Abstract
This is a family-presented case study on how cerebral visual impairment (CVI) impairs my 13-year-old child's ability to read print and how I discovered that braille is the only effective way for her to access literacy. I found that, under the neurological umbrella of CVI, several diagnosed impairments to higher visual processing, including a visual agnosia of letters, prevent her from fluently reading print. Discovering she subconsciously utilizes a link between her visual and tactile senses as a compensatory strategy when she encounters print ultimately encouraged us to introduce braille. Introduction of braille and reduction of visual demands at age 12 has given her access to literacy with pleasure, common understanding, academic growth, and independence.
CVI Diagnosis at 11 Years of Age
CVI is “a verifiable visual dysfunction which cannot be attributed to disorders of the anterior visual pathway or any potentially co-occurring ocular impairment” (Sakki et al., 2018) and Mae was diagnosed with CVI at New England Eye Low Vision Clinic at Perkins, with follow-up at Boston Children's Hospital, after 11 years of my partner and I having no idea she experienced any problems with her vision. Mae's formal diagnoses include simultanagnosia, prosopagnosia, optic ataxia, visual agnosias (letter, form, and topographic), oscillopsia, and convergence insufficiency. All of Mae's pediatrician well-child visit eye screenings were typical and she has typical visual acuity. The professionals we consulted throughout her childhood never suggested Mae's difficulties could be related to impaired visual function. Mae has not experienced other medical or learning challenges and is a curious, talkative, bright young person.
The CVI diagnosis came after approximately 18 months of seeking answers to unresolvable visual anomalies that Mae reported during reading: trouble holding print in focus; oscillating text; difficulty finding her place; and eye strain, nausea, and headaches. In this period, she was diagnosed with convergence and accommodative insufficiency. Six months of vision therapy was an incredibly difficult experience for Mae that did result in stronger oculomotor control, which in turn allowed Mae to describe a range of serious lifelong visual anomalies of which I had not been aware and led me to question if these problems were neurological.
Having learned that perinatal oxygen deprivation, head trauma, and hypoglycemia can result in damage to the visual pathways in the brain, I realized that Mae's traumatic birth put her at risk for CVI—even though her Apgar score was 9 and her neonatal medical care never indicated any problem. I did not realize at that time that her oculomotor symptoms were actually associated with a host of CVI challenges that affect print reading. She is unable to access literacy through visual means.
There is a cohort of individuals with CVI who subconsciously take advantage of other methods to understand the world around them. Some appear to use a combination of self-narration, kinesthetic, tactile, and auditory skills to compensate for their atypical vision (Zahide et al., 2021). The optometrist who diagnosed Mae, when evaluating her for CVI, invited her to answer questions about her functional vision including, “How are you reading print?” At home, I followed his curious, conversational approach and, thereby, learned that our daughter cannot recognize or visualize letters and utilizes a compensatory strategy to appear to read print. I also learned that in order for a beginner reader to progress beyond rudimentary stages, a leap must be made from visual identification of letters, which requires the conscious consideration of salient features to accomplish, to visual recognition of letters, a subconscious and effortless process (Kilpatrick, 2016, pp. 5–6). Mae is unable to recognize, but has developed compensatory strategies to identify, the letters at which she is looking.
The research of David Kilpatrick explains the distinction between identifying and recognizing words in early reading acquisition, using an analogy to facial recognition for word identification versus word recognition (the emphasis is Dr. Kilpatrick's): Someone may say to you “go into the meeting in the next room and tell the tall man with red hair and glasses that he has a phone call.” Based on such cues, you could identify that man even if you have never seen him before. But sending you in the next room to tell your best friend she has a phone call is quite a different task. You already know your best friend. You don't need cues to identify her. Rather, you simply recognize her when you see her. It is similar with reading words. We can distinguish between the terms word identification and word recognition. Identification is a broad term that means that a student correctly reads a word, regardless of whether he sounded it out, guessed, or retrieved it from memory. Identification takes effort. By contrast, word recognition refers to the retrieval of a familiar word from memory. There is no need to guess or sound out the word. Recognition is instant and effortless. It is based on whether or not that particular word is in a student's sight vocabulary (i.e., the words he can recall from memory). Word identification takes phonic decoding, guessing, and word recognition. . . .
Dr. Kilpatrick makes a direct analogy between the sorts of salient feature identification a person must employ out of necessity to identify an unfamiliar person with the process of identifying letters and words in the absence of recognition (Kilpatrick, 2016, pp. 5-6). In Mae's case, she is unable to recognize letters, which is a necessary prerequisite to recognizing words. In our family's experience, an intelligent child with CVI can have compensatory strategies for identifying, but not recognizing, letters and words so that no one around her realizes it is happening, but it is tiring and takes extreme effort nonetheless.
Strategies to Decode Print
Mae calls her strategy for engaging with print “tactile vision mediation.” She imagines feeling whatever she is looking at, including print. Her brain has integrated her visual acuities with her tactile memory to interpret word shapes as “nonvisual stencils.” Mae clarifies, In order to recognize a word, the stencil must fit on the shape of the word. The internal tactile feeling of this is like pressing a brick to open a hidden door, except it has to match the shape of the word in order to open the door. . . . My stencils take almost daily maintaining to remember.
She considers what the writing is about. “I am actively looking for clues everywhere other than text to see what the book is about, so I can have more context, which gives me the best advantage.” Mae uses prediction based on context to guess what word will come next in a sentence and what the text is likely to be about.
She aligns her eyes to focus as best she can, is seated with text very still, uses a tinted typoscope or blue back-lit screen, and needs increased font size and reduced visual complexity.
Mae mentally perceives the tactile outline of the word shape, but not the individual letters in the word.
Mae runs through her Rolodex of many “word stencils” to find one that fits the shape of the word. Her stencils do not tell her what letters are inside of the word.
If Mae cannot tell what a word is from her stencils, she moves to “Plan B: rolling out my toolbox to look inside the word” (spoken with true disdain). Mae's “toolbox” means bringing to bear her structured literacy training to decode words letter-by-letter.
When Mae uses her compensatory strategies for print, it causes an exhaustion that is common among people with CVI, which, within 5–15 min, manifests with drooping down to the table, rubbing or blocking eyes, laying down the head, bouncing or rocking, losing voice inflection, being distracted from the visual task, or crying (Bennett, 2021). Mae explains the experience of feeling fatigued from reading print: I lose any initial excitement I had about reading the text. It becomes the most horrible of chores, and the only reason I keep reading is so that it is over. Reading never gets easier for me; I just get faster at my work-arounds. Print repels me.
Visual Fatigue
Convergence and accommodative insufficiency, difficulty tracking, and trouble with saccades conspire to make holding text in focus nauseating for Mae, even when she is using a typoscope. Visual fatigue plagues her. She explains, My eyes aren’t just having trouble going from left to right smoothly. My eyes are having trouble being aware of the confinement that is the left-to-right line structure of reading. I often find myself jumping to the next word not by going east, but by going northeast, or southeast; I’ll find myself reading the beginning of the line I just finished, and become quite confused and disoriented as to where I came from.
The direct effect of extended print reading is losing her ability to regulate her nervous system. Rage and “reading tantrums” are reasonable responses to this impossible situation for a child (Damato, 2021). CVI NOW, a resource created by the Perkins School for the Blind, includes a series of articles outlining the neurological and physiological reasons why individuals with CVI can become so easily fatigued (Bennett, 2021). Chronic nervous system activation, due to the stress a person experiences while trying to read print, quickly turns into a trauma response.
Mae has particular difficulty distinguishing letters that are mirror images of each other. Suppression of reversals is a learned property of the visual system that a person relies on while reading. Mirror image confusion is not dyslexia, but rather a vestige of our primate brain's visual circuitry that allows generalization across mirror images that is necessary to identify 3D objects but is detrimental to reading. Mirror errors peak between 7 and 10 years of age; it can take a typical child several months to unlearn mirror errors for letters (Dehaene, 2009). Mae struggles to distinguish mirrored letters, despite many writing and reading experiences (including multiple approaches to using kinesthetic learning), further challenging instant letter recognition.
Letter invariance is part of the visual system's ability to subconsciously recognize letters regardless of major differences in the shape of the letter symbol (like uppercase cursive “G,” uppercase print “G,” and lowercase “g”) (Dehaene, 2009; Kilpatrick, 2016). Mae cannot make a visual memory of letters, so every letter, in each case and in any font, must be identified almost like a code. Something as ubiquitous as a font with serifs is a barrier to Mae; she explains, With pointy fonts [serifs], I have to mentally remove all the little pieces [serifs] and it will fit in my stencil. Or, I have to blur out my stencil, to see if the word in question will fit in, but this is not very accurate. I don’t recognize words as well because my stencils are blurry. If I really can’t tell, I have to identify where a distinct letter is, or identify where I don’t understand, and peel off the extra pointy parts [serifs]. The thing about simultanagnosia is when the scene I'm looking at gets focused in closer, then the amount of parts I can see all at once shrinks with it. When I go to look at one letter, I will not see all of it at once.
An Illustration of How Print Appears to Mae.
I first responded to these visual challenges with various adaptations to improve Mae's access to print, to observe if and how she could progress from letter and word identification to recognition without debilitating consequences. What I found was a lifelong, underlying inability to become familiar with letters to the point of automaticity. Mae's diagnosed visual agnosias (face, letter, form, and topography) means she can identify, but not recognize, these items. I found scant information to guide my own understanding of Mae's experience with letters, and questioned how visual agnosia of letters (and therefore words) presents. The following section describes my observations of clues from Mae's life that have indicated her letter agnosia.
Letter Agnosia
Mae assigns letters salient features and stories to “know” them without being able to make any visual memory of them. For example, “[Lowercase] ‘f’ is important because it has a shape that not any others have. It is so nice and distinctive, and whimsical.” Mae would become very angry about words containing “F” or “ph,” because they remove one of the few letters that is easy for her to identify. Or, “I do not trust ‘u,’ because it is a very tricky letter. It is like a petulant child, who always hides behind another friend to get them to do his dirty work.” This statement is in reference to the fact that “ou” makes four sounds (as in: “mouse,” “soul,” “soup,” and “touch”).
Mae's lack of visual memory for letters caused her to struggle continuously with recognizing words. Orthographic mapping occurs when the letters a person sees and the sounds they hear in that word get processed and stored together in the brain. It is not the same as memorizing a word's exterior shape (Sedita, 2020). Orthographic mapping is how a person learns to read words by sight and spell by memory, and is a cognitive process at the level of letter-phoneme pairing. For Mae, who has never seen any letter in its entirety and has no visual memory of letters, there is no amount of exposure to the print word plus sounds that resulted in storage of a visual-phonological unit.
Mae can only write in print using phonological guesses, and she almost never reads back her own writing. With wildly inconsistent spelling and no visual memory of the letters inside words, she increased the likelihood she would be able to read back what she wrote by modifying the shapes of letters to create identifiable salient features for sounding out words letter-by-letter. Mae says, “Lowercase ‘d’ is a circle with a stick and you barely notice the stick because it is like an ‘l’; ‘d’ looks like an ‘o’ and an ‘l’ together, so I draw it curved, like a 6.” (See Figure 2 for an example of Mae's adapted handwriting.)
An Example of Mae's Adapted Print Handwriting, in Which She Adds Identifiable, Salient Features to Letters.
Braille Introduction
Mae was experiencing excruciating exhaustion, frustration, and academic stagnation in reading print despite one-to-one, multisensory, and research-based interventions. Although she has typical visual acuities, I decided that braille was an appropriate next step in Mae's literacy education. Mae is homeschooled, and I (her mother) provide braille instruction without access to a teacher of students with visual impairments, although we do take part weekly in a 30-min private consultation with a fluent braille reader and assistive technology specialist. I did not appreciate the extent of Mae's letter agnosia until I witnessed firsthand the contrast between Mae reading print compared to braille.
Mae started learning braille tactually (not visually) at 12 years old, and the astounding contrast between her reading experiences in print and braille was immediately evident. Mae was enthusiastic and did not become exhausted when learning, reading, or writing braille. She quickly devised a system for punching her own braille using a piece of foam and a pin head—and she was able to read back what she had written in braille.
As she learned braille, Mae shocked me by spontaneously verbalizing many early-reader foundational insights such as recognition that letters are inside of a word, can be rearranged into new words, and represent sounds; and the discovery that repeated braille reading of simple words (i.e., “cow” and “said”) supports her reading and spelling memory. These insights, occurring for the first time at 12 years old, are remarkable, given Mae's background in a highly supported, print-rich learning environment and one-on-one didactic literacy instruction. I believe Mae has a tactile memory for braille letters that functions typically, which allows her to make these discoveries. Her inability to access letters through visual memory because of CVI prevents visual learning through print, despite her demonstrated ability to appear to read print using her self-described “workarounds.”
When Mae reads in braille, letters can be memorized using tactile input and linked with their auditory sounds. Braille is a tool that is finally allowing Mae to tactually and orthographically map words in the same way that typical readers do using vision. Mae's brain, out of necessity, already created a tactile means for interpreting print language that is far less efficient and flexible than braille. We believe Mae's self-taught compensatory strategies primed her to use braille to link auditory and tactile sensory information. One year after starting braille, Mae is tactually reading a Unified English Braille (UEB) grade 2 braille young-adult novel independently, with age-appropriate comprehension, is using the Nemeth Braille Code for Mathematics and Science Notation to study middle-grade mathematics, and is proficient on her refreshable braille display and screen reader.
Discussion
I share this description of surviving in a print-dominant world to contribute to what it can be like when a child with CVI learns to identify, but not recognize, letters, and words. Without lightning-speed visual memory for letter-sound associations, fluent print reading simply is not possible. As reading demands increase exponentially beyond fourth grade, a child who is already working overtime to pass as sighted and a print reader is set up for failure, burnout, and despair. With greater recognition of the existence of visual agnosia of letters, including when it is hidden under compensatory strategies, I believe those supporting literacy access for children with CVI should consider braille, including for children with typical visual acuities.
Footnotes
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.