti, & Harskamp, 2001),by developmental studies showing that counting works are necessary for counting further than four ( Gelman& Gallistel, 1978), and most interestingly by cultural studies which have found that Amazonian cultures which lack words for exact numbers larger than 5 have difficulties representing such numbers (Gordon , 2004). Although there are authors that challenge this view by highlighting that whilst language facilitates numerical concepts it does not necessarily underpin it (Gelman & Butterworth, 2005), language ability should be taken into account when developing interventions dyscalculia,as the disorder often occurs in concurrence with disturbances in the field of language (Silver, Pennett et al 1999 - Stability of arithmetic disability subtypes) For children with dyscalculia this implies that they may not understand the language they recite and that they may not be able to use internal language to help with mathematics (Hannell, 2005). This constitutes a substantial challenge in the development of dyscalculia interventions. In order to address these deficit and enable students to communicate effectively in relation to mathematics, Wadlington and Wadlington (2008) suggest that new terms should always be explained using concrete material in order to make number concepts meaningful. Students should also be allowed to develop their own dictionaries in order to illustrate new terms. As language disability might also impair dyscalculic children’s ability to monitor thinking and learning through internal language-related thinking, interventions should prompt students to write in a journal entries in order to allow them to make sense of their successes, difficulties and thought processes (Wadlington and Wadlington (2008). Visuospatial impairment Deficits in visuo-spatial skills have also been associated with dyscalculia (Geary, 2004; Szucs, Devine, Soltesz, Nobes & Gabriel, 2013). Children with dyscalculia have been found to perform worse than controls on tests of attention and visual-spatial processing (Shalev,

43 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Auerbach, and Gross-Tsur, 1995; Lindsay, Tomazic, Levine, and Accardo, 2001). Recently a study by Crollen and Noel (2015), compared children with high and low visual spatial ability,and found that those with low abilities performed worse in relation to basic numerical processing tasks such as number-to-position task which assesses the understanding of representations of numerical value. Consequently, children with dyscalculia may display a deficit concerning calculations which require logic dependent on holistic, spatial reasoning. As part of his Dyscalculia Toolkit, Bird (2013), suggests the use of Cuisenaire Rods in order to emphasise the relationships between numbers and therefore facilitate the construction of a coherent number system. Visuospatial impairment in children with dyscalculia also means that they will have difficulty with shape and space. For example they might struggle with 2D representations of 3D shapes, and may find it difficult to copy or draw shapes accurately. In order to support drawing, Dowker (2009) recommends activities such as joining dots and modelling in plasticine and the use of specialist equipment when drawing e.g. rulers, templates, curves. Additionally 3-D shapes should always be presented in conjunction with 2-D representations. Memory impairment Multiple studies have established that children with dyscalculia experience memory deficits (Wilson and Swanson, 2000; Geary, 2004). These deficits relate to working memory as well as long-term memory. Working memory refers to the temporal capacity to process and store information. Poor working memory implies that children with dyscalculia might struggle with following instructions and as a result will need to rely on finger based representations in order to keep track of what they are doing whilst performing calculations(Hannell, 2005). They may also find it difficult to keep focused, so they will struggle to recite multiplication tables as it is easy for them to forget the sequence that they follow (Hannell, 2005). Ways to address shortages in working memory require the use of strategies and equipment which limit the requirements placed on memory during problem solving, such as calculators, concept cards, and maths videotapes (Nolting , 2000). The DynamoMaths programme (Esmail, 2008), accounts for working memory impairments by using instructions which are short, simple and repeatable (i.e. the child can listen to instructions whenever they want), and by clear providing instant feedback in order to hold the students attention and by using an uncluttered display. Dyscalculic students also experience difficulties with long term memory imp[lying s that they struggle with remembering written symbols and correct application of procedural rules. Dowker and Morris (2014) suggest in order to reinforce learning , students should be reminded at the beginning of each session and also reminded briefly but frequently of what they had previously achieved. Dyscalculia guidance summary Based on the cognitive principles highlighted above the consensus on guidelines for effective intervention in dyscalculia can be summarized as follows: 44 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

1. Interventions should be personalised according to individual needs 2. Instructions should be simple and well organised 3. Initially, abstract concepts should be made concrete 4. Big concepts should be broke down into smaller parts and introduced step by steps 5. Ample time should be allowed for students to practice new concepts, ideally students should be allowed o decide when they are comfortable to move on. Speed should not be emphasised until facts are mastered. 6. Provide pictures, graphs, charts and encourage drawing the problem in order to enable visualization of problems. 7. Provide real life examples relevant to the student's age and experiences 8. Provide immediate feedback and opportunities for students to revise their answer. 9. Allow students to communicate about mathematics in multiple ways, be it orally or through journal entries. 10. New vocabulary should be adequately explained These guidelines are supported by the Department for Education and Skills (DfES, 2001). Benefits of digital technologies Interventions for children with dyscaculia have traditionally used small teacher student groups , the most popular being Numeracy Recovery (Dowker, Hannington & Matthew, 2000) and Catch Up Numeracy (Holmes & Dowker, 2013) as well as pen and paper games and toolkits (Hanell 2005; Brid, 2013) . In recent times however computerised technologies have radically changed the way children acquire information (Christakis, Ebel, Rivara & Zimmerman, 2004). As a consequence interventions for dyscalculia are moving towards digitised alternatives. Although not specifically directed towards children with dyscalculia, computer-assisted mathematical interventions for school children have been proven to be effective since the 1960's. A review by Rasanen, Salminen, Aunio, Wilson and Dehaene (2009) found that computer assisted interventions have seen shown to be especially effective in primary grade because they can improve children's early mathematical knowledge by enhancing counting skills, numeral recognition, numerical concept learning. As highlighted in the previous section, in order to be effective, interventions for dyscalculia have adhered to specific guidelines, and digital environments have been shown to mould very well with these requirements (Butterworth & Laurillard, 2010). Computerised technologies can easily be personalised to individual's needs while also enabling a self- paced learning approach which allows the individual ample opportunity for repeated practice. Additionally, digital programmes can be administered in private, so that students

45 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

can manage math anxiety by making use of a threat-free tolerant approach. The flexibility to manipulate digital objects inherent in computer assisted technologies also allows students to experience multisensory instructions, which are especially important in dyscalculia (Clements, 2000) - and not provided by paper and pencil. Examples of digital interventions which could be of help in dyscalculia Although a range of interventions for improving mathematics ability are currently commercially available, a review of interventions for low mathematical ability found that only a small proportion of interventions are underpinned by strong neuroscience or cognitive research (Kroeger, Brown and O'Brien, 2012). The review further highlighted that only a quarter of interventions were supported by empirical evidence . Furthermore, whilst they are developed on principles known to benefit children with dysalculia, most current digital interventions are not necessarily targeted directly towards children with dyscalculia, but towards a more general group of children that present with difficulties in acquiring mathematical skill. Table 1 presents currently available programs that are based on empirical evidence and that also integrate, at least partly a digital component. The list includes both teacher led and classroom independentprogrammes.

46 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Table 1. List of currently available evidence based intervention programmes designed for children with difficulties in acquiring mathematical skill.

Intervention Target Focus Description Features population

The Number Age 4 to 8 Focuses on Free adaptive software programme Adjusts level of difficulty Race number formats, based on neuroscience findings related to to match learner’s counting and the numerosity system in the interparietal performance (Wilson et al., simple e additions sulci. The task requires students to select 2006) and subtractions the larger of two arrays of dots focussing Stepwise approach initially on large differences and moving on to smaller differences as the student is successful.

Graphogame – Focuses on Finnish adaptive game based which aims Informational feedback Math (Numerate) number to target the inherent system for comparison and representing and manipulating sets in the Stepwise approach number symbols interparietal sulci. Task requires the comparison of visual arrays of object, focusing initially on small sets which can be counted, and moving on to tasks which require comparison processes and knowledge of verbal numerical labels.

Fluency and Programme which includes series of Automaticity Grades 2 Focuses on engaging and motivating math Controlled response through Systematic to 12 developing games which focus on the relationship times Teaching with fluency with basic between numerical symbols and their Technology (FASTT mathematical associations with verbal representations. Personalised learning Math) operations By personalising learning material the path programme determines the level of (FASTT Math, automaticity and builds on existing Includes educator n.d.) declarative knowledge so that students can components practice just their newly learned and fluent facts and therefore helps build confidence.

Number Worlds Pre-K to Focuses on Interactive teacher-led group Individualised approach 8th grade teaching instructional programme aims to engage (Griffin, 2004) mathematical students in mathematical thinking by Adresses changes in concepts helping them develop an understanding of working memory by the meaning behind quantities. Tasks offering ample practice involve interactive games such as building opportunities blocks activities and strategic digital modelling as well as digital game boards Weekly planner for group interaction.

47 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

Accelerated Grades 1 Magnitude Daily progress-monitoring software tool Personalized pacing Math to 12 comparison and that can be used in conjunction with any estimation tasks core curriculum. It allows teachers to Goal setting feature (FASTT Math, differentiate instructions based on n.d.) Automaticity of student’s needs. Based on an initial Immediate feedback fact retrieval diagnostic assessment the software generates learning objectives specifically Multidigit aimed at the student’s needs. Based on computation personalised objectives the programme then generates individualized paper-and- pencil worksheet of practice problems which once completed and scanned and scored by the programme.

Tom’s rescue Arithmetical Uses a virtual environment in 18 Use of virtual operations, computer games which aim to confront environment (de Castro, number children with fun situational problems in Bissaco, Panccioni, sequences, visual order to stimulating reasoning in children Playful setting Rodrigues & reasoning, and generating positive attitudes towards Domingues, 2014) geometric shapes mathematics. All games are developed based on a storyline in order to promote child interaction whilst also addressing specific cognitive skills such as working memory and spatial reasoning

48 This project was financed with support from the European Commission. This publication reflects the views only of the author, and the Commission cannot be held responsible for any use which may be made of the information contained therein.

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