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Treatment of Hypernasality in Children with Down Syndrome - featured September 12, 2011

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Treatment of Hypernasality in Children with Down Syndrome

By: Bridget A. Russell, Ph.D., CCC-SLP
Department of Speech Pathology & Audiology
State University of New York Fredonia

Abstract:

Adenoidectomy has been reported to be a causal factor of velopharyngeal insufficiency (VPI) and associated hypernasal speech in patients with and without palatal and oral-pharyngeal defects. Children with Down Syndrome (DS) often have tonsillectomy and/or adenoidectomy to treat otologic, upper airway and sinonasal disease. Secondary to their altered head and neck structures, there is an increased possibility for VPI. Treating hypernasality may require surgical intervention including sphincter pharyngoplasty or pharyngeal flap, prosthetic devices or behavioral speech therapy to improve velar contact with posterior and lateral pharyngeal walls. Specific speech treatment regimens may include direct articulation-phonological therapy, biofeedback and muscle training. Combined surgical intervention and speech therapy may also be implemented. The following discussion addresses previous and current surgical, physical and behavioral treatment regimens to improve hypernasality in patients with VPI.

Introduction:

Down Syndrome or chromosome 21-trisomy syndrome is a type of mental retardation, occurring in approximately 1 in 800 live births. Typical physical deficits in children with DS include abnormal head and neck structures which result in sinonasal disease, upper airway and otologic problems (Price, Orvida, Weaver & Farmer, 2004). Anatomical differences include macroglossia, hypoplastic nasal bones, a narrower, less voluminous nasopharynx and oropharynx and eustachian tubes which have a less acute angle to the hard palate and a smaller diameter (Brown, Lewis, Parker, and Maw, 1989).

VPI is described by any of the following: velopharyngeal inadequacy, velopharyngeal insufficiency, velopharyngeal incompetence, or velopharyngeal dysfunction. These terms are used interchangeably to denote any type of velopharyngeal closure problem. This article uses the term velopharyngeal insufficiency or rhinolalia aperta to describe the failure of apposition of the soft palate and orophayngeal wall during speech, which is the main cause of hypernasality. In hypernasality, air escapes into the nose causing difficulty with speech, especially high pressure consonants such as plosives and fricatives. However, in the clinical management of these disorders, various etiologies require different management approaches.

As a result of anatomical differences, children with DS often present with symptoms such as; snoring, sleep apnea, nasal drainage, nasal congestion, mouth breathing, acute and chronic otitis media, drooling and tongue protrusion. Due to upper respiratory and structural differences, children with DS often undergo surgical procedures to help alleviate these symptoms. One procedure regularly performed is tonsillectomy with/without adenoidectomy (“T&A”). These procedures are often recommended to correct sleep apnea and other obstructive symptoms, despite controversy regarding their effectiveness (Price, et.al., 2004). Often, these surgical treatments do not account for the possible complications or side effects that may occur secondary to changing the anatomical structures in these patients. DS children have smaller-than-normal adenoidal pads and adenoidectomy may not have the expected positive result on nasal respiration and otologic problems as was previously thought (Kanamori, Witter, Brown, Williams-Smith, 2000). Furthermore, it has been noted that reducing the already small adenoidal pad in DS children may cause associated velopharyngeal insufficiency and therefore possibly creating hypernasal speech (Kavanagh, Kahane & Kordan, 1986).

Estimates of the incidence of velopharyngeal insufficiency after an adenoidectomy in non-syndromic patients has been estimated between 1 in 1500, to 1 in 10,000 procedures (Parton, & Jones, 1998). Other studies indicated that DS children post-T&A have benefited from the procedure by eliminating or reducing symptoms such as snoring, sleep apnea, nasal drainage, and mouth breathing, but not drooling or tongue protrusion. This data was reported via a parental questionnaire survey of 74 parents of children with DS (Price et al., 2004). The same report indicated two children had hypernasality after surgery. The children were given complete speech and language evaluations and additional assessment using cinefluoroscopy. Structural and functional causes of hypernasality were identified. Structural abnormalities included a high-arched short hard palate and a short soft palate. Contributing functional factors included hypotonia, slowed motor learning and oral motor developmental delay. The incidence of postoperative hypernasality found in these patients was higher than in the general population and therefore is essential to consider prior to the performance of an adenoidectomy in DS children (Kavanagh, 1986). The likelihood of post-surgical complications and possible secondary surgical procedures included chronic ear drainage and subsequent ear surgery, post-extubation stridor and respiratory problems. Whichever decision is made regarding adenoidectomy, the surgeon and family should be well informed of these recent findings to appropriately weigh the potential benefits and risks.

Etiology of VPI:

VPI originates from a number of possible etiologies; structural (i.e. cleft palate and post-surgical insufficiency), neurologic (i.e. cerebral palsy, dystrophies and myotonias) and speech sound “mislearning” (i.e. compensatory misarticulations, hearing impairment, and postoperative nasal emission). The specific etiology needs to be discerned in each case to provide a tailored treatment. VPI in DS children typically originates with non-cleft VPI caused by mechanical interference and post-surgical insufficiency. Adenoid removal may indeed impair velopharyngeal closure, as the previously-in-place bulky adenoid potentially facilitated velopharyngral closure. However, once the bulky adenoid is removed, hypernasal speech may result. This condition will further degrade intelligibility of children with and without DS and thus, surgical, physical or behavioral remediation would be employed to treat the speech deficit.

Current treatments for hypernasality in cleft and non-cleft VPI may include surgery, prosthetic devices, and physical management including muscle training and traditional speech therapy, or a combination of these treatments. Speech therapy is usually combined with other treatments to provide long-term benefits. If hypernasality is the result of learned behavior, speech therapy alone may be recommended. If a problem in the patient’s anatomical structures is causing hypernasal speech, surgery may be the suggested treatment.

Surgical Protocols to Reduce Hypernasality:

Surgical procedures can reduce the gap between the palate and pharynx and lessen the air leakage into the nose, to make speech sound more normal. Typically, surgical procedures for hypernasality take about 1½ hours to perform and require one or two nights in the hospital. In a small number of children, a second operation may be necessary. The following list of surgical procedures will be tailored to the nature of the problem, included are those procedure that are a possibility with DS children with VPI issues.
  • Furlow Palatoplasty – This technique is typically performed on children with cleft palate. The Furlow Palatoplasty realigns the muscles of the soft palate while lengthening the structure. The additional length makes it easier for the palate to contact the back of the throat, closing the gap which previously facilitated hypernasality. Realigning the muscles of the palate may also reduce middle ear disease in children with cleft palate and DS. In the 1990’s, Children’s Hospital of Philadelphia reported a substantial improvement in the results of ameliorating VPI through primary and secondary Furlow surgery (i.e. Furlow double reversing Z-plasty (Furlow, 1986). Their success was apparently due to lengthening and improving the function of the velum.
  • Pharyngeal Flap – This technique consists of sewing a flap of tissue from the back of the throat into the palate to block the back of the throat and alleviate air leakage into the nose. Two holes are left on either side of the flap for breathing and nasal drainage. Sometimes this procedure “overcorrects” the deficiency, causing obstruction of the nose. Obstructive sleep apnea is a relatively common complication after pharyngeal flap surgery. Although pharyngeal flap surgery was previously a common surgical technique, newer techniques with fewer complications are becoming more popular.
  • Sphincter Pharyngoplasty – During this procedure, flaps of tissue from the back of the throat are used to build a "speed bump" in the nasopharynx (area behind the soft palate) which helps the soft palate connect with the back of the throat. The size of the "speed bump" is tailored to the size and shape of the gap at the back of the throat and cannot be seen when looking into one’s mouth.

Primary and secondary surgeries to improve VP closure in DS (and other patients) do not always improve VPI symptoms. Although it is helpful to know the details of the various surgical procedures, the SLP’s primary interests are more often examination, evaluation and treatment of the post-surgical patient. For example, the oral peripheral examination might entail recognizing a mid-sagittal pharyngeal flap in the client’s mouth. The flap might, or might not, be low enough on the pharyngeal wall to be visible to the clinician. During the oral-peripheral exam, SLP’s evaluate velar and pharyngeal wall movement during production of a repeated vowel (typically /a/). However, velar movement might be greater during connected speech, rather than isolated vowel production. Velar movement cannot typically be visualized or evaluated via the oral examination.

Prosthetic Treatment to Reduce Hypernasality:

A speech prosthesis is a removable plastic plate that fits over the hard and soft palates and is anchored to the upper teeth by metal clips. At the back of the plate is an extension of plastic, which acts to improve speech. Speech prostheses are similar to removable dental braces used by orthodontists to straighten teeth. A speech prosthesis is recommended when children are considered poor surgical candidates. A trial period with a removable prosthetic may be recommended to gauge how successful a surgical procedure might be, if performed at a later date. Creating the removable device requires weekly or biweekly visits over the course of several months. Two devices that can be used by patients with VPI are palatal lift appliances (Figure 2) and speech bulbs (Figure 3).

Figure 2: Palatal Lift Appliance

[Image: hypernasality2.jpg]
(Reproduced with kind permission of North Thames Regional Cleft Centre, Great Ormond Street Hospital.
Copyright Great Ormond Street Hospital 2001.)

A palatal lift device acts to lift the soft palate upwards and backwards into full contact with the posterior and sometimes lateral pharyngeal walls. The appliance also positions the velum so that the least amount of muscular motion is necessary in the pharyngeal walls to attain VP closure. This device is recommended if the soft palate does not move very much during speech, but appears long enough to reach the back of the throat. These devices are also useful in non-cleft patients when there is minimal movement of the velum such as neurologically impaired patients. Problems with this device include articulation precision after the initial positioning of the appliance and dental stress.

Figure 3: Palatal Speech Bulb
[Image: hypernasality3.jpg]
(Reproduced with kind permission of North Thames Regional Cleft Centre, Great Ormond Street Hospital.
Copyright Great Ormond Street Hospital)

Speech bulbs work best when the device does not have to close a large VP gap and can be designed to close off “most” of the gap and leave a small space for nasal respiration. The device is most successful in patients that have adequate inward movement of the pharyngeal walls to improve closure. Speech bulbs are recommended when surgery is not possible because of medical or anesthetic risks, or sometimes when the gap is so large that surgery is unlikely to work.

After the device is fitted on the patient, the SLP’s direct therapy will involve: 1) changes in speech sound production, 2) teaching the differentiation of production of sounds produced with nasal emissions and those produced with oral pressure and airflow, 3) remediate compensatory strategies used prior to the placement of the device, and 4) develop procedures to practice and facilitate oral production of various sounds. Additionally, there are social aspects of wearing prosthetic devices, and the SLP, parent and patient can work through and address these concerns, too.

Physical Exercise to Improve Hypernasality:

Much of the early literature on treating VPI centered on physical therapy principles which stressed improving muscle tone and strength through tactile stimulation, repeated contraction of muscles and resistance exercises. Speech pathologist’s activities included; blowing, whistling, swallowing, sucking, cheek puffing and blowing against resistance. These activities were controversial and results were bleak in terms of their ability to effectively change velar movement during speech activities.

Nonetheless, temporary changes in VP activity were reported (Peterson-Falzone, Hardin-Jones, and Karnell, 2001). However, the idea that these “exercises” strengthened the palatal musculature came under scrutiny. Kantner, 1947, suggested the act of “blowing” will not exercise the palate unless the palate moves during the exercise. Kantner indicated any gains made during the exercise would not automatically carry over to connected speech. McDonald and Koepp-Baker (1951) added to the downfall of these exercises as a palatal strengthener. Powers and Starr (1974) demonstrated that palatal exercises involving sucking, blowing, swallowing and gagging resulted in no significant improvements in nasality or VP functioning.

Another prosthetic intended to serve as a palatal exerciser was developed and tested in 1969 (Lubit & Larsen, 1969). The device functioned using a maxillary-retention segment and an attached inflatable bag. By inflating the bag, the velum was moved in a superior-posterior direction and exerted pressure against the posterior wall of the pharynx. The results showed no indication that VP functioning was improved and it was suggested that the device provided resistance to the velar depressors, rather than elevators (Lubit & Larson, 1969).

The majority of studies regarding palatal exercises to improve VP closure have resulted in a lack of success with these treatment methods. There is no evidence these exercises can be used specifically to develop the range, skill of execution, and flexibility of movement required during normal speech production. Therefore, it can be concluded these exercises should not be used routinely in clinical practice.

Palatal exercises have not been successful in improving VP functioning in part because the mechanism for closure is different during speech and non-speech activities. Therefore, any attempt to improve VP closure through muscle training has to be specific to “speaking tasks” and if muscle strength and endurance are goals of treatment, resistance must be used in the training principle. Recently, a promising new therapy approach using resistance training methods has been investigated using continuous positive air pressure (CPAP) (Kuehn, 1991). This technique assumes that the individual can "learn" how to use the mechanism in a more appropriate manner thereby improving functionality for speech. However, there may be physical limits that must be overcome before additional modification in behavior can be achieved. An example of such a limitation might be insufficient muscle strength or endurance coupled with a threshold of fatigue that is relatively low. Children with DS might benefit from the general muscle strengthening provided by CPAP, to the muscles of the VP mechanism.

Continuous Positive Airway Pressure (CPAP):

One approach to alleviate low muscle tone is to increase muscle strength or endurance using a resistance exercise regimen focused on the muscles of the soft palate. Kuehn (1991), proposed a resistance-based treatment for strengthening the muscles of velopharyngeal closure that utilize principles of exercise physiology. Resistance to the muscles of the VP mechanism is delivered via artificially increased nasal resistance through increased air pressure to the nasal cavities. Air pressure is delivered using a commercially available device referred to generically as continuous positive airway pressure (CPAP). The operational hypothesis is; if patients had to work against resistance (i.e. nasal air pressure), the muscles of the soft palate would work against the added intranasal pressure and thus gain strength.

The physiological principles of CPAP-based treatment include: overloading, progressive resistance, and specificity of the exercise regimen, all of which are known to increase muscle strength. By increasing the load on a muscle group it adapts and strengthens in direct correlation to the weight used. Progressive resistance refers to a gradual increase in exercise intensity over time, i.e., muscles adapt to an increased load. Specificity involves exercising the muscles in question so their corresponding motor nerve pathways will be activated during the task and increase strength. This is an important consideration with regard to the muscles of VP closure because non-speech activities, such as swallowing and blowing, are still frequently used in an attempt to strengthen the muscles. However, it is known that non-speech tasks, such as swallowing, involve very different VP movement patterns compared to that for speech. Thus, it is advantageous to provide resistance to the muscles of velopharyngeal closure during speech.

The CPAP instruments consists of an air pressure generating source and a hose and mask assembly and is commercially available for use in a patient’s home. The treatment protocol used by Kuehn, was 8 weeks in duration with incremental increases in intranasal pressure and duration of application each week over the entire treatment period. The subject produced specially developed utterances; vowel-nasal consonant-[pressure] consonant- vowel (VNCNs), such as /insi/. These nonsense words were designed to lower the velum during the nasal consonant (N) followed by a rigorous elevation of the velum for the following pressure consonant © much like the power lift in weight training. Initially, only single words are produced, and then, over time, sentences are produced to imitate conversational speech.

The CPAP approach is different from previously described muscle training exercises in that exercises are preformed during speech rather than non-speech tasks. CPAP results indicate a decreased perception of hypernasality determined by listener judgments, although average decreases in hypernasality scores were small (Kuehn, Imrey, Tomes, Jones, O’Gara, Seaver, Smith, Denmark, & Wachtel, 2002). Although none of the subjects had severe hypernasality, it was thought that patients with severe hypernasality would have potentially demonstrated a greater response to the treatment (i.e. greater decrease in mean hypernasality scores). Additionally, instrument-based measures might provide a greater change in hypernasality than listener perceived changes.

Biofeedback Techniques to Decrease Hypernasality:

The difficulty in using physical therapy principles with muscles of the VP mechanism relate to the sensory input differences present, as compared to other oral structures, such as the tongue. We have a great awareness of lip, tongue and teeth movement and can exactly create movements when verbally commanded, such as tongue movements during an oral examination). However, if asked to maneuver our soft palate, most people would not be able to tell you what position their velum is in from one moment to the next. Thus, most SLP’s rely on the patient’s speech production (i.e. auditory information) to provide feedback on the adequacy of VP closure. Treatment outcomes improved when therapists began to use a variety of biofeedback techniques that provided visual and/or auditory feedback to the patient. Biofeedback attempts to change a bodily function through self-observation of that function, using a monitoring device. Several devices have been used to provide visual and auditory feedback. Some of these will be discussed below.

Sea-Scape:

The Sea-scape device is one of the oldest biofeedback methods to indicate nasal airflow and the general functioning of the VP mechanism. This device does not measure VP closure deficits, but rather monitors them. The device is comprised of a rigid plastic or glass tube containing a small Styrofoam ball. The base has a flexible tube attached on one end and a nasal olive attached on the other end. The nasal olive is to be partially inserted into the nostril of the patient. Then the Sea-scape is used as a monitoring device for excessive nasal airflow during the production of oralized consonants. The Styrofoam ball rises if excessive airflow is present during speech. However, sometimes during normal breathing the Styrofoam ball will rise, too. Both sides of the nose should be tested as blockage could occur on only one side, such as might be found with a deviated nasal septum. Lastly, children tend to play with the device to watch the ball move and inaccurate assessment of nasal flow may occur. Although awareness of these potential pitfalls is important, the usefulness of this device to monitor and provide visual feedback to the patient remains strong.

Using the Sea-Scape during the Whistling-Blowing Technique:

One therapy using the Sea-Scape was designed to develop VP closure during speech by using the natural VP closure that occurs during the act of whistling or blowing (Shprintzen, McCall, and Skolnick, 1975). Four subjects (4-19 years) were asked to phonate during whistling and/or blowing and a Sea-Scape was used to monitor nasal airflow. Once the subject practiced whistling/blowing and phonating, the non-speech task was slowly eliminated. All subjects improved VP functioning as observed using videofluoroscopic examination tapes and reduced air leakage as monitored using the Sea-Scape.

Endoscopy:

In the 1970’s and 1980’s, improved visualization of the laryngeal and VP structures was developed through oral video-endoscopy. Endoscopes allowed “live” visualization of VP musculature and helped ensure proper diagnosis of VPI and related disorders. Some studies examined using endoscopy as a biofeedback tool.

In one study of normal subjects (Siegel-Sadewitz and Shprintzen, 1982), the ability to alter VP closure patterns from coronal to sagittal was explored. However, none of the subjects in that study had VPI. Other studies demonstrated success using nasal endoscopic feedback to remediate VPI in patients with palatal clefts. These studies showed subjects had to learn to achieve VP closure during swallowing, blowing and the production of pressure consonants. One common result was that “swallowing VP closure” did not translate into “phonation VP closure”. However, once closure was attained in blowing, it did not take long for VP closure during phonation to follow (Miyazaki, Matsuya, Yamashita, and Nishio, 1974; Nishio, Yamaoka, Matsuya, and Miyazaki,1976). Subjects able to achieve VP closure during blowing seem to be able to maintain VP closure during phonation, and they are the best candidates for success using endoscopic biofeedback

Nasometer:

The Nasometer (Kay-Pentax, Inc.) is a device which measures acoustic energy during speech. A ratio of acoustic data is acquired by two microphones, situated on a metal plate on a headset that separates the nose from the mouth (see figure 4). This ratio is termed nasalance and is defined as the acoustic correlate of perceived nasality. Nasalance is displayed as a percentage on the software program, with higher percentages representing increased nasalance.

Figure 4: Child wearing Nasometer headset (Kay-Pentax, 2006).

[Image: hypernasality4.jpg]

Taken from:
http://www.kayelemetrics.com/

Although the Nasometer does not give the patient a view of the VP mechanism, it does give auditory feedback coupled with the instrument’s real-time visual feedback during therapy or assessment tasks. Other advantages include; it is noninvasive, easy to clean and has an established normative database. The data collected is often useful in documenting patient performance before, during and after therapy, surgery, or prosthetic fitting.

The Nasometer can be used in therapy to provide visual, real-time nasalance percentages as the patient reads, talks or repeats prescribed material. One useful protocol is to set a threshold 10% higher than the patient’s average nasalance score, while the patient attempts to stay below the set threshold. The threshold is lowered in 5-10% increments as the individual successfully produces the treatment stimuli at or below the target.

The use of the Nasometer to reduce nasality was researched by Fletcher (1972) and was generally found to be successful with most VPI patients. Results indicated that patients with various VPI conditions were able to decrease nasalance by a significant margin and perceptual judgment of nasality also decreased (Fletcher, 1978; Starr, 1993). Those with mild, inconsistent hypernasality are the best candidates for nasality reduction using the Nasometer.

Palatal Efficiency Rating Computed Instantaneously (PERCI):

Nasal airflow can also be detected and monitored through various instruments that measure aerodynamic data such as subglottal pressure, translaryngeal and nasal airflow. The PERCI system was developed by Warren and Dubois in 1964, and has been modified and packaged to be commercially available. The PERCI system is typically used for assessment purposes but can be used during treatment by providing visual feedback of nasal and oral flow during speech production. PERCI is a noninvasive method for assessing and monitoring VP function.

Direct Articulation-Phonological Therapy for Hypernasal Speech:

In patients with VPI, there are specific articulatory or phonological treatment strategies that minimize the perception of hypernasality or audible nasal emission. One common goal of these strategies is to manipulate the oral cavity in an attempt to alter the resonance. This is an important point because behavioral therapy cannot alter structural deficits, such as lack of VP closure. Although the effectiveness of the following activities has not been systematically tested, many researchers have found anecdotal success with their use (Hardin, 1991; Kummer and Lee, 1996). Most of these activities focus on encouraging oral resonance and articulation through modification of the oral cavity.

Numerous studies have recommended increasing oral resonance by further opening the oral cavity, as in increasing the mouth opening (Boone and McFarlane, 1994; Coston, 1986; McDonald and Koepp-Baker, 1951; Van Denmark and Hardin, 1990). Increasing the oral opening decreases oral resistance to airflow, thereby decreasing nasal airflow. By decreasing the nasal airflow hypernasal speech will diminish naturally. Therapy simply involves providing a mirror for visual feedback and asking the patient to observe increased mouth opening during the production of liquids, glides and vowels. Another strategy is exaggerated articulatory movements leading to larger mouth opening. The best candidates for this approach are patients with a small VP gap and with a lack of oral movement during speech.

Another approach to reduce the perception of nasal emission in patients with VPI is to use a light articulatory contact (Coston, 1986, Van Denmark and Hardin, 1990). The idea is to contact the articulatory structures with less pressure, reducing the amount of time they are held in a single position. The idea is that by decreasing the duration of the articulatory posture you decrease the time nasal emission is heard. Most authors believe light articulatory contact presents a detrimental result because more forceful contacts are associated with improved VP muscular movement and closure. Light contact would oppose such muscular movement and increase VP opening (Golding-Kushner, 1995; Hoch, Golding-Kushner, Siegel-Sadewitz, and Shprintzen, 1986). The suggested population for this technique might include patients with mild nasalization and no prospects for surgical intervention.

Lang’s approach to systematically reduce and eliminate hypernasal speech (Lang, 1974) was based on the varying degrees of nasality in each phoneme. Lang suggested that vowels from least to most nasal are /? /, /a/, /? /, /æ /, /u/, and /i/. Consonants from least to most are glides, glottal fricatives, plosives, fricatives, affricates, and /z/. 100% nasal-free speech was required at each step before moving to the next step. A table to describe each therapy step is provided in Table 1. Lang reported positive results in a study of 11 children (ages 9-13 years) enrolled in an 8 week program to reduce hypernasal speech.

Table 1: Lang’s Hypernasality Reduction program (1974).
  1. Correct production of the following vowels. Practice in order listed: /? /, /e/, /? /, /æ /, /u/, and /i/.
  2. Correct production of VCV combinations utilizing each vowel (from least to most nasal) in combination with each consonant (least to most nasal).
  3. Correct production of CVC combination utilizing each vowel (from least to most nasal) in combination with each consonant (least to most nasal).
  4. Correct production of the varied vowel and consonant combinations (from least to most nasal) in the initial and final position of monosyllabic words.
  5. Correct production of the varied vowel and consonant combinations (from least to most nasal) in bisyllabic words.
  6. Correct production of the 10 short phrases and sentences loaded with the following phonemes in combination with all the vowels: /r/, /w/, /h/, /l/, /j/.
  7. Correct production of the 10 short phrases and sentences loaded with the following phonemes in combination with all the vowels: /t/, /p/, /k/, /g/, /b/ and /d/.
  8. Correct production of the 10 short phrases and sentences loaded with the following phonemes in combination with all the vowels: /v/, /f/, /?/, /?/, /s/.
  9. Correct production of the 10 short phrases and sentences loaded with the following phonemes in combination with all the vowels: /d?/, /?/, / t?/, /?/, /z/.
  10. Correct production of all consonants and vowel combinations in long sentences
  11. Correct production of all consonant and vowel combinations while reading a short paragraph. (if the patient does not read, poems or nursery rhymes can be substituted).
  12. Correct production of all consonants and vowel combinations in structured conversational speech.
  13. Correct production of all consonants and vowel combinations in spontaneous conversational task.


**From Sommers, R.K.: Articulation Disorders. Englewood Cliffs (NJ): Prentice-Hall, 1983.

Other Considerations in Treating Hypernasality in Down Syndrome Patients

Other areas that may need to be addressed with DS patients may include treating phonological deviations, rate, fluency, voice quality, resonance, and motor planning for speech. Possible phonological deviations may include final consonant deletion, which can be addressed by instructing the difference between word pairs such as bow and boat, and bat and back. The pacing board concept (Kumin, 2006) is also applicable to intervention for homological deviations, primarily for initial consonant deletion, final consonant deletion or weak syllable deletion. For example, the pacing board enables the child to see and feel the presence of the final consonant, in addition to hearing the final consonant in the word. The multi-sensory nature of homological intervention using the pacing board leads to increased awareness, greater success in producing the structure, and more consistent generalization of the skill.

Programming the articulators to produce a string of phonemes together to produce words and sentences is known as motor planning (Love, 2000). Deficits in motor planning are often referred to as developmental apraxia of speech. DS patients exhibit deficits in motor planning and it is important to identify these symptoms early because developmental apraxia of speech requires a different protocol compared with articulation difficulties. For developmental apraxia of speech, therapy trains children to produce gradually lengthening combinations of overlapping speech movements, paying particular attention to the consonant-vowel combination as well as the length of the word or phrase.

Dysfluencies are often observed in children with DS when they begin to produce sentences and phrases and their rate of speech increases. Published fluency programs such as Easy Does it for Fluency and the Fluency Criterion Program have proven successful in treating this problem (Heinze and Johnson, 1985).

Conclusion:

The assessment process will identify the specific needs of the patient and therapy will than target the specific issues, whether rate, fluency, voice, resonance, articulation or intelligibility. It is important to note that the therapy with children with DS cannot simply address one specific problem such as hypernasality, it must systematically address all speech deficits. A well thought out and planned treatment regimen should improve structural and functional aspects of speech production.

References:

Boone, D.R. and McFarlane, S.C. The voice and voice therapy. Englewood Cliffs (NJ): Prentice-Hall, 1994.

Brown, P.M., Lewis, G.T., Parker, A.J. and Maw, A.R. (1989). The skull base and nasopharynx in Down’s syndrome in relation to hearing impairment, Clinical Otolaryngology, 14, 241-246.

Coston, G.N. (1986). Therapeutic management of speech disorders associated with velopharyngeal inadequacy. Journal of Childhood Communication Disorders, 10, 75-85.

Fletcher, S.G. (1972). Contingencies for bioelectric modification of nasality. Journal of Speech and Hearing Disorders, 37, 329-346.
Fletcher, S.G., Diagnosing speech disorders from cleft palate. New York: Grune and Stratton, 1978.

Furlow, L.(1986). Cleft palate repair by double opposing Z-plasty, Plastic Reconstructive Surgery, 78, 724-736.

Golding-Kushner, K. Treatment of articulation and resonance disorders associated with cleft palate. In Shprintzen, R.J. and Bardach, J. (eds). Cleft palate speech management: a multidisciplinary approach. St. Louis: Mosby, 1995.

Hardin, M.A. (1991). Cleft palate: intervention. Clinics in Communication Disorders, 1, 12-18.

Heinze, B. A. & Johnson, K. L. (1985) Easy does it 1: Fluency activities for young children. Moline, IL: LinguiSystems.

Heinze, B. A. & Johnson, K. L. (1987) Easy does it 2: Fluency activities for school-aged stutterers. Moline, IL: LiguiSystems.

Hoch, L., Golding-Kushner, K., Siegel-Sadewitz, V.L., and Shprintzen, R.J. (1986). Speech Therapy. Seminars in Speech and Language, 7, 313-326.

Kanamori, G.,Witter, M., Brown, J., and Williams-Smith, L. (2000). Otolaryngologic manifestations of Down syndrome, Otolaryngology Clinics of North America, 33, 1285-1292.

Kantner, C.E. (1947). The rationale for blowing exercises for patients with repaired cleft palates. Journal of Speech Disorders, 12, 281-286.

Kavanagh, K.T., Kahane, J.C. and Kordan, B. (1986). Risks and benefits of adenotonsillectomy for children with Down Syndrome, American Journal of mental deficiency, 91:1, 22-29.

Kuehn, D.P. (1991). New therapy for treating hypernasal speech using continuous positive airway pressure (CPAP). Plastic & Reconstructive Surgery, 88, 959-966.

Kuehn, D.P., Imrey, P.B., Tomes, L., Jones, D.L., O’Gara, M.M., Seaver, E.J., Smith. B.E., Van Demark, D.R., & Wachtel, J.M. (2002). Efficacy of continuous positive airway pressure for treatment of hypernasality. Cleft Palate-Craniofacial Journal, 39 (3), 267-276.

Kumin, L (2006). Comprehensive speech and language treatment for infants, toddlers, and children with Down syndrome. http://www.ds-health.com/speech.htm

Kuehn, D.P., Moon, J.B., & Folkins, J.W. (1993). Levator veli palatini muscle activity in relation to intranasal air pressure variation. Cleft Palate- Craniofacial Journal, 30, 361-368.

Kuehn, D.P. & Moon, J.B. (1994). Levator veli palatini muscle activity in relation to intraoral air pressure variation. Journal of Speech and Hearing Research, 37, 1260-1270.

Kuehn, D.K. & Moon, J.B. (1995). Levator veli palatini muscle activity in relation to intraoral air pressure variation in cleft palate subjects. Cleft Palate- Craniofacial Journal, 32, 376-381.

Kummer A. and Lee, L. (1996). Evaluation and treatment of resonance disorders. Language Speech and Hearing Services in Schools, 27, 271-281.

Lang, M.:Program for the elimination of hypernasality. Unpublished manuscript, Kent State University, 1974.

Love, R.J. (2000).The neurology of childhood motor speech disability. In R.J. Love (Ed.), Childhood Motor Speech Disability (2nd edition). Allyn & Bacon.

Lubit, E.C. and Larsen, R.E. (1969). The Lubit palatal exerciser: a preliminary report. Cleft Palate Journal 6:120-133.

McDonald, E.T. Koepp-Baker, H. (1951). Cleft palate speech: an integration of research and clinical observation. Journal of Speech and Hearing Disorders, 16: 9-20.

Miyazaki, T., Matsuya, T., Yamashita, Y., and Nishio, J.: A nasopharyngeal fiberscope (film). Boston: American Cleft Palate Association, 1974.

Nishio, J., Yamaoka, M., Matsuya, T., and Miyazaki, T.: How to exercise the velopharyngeal movement by the velopharyngeal fiberscope. Japanese Journal of Oral Surgery, 20, 450, 1974. Abstracted in Cleft Palate Journal, 13:310, 1976.

Parton, M.J. and Jones, A.S. (1998). Clinical Otolaryngology, 23, 18-19.

Peterson-Falzone, S.J., Hardin-Jones, M.A.and Karnell, M.P.: Cleft Palate Speech (3rd edition). St. Louis: Mosby, 2001.

Powers, G.L. and Starr, C.D. (1974). The effects of muscle exercises on velopharyngeal gap and nasality. Cleft Palate Journal 11: 28-35.

Price, D.L., Orvida, L.J., Weaver, A.L., and Farmer, S.A. (2004). Efficacy of adenoidectomy in the treatment of nasal and middle ear symptoms in children with Down syndrome, International Journal of Pediatric Otolaryngology, 68, 7-13.

Ruscello, D. M.. Considerations for behavioral treatment of velopharyngeal closure for speech. In KR Bzoch (ed): Communicative disorders related to cleft lip and palate (5th edition). Austin, Texas: Pro-Ed, 763-796, 2004.

Shprintzen, R.J., McCall, G.M., and Skolnick, L.: A new therapeutic technique for the treatment of velopharyngeal incompetence. Journal of Speech and Hearing Disorders 40:69-83, 1975.

Siegel-Sadewitz, V.L., and Shprintzen, R.J. (1982). Nasopharyngoscopy of the normal velopharyngeal sphincter: and experiment in biofeedback. Cleft Palate Journal, 19, 194-200.

Starr, C.D. Behavioral approaches to treating velopharyngeal closure and nasality. In Moller, K.T., and Starr, C.D. (eds.). Cleft palate: interdisciplinary issues and treatment. Austin: Pro-Ed, 1993.

Tomes, L., Kuehn, D.P., & Peterson-Falzone, S. (in press). Behavioral therapy for speakers with velopharyngeal impairment. In K.R. Bzoch (Ed.), Communicative Disorders Related to Cleft Lip and Palate, 4th Ed., Austin, TX: Pro-Ed.

Van Denmark, D. R. and Hardin, M. Speech therapy for the child with cleft lip and palate. In Bardach, J., and Morris, H.L. (eds.). Multidisciplinary management of cleft lip and palate. Philadelphia: WB Saunders, 1990.

Warren, D.W. and Dubois, A.B. (1964). A pressure-flow technique for measuring velopharyngeal orifice area during continuous speech. Cleft Palate Journal, 1, 52-57.


Featured Author: Dr. Bridget A. Russell

Dr. Bridget A. Russell received her B.A., M.A., and Ph.D. from the State University of New York at Buffalo. She is an Associate Professor at the State University of New York College at Fredonia. Her research interests are professional voice, and voice/respiratory disorders affecting speech production. She has published works in JSHLR and Voice and Speech Review and has served as editorial consultant for JSHLR and Delmar Publishing Group. She is director of the Speech Production Laboratory at SUNY College at Fredonia and is researching the efficacy of voice therapy with patients at the Voice Center of Western New York.

Dr. Bridget Russell received her PhD from SUNY Buffalo. She is an Associate Professor at SUNY Fredonia and has published in JSHLR and Voice/Speech Review and served as an editorial consultant for JSHLR. She is currently researching the efficacy of voice therapy at the Voice Center of Western New York.

To learn more about Dr. Russell visit the website of Western New York Voice Consortium and on the Fredonia faculty pages at http://www.fredonia.edu/department/commd...aculty.asp


This article originally appeared as: Russell, B. (2006). Treatment of Hypernasality in Children with Down Syndrome Post Adenoidectomy. SpeechPathology.com Text-Based CEU Course Journal Articles, http://speechpathology.com/articles/index.asp and is reprinted here with permission of the author Dr. Bridget Russell.


Tags: Down Syndrome SLP Voice Disorders Resonance Disorders Cleft Palate Article Newsletter 16 September 2011