700
N.K.K. Prasad et al.
a
c
b
Fig. 31.3 ( a – c ) Lateral cephalograms at different intervals as follows. ( a ) Before commencement of distraction,
( b ) immediate post distraction period, and ( c ) three year following completion of distraction
patient treated with rigid external distraction are
illustrated in Fig. 31.3a–c .
The radiographs were traced and evaluated
by a single experienced investigator. An X – Y
coordinate system was made by drawing a line
at an angle of 7° below SN plane for x-axis.
Y-axis was drawn perpendicular to this and
intersecting it at the sella (Fig. 31.4 ). The
changes in position of ANS, point A, mandibu-
lar incisor, and pogonion (Pog) were measured
in the x - and y -axis in cephalograms T1–T4 seri-
ally. A mean value was arrived at from all
701
31 Preventing Relapse Following Distraction Osteogenesis for the Cleft Midface in Adults
S
Vertical
reference line
Horizontal
reference line
ANS
Point A
Mand incisor
Pogonion
7°
N
Fig. 31.4 X – Y coordinate
system was used. The
changes in position of
ANS, Point A, Mandibular
incisor, Pogonion (Pog)
were measured on the
X and Y axis
individual measurements, and paired sample t
test was used to compare the difference between
T1–T2, T2–T3, and T3–T4.
31.3
Results
Immediately following distraction (T1–T2),
the maxilla moved forward at the ANS by
12.4 mm and also downward by 1.56 mm. In
the fi rst year following distraction (T2–T3),
the maxilla moved backward by 1.5 mm, sug-
gesting signi fi cant relapse. Between 1 and 3 (or
more) years (T3–T4), maxilla remained stable
in horizontal and vertical position. Similar
changes with insigni fi cant differences in val-
ues were seen in the positions of point A over
the same time frame. The position of the tip of
mandibular incisor showed signi fi cant down-
ward movement of the mandibular incisors
immediately after distraction; however, after
1 year following distraction, it moved forward.
Similarly, pogonion (Pog) changed as follows:
Between T1 and T2, there is backward and
downward movement, and between T3 and T4,
it remained stable in its new position.
Comparison of maxillary positions between
bilateral and unilateral cleft lip and palate that
was done using the unpaired t tests showed no
signi fi cant difference between these groups
(Fig. 31.5 ). Comparison between patients who
wore a retention device for a year as suggested
by the protocol and those who did not wear it
or partially complied with the instructions
showed that the former group had better reten-
tion (Fig. 31.6 ).
702
N.K.K. Prasad et al.
67.8
80
60
40
20
Mean change in ANS (mm)
0
ANS T2 x ANS T3 x ANS T2 y ANS T3 y
ANS T2 x ANS T3 x ANS T2 y ANS T3 y
68.6
Reverse pull headgear
n = 9
n = 9
Without reverse pull headgear
48.7
46.9
77.9
74.1
44.7
44.6
Fig. 31.6 Comparison between patients who wore a retention device and who did not wear retention device
80
61.2
42.8
74.4
45.8
58.7
49.8
69.7
ANS T1 x
ANS T1 y
ANS T2 x
ANS T2 y
48.3
60
40
Mean change in ANS (mm)
20
0
Unilateral
Type of cleft
n = 12 Unilateral
n = 6 Bilateral
Bilateral
Fig. 31.5 Comparison of
maxillary positions between
bilateral and unilateral cleft
lip and palate
703
31 Preventing Relapse Following Distraction Osteogenesis for the Cleft Midface in Adults
31.4
Discussion
From the above mentioned results, one may rea-
sonably conclude that maximum relapse occurred
during the fi rst year following distraction. The
various factors in fl uencing this may be catego-
rized into preexisting factors like number and
quality of previous operations, preoperative fac-
tors, and postoperative management. While the
fi rst variable cannot be controlled since it is pre-
existent, the second and third may be in fl uenced
to achieve a more favorable long-term result.
Care needs to be taken to ensure that the osteot-
omy is completed with no remaining bony attach-
ments which may hamper distraction and cause
early relapse. At the same time, the segments
should not be down fractured or extensively
mobilized, since this may lead to fi brous union or
nonunion with a subsequent higher rate of relapse
(He et al. 2010 ) . The distraction needs to be car-
ried out at the rate of 0.5 mm twice a day as has
been established in various studies (Ilizarov et al.
1969 ; Polley and Figueroa 1997 ; Cho and Kyung
2006 ; Bell and Guerrero 2007 ) with careful con-
trol of the vectors. The orthodontic management
needs to be closely controlled in the fi rst year
since maximum relapse happens during this
period (Cheung and Chua 2006 ) . In our series,
we have retained the external frame distraction
device for a period of 3 months followed by
removable retention devices for a further period
of one year. These ranged from reverse pull head-
gear or directional elastics, and the study has
shown lesser relapse in patients who complied
with this.
Conclusion
Distraction osteogenesis is a powerful tool in
the armamentarium of the cleft team for cor-
rection of midface recessiveness. However, it
should be used judiciously and needs careful
patient selection, meticulous planning, and
execution. Relapse is inevitable, but its
effects on the fi nal result can be minimized.
This needs incorporating a degree of overcor-
rection during planning, precautions and
meticulous technique during osteotomy, and
careful monitoring during the distraction and
consolidation phases with prolonged use of
retention devices. The potential complica-
tions need to be anticipated, identi fi ed early,
and
corrected
during
the
course
of
treatment.
References
Aronson J, Shen XC, Skinner RA, Hogue WR, Badger
TM, Lumpkin CK Jr (1997) Rat model of distraction
osteogenesis. J Orthop Res 5:221–1
Bell WH, Guerrero CA (2007) Distraction osteogenesis
of the facial skeleton. BC Decker Inc, Hamilton,
ON
Cheung LK, Chua HDP (2006) A meta-analysis of cleft
maxillary osteotomy and distraction osteogenesis. Int
J Oral Maxillofac Surg 35:14–24
Cheung LK, Chua HD, Hägg MB (2006) Cleft maxillary
distraction versus orthognathic surgery: clinical mor-
bidities and surgical relapse. Plast Reconstr Surg
118:996–1008
Cho BC, Kyung HM (2006) Distraction osteogenesis of
the hypoplastic midface using a rigid external distrac-
tion system: the results of a one- to six-year follow-up.
Plast Reconstr Surg 118(5):1201–1212
Cohen SR, Burstein FD, Stewart MB et al (1997)
Maxillary-midface distraction in children with cleft lip
and palate: a preliminary report. Plast Reconstr Surg
99:1421–1428
Figueroa AA, Polley JW, Ko EW (1999) Maxillary dis-
traction for the management of cleft maxillary hyp-
oplasia with a rigid external distraction system. Semin
Orthod 5:46–51
Gulsen A, Ozmen S, Tuncer S et al (2007) Maxillary
advancement
with
internal
distraction
device
in cleft palate patients. J Craniofac Surg 18:
177–185
He D, Genecov DG, Barcelo R (2010) Nonunion of the
external maxillary distraction in cleft lip and palate:
analysis of possible reasons. J Oral Maxillofac Surg
68:2402–11
Houston WJB, James JE, Kavvadia S (1989) Le fort 1
maxillary osteotomies in cleft palate cases-surgical
changes and stability. J Craniomaxillofac Surg 17(1):
9–15
Hussain SA (2009) External frame distraction osteogene-
sis of the midface in the cleft patient. Indian J Plast
Surg 42(suppl 1):s168–s173
Ilizarov GA, Lediaev VI, Shitin VP (1969) The course
of compact bone reparative regeneration in distrac-
tion osteosynthesis under different conditions of bone
fragment fi xation (experimental study). Eksp Khir
Anesteziol 14(6):3–12
Karp NS, McCarthy JG, Schreiber JS et al (1992)
Membranous bone lengthening: a serial histological
study. Ann Plast Surg 29:2–7
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Kozák J, Hubácek M, Müllerová Z (2005) Midface dis-
traction in patients with cleft palate. Acta Chir Plast
47(3):71–6
Krimmel M, Cornelius CP, Roser M (2001) External dis-
traction of the maxilla in patients with craniofacial
dysplasia. J Craniofac Surg 12(5):458–463
Lilja J, Mars M, Elander A et al (2006) Analysis of dental
arch relationships in Swedish unilateral cleft lip and
palate subjects: 20-year longitudinal consecutive
series treated with delayed hard palate closure. Cleft
Palate Craniofac J 43:606–11
Mars M, Asher-McDade C, Brattström V et al (1992)
A six-center international study of treatment outcome in
patients with clefts of the lip and palate: part 3 dental
arch relationships. Cleft Palate Craniofac J 29:405–8
Nout E, Wolvius EB, van Adrichem LN et al (2006)
Complications in maxillary distraction using the RED
II device: a retrospective analysis of 21 patients. Int J
Oral Maxillof Surg 35:897–902
Panula K, Lorius BBJ, Pospisil OA (1993) The need for
orthognathic surgery in patients born with complete
cleft palate or complete unilateral cleft lip and palate.
Oral Surg Oral Diagn 4:23–8
Polley JW, Figueroa AA (1997) Management of severe
maxillary de fi ciency in children and adolescence through
distraction osteogenesis with an external, adjustable,
rigid distraction device. J Craniofac Surg 8:181–185
Rachmiel A, Potparic Z, Jackson IT (1993) Midface advance-
ment by gradual distraction. Br J Plast Surg 46:201–207
Rachmiel A, Laufer D, Jackson IT et al (1998) Midface
membranous bone lengthening: one year histological
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Ross RB (1987) Treatment variables affecting facial
growth in complete unilateral cleft lip and palate. Cleft
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Wang XX, Wang X, Yi B et al (2005) Internal midface
distraction in correction of severe maxillary hypopla-
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Williams AC, Sandy JR (2003) Risk factors for poor den-
tal arch relationships in young children born with uni-
lateral cleft lip and palate. Plast Reconstr Surg
111:586–593
Williams AC, Bearn D, Mildinhall S et al (2001) Cleft
lip and palate care in the United Kingdom (UK) - the
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part 2-dentofacial outcomes, psychosocial status and
patient satisfaction. Cleft Palate Craniofac J 38:
24–29
Yamauchi K, Mitsugi M, Takahashi T (2006) Maxillary
distraction osteogenesis using Le Fort 1 osteotomy
without intra operative down-fracture. Int J Oral
Maxillofac Surg 35:493–498
Part XIII
Speech
707
S. Berkowitz (ed.), Cleft Lip and Palate,
DOI 10.1007/978-3-642-30770-6_32, © Springer-Verlag Berlin Heidelberg 2013
32
32.1
Articulation Tests
An articulation tests is a paper and pencil test
used by speech-language pathologists to sys-
tematically evaluate the formation and produc-
tion of the sounds of speech in different contexts
in words and sentences. It is used to record the
sounds that are produced correctly as well as the
errors in speech including omissions, distor-
tions, and substitutions of normal or compensa-
tory sound errors. Systematic evaluation of the
articulation of speech is helpful in ensuring
complete and consistent analysis of the prob-
lems so that effective and ef fi cient treatment can
be planned.
Many different types of tests are available,
but the most useful tests for patients with cleft
palate are those that assess both place and
manner of production of speech sounds in the
vocal tract. Individuals with cleft palate often
produce sounds in a more anterior or more
posterior (this is a more frequent error) loca-
tion in the vocal tract than normal speakers.
Careful notation of the types of errors and
the probable causative factors sets the stage
for determining the method and duration of
treatment. These tests also provide a baseline
measure or description of speech articulation
against which progress, improvement, devel-
opmental changes, and treatment outcome can
be evaluated. The examiner scores articulation
of individual sounds or sound units by listen-
ing to and observing the production of these
sounds. In some cases, traditional articula-
tion testing alone cannot completely describe
the manner or place of the error sounds, and
visualization techniques such as multiview
video fl uoroscopy and nasopharyngoscopy are
useful in completing the description and diag-
nosis of the error(s).
32.2
Rating Scales of Speech
Intelligibility and Acceptability
and Acceptability
Rating scales are often used by speech-language
pathologists and other members of the cleft palate
team to score the overall severity of an individual’s
communication impairment in several categories.
Ratings of intelligibility describe how well an indi-
vidual’s speech can be understood by others,
whereas ratings of acceptability describe the
pleasingness of the sound and appearance of
Diagnostic Procedures
and Instruments Used
in the Assessment and Treatment
of Speech
Samuel Berkowitz
S. Berkowitz , DDS, M.S., FICD
Adjunct Professor, Department of Orthodontics ,
College of Dentistry, University of Illinois ,
Chicago , IL , USA
Clinical Professor of Surgery and Pediatrics (Ret),
Director of Research (Ret),
South Florida Cleft Palate Clinic,
University of Miami School of Medicine ,
Miami , FL , USA
Consultant (Ret), Craniofacial Anomalies Program ,
Miami Children’s Hospital , Miami , FL , USA
e-mail: sberk3140@aol.com
708
S. Berkowitz
speech. These scales usually consist of 5 or 7
points, with 1 being normal and 5 or 7 indicating
unintelligible or unacceptable speech. Effective
use of these scales is related to rater reliability, and
raters must establish and reestablish their reliabil-
ity on a regular basis when using these scales for
clinical or research purposes. These scales are use-
ful as overall measures of severity and treatment
outcome; however, they are descriptive in nature
and are not useful in isolating causative factors.
32.3
Cephalometrics
Orthodontists and specialists in facial growth have
developed the technique of cephalometrics. This
is a still sagittal x-ray taken with the patient stabi-
lized in a headholder which positions the head
relative to a cranial landmark called the Frankfort
horizontal plane. The head is secured by earposts,
and the subject’s midline is positioned at a con-
stant distance from the x-ray source. The standard
position and photon source relationship to the
subject provide a means of comparing the mea-
surements from the resulting x-rays. It should be
understood that x-ray fi lms provide an image that
is a summation of the tissue through which the
x-ray beam has passed. If a person had soft palatal
closure on one side but not on the other, the mid-
sagittal view would show velopharyngeal closure
because the beam would pass through the tissue
on the closed side, and the resulting image would
not yield information about the opening. The
velopharyngeal valve is three dimensional, and its
attributes cannot all be captured in the midsagittal
view alone. This is a shortcoming of cephalomet-
rics when used for speech purposes. Another
problem with the technique is that it is capable of
fi lming only a single point in speech production.
Connected discourse cannot be studied by this
means, nor is it possible to specify precisely what
will be fi lmed in the course of production of a
single sound.
Of particular interest to speech physiologists
are the cephalometric studies that have de fi ned
structures and growth patterns of pharynx, velum,
and lymphatic tissue (King 1952 ; Rosenberger 1934 ;
Subtelny and Baker 1956 ; Subtelny 1957 ) .
Compositely, these cephalometric studies have
provided objective data pertaining to normal
skeletal and soft tissue structures and their pos-
tural relationships.
This information also has been stated within
the complex and important reference of a speci fi ed
stage of growth and development. The diagnostic
value of such normative data becomes obvious
in considering the oral examinations speech-
language pathologists routinely perform to evaluate
speech structures. In such examinations, skeletal,
dental, and soft tissue components of the speech
apparatus are evaluated.
In addition to the normative data provided,
cephalometric analyses have also been under-
taken to evaluate skeletal characteristics and
growth disturbances in pathologic conditions.
Especially valuable for speech-language patholo-
gists are the studies which have de fi ned the
intraoral, intranasal, and pharyngeal architecture
of individuals with clefts of the lip and palate
(Brader 1957 ; Prusansky 1953 ; Ricketts 1954 ;
Slaughter and Prusansky 1954 ; Subtelny 1955 ) .
Speech-language pathologists have learned a
great deal about morphologic variation in cleft
palate patients, whose total rehabilitation, of
necessity, includes multiprofessional concerns,
from these cephalometric studies.
The fact that cephalometric head plates can be
reliably compared has made them attractive to
speech-language pathologist’s intent on de fi ning
physiological differences that result in defective
speech production.
There is probably universal agreement that a
midsagittal cephalometric view, although useful,
yields incomplete information about soft palate
and lateral pharyngeal valving. It provides little
insight into the location, con fi guration, or move-
ment of structures off the midsagittal plane. In
particular, it offers no information about move-
ment of the lateral pharyngeal walls.
32.4
Cine- and Video fl uoroscopy
Cine fl uoroscopy (x-rays recorded on motion
picture fi lm) and video fl uoroscopy (x-rays
recorded on videotape) with simultaneous
709
32 Diagnostic Procedures and Instruments Used in the Assessment and Treatment of Speech
voice recordings are useful procedures in the
evaluation of individuals with cleft palate. The
key to development of motion x-rays was the
advent of image intensi fi cation, which permit-
ted greater contrast with reduced x-ray dosages.
Video recording involves lower radiation dosage
than does cine fl uoroscopy. Video fl uoroscopic
recording can be done with less radiation than is
required for a single still x-ray of the head.
To extract more information from video-
fl uoroscopy, Skolnick ( 1970 ) introduced mul-
tiview video fl uoroscopy, a technique that adds
a base view to the traditional lateral and frontal
projections.
32.5
Multiview Video fl uoroscopy
This fl uoroscopic x-ray technique allows visual-
ization of the velopharyngeal port, or valve, dur-
ing speech in several different planes (Skolnick
1970 ; Skolnick et al. 1975 ) . This is important
because the mechanism of velopharyngeal clo-
sure is three dimensional and may involve move-
ment not only of the soft palate but also of the
lateral
and
posterior
pharyngeal
walls.
Descriptions of the technique include the lateral,
base, Towne’s, frontal, Waters’, and oblique
views. Not all views are required for a complete
examination. The view selected depends on the
information needed, the anatomy of the skull,
and the anatomy and function of the vocal tract.
In most cases, a complete examination consists
of three views, including the lateral view, the
base or Towne’s view, and the frontal, Waters’,
or oblique view. The examinations are usually
recorded and maintained on videotape with
audio recording for purposes of interpretation
and comparison. The procedure is conducted
and interpreted jointly by a radiologist and
speech-language pathologist. This contributes to
a valid study which includes an adequate speech
sample, appropriate visualization of the velopha-
ryngeal valve, and interpretation of the fi ndings
which are consistent with the patient’s speech.
Radiation dosage is kept to a minimum by using
video fl uoroscopy, as opposed to cine fl uoroscopy;
coning the x-ray beam to the smallest area;
ensuring that the equipment is emitting the min-
imum radiation necessary to produce the image;
and using lead sheets to shield the patient except
for the area of interests.
32.5.1 Technique
To enhance the velopharyngeal area, high-density
barium is instilled into each nostril using a syringe
with a plastic tip. This results in barium coverage
of the soft palate, lateral and posterior pharyngeal
walls, and posterior aspect of the tongue. A stan-
dard speech sample is used during each view. The
lateral view is obtained fi rst, with the patient sit-
ting upright. In this view, judgments can be made
about the length and thickness of the soft palate,
the depth of the pharynx, and the size and loca-
tion of adenoid and tonsillar tissue. The anterior-
posterior excursion of the soft palate and, in some
cases, the anterior movement of the pharynx are
observed, and judgments of velopharyngeal con-
tact during speech are made. This view alone is
not suf fi cient to determine velopharyngeal clo-
sure because it does not con fi rm velopharyngeal
contact along its width.
The lateral view permits excellent visualiza-
tion of the function of the tongue during speech
and is helpful in the differential diagnosis of
compensatory articulation errors. In some cases,
the tongue is observed to elevate the soft palate in
an attempt to effect velopharyngeal closure, par-
ticularly for the velar stop sounds /k/ and /g/.
Abnormal posterior movements of the epiglottis
and elevation of the larynx during speech are also
apparent in this view.
The frontal view is usually performed next to
determine the degree and location of mesial
movements of the lateral pharyngeal walls.
Lateral pharyngeal wall movements may occur
at a speci fi c location in the vocal tract or along
an extensive area. This information is thought to
be useful in planning the approximate width of a
pharyngeal fl ap or designing a prosthetic speech
appliance
in
cases
of
velopharyngeal
insuf fi ciency. To obtain this view, the patient is
seated upright facing the image intensi fi er with
his or her head positioned in the Frankfurt
710
S. Berkowitz
horizontal plane. Complete coating of the
nasopharynx with barium is essential to gather
information for adequate interpretation. The
standard speech sample is used.
In cases where the overlying bony structures
obscure the lateral pharyngeal walls and their
movements, the Waters’ view is a helpful alterna-
tive. In this view, the head is tilted upward
approximately 450° from the Frankfurt horizon-
tal plane so that the bony structures do not impede
the view of the lateral walls. When asymmetric
movement of the lateral pharyngeal walls is
observed, Shprintzen et al. ( 1977 ) recommend
rotation of the head to the right and left along the
x-axis to more clearly identify the extent of
movement.
The third view to be performed is either the
base or Towne’s view. These views outline the
shape of the velopharyngeal valve; the pattern,
symmetry, and consistency of velopharyngeal
valving movements; and the size and location of
velopharyngeal gaps during speech. The Towne’s
view is the more useful of the two views when the
soft palate approximates adenoid tissue during
valving creating an oblique axis of velopharyn-
geal closure or when patients have large tonsils or
posterior compensatory tongue movements.
These situations interfere with adequate visual-
ization and interpretation of velopharyngeal
movements in the base view.
To obtain the base view, the patient lies prone
on the x-ray table in a sphinxlike position with
the head hyperextended. To obtain the Towne’s
view with over-table tube fl uoroscopy equipment,
the patient is seated upright with his or her head
in the horizontal plane. The camera is then rotated
in relation to the face until the velopharyngeal
valve is visualized. This view is similar to that
used in nasopharyngoscopy. The standard speech
sample is used for both the base and Towne’s
views.
Multiview video fl uoroscopy may be reliably
performed in patients as young as 3 or 4 years
old. Because this test involves radiation, it should
be used judiciously. It is most reliable when the
child is mature enough to cooperate for the exam-
ination and has suf fi cient speech development to
allow visualization of the valve throughout all
classes of speech sounds. The test should be used
when the clinical speech assessment suggests
velopharyngeal inadequacy, particularly when
surgical or prosthetic management is being
considered.
32.6
Ultrasound
Ultrasound is a device that has been employed in
the evaluation of velopharyngeal function, par-
ticularly movements of the lateral pharyngeal
walls. It is not suitable for displaying motion of
the velum because of problems in transmitting
ultrasound through bone overlying the palate
(Hawkins and Swisher 1978 ) .
32.7
Video Nasopharyngoscopy
This technique involves inserting a fl exible fi ber-
optic tube into the nose to obtain a direct supe-
rior view of the velopharyngeal valve and vocal
tract during speech. In the hands of an experi-
enced and patient examiner, the test provides
information about the anatomy and function of
the velopharyngeal valve during speech; the rela-
tive size, location, and consistency of velopha-
ryngeal gaps; the function of the posterior aspect
of the tongue during speech, which is helpful in
the differential diagnosis of compensatory artic-
ulation errors; and the anatomy and function of
the laryngeal structures. It is also useful in the
identi fi cation of pulsations in the pharynx which
may be indicative of abnormally placed carotid
arteries that might preclude pharyngeal fl ap or
sphincter pharyngoplasty surgery. Although
the latter fi nding is rare, it occurs most often in
patients with velocardiofacial syndrome. This
technique is useful not only for pretreatment
diagnostic assessment of velopharyngeal func-
tion but also to evaluate the outcome of surgi-
cal, prosthetic, and/or speech therapy treatment.
It may also be used for biofeedback therapy to
enhance velopharyngeal movements during
speech and correct some compensatory articu-
lation errors. For ease of examination in young
children, a fl exible nasopharyngoscope with
711
32 Diagnostic Procedures and Instruments Used in the Assessment and Treatment of Speech
a distal tip diameter between 2 and 3.7 mm is
recommended.
McWilliams et al. ( 1984 ) note that lateral wall
movement is not reliably assessed by either mea-
surements or judgments of nasoendoscopic
images. A major reason for using endoscopic
procedure is to learn about the contribution of the
lateral pharyngeal walls to velopharyngeal func-
tion. Some investigators have assumed symmetry
of lateral wall movement in making their judg-
ments, but this assumption is questionable.
Further data relative to the realizability and valid-
ity of endoscopic measurements are needed. In
the meantime, the technique is quite commonly
used clinically and has much to offer in the hands
of trained examiners.
32.7.1 Technique
A topical anesthetic such as 3 % lidocaine or 2 %
tetracaine hydrochloride mixed in equal parts
with .5 % phenylephrine is applied through one
side of the patient’s nasal cavity to allow com-
fortable insertion of the scope and enhance coop-
eration. Some patients are able to tolerate the
procedure without anesthesia, particularly when
a smaller scope is used. The nasopharyngoscope
is inserted through the middle meatus of the nasal
passage to fi rst visualize the velopharyngeal area
to document anatomy and function and scan the
pharynx for abnormal pulsations. The scope is
then passed down into the vocal tract to docu-
ment the anatomy and function of the tongue and
larynx. In patients with a preexisting pharyngeal
fl ap, the scope can be passed through one or both
ports to visualize the lower vocal tract as long as
the ports are not stenosed. A standard speech
sample is used at each observation point in the
vocal tract. Care must be taken when positioning
the scope in the velopharynx. A false-positive or
false-negative diagnosis of velopharyngeal clo-
sure will be obtained if the distal end of the scope
is not positioned directly above the velopharyn-
geal port. The quality of the study and interpreta-
tion, treatment planning, and outcome analysis
are enhanced by audio-video recording which
allows playback of the study for repeated analysis,
comparisons with previous examinations, and
demonstration of the fi ndings to the professionals
involved in the treatment and the patient and/or
parents.
32.8
The Nasometer
The nasometer is a computer-assisted instru-
ment produced by Kay Elemetrics (Pinebrook,
New Jersey) which is designed to measure the
relative amount of nasal acoustic energy com-
pared to oral acoustic energy during continuous
speech production (Dalston et al. 1981 ) . This
instrument uses a sound separator that rests on
the patient’s upper lip. Microphones on either
side of the sound separator sense oral and nasal
acoustic energy during speech, and this energy
is fi ltered and digitized by custom electronic
modules. The computer with Kay Elemetrics
software (Version 1.7) processes the informa-
tion and produces a “nasalance score.” This
score is a ratio of nasal to oral acoustic energy
multiplied by 100. The nasal and oral acoustic
energy is averaged during the production of
vowels and consonants in test sentences to pro-
duce the nasalance score. The length of the
speech sample used to calculate the nasalance
score may be up to 100 s. This instrument is
based on the Tonar II developed by Fletcher in
1976 (Fletcher and Bishop 1970 ) . An abnor-
mally high nasalance score during production of
nonnasal consonants suggests velopharyngeal
inadequacy and hypernasality, and an abnor-
mally low nasalance score during production of
nasal consonants is suggestive of hyponasality
and/or nasal airway impairment (Dalston et al. 1981 ) .
Dalston et al. ( 1981 ) studied the sensitivity and
speci fi city of the nasometer and found it to be an
appropriate instrument for use in corroborating
listener judgments of hypernasality. Sensitivity
and speci fi city values for assessing hyponasality
were in the expected range for patients without
any indication of concurrent velopharyngeal
inadequacy, but the scores did not identify hypo-
nasality in patients who exhibited both hypona-
sality and excessive nasal air emission.
Therefore, the instrument may be less useful as
712
S. Berkowitz
a diagnostic procedure in patients who exhibit
both velopharyngeal inadequacy and nasal air-
way impairment (Dalston et al. 1981 ) . The
nasometer is not a substitute for listener judg-
ments of hyper- and hyponasality but can be
useful in providing baseline data to assist in the
identi fi cation of velopharyngeal inadequacy,
assessment of treatment outcome, fi tting of a
palatal prosthesis, and providing visual biofeed-
back in speech therapy.
32.8.1 Technique
Care should be taken to ensure that the system is
calibrated according to the speci fi cations of the
manufacturer. The headgear is then adjusted to fi t
the patient, and the patient is asked to read or
repeat a standard speech sample. This procedure
is usually used with patients age 3 and older
because younger patients may have shorter atten-
tion spans and less well-developed speech and
language skills. Once the speech sample is
recorded on the computer terminal, the software
cursors are used to mark the beginning and end of
the speech display. The “calculate” function is
then activated, and the mean and standard devia-
tion of the nasalance score are determined.
32.9
Aeromechanical Measurement
32.9.1 Warren and Dubois Technique
(Warren and DuBois 1964 )
Measurements of nasal air fl ow and of the differ-
ence in air pressure above and below the velopha-
ryngeal port may be used to estimate both the
area of the velopharyngeal ori fi ce, if any, during
the production of stop consonants and the resis-
tance of the port to air fl ow. Pressure- fl ow mea-
surements provide information about the coupling
of the oral and the nasal cavities during speech
and about resistance in the system. They do not
describe the movement of particular structures,
such as the velum and lateral pharyngeal walls, or
the location and con fi guration of any opening
that is present.
32.9.1.1 PERCI
Warren ( 1979 ) introduced an instrument called
the PERCI (Palatal Ef fi ciency Rating Computed
Instantaneously) for use in the evaluation of the
velopharyngeal
mechanism
during
speech.
PERCI records and displays the difference in air
pressures in the mouth and in the nose. From a
study of 75 cleft palate patients, Warren reported
that patients with differential pressure readings
>3.0 on the PERCI had velopharyngeal ori fi ce
areas of 10 mm 2 or less, whereas those with
PERCI readings of <1.0 had areas greater than
20 mm 2 . PERCI readings of 1.0 through 2.9 were
associated with velopharyngeal areas between 10
and 20 mm 2 .
32.9.1.2 TONAR
Fletcher and Bishop ( 1970 ) advanced the study
of oral and nasal sound intensity measures as
indices to hypernasality through the development
of an instrument which they named TONAR (The
Oral-Nasal Acoustic Ratio). The instrument
prints out voltages associated with the nasal and
oral signals and also a trace re fl ecting the ratio of
the voltages from the sound detected in the oral
and nasal chambers.
32.10 Summary
Several instrumental procedures are available for
assessing the velopharyngeal mechanism and its
function. Each has advantages and disadvantages,
and choosing among them depends on the speci fi c
purpose of the evaluation. The reliability of endo-
scopic procedures is not well documented:
Aerodynamic measures provide data about the
area of the velopharyngeal opening, velopharyn-
geal resistance to air fl ow, and air pressure avail-
able for the production of obstruent sounds.
These measures provide no information about the
relative contributions of the velum and the pha-
ryngeal walls to velopharyngeal function.
An important warning in the use of any instru-
mentation for the study of speech is that data
taken during speech production must be inter-
preted within the context of the patient’s reper-
toire of speech pro fi ciency.
713
32 Diagnostic Procedures and Instruments Used in the Assessment and Treatment of Speech
References
Brader AA (1957) A cephalometric appraisal of morpho-
logic variations in cranial base and associated pharyn-
geal structures: implications in cleft palate therapy.
Angle Orthod 27:179
Dalston RM, Warren DW, Dalston ET (1981) The use
of nasometry as a diagnostic tool for identifying
patients with velopharyngeal impairment. Cleft Palate
Craniofac J 28:184–188
Fletcher SG, Bishop ME (1970) Measurement of nasality
with TONAR. Cleft Palate J 7:610–621
Hawkins CF, Swisher WE (1978) Evaluation of real-time
ultrasound scanner in assessing lateral pharyngeal
wave motion during speech. Cleft Palate J 15:161
King EW (1952) A roentgenographic study of pharyngeal
growth. Angle Orthod 22:23
McWilliams BJ, Morris HS, Shelton RL (eds) (1984)
Instruments for assessing velopharyngeal mechanisms
in cleft palate speech. CV Mosby, St. Louis
Prusansky S (1953) Description, classi fi cation, and analy-
sis of unoperated clefts of the lip and palate. Am J
Orthod 39:590
Ricketts RM (1954) The cranial base and soft structures in cleft
palate speech and breathing. Plast Reconstr Surg 14:74
Rosenberger HC (1934) Growth and development of the
nasorespiratory area in childhood. Ann Otol Rhinol
Laryngol 43:495
Shprintzen RJ, Rakoff SJ, Skolnick ML, Lavaroto AS
(1977) Incongruous movements of the velum and lat-
eral walls. Cleft Palate J 14:148–157
Skolnick ML (1970) Video fl uoroscopic examination of
the velopharyngeal portal during phonation in lat-
eral and base projections – a new technique for
studying the mechanics of closure. Cleft Palate J 7:
803–816
Skolnick ML, Azgzebski JA, Watkin KL (1975) Two-
dimensional ultrasonic demonstration of lateral pha-
ryngeal wall movement in real time – a preliminary
report. Cleft Palate J 12:299
Slaughter WB, Prusansky S (1954) The rationale for velar
closure as a primary procedure in the repair of cleft
palate defects. Plast Reconstr Surg 13:341
Subtelny JD (1955) Width of the nasopharynx and related
anatomic structures in normal and unoperated cleft
palate children. Am J Orthod 41:889
Subtelny JD (1957) A cephalometric study of the growth
of the soft palate. Plast Reconstr Surg 19:49
Subtelny JD, Baker HK (1956) The signi fi cance of ade-
noid tissue in velopharyngeal function. Plast Reconstr
Surg 17:235
Warren DW (1979) PERCI: a method for rating palatal
ef fi ciency. Cleft Palate J 16:279–285
Warren DW, DuBois AB (1964) A pressure- fl ow tech-
nique for measuring velopharyngeal ori fi ce area dur-
ing continuous speech. Cleft Palate J 1:52–71
715
S. Berkowitz (ed.), Cleft Lip and Palate,
DOI 10.1007/978-3-642-30770-6_33, © Springer-Verlag Berlin Heidelberg 2013
33
33.1
Muscles
33.1.1 Pharynx and Velum (Dickson
and Dickson 1982 )
The adult pharynx is the common pathway for
food and air in human beings. It extends from the
cranial base behind the nasal cavities to the upper
end of the esophagus behind the larynx. The phar-
ynx is widest at its upper portion and narrows as
it descends to the esophagus. The pharynx lies
immediately in front of the vertebral column, sep-
arated from it by prevertebral muscles and fascia.
Anteriorly, the pharynx communicates with the
nasal cavities, the oral cavity, and the aditus of the
larynx. Inferiorly, the pharynx is continuous with
the esophagus. The part of the pharynx that extends
upward to the level of the velum (soft palate) is
commonly called the nasopharynx (Fig. 33.1 ).
The pharyngeal ori fi ces of the auditory tubes
open into the lateral walls of the nasopharynx.
The auditory tube connects the pharynx with the
middle ear and serves to maintain an equilibrium
of air pressure between the middle ear and the
external atmosphere. The superoposterior part of
the pharynx contains the pharyngeal tonsil (also
called the adenoid).
The velum (soft palate) forms the boundary
separating the nasopharynx from the oropharynx.
Because the velum is mobile, this boundary is
arbitrary. The velum is a muscular body that
attaches to the posterior rim of the hard palate
and the lateral walls of the posterior part of the
oral cavity. The posterior free border of the velum
hangs into the oropharynx and ends in a small
midline projection, the uvula.
The oropharynx extends inferiorly to the level
of the hyoid bone. The anterior wall of the
oropharynx, inferior to the opening into the oral
cavity, is formed by the posterior surface of the
root of the tongue.
The palatopharyngeal fold (posterior faucial
arch) extends from the sides of the velum into the
lateral walls of the pharynx. This muscular fold
forms the greatest lateral constriction between
the oropharynx and oral cavity. The superior end
of the epiglottis extends superiorly from the lar-
ynx into the oropharynx immediately posterior to
the root of the tongue.
The nasopharynx and auditory tube are lined
by ciliated, pseudostrati fi ed columnar epithelium
rich in goblet cells and glands that secrete mucus.
The pharynx is lined with strati fi ed squamous
epithelium where contact between the pharynx
Variations in Nasopharyngeal
Skeletal Architecture
Samuel Berkowitz
S. Berkowitz , DDS, M.S., FICD
Adjunct Professor, Department of Orthodontics ,
College of Dentistry, University of Illinois ,
Chicago , IL , USA
Clinical Professor of Surgery and Pediatrics (Ret),
Director of Research (Ret),
South Florida Cleft Palate Clinic,
University of Miami School of Medicine ,
Miami , FL , USA
Consultant (Ret), Craniofacial Anomalies Program ,
Miami Children’s Hospital , Miami , FL , USA
e-mail: sberk3140@aol.com
716
S. Berkowitz
and velum takes place and in the portion that
serves as a food channel.
33.2
Nasopharyngeal Growth
Variations in the height and depth of the nasophar-
ynx are apparent from birth through the early years
of growth and development. Rosenberger ( 1934 )
made a longitudinal study of nasorespiratory areas
in children from 3 months to 5 years of age. It was
his opinion that an enlargement of the nasorespira-
tory area accompanied growth in the body and
great wing of the sphenoid as well as the forward
drift of the hard palate. Brodie ( 1941 ) demonstrated
that the hard palate moves downward, away from
the base of the skull, in a parallel manner with
increasing age and that this results in a progressive
increase in the height of the nasal and nasopharyn-
geal areas.
a
b
At rest
At rest
Vocalizing ‘Uuu...
Vocalizing ‘Uuu...
Fig. 33.1 ( a ) Lateral cephaloradiograph shows the skeletal
structures surrounding the pharyngeal space and allows
evaluation of the pharyngeal depth, shape of cervical spine,
soft palate size and length, and extent of soft palate eleva-
tion. Because it is a two-dimensional representation of the
velopharyngeal area and does not show lateral pharyngeal
wall motion, this record cannot be used to diagnostically
determine velopharyngeal functions. ( a ) Left : Structures
involved in controlling air fl ow as seen in a lateral cephalo-
graph at 5 years of age ( A anterior tubercle of the atlas, CS
cervical spine, S odontoid process, W posterior pharyngeal
wall, PS pharyngeal space, SP soft palate (velum), HP
hard palate, T tongue, M mandible). Right : Air fl ow when
vocalizing “Uuu” during normal speech. The soft palate
elevates and makes contact with the adenoids (if present)
or the posterior pharyngeal wall during normal speech and
swallowing. When the lateral pharyngeal muscles operate
in coordination with a competent (adequate length, width,
and timing of action) soft palate in a normal skeletal
environment most of the air is channeled through the
mouth while some enters the nose. This is designated as
velopharyngeal competency (VPC). ( b ) Velopharyngeal
Incompetency (VPI). There are usually many reasons for
inadequate air fl ow control. Some are (1) a relatively deep
pharynx when related to velar length, (2) inadequate velar
elevation and/or pharyngeal wall motion (neuromuscular
function), and (3) poor timing of speech with pharyngeal
and velar muscle (sensory-motor) function
717
33 Variations in Nasopharyngeal Skeletal Architecture
In a study of the growth of the pharynx, King
( 1952 ) concluded that there is a considerable
increase in the vertical dimension of the pharynx
resulting from the descent of the hard palate,
mandible, and hyoid bone, as well as from an
increase in the height of the cervical vertebrae.
He concluded that there is no appreciable increase
in the anteroposterior dimension of the pharynx
after an early age.
According to Scott ( 1953, 1957, 1958 ) ,
growth in the height of the pharyngeal area
is regulated by the cartilages of the cervi-
cal vertebrae. He, as well as Todd and Tracy
( 1930 ) , attributed growth in the anteroposte-
rior dimension of the nasopharynx to growth at
the spheno-occipital synchondrosis. Subtelny
( 1955 ; Subtelny and Baker 1956 ) studied verti-
cal growth of the nasopharynx and observed a
steady rate of increase up to 15 years of age.
Using a measurement from the posterior nasal
spine to the soft tissue of the posterior wall of
the pharynx, he observed a general increase in
depth of the nasopharynx at the level of the pal-
atal plane. Berkowitz ( 1989 ) believes that this
increase is due mainly to a change in the curva-
ture of the posterior pharyngeal wall.
Several investigators studying cleft palate sub-
jects have focused attention on skeletal structures
closely related to the nasopharynx. Ricketts
( 1954 ) found that the basilar portion of the occip-
ital bone varies in its position in relation to the
anterior cranial base and that these variations can
in fl uence the anteroposterior dimension of the
nasopharynx. Brader ( 1957 ) reported signi fi cantly
smaller anteroposterior and vertical nasopharyn-
geal dimensions in cleft palate subjects than in
noncleft individuals and noted that his cleft pal-
ate subjects had relatively larger masses of ade-
noid tissue within the nasopharynx than noncleft
individuals. He also observed no signi fi cant dif-
ference in the angularity of the cranial case in
individuals with cleft palate when compared with
individuals without clefts.
Moss ( 1965 ) studied malformations of the
skull base associated with cleft palate deformities
and indicated that a fl exion of the cranial base
(N-S-Ba) placed the basioccipital bone “more
anteriorly relative to the sphenoid bone,” thus
creating a smaller anteroposterior nasopharyn-
geal dimension. Subtelny ( 1955 ; Subtelny and
Baker 1956 ) showed that width of the skeletal
nasopharynx was larger in persons with cleft pal-
ate than in normal individuals.
Coccaro et al. ( 1962 ) studied nasopharyngeal
growth in children with cleft lip and cleft lip and
palate using serial cephalometric fi lms on 57 sub-
jects from 3 months to 7 years of age. Comparisons
were made with a noncleft group and with narrative
data supplied by Subtelny. Results showed that: (1)
The cleft palate group had smaller nasopharyngeal
height measurements than the normal group, but
only up to 3 years of age. (2) By 3 years of age, all
three groups had attained approximately 80 % of
their respective increases in nasopharyngeal height.
(3) Nasopharyngeal depth in the cleft palate group
increased slightly more than in the noncleft group
(6.8 mm vs. 4.3 mm). (4) The horizontal nasopha-
ryngeal changes in the cleft palate group were
shorter than in the noncleft group for most of the
age levels studied.
33.3
Functions
The pharynx serves as an air passage connecting
the mouth and nose to the lungs via the larynx
and trachea and as a food passage connecting the
mouth to the stomach via the esophagus. Because
these channels are common over part of their
length in humans, a sphincter is found at the level
of the velum to prevent food from entering the
nasal cavities during swallowing.
33.3.1 Swallowing
Constriction of the pharyngeal walls results in
changes in the diameter of the pharynx. These
changes are important to both swallowing and
speech. During swallowing, the lateral and poste-
rior walls of the pharynx move anteromedially,
reducing the cross-sectional area of the pharynx.
This results in a wavelike constriction that fl ows
down the pharynx and continues throughout the
entire digestive tract. The wavelike motion is
called peristalsis.
In the initial stage of swallowing, the phar-
ynx constricts around the velum while the
718
S. Berkowitz
velum moves somewhat dorsally and superiorly,
closing off the nasopharynx from the oropharynx
(a movement called velopharyngeal closure).
The oropharynx expands somewhat, and the lar-
ynx moves superiorly and ventrally, opening the
pharynx to receive the bolus of food. As the bolus
moves into the pharynx, the pharyngeal walls
constrict behind it, propelling the bolus toward
and into the esophagus.
33.3.2 Speech
Oropharyngeal constriction and changes in
length also occur during speech. These changes
affect the resonant frequencies of the vocal tract
and thus change the quality of the voice and
in fl uence the perception of what phoneme is pro-
duced. The lateral walls of the oropharynx move
medially on low vowels and laterally on high
vowels.
Velopharyngeal closure for speech eliminates
the nasal cavities from the resonance system and
permits the buildup of intraoral breath pressure to
produce fricative and plosive consonants. The
mechanics of velopharyngeal closure for speech
are quite different from those for swallowing.
During speech, the velum moves superiorly and
posteriorly against the posterior pharyngeal wall
(Fig. 33.2 ), while the lateral pharyngeal walls
move medially against the sides of the velum
(Fig. 33.3 ). By contrast, velopharyngeal closure
for swallowing is primarily pharyngeal.
Radiological studies have demonstrated that,
during closure for speech, the velum also
increases in length, particularly in its posterior
a
b
c
Fig. 33.2 ( a – c ) Passavant’s
Pad. The superior border of
the superior constrictor
pharyngeus muscle on
contraction forms a ridge or
pad on the posterior wall. It is
observed in both noncleft and
cleft patients. There is no
consistent relationship of the
pad to the velum, and it is
usually seen during swallow-
ing as well as during speech,
whistling, and blowing. Some
state that it functions
adequately for compensatory
purposes and does not appear
to contribute to velopharyn-
geal closure in all patients.
( a ) An intraoral view of
Passavant’s pads ( p ). ( b )
Lateral cephaloradiograph –
at rest. ( c ) While phonating
“Uuu,” showing Passavant’s
pad ( p ) at the level of the
anterior tubercle of the atlas
making contact with the
uvulae
719
33 Variations in Nasopharyngeal Skeletal Architecture
part. The velum contacts the posterior wall just
above the level of the anterior tubercle of the atlas
and on a plane with the hard palate. Velar elevation
is somewhat greater for males than for females,
and the greatest velar motion is at the junction of
its middle and posterior thirds.
Coronal
Sagittal
Circular
Circular-with
passavant’s ridge
Fig. 33.3 Four basic velopharyngeal closure patterns. The
size and shape of velopharyngeal valving patterns is very
variable (Skolnick 1975 ) . Although the closure patterns are
not actually discrete, for convenience sake, Skolnick et al.
( 1973 ) categorized velopharyngeal valving into four pat-
terns. These patterns are: Coronal pattern: The majority of
valving is palatal with the full width of the velum contact-
ing the posterior pharyngeal wall. The lateral walls move
medially to the lateral edges of the velum. There is no
motion in the posterior wall. Sagittal pattern: The majority
of valving is pharyngeal. The lateral walls move to the mid-
line and approximate each other. The soft palate does not
contact the posterior pharyngeal wall but instead abuts up
against the approximated lateral pharyngeal walls, thus
completing the closure pattern. Circular pattern: There is
essentially equal contribution from the velum and lateral
pharyngeal walls with the bulk of the musculus uvulae
acting as the focal point. The dorsum of the musculus
uvulae contacts the posterior wall (which does not move).
The lateral walls squeeze around the bulk of the musculus
uvulae. Passavant’s ridge pattern: As in the circular pattern,
there is essentially equal contribution from the velum and
lateral pharyngeal walls, but in addition, the posterior phar-
ynx moves forward. The musculus uvulae also serves as the
focal point for closure in this pattern (Reproduced with
permission from Siegel-Sadewitz and Sphrintzen (1982) )
720
S. Berkowitz
It is of interest to note that both velar elevation
and the degree of motion of the lateral nasopha-
ryngeal walls during speech are greater for high
vowels than for low vowels. This is the reverse
of the activity of the lateral walls of the orophar-
ynx. The degree of motion, and in fact the degree
to which complete closure is achieved, depends
on both the phoneme being produced and its
phonemic context. Motion is greatest for frica-
tives, less for high vowels, and least for low vow-
els. The velopharyngeal function is greater for
vowels in a nonnasal context than in a nasal con-
sonant context. Finally, the extent of lateral wall
motion and the degree of velar motion are
synchronized and directly and highly related to
each other.
Velopharyngeal closure during speech has
received considerable attention, and properly so
because it is an important factor in the treatment
of cleft patients. It is evident that the tensor veli
palatini muscle is not active in velopharyngeal
function. The palatopharyngeus is active during
velar lowering, but its activity is inconsistent and
vowel-dependent. Palatoglossus activity relates
more to tongue motion than to velar activity. The
superior constrictor and levator veli palatini
muscles are the only two velar muscles studied
(the uvulus muscle has not been de fi nitively
studied) that are active during velopharyngeal
function for speech. All investigators agree that
levator veli palatini activity is synchronized with
velar height.
Indirect evidence indicates that the uvulus
muscle plays an essential role in velopharyngeal
function. Nasopharyngoscopy has revealed a
patient population with hypernasality, a midline
longitudinal concavity rather than convexity of
the dorsal velar surface, translucency of the lon-
gitudinal velar midline, and a small midsagittal
gap between the velum and posterior pharyngeal
wall during velopharyngeal closure for speech.
This lends presumptive evidence that what has
been called the levator eminence is actually a
function of the uvulus muscle. In lateral projec-
tion, the velum may seem to touch the posterior
pharyngeal wall, even when there is actually a
small central gap because of the absence of the
musculus uvulae.
Thus, velopharyngeal closure for swallowing
is accomplished by the superior constrictor, leva-
tor veli palatini, and uvulus. Closure for speech is
accomplished by the levator veli palatini and
uvulus. The difference in function during swal-
lowing and speech is also apparent among per-
sons with velopharyngeal insuf fi ciency due to
congenital disproportion between the pharyngeal
depth and velar length or disproportion following
surgery for cleft palate. In our experience, most
individuals in this group who are unable to
achieve velopharyngeal closure for speech do so
consistently during swallowing.
33.4
The Role of the Nasal Cavity
(Fig. 33.4a )
The nasal cavities are the fi rst points of entry and
the last points of exit of air fl ow to and from the
lungs. They serve to fi lter the air as it enters the
airway and to control air temperature and humid-
ity. They also serve as resonators for nasal sounds
that are produced with the velopharyngeal valve
open. The two nasal cavities are separated from
each other by the nasal septum in the midsagittal
plane. The nasal septum is composed of three
parts. The superior part is the perpendicular plate
of the ethmoid bone. The inferior and posterior
parts are composed of the vomer bone. The ante-
rior part is composed of the cartilage of the nasal
septum.
The roof of the nasal cavities is formed by the
cribriform plate of the ethmoid bone, which is
penetrated by the olfactory nerves. Immediately
posterior to the perpendicular plate of the eth-
moid is the body of the sphenoid, which contains
the sphenoid sinus. The fl oor of the nasal cavities
is formed by the hard palate. Anteriorly, the nasal
cavities are bounded by the external nose. The
framework of the external nose includes the nasal
bones and the nasal cartilages. The bridge of the
nose is formed by the nasal bones. Inferiorly, the
nasal bones articulate with the lateral nasal carti-
lages. The tip of the nose is supported by the
greater alar cartilages. Small lesser alar cartilages
support the lateral walls of the anterior nasal
openings.
721
33 Variations in Nasopharyngeal Skeletal Architecture
The lateral wall of each nasal cavity is convo-
luted owing to the presence of the superior, mid-
dle, and inferior nasal conchae. The conchae have
an extremely rich blood supply and provide a
large surface area for humidity and temperature
control of the air passing over them.
Although the terms VP “incompetence,”
“inadequacy,” and “insuf fi ciency” historically
have been used interchangeably, they do not
necessarily mean the same thing. For this reason,
standardization of nomenclature has been recom-
mended. Trost-Cardamone ( 1989 ) has proposed a
taxonomy for VP disorders, based on causative
factors in which “velopharyngeal inadequacy is
the generic term used to denote any type of abnor-
mal velopharyngeal function.” In the broad group
of inadequacies, there are subgroups of structural
(VPI), neurogenic (VP incompetence), and mis-
learning (VP mislearning) or functional origins.
VPI includes any structural defects of the velum
or pharyngeal walls at the level of nasopharynx
with insuf fi cient tissue to accomplish closure or
Nasal septum
Sup.Articular
Surface
Basion
Post.
tubercle
ANT.Tubercle
Occipital condyle
Odontoid process
of axis
Inf.articular
process
Opisthion
Bolton
point
Perpendicular p
of ethmoid bone
Cartilage of
nasal septum
Vomer
a
c
b
Fig. 33.4 ( a ) The normal nasal septum: The inferior ( i ),
middle ( m ), and superior turbinates are attached to lateral
nasal walls. ( b ) The nasal septum is composed of the per-
pendicular plate of the ethmoid, the cartilage of the nasal
septum, and the vomer. In complete clefts of the lip and
palate at birth, the nasal septum is displaced to the non-
cleft side in varying degrees. The con fi guration of the
nasal septum and the turbinates on the cleft side limits the
medial movement of the cleft palatal segment after lip and
palate repair. ( c ) Cervical spine: The condition and the
relationship of the various vertebrae to each other can
in fl uence the depth and con fi guration of the pharyngeal
space. For example, the anterior tubercle of the atlas (1 C,
fi rst cervical vertebrae) supports the posterior pharyngeal
wall. If it is absent or dislocated, the functional pharyn-
geal depth may increase to a nonfunctional state
722
S. Berkowitz
some kind of mechanical interference with clo-
sure. The all-encompassing term “velopharyn-
geal dysfunction (VPD)” does not assume or
exclude any possible cause of the perceived
speech symptoms or management approach. It
applies to speech disorders that may be the result
of structural de fi cits, neurologic disorders, faulty
learning, or a combination of sources (Witt and
D’Antonio 1993 ) .
33.5
The Use of Lateral
Roentgencephalometrics in
Evaluating Skeletal Pharyngeal
Architecture and Velar
Elevation
Velopharyngeal valving is dependent not only on
the sensorimotor adequacy of the velum and its
synergistic musculature but also on the morpho-
logic skeletal dimensions of the nasopharyngeal
port. The size and shape of the nasopharynx are
determined, in part, by the contiguous osseous
anatomy of the maxilla (Subtelny 1955 ) , cranial
base, and vertebral column (Figs. 33.4 and 33.5 ).
The mechanism of velopharyngeal closure can
be studied to some degree by cephalometric
roentgenography, but it must be stressed that this
is not a functional test for velopharyngeal compe-
tency (Fig. 33.6 ). The vowel /u/, as in “boom,” is
generally employed to achieve maximal eleva-
tion of the soft palate during vowel production.
After careful rehearsal, the subject is instructed
to maintain the sound at a constant pitch and
intensity for a time suf fi cient to cover the roent-
genographic exposure period. This procedure
ensures that the soft palate will remain in a rea-
sonably stable position during the recording
interval.
In a noncleft individual, as seen in the lateral
headplate, the soft palate elevates to contact the
posterior pharyngeal wall. In doing so, the longi-
tudinal axis of the soft palate becomes continu-
ous with that of the total palatal plane, while the
uvula projects downward and nearly at right
angles to the rest of the velum. If for any reason
this velopharyngeal closure cannot be achieved,
deglutition is impaired, and in phonation, the air
stream is misdirected through the nose. However,
it is possible to have good swallowing activity but
poor velopharyngeal closure during speech. The
term “palatal insuf fi ciency” is more descriptive
of a physiologic de fi ciency than of an anatomic
defect.
33.6
Cervical Spine Anomalies
(Fig. 33.7 )
Osborne ( 1968 ) wrote that fusion of the posterior
spines at C2–C3 does not appear to in fl uence the
osseous anteroposterior diameter of the nasopha-
ryngeal port. On the other hand, occipitalization
of the atlas, a smaller-than-normal anterior arch
of the atlas, or atlantoaxial dislocation will have a
direct effect on the anteroposterior dimension of
the pharynx. It is clear that the population of con-
genital palatopharyngeal incompetence (CPI)
patients with cervical anomalies, of whatever
kind, present greater osseous nasopharyngeal
depth (the distance between the hard palate and
retropharyngeal wall) than do CPI patients with-
out cervical anomalies.
The importance of the anterior tubercle of the
atlas and the upper cervical vertebrae in achiev-
ing adequate velopharyngeal closure and speech
is well established. One need know only that the
musculofascial layer covering the upper cervical
vertebrae, and forming the posterior pharyngeal
wall, is only 2–5 mm thick to realize the morpho-
logic importance of these vertebrae to velopha-
ryngeal closure.
The in fl uence of anomalies of the upper cer-
vical vertebrae on the size and shape of the
nasopharynx, with resulting effects on velopha-
ryngeal valving, was fi rst reported by McCarthy
( 1925 ) and, shortly thereafter, reemphasized by
Schuller ( 1929 ) . Epidemiologic studies have
shown that patients with craniofacial birth
defects, many of which are accompanied by
velopharyngeal incompetence, have a higher
prevalence of upper cervical spine anomalies
than the general population (Osborne et al.
1971 ) . This survey also found that a surprisingly
large number (18.8 %) of patients with CPI
demonstrated anomalies of the upper cervical
723
33 Variations in Nasopharyngeal Skeletal Architecture
D.D
13.6
SN
144
GB
10.0
KC
S.G
9.1
PO
6.8 00 6.10
L.I
7.0
7.10
G.E
6.4
CB
9.0
SN20
120
Fig. 33.5 Functional problems related to cervical verte-
brae, cranial base, nasopharynx, adenoid, tonsils, and the
tongue. DD dorsal displacement of vertebrae, GE con-
genital deformity with ventral displacement of cervical
vertebrae, GB obtuse cranial base upward and backward
location of anterior arch of the atlas and severe cleft palate
speech (From functional diagnosis of malocclusion)
724
S. Berkowitz
vertebrae, as discerned in lateral cephalometric
head fi lm.
Simply stated, congenital palatopharyngeal
incompetence (CPI) can be de fi ned as the pres-
ence of hypernasality (rhinolalia aperta) in the
absence of an overt cleft palate. Based on detailed
studies of 110 subjects, Pruzansky and Mason
( 1966 ) found that CPI may be caused by one or
more of the following variables: a bi fi d uvula; a
short or thin soft palate, with or without a pink
translucent area (“zona pellucida”) usually seen
in submucous cleft palate; anomalies of the upper
cervical vertebrae and cranial base; and scanty
adenoid development or early involution or
excision of the adenoid.
As we shall point out in more detail later, our
own concept of the defect in congenital palatal
insuf fi ciency has been extended to include a
broader view of the pharynx and its contiguous
structures.
s
s
1
23
4
N
N
FH
FH
Ba
35 mm
At rest
Phonating “U”
PNS
ANS
Ba
Ptm
PNS
ANS
AA
35 mm
66º
58º
50º
40º
138º
120º
Fig. 33.6 The in fl uence of the skeletal architecture on
the pharyngeal form and size. The pharyngeal space is
bounded superiorly by the cranial base and laterally by
the cervical spine on one side and the tongue/hard palate
complex on the opposite side. The odontoid process
( axis ) of the cervical spine points to the posterior extent
(Basion-Ba) of the basilar portion of the occipital bone. It
is the median point of the anterior margin of the foramen
magnum. The hard palate of the maxillary complex is
anatomically associated with the anterior cranial base
and can vary in its anteroposterior dimension. The impor-
tance of cranial base angle (Ba-S-N). Left : Obtuse cranial
base angle. In cases with a severe obtuse cranial base
angle, the pharyngeal space is usually deeper than normal
even in the presence of a long hard palate. An obtuse cra-
nial base positions the cervical spine more posteriorly
since it must be associated with the basilar portion of the
occipital bone. This condition is usually seen when
hypernasality exists in the absence of an overt palatal
cleft and is called congenital palatal insuf fi ciency (CPI).
Right : With an acute cranial base angle, the cervical spine
is positioned close to the hard palate creating a shallow
pharyngeal depth. Comment: These relationships change
with growth. The palate descends from the anterior
cranial base in a parallel fashion. Due to changing slope
of the posterior pharyngeal wall, the pharyngeal depth
increases with age. The in fl uence of adenoids: The
adenoid is attached to the posterior pharyngeal wall above
the level of the hard palate. Its size is highly variable
according to age. It usually increases until 13 years of age
and then retrogresses, but many variations in this growth
pattern have been observed. The in fl uence of cleft size:
The severity of the cleft size in complete clefts of the lip
and palate at birth does in fl uence the nasopharyngeal
width before lip and/or palatal surgery, but not after the
lip is united. The degree of mesodermal de fi ciency of
the hard and soft palate can determine not only the size of
the cleft space by in fl uencing palatal size but also the
length and width of the soft palate and, therefore, its
functional ability to affect velopharyngeal closure (VPC).
A short anteroposterior maxillary dimension, as is usually
found with submucous clefts, does not by itself signify
that VPC will be inadequate. Short anteroposterior hard
palate dimension can be associated with a shallow
pharyngeal depth
725
33 Variations in Nasopharyngeal Skeletal Architecture
Fig. 33.7 ( a – e ) Cervical spine anomalies and their
effects on the pharyngeal space con fi guration. ( a ) The
absence and ( b ) malformation or dislocation of the ante-
rior tubercle of the atlas result in the lack of support to the
posterior pharyngeal wall, thus increasing the depth of the
pharyngeal space. These anomalies coupled with a small
velum will compound the problem. Left : Velum at rest.
Right : Velum in function. No contact with the posterior
pharyngeal wall
726
S. Berkowitz
c
Fig. 33.7 (continued) Other cervical spine anomalies include: ( c ) Cervical spine anomalies abnormal atlanto-dental
angulation
727
33 Variations in Nasopharyngeal Skeletal Architecture
Fig. 33.7 (continued)
d
e
( d ) Cervical spine anomalies. Fusion of posterior arches C2–C3. ( e ) Abnormalities to the axis
and atlas (Courtesy of S. Pruzansky)
728
S. Berkowitz
33.7
Velar Closure
During the clefting process, the muscular forces
that act in the region of the pterygoid plates
account for the divergence of the pterygoid plates
and the consequent increased lateral width of the
nasopharynx, the separation of the tuberosities of
the maxillae, and the width of the cleft itself. The
failure of the hard and soft palate to unite in the
midline produces a profound disturbance in the
interplay and balance among the involved muscle
groups. The tongue, pterygoid muscles, levators,
and tensors of the velum tend to aggravate the
malformation that has resulted from the failure of
union in the midline of the palate.
A more favorable alignment of the osseous
segments of the palate will follow reestablish-
ment of balanced muscle forces. Slaughter and
Pruzansky ( 1954 ) concluded that, since repairing
the lip altered the spatial con fi guration of the
skeletal framework so profoundly in the anterior
segment of cleft, repairing the velum might
achieve comparably bene fi cial results in the
region of the nasopharynx.
In reviewing the morphologic variants that
might contribute to inadequate velopharyngeal
function, a number of factors must be considered.
For example, the velum might be too short, the
hard palate could be de fi cient in its anteroposte-
rior dimension, or there may be unilateral or bilat-
eral and posterior pharyngeal wall dysfunction. In
other cases, both the hard and soft palates might
be shorter than normal (Fig. 33.8a–e ). In rare
instances, the soft palate may be paralyzed and
incapable of movement (Fig. 33.8f ). The cases
presented in Figs. 33.9 and 33.10 show varia-
tions in adenoid size and pharyngeal architecture
which in fl uence velar pharyngeal closure.
Fig. 33.8 ( a – e ) Variations in the pharyngeal space, velar
elevation, and adenoid size. ( a ) Left : Normal pharyngeal
architecture at rest, 10 years of age. Right : While vocal-
izing “Uuuu….” Note the velum of good length making
contact against a small adenoid – the hard palate is posi-
tioned above the anterior tubercle. ( b ) Short, stubby velum
in a relatively small pharyngeal space making good con-
tact with the posterior pharyngeal wall at the level of the
anterior tubercle, 12 years of age, no adenoids. 1 . At rest,
2 . Vocalizing “Uuuu….”
729
33 Variations in Nasopharyngeal Skeletal Architecture
Fig. 33.8 (continued) ( c ) Cephaloradiographs taken at
4 years of age while vocalizing “Uuuu….” The small
velum is seen making contact with large adenoid creating
a shallow pharyngeal depth. ( d ) A small, thin paralyzed
velum in a deep pharynx at 5 years of age. ( e ) A long
velum of good thickness showing poor elevation resulting
in a lack of contact with posterior pharyngeal wall. The
velum does not elevate on vocalizing “Uuuu….” The
velum may or may not make contact in sustained speech.
That is why this record is not a functional test
730
S. Berkowitz
a
b
Fig. 33.9 ( a , b ) Variations in adenoid size and its effect
on nasal air fl ow. ( a ) Partial nasal obstruction at 4 years of
age ( left ) and 11 years of age ( right ). ( b ) Complete nasal
obstruction shown at 8 years of age ( left ) and 2 years of
age ( right ) ( * , adenoid)
731
33 Variations in Nasopharyngeal Skeletal Architecture
Fig. 33.10 ( a – c ) Nasal obstruction associated with mid-
facial hypoplasia and cranial base abnormalities in three
different craniofacial syndromes: ( a ) Apert syndrome
( a adenoid, b soft palate), ( b ) mandibulofacial dysostosis,
and ( c ) Crouzon syndrome
33.8
Improving Velopharyngeal
Closure
Traditionally, surgeons have focused on the symp-
tom of hypernasality and have attributed it to inad-
equate palatal length. Accordingly, operations
have been devised to address these structural
de fi cits. Many procedures can be used to control
air fl ow movements. Some have been successful;
others have had limited success. The relationships
among perceptual, anatomic, and physiologic vari-
ables have not been appreciated until relatively
recently. To believe that hypernasality generally is
the result of inadequate anatomy alone represents
a simplistic view of VP function (Witt and
D’Antonio 1993 ) .
33.8.1 Pharyngeal Flaps
The pharyngeal fl ap is the surgical procedure
most widely used to correct velopharyngeal
insuf fi ciency for speech. One end of the fl ap
remains attached to the pharyngeal wall, while
the other is sutured to the palate to occlude most
of the velopharyngeal space. There are a number
of variations of this procedure which involve
where the fl ap from the posterior wall is inserted
in the velum. It may be positioned midway on the
superior surface, sandwiched (velar split) within
the velum, or placed at the end and superior sur-
face of the velum. The posterior fl ap also may be
superiorly or inferiorly based (Figs. 33.11 and
33.12 ). Most surgeons and speech-language
732
S. Berkowitz
pathologists seem to favor the superiorly based
fl ap in which the base of the fl ap is positioned in
line with the anterior tubercle of the atlas. This is
the point where the velum in most adults makes
contact with the posterior pharyngeal wall during
normal speech.
The pharyngeal fl ap is an unphysiological
substitute for the velopharyngeal mechanism
because it acts as a sail to capture and channel
most of the air through the mouth. The remaining
pharyngeal port size will determine whether
hypernasality or hyponasality will be present
after surgery. Warren ( 1964a, b ) , analyzing
aerodynamic pressure fl ow patterns in normal
speech, determined that hypernasality occurred
when the velopharyngeal port exceeded 10 mm 3
and that nasal escape of air was clearly evident at
20 mm 3 . Bjork ( 1961 ) , using basal radiographic
techniques, estimated that a port size of 20 mm 3
in an area was the threshold between hypernasal
and hyponasal speech.
Shprintzen et al. ( 1979 ) reported that the fl ap
may have to be positioned more to one side than
another according to the location of the port
Fig. 33.12 Inferiorly based pharyngeal fl ap in the pres-
ence of a small adenoid. Even with good velar length and
elevation, a pharyngeal fl ap was necessary because of
poor lateral pharyngeal wall muscular movement. Left : At
rest. Right : While vocalizing “Uuuu....” Inferiorly based
fl aps and/or fl aps placed below the anterior tubercle of the
atlas usually do not function adequately because that may
not be the position for maximum lateral pharyngeal
muscle action
Fig. 33.11 ( a – d ) Variations in the placement of pharyn-
geal fl aps. Superiorly based fl aps are taken from the pos-
terior pharyngeal wall above or at the anterior tubercle of
the atlas. ( a ) Anteriorly superiorly based fl ap. ( b )
Posteriorly superiorly based fl ap. ( c ) Velar split – superiorly
based. Flap is taken from the pharyngeal wall at the level
of anterior tubercle. ( d ) Inferiorly based fl ap was taken
from the posterior pharyngeal wall below the anterior
tubercle of the atlas
733
33 Variations in Nasopharyngeal Skeletal Architecture
space. With recent improvements in diagnostic
abilities to determine the location and degree
of air escape, nasopharyngoscopy has become
the procedure of choice to predetermine the
lateral position to place the pharyngeal fl ap.
Unfortunately, surgical skill is not the sole deter-
minant of the procedure’s success, and patients
still need to undergo extensive speech therapy.
Occasionally, a patient will remain hypernasal
and continue to produce articulation errors in
spite of a seemingly good surgical result. This
can occur even when there seems to be a good
speech mechanism, good muscular motion, and a
good family environment.
An excessively wide fl ap can block off too
much air fl ow into the nose, leading to mouth
breathing and denasalized nasal consonants, and
patients may suffer other side effects ranging
from severe snoring to sleep apnea. As in normal
speech, the opening and closing functions of the
nasopharynx necessary for speech must be
achieved by lateral wall movements in the
nasopharynx. The lateral walls need to move
medially to make contact with the fl ap during
speech to prevent nasal escape of air.
A pharyngeal fl ap was also used in conjunc-
tion with push-back procedures at the time of
palatoplasty, thinking that when combined, the
retropositioned velum would be better maintained
in its posterior position. Experience has shown
that this should never be performed as a primary
procedure because there is no evidence that
velopharyngeal incompetency can be detected
with certainty prior to speech production. Scar
contracture resulting from push-back procedures
not only inhibits palatal growth but causes any
velar length increase to relapse. It does not seem
wise to tether the push-back tissue with the pha-
ryngeal fl ap because negative changes occurring
to the palatal tissue can distort the fl ap.
In the treatment of cleft palate, the superiorly
posterior-based pharyngeal fl ap is almost
universally favored. Some clinicians are also
using inferiorly based fl aps in some cases (Millard
1963, 1980 ) . It is still debated whether the supe-
riorly or inferiorly based procedure is better.
However, Trier ( 1985 ) points out there are com-
pelling reasons why the superiorly based fl ap is
employed more frequently. The inferiorly based
fl ap not only has severe length limitations but
also has the disadvantage of tethering the fl ap in
an inferior direction, away from the palatal plane
and motion for affecting VP closure.
Most speech-language pathologists tend to favor
performing a pharyngeal fl ap at 4 years of age to
avoid the problems of prolonged training. Early
pharyngeal fl ap surgery carries no systemic risk of
interference with facial growth but can cause some
reduction in forward maxillary growth. It also can
cause hyponasality and sleep apnea if it signi fi cantly
obstructs air fl ow through the nose (Subtelny and
Nieto 1978 ) . The ability of patients to achieve
proper velopharyngeal closure after pharyngeal
fl ap surgery is unpredictable. Retrospective studies
have shown that, when performed prior to 3 years
of age, there are no signi fi cant morphological
differences in the velopharyngeal area among the
various cleft groups. Harding et al. ( 1975 ) and his
colleagues advocated a superiorly based pharyn-
geal fl ap at a mean age of 6.5 years. Randall et al.
( 1978 ) advocated performing the procedure even
earlier, between 2 and 3 years of age, especially
when associated with a short, scarred, immobile
palate with severe hypernasality and virtually no
anterior articulation.
Failed pharyngeal fl aps are generally due to
the inability of the lateral pharyngeal walls to
gain fi rm contact with the sides of the pharyngeal
fl ap. This can result if the fl ap is too narrow and/
or the lateral pharyngeal walls do not move far
enough medially to reach it. In some instances,
the fl ap may be set below the point of maximum
medial movement, which is usually at the level
of the hard palate. He also states that, under no
circumstances, should a pharyngeal fl ap opera-
tion be performed simultaneously with other
procedures.
33.8.2 Speech Aid Appliances
Wolfaardt et al. ( 1993 ) noted that the functional
integrity of the palatopharyngeal valve can be
compromised by a number of factors, including
neurologic disorders such as stroke or head
trauma; degenerative diseases such as multiple
734
S. Berkowitz
Fig. 33.13 ( a – c ) Speech aid appliances. In the event that
surgery is contraindicated or delayed or, if performed,
does not produce proper palatopharyngeal closure, a den-
tal speech appliance may be substituted. Such appliances
may be made of acrylic plastic retained by clasps about
the teeth. Metallic castings of nonprecious and precious
metals may also be used. ( a ) The appliance consists of
three parts: ( a ) the palatal, ( b ) the velar, and ( c ) the pha-
ryngeal sections. The palatal section ( a ) covers the palate
and carries the clasps for attachment to the teeth. ( b ) The
palatal section supports placement for the missing teeth
and may also plump the upper lip to improve the facial
pro fi le. The velar section ( b ) is in the shape of a bar con-
necting the palatal section to ( c ) the pharyngeal portion of
the appliance which extends into the nasopharynx and
assists in palatopharyngeal closure and reducing nasal
resonance. ( c ) When midfacial vertical dimension is lost
( top ) and must be increased, the palatal section will need
to replace missing teeth and cover the natural teeth ( bot-
tom , wearing appliance). In such cases, the teeth need to
be crowned in gold to protect them from dental caries. The
unoperated cleft space ( a ) is covered with a posterior
extended acrylic section ( c ) to help control air fl ow and
permit proper feeding. ( d ) Replacement teeth. The pha-
ryngeal section needs to extend into the nasopharynx and
make contact with the posterior pharyngeal wall (Courtesy
S. Pruzansky)
sclerosis, Parkinsonism, or bulbar polio; cranio-
facial birth defects, such as overt and covert clefts
of the palate; and some behavioral disorders
(e.g., functional articulation disorders).
In addition, in the absence of a palatopharyn-
geal control problem, resonance balance in
speech also may be rendered aberrant by hearing
loss or faulty sensorimotor learning patterns.
These include impairments associated with con-
genital neurologic disorders (e.g., cerebral palsy)
that impair a speaker’s ability to regulate the
articulatory dimensions of speech that in fl uence
the acoustical expression of nasality.
Prosthetic dental appliances are used for the
replacement of missing or malformed teeth in the
line of the cleft and, when indicated, in the design
of speech appliances.
In the event that surgery is contraindicated,
delayed, or performed unsuccessfully, a den-
tal speech appliance may provide a satisfactory
substitute. Such appliances are usually made
of acrylic plastic and retained by clasps about
the teeth. Metallic castings of nonprecious and
precious metals also may be used in the design of
speech appliances.
For purposes of description, the appliance
consists of three parts: the (a) palatal, (b) velar,
and (c) pharyngeal sections (Fig. 33.13 ). The
palatal section may consist of little more than a
covering for the hard palate and carry the clasps
for attachment to the teeth. This section may also
supply replacements for missing teeth. In cases in
which vertical dimension must be increased
because of arrest of vertical growth of the middle
735
33 Variations in Nasopharyngeal Skeletal Architecture
face, the palatal section may cover the natural
teeth. In these circumstances, the teeth should be
crowned to protect them from dental caries. By
forward extension, the palatal section may also
plump the upper lip and improve the facial
pro fi le.
The velar section is in the shape of a bar con-
necting the palatal section to the pharyngeal por-
tion of the appliance. The pharyngeal portion
extends into the nasopharynx and assists in
palatopharyngeal closure (Figs. 33.14 and 33.15 ).
The skill of prosthodontists in designing these
devices has added a fortunate dimension to
speech habilitation of cleft palate whenever
surgery is unable to provide good results.
This treatment technique is logically com-
bined with auditory training. The patient needs to
learn to rely on auditory and intraoral somesthetic
Fig. 33.14 ( a – c ) The pharyngeal bulb in a speech
appliance is viewed in cross section in place in the
nasopharynx. ( a ) Incompetent velopharyngeal closure.
( b ) Inadequate nasopharyngeal bulb. ( c ) Pharyngeal bulb
(pharyngeal extension) is well positioned within the nasal
chamber (Courtesy J.D. Subtelny)
Rest
Phonation of [a]
Fig. 33.15 The pharyngeal bulb on a speech aid appli-
ance is viewed in the nasopharynx. At rest ( left ), a lateral
space on either side of the bulb allows for normal nasal
drippings to enter the mouth. When phonating /a/, ( right )
the lateral pharyngeal walls make contact with the speech
bulb, reducing nasal air fl ow (Courtesy of J.D. Subtelny)
736
S. Berkowitz
perceptions of resonance balance and palatopha-
ryngeal control. Articulation training is likewise
conducted to normalize consonant distortion or
substitution patterns that patients may have devel-
oped as compensatory maneuvers in the face of
palatopharyngeal incompetence.
Some speech-language pathologists and den-
tists utilize a palatal lift appliance (PLA) which is
designed to hold the soft palate up and backward
and to ultimately improve its function by acting
as an isometric training appliance (Fig. 33.16 )
(Wolfaardt et al. 1993 ; Mazaheri and Mazaheri
1974 ) . D’Antonio (1995, personal communication)
suggests that the PLA be used for patients in
whom there is adequate tissue but poor control of
coordination and timing of VP movements. It is
hoped that the degree of lift can be reduced grad-
ually until the appliance can be discarded.
Unfortunately, in many instances the prosthesis
must be worn inde fi nitely. The use of a palatal lift
appliance (PLA) is still controversial. Some
believe that, at best, its use produces inconsistent
results even with speech therapy. The palatal lift
appliance (PLA) has also proved useful in reduc-
ing drooling and improving mastication and
tongue movement. Speech therapy associated
with use of a PLA may include any one or a num-
ber of techniques, many of which are practiced
by the patient with and without the appliance in
place (Wolfaardt et al 1993 ) .
Auditory training should be used to enhance
the patient’s auditory awareness of normal and
deviant resonance balance. Articulation training
is used to develop a patient’s palatopharyngeal
control to regulate nasal resonance.
33.8.3 Push-Back Procedures
(Velar Lengthening)
These are surgical procedures designed to
lengthen the soft palate done either in conjunction
with primary palatoplasty or as a secondary pro-
cedure after the palatal cleft has been closed and
velopharyngeal incompetence has been detected.
The most common surgical procedure is the V-Y
push-back, commonly called the Wardill-Killner,
Wardhill ( 1933, 1937 ) or Veau-Wardill-Killner
procedure. There are a number of variations of
this basic design. This procedure involves the use
of two unipedicle or single-base fl aps of palatal
mucoperiosteum, the soft tissue on both sides of
the cleft space. The fl aps are elevated from the
bony palate and retropositioned so that the cleft
space is covered and the length of the soft palate
increased. Push-back palatoplasty procedures
a
b
Fig. 33.16 ( a , b ) A palatal lift appliance. It is used to
improve velopharyngeal function by improving velar
muscle action. ( a ) The appliance on a dental model. The
velar extension extends posteriorly to the uvulae. ( b )
Lateral cephalometric tracing showing the appliance
( A-A ¢ ) velar section making contact up to the uvulae ( B ).
The appliance can be well stabilized by using orthodontic
bands with soldered buccal shelves to establish undercuts
for the clasps
737
33 Variations in Nasopharyngeal Skeletal Architecture
performed at an early age require an extensive
amount of mucoperiosteal undermining and soft
tissue displacement leaving large areas of
denuded bone. These areas are later covered over
by scar tissue which inhibits palatal bone growth
and causes palatal deformation. This problem
exists even when the push-back procedure is per-
formed as a secondary procedure after the palatal
cleft has already been closed.
Lateral cephalometric evaluation of this and
other push-back techniques has shown that the
net gain in soft palatal length is not as great as the
amount of mucoperiosteum brought posteriorly
because of contraction of scar tissue. Yet, some
speech-language pathologists believe this tech-
nique yields a higher success rate, as measured
by speech results, than any other surgical tech-
nique. Harding et al. ( 1977 ) criticized this proce-
dure because large surfaces of denuded bone are
left in the anterior palate, which result in exces-
sive scarring and midfacial growth retardation.
Other palatal lengthening procedures, such as
Millard’s Island Flap, have not been successful
because the extensive mucoperiosteal shifting
leaving large areas of denuded bone has been
shown to have a deleterious effect on palatal
development.
Witt and D’Antonio ( 1993 ) recommend
Furlow’s double-opposing Z-plasty for patients
with a small central VP gap seen in submucous
cleft palate or in patients with previously repaired
cleft palate who demonstrate a midline “trough”
or muscular diastasis.
33.8.4 Sphincteric Pharyngoplasty (SP)
of Orticochea ( 1970, 1990 )
No discussion of pharyngeal surgery could be
complete without mentioning the Orticochea
operation. Jackson ( 1985 ) described the proce-
dure most commonly used today. This surgical
procedure was designed to occlude the lateral
ports by changing the lower insertion of the
posterior pillars from the lateral walls to the
posterior wall of the pharynx. Orticochea
believed that this created a dynamic muscle
sphincter of the pharynx, which could open and
close dozens of times a minute. Furthermore,
this sphincter has the same cerebrocortical role
as the nasopharyngeal sphincter described by
Passavant because the palatopharyngeus and
superior constrictor muscles that are part of the
Passavant nasopharyngeal sphincter are used in
its constriction.
In patients with very short palates in whom
there is a greater risk of dehiscence, only one of
the palatopharyngeus muscles is transplanted.
Three months later, in a second operation, the
sphincter is completed by transplanting the
second palatopharyngeus muscle.
The sphincter is created by transposing the
posterior tonsillar pillars with the enclosed
palatopharyngeus muscles from the lateral
pharyngeal walls to the midsection of the poste-
rior pharyngeal wall. Three openings conduct air
between the oral and nasal pharynges. The cen-
tral opening’s shape and width remain unchanged
postoperatively, with no reduction of the lumen.
The two lateral openings completely disappear.
This sphincter tends to close off the nasopharynx.
Some surgeons use it as a secondary corrective
procedure.
Sphincter
palatoplasty’s
cited
advantages
include (1) dynamic sphincteric closure as a result
of retained neuromuscular innervation, (2) techni-
cal ease of execution, (3) a low complication rate,
(4) nonobstruction of the nasal airway, and (5) no
violation of the velum. Unfortunately, the question
of whether it attains superior speech results is still
open. It also must be emphasized that many sur-
geons have developed variants of the original pro-
cedure. The SP procedure has theoretic advantages
and is an effective means of management of VPI
for some patients. More studies need to be per-
formed to determine when this or the pharyngeal
fl ap should be the treatment of choice ( Witt and
D’Antonio 1993 ) .
33.8.5 Posterior Pharyngeal Wall
Augmentation
Te fl on and other materials have been implanted
in the posterior pharyngeal wall with no reported
long-term success. Potential problems include
738
S. Berkowitz
tissue incompatibility and migration of the
implant. Injectable forms of these substances also
can create problems with respect to embolism or
transport of the prosthetic matter to regional
lymph nodes.
Although pharyngeal fl ap surgery is the treat-
ment of choice in most cleft palate patients,
Denny et al. ( 1993 ) believe that a posterior pha-
ryngeal wall implant of costal cartilage should be
used in cases of extremely shallow pharyngeal
spaces with inadequate lateral pharyngeal wall
motion and when the side effects of a pharyngeal
fl ap should be avoided. Shprintzen et al. ( 1985 )
and Sher et al. ( 1986 ) believe that pharyngeal
fl aps should be avoided in patients with primary
neuromuscular disease or pharyngeal hypotonia.
Pharyngeal implants have been carried out
with a variety of materials by a number of sur-
geons over the years (e.g., Te fl on, by Smith and
McCabe ( 1977 ) ; Silastic by Blocksma ( 1963 ) ;
Silicone Gel by Brauer ( 1973 ) ; Proplast by
Wolford et al. ( 1989 ) ; and Homologous Cartilage
by Trigas et al. ( 1988 ) ).
Surgical procedures as well as injection tech-
niques have been used. Furlow et al. ( 1986 )
reported one instance of obstructive sleep apnea
(OSA) 6 years post-Te fl on injection due to the
downward displacement of the Te fl on particles
resulting in a narrowing of the patient’s airway.
In a series of studies, Vinas and Jager ( 1971 )
obtained the best results with surgically inserted
cartilage implants.
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741
S. Berkowitz (ed.), Cleft Lip and Palate,
DOI 10.1007/978-3-642-30770-6_34, © Springer-Verlag Berlin Heidelberg 2013
34
34.1
Introduction
Normal speech is dependent on the modulation
of voice and sound and the appropriate direction
of air fl ow from the lungs as it travels through the
vocal tract and the upper airway. One of the most
important components of speech is the ability to
separate the oral cavity from the nasal cavity, a
process that is unique to humans (Shprintzen
2003 ) . The majority of meaning in speech is
delivered by the production of consonants
(Shprintzen 2003 ) , and the all but three English
language consonants are produced orally with
essentially no nasal resonance. The only conso-
nant sounds that have signi fi cant nasal resonance
in English (and most other languages) are /m/, /n/,
and /ng/. All other consonant sounds require or
involve the separation of the oral cavity and nasal
cavity that is accomplished by the movements of
the velum and pharyngeal walls. This process is
referred to as velopharyngeal closure. Failure to
achieve velopharyngeal closure has been vari-
ously referred to as velopharyngeal insuf fi ciency
(VPI), velopharyngeal incompetence, velopha-
ryngeal inadequacy, and velopharyngeal dysfunc-
tion (VPD). Arguments over the validity of any
or all of these terms are not really important, and
for the purpose of this chapter, we will simply
refer
to
the
problem
as
velopharyngeal
insuf fi ciency (VPI), the most widely used and
accepted term historically.
34.2
Normal Velopharyngeal
Closure
The opening between the oral cavity and nasal
cavity is demarcated anteriorly by the velum,
posteriorly by the posterior pharyngeal wall, and
laterally by the lateral pharyngeal walls. These
muscular structures are multifunctional in that
they are normally active during swallowing,
speech, and other activities related to airway
maintenance. The passageway connecting the
oropharynx and nasopharynx, sometimes referred
to as a “port” or “portal,” is really a tube oriented
in a relatively vertical manner (Fig. 34.1 )
(Shprintzen 1995 ). The opening and closing of
this passageway between the oropharynx and
nasopharynx actually represents a valve or valv-
ing mechanism, similar to other valves in the
body that regulate the fl ow of air, blood, or other
fl uids by modulating the size of the opening. The
The Velopharyngeal Mechanism
Robert J. Shprintzen
R. J. Shprintzen , Ph.D.
President and Chairman of the Board ,
The Virtual Center for Velo-Cardio-Facial
Syndrome, Inc., NY , USA
e-mail: robert.shprintzen@vcfscenter.com
742
R.J. Shprintzen
movements for speech in the velopharyngeal
mechanism are controlled by the motor cortex
and are learned, just as the movements of the
tongue, lips, and teeth are learned for speech. The
movements of the same structures during swal-
lowing are primarily re fl exive and mediated by
the brainstem. Velopharyngeal movements for
swallowing are unrelated to those of speech, and
the muscles used and timing of movements are
different (Shprintzen et al. 1974 ) . For the velopha-
ryngeal valve to close during speech, there needs
to be movement from the velum and the pharynx
along three planes: the anteroposterior plane, the
medial plane, and the vertical plane (Fig. 34.2 ).
Anteroposterior movement is caused by the con-
traction of the levator veli palatini muscle which
acts like a sling to draw the velum back toward
the posterior pharyngeal wall. Depending on the
individual craniofacial structure of the patient,
that movement can be primarily posterior, pri-
marily superior, or posterior-superior (Fig. 34.3 ).
The medial movement of the pharyngeal walls is
caused by contraction of the uppermost fi bers of
the superior constrictor pharyngeus. Lateral pha-
ryngeal wall motion is highly variable ranging
from barely detectable motion to lateral pharyn-
geal walls that approximate in the midline of the
pharynx. Anterior motion of the posterior pha-
ryngeal wall, usually referred to as Passavant’s
ridge, is also caused by contraction of the superior
Fig. 34.1 Two views of the anatomy of the velopharyn-
geal valve. At left is an axial view from above showing
what looks like a two-dimensional opening, but when the
same specimen is viewed from a different angle (from the
right posterolateral aspect of the pharynx), the vertical
height of the velopharyngeal mechanism can be appreci-
ated. Therefore, the velopharyngeal valve is a three-
dimensional tube with width, breadth, and height
Fig. 34.2 Drawing showing the planes of movement
necessary for velopharyngeal closure
743
34 The Velopharyngeal Mechanism
constrictor and can be variable in shape, size, and
position (Fig. 34.4 ).
The variability of movement in the velopha-
ryngeal valve from person to person was fi rst
described in a landmark article by Skolnick and
colleagues ( 1973 ) . They showed that some indi-
viduals have more lateral pharyngeal wall motion
than others, some have Passavant’s ridge and
some do not, and that this variability of move-
ment is true for both normals and people with
VPI. Subsequent research in large samples of
both normals and people with VPI con fi rmed this
fi nding (Croft et al. 1981b ) . This variability may
be related in part to structural differences from
person to person, but learnng is also clearly a fac-
tor. The role of learning has been demonstrated
experimentally by using visual nasopharyngo-
scopic biofeedback to alter patterns of movement
(Siegel-Sadewitz and Shprintzen 1982 ) , therefore
indicating that it can be altered by learning new
muscular movements.
Therefore, velopharyngeal closure is a volun-
tary, CNS-mediated motor activity that is learned
during early speech acquisition. The role of diag-
nosis needs to be aimed at determining if a failure
of the velopharyngeal mechanism to close com-
pletely during normally nonnasal speech is an
anatomic or physiologic limitation versus an
error in learning.
34.3
Failures of Velopharyngeal
Valving
There are a substantial number of reasons why
velopharyngeal closure might not occur during
speech that normally requires it. VPI is usually
associated with palatal anomalies, but the fre-
quency of VPI in disorders unrelated to structural
malformations of the palate is also very common.
There are no de fi nitive data available that help us
to determine what the most common cause of
VPI might be, but suf fi ce it to say that it is a com-
mon disorder. For the purposes of this text, we
will focus primarily on clefting anomalies, but
the reader should be aware that problems with the
velopharyngeal valve associated with other disor-
ders require careful scrutiny, as well.
Disorders of velopharyngeal function might best
be divided into three major categories: structural,
Rest
Speech
Fig. 34.3 Three lateral view video fl uoroscopies showing the different types of movement of the velum that can occur
depending on the orientation of the levator palatini
744
R.J. Shprintzen
neurogenic/myopathic, and learned. These cate-
gories are not mutually exclusive. In other words,
it is typical to have only one dysfunction causing
the problem, but it might also be possible to have
two or three of these factors in operation
simultaneously.
34.4
Disorders of Structure
Structural anomalies include clefting of the pal-
ate (in all of its variations), anomalies of symme-
try of the palate and/or pharynx, and anomalies
of the surrounding pharynx (including abnormal-
ities of the lymphoid tissue). Cleft palate is a
broad designation for a broad spectrum of anom-
alies that range from complete clefts of the lip
and palate to nearly an undetectable malforma-
tion known as occult submucous cleft palate.
However, all of these anomalies could result in
the same disorder of VPI.
34.4.1 Overt Clefts
Overt clefts of the palate are the most easily recog-
nizable form of palatal anomaly and may range
from complete bilateral clefts of the hard and soft
palate to a cleft of the posterior aspect of the soft
palate (Fig. 34.5 ). The extent of the cleft varies in
both syndromic and nonsyndromic cases. The fre-
quency of velopharyngeal insuf fi ciency does not
really vary according to cleft type, but cleft type
does vary according to the primary diagnosis. In
nonsyndromic clefts where there are no associated
anomalies to affect velopharyngeal closure, the
frequency of VPI after repair is probably primarily
related to a combination of surgical variables and
the amount of muscle de fi ciency associated with
the cleft. However, in syndromic cases that account
for more than half of all clefts (Shprintzen et al.
1985a, b ) , other syndromic features can contribute
to VPI. For example, in velo-cardio-facial syn-
drome, besides cleft palate, there are three other
Rest
Speech
Fig. 34.4 Several different examples of Passavant’s ridge ( arrow )
745
34 The Velopharyngeal Mechanism
clinical fi ndings that contribute to VPI, none of
which are mutually exclusive. Individuals with
VCFS are known to have a high frequency of pha-
ryngeal hypotonia (Zim et al. 2003 ) , absent or
hypoplastic adenoid tissue (Williams et al. 1987 ) ,
and platybasia (Arvystas and Shprintzen 1984 )
that can increase pharyngeal depth. Therefore, pri-
mary cleft repair in VCFS almost always fails to
resolve VPI (Shprintzen 2001 ) .
Surgical repair of the palate is important for
only one reason, to provide the patient with the
possibility of developing normal speech. Feeding,
ear infection, growth, etc.—none of these are fac-
tors of importance to consider in relation to the
need for palatoplasty. Babies with clefts feed
quite successfully when proper technique is used,
as will be discussed brie fl y later. In fact, because
palate repair is not usually completed until
approximately 1 year of age, babies must feed
over that period of time with a cleft. Middle ear
disease is common in babies with clefts but is
related to malformation of the Eustachian tube
rather than muscular action of the velum
(Shprintzen and Croft 1981 ) . Middle ear disease
can also be successfully managed medically or
with myringotomy and tubes. Although feeding
and hearing can be normal with an unrepaired
cleft palate, speech cannot. Achieving acousti-
cally acceptable speech is dependent on normal
resonance and the ability to produce pressure
consonants orally, both highly dependent on
velopharyngeal closure.
Surgical repair of clefts are highly successful
when they are accomplished prior to 18 months of
age, and most surgeons in the USA are timing pri-
mary palatoplasty at or before the fi rst birthday in
Fig. 34.5 Four different types of overt palatal clefts,
including bilateral complete cleft of the hard and soft pal-
ate ( top left ), unilateral cleft of the hard and soft palate
( top right ), cleft of the entire soft palate ( bottom left ), and
a cleft of the posterior third of the soft palate ( bottom
right )
746
R.J. Shprintzen
order to intercept the early phases of speech devel-
opment. It is clear that the majority of children who
are born with overt clefts do not have VPI following
primary palatoplasty (Salyer et al. 2006 ) . Although
surgical approaches to cleft repair have varied in
relation to technique and timing, the majority of
experienced surgeons who complete primary palato-
plasties prior to 18 months of age achieve between
80 and 90 % positive outcomes de fi ned as an absence
of VPI after palatoplasty. Although this leaves a
core of patients who will require secondary surgical
procedures at a later date to resolve VPI, the overall
percentage of individuals with VPI who have
repaired clefts is probably quite low in most treat-
ment centers.
The logical conclusion regarding the successful
outcome of palate repair is that it is highly depen-
dent on surgical technique. Actually, this has never
really been proven, and there are many factors
involved, including the age at time of surgery, the
type of operation, other anatomical variations in
the pharynx, muscle tone and developmental fac-
tors, and one factor that has received very little
attention in the literature, the surgeon and his or her
skill level. It is not within the scope of this chapter
to discuss each of these issues, but it is important to
show that the successful outcome of palate repair is
dependent on more than one factor.
Although the palate is an important component
of velopharyngeal valving, it is not the only one.
The palate, and the velum in particular, is simply
one component of the velopharyngeal mechanism,
and there is quite a bit of variability of the size and
shape of the other structures within the pharynx.
The height and width of the pharynx varies from
person to person just as height, weight, and head
circumference do. The size of the adenoid is of
particular importance because the adenoid sits in
the plane of velopharyngeal closure in young chil-
dren. In fact, velopharyngeal closure in children
until at least 6 years of age is actually velar-ade-
noidal (Williams et al. 1987 ) (Fig. 34.6 ). Adenoids
vary considerably in size, and there is some evi-
dence that suggests that adenoid size may be the
most important factor for successfully achieving
velopharyngeal closure (Havkin et al. 2000 ) . The
larger the adenoid, the less movement is required
of the velum. Although the adenoids are impor-
tant to normal speech production, the tonsils are
not and will be considered separately later in this
chapter. It is clear that adenoidectomy should be
avoided (unless there is severe upper airway
obstruction in which case partial adenoidectomy
may be indicated) in individuals with cleft palate
or submucous cleft palate because of the potential
for the development of the VPI once the adenoid
Rest
Speech
Fig. 34.6 Lateral view video fl uoroscopy in a normal 4-year-old child demonstrating that the velum ( v ) never contacts
the posterior pharyngeal wall during speech but instead approximates to the adenoid ( a )
747
34 The Velopharyngeal Mechanism
tissue has been removed having the effect of
increasing the volume of the nasopharynx (Croft
et al. 1981a, b ; Havkin et al. 2000 ) .
Another important factor is the volume of the
nasopharynx. An increased volume of the nasophar-
ynx can be caused by an abnormally obtuse angu-
lation of the skull base (Shprintzen 2001 ) . Many
clinicians who use lateral view radiographs of the
head and neck may interpret an increased nasopha-
ryngeal volume as a “deep pharynx.” Alternatively,
because the palate appears short within the presence
of an increased pharyngeal volume, some clinicians
may interpret this as a “congenitally short palate.” As
will be discussed later, structural integrity of the palate
cannot be assessed without a direct endoscopic view
of the nasal surface of the velum. However, the depth
of the nasopharynx can be assessed radiographically
using the standardized measurements provided by a
lateral cephalograph. Neither procedure (endoscopy
or cephalometrics) is individually perfect for describ-
ing the anatomy of the pharynx. Rather, the informa-
tion obtained from each is complementary.
34.4.2 Submucous Cleft Palate
Submucous cleft palate has traditionally been
de fi ned as the triad of bi fi d uvula, notching of the
posterior border of the hard palate, and a visible dia-
stasis of the musculature in the soft palate referred
to as a zona pellucida (Fig. 34.7 ) (Calnan 1954 ) .
However, although all cases with these clinical
fi ndings have submucous cleft palate, not all people
with submucous cleft palate have all three of these
fi ndings. Some have two of these fi ndings, usually a
bi fi d uvula and hard palate notch, some have only
one of these fi ndings, almost always a bi fi d uvula,
and more interestingly, some have none of these
fi ndings. Submucous cleft palate is simply a milder
or less obvious expression of cleft palate. In this
case, the skin, or mucous membrane, is intact but
the underlying musculature is not and some muscle
may be congenitally absent (Croft et al. 1978 ;
Lewin et al. 1980 ) . Of interest, although people
with submucous cleft palate do have velopharyn-
geal insuf fi ciency and hypernasal speech, the large
majority do not (Shprintzen et al. 1985a, b ) . This is
interesting in light of the fact that many surgeons
perform palatoplasty in infancy, before the onset of
speech in an attempt to avoid speech problems by
rearranging the muscles to a more normal
con fi guration in the palate for individuals with sub-
mucous cleft palate even though the large majority
of people with submucous clefts have normal
speech without such surgery. This may indicate that
other factors besides muscle integrity play a large or
even larger role in normal speech production than
simply the degree of palatal normality.
34.4.3 Occult Submucous Cleft Palate
In 1975, Kaplan reported an abnormality of the
palate he labeled the occult submucous cleft palate
Fig. 34.7 Obvious
submucous cleft palate
showing a bi fi d uvula ( dual
arrow ), a notch in the
posterior border of the hard
palate (*) and zona pellucida
( single arrow )
748
R.J. Shprintzen
(Kaplan 1975 ) . Kaplan’s report described four indi-
viduals with VPI who had normal appearing pal-
ates on oral examination, without evidence of bi fi d
uvula, a zona pellucida, or notching in the hard pal-
ate. At surgery for correction of their VPI, he dis-
sected the palate and studied the musculature noting
that the muscle fi bers were abnormal in position
and orientation as might be found in a cleft palate or
submucous cleft palate, hence the label occult sub-
mucous cleft palate (occult meaning mysterious).
He concluded that the only way one could detect
this anomaly was by surgical dissection. However,
subsequent publications have demonstrated that
occult submucous cleft palate can be detected by
nasopharyngoscopy (Croft et al. 1978 ; Lewin et al.
1980 ; Shprintzen 1979a, b, 2000 ) . Unfortunately,
many clinicians and researchers have not applied
this diagnostic approach so that many cases of occult
submucous cleft go undetected and the frequency
of clefting becomes signi fi cantly underestimated. It
is interesting to note in retrospect that three of the
four patients shown by Kaplan clearly have velo-
cardio-facial syndrome, a condition known to have
a high frequency of occult submucous cleft palate
(Shprintzen 1982 ) . It is not known what the fre-
quency of hypernasal speech is in association with
occult submucous cleft palate because it is very
unlikely that individuals with normal speech will
ever have the anomaly detected unless nasopharyn-
goscopy is performed for some other reason.
34.5
Normal Anatomy of the
Velopharyngeal Mechanism:
Developmental Changes
The structures of the palate, nasopharynx, and
oropharynx (the structures that constitute the
velopharyngeal mechanism) are not static. They
undergo signi fi cant alteration and remodeling over
time relative to their functions and age. Therefore,
mechanisms of velopharyngeal function at one point
of time in life may not be the same as another. This
means that broad generalizations about how the pal-
ate and pharynx function should be avoided because
they may be age dependent. As mentioned previ-
ously, the palate and pharynx are multifunctional.
Modulating the size and shape of the upper airway,
sometimes referred to as the aerodigestive tract, is
necessary for proper function during speech, feed-
ing, and airway maintenance. However, these func-
tions vary with age. Infants have the ability to
separate the oropharyngeal airway from the nasopha-
ryngeal airway making it easier for them to breathe
through their noses while they eat than is true for
adults. This is because the upper airway in infants is
vertically shorter and oriented in a more horizontal
than vertical angle (Shprintzen 2003 ) (Fig. 34.8 ).
The shorter upper airway in infants allows the epi-
glottis to be in close proximity to the soft palate cre-
ating a conduit for milk to fl ow unobstructed into the
INFANT
ADULT
Nasopharynx
Oropharynx
Vertical
orientation
Nasopharynx
Oropharynx
Horizontal
orientation
Fig. 34.8 Comparison of the upper airway in an infant ( left ) to that of an adult ( right ) showing a more vertical orienta-
tion of the pharynx in the adult compared to the more horizontal position in the infant
749
34 The Velopharyngeal Mechanism
pyriform sinuses laterally while the baby breathes
through the nose. Because infants’ ventilatory efforts
are faster and more frequent than adults and their
smaller lung capacity and blood volume makes them
more prone to rapid oxygen desaturation than adults,
it is important for them to breathe uninterrupted dur-
ing feeding to prevent hypoxia and hypercarbia.
Babies typically eat semi-reclined, a position that
actually makes the upper aerodigestive tract more
vertical so that feeding is facilitated by gravity and
an easy fl ow of fl uids into the hypopharynx and
esophagus.
As children grow, growth in the maxilla and
mandible is both horizontal and vertical. This
expands the upper airway vertically and draws
the palate and velum away from the epiglottis and
larynx. The entire pharynx expands in three
dimensions (width, depth, and height) as the
entire palate grows in length. In fact, if the upper
airway did not increase in height, the palate
would hang too deeply into the pharynx resulting
in upper airway obstruction. In fact, in some
genetic disorders with midfacial anomalies, such
as Crouzon syndrome, Apert syndrome, and
Down syndrome, where the maxilla is hypoplas-
tic in all dimensions, the phenomenon of the
velum contributing to upper airway obstruction is
commonplace, resulting in obstructive sleep
apnea as well as obstructive daytime respiration.
As the pharynx becomes more vertical with
age, the adenoids begin to involute. As reported
previously, if the adenoid did not involute, the
pharynx would become obstructed because of its
change in shape and orientation (Shprintzen
2003 ) . This is because the hard and soft palates
actually migrate to a position relatively closer to
the posterior pharyngeal wall over time. If the
adenoid remained large (typically occupying
about 25–50 % of the postnasal space before
involution begins between 8 and 10 years of age),
the palate would be fl ush against it, thereby com-
promising the nasopharyngeal airway.
The changes in the shape, volume, and posi-
tion of the upper airway have been speculated to
potentially result in changes in velopharyngeal
function over time. Clinically, I have often heard
professionals suggest that gradual adenoid invo-
lution can result in the development of velopha-
ryngeal insuf fi ciency. Although there are a very
small number of anecdotal cases reported with a
late onset of velopharyngeal insuf fi ciency (Mason
and Warren 1980 ) , such events are very rare, and
I have never encountered such a case in more
than 38 years of practice with the exception of
late onset muscular dystrophies, myasthenia
gravis, or other neuromuscular diseases.
34.6
Other Structural Disorders
Although the adenoid is important to normal
speech production, other lymphoid tissues do
not play a role and may be detrimental to nor-
mal speech production. There are three other
masses of lymphoid tissue within the oral and
pharyngeal airway: two palatine tonsils and one
lingual tonsil. The lingual tonsil sits at the base
of the tongue anterior to the vallecula and plays
no role whatsoever in speech production. When
enlarged, the lingual tonsil can cause the sen-
sation of globus, or a feeling that something is
stuck in the bottom of the throat. If very large,
a muf fl ed resonance may also result from an
enlarged lingual tonsil because it may physi-
cally damp the sound waves produced by the
vocal cords just millimeters below the base of
the tongue. However, the lingual tonsil does not
cause hypernasality or hyponasality and is too far
away from the velopharyngeal valve to interfere
with it.The palatine tonsils, however, may alter
nasal resonance patterns as has been documented
in the medical literature on several occasions
(Henningsson and Isberg 1988 ; MacKenzie-
Stepner et al. 1987 ; Shprintzen et al. 1987 ) .
The palatine tonsils are situated in the poste-
rior oral cavity with their attachment in the fau-
cial arch in between the anterior and posterior
tonsillar pillar. Assessment of tonsillar size is
often relegated to oral assessment with a fl ashlight
and tongue blade. It is typical for clinicians to use
a rating scale between 0 and 4+ based on the
degree of medial excursion of the tonsils toward
the midline of the posterior oral cavity with 0
implying that the tonsils are not visible, 1+ refer-
ring to tonsils fully contained within the faucial
pillars, 2+ meaning the tonsils extrude medially
beyond the pillars but less than halfway to mid-
line, 3+ indicating that the tonsils extend more
than half of the way to midline, and 4+ meaning
they are touching in the midline (kissing tonsils).
750
R.J. Shprintzen
The problem with this scale is that no one has told
the tonsils that if they get big that they must grow
medially. In fact, in many cases, they grow back
into the pharyngeal airway and may grow down
into the hypopharynx or up into the nasopharynx
(Fig. 34.9 ). If the tonsils extrude into the pharynx,
they may interfere with velar elevation or lateral
pharyngeal wall motion, resulting in VPI
(MacKenzie-Stepner et al. 1987 ; Shprintzen et al.
1987 ) . In patients with cleft palate, this may pres-
ent as a particularly dif fi cult diagnostic dilemma
and potentially dangerous circumstance. Because
VPI is relatively common in children with cleft
palate, the presence of hypernasality or audible
nasal air escape during speech may lead clini-
cians to conclude that the problem is an anatomic
problem of the palate, and therefore a recommen-
dation for pharyngeal fl ap or other surgery may
be made. However, if the tonsils are large and
contributing or causing the problem, performing
surgery in the pharynx may be unnecessary, but
even more important, may prompt the develop-
ment of obstructive sleep apnea. Tonsils have
been shown to be a primary factor in inducing
obstructive apnea following pharyngeal fl ap sur-
gery (Shprintzen et al. 1992a, b ) because they sit
beneath the fl ap and narrow the oropharyngeal
airway, while the fl ap compromises the nasal air-
way. It has been reported that if pharyngeal fl ap is
indicated, tonsillectomy or adenotonsillectomy
should be performed fi rst in order to avoid
obstructive sleep apnea as a complication (Chegar
et al. 2007 ; Shprintzen et al. 1992a, b ) . In some
cases, tonsillectomy may resolve the VPI if the
tonsils were the primary cause for limiting move-
ment in the velopharyngeal valve (Shprintzen
et al. 1987 ) .
34.7
Neurogenic and Myopathic
Disorders of Velopharyngeal
Function
VPI is a common disorder among individuals
with disorders of the central nervous system or
who have primary myopathies. Although the fre-
quency of VPI is high, the true prevalence is not
known because many of these individuals have
other severe disorders of speech, and the VPI is
simply a component of a broader spectrum of
speech abnormality that may be very severe. In
such cases, resolving VPI alone will do little or
nothing to improve the intelligibility of speech,
and therefore such individuals are not often
referred for diagnosis or treatment. Neurogenic
disorders that might frequently result in VPI are
strokes and other cerebrovascular accidents, open
and closed head trauma, cerebral palsy resulting
Epiglottis
Velum
Tonsil
Tonsil
PENTAX
COMMENT : airway
Posterior tonsillar pillar
NAME :
ID :
AGE : 5
SEX : M
06/24/04
15:14:36
Tonsil
Fig. 34.9 Hypertrophic tonsils seen endoscopically and on lateral view radiographs showing that they intrude posteri-
orly into the pharyngeal airway behind the faucial pillars
751
34 The Velopharyngeal Mechanism
from neonatal hypoxia or brain malformation,
infections such as encephalitis, and neurodegen-
erative diseases, such as myasthenia gravis.
Primary myopathies that have VPI as a common
fi nding include (but are not limited to) Steinert
syndrome (myotonic dystrophy), facioscapu-
lohumeral muscular dystrophy, nemaline myopa-
thy, and oculopharyngeal muscular dystrophy.
The management in myopathies is very different
than in cleft palate or other structural anomalies
of the head and neck. Surgical intervention is
often problematic in myopathies because of respi-
ratory concerns, and even the operation itself may
be compromised by severe adverse anesthesia
reactions, particularly the development of malig-
nant hyperpyrexia which can be a fatal complica-
tion (Shprintzen 1997 ) . Therefore, the diagnosis
of myopathic disorders and the recognition of
hypernasal speech as a potential clinical feature
are critical for proper clinical management.
34.8
Evaluation of Velopharyngeal
Insuf fi ciency
Two issues established in the previous pages point
out the need for detailed and comprehensive evalu-
ation of VPI. First, velopharyngeal valving is
highly variable from person to person, both in nor-
mals and in those with velopharyngeal insuf fi ciency.
Therefore, assumptions cannot be made about the
nature of velopharyngeal valving based on the
sound of speech or preconceived notions about
how the mechanism works for the “average” indi-
vidual. Second, VPI occurs in many different con-
ditions, and the reason for the VPI may not always
be obvious, including structural anomalies that
cannot be seen on oral clinical examination. The
diagnosis of more obscure yet common conditions
such as occult submucous cleft palate, tonsillar
hypertrophy, or myopathies is highly dependent
on a precise diagnostic approach.
In general, procedures used to assess velopha-
ryngeal insuf fi ciency can be divided into two
broad categories: direct and indirect. Direct pro-
cedures can be de fi ned as those that allow direct
visualization of the velopharyngeal valve during
the unimpeded fl ow of speech typical for the indi-
vidual while not interfering with the movements
of the valve. Indirect procedures can be de fi ned
simply as everything else. Indirect procedures
typically measure some type of artifact or by-
product of velopharyngeal valving rather than
assessing the act itself. Indirect procedures con-
sist of listener judgment of resonance and nasal
air fl ow, instrumental techniques for measuring
nasal resonance, and techniques for measuring
nasal air fl ow or pressure. Direct procedures
include both radiographic and endoscopic tech-
niques, although ultrasound and real time CT and
MRI have all been proposed to study VPI.
However, only fl uoroscopic and endoscopic pro-
cedures have stood the test of time and remained.
34.9
Indirect Assessments of VPI
Every diagnostic process starts with an assess-
ment of the way a patient sounds to the human
ear. Parents may perceive something is wrong
with the way the child’s speech sounds, or if the
child has a repaired cleft, a clinician schedules
periodic assessments to determine if speech will
develop normally. At some point, however, some-
one has to perceive abnormal hypernasality or
nasal air escape during speech. The listener must
determine if resonance is normal, hypernasal, or
hyponasal. Occasionally, there may be mixed
hyper-/hyponasality if there is VPI in association
with nasal blockage at some location anterior to
the velopharyngeal valve. In addition to reso-
nance, the listener must determine if there is an
abnormal escape of air during speech and, if so,
on what sounds and how often it is occurring. If
the speech pattern is deemed to be abnormal, then
the necessity for treatment is determined. In other
words, perceptual assessment becomes the entry
point for further evaluation and is therefore the
most critical portion of the assessment.
Once a patient’s speech has been determined to
show the effects of VPI, then the clinician must
choose what to do next. The clinician may choose to
con fi rm nasal air escape. This can be done in a num-
ber of ways from very simple to very complex.
752
R.J. Shprintzen
Simple procedures would include the use of a nasal
mirror, a listening tube or stethoscope, or devices
that might detect nasal air escape such as the
SeeScape®. All of these devices are used to con fi rm
the presence of abnormal nasal air escape during the
utterance of normally nonnasal sounds, words, or
phrases. They are “yes vs. no” devices rather than
quantitative instruments. Although sensitive to nasal
air escape, they are not speci fi c as to the source of
the air escape. In individuals with clefts, the air may
be leaking from a fi stula even if there is no VPI, thus
resulting in a false positive. False negatives are com-
mon. Nasal congestion or a deviated septum will
block air escape from the nostrils even if VPI is
present resulting in a false negative. Therefore, pro-
cedures for detecting nasal air escape may be decent
“quick and dirty” techniques for identifying nasal
air escape, but they are not really diagnostic for VPI
in many cases. Perhaps the easiest procedure for a
“quick and dirty” technique is to use nostril occlu-
sion during normally nonnasal speech. Occluding
the nostrils during a normally nonnasal speech sam-
ple should result in no change of resonance if
velopharyngeal closure is being achieved. If there is
VPI, then pinching the nostrils shut will result in a
cul-de-sac nasal resonance that will cause an imme-
diate change in resonance pattern. If nasal air escape
is audible during spontaneous speech, occluding the
nostrils will eliminate the nasal turbulence. Although
this procedure is not quantitative in any way, it is a
simple procedure for making a quick determination
of the potential presence of VPI.
There are several devices that have been
designed as quantitative. These include manome-
ters and rhinometers that measure pressure from
the mouth or nose, but these devices have been
proven to be irrelevant in relation to speech pro-
duction. Two procedures have garnered some
attention from their use in research and some clini-
cal settings: the nasometer and pressure- fl ow
instrument. The nasometer and pressure- fl ow
device both apply numbers to nasal air escape dur-
ing speech based on resonance (nasometer) or the
pressure of air coming from the nose and mouth
(pressure fl ow) during speech. The nasometer uses
microphones to compare resonance between the
nasal and oral cavities during speech, providing a
number (nasalance, a ratio of acoustic energy from
the nose compared to the mouth), while pressure-
fl ow instrumentation utilizes a mathematical com-
putation, the theoretic hydraulic principle, to
calculate an ori fi ce size in mm 2 . Although both of
these devices provide numerical data, one should
not equate a number with “objective” data. Albert
Einstein was reputed to have a sign hanging in his
of fi ce at Princeton that said, “Not everything that
counts can be counted, and not everything that can
be counted counts.” Further, relevant to the hydrau-
lic principle calculation, Einstein also said, “As far
as the laws of mathematics refer to reality, they are
not certain; and as far as they are certain, they do
not refer to reality” and “If the facts don’t fi t the
theory, change the facts.” In other words, validity
of a measure has more to do with its relevance than
anything else. The nasometer is a device for detect-
ing resonance patterns, but the ultimate tool for this
is the examiner’s ear. It is the perception of normal
versus abnormal that is important. If the ear per-
ceives speech as normal, it matters not what a
machine assigns as a numerical value. The second
and third quotes from Einstein reinforce a primary
problem with pressure- fl ow instrumentation. The
entire principle behind pressure fl ow is that the
opening detected in the velopharyngeal valve is
measured in mm 2 . As was emphasized earlier in
this chapter and as is shown in Fig. 34.1 , the
velopharyngeal valve is a three-dimensional struc-
ture, not two dimensional. A three-dimensional
object needs to be measured in mm 3 , not mm 2 . This
misinterpretation of velopharyngeal valving that
serves as the basis for pressure- fl ow studies ren-
ders its calculations as irrelevant. Some clinicians
use nasometers and similar instruments for bio-
feedback in order to obtain comparisons following
treatment or during the application of some forms
of speech therapy, but de fi nitive data in terms of
effectiveness of this approach is not available.
34.10 Direct Assessments
34.10.1 Multi-view Video fl uoroscopy
It is interesting to note that the two gold standards
for direct assessment of velopharyngeal valving