Nonverbal Learning Disabilities in Turner Syndrome

Jim Abbott, a former Major League Baseball pitcher, despite having been born without a right hand, once stated that “it’s not the disability that defines you; it’s how you deal with the challenges the disability presents you with. We have an obligation to the abilities we DO have, not the disability” (Jim Abbot). This has never been more important to instill in our students today. They learn from our actions and language how to treat others, so modeling appropriate behavior in the classroom is essential. Understanding and respecting our students as individuals, each with their own special needs, will better prepare them for success. It is our job as educators to facilitate that success, and tailor our classrooms to fit their needs. We should be aware of what our students may be going through and the challenges they face. For example, the chromosomal abnormality Turner Syndrome (TS) has many symptoms and implications in the classroom.

TS occurs in about 1 in 2,500 newborn girls worldwide. According to the U.S. National Library of Medicine’s webpage Turner Syndrome – Genetics Home Reference, “most cases of Turner Syndrome are not inherited… the chromosomal abnormality occurs as a random event during the formation of reproductive cells in the affected person’s parent…[or] during cell division in early fetal development” (Turner Syndrome -–Genetics Home Reference). Common features associated with TS include distinct physical characteristics, heart and kidney complications, visual and hearing impairments, nonverbal learning disabilities and ADHD, and ovarian failure. LeAdelle Phelps states in the book Health-related Disorders in Children and Adolescents: A Guidebook for Understanding and Educating that, “the distinct developmental and physical characteristics of these children require a lifetime of medical intervention and careful monitoring of health status. However, the most long-lasting challenges associated with Turner’s syndrome are not medical or physical but rather emotional and social” (Phelps, p. 693). There are numerous factors to take into account when accommodating students with TS; classroom modifications, student self-esteem, and teaching techniques.

Due to the lack of pseudoautosomal regions, Turner syndrome has distinct physical and health-related characteristics. The pseudoautosomal regions “(PAR1 and PAR2) of the human X and Y chromosomes pair and recombine during meiosis. With the deletion of PAR1, the SHOX (short stature homeobox-containing) gene’s haploinsufficiency contributes to certain features of TS” (Mangs and Morris). Haploinsufficiency is the phenomenon where a diploid organism has only a single functional copy of a gene (with the other copy inactivated by mutation) and the single functional copy of the gene does not produce enough gene product, leading to an abnormal state ("Medical Definition of Haploinsufficiency"). The most common features of TS include its distinct physical characteristics: short stature, webbed neck, flat or broad chest, and shortened metacarpal IV. Growth hormone has been used to increase a patient’s final adult height, and is usually received through injections several times a week until the desired pubertal age. Without this, the average adult height of a woman with Turner syndrome is about 4 ft. 8 in. When to start estrogen replacement therapy is usually a complicated question:

Since estrogen effectively brings about the end of bone growth, the decision to start estrogen and stop growth hormone is an important one. Allowing the girl to grow to her full potential is a priority—but starting puberty after her peers have finished it can present other difficulties. (“Estrogen Replacement Therapy for Turner Syndrome”)

The average age to begin the replacement therapy is usually between ages 12 to 15, around the age for typically developing peers. As Richard Lippa explains in his book Gender, Nature, and Nurture, “Turner’s syndrome females have gonads that degenerate during fetal development, and they are born without ovaries and uteri. Because of their lack of ovaries, they do not produce natural estrogens, and of course, they are infertile” (Lippa, p. 107). Pills, patches, or topical creams will need to be taken for the rest of the patient’s life. Hormones are not only crucial for puberty, but for maintaining good bone integrity, cardiovascular health, and tissue health as well. Another common disorder that affects the endocrine system of TS women is Hashimoto’s thyroiditis, an autoimmune disease in which the thyroid gland is gradually destroyed by antithyroid antibodies. It can, however, be treated with thyroid hormone supplements.

One third of girls with TS have a heart abnormality, and twenty to forty percent have kidney abnormalities. Heart problems include coarctation or a narrowing of the aorta, bicuspid aorta valve, aortic valve stenosis, and dissection of the aorta. There is also a higher risk for hypertension. However, with proper monitoring and management, the prognosis for many TS patients is relatively good. According to SM Yuan in the article "Cardiovascular Disorders of Turner’s Syndrome: A Review", “in all individuals with hypertension, with or without aortic dilation or aortic dissections, medications should be given to control the blood pressure in order to prevent coronary heart disease, heart attack or other potentially fatal complications. Beta blockers or rennin-angiotensin system antagonists may benefit TS patients in retarding the progression of aortic dilation” (Yuan, p. 6). In more critical cases of coarctation repair, stent graft deployment has been a well-developed alternative choice to open surgery (Yuan, p. 6). As well as these possible heart complications, there can also be several kidney abnormalities. A horseshoe kidney is one of the most frequent renal anomalies. Other anomalies include hydronephrosis, renal agenesis, double pelvocalyces and ureter, etc. Doctors Dong Uk Yu, Jae Kyun Ku, and Woo Yeong Chung state in the article Renal Problems in Early Adult Patients with Turner Syndrome that the “development of renal malformation in TS is known to be caused by a defect in the ureteric budding during early stage of pregnancy or by an abnormality in migration of the kidney from the pelvis” (Yu, Ku, and Chung, p. 156). Both renal and cardiovascular abnormalities require careful attention and medical intervention.

Hearing and vision problems are also recurrent with TS. Because of the unique shape and structure of their ears, “infections are common. [Subsequently,] about 15 percent of adults with the syndrome have significant hearing loss” (Phelps, p. 692). Pediatric Endocrinologist Cresio Alves explains in the article Hearing Loss Among Patients with Turner’s syndrome: Literature Review that as the pharyngeal arches develop into the maxilla, mandible, and ossicles of the middle ear, the muscles involved in opening the Eustachian tube, dampening sounds, chewing, modulating tension of the soft palate, and changing facial expressions are affected. Skeletal dysplasia or a lymphatic insufficiency may cause obstructed drainage and thus otitis media, an inflammatory disease of the middle ear. Therefore, “the haploinsufficiency of SHOX expression is a possible explanation for features such as a high palate, prominent ears, chronic otitis media, obstructive sleep apnea, [and] increased sensitivity to noise” (Alves, p. 258). Eye complications seem to be less studied, but include strabismus, glaucoma, and amblyopia. Phelps agrees that “regular vision and hearing screening is especially important for children with Turner’s syndrome because auditory and visual difficulties increase the probability of academic problems” (Phelps, p. 692).

Considered to be neurologically based, nonverbal learning disorder (NLD) affects skills like abstract thinking and spatial relationships. Girls with TS have a higher chance of developing a Nonverbal Learning Disorder. According to the Turner Syndrome Society of the United States’ website, NLD is usually characterized by a significant discrepancy between higher verbal skills and weaker motor, visual-spatial and social skills. It “affects an individual's ability to read and interpret nonverbal communication such as body language, social cues, sarcasm, and facial expressions” (The Turner Syndrome Society of the United States). Visual-spatial processing can be difficult for TS girls, including driving, memory, understanding time, and math. The sequencing and abstract concepts in math are of particular difficulty. Joseph Baker and Allan Reiss further emphasize in their article A Meta-Analysis of Math Performance in Turner Syndrome that “Turner syndrome performance was severely affected by questions that required a speeded response time” (Baker and Reiss, p. 128). Lucie Attout, Marie-Pascale Noel, Marie-Cecile Nassogne, and Laurence Rousselle state in their research article "The role of short-term memory and visuospatial skills in numerical magnitude processing: Evidence from Turner syndrome that the risk of presenting mathematical learning disabilities is about four to five times greater among girls with TS than in the general population":

[Short term memory] STM is a good candidate to explain both deficits [sequential numerical information processing and in arithmetic]. Indeed, when controlling for memory capacities…STM measures were the stronger predictors of mathematical achievement in our study. (Attout et al, p. 19)

Attention deficit hyperactivity disorder (ADHD) also affects girls with TS. It is characterized by problems paying attention, excessive activity, or difficulty controlling behavior. These symptoms begin by age 6 to 12, are present for more than six months, and cause problems in at least two settings (such as school, home, or recreational activities). As Phelps explains, “these children are at risk to experience social isolation and low self-esteem” (Phelps, p. 693). Because of their social isolation, making new friends and keeping personal space is important. Premature ovarian failure and fertility issues may lead to shyness, social anxiety, and reduced self-esteem. These conditions have many implications for the classroom.

These implications affect the physical modifications, the social sphere, and the organization of the classroom. Teachers should be very aware of the setup of their classroom, and the needs of the students. Step stools, low shelves, and visual schedules may be needed to help with the access to and understanding of routines. Sue Thompson states in her article "Developing an Educational Plan for the Student with NLD", “the student with NLD generally copes well in a structured predictable environment. However, [she] will experience extreme stress when faced with forced or unexpected changes in routine” (Thompson, p. 2). Because of this, she will need extra assistance ahead of time for all changes in routine and transitions. This can be for anything from field trips, assemblies, substitute teachers and modified days. Reducing background noise can also help students focus, as well as providing them a seat facing the teacher. When teachers set realistic goals that rewards rather than punishes students with NLD, students will achieve a higher rate of success. This influences their self-esteem and allows them to participate more fully in the social environment. All students deserve consistent and high expectations. As stated in the article Turner Syndrome & Education Guide for Parents and Teachers “most children tend to live up to the expectations of their teacher and their peers, and if a child hears often enough that they [aren’t] clever/kind/talented then they may well end up being just that!” (The Turner Syndrome & Education Guide for Parents and Teachers, p. 3). The self-fulfilling prophecy becomes a very important indicator of student success, and something teachers should avoid. Speaking directly, using clear language and short sentences, and using appropriate and unambiguous facial expressions as well as visual demonstrations can help students with NLD. Group projects and social skill groups may not only provide an opportunity for some students to learn from their typically developing peers, but also introduces them to peers with whom they would not have normally interacted. They can present strategies for confronting challenges to being socially successful. One strategy is workshop drama, which may be useful in better understanding social interactions in a safe environment (p. 4).

Many girls with Turner syndrome begin to understand themselves as different than their peers, especially concerning areas of puberty; “although 10-20 percent of girls with Turner syndrome may experience puberty without hormonal intervention, most do not” (Phelps, p. 692). This is particularly concerning because, "when the child reaches the age of puberty, social and emotional concerns typically become paramount to the child and family" (Phelps, p. 693). Having a sense of belonging is essential for a cohesive classroom, as a deficiency in their social skills can make TS girls a target for bullying. Lynn-Georgia Tesch unfortunately “indicated that this sense of difference is not typically addressed by medical professionals” and has received little attention (Phelps, p. 693). There are anti-bullying programs in many school districts, and many schools have provisions in their codes of conduct about its consequences. However, talking to students about their concerns and intervening when necessary is vital. The Turner Syndrome & Education Guide for Parents and Teachers states that “given their small stature there may be a danger of a girl with TS either being picked on as someone small and vulnerable, or patronized and treated as a mascot by peers” (Turner Syndrome & Education Guide for Parents and Teachers, p. 5). Women’s self-esteem in particular seems to be linked to their personal relationships. The more all children can participate in the classroom social sphere, the more their peers observe everything they can accomplish. Moreover, they may be less likely to “baby” TS girls in return. The “Right to Be Me” lesson plan demonstrates this idea of emphasizing students’ positive traits together as a class, and promotes acceptance of differences.

The organization of the classroom is also extremely important. Students with Turner syndrome and NLD may be disorganized, and need help with the weekly cleanup. This can include their desk, backpack, cubby, or current activity. According to James Long and Robert Williams in the book Making it till Friday: Your Guide to Successful Classroom Management, teachers should “have a scheme for putting things away when they are not in use, develop a system for minimizing clutter in the classroom, establish a routine for periodically cleaning furniture and work space, and add items that will increase the aesthetic appeal of the classroom” (Long & Williams, p. 204-205). Most children, atypically developing or not, benefit from this. Schedule notebooks can be used to prepare priorities, reminders, and instructions when students are at home. Developing a plan reduces confusion and frustration for both children and teachers. When task priorities are set, they can subsequently be divided into subtasks which provide more clarity. Long and Williams further mention that “the difficulty level of a task is directly affected by the clarity of instructions for doing the task… in classroom instruction, task difficulty can be greatly reduced if the teacher divides tasks into smaller steps and then presents those steps in a logical sequence” (Long & Williams, p. 205). Instructors may also need to spend extra time teaching the structures and templates for various tasks, including book reports, spelling assignments, essays, etc. The major objective for these organizational tools is to promote easy retrieval of needed materials and to maximize concentration on assignment tasks.

There are several therapies that could be very effective in the classroom learning environment: auditory integration, cognitive behavioral, and sensory integration therapy. They have been used to reduce the effects of learning disabilities, attention deficit hyperactive disorder (ADHD), autism spectrum disorder, and hearing challenges. In auditory integration therapy, modulated and filtered sound is played through high quality headphones. The program exercises and educates the listener through the auditory system, promoting normal hearing, and educational and social wellbeing. The article "Turner Syndrome at a Glance" suggests that it “improves auditory processing and the way the brain processes information, particularly in normally noisy environments” (Turner Syndrome at a Glance, p. 9). Through the increased development of neural pathways, there may also be an improvement in reading, writing, speech, and language development, and the ability to handle noisy environments and social situations. Therapists or computer-based programs use cognitive behavioral therapy (CBT) techniques to help individuals replace errors in thinking such as overgeneralizing, magnifying negatives, minimizing positives and catastrophizing with more realistic thoughts, thus decreasing emotional distress and self-defeating behavior (Turner Syndrome at a Glance, p. 9-10). As Long and Williams state, “control might be achieved by learning new techniques to influence behavior. Controllability is almost always a function of skill level” (Long and Williams, p. 15). Teachers should not simply admonish students, but have a system in place to ameliorate deportment; “if a teacher uses reprimands repeatedly, students can develop negative reactions toward him or her in the classroom setting” (Long and Williams, p. 126). Sensory integration therapy “helps with organizing and interpreting sensory signals in the environment” (Turner Syndrome at a Glance, p. 10). They describe how the neurological process and integration of sensory information from the body and environment contribute to emotional regulation, learning, behavior, and participation in daily life (Turner Syndrome at a Glance, p. 10).

Poor homework performance is also associated with TS, and NLD challenges in particular. Teachers should help students plan their study time with a schedule, or provide parents with materials and support. As "Turner Syndrome at a Glance" suggests, a workspace should be available that has good lighting, plenty of space, no clutter, and little or no noise. Establishing guidelines such as the avoidance of loud music, TV, and text messaging while completing homework can augment concentration. Students should be “encouraged to ask for help and to use a study buddy” (Turner Syndrome at a Glance, p. 10). Although high school students may benefit minimally from homework, younger students, especially those with special needs, do not receive much of an advantage. Teachers need “to ensure that homework assignments are practical as well as academic…You will need to help students be aware of how an assignment is linked to important life goals that will benefit them both now… and later as adults” (Long & Williams, p. 154). Doing difficult, seemingly arduous tasks that feel extraneous eventually leads to student enervation. Thompson further explains that “in addition to this extreme exhaustion, slow processing speed and severe organizational deficits make it necessary to lessen the homework/class work load… the student with NLD is usually so exhausted by the time school lets out, she literally collapses upon arriving home” (Thompson, p. 6). Children should be assisted in organizing binders for each subject and prioritizing a weekly to-do list. The list may include homework that needs to be done, activities, chores, and plans that have been made (Turner Syndrome at a Glance, p. 10). It’s essential to note that the average intelligence of girls and women with TS is similar to the general population. It is the responsibility of educators to ensure an appropriate learning environment for all.

Many of the lessons and activities I have included have been designed to promote inclusion. Inclusion, as I understand it, “means that children with special needs attend… school with typically developing peers” (Allen and Cowdery, p. 4). It requires the teamwork of students, parents, teachers, and other professionals. As the DEC/NAEYC article "Early Childhood Inclusion" states, the growing concern with new regulations and laws “reflects a reaction against previous educational practices of separating and isolating children with disabilities” (Early Childhood Inclusion, p. 1). Its defining features include high quality access, participation, and support for all children. There are many pros and cons for children with and without disabilities. However, with new laws such as the Individuals with Disabilities Education Act (IDEA), teachers especially should work with families to create the least restrictive environment possible for all students. Their participation and knowledge is crucial. All students deserve a free, appropriate public education that is tailored to their individual needs. “Inclusion is a right, not a privilege for a select few” (Oberti v. Board of Education in Clementon School District, 1993). Any program catering to young children has the potential to be inclusive. It “can take many difference forms and occur in various organizational and community contexts, such as homes, Head Start, child care, faith-based programs, recreational programs, preschool, public and private pre-kindergarten through elementary education, and blended early child education/early childhood special education programs” (Early Childhood Education, p. 2).

As has been mentioned, Turner syndrome has distinct physical and health-related characteristics. One third of girls with TS have a heart abnormality, and 20 to 40 percent have kidney abnormalities. Hearing and vision problems are also recurrent with TS. Nonverbal learning disorder (NLD) affects skills like abstract thinking and spatial relationships. Girls with TS have a higher chance of developing a Nonverbal Learning Disorder. These conditions have many implications for the classroom. They affect the physical modifications, the social sphere, and the organization of the classroom. Many girls with Turner syndrome begin to understand themselves as different than their peers, especially concerning areas of puberty. The organization of the classroom is also extremely important. There are several therapies that could be very effective in the classroom learning environment: auditory integration, cognitive behavioral, and sensory integration therapy. Poor homework performance is also associated with TS, and NLD challenges in particular. A growing concern of many teachers, parents, and students, inclusion is a significant classroom technique to ameliorate academic performance. Its defining features include high quality access, participation, and support for all children. Although TS women display an average intelligence, their specific NLD, ADHD, medical, and other concerns leave classroom support as particularly necessary. It is our job as educators to facilitate their success, and tailor our classrooms to fit their needs.

Works Cited

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Yuan, S-M, and Hua Jing. "Cardiovascular Disorders of Turner's Syndrome: A Review." Balkan Journal of Medical Genetics. Web.

Yu, Dong Uk, M.D, Jae Kyun Ku, M.D, and Woo Yeong Chung, M.D. "Renal Problems in Early Adult Patients with Turner Syndrome." The Korean Society of Pediatric Nephrology, 2015. Web.

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