Speech, language, voice and swallowing in the Ehlers-Danlos syndromes
Angela Hunter, Speech and Language Therapist (retired) and trustee, EDS UK
Please note: The following text cannot and should not replace advice from the patient's healthcare professional(s). Any person who experiences symptoms or feels that something may be wrong should seek individual, professional help for evaluation and/or treatment. This information is for guidance only and is not intended to provide individual medical advice.
This information is based on symptoms that can sometimes affect people with EDS. There is limited published evidence to support this and further research is necessary to confirm the relationship between these symptoms and the condition. However, there is a significant amount of clinical evidence and prevalence among EDS patients. It is important to remember that just because you have EDS does not mean you will necessarily have these symptoms.
Having Ehlers-Danlos syndrome (EDS) does not mean you will have any or all of the following difficulties. However, the underlying altered collagen opens you up to the possibility. This is because collagen is in all the tissues and structures involved in speech and swallowing, leading to: stretchiness of ligaments, potential muscle fatigue or weakness, and altered structure of the mucous membrane of the mouth and teeth. Ear-related difficulties such as recurrent infections, reduced function and effectiveness of the jaw, oral soreness and dental pain can all reduce our ability to maintain clear speech, creating difficulties with voice, chewing, speaking and swallowing. It is now known that in EDS proprioception (your sense of where your joints are in space) can be altered, creating further difficulties. The ‘biting position’ of the teeth can also be altered with the changed movement of the jaw increasing the risk of delayed swallowing. The occurrence of these problems is now known to be above that of the general population. The effects of these issues may be mild or severe, periodic or permanent. They may be evident during childhood, puberty, adulthood or appear with ageing.
If you are concerned about any of these areas the advice of a Speech and Language Therapist (SLT) should be sought, as there may be remediation techniques that can improve your quality of life. Advice sooner rather than later is important to reduce unnecessary wear and tear, as well as to highlight where referral to another specialist is required. This may be an ear, nose and throat specialist, an oral surgeon, a gastroenterologist or educational support. Communication and eating difficulties can create immense frustration for the sufferer and their partner. Help is needed to avoid these difficulties affecting learning and quality of life more than they need to.
This is the ability to produce the sounds of speech clearly and repetitively in the sequences of words, at speed. It is only when articulation skills do not develop as expected that we become aware of the complexity of this skill.
Attaining clear articulation can be delayed with the EDS child, needing longer to acquire clarity. An inability to talk clearly and quickly at all times may persist into adulthood. Many people with EDS report quickly tiring to the point where their speech is no longer clear. Using a telephone can become very painful. Articulation can also be affected by factors such as pain or dental problems.
Voice is another area of communication we all take for granted until it does not function as we need it to. Producing and maintaining voice is a reported difficulty. The way posture, breathing and the voice box need to coordinate to achieve a voice can be affected. It is becoming known that those with EDS can have respiratory difficulties, including asthma. Younger children can only produce a vocal sound if they shout, reaching their first decade before their ability to consistently initiate voice is established. In adulthood, there are reports from people of poor voice maintenance and a reduction of the vocal range, even from their 20s and 30s. People who have been competent singers find their ability reducing a couple of decades early. In women, hormonal ﬂuctuations exacerbate difficulties. Difficulties with a husky voice on waking could indicate acid reflux has occurred overnight.
There are reports of word-finding difficulties across all age groups, with children needing longer to acquire complete, grammatically correct language. Again, these difficulties occur more frequently than in the general population. Problems with memory and alertness are also often reported by those with EDS and, while not researched, it is again possible that tiredness can increase the word-finding problems. As well as physical and mental fatigue, it is possible that chronic pain, medication and/or postural tachycardia syndrome affect word retrieval. In vascular EDS, word-finding and other communication difficulties can occur following vascular incidents within the brain, e.g. stroke, transient bleeds.
Chewing and swallowing
The jaw or ‘temporomandibular joint’ (TMJ) is one of the more complex joints in the body. It is open to the same additional wear and tear and strain of any other EDS joint. The joint contains a disc (meniscus) so any incorrect movement and/or subluxation can cause damage creating tiny pieces of debris. This debris within the joint can further impair functioning. If this happens the advice of an Oral or Maxillofacial Surgeon must be sought. This disc can also become displaced.
To chew and swallow well we need to be able to bite our food, then chew it by moving it around in our mouths, until it is ready to swallow. A very complex combination of chemical, mechanical and neurological receptors send messages to our brain that a swallow is needed. This in turn stimulates the muscles of the respiratory and digestive systems to coordinate so that the swallow is triggered and achieved safely. A safe swallow is where the food goes smoothly down the oesophagus (gullet) into the stomach while the airway is protected for the short period of time needed for this to happen.
There is increasing awareness that many of the connective tissue disorders (such as EDS) have implications for swallowing, causing pain and difficulty with eating. Delay in triggering the swallow, food residue being left behind in the throat after a swallow, and slowed movement through the oesophagus have been reported. It is also possible that any difficulties which exist are exacerbated by normal hormonal ﬂuctuations.
Any difficulties with safe eating and drinking and comfortably achieving adequate nutrition should be discussed with a medic, and a Speech and Language Therapy consultation should be sought.
Self-help and remediation techniques
Voice and vocal care
Producing the voice makes use of many muscles and ligaments attached to the cartilages of the voice box. These in turn produce the tensions in the vocal cords needed to produce voice. Along with these movements there must be the coordination of posture and breathing. As with any physical exercise, a degree of ‘warming up’ before use can improve performance.
Any general bodily hypermobility or fragility of tissues could, therefore, upset the smooth working of the voice by making these movements more difficult or by the tissues of the voice box becoming more easily inﬂamed. Ongoing vocal discomfort lasting longer than six weeks should always be discussed with a doctor even if the diagnosis is that the problems are ‘functional’.
The voice in EDS may be subject to extra pressures, however there are many ways in which the speaker can help him/herself. In fact, the intricacies of vocal care are so individual that advice from a SLT should be sought if possible. Because of the individual nature of vocal needs, it is only possible to give general advice on good voice care and production. Usually a series of exercises would be recommended to build up vocal stamina. Where EDS is concerned, any repetitive exercising must be done with care. Warming up can be done at the beginning of the day by breathing as outlined below, humming and repeating phrases which contain nasal sounds, e.g. “man in the moon”, “meet me at noon”.
Factors involved in good voice production
Always ensure that you are as upright as possible and that your head and neck are in line, with your chin slightly tucked in.
To vocalise well we need a good supply of breath. This is achieved by breathing from the diaphragm and filling the lungs with air by allowing them to expand outwards. It is possible to breathe incorrectly by pulling the lungs in during inhalation. This is not useful and reduces the amount of air available. It is also harder work. It can help to become more conscious of your breathing pattern by placing your hands just below your ribs and feeling the movements. If you lie on your back gravity will make it easier for you to feel the expansion and contraction of your diaphragm. As you exhale, let your breath out firstly to a count of three and then increase the count to six or eight.
There are many exercises which can be done when development is delayed to help development of clearer speech. Initially the youngster needs to be able to hear the difference between the target sound and what they actually say. For example, if they say t for k can they show tap or cap, when asked, if shown the two pictures. If trying to use a newly-learnt sound in words, it can help to practise saying the words with the sound slightly separated, e.g. s-spoon, c- ake. Where there is a difficulty with blends of consonants, e.g. sk, sl, sm, or clusters, e.g. skr, spl, str, these too can be improved by practising them in parts and then adding them to the word. A SLT can advise on the expected ages for sound acquisition.
Where the problem is in maintaining clarity over phrases and sentences, then again, some practice of exercises may help. Repeat strings of consonants such as ptk (puh tuh kuh) or f-s-sh or the words “buttercup, buttercup, buttercup” which mean the tongue and lips are having to move about. Begin at a pace which allows clarity and then aim to speed up. Repeat each string six to ten times.
It may also help to remember to keep phrases short. If the speech is unclear to the point of needing repetition, then begin the repetition at the unheard word, rather than going back to the beginning of the utterance each time. Always make sure you have enough breath to finish the phrase, if not then breathe more often.
Whatever the problems, do not avoid difficult situations or words, as this eventually severely cuts down the words your confidence will allow you to try. This can really restrict your overall freedom in conversation.
We all develop language at different rates and in various ways, and some apparent delays are remediated on their own. Once again, any cause for concern should be investigated so that help can be given, if appropriate.
This is the bringing together of the vocal cords as air passes over them. The cords need to be brought together firmly. Using them only partly to whisper, either during voice loss or at other times, increases the strain. This adds to the wear and tear, lengthening the period of voice loss and overall problem. Sometimes nodules (benign growths) can prevent the cords from working well. People who use their voices professionally are subject to them. Therefore any ongoing voice loss must be discussed with a doctor.
Obtaining a voice may be difficult in EDS, possibly because the cords or ligaments are not holding firmly enough. It may help to achieve initial closure by imagining that you are pushing a heavy box and making an “ugh” sound as you push. I stress you ONLY IMAGINE the act of pushing (you don’t want to increase pressure inside the skull which could affect the eyes and vessels). In this way, the cords get used to hitting together. Particularly with voice production issues, I recommend individual programmes designed by a SLT.
Hypernasality (a speech disorder) may occur too, due to the laxity of the muscles allowing too much air to flow through the nose. A SLT can measure this very easily and give exercises to help increase the muscle tone. This laxness can also effect swallowing.
We all have natural resonators in our heads – the sinuses and the bones of the skull. Use of these will add body to the voice. Humming exercises can make you more aware of the effect of resonance. Also remember that our environments are resonators and if you have to speak for a long time then ensure there is some natural resonance around, i.e. do not try giving a talk in a bare warehouse!
Fluency of speech
During the development of articulated speech some non-ﬂuency can occur. This will usually resolve itself. However, if self-resolution does not seem to be occurring or if there is a family history of non-ﬂuency, then help should be sought.
Most of us find our pitch naturally, but it is possible to begin using a different pitch during voice loss or at other times when a change in pitch seems to help. This can then lead to ongoing use of incorrect pitch which leads to strain. To find your optimum pitch, sing up and down a few scales until you find a comfortable point.
There is nothing wrong with using increased volume for short periods of time but this should not be a regular thing. If you need volume, then ensure you are following all of the suggestions here. If you have a problem maintaining voice at volume, this relates to the working of the cords and breath coordination. Again, individual advice should be sought.
Although we can do nothing to stop the effect of hormones on our voices we do need to be aware of them. Puberty is obviously one time when things may either improve or worsen. The menstrual cycle can also affect the voice. Opera singers in the international companies are not expected to sing at a certain time in their cycle.
The environment in which you need to use your voice, or to which you are frequently exposed, can affect the overall health of your voice. Dusty areas, air which may contain irritants or the need to speak against background noise should be taken into consideration if you have ongoing vocal strain, hoarseness etc.
To help in good voice production, we need to be generally relaxed. Bodily tension will add to vocal difficulties. It is possible to be more tense than we realise and carrying out a general programme of relaxation can help.
It is, of course, quite possible for psychologically-induced vocal problems to occur. However, this is not a typical cause in EDS, and even if these factors are at play you can still benefit from vocal therapy and the insight this can give.
Maintaining your voice
This can be as difficult as maintaining any other repetitive, physical activity. Therefore, voice self-help is more about planning. Make sure you can have times when you can stop talking, e.g. a teacher writing on the board. If possible take frequent rests. Having a drink such as water to hand to keep the voice lubricated may help if swallowing during the need for talking is not likely to add to your problem. Sips every couple of minutes have been found useful by some people, as this keeps the oral structures and vocal cords hydrated. Avoid drinks with caffeine or alcohol as these tend to dry out the tissues.
Eating, drinking and swallowing
Dietary modifications to a softer diet may be needed. Biting of things such as apples, chocolate bars and even toast can overstretch and aggravate the jaw (TMJ). This is compounded if the ‘bite’ achieved between top and bottom teeth is poorly aligned.
Avoid foods which require long chewing times such as steak or else prepare them in ways which ease the chewing. Do not chew gum. Not only does this create wear and tear, it can also lead to problems with the excess gastric juices which are created.
Maintain good neck and oesophageal posture during eating and swallowing as this will help the transit of the food or ﬂuid. The chin should be slightly tucked in as we swallow.
If you need to chew for a long time or your swallow is hard to make or does not happen, seek help.
If you feel a lump, or food returns or you cough/choke often after a swallow, discuss this with your doctor. Recurrent chest infections can be an indicator of swallowing difficulties and/or acid reflux.
Try to talk in shorter rather than longer bursts, as talking will aggravate a problematic TMJ. A simple TMJ exercise programme may help too, as underuse can create stiffness in the joint.
Peer reviewed by: Bonnie Heintskill, Speech Language Pathologist and Sue Rae Craemer, Speech Language Pathologist (retired)
Date of last review: 01/10/2017