Cleft Palate Development & Anatomy
You have been asked to give an in-service to a group of students who wish to eventually specialize in service delivery to children with cleft palates and their families. The students want to know detailed information about in utero development of the hard and soft palate (among other things). You can accurately tell them that in utero, the hard palate fuses between the developmental ages of
A. 1 and 2 weeks.
B. 5 and 6 weeks.
C. 8 and 9 weeks.
D. 10 and 12 weeks.
C. In utero, the hard palate fuses between the developmental ages of 8- and 9-weeks' gestation.
A clinician in a private practice is approached by the parents of Tommy D, a 5-year-old boy. The parents want to place Tommy in kindergarten in the fall but say, "We know there's something wrong with him—we're just not sure what." According to Tommy's parents, he is a "sweet, lovable boy who will go to anybody. He likes to sing a lot, too." Because the parents live in a rural area, health care access has been limited. After seeing Tommy for the first time, the clinician refers his parents to a neurologist because she suspects that Tommy has a syndrome. He is small for his age and has an elfin-like appearance characterized by a small chin, turned-up nose, puffiness around the eyes, a long upper lip, and a wide mouth. His teeth are small and widely spaced.
The clinician suspects that Tommy has
A. Williams syndrome.
B. Apert syndrome.
C. Moebius syndrome.
D. Turner syndrome.
A. Because Tommy is small for his age and has an elfin-like appearance characterized by a small chin, turned-up nose, puffiness around the eyes, a long upper lip, and a wide mouth with small and widely spaced teeth, he probably has Williams syndrome.
The surgical method of cleft palate repair that involves raising two bipedicled flaps of mucoperiosteum, bringing them together, and attaching them to close the cleft is called the
A. von Langenbeck surgical method.
B. V-Y retroposition.
C. Veau-Wardill-Kilner method.
D. pharyngeal flap procedure.
A. The surgical method of cleft palate repair that involves raising two bipedicled flaps of mucoperiosteum, bringing them together, and attaching them to close the cleft is called the von Langenbeck surgical method.
A child transfers from Apple City to Middleton City, and his file indicates that he has been receiving speech-language services in Apple City. To the chagrin of the Middleton City clinician, some pages of the report from the SLP in Apple City are missing. However, on the first page, it is indicated that this child has Moebius syndrome. He also has a history of frequent hospitalizations. The Middleton City clinician can probably expect to find that this child
A. has low muscle tone, a history of early feeding difficulties, initial failure to thrive, obesity after the first year, and underdeveloped genitals.
B. has underdeveloped facial bones, including mandibular hypoplasia, malar hypoplasia, dental malocclusion, and downwardly slanted palpebral fissures.
C. has a small maxillary structure, spheno-ethmoidal synchondroses, ocular hypertelorism, facial asymmetry including a tall forehead, and brachycephaly.
D. may have delayed language and an articulation disorder, as well as bilabial paresis and weak tongue control for lateralization, elevation, depression, and protrusion; a masklike face; a history of feeding problems in infancy; and unilateral or bilateral paralysis of the abductors of the eye.
D. A child with Moebius syndrome can be expected to have possible delayed language and an articulation disorder, as well as bilabial paresis and weak tongue control for lateralization, elevation, depression, and protrusion; a masklike face; a history of feeding problems in infancy; and unilateral or bilateral paralysis of the abductors of the eye.
In infants and children with cleft palates, Eustachian tube dysfunction is probably mostly related to the lack of contraction of the
A. levator veli palatini muscle.
B. veli palatini muscle.
C. tensor veli palatini muscle.
D. palatopharyngeus muscle.
C. In infants and children with cleft palates, Eustachian tube dysfunction is mostly related to the lack of contraction of the tensor veli palatini muscle.
A condition in which the surface tissues of the soft or hard palate fuse but the underlying muscle or bone tissues do not is called
A. fusion disorder.
B. submucous or occult cleft palate.
C. class III palatal cleft.
D. submucosal cleft class IV.
B. A condition in which the surface tissues of the soft or hard palate fuse but the underlying muscle or bone tissues do not is called submucous or occult cleft palate.
This syndrome is caused by a rare genetic disorder that affects an estimated 1 out of every 20,000 babies. It is caused by
A. a missing part of chromosome 22, known as 22q11.
B. an expanded number of cytosine-guanine-guanine (CGG) nucleic acid repeats on a specific gene on one of the distal ends of the X chromosome.
C. a spontaneous autosomal dominant mutation, whose gene and locus is FGFR2 at 10q25-26.
D. the deletion of approximately 25 genes on one copy of chromosome 7q11.23.
D. Williams syndrome is caused by the deletion of approximately 25 genes on one copy of chromosome 7q11.23.
A school-based clinician, Joe, is assessing the velopharyngeal adequacy of Janie K., a 17-year-old immigrant high school student from the Philippines. Janie was born with a cleft of the palate and lip; there was no repair until her family came to the United States when Janie was 16 years old. In the Philippines, Janie and her family lived on Tablas, a small and rural island, where surgery was unavailable. Though the repair surgery in the United States a year ago was successful and Janie now has a more aesthetically pleasing appearance and better speech, there is still audible nasal emission and hypernasality when she speaks. Joe plans to refer her to a local craniofacial team, but he also wants to conduct as thorough an examination as he can despite the lack of instrumentation available at his school site. He does have access to an oral manometer. He uses this to provide a beginning point from which to refer Janie to the craniofacial team. After obtaining a ratio by comparing pressures achieved in the nostrils-occluded and the nostrils-open conditions, the clinician concludes that Janie especially needs to be referred to the craniofacial team for possible further surgery or a pharyngeal flap. When he used oral manometry, the clinician probably found that Janie
A. had a ratio of 1.0.
B. had a ratio of 1.2.
C. had a ratio of .96.
D. had a ratio of .87.
D. When the clinician did oral manometric testing, he probably found that Janie had a ratio of .87. Anything below .89 is especially indicative of problems.
You have received a referral of Akhtar, a 4-year-old refugee child from a Dari-speaking family from Afghanistan. Akhtar has never been evaluated or received speech-language services. Before you meet Akhtar, you speak with the doctor, who explains that Akhtar has midface dysplasia, a high and narrow arched palate, a relatively large and fissured tongue that protrudes, hearing loss, and speech sound disorder. Akhtar also has generalized hypotonia and brachycephaly (small head). You can confidently conclude that Akhtar has
A. Crouzon syndrome.
B. Cri du chat syndrome.
C. Down syndrome.
D. Hurler's syndrome.
C. Akhtar has Down syndrome, which is characterized by generalized hypotonia, brachycephaly, midface dysplasia, a high and narrow arched palate, and a relatively large and fissured tongue that tends to protrude. Persons with Down syndrome may also have hearing loss and speech sound disorder.
You are on a cleft palate and craniofacial anomalies team that evaluates children with cleft palates. When a child has a resonance problem, the team decides whether the child will benefit from speech therapy alone or more intensive medical intervention (e.g., surgery) is needed. As part of the assessment process, cephalometric analysis is used. In cephalometric analysis, the Cephalometric Assessment of Velopharyngeal Structures computer program analyzes the ratio relationship between the length of the soft palate and the depth of the nasopharynx. Which one of the following statements best summarizes a key principle that guides treatment decisions?
A. A ratio of less than 60 is usually found when the soft palate is too short and the nasopharynx is too shallow.
B. A ratio of less than 60 is usually found when the nasopharynx is too deep or the soft palate is too long.
C. A ratio of 60-80 generally indicates adequate tissue for velopharyngeal closure for speech.
D. If a ratio is higher than 80, this means the nasopharynx is too shallow or the velum is too long.
C. A ratio of 60-80 generally indicates adequate tissue for velopharyngeal closure for speech.
Though cleft palate is often caused by genetic factors, it can also be related to mechanical factors. Which one of these is NOT a mechanical factor related to cleft palate?
A. Intrauterine crowding
B. Twinning
C. Uterine tumor
D. Drug use by the birth mother
D. Drug use by the birth mother is not a mechanical factor related to clefting.
The clinician knows that she will probably end up seeing Tommy for intervention if his parents are able to bring him on a weekly basis. She will probably be working on which of the following goals?
A. Pragmatics, to increase Tommy's ability to interact with others
B. Oral-motor coordination, because children with this syndrome usually have oral-motor coordination problems, which contribute to decreased intelligibility
C. Overall expressive and receptive language, because children with this syndrome generally have IQs of 50-70 (although some have good language skills)
D. Morphological skills, because although children with this syndrome usually have above average IQs, they frequently delete bound morphemes from the beginnings and ends of words
C. The clinician will probably work on increasing overall expressive and receptive language, because children with Williams syndrome generally have IQs between 50 and 70 (although some of these children have good language skills).
You are serving a child with a repaired cleft palate. Marissa is 11 and still somewhat unintelligible, manifesting difficulty with some speech sounds. To help her, you employ a specialized procedure. In this procedure, an orthodontist will design an artificial palate containing 62 embedded electrodes connected to a computer. This will be fitted into Marissa's mouth, and when her tongue contacts the electrodes during speech production, articulatory patterns can be seen on the computer screen. This procedure is called
A. electropalatography.
B. electroglottography.
C. palatomyography.
D. myomanometry.
A. This procedure is called electropalatography.
You are providing support for a man with Marfan syndrome; he has difficulties with respiration, and you are working on breathing techniques. He shares with you that he and his wife want to have a child, and they are concerned about their child possibly having Marfan syndrome. You refer him to a genetic counselor, who will share with him that Marfan syndrome is
A. an autosomal dominant inherited disorder caused by mutations in the FBN1 gene.
B. an autosomal recessive disorder caused by mutations in the FBN1 gene.
C. a partial deletion syndrome caused by mutations in the FBN2 gene.
D. not genetically transferred to offspring, so the parents do not need to be concerned.
A. Marfan syndrome is an autosomal dominant inherited disorder caused by mutations in the FBN1 gene.
In your private practice, a father brings his 5-year-old son, Jordan, to see you. The kindergarten teacher has noticed that Jordan "talks kind of funny," and sometimes other children make fun of him. Jordan was born with a cleft palate and has had repair surgery, and he has a history of conductive hearing loss secondary to otitis media with effusion. During your evaluation of Jordan's speech, you note that he is especially having difficulty producing affricates, fricatives, and plosives. This is probably because he is having continued difficulty with
A. vocal nodules.
B. hypertrophied adenoids.
C. velopharyngeal closure.
D. maxillary deficiency.
C. If a child with a repaired cleft palate specifically has continued difficulty with fricatives, affricates, and plosives, this generally indicates continued inadequate velopharyngeal closure.
Children with bilateral cleft lip or palate may have problems with their teeth. A common problem for these children is
A. supernumerary teeth.
B. teeth erupting out of the hard palate.
C. hypodontia or missing teeth.
D. teeth that are too small.
C. Children with bilateral clefts of the palate and lip commonly have hypodontia, or missing teeth.
A child comes to a clinic with her mother for articulation therapy. The mother tells the clinician that her daughter has Hurler's syndrome. Hurler's syndrome is caused by
A. autosomal recessive deficiency of α-L-iduronidase.
B. a spontaneous autosomal dominant mutation of FGFR2 at 10q25-26.
C. autosomal dominant inheritance and deletion in the region of the long arm of chromosome 15 (15q11-15q13).
D. an expanded number of cytosine-guanine-guanine (CGG) nucleic acid repeats on a specific gene on one of the distal ends of the Y chromosome.
A. Hurler's syndrome is caused by autosomal recessive deficiency of α-L-iduronidase.
A clinician is a member of a cleft palate team that asks him to conduct an objective assessment of an 8-year-old child's velopharyngeal mechanism. The clinician decides to do nasopharyngoscopy, in which a nasopharyngoscope is passed through the middle meatus and back to the area of velopharyngeal closure. This will enable the clinician to observe the child's
A. adenoid pads and anterior pharyngeal walls as the child prolongs /s/.
B. posterior and lateral pharyngeal walls, as well as the adenoid pad, as the child sustains /a/.
C. nasal aspect of the velum and the adenoid pad as the child produces CVC words.
D. posterior and lateral pharyngeal walls, as well as the nasal aspect of the velum and the adenoid pad, as the child produces sentences.
D. Nasopharyngoscopy enables the clinician to observe the posterior and lateral pharyngeal walls, as well as the nasal aspect of the velum and the adenoid pad, as the child produces sentences.
As part of your job in a public school district, you serve several preschools. You get an urgent call from a parent at one of the schools. She states that her daughter Michelle (age 4), who previously had typical language skills, has suddenly "lost her words" and is having seizures. She shares that Michelle "is so much more hyper now, and we don't know what's wrong." The mom shares that they have an appointment with Michelle's pediatrician to find out more about what is happening. You support this decision, especially because you suspect that Michelle has
A. Williams syndrome.
B. Landau-Kleffner syndrome.
C. Treacher Collins syndrome.
D. Pierre-Robin syndrome.
B. In Landau-Kleffner syndrome, children may experience sudden loss of language, seizures, and hyperactivity.
You are serving in a juvenile detention facility and receive a referral of Max, a 16-year-old with documented fragile X syndrome. Which of the following will you expect to find when you evaluate Max?
A. Attention deficits, a large tongue, sleep disturbances, and a high, protruding forehead.
B. Speech sound disorders, normal intelligence, expressive language delays, and micrognathia.
C. Hunched back, tracheal stenosis, brachycephaly, and hypertonia.
D. Intellectual disability and delays in pragmatic, semantic, phonologic, and syntactic aspects of language, with syntax being especially affected.
D. Teen boys with fragile X syndrome commonly show intellectual disability and delays in pragmatic, semantic, phonologic, and syntactic aspects of language, with syntax being especially affected.
You work in a high school that offers family life education classes for all its students. You have been asked to talk with the female students about the importance of abstaining from drugs and alcohol during pregnancy because of potential threats to the fetus's well-being. One fact you will be sure to emphasize, relevant to preventing cleft palate, is that
A. the 4th-6th week of pregnancy poses the greatest threat of embryonic disruptions, as it is the most sensitive period of growth.
B. the 9th-10th week of pregnancy poses the greatest threat of embryonic disruptions, as it is the most sensitive period of growth.
C. the 12th-14th week of pregnancy poses the greatest threat of embryonic disruptions, as it is the most sensitive period of growth.
D. the 15th-17th week of pregnancy poses the greatest threat of embryonic disruptions, as it is the most sensitive period of growth.
A. The 4th to the 6th week of pregnancy poses the greatest threat of embryonic disruptions, as it is the most sensitive period of growth.