Cleft Lip and Palate

Craniofacial conditions, including cleft lip and palate, are congenital structural anomalies caused by atypical embryological development. Craniofacial differences are a result of interruption in embryologic growth between the 4th and 10th week of the developing embryo or fetus (Peterson-Falzone, Hardin-Jones, & Karnell, 2010).

Clefts are described based on the structures involved (lip, alveolus, hard palate, soft palate), laterality (unilateral left, unilateral right, or bilateral), and severity (width and extent of structures involved). Clefts can be isolated (cleft lip only or cleft palate only), or they can occur in combination. They can be unilateral or bilateral, and they can be complete or incomplete.

Figure 1 contains images of the most common types of cleft affecting the palate, along with a labeled image of intact structures. The primary palate is the triangular area of the hard palate anterior to the incisive foramen and includes a portion of the alveolar ridge. The secondary palate consists of the remaining hard palate and all of the soft palate.

Palatal clefts can be overt or submucous. An overt palatal cleft is visibly open and can be observed on intraoral inspection. In a submucous cleft palate (SMCP), oral mucosa is intact, but the underlying velar musculature has failed to attach at the midline. The cleft is not visibly open and often is not obvious on intraoral inspection. There are three signs of an SMCP: (a) zona pellucida—a blue discoloration due to levator veli palatini muscle diastasis (i.e., separation in the midline); (b) a bifid uvula; and (c) a palpable bony notch at the edge of the hard palate.

Another type of SMCP referred to as an occult (hidden) submucous cleft palate (OSMCP) has no visual oral manifestations and can be diagnosed only by direct observation during surgery or by magnetic resonance imaging (MRI). Nasendoscopy may demonstrate a concave or flattened nasal surface of the palate, indicating possible diastasis and/or absence of the musculus uvulae muscle.

Cleft lip and cleft palate can be associated with a large number of craniofacial and genetic sequences or syndromes, some of which are rare. Some examples include the following:

• 22q11.2 deletion syndrome (a.k.a. velocardiofacial syndrome [VCFS] and DiGeorge sequence)
• Stickler syndrome
• Pierre Robin sequence
• Van der Woude syndrome
• Treacher-Collins syndrome
• Craniofacial microsomia (spectrum of disorders, including Goldenhar syndrome)
• Neonatal Abstinence Syndrome (NAS), which includes Fetal Alcohol Spectrum Disorder (FASD)

Incidence is the number of new cases of a disorder or condition identified in a specific period. Prevalence is the number of individuals who are living with the disorder or condition in a given period.

Epidemiological estimates of orofacial clefts vary substantially based on a variety of factors, including the sample population, the surveillance methodology, and the clinical classification (International Perinatal Database of Typical Oral Clefts [IPDTOC] Working Group, 2011).

Worldwide, oral clefts in any form (i.e., cleft lip, cleft lip, and palate, or isolated cleft palate) occur in about one in every 700 live births (World Health Organization [WHO], 2001). International estimates that are limited to cleft lip with or without cleft palate range from 7.94 to 9.92 per 10,000 live births (IPDTOC, 2011; Tanaka, Mahabir, Jupiter, & Menezes, 2012).

Cleft lip with or without a cleft palate is the second most common birth defect in the United States, affecting one in every 940 births and resulting in 4,437 cases every year (Parker et al., 2010). Reported prevalence estimates range from 7.75 to 10.63 per 10,000 live births (Parker et al., 2010; Tanaka et al., 2012). Isolated cleft palate is less common, presenting in one in every 1574 births (Parker et al., 2010).

The occurrence rate of orofacial clefts varies by population. Overall, higher rates have been reported in Asians and American Indians (one in 500 births), and lower rates have been reported in African-derived populations (one in 2,500 births; Dixon, Marazita, Beaty, & Murray, 2011). Isolated cleft palate is more frequently found in females than in males, at a ratio of 2:1. In contrast, there is a 2:1 male-to-female ratio for cleft lip with or without cleft palate (Mossey, Little, Munger, Dixon, & Shaw, 2009).

The signs and symptoms associated with clefting depend on a variety of factors, including type and severity and whether both lip and palate are involved. Individuals with cleft lip and palate may experience difficulties in one or more of the areas listed below.

Resonance and Airflow

Normal velopharyngeal (VP) closure separates the nasal and oral cavities and allows for speech to be produced with a balance between oral and nasal resonance. VP closure allows for (a) oral resonance of vowels, liquids, and glides and (b) the buildup of intraoral pressure and facilitation of orally directed airflow required for the production of the oral pressure consonants (stops, fricatives, and affricates). With normal resonance balance, nasal consonants /m/, /n/, and “ng” have nasal energy enhancement.

Velopharyngeal dysfunction (VPD; also referred to as velopharyngeal inadequacy [VPI]) may result from palatal clefting or other noncleft causes such as mechanical interference (e.g., large tonsils), neurogenic etiologies, or velopharyngeal mislearning (see Classification of Velopharyngeal Dysfunction [PDF] [Peterson-Falzone, Trost-Cardamone, Karnell, & Hardin-Jones, 2006; Trost-Cardamone, 1989]).

During speech production, VPD can cause hypernasality (a resonance disorder), nasal air emission, or both. Hypernasality and nasal air emission that result from an anatomical or physiological inability to close the VP port are considered obligatory (see “obligatory errors” in the Articulation subsection below).

• Hypernasality is an acoustic phenomenon that occurs when there is excessive resonance in the nasal cavity during the production of vowels and vocalic consonants (i.e., the liquids and glides), due to the coupling of the oral and nasal cavities. Hypernasality may increase in connected speech due to increased demands on the VP mechanism.
• Nasal air emission is an aerodynamic phenomenon resulting in the audible or inaudible release of air from the nasal cavity during the production of oral pressure consonants (i.e., the stops, fricatives, and affricates). It can be heard as nasal turbulence (nasal rustle), especially if the VP gap is small. Nasal air emission can also be caused by airflow through a palatal fistula into the nasal cavities.

Articulation

The resulting impact of VPD on articulation can be described as “cleft palate speech” or “cleft-type speech” and may include obligatory errors and/or compensatory (learned) errors.

Obligatory Errors

Obligatory errors exist due to structural abnormalities that result in velopharyngeal insufficiency and oral structural deviations (e.g., oronasal fistulas, dental deviations, or malocclusions).

They are not likely to improve until the structural cause is addressed through physical management (e.g., surgery or prosthetic intervention).

Obligatory errors include the following:

• Hypernasality on vowels, liquids, and glides (in severe cases, voiced oral consonants may also be nasalized).
• Articulation errors related to dental status or malocclusion (e.g., sibilant distortion with Class III dental malocclusion).
• Obligatory nasal air emission due to VPD or fistula, that is consistent across the oral pressure consonants (stops, fricatives, and affricates). This pattern of airflow into the nasal cavity can persist postoperatively, despite adequate VP closure.

Compensatory Errors

Compensatory errors are learned articulation errors. They are, for the most part, errors in place of articulation or direction of airflow.

These errors are thought to develop for several reasons, including the inability to generate adequate intraoral air pressure for consonant production, the presence of anterior structural anomalies, abnormal auditory–perceptual learning, or other factors.

Compensatory errors that developed due to anatomical inability to close the VP port can persist even after successful physical management of the VP mechanism.

Compensatory errors

• can exacerbate the perception of nasality and can inaccurately represent the child’s ability to use the VP valve;
• are treated through behavioral intervention (i.e., traditional speech therapy) to teach proper articulatory placement/patterns; and
• can be described as those that
  • occur below the level of the VP port and eliminate the need for valving of airflow at the level of the oral cavity and/or the VP port by allowing the individual to use the pharynx or larynx to valve air or pressure (e.g., glottal stops and fricatives [e.g., /h/], pharyngeal stops, fricatives, and affricates);
  • occur in front of the VP port and involve a backed place within the oral cavity (e.g., velarized alveolars and palatals, mid-dorsum palatal stops, fricatives, and affricates);
  • use the opening of the VP port as part of the articulation for intentional nasal release of airflow and simultaneous bilabial, alveolar, or velar closure (realized as voiceless nasal fricatives); or
  • involve ingressive rather than egressive airflow to eliminate the need for closure of the VP port (e.g., bilabial smacks, tip–alveolar clicks, and blade–alveolar fricatives)

Learned Nasal Emission

Learned nasal emission can be realized as nasal fricative substitutions or nasal emission that is co-produced with the target sound and may be associated with nasal turbulence or snorting.

The following two patterns may be seen:

• Phoneme-specific nasal emission (PSNE) — nasal emission that is limited to one or more specific sounds.
  • PSNE typically affects fricatives and may also affect affricates.
  • It may occur in individuals without cleft palate.
  • It is treated through behavioral intervention (i.e., traditional speech therapy).
• Persisting postoperative nasal emission — nasal emission that continues after surgical repair of the cleft palate when VP closure appears to be adequate.
  • The individual continues to use the pattern of directing air into the nasal cavity.
  • The clinician may need to consult with the team SLP to confirm adequate closure before behavioral intervention.

(See e.g., Golding-Kushner, 2001, 2015; Golding-Kushner & Shprintzen, 2011; Morgan & O’Gara, 2014; Peterson-Falzone, Trost-Cardamone, Karnell, &Hardin-Jones, 2016; Trost-Cardamone, 2013; Zajac & Vallino, 2017d)

Developmental Speech Errors

Children may also present with articulation and/or phonological errors that are unrelated to cleft palate. See ASHA’s Practice Portal page, Speech Sound Disorders: Articulation and Phonology, for a description of these error types and detailed information on assessment and treatment.

Early Speech and Language Characteristics

When compared with age-matched peers without cleft palate, babies with cleft palate often show both quantitative and qualitative differences in their early prelinguistic and linguistic development.

Speech and language development in children with clefts depends on several factors, including hearing status, type and severity of the cleft, and the presence of a syndrome.

• Babies with cleft palate
  • vocalize as frequently as do babies without cleft palate but may have delayed onset of canonical babbling;
  • have less variety in the canonical forms produced;
  • have a more restricted consonant inventory during babbling;
  • demonstrate fewer total consonant productions (e.g., fewer oral stops and more glottal stops);
  • have a preference for nasal glides and the glottal fricative /h/ (compared with a typical preference for alveolar stop /d/);
  • have delayed onset of first words and acquire words more slowly; and
  • demonstrate a preference for words beginning with sonorants (nasals, liquids, glides, vowels).

• As children get older,
  • articulation may be characterized by a restricted phonetic inventory, sound substitutions/omissions, compensatory articulation errors; and
  • expressive language development can be delayed but often catches up with age

• Children with a syndrome may experience more severe language disorders than children without a syndrome due to developmental delays associated with the syndrome.

(See e.g., Chapman, 1991; Chapman & Hardin, 1992; Chapman, Hardin-Jones, Schulte, & Halter, 2001; Estrem & Broen, 1989; Hardin-Jones & Chapman, 2014; O’Gara & Logemann, 1988; O’Gara, Logemann, & Rademaker, 1994; Olson, 1965; Peterson-Falzone et al., 2010; Scherer, Williams, & Proctor-Williams, 2008; Shprintzen, 2000; Trost-Cardamone, 2013)

Voice

Voice problems in children with a cleft palate may include the following:

• Laryngeal hyperfunction from attempting to compensate for the loss of pressure at the VP valve may result in muscle tension dysphonia with or without changes to the vocal folds (e.g., vocal nodules; inflammation, and edema).
• Soft voice syndrome due to loss of pressure through the VP port or when the child reduces vocal intensity as a compensatory strategy to minimize or disguise hypernasality, nasal emission, or hoarseness (see, e.g., Peterson-Falzone et al., 2010).

Syndromic conditions associated with a cleft palate may be associated with organic laryngeal disorders that affect the voice. Examples include calcification of the larynx in Apert syndrome and vocal fold paralysis and glottic webbing in 22q11.2 deletion syndrome (Cavalli, 2011; Shprintzen, 2000; Shprintzen & Golding-Kushner, 2008).

Feeding and Swallowing

Feeding problems can vary considerably in infants with cleft, depending on the type and severity of the cleft. In most cases, infants with clefts who are otherwise typically developing have normal pharyngeal swallowing function; once the milk reaches the oropharynx, the swallow is initiated with normal airway protection (Miller & Kummer, 2014; Shprintzen & Bardach, 1995).

Babies with cleft lips only typically have little feeding difficulty. Once the nipple is positioned in the baby’s mouth, he or she can usually achieve sufficient compression of the nipple against the intact palate.

Babies with cleft palate—with or without cleft lip—may have more significant feeding difficulty. They are unable to separate the nasal cavity from the oral cavity and therefore cannot create the negative pressure necessary for sucking. In addition, they may have difficulty compressing the nipple to express milk because the palatal surface is not intact.

Potential problems associated with feeding difficulties include

• fatigue due to excessive energy expended during feeding;
• poor weight gain due to inadequate nutritional intake;
• excessive air intake; and
• nasal regurgitation (Dailey, 2013; Miller & Kummer, 2014; Peterson-Falzone et al., 2016; Zajac & Vallino, 2017a).

Infants with cleft palate and other craniofacial anomalies associated with genetic syndromes or sequences may have more significant feeding and swallowing difficulties (Cooper-Brown et al., 2008; Dailey, 2013). For example, children with the Pierre Robin sequence are at increased risk for airway obstruction, dysphagia, and aspiration due to micrognathia and glossoptosis, which position the tongue toward or even against the posterior pharyngeal wall (Monasterio et al., 2004; Nassar, Marques, Trindale, & Bettiol, 2006; Shprintzen & Singer, 1992). In infants with 22q11.2 deletion syndrome, laryngeal, neurologic, or cardiac abnormalities may exacerbate feeding difficulties (e.g., Cuneo, 2001; Golding-Kushner & Shprintzen, 2011) and may contribute to fatigue (Cuneo, 2001).

Infants with cleft lip and palate who are preterm may have feeding and swallowing problems, which, in addition to those problems associated with clefting, place these infants at high risk for aspiration and poor nutritional intake.

More significant feeding and swallowing difficulties seen in these populations may be signaled by the following:

• Inability to establish suck–swallow–breathe sequence
• Arching of back or refusal of nipple
• Coughing
• Choking and gagging
• Increased respiration rate
• Oxygen desaturation

Dental/Orthodontic

Dental deviations and malocclusion can affect articulatory placement.

Dental deviations include the following:

• Congenitally missing teeth
• Malrotated teeth
• Ectopic teeth (abnormally located)
• Supernumerary or duplicated teeth

Malocclusions include the following:

• Overjet/overbite, often associated with Class II malocclusion (upper teeth protrude beyond lower teeth)
• Underjet/underbite, often associated with anterior crossbite or Class III malocclusion (lower teeth protrude beyond upper teeth)
• Open bite (teeth do not fully occlude)
• Lateral (buccal) crossbite (lower teeth buccal to upper teeth)

Hearing

Children with cleft palate with or without cleft lip may be at high risk for middle ear effusions and associated conductive hearing loss because of eustachian tube malfunction (Flynn, Möller, Jönsson, & Lohmander, 2009). The prevalence of middle ear fluid in children with unrepaired cleft palate has been estimated at more than 90% (Paradise, Bluestone, & Felder, 1969; Stool & Randall, 1967). Middle ear fluid is also seen in children with isolated cleft lips, although the prevalence is lower than in children with cleft palate (Deedler et al., 2011; Ruegg et al., 2015; Vallino, Zuker, & Napoli, 2008).

Other potential causes of conductive hearing loss include anomalies of the outer ear (e.g., stenotic or narrow external auditory canal) and anomalies of the middle ear (e.g., malformation of the ossicles; Zajac & Vallino, 2017b).

Fluctuating and long-standing conductive hearing loss is the primary concern for children with cleft palate. However, sensorineural hearing loss (e.g., due to inner ear anomalies), or mixed hearing loss may also be present, especially in children with syndromes (e.g., Stickler syndrome; Nowak, 1998; Zajac & Vallino, 2017b).

Psychosocial Impact

Cleft lip and palate can have an impact on the individual’s psychological and social well-being; this impact can vary based on cultural beliefs.

The potential psychosocial impact can include the following:

• Psychosocial issues stem from the realization of one’s facial differences and the reactions/judgments of others. These issues include
  • poorer self-concept;
  • lower self-confidence;
  • feelings of depression and anxiety; and
  • difficulties initiating and maintaining friendships (see, e.g., Broder & Strauss, 1989; Noar, 1991; Ramstad, Otten, & Shaw, 1995; Turner, Thomas, Dowell, Rumsey, & Sandy, 1997).
• Impact of hearing loss on socialization—even mild hearing loss can result in missed portions of fast-paced conversations and can hurt social interactions (Anderson & Matkin, 1991).
• False stereotypes and misconceptions may lead to lower expectations in the classroom—for example, teachers may assume that the child has a lower IQ and consequently may lower the student’s learning standards (Richman, 1978a, 1978b).
• Vulnerability to teasing/bullying and discrimination based on appearance (e.g., Turner et al., 1997).

See Hunt, Burden, Hepper, and Johnston (2005) and Peterson-Falzone et al. (2010) for more detailed discussions of the psychosocial impact of cleft lip and palate.

Earlier theories suggested that craniofacial conditions may negatively affect mother-infant interaction and attachment (Clifford, 1969; Endriga, Speltz, & Wilson, 1992; Field & Vega-Lahr, 1984; Waechter, 1977). However, recent research indicates that the mother-infant relationship does not appear to be affected by facial differences associated with clefting (Maris, Endriga, Speltz, Jones, & DeKlyen, 2000). By 2 years of age, most infants with cleft lip and palate demonstrate secure maternal attachments (Maris et al., 2000).

There is no one cause of cleft lip and palate. However, most cases are thought to result from multifactorial inheritance—an interaction between the person’s genes (genetic predisposition) and specific environmental factors (see, e.g., Beaty et al., 2011). Clefting can also be caused by chromosomal differences in individuals born with genetic syndromes.

Risk factors that increase the likelihood of cleft lip and palate include the following:

• Strong family history of cleft lip and palate (e.g., Grosen et al., 2010; Natsume, Kawai, Ogi, & Yoshida, 2000; Sivertsen et al., 2008).
• Race—cleft lip and palate are more common in Asians and Native Americans than in African Americans (see Incidence and Prevalence section).
• Sex—males are more likely to have cleft lip with or without cleft palate; females are more likely to have cleft palate without cleft lip (see Incidence and Prevalence section).
• Exposure to certain environmental substances, such as tobacco and alcohol, prescription drugs, and illegal drugs (e.g., Arpino et al., 2000; DeRoo, Wilcox, Drevon, & Lie, 2008; Källén, 2003; Li et al., 2010; Little, Cardy, & Munger, 2004; van Gelder, Reefhuis, Caton, & Werler, 2009).

Speech-language pathologists (SLPs) play a central role in the screening, assessment, diagnosis, and treatment of speech and language problems, as well as feeding and swallowing problems, associated with cleft lip and palate. The professional roles and activities in speech-language pathology include clinical/educational services (diagnosis, assessment, planning, and treatment); prevention and advocacy; and education, administration, and research.

A team approach to service delivery is essential throughout the individual’s care, and SLPs typically provide services as part of, or in collaboration with, a team. See Standards for Cleft Palate and Craniofacial Teams from the American Cleft Palate-Craniofacial Association (ACPA, 2016) for essential characteristics of quality for team composition and functioning. See also ASHA’s web page, Interprofessional Education/Interprofessional Practice (IPE/IPP).

Appropriate roles for SLPs include, but are not limited to,

• counseling persons with cleft lip and palate and their families regarding communication- and feeding/swallowing-related issues and providing education aimed at preventing further complications relating to these conditions;
• educating other professionals about the needs of persons with cleft lip and palate and the role of SLPs in diagnosis and management;
• screening individuals who present with cleft lip and palate; determining the need for further assessment and/or referral for other services;
• conducting a comprehensive, culturally and linguistically appropriate assessment of speech, language, resonance, voice, or feeding problems associated with cleft lip and palate;
• diagnosing speech, language, resonance, voice, or feeding disorders associated with cleft lip and palate;
• referring to other professionals to rule out co-occurring conditions, determine etiology, and facilitate access to comprehensive services;
• making decisions about the management of speech, language, resonance, voice, and feeding/swallowing problems related to cleft lip and palate;
• developing treatment plans, providing treatment, documenting progress, and determining appropriate dismissal criteria;
• consulting and collaborating with other professionals, family members, caregivers, and others to facilitate program development and to provide supervision, evaluation, and/or expert testimony, as appropriate;
• remaining informed of research in the area of cleft lip and palate and helping advance the knowledge base related to the nature and treatment of these conditions;
• advocating for individuals with cleft lip and palate and their families at the local, state, and national levels;
• serving as an integral member of an interdisciplinary team working with individuals who have cleft lip and palate and their families/caregivers; and
• providing quality control and risk management.

As indicated in the Code of Ethics (ASHA, 2016a), SLPs who serve this population should be specifically educated and appropriately trained to do so. This is of paramount importance for those serving on a cleft palate-craniofacial team, where decisions regarding surgical intervention require significant SLP input.

Screening

Speech screening for the child with clefting or suspected VP dysfunction may be completed by an SLP in a variety of settings, including a cleft palate-craniofacial team visit.

The SLP attends to signs of

• anatomical/structural differences (e.g., malocclusion, submucous cleft, fistula, or bifid uvula);
• resonance or nasal airflow problems;
• articulation errors that may be characterized as obligatory errors (due to structural differences) or as compensatory (learned) errors; and
• articulation errors that may be characterized as developmental (i.e., traditional articulation and phonology disorders).

The screening protocol may also include a hearing screening and screening of expressive and receptive language skills. Screening may result in recommendations for additional assessments/examinations by the team and/or a comprehensive speech and language assessment.

Comprehensive Assessment

Assessment and treatment of clefts may require the use of appropriate personal protective equipment.

An SLP conducts a comprehensive speech and language assessment using both standardized and nonstandardized measures. (See ASHA’s resource on assessment tools, techniques, and data sources).

Some infants and toddlers with clefts may be evaluated by an SLP in early intervention to identify language delays, even before they are seen by a team. An understanding of the speech and language characteristics of these children is essential for accurate assessment. For children who are not yet talking, the assessment includes measures of vocalization diversity and complexity, vocalization rate, and use of communicative gestures (Scherer, 2017).

For school-age children, the assessment may be conducted by the school SLP, who provides services to the child within the context of an educational setting. The assessment may also be conducted by the team SLP or by an SLP in another outpatient or private clinic. Collaboration between the team SLP and other SLPs involved in service delivery for a child with a history of cleft lip and palate is encouraged (ACPA, 2016).

Due to the likelihood of transient or permanent hearing loss in this population, the comprehensive assessment typically includes an audiological assessment by an audiologist.

The assessment is conducted in the language(s) used by the child and his or her family, with the use of interpretation services as necessary (see ASHA’s Portal Page, Collaborating With Interpreters). Assessment takes into account speech and language characteristics unique to the linguistic background of the individual and the cultural variables that affect communication.

Consistent with the World Health Organization’s (WHO) International Classification of Functioning, Disability, and Health (ICF) framework (ASHA, 2016a; WHO, 2001), a comprehensive assessment is conducted to identify and describe

• impairments in body structure and function, including underlying strengths and weaknesses in speech sound production and verbal/nonverbal communication;
• co-morbid deficits or conditions, such as developmental disabilities or syndromes;
• limitations in activity and participation, including functional communication, interpersonal interactions with family and peers, and learning;
• contextual (environmental and personal) factors that serve as barriers to or facilitators of successful communication and life participation; and
• the impact of communication impairments on the quality of life of the child and family.

Assessment may result in

• diagnosis of a speech, language, resonance, voice, and/or feeding and swallowing disorder;
• determination of the type, severity, and possible cause of the disorder;
• recommendation for therapy identification of barriers to child and family participation in everyday situations;
• referral for surgical, prosthetic, or other medical or dental intervention; and/or
• referral for genetic testing to rule out syndromes.

Cultural Considerations

Studies of populations in Cambodia, China, Egypt, India, Kenya, Nigeria, Peru, Russia, and South Africa, have found that causal explanations of cleft lip and palate sometimes reflect religious beliefs (e.g., evil spirits or punishment for a past sin). Other causal explanations include a poor diet or starvation during pregnancy, viewing a solar eclipse during pregnancy, or having lived near a nuclear plant during pregnancy (Loh & Ascoli, 2011; Mednick et al., 2013).

Cultural beliefs about the cause of cleft lip and palate may influence a family’s decisions about seeking treatment (Loh & Ascoli, 2011; Louw, Shibambu, & Roemer, 2006; Mednick et al., 2013). For example, individuals who believe that clefting is “fate” or “God’s will” may not seek treatment, so as not to interfere with the spiritual world (Loh & Ascoli, 2011).

Cultural differences might also affect the interaction between the clinician and family, and cultural sensitivity on the part of the clinician is essential for developing trust. Professionals need to understand cultural differences—including differences in maternal reactions to the birth of a child with clefting—and learn to communicate across cultural lines when providing care (Black, Girotto, Chapman, & Oppenheimer, 2004; Strauss, 1997). Using ethnographic interviewing techniques can be an effective strategy for gathering information about cultural beliefs and concerns.

Team Approach to Treatment

Treatment for children with cleft palate (with or without cleft lip) and associated craniofacial or velopharyngeal conditions involves a team approach. The team includes a patient care coordinator or nurse who facilitates team function and efficiency, assists families, and coordinates care for individuals and families. The cleft palate team must have as a minimum core a surgeon, an orthodontist, and an SLP.

Teams may also include or have access to professionals in other fields, such as audiology, general dentistry, genetics, nursing, otolaryngology, pediatrics, psychology, and social work. Additional members vary depending on the individual’s developmental, medical, and psychological needs.

Components

The primary components of treatment are surgical repair and behavioral intervention (i.e., speech therapy).

• The goal of surgery is to repair affected structures to establish adequate VP function, facilitate orofacial growth, and improve appearance.
• The goal of speech therapy is to establish normal articulation behaviors.

Treatment may also include early feeding intervention, dental care and orthodontics, audiological care and monitoring of hearing status, and psychological services.

In addition to speech therapy for cleft palate speech deviations, treatment may also address developmental articulation and phonology disorders and language delays or disorders, if present.

Approaches to Treating Cleft-Type Speech

Approaches to treatment for articulation disorders associated with a cleft palate or VPD will depend on whether or not speech deviations are obligatory (i.e., related to atypical anatomy and/or structural defects) or learned.

• Obligatory speech deviations that are related to true VPD or other structural deviations such as fistulas are not responsive to speech therapy and will likely require surgical intervention or other physical management.
• Learned articulation errors (e.g., compensatory errors and phoneme-specific nasal air emission) should be responsive to speech therapy.

Surgical Intervention and Other Physical Management

Surgical intervention or other physical management is needed for speech deviations resulting from

• resonance disorders (hypernasality or hyponasality);
• pervasive nasal air emission (and/or weak pressure on oral consonants); and
• other obligatory errors (e.g., related to dental status/malocclusion).

Presurgical orthopedics may be used to move cleft segments closer together and into better alignment before surgical repair. These include

• lip taping;
• lip adhesion;
• nasoalveolar molding (NAM) appliances; and
• devices attached by pins and screws that force the alignment of maxillary segments.

Surgeries can include one or more of the following:

• Repair of cleft lip (usually performed within the first 6 months of life)
• Repair of cleft palate (usually by 12 months of age in a child with typical development)
• Repair of submucous cleft palate
• Procedures to correct noncleft VPD or to correct postoperative velopharyngeal insufficiency (e.g., pharyngeal flap; sphincter pharyngoplasty; palatal lengthening procedures; posterior pharyngeal wall augmentation)
• Tonsillectomy and/or adenoidectomy (may be needed before pharyngeal flap or other pharyngoplasty)
• Secondary surgeries to repair palatal fistulae (if symptomatic)
• Alveolar bone grafting (determined by the stage of dental development and in collaboration with an orthodontist)
• Surgical treatment of malocclusion (e.g., maxillary advancement—typically completed in adolescence)

Other physical management may include the following:

• Orthodontics—to correct various malocclusions and other dental alignment deviations
• Prosthodontics—including palatal obturators, speech bulbs, and palatal lifts

Behavioral Intervention: Speech Therapy for Cleft Palate Speech Errors

Direct speech therapy is appropriate for treatable speech problems (i.e., articulation errors), including compensatory misarticulations and phoneme-specific nasal air emission.

The objectives of therapy are to

• correct oral placements for consonant misarticulations (e.g., bring backed oral articulations forward, teach correct oral place, and establish oral pressure buildup and release) and
• establish oral pathway/direction of airflow and appropriate valving of airflow at the target place during the production of oral sounds (Golding-Kushner, 2001; Ruscello, 2017; Trost-Cardamone, 2013).

Therapy can be diagnostic. Progress can help inform the cleft palate team about VP function and the possible need for (further) surgical intervention

Treatment Strategies

Therapy typically targets the elimination of compensatory misarticulations first, as these tend to have a greater impact on intelligibility and VP function than do other types of speech sound errors. Later therapy addresses developmental articulation or phonological errors if present.

Initial Therapy Targets

Initial therapy targets for children with compensatory misarticulations may include

• sounds for which the child is stimulable (i.e., can articulate, given a model);
• nasal and low-pressure consonants, if they are produced or co-produced with glottal stops;
• high-pressure consonants that are not in the inventory, are age-appropriate, or have a high impact on intelligibility/understandability; and
• anterior consonants that are visible (e.g., p, b, t, d, f), given that children often compensate with posterior placement.

Strategies and Techniques

Strategies and techniques used in therapy include

• Using phonetic placement techniques, typically starting with bilabials and then moving to alveolars.
• Introducing gentle whispering or using sustained /h/ to break the glottal pattern and to teach easy oral airflow with an open glottis.
• Inserting /h/ after oral stop consonants to discourage use of glottal stops prior to vowel onset [e.g., p(h)op for “pop”].
• Teaching auditory discrimination between the correct target and the compensatory error to facilitate self-monitoring.
• Providing visual cues, including
  • using a lateral diagram or a model of the mouth for visual placement cues;
  • pointing to lips/tongue to cue placement;
  • using visual airflow cues (e.g., using a feather); and
  • using mirrors for observing articulatory positioning—sit side-by-side, so that the child can view the reflection of themselves and the therapist.
• Providing verbal cues such as instructions for correct placement and manner or airstream.
• Providing tactile cues, such as
  • feeling one’s neck musculature to help identify incorrect placement for glottal stops and pharyngeal stops and fricatives;
  • feeling a released puff of air on one’s hand during the production of plosives;
  • using one’s finger to feel bilabial closure and oral air pressure on plosives; and
  • using nose plugging/pinching (nasal occlusion) to provide the individual with the sensation of oral pressure and to discourage nasal airflow errors.
• Using established speech therapy procedures and techniques (e.g., minimal pairs, traditional shaping hierarchy).

(Golding-Kushner, 2001; Peterson-Falzone et al., 2016; Trost-Cardamone, 2013)

Use of Technology in Speech Therapy

Several technologies can be used in speech therapy to provide real-time feedback. However, except for the low-tech tools listed below, many are not available to SLPs in early intervention, early childhood, or school-based programs.

Technologies for real-time feedback include the following:

• Low-tech tools for targeting PSNE:
  • A dental mirror was placed under the nose
  • See-Scape™
  • Stethoscope (placed against the side of the nose)
  • Plastic tubing or drinking straw for self-monitoring one’s production (one end is placed at the patient’s nostril entrance, and the other end is placed by the ear)
• Nanometer to monitor oral versus nasal speech and provide real-time visual feedback; used in cases of phoneme-specific disorders or nasalization errors (not to treat consistent hypernasality).
• Nasopharyngoscopy provides visual feedback about the actions of the VP mechanism during speech (Brunner, Stellzig-Eisenhauer, Pröschel, Verres, & Komposch, 2005), which may help individuals improve VP movements when they have the physical ability to achieve VP closure but do not do so because of faulty articulation (e.g., PSNE; Witzel, Tobe, & Sayler, 1988).
• Electropalatography (EPG)—is a computer-based technique for training correct oral articulation placements; EPG provides a visual feedback display of the tongue’s contact with the hard palate during speech (Lee, Law, & Gibbon, 2009).

See Kummer (2014b) for a description of various technologies used in speech therapy for cleft-type speech errors.

Early Speech and Language Intervention

Delays in the onset of babbling and the lack of diversity in consonant production during the prelinguistic period have been linked to delays in speech and language development (Stoel-Gammon, 1994). Early speech and language intervention can lessen the impact of these factors on the development of communication skills in infants and toddlers (see, e.g., Scherer, 1999; Scherer & Kaiser, 2007).

The most important goal of early intervention for babies and toddlers with cleft lip and palate is to help them develop oral speech by increasing consonant inventory, increasing expressive vocabulary, and teaching oral airflow for early emerging fricatives (e.g., /h/; Hardin-Jones, Chapman, & Scherer, 2006; Trost-Cardamone, 2013).

Parent education and training are essential in early intervention. SLPs counsel parents about the expected impact of clefting on speech and encourage parents to foster good communication skills in their babies (Peterson-Falzone et al., 2010). Parent-implemented early intervention programs have been shown to facilitate speech and language development in young children with cleft palate (e.g., Scherer, 2003; Scherer, D’Antonio, & McGahey, 2008).

SLPs play a role by teaching parents

• how to engage in vocal play with their children before surgery;
• how not to reinforce compensatory productions (e.g., laryngeal growls and nasal fricatives); and
• how to provide intensive language stimulation (Golding-Kushner, 2001; Hardin-Jones, Chapman, & Scherer, 2015; Kummer, 2014b; Scherer, 2017; Trost-Cardamone, 2013).

Before Palatal Repair

Before palatal repair, goals include the following:

• Using vocal play activities to stimulate the production of speech sounds least affected by the cleft (e.g., nasals, glides, glottal fricative /h/, and vowels) by
  • modeling varied sounds and encouraging imitation of sounds as well as lip, tongue, and facial movements and
  • reinforcing attempts to produce oral stop consonants and ignoring any obligatory nasal emissions.
• Modeling alternatives for compensatory productions (e.g., “no no” instead of “uh uh”).

After Palatal Repair

After palatal repair, goals include the following:

• Using sound play and early word productions to increase the variety of vocalization.
• Increasing consonant inventories, especially oral pressure consonants.
• Increasing communication opportunities.
• Increasing vocabulary, with a focus on functional words.
• Increasing awareness of oral airflow for speech.
• Monitoring persistent nasal substitutions and glottal stops.
  • If present after surgery, this might indicate learned behaviors (emerging compensatory misarticulations) despite a potentially adequate VP mechanism.

(Hardin-Jones et al., 2006, 2015; Peterson-Falzone et al., 2016; Scherer, 2017)

Feeding and Swallowing

Feeding issues are among the first concerns following the birth of a child with cleft lip and palate. The goal of feeding intervention is to ensure adequate and efficient intake for appropriate hydration and nutrition, growth and development, and adequate medical status before surgery.

An important additional goal is to ensure that feeding is a low-stress experience for infants and families (Reid, 2004). Healthcare providers (e.g., SLPs, nurses, or lactation consultants) instruct mothers in feeding techniques and reassure them that successful feeding can be accomplished with the least amount of stress for the infant and family (Goyal, Chopra, Bansal, & Marwaha, 2014).

Before Surgery

Several strategies and techniques can be used to facilitate feeding success before surgery, including

• placing the baby in an upright position while feeding to help prevent nasal regurgitation;
• providing jaw and cheek support as needed;
• using the appropriate nipple size;
• positioning the nipple optimally (e.g., away from the side of the cleft, if unilateral);
• pacing flow rate and providing fluid in rhythm with baby’s cues (e.g., movements and reactions);
• burping frequently to counter excessive air intake; and
• limiting feeding time (e.g., no more than 30 minutes) to avoid fatigue (Dailey, 2013; Peterson-Falzone et al., 2010, 2016).

nasoalveolar molding (NAM) appliances are used before surgery to align maxillary segments; however, a secondary benefit is that the appliance can function as a feeding plate. Some parents report improved feeding with a NAM appliance (Zajac & Vallino, 2017a).

Bottle Feeding

Most babies who have a cleft palate with or without a cleft lip will need a modified bottle to feed successfully. Nipples can vary in shape, size, and pliability to accommodate each baby’s unique needs. For example, a nipple with a wide base may be sufficient for some babies to achieve adequate lip seals despite a wide cleft lip.

Other frequently used modifications include the following:

• Nipples with a one-way valve at the base. This valve allows milk to flow into the nipple but not back into the bottle when the nipple is compressed; milk in the nipple flows into the baby’s mouth on compression (e.g., Special Needs Feeder, Pigeon Bottle, and Dr. Brown’s Standard Specialty Feeding System).
• Soft, squeezable bottles that allow the feeder to express milk into the baby’s mouth by compressing the bottle in sync with each sucking motion (e.g., Mead Johnson Cleft Palate Nurser).
• Nipples with enlarged (e.g., cross-cut) holes that allow milk to flow into the baby’s mouth via force of gravity; the feeder must monitor the baby’s cues and control the rate of flow to avoid compromising the baby’s suck–swallow–breathe coordination.

See the Cleft Palate Foundation video, “Feeding Your Baby,” for demonstrations of feeding using various bottle and nipple types.

Breastfeeding

Mothers interested in breastfeeding should be supported and given all necessary guidance in their efforts. Consultation with a lactation consultant or feeding specialist is often recommended, and the baby’s weight gain should be closely monitored. If breastfeeding is not sustainable for maintaining adequate nutrition and hydration, mothers who are interested in bonding with their babies during nonnutritive times at the breast after feeding should be supported.

The type and severity of the cleft can affect the degree of success with breastfeeding. For example, babies with isolated cleft lips, small soft palate clefts, or submucous clefts will have less difficulty than babies with larger clefts of the lip and/or palate (see, e.g., Arvedson & Brodsky, 2001; Clarren, Anderson, & Wolf, 1987; Garcez & Giugliani, 2005; Grady, 1977; Mei, Morgan, & Reilly, 2009; Miller, 2011).

As with bottle feeding, making modifications in the feeding position and providing cheek and lip support can help make breastfeeding successful. Often, the baby is unable to empty the breast; however, milk can be expressed and placed into a bottle to complete the feeding or to store for later feeding.

After Surgery

Decisions about the timing and methods of feeding after surgical cleft repair vary and depend on several factors, including

• the type (e.g., lip or palate; unilateral or bilateral) and size of the original cleft;
• the particular surgical procedure (e.g., primary lip repair or primary palate repair); and
• the preferences of the surgeon and the cleft-craniofacial team.

Some babies are allowed to feed from a bottle or breast immediately after surgery, while others will use alternate methods (e.g., syringe or spoon feeding) for the first month or so following surgery (see, e.g., Cohen, Marschall, & Schafer, 1992; Darzi, Chowder, & Bhat, 1996; Peterson-Falzone et al., 2016).

Feeding Considerations: Babies With Clefts Related to Craniofacial and Genetic Sequences or Syndromes

Clefting is sometimes one of several anomalies in children with craniofacial and genetic sequences or syndromes. Some anomalies associated with these conditions may place the child at risk for airway compromise during feeding. These anomalies may include differences that are both structural (e.g., anatomically smaller airway) and neurological (e.g., weakened muscles due to hypotonia).

Careful assessment is conducted, with particular consideration for potential airway compromise during feeding. The infant’s nutritional needs might necessitate alternate feeding methods (e.g., tube feeding).

Audiologic Care

Early audiological assessment and ongoing monitoring of hearing status by an audiologist are essential for children with cleft lip and palate, especially because of the potential impact of hearing loss on speech and language development (Holm & Kunze, 1969; Yoshinaga-Itano, Coulter, & Thomson, 2000).

Audiologic assessment and management depend on the type and degree of hearing loss and will include

• diagnostic auditory brainstem response (ABR) testing shortly after birth;
• frequent monitoring of hearing status and middle ear function, especially from birth to age 5–6 years old;
• referral and ongoing monitoring of middle ear health and ventilation tube function by the otolaryngologist; and
• consideration for amplification and personal FM or Bluetooth system for educational settings when persistent or permanent hearing loss is identified.

Teachers and other school personnel should be informed of any necessary services and accommodations for school-age children with fluctuating or permanent hearing loss (ACPA, 2009).

Role of the Community-Based SLP in Treatment

Collaboration

Successful speech therapy involves a partnership between the community-based SLP (e.g., school, private clinic, etc.), the cleft palate team, and the child’s parents and caregivers.

Collaboration between the community-based SLP and the SLP on the cleft palate team is essential to ensure optimal speech outcomes (Golding-Kushner, 2001; Peterson-Falzone et al., 2016).

Models of collaboration vary and may change throughout treatment, based on the needs of the individual and the availability of resources (Dailey & Wilson, 2015; Grames, 2004). Models include informal consultation, formal consultation, parallel service delivery, and co-provision of care (Lorenz, Mauksch, & Gawinski, 1999).

Education and Training

Community-based SLPs involved in speech intervention must be familiar with the timeline for surgical and orthodontic intervention, the impact of oral and pharyngeal structures on speech, and the types of errors related to clefts and VPD (Dailey & Wilson, 2015).

Adequate preparation at the graduate school level as well as continuing education and training opportunities are essential for providing the community-based SLP with the knowledge and skills necessary to treat this population (Bedwinek, Kummer, Rice, & Grames, 2010; Grames, 2008).

School-based SLPs report that information in several areas would be helpful. These areas include specific speech treatment techniques, assessment, and treatment of articulation disorders related to VPD, treatment of resonance disorders, and language problems of children with cleft lip and palate (Bedwinek et al., 2010).

Persisting Speech Difficulties

Speech difficulties associated with cleft lip and palate can persist into adolescence and adulthood and can have an impact on self-esteem and ease of communication in educational, social, and vocational settings.

Speech difficulties can persist for several reasons, including

• lack of access to adequate speech services when the child was younger, resulting in habituated speech patterns (compensatory articulations) that may be more difficult to change;
• persistent anatomic obstacles to normal speech, such as severe malocclusion or untreated VPD;
• expectations of failure;
• poorly developed self-monitoring skills; and
• lack of motivation.

Some of the speech errors that persist into adolescence are related to past or ongoing dental and occlusal problems. Some surgical procedures (e.g., maxillary advancement) are done in adolescence, and treatment of related errors or distortions may have been deferred until then. Speech is reevaluated after the surgical procedure to check for articulation and resonance and to ensure that no new or worsening symptoms are present. Additional speech therapy may be appropriate for these individuals. They will need to be highly motivated to improve speech to overcome learned misarticulations that have increased in habit strength over time (see, e.g., Kummer, 2014b; Peterson-Falzone et al., 2010).

Transitioning to Adult Services

Individuals transitioning from child-centered multidisciplinary care to adult-centered care may face several challenges, including difficulties locating appropriate services on their own. Barriers associated with the transition may hurt the quality of care and the individual’s quality of life. A model of adult care that incorporates a team approach would likely facilitate this transition (Zajac & Vallino, 2017e).

Cultural Considerations

Cultural sensitivity is essential when discussing potential treatment recommendations and outcomes. Careful consideration of the family’s beliefs and values is a an import to factor when designing treatment goals. The clinician must consider the individual’s linguistic environment to ensure that goals will meet the linguistic demands of daily life for the individual and his or her family.

Service Delivery

In addition to determining the type of speech and language treatment that is optimal for individuals with cleft lip and palate, SLPs consider other service delivery variables—including format, provider, dosage, timing, and setting—that may affect treatment outcomes.

• Format — whether a person is seen for treatment one-on-one (i.e., individual) or as part of a group; the format of service delivery for this population can include in-office and telepractice models (Golding-Kushner, 2015)
• Provider — the person providing treatment (e.g., SLP, trained volunteer, caregiver)
• Dosage — the frequency, intensity, and duration of service
• Timing — the timing of intervention relative to the diagnosis—this includes the timing of behavioral intervention about surgical/physical management
• Setting — the location of treatment (e.g., home, community-based, school)

References