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Cortical visual impairment (CVI) is a form of
visual impairment Visual impairment, also known as vision impairment, is a medical definition primarily measured based on an individual's better eye visual acuity; in the absence of treatment such as correctable eyewear, assistive devices, and medical treatment� ...
that is caused by a brain problem rather than an eye problem. (The latter is sometimes termed "ocular visual impairment" when discussed in contrast to cortical visual impairment.) Some people have both CVI and a form of ocular visual impairment. CVI is also sometimes known as
cortical blindness Cortical blindness is the total or partial loss of vision in a normal-appearing eye caused by damage to the brain's occipital cortex. Cortical blindness can be acquired or congenital, and may also be transient in certain instances. Acquired cortic ...
, although most people with CVI are not totally blind. The term neurological visual impairment (NVI) covers both CVI and total cortical blindness. Delayed visual maturation, another form of NVI, is similar to CVI, except the child's visual difficulties resolve in a few months. Though the vision of a person with CVI may change, it rarely if ever becomes totally normal. The major causes of CVI are as follows: asphyxia, hypoxia (a lack of sufficient oxygen in the body's blood cells), or ischemia (not enough blood supply to the brain), all of which may occur during the birth process; developmental brain defects; head injury; hydrocephalus (when the cerebrospinal fluid does not circulate properly around the brain, and collects in the head, putting pressure on the brain); a
stroke A stroke is a disease, medical condition in which poor cerebral circulation, blood flow to the brain causes cell death. There are two main types of stroke: brain ischemia, ischemic, due to lack of blood flow, and intracranial hemorrhage, hemorr ...
involving the
occipital lobe The occipital lobe is one of the four major lobes of the cerebral cortex in the brain of mammals. The name derives from its position at the back of the head, from the Latin ''ob'', "behind", and ''caput'', "head". The occipital lobe is the vi ...
; and infections of the central nervous system, such as meningitis and encephalitis.


Visual and Behavioural Characteristics

Visual and Behavioural Characteristics of CVI are individual and may include several (but not necessarily all) of the following: * The person with CVI may exhibit what at first appears to be frequent variable changes in vision. However, it is the environment that impacts their visual function rather than their neurology or capacity for vision at that moment. A persons change in visual function when processing visual information in a complex environment, or trying to process a certain complex or unfamiliar visual target likely accounts for what looks like their visual ability changing from one day to the next, or from minute to minute. Feeling tired or unwell may also compound this challenge. For some people with CVI the complexity of the environment, including variables like the complexity and familiarity of the visual input, or processing various forms of secondary sensory input (for instance, sound or touch) may cause them to have difficulty using their vision to their full potential. The amount of sensory input an individual can tolerate without impacting function significantly may change throughout development and person to person. Managing
fatigue Fatigue describes a state of tiredness that does not resolve with rest or sleep. In general usage, fatigue is synonymous with extreme tiredness or exhaustion that normally follows prolonged physical or mental activity. When it does not resolve ...
may reduce fluctuations but does not eliminate them, however utilizing breaks and being well rested may help to increase resilience during these times. Addressing these changes may mean adapting strategies that work for the individual person. For example, When undertaking critical activities, people with CVI should be prepared for their vision to fluctuate, by taking precautions such as always carrying a
white cane A white cane is a device used by many people who are visual impairment, blind or visually impaired. A white cane primarily allows its user to scan their surroundings for obstacles or orientation marks, but is also helpful for onlookers in identif ...
, if they use one, even if they don't always use it to the fullest. Another example is having very large print available, just in case it's needed (For example, consider the consequences of losing vision while giving a public speech). Other potential adaptations to improve visual function in higher environmental complexity may include strategies like clearing away excess clutter from a work spaces, or providing a plain background to view objects against, reducing noise levels, or other methods of monitoring and adapting for environmental complexity. * One eye may perform significantly worse than the other, and
depth perception Depth perception is the ability to perceive distance to objects in the world using the visual system and visual perception. It is a major factor in perceiving the world in three dimensions. Depth perception happens primarily due to stereopsis ...
can be very limited (although not necessarily zero). * The
field of view The field of view (FoV) is the extent of the observable world that is seen at any given moment. In the case of optical instruments or sensors it is a solid angle through which a detector is sensitive to electromagnetic radiation. Human ...
may be severely limited. The best vision might be in the centre (like tunnel vision) but more often it is at some other point, and it is difficult to tell what the person is really looking at. Note that if the person also has a common ocular visual impairment such as
nystagmus Nystagmus is a condition of involuntary (or voluntary, in some cases) eye movement. Infants can be born with it but more commonly acquire it in infancy or later in life. In many cases it may result in reduced or limited vision. Due to the invol ...
then this can also affect which part(s) of the visual field are best. (Sometimes there exists a certain gaze direction which minimises the nystagmus, called a "null point.") * Even though the field of view may be very narrow indeed, it is often possible for the person to detect and track movement. Movement is handled by the 'V5' part of the
visual cortex The visual cortex of the brain is the area of the cerebral cortex that processes visual information. It is located in the occipital lobe. Sensory input originating from the eyes travels through the lateral geniculate nucleus in the thalamus ...
, which may have escaped the damage. Sometimes a moving object can be seen better than a stationary one; at other times the person can sense movement but cannot identify what is moving. (This can be annoying if the movement is prolonged, and to escape the annoyance the person may have to either gaze right at the movement or else obscure it.) Sometimes it is possible for a person with CVI to see things while moving their gaze around that they didn't detect when stationary. However, movement that is too fast can be hard to track; some people find that fast-moving objects "disappear." Materials with reflective properties, which can simulate movement, may be easier for a person with CVI to see. However, too many reflections can be confusing (see cognitive overload). * Some objects may be easier to see than others. For example, the person may have difficulty recognising faces or facial expressions but have fewer problems with written materials. This is presumably due to the different way that the brain processes different things. * Colour and contrast are important. The brain's colour processing is distributed in such a way that it is more difficult to damage, so people with CVI usually retain full perception of colour. This can be used to advantage by colour-coding objects that might be hard to identify otherwise. Sometimes yellow and red objects are easier to see, as long as this does not result in poor contrast between the object and the background. * People with CVI strongly prefer a simplified view. When dealing with text, for example, the person might prefer to see only a small amount of it at once. People with CVI frequently hold text close to their eyes, both to make the text appear larger and to minimise the amount they must look at. This also ensures that important things such as letters are not completely hidden behind any
scotoma A scotoma is an area of partial alteration in the field of vision consisting of a partially diminished or entirely degenerated visual acuity that is surrounded by a field of normal – or relatively well-preserved – vision. Every normal mam ...
s (small defects in parts of the functioning visual field), and reduces the chances of getting lost in the text. However, the simplification of the view should not be done in such a way that it requires too rapid a movement to navigate around a large document, since too much motion can cause other problems (see above). *In viewing an array of objects, people with CVI can more easily see them if they only have to look at one or two at a time. People with CVI also see familiar objects more easily than new ones. Placing objects against a plain background also makes them easier for the person with CVI to see. * For the same reason (simplified view), the person may also dislike crowded rooms and other situations where their functioning is dependent on making sense of a lot of visual 'clutter'. * Visual processing can take a lot of effort. Often the person has to make a conscious choice about how to divide mental effort between making sense of visual data and performing other tasks. For some people, maintaining
eye contact Eye contact occurs when two people look at each other's eyes at the same time. In humans, eye contact is a form of nonverbal communication and can have a large influence on social behavior. Coined in the early to mid-1960s, the term came from ...
is difficult, which can create problems in Western culture (for example, bonding can be difficult for some parents who have an infant with CVI, and lack of contact in an older child can cause others to regard him or her with suspicion). *It can also be difficult for some people with CVI to look at an object and reach for it at the same time. Looking and reaching are sometimes accomplished as two separate acts: look, then look away and reach. * People with CVI can sometimes benefit from a form of blindsight, which manifests itself as a kind of awareness of one's surroundings that cannot consciously be explained (for example, the person correctly guesses what they should do in order to avoid an obstacle but does not actually see that obstacle). However, this cannot be relied on to work all the time. In contrast, some people with CVI exhibit spatial difficulties and may have trouble moving about in their environment. * Approximately one third of people with CVI have some
photophobia Photophobia is a medical symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of ...
. It can take longer than usual to adjust to large changes in light level, and flash photography can be painful. On the other hand, CVI can also in some cases cause a desire to gaze compulsively at light sources, including such things as
candle A candle is an ignitable wick embedded in wax, or another flammable solid substance such as tallow, that provides light, and in some cases, a fragrance. A candle can also provide heat or a method of keeping time. A person who makes candle ...
flames and fluorescent overhead lights. The use of good
task lighting Often task lighting refers to increasing illuminance to better accomplish a specific activity. However, the illuminance level is not the only factor governing visibility. Contrast is also important, and a poorly positioned light source may cause ...
(especially low-temperature lamps which can be placed at very close range) is often beneficial. * Although people (with or without CVI) generally assume that they see things as they really are, in reality the brain may be doing a certain amount of guessing and "filling in", which is why people sometimes think they see things that turn out on closer inspection not to be what they seemed. This can occur more frequently when a person has CVI. Hence, a person with CVI can look at an optical illusion or abstract picture and perceive something that is significantly different from what a person without CVI will perceive. The presence of CVI does not necessarily mean that the person's brain is damaged in any other way, but it can often be accompanied by other neurological problems, the most common being
epilepsy Epilepsy is a group of non-communicable neurological disorders characterized by recurrent epileptic seizures. Epileptic seizures can vary from brief and nearly undetectable periods to long periods of vigorous shaking due to abnormal electrica ...
.


Diagnosis

Diagnosing CVI is difficult. A diagnosis is usually made when visual performance is poor but it is not possible to explain this from an eye examination. Before CVI was widely known among professionals, some would conclude that the patient was faking their problems or had for some reason engaged in
self-deception Self-deception is a process of denying or rationalizing away the relevance, significance, or importance of opposing evidence and logical argument. Self-deception involves convincing oneself of a truth (or lack of truth) so that one does not rev ...
. However, there are now testing techniques that do not depend on the patient's words and actions, such as
fMRI Functional magnetic resonance imaging or functional MRI (fMRI) measures brain activity by detecting changes associated with blood flow. This technique relies on the fact that cerebral blood flow and neuronal activation are coupled. When an area ...
scanning, or the use of electrodes to detect responses to stimuli in both the retina and the brain. These can be used to verify that the problem is indeed due to a malfunction of the visual cortex and/or the posterior visual pathway.


See also

*
Cortical blindness Cortical blindness is the total or partial loss of vision in a normal-appearing eye caused by damage to the brain's occipital cortex. Cortical blindness can be acquired or congenital, and may also be transient in certain instances. Acquired cortic ...


References


Further reading

* * * * * * * "Fact Sheet: Cortical Visual Impairment" by Mary Ann Demchak, Charmaine Rickard and Marty Elquist, published by The University of Nevada, Reno in the Nevada Dual Sensory Impairment Project 2002.


External links


CVI Now
A CVI resource hub associated with Perkins School For the Blind
Little Bear Sees
A foundation providing families with information, tools and research which improves the lives of children with CVI
PCVIS
The Pediatric Cortical Visual Impairment Society is a nonprofit promoting research related to the improvement of vision care for children with CVI {{CNS diseases of the nervous system, state=collapsed Blindness Brain disorders