Rev. 2008; originally published 2000
SPEECH AND LANGUAGE
All of us communicate throughout our day from the time we’re infants until we grow old. To get our messages across, we use both verbal and nonverbal methods when we communicate whether it’s with one person or groups of people. When the process of getting our message across is disrupted, either by something we have done or otherwise, we experience varying levels of frustration. The level and amount of frustration we experience is dependent on how important our message is and how attached we are to it emotionally.
When there are consistent concerns about a person’s ability to communicate, a first step is to have a speech and language evaluation performed. Generally, an evaluation looks the key, important components of speech and language skills. These include a person’s ability to say sounds clearly and accurately or “speech.” Speech can also be referred to as “sound production” or “articulation.” Language looks at a person’s ability to understand language (also known as receptive language or auditory comprehension) and his/her ability to make his/her needs and wants known (also known as expressive language). Associated language areas include looking at a person’s reading skills and written language skills.
To assess someone’s speech and language skills, formal measures using standardized tests are completed whenever possible. This is true for someone who has A-T, although sometimes the tests have to be changed or modified to adjust for some of the areas of challenge. For example, we often ask a child who has A-T whether he/she likes to look at material in books flat on a table or up off the table in an easel presentation. Since volitional eye movements are sometimes affected, it’s common for the child to say that they prefer the books being presented up in an easel presentation. This, then, can be an easy and functional recommendation that can be implemented in the child’s every day environment.
In devising an appropriate test battery for a speech and language evaluation, we often have to modify or eliminate tasks that the child isn’t able to perform or are particularly challenging. For example, reading can be challenging for an older child who has A-T and reading is often a key component to language evaluations for this age child. During the administration of the tests, it’s helpful to look at and analyze not only the person’s performance on the tasks but to make note of and try different therapeutic techniques. These can have important implications for the child’s functioning at home and school.
From a speech and language perspective, speech is often the most noticeable characteristic of someone who has A-T and is an important component to a speech and language evaluation. Specifically, speech is often non-distinct or slurred, which is known as dysarthria. During a speech and language evaluation, it’s important to look at the person’s speech production skills, both in single words and in connected speech including sentences and conversation. When looking at specific sound production skills and where someone has trouble, it’s helpful to understand how A-T affects motor movements.
All of these areas combined are considered in the evaluation process when looking at the person’s ability to functionally communicate for his/her current daily needs and requirements. These needs obviously change as a person grows and circumstances change.
General Speech and Language Findings
Speech and language abilities vary from one person with A-T to another, and vary over time in individual patients. Each individual will have areas of relative strength and weakness. It is hard to compare children’s abilities simply because they share the diagnosis of A-T. The young child with A-T may or may not have delayed language. Although expressive language, specifically trouble saying sounds, is often the most apparent area of difficulty for the child with A-T, receptive language can also be affected.
The most obvious and apparent communicative area of difficulty for the person with A-T is slurred speech or dysarthria. It often is the first sign of speech and language involvement. This difficulty with speech production can be present in the very young child with A-T or may develop as the child grows. Muscle tone throughout the face and lips is often decreased, tongue strength may also be decreased, and there is incoordination of the muscles of speech. This difficulty with the oral musculature affects the clarity with which speech sounds can be said. Sometimes, as a result of the muscle incoordination and weakness, drooling may be present and there may be a decrease in the rate of speech or the person may talk slowly.
A common characteristic of many children with A-T is the need for increased time to process information and respond appropriately. Often the person does not give any indication that he/she is actually thinking and it may appear that he/she is simply sitting. Given time, however, the person is often able to respond appropriately. Obviously, this could have impact on the person’s ability to perform, especially in school, where immediate responses are sought. What sometimes makes this more difficult is that the person with A-T often does not let the other person know that he/she is thinking or that he/she needs additional help or time to respond. So, it appears that he/she is not responding to the request or to the information presented.
Some people with A-T have subtle trouble with word retrieval and word recall. In other words, the person is not able to think of a word that he/she wants to say. Often times the student may respond that “I knew what I wanted to say but couldn’t think of it.” Rather than say something wrong, he/she decides not to say anything at all. When given cues such as a description of the word (semantic cues) or the beginning sound of the word (phonemic cues), the person is often able to think of the word and say it.
Thought organization and process is sometimes involved, as demonstrated in structured and formal tasks. The person may be able to talk and carry on a conversation without any apparent trouble. However, when asked to develop sentences with a specific word or words, the person is unable to complete the task. This becomes increasingly apparent as the words and tasks become more complex. On the surface it may appear that the person has no difficulty with organizing language, but when asked to demonstrate some depth of language skill, the deficit becomes more apparent.
Following directions, especially when they require visual scanning or reading, may be especially hard. As the directions become more complex and contain more language embedded concepts, more difficulty is observed. The difficulty with volitional (apraxic) eye movements can make these tasks even more difficult, and sometimes impossible, to complete. This might be most apparent in school where the child is looking at visual material in books and have to shift their eyes quickly from the items on the page to the teach and back again while trying to complete the command.
The person with A-T may have poor respiratory control, resulting in quiet speech, and minimal changes in intonation or pitch inflection with speech. Usually those familiar with the person who has A-T are able to understand his/her speech although repetitions are sometimes necessary. It often is more difficult for strangers, or those unfamiliar with the person, to understand what has been said. This can be especially true if the context or general topic area is not known. As the person gets older or if symptoms of A-T worsen over time, it may become more and more tiring for the person to talk; what seems like the simple task of talking actually results in fatigue. As a result, the person may talk less and may avoid communicative situations. It also can affect the person’s ability to interact with other people, especially in school and is social situations.
Facilitating communication skills for the person who has A-T can be achieved by having the person implement techniques and helping him/her learn strategies. There also are things that others around him/her can do to help. It is a combination of both of these that results in positive communicative interactions.
Speech and language services are often recommended to help the person learn functional ways to address areas of weakness and to compensate for areas of difficulty. Rehabilitative recommendations may include use of speech and language services provided through the school. Providing services in school offers functional ways for the person to implement strategies. It’s often helpful to have both classroom-based services and individual or group services where the child is taken out of the classroom. Providing services in the classroom gives opportunities for the student to practice skills and techniques that he/she has learned in the therapy setting. The speech-language pathologist can also give suggestions to the child’s teachers to help him/her be more successful. Suggestions can be provided as necessary to assist the student with communicating when talking is too tiring and to teach compensatory strategies.
Generally, the earlier compensatory strategies can be taught and implemented, the easier it is for the person to use them over time. By learning the strategies at younger ages, using them becomes a way of life for the person who has A-T and those around him/her at school, home, and socially. The most important factor to consider is to keep all suggestions and techniques functional, practical, and simple to implement.
Since speech is often noticeably affected in someone who has A-T, questions often arise about how to deal with this from a therapy perspective. Usually when someone has trouble saying sounds accurately and being understood, a primary therapy goal is to increase speech production skills and increase intelligibility. However, for the child with A-T, this may not be a reasonable focus of therapy. Specifically, a person with A-T often can’t say speech sounds crisply and accurately because of the associated physical components related to A-T. Frequently the child can say sounds in all positions of words when they’re said in single words, but aren’t able to maintain the skill when talking in sentences and in conversational speech. Even in single words saying some sounds can be challenging, especially when they occur at the end of a word. From a physiological standpoint, it can be hard to move the tongue quickly and accurately to the different positions in the mouth to say speech sounds. As a result, sounds may be left out or omitted. Also, a person may “mark” a sound or get the tongue in the right place to say the sound, but lacks the other physiological components to actually get the sound out.
A good example of this is with the “s” sound at the end of a word, as in “us” and “windows.” When watching the person’s face and mouth while they talk, the tongue gets in the right place to say the “s” sound, but isn’t able to coordinate the airflow appropriately to actually get the sound out. It’s not that the person needs help to learn how to say the “s” sound when it comes at the end of a word; rather, he/she knows that it should be there and tries to say it, but can’t.
As a result, working specifically on speech production skills often isn’t recommended to address in speech-language therapy, although this is decided on a case-by-case basis and according to a child’s age. Deciding what sounds to work on should also be based on an understanding of how A-T affects motor skills. In addition, knowing the association between motor patterns and fatigue is important to keep in consideration. Doing a lot of drill-work or saying a sound or word repetitively wouldn’t be indicated because of the underlying motor-related fatigue that would occur. In the long-run, this would be counter-productive.
Instead of working on specific sounds in therapy, it’s better to focus on compensatory strategies that can be implemented to increase a child’s ability to communicate and get his/her message across. As with other therapy techniques for children who have A-T, some of the strategies include things that the child can do and things that are helpful for others to do.
One important area that frequently occurs is how hard it is for the child who has A-T to talk when they get tired. As he/she gets tired, speech often gets harder to understand. During those times, it is important to understand the energy that talking requires and respect how the person may need to adjust his/her communicative techniques. Specifically, when the person is tired, do not expect or require full sentences. Encourage the person to use the key, important words in the message. For example, instead of requiring the person to say, “I’d like a drink of orange juice, please,” he/she could say, “drink, orange juice.” It is important to remember that when the person is tired, he/she shouldn’t be expected to say social, “polite” words, such as “please” and “thank you.” These words require just as much effort and energy to say as content words, but do not contain any meaning. Obviously, when the person is feeling “full of energy,” the person should be expected to say them. If the person is too tired to use key or important words, offer choices where he can respond with one or two words. Also, it may be helpful to pose simple yes/no questions so that the person can nod or shake his/her head in response and not talk at all.
When talking with a child who has A-T, it’s common to understand part of what he/she said, but not the entire sentence. In these situations it’s often helpful to let the person know and confirm what was understood. That way, the person has to repeat only the second part of the sentence. This is an important technique to use and consider. It’s likely that if the person keeps repeating the entire sentence, he/she will continue having trouble around the same point in the sentence. The difficulty may be the result of decreased breath support necessary for speech in combination with trouble saying sounds efficiently. This can become a frustrating circle for everyone involved and sometimes results in the person saying “forget it” because the process is too hard. After the person knows what wasn’t understood, he/she can continue or repeat the sentence from that point and hopefully this will break the cycle of constant repetitions. Over time, we’ve learned that some children who have A-T will take advantage of this technique and others won’t, regardless of how much they are encouraged by their family members. Given that it can reduce frustration with communicating, it’s often worth trying, but it may be a personal choice that the person with A-T ultimately makes.
Language weakness may create difficulty with social interactions and school performance. When a weakness cannot be corrected by therapy, teaching the person ways to compensate for the area of weakness is important. Fortunately, an area of strength for many persons with A-T is the ability to listen to information presented auditorily. The ability to remember things immediately after they are said (e.g., immediate recall) and interpretation of the material often remains stable, even when other neurologic functions deteriorate. This auditory strength can be used to offset other areas of language weakness.
Often the person with A-T needs more time to process information and to respond to a request, verbal or otherwise. Since the person with A-T doesn’t always indicate that he/she needs time, it is helpful for others to know that this may be an area of need. Whenever possible, it would be helpful if the person could indicate that he/she needs more time or assistance. It may be helpful to develop a nonverbal system such as lifting up a hand to signal that more time is needed. If the child has a 1:1 assistant in school, this person could also help communicate the need for more time. If the person isn’t able to convey the need for more time, it would be helpful for others to ask, “Do you need more time or is this too hard?” This would provide the person with the option of providing a response or to move on.
If a person has trouble with word retrieval and word recall, he/she needs continued experience and exposure with a variety of activities involving vocabulary. Obviously using classroom vocabulary is important for students, since the person will need to have easy access to these words while in school. In addition to understanding a word, the person needs to develop and expand word knowledge. Knowing the word category and the descriptive qualities of the word will be helpful ways of expanding word knowledge. Playing games where one person states items of inclusion and exclusion for specific categories is also a good way of expanding word knowledge. One example of a game would be to take turns choosing different categories. One player would list as many things as he/she within the category and count who can think of the most items. Another game would be to state two or three items in a category along with an item that doesn’t belong and have the person find the inappropriate item. For example, for the category “fruits,” state the items “apple, orange, stove, banana” and see if the person is able to find “stove” as the inappropriate item. Another version would be to describe objects by explaining the color, function, and location and having the other person name the object. Also, a more challenging version would be to determine a category and then a specific characteristic from within the category and name related items. For example, for “fruits,” you could find all the fruits that grow on trees or describe all the different fruits that are “red.”
In general, when it’s obvious that the person is having trouble finding a word, there are many ways to help. Encourage the person to describe the word as extensively as possible, or ask questions to try to identify the word. Then provide verbal or visual cues to the word category or the function of the word. As cues are provided, allow some time to see if the word can be recalled. If continued assistance is needed, provide just the beginning sound or the first syllable of the word, followed by a pause to see if the word is recalled. If the word is a multi‑syllabic word, continue in this manner to see if recall can be stimulated. Cueing should be provided from the lesser to greater degrees to assist in recall (i.e., an active thinking process) instead of simply stating the word (i.e., a passive response).
When following directions, it may be helpful for the person to learn to listen for key words and to “chunk” (e.g. group) important words that are essential to completing the task. The person may need some help and training to be able to recognize the important words in the direction. For this purpose, it may be helpful for the person to repeat the direction after it is given to him/her, either in its complete form or after he/she has “chunked” the direction. This gives the person practice and it lets the listener know if the direction was heard correctly. After the person has completed the direction, he/she should repeat the direction again to make sure his/her response was accurate.
Oral motor skills are affected in A-T, impacting both speech production and the ability to eat/drink. It would be logical to think that addressing oral motor skills would be an important component to address for the person who as A-T, especially if someone isn’t familiar with A-T or its characteristics. Even though the oral system is involved, working on improving muscle strength may not be helpful. Also, the long-term benefit of performing oral motor exercises is not clear, either for functioning related to swallowing/eating and saying speech sounds for talking. For that reason, addressing oral motor skills in someone who has A-T usually isn’t a general or standard recommendation. If it is decided to implement an oral motor program for someone with A-T, objective ways of measuring progress should be established and progress should be closely monitored. As with other forms of muscle use and exercise, however, it is important that the person does not overuse his/her muscles to the point of fatigue. The person’s progress and performance should be closely monitored and continuing with these exercises should be decided by the child (when appropriate), family, and therapist (when involved). Again, it should be remembered that there is not long-term data that concludes whether these exercises are beneficial.
Drooling is a common problem for a person who has A-T because of the decreased oral motor skills. Sometimes difficulty with drooling changes and may be absent or present as the child grows. Also, this is an area of difficulty for some children and not for others. It’s not clear why some children experience drooling while others don’t or why the problem can fluctuate over time. To address the drooling, it may be helpful for the person to “slurp” or retract the saliva into the mouth. As a first step, this technique may need to be taught, although it may be hard for the person to learn. If the technique is learned, it may be helpful to create a nonverbal cue, such as touching your cheek, combined with saying the person’s name to signal the need for it. Developing a neutral nonverbal cue has two purposes. First, it requires active involvement from the person instead of expecting a passive response to a verbal directive. Second, it removes any social stigma the person may feel by having attention drawn to the problem.
If the person isn’t able to control the drooling, it may be helpful to consider other options. One would be to wear a tennis band around the wrist that the person can dab his/her mouth with. Another option would be for the person to wear a kerchief around the neck that would absorb the salvia. The kerchief could be changed after it gets wet. This provides a way to address the problem without it being too noticeable or obvious.
Respiratory skills are driven by underlying trunk support and overall motor skills, which then have an impact on oral motor skills and affect speech production. Having adequate breath support is necessary to maintain voice volume so that speech can heard and understood. Sometimes as motor skills are affected respiratory skills decrease with a direct result of speech becoming quiet and hard for others to hear. Again, it would seem logical that a person’s respiratory skills be a focus of therapy tasks. However, once again, long term effects and benefits to performing respiratory exercises in A-T patients are not known. As a result, including these in a person’s therapy plan usually isn’t recommended.
Sometimes postural modifications can be done to help increase underlying respiratory support necessary for speech. These can include making sure that the person sits with his/her feet firmly on the floor. In addition when seated in a chair, it may help to take a small towel or cloth and roll it up either length- or width-wise. The towel could be placed between the shoulder blades, length-wise, or across the back at the level of the shoulder blades or at the small of the back, width-wise. Sometimes it helps to have two small towels at both of these points across the back. Providing this support may bring back a person’s arms and shoulders, which will also open up his/her chest. This should allow for more respiratory support and may help with speech, especially the loudness. If the person moves around a lot in the chair, keeping these supports in place may be hard. Even so, it may be worth trying.
Even though a person with A-T has dysarthric speech, his/her speech usually can be understood with careful listening and by watching his/her face while talking. As a result, the person with A-T usually can make his/her needs and wants know verbally, especially someone gets more familiar with his/her speech. Implementing these compensatory strategies also assists in getting the person’s message across. Even with slurred speech or dysarthria, alternate methods of communication through an augmentative communication device are not always needed. Also, the presence of ocular motor apraxia can make using a device challenging.
Even so, if the person isn’t able to make their needs and wants known verbally or be understood more than half the time (around 60%), then the use of an augmentative communication device may be helpful. Using both low-tech picture boards and high-tech voice output devices should be investigated by someone familiar and comfortable with augmentative communication devices and the different components related to A-T. Sometimes it’s helpful to use an augmentative communication devices and picture communication boards to assist the person in communicating in particularly challenging situations (e.g., unfamiliar listeners, noisy environments, etc.). It’s important to keep in mind that even the most high-level, dynamic devices can’t take the place of and aren’t as fast as the brain-mouth connection. When someone is frustrated with his/her attempts at communicating and avoids talking because his/her message can’t be understood, then investigating alternate means of communication is important.
Using and implementing these strategies with the person who has A-T can have a powerful effect. Communication is a potent force in our lives and helping someone maintain skills in this area provides positive rewards for everyone involved.
Disclaimer: The information provided on this website should NOT be used as a substitute for seeking professional medical diagnosis, treatment or care. You should not rely on any information in these pages to replace consultations with qualified health professionals.