Patrick J. Antonelli
Oral clefts can profoundly impact the physiology of the entire upper aerodigestive tract. Although disorders of the upper aerodigestive tract are central to the field of otolaryngology, patients with oral clefts are best managed by a multidisciplinary team. Many of the issues related to the Otolaryngologic needs of individuals with oral clefts have been addressed in other chapters (e.g., velopharyngeal insufficiency). Accordingly, this chapter focuses on the ear, nose, and throat issues for which individuals with oral clefts most commonly seek Otolaryngologic care (Table 30.1).
Ear
Disorders of the ear are the most common reason that children with cleft palate seek care from an otolaryngologist. Otologic problems in individuals with oral clefts, like those in individuals without clefts, change with age. In early childhood, the primary concerns include acute otitis media, chronic otitis media with effusion, and the cognitive sequelae of hearing loss. With advancing age, otologic concerns shift to the middle ear sequelae of chronic eustachian tube dysfunction and its treatment.
Otitis Media: Classification and Prevalence
Otitis media is defined as any inflammation within the middle ear. The sine qua non of otitis media is effusion within the middle ear space. Further classification is based largely on duration, the nature of the effusion, and the presence or absence of symptoms (Table 30.2). Though these definitions may seem unambiguous, there are no clear distinctions in clinical practice. Otoscopy is far from 100% accurate (Finitzo et al., 1992; Jensen and Lous, 1999). Otitis media is a dynamic process that may vacillate along a continuum (Paparella et al., 1990). Extrinsic factors may also complicate the distinction between types of otitis media (Kempthorne and Giebink, 1991). For example, a child with persistent otitis media with effusion may become febrile because of a bacterial superinfection in the middle ear. Alternatively, the fever may be due to an unrelated viral illness. These issues must be kept in mind when making therapeutic decisions concerning children with otitis media, particularly children with persistent otitis media with effusion, such as children with cleft palate.
Though clinicians may use many different criteria to diagnose acute otitis media (Hayden, 1981), the criteria proposed by Paradise (1995) have gained the widest acceptance. Acute otitis media is generally defined as acute, symptomatic, suppurative middle ear inflammation. Acute otitis media is likely to afflict most children with cleft palate at some point in their early childhood (Skolnik, 1958), but the same is true for children without cleft palate (Teele et al., 1989). Recurrent acute otitis media is only slightly more common in children with cleft palate (Rynnel-Dagoo et al., 1992).
Otitis media with effusion is defined as painless, persistent middle ear effusion. Otitis media with effusion is found in nearly all infants born with cleft palate (Stool and Randall, 1967; Paradise et al., 1969, Dhillon, 1988; Grant et al., 1988). Controlled studies ave confirmed that otitis media with effusion is far more common in children with cleft palate (Kemaloglu et al., 1999).
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TABLE 30.1. Most Common Otolaryngological Issues in Individuals with Oral Clefts |
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Chronic suppurative otitis media is broadly defined by persistent suppuration of the middle ear, manifested by otorrhea through a defect in the tympanic membrane (e.g., a tympanic membrane perforation). Though persistence is usually defined as being greater than 6 to 8 weeks, it also implies failed conservative management. Chronic suppurative otitis media is further categorized by the presence or absence of cholesteatoma (i.e., the presence of squamous epithelium and accumulation of desquamated skin debris within the middle ear space). The latter classification is of great importance with respect to therapeutic options. Cholesteatoma (Dominguez and Harker, 1988) and tympanic membrane perforation (Gordon et al., 1988) have long been reported to be more common in patients with cleft palate than in those without it (Podoshin et al., 1986; Mawson and Ludman, 1979). This has been substantiated in more rigorous epidemiologic studies (Kemppainen et al., 1999).
Eusfachian Tube Dysfunction and the Pafhogenesis of Ofitis Media
Eustachian tube dysfunction is thought to be central to the pathogenesis of otitis media. The main effect of eustachian tube dysfunction is a gradual absorption of oxygen and carbon dioxide from the middle ear space and the development of negative middle ear pressure (Elner, 1977; Doyle and Seroky, 1994). The negative pressure leads to transudation and accumulation of sterile middle ear effusion (Casselbrandt et al., 1988). This is known as the “hydrops ex vacuo” theory of pathogenesis of chronic otitis media with effusion.
Otitis media with effusion in individuals with cleft palate is due primarily to eustachian tube dysfunction resulting from tensor veli palatini incompetence (Doyle et al., 1980; Casselbrandt et al., 1988). Although tensor veli palatini dysfunction improves after definitive palatal reconstructive surgery, this may never normalize, and otitis media with effusion remains a common problem after palatal surgery (Matsune et al., 1991; Dhillon, 1988; Tasaka et al., 1990; Rynnel-Dagoo et al., 1992; Robinson et al., 1992; Nunn 1995). This may result from congenital abnormalities of the eustachian tube that are not, and cannot be, addressed by palatoplasty (Yamaguchi et al., 1990; Matsune et al., 1991, 1992; Kemaloglu et al., 1999).
Tubal dysfunction and negative middle ear pressure also predispose to bacterial infection (Meyerhoff et al., 1981). This may lead to recurrent acute otitis media with common upper respiratory pathogens, Streptococcus pneumoniae, Haemophilus influenzae, and Moraxella catarrhalis. Tubal dysfunction is very common in all children (Bluestone and Klein, 1996a) and after viral upper respiratory dysfunction (Buchman et al., 1994). Children with cleft palate, like any other children, are vulnerable to other risk factors for the development of acute otitis media, including day-care attendance, bottle-feeding (vs. breast-feeding), and environmental tobacco smoke exposure. Hence, it can be difficult to isolate the effects of cleft palate from these other factors.
Chronic otitis media with effusion may also be perpetuated, at least in part, by bacterial infection. Though chronic otitis media with effusion (nonsuppurative otitis media) has traditionally been thought of as a sterile process, the same spectrum of bacteria found in acute otitis media may be cultured from the middle ear effusion in up to 30% of cases (Liu et al., 1976; Post et al., 1995). Polymerase chain reaction techniques have suggested that the vast majority of chronic otitis media with effusion cases may yield viable bacteria (Post et al., 1995, 1996; Rayner et 1998; Aul et al., 1998). Studies on the role of bacteria in pathogenesis chronic otitis media with effusion have not been performed on children with cleft palate.
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TABLE 30.2. Classification of Otitis Media |
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FIG. 30.1. Progression of middle ear atelectasis to cholesteatoma. |
Persistence of eustachian tube dysfunction after palatoplasty is the primary basis for the development of more serious middle ear pathology in children with cleft palate (Bluestone and Klein, 1996a; Dominguez and Harker, 1988; Goldman et al., 1993; Muntz, 1993). Persistent eustachian tube dysfunction leads to chronic negative middle ear pressure. Chronic negative middle ear pressure leads to inward distention of the tympanic membrane. This may eventually cause irreversible tympanic membrane pathology, such as atelectasis (collapse), perforation, or cholesteatoma (epidermoid cyst) (Bluestone and Klein, 1996a). These conditions are thought to occur along a continuum, and atelectasis is considered a precursor to perforation and cholesteatoma (Fig. 30.1) (Sade and Berco, 1976; Wells and Michaels, 1983; Wolfman and Chole, 1986). If the tympanic membrane ruptures in response to the chronic negative middle ear pressure, a chronic tympanic membrane perforation may result. If the tympanic membrane remains intact, atelectasis will usually develop. Less commonly, atelectasis will progress to cholesteatoma formation.
Atelectasis of the pars tensa (Color Fig. 30.2) is commonly graded using the classification system of Sade and Berco (1976) (Fig. 30.1). Stage 1 involves tympanic membrane retraction toward the promontory with loss of its biconvex contour. Stage 2 involves further retraction of the pars tensa such that the tympanic membrane contacts the incus and/or the stapes. In stage 3, the tympanic membrane contacts the promontory but does not adhere to it. In stage 4, the tympanic membrane adheres to the promontory and ossicular damage is commonly seen. Attic retraction (Color Fig. 30.3) is graded similarly (Tos and Poulson, 1980). This is considered mild (stage 1) when retraction of the pars flaccida is not accompanied by erosion of the scutum (the superomedial tympanic ring). Moderate attic retraction (stage 2) is manifested by slight bone erosion (exposure of malleus neck). Severe retraction (stage 3) involves exposure of the malleus head.
A cholesteatoma (Color Fig. 30.4) results when skin that is sloughed from the atelectatic tympanic membrane begins to accumulate, forming an expansile cyst. Accumulation of debris in the middle ear space, in conjunction with body heat and moisture from the external ear, creates a nidus for chronic suppurative otitis media. Chronic or recurrent purulent drainage usually occurs. In some cases, no drainage may be seen. In most cases without apparent drainage, the drainage will dry over the defect in the tympanic membrane, giving the appearance of cerumen (Color Fig. 30.5).
Tympanic membrane perforation may lead to recurrent or chronic middle ear infection in one of two ways (Bluestone and Klein, 1996a). The defect in the tympanic membrane may allow contamination of the middle ear with water and pathogens from the ear canal.
Such an infection may be seen after an individual with a tympanic membrane perforation has been swimming. Alternatively, the defect in the tympanic membrane may allow reflux of nasopharyngeal secretions.
In contrast to the pathogens isolated from acute otitis media and otitis media with effusion, the bacteria isolated from chronic suppurative otitis media, with or without cholesteatoma, include Pseudomonas aeruginosa, anaerobes, Enterobacteriaceae, and Staphylococcus species (Kenna and Bluestone, 1986; Papastavros et al., 1986; Harker and Koontz, 1977). Experimental animal studies have indicated that middle ear infection with P. aeruginosa can lead to both conditions (Friedmann, 1955a,b; Kenna, 1988; Antonelli et al., 1988, 1992, 1993). In humans, antipseudomonal treatment can eradicate chronic suppurative otitis media without cholesteatoma (Kenna et al., 1986; Fliss 1990). However, chronic suppurative otitis media can be cured with tympanaplasty (repair of the tympanic membrane perforation and removal of the cholesteatoma), without the use of antibiotics. Hence, it is unclear whether bacteria are truly pathogenic (i.e., contribute to the development of tympanic membrane perforation or cholesteatoma) or merely colonize the middle ears with tympanic membrane perforations or cholesteatoma.
Hearing Loss and Cognitive Sequelae of Media
Chronic otitis media with effusion is clinically relatively silent. Few children with chronic otitis media with effusion have symptoms referable to the ear (Paradise et al., 1969). The most common clinical manifestation of chronic otitis media with effusion is conductive hearing loss (Paradise et al., 1969). Hearing loss due to otitis media with effusion averages 25 to 30 dB but may be as high as 50 dB (Cohen and Sade, 1972; Brown et al., 1983; Fria et al., 1985), yet many parents and treating physicians may be unaware that a child has hearing loss due to otitis media with effusion (Rosenfeld et al., 1998; Brody et al., 1999).
Though hearing loss due to otitis media with effusion is considered mild in children with normal inner ear function, children with chronic otitis media with effusion may experience greater problems in speech and language acquisition, cognitive development, and social maturation than children without it (Stewart et al., 1996; Silverman, 1996). Although a great deal of research continues to look for definitive evidence of lasting speech and cognitive sequelae of chronic otitis media with effusion, the data are conflicting or show only mild effects (Hubbard et al., 1985; Schilder et al., 1993; Welsh et al., 1996; Roberts et al., 1998). For example, Teele and colleagues (1990) found a significant, lasting effect of chronic otitis media with effusion on intelligence quotient, math skills, and verbal language scores; but these findings have not been corroborated in other well-controlled studies (Roberts et al., 1994). Roberts and colleagues (1995, 1998) have found the greatest correlation between chronic otitis mediarelated hearing loss and the quality of the home and day-care environments. Hence, the impact of hearing loss due to otitis media with effusion remains unclear.
Treatment of Ofifis Media with Effusion and Hearing Loss
Because intratemporal or intracranial complications of otitis media are rare (Bluestone and Klein, 1996b) and hearing loss may have a lasting impact on cognitive development, treatment of otitis media, most commonly chronic otitis media with effusion, has been directed at correcting the associated conductive hearing loss.
Speech and cognitive sequelae with much more severe (sensorineural) hearing loss can be minimized or prevented with appropriate early identification and rehabilitation (Yoshinaga-Itano et al., 1998). However, conductive hearing loss due to chronic otitis media with effusion, unlike sensorineural hearing loss, presents unique difficulties, principally as a result of its fluctuating nature. Even relatively brief periods of conductive hearing loss, in both childhood and adulthood, can have lasting effects on central auditory system processing (Gunnarson and Finitzo, 1991; Moore et al., 1999). Treatment for brief or transient conductive hearing loss, with either surgery or amplification, has not been advocated. Central auditory processing difficulties resolve slowly after resolution of bilateral chronic otitis media with effusion (Hall et al., 1995) or unilateral conductive hearing impairment (Hall and Derlacki, 1986; Magliulo et al., 1990; Hall and Grose, 1993; Snik et al., 1994; Wilmington et al., 1994). Because this has been found in both children and adults, adaptation following treatment is thought to be independent of critical periods for speech and language development (Hall et al., 1990; Ferguson et al., 1998).
Conductive hearing loss may be readily treated either surgically or with amplification. Unfortunately, no studies have compared language and cognitive development in children with chronic otitis media with effusion treated by amplification against those treated surgically.
Amplification has traditionally relied on the use of hearing aids. Fitting young children with hearing aids can be difficult because hearing loss due to chronic otitis media with effusion may fluctuate significantly and young children cannot provide the feedback necessary to readily handle such fluctuations. Although the use of hearing aids for hearing loss due to chronic otitis media with effusion requires frequent audiologic assessment and intervention, they are well tolerated by most children (Updike, 1994; Flanagan et al., 1996; Jardine et al., 1999). Alternative amplification strategies include the use of FM trainers, modification of environmental acoustics, sound-field amplification, and home stimulation language programs (Bergstrom, 1980; Bess et al., 1984; Crandell, 1993).
Surgical treatment of chronic otitis media with effusion involves myringotomy (incision through the tympanic membrane) and insertion of a tympanostomy tube through this incision, as described by Armstrong (1954). This bypasses the eustachian tube, allowing ventilation of the middle ear and clearance ear effusion. Clearance of middle ear effusion leads to correction of hearing impairment in the vast majority of children with chronic otitis media with effusion (Fria et al., 1987). The simplicity and prompt auditory benefits of tympanostomy tube placement have led to this procedure becoming the standard of care for chronic otitis media with effusion in the United States (Paradise, 1976; Robinson et al., 1992; Otitis Media Guideline Panel, 1994). The placement of tympanostomy tubes has been cited as the most commonly performed surgical procedure in the United States. Nearly one-third of children less than 2 years old are treated with tympanostomy tubes (Myer and France, 1997). Because chronic otitis media with effusion in children with cleft palate commonly persists for the first few years of life, even after palatoplasty, and tympanostomy tubes commonly extrude in a shorter period of time (Weigel et al., 1989), children with cleft palate who are managed with tympanostomy tubes commonly require multiple tympanostomy tube placement procedures (Muenker, 1980; Robinson et al., 1992; Muntz, 1993). Use of tympanostomy tubes has not been globally adopted as the standard of care. This is likely a function of the limited access to care in developing countries, the expense of surgical intervention, and the potential for sequelae due to tympanostomy tube placement.
A decrease in the incidence of irreversible tympanic membrane conditions (e.g., atelectasis and cholesteatoma) has been perceived by many clinicians. Most have related this decline to an increase in the use of tympanostomy tubes, but Roland et al. (1992) found no correlation between the use of tympanostomy tubes and the incidence of cholesteatoma. On the contrary, a number of studies have implicated tympanostomy tubes in the pathogenesis of middle ear pathology, such as atelectasis and tympanic membrane perforation (Moller, 1975, 1981; Sade and Berco, 1976; Skinner et al., 1988; Sederberg-Olsen, et al.,1988; Gordon et al., 1988; Le et al., 1991; Robson et al., 1992; Schilder, et al., 1993; Maw and Bawden, 1994). More specifically, the tympanostomy may serve as the nidus for the development of irreversible tympanic membrane pathology, particularly with long-term tube placement (Moller, 1981; Lildholdt, 1983; Gundersen et al., 1984; Mortensen and Lildholdt, 1984; Soderberg et al., 1986; Skinner et al., 1988; Le et al., 1991; Robinson et al., 1992; Robson et al., 1992; Maw and Bawden, 1994; Oluwole and Mills, 1996; Golz et al., 1999a,b). Oluwole and Mills (1996) reported that 70% of tympanic membrane perforations are tube-related. Similar associations have been drawn for cholesteatoma (Golz et al., 1999a).
Adverse middle ear outcomes appear to be more common in children treated with tympanostomy tubes than with myringotomy alone. Compared to treatment with tympanostomy tubes, chronic otitis media with effusion treated by myringotomy alone has been associated with a lower rate of myringosclerosis (deposition of hyalinized scar tissue within the lamina propria of the tympanic membrane or submucosally in the middle ear) (Sade and Berco, 1976). Le et al. (1991) found a significantly higher rate of tympanic membrane perforations in ears treated with tympanostomy tubes than with myringotomy alone. Thus, tympanostomy tubes do not protect against long-term adverse middle ear outcomes of eustachian tube dysfunction. Rather, they may increase the risk of their development.
Lildholdt (1983), Skinner and colleagues (1988), and Maw and Bawden (1994) performed long-term, controlled studies on the effects of tympanostomy tubes (with or without concomitant adenoidectomy) for chronic otitis media with effusion in children without cleft palate. All enrolled patients had bilateral chronic otitis media with effusion, but tympanostomy tubes were placed in only one ear. These authors assessed the incidence of several forms of tympanic membrane pathology observed in children with chronic otitis media with effusion, including segmental atrophy (loss of the lamina propria, the fibrous, strength layer) of the pars tensa, scarring and thickening of the pars tensa, atelectasis of the pars tensa, attic retraction, myringosclerosis, cholesteatoma, and tympanic membrane perforation. Though ears treated with tubes took less time to resolve the middle ear effusion, other problems were more common in these surgically treated ears. Problems such as myringosclerosis and segmental tympanic membrane atrophy were statistically significantly more common in ears treated with tympanostomy tubes as early as 3 years of age. Skinner et al. (1988) found that less common but more serious problems, such as cholesteatoma, were more common in ears treated with tympanostomy tubes (6.5% vs. 0%), but these differences were not statistically significant. Higher rates of myringosclerosis with the use of tympanostomy tubes have been reported (Sederberg-Olsen et al., 1988; Schilder et al., 1993).
Both Skinner et al. (1988) and Maw and Bawden (1994) treated many of their patients with concomitant adenoidectomy. Adenoidectomy has been shown to significantly impact the resolution of chronic otitis media with effusion and to minimize the recurrence of middle ear effusion, i.e., to minimize the need for repeat tympanostomy tube placement (Gates et al., 1987; Paradise et al., 1990). The benefits of adenoidectomy on chronic otitis media with effusion are greater than those of tympanostomy tubes (Gates et al., 1987; Paradise et al., 1990). The benefit of adenoidectomy is presumably due to its effects on tubal function and nasopharyngeal microflora (Bluestone and Klein, 1996a). Unfortunately, adenoidectomy is relatively contraindicated in children with cleft palate because it places them at an increased risk of velopharyngeal insufficiency (Haapanen et al., 1993). Only a single, small retrospective study has addressed the impact of adenoidectomy on otitis media with effusion in children with cleft palate (Severeid, 1972).
In light of the contraindication for adenoidectomy and the more lasting difficulties with eustachian tube dysfunction in children with cleft palate, it is not surprising that these children undergo more treatment with tympanostomy tubes than children without cleft palate (Muenker, 1980; Robson et al., 1992; Muntz, 1993). As standard tympanostomy tubes extrude or become nonfunctional in 1 to 2 years (Weigel et al., 1989), they are often repeatedly placed in children with cleft palate (Muenker, 1980; Robson et al., 1992). Though longeracting tympanostomy tubes are available, these are associated with significantly higher rates of serious tympanic membrane pathology, such as cholesteatoma and persistent tympanic membrane perforation (Weigel et al., 1989). Robson et al. (1992) found that children with cleft palate needed an average of 1.66 tympanostomy tubes, despite the use of long-acting tubes. Thus, children with cleft palate and chronic otitis media with effusion are at higher risk than children without cleft palate of experiencing any possible adverse outcomes related to tympanostomy tubes.
To date, only retrospective studies have addressed the long-term middle ear outcomes in children with cleft palate. Gordon et al. (1988) reported that myringosclerosis was significantly more common in the ears of cleft children with chronic otitis media effusion who had been treated with tympanostomy tubes. Robson et al. (1992) similarly found higher rates of otoscopic abnormalities in the ears of cleft children treated with tympanostomy tubes. Seagle et al. (1998) reported higher rates of otologic and audiologic problems in American children with cleft palate who had been treated with tympanostomy tubes than in Russian children with cleft palate who had not received tympanostomy tubes. Hence, some clinicians have long been advocating more limited use of tympanostomy tubes (Moller, 1981; Gundersen et al., 1984; Robinson et al., 1992; Robson et 1992). Use of tympanostomy tubes has been similarly questioned in other craniofacial disorders (e.g., Down syndrome) because of higher rates of such sequelae (lino et al., 1999). Unfortunately, the long-term value and consequences of tympanostomy tube placement in children with cleft palate and chronic otitis media with effusion are still not clearly understood. This issue has not been formally, prospectively studied in children with cleft palate and chronic otitis media with effusion.
Treatment of Chronic Suppurative Otitis Media
As with chronic otitis media with effusion, chronic otitis media with tympanic membrane perforation or cholesteatoma commonly manifests by hearing loss (Paparella and Schachern, 1984). However, untreated, cholesteatoma and chronic infection can spread into surrounding structures (e.g., the inner ear or cranium) with potentially life-threatening consequences (Sheehy and Brackmann, 1979; Kangsanarak et al., 1993; Burggraff et al., 1995). Simple procedures, such as tympanostomy tube placement, will not correct either of these conditions or their associated hearing loss. Middle ear reconstructive surgery (tympanoplasty with or without mastoidectomy), often requiring multiple procedures, is commonly necessary (lino et al., 1998; Stangerup et al., 1999; Silvola and Palva, 1999). Although surgical treatment is highly effective for preventing serious intratemporal and intracranial suppurative complications, hearing may remain compromised (Silvola and Palva, 1999). This can have a lifelong impact on quality of life (Mulrow et al., 1990).
Ideally, cholesteatoma should be identified as early as possible, before significant ossicular damage has been incurred and before ossicular removal would be necessary to eradicate the disease. Distinguishing cholesteatoma from end-stage atelectasis can prove challenging. Debris or drainage from a cholesteatoma pocket can look very similar to cerumen (Color Fig. 30.5). Cholesteatoma should be considered if there is any sign or symptom of recurrent or progressive middle ear inflammation (Table 30.3).
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TABLE 30.3. Clinical Findings Consistent with the Diagnosis of Cholesteatoma |
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Nose
The nasal airway is commonly compromised in individuals with oral clefts (Chaudhuri and Bowen-Jones, 1978; Warren et al., 1990; Ishikawa and Amitani, 1994). Nasal airway obstruction is generally not manifest until after cheiloplasty, palatoplasty, or pharyngoplasty (Orr et al., 1987; Josephson 1996; Witt et al., 1996). This can seriously affect spontaneous ventilation in obligate nasal breathers, such as young infants (Sculerati et al., 1994). Older individuals may manifest nasal airway obstruction in more subtle ways, such as obstructive sleep apnea (Nowak and Weider, 1998).
In most cases, the nasal obstruction is ipsilateral to the cleft palate, but both sides may be involved (Sandham and Murray, 1993; Wahlmam et al., 1998; Suzuki et al., 1999; Kunkel et al., 1999). Anatomic deformities include septal deviation (Suzuki et al., 1999), nasal valve stenosis (Wahlmam et al., 1998), posterior choanae (Wahlmam et al., 1998), ectopic teeth (Ranalli et al., 1990; Yeung and Lee, 1996), adenoid hypertrophy (Shapiro, 1982), and skull base encephalocele (Shimizu et al., 1999). Septoplasty and rhinoplasty are often helpful (Lowenthal, 1981), but results may be less than ideal because of midfacial developmental problems (Drake et al., 1993; Mooney et al., 1994; Anastassov et al., 1998).
Adenoid hypertrophy can obstruct the choanae of children with cleft palate. Adenoidectomy is relatively contraindicated in children with cleft palate because of the increased risk for velopharyngeal insufficiency (Pickrell et al., 1976; Croft et al., 1981; Witzel et al., 1986; Haapanen et al., 1993). However, a limited adenoidectomy (i.e., from high within the choanae) may significantly improve the nasal airway with a low rate of velopharyngeal insufficiency in selected patients (Shapiro, 1982; Kakani et al., 2000).
Rhinitis and sinusitis are also relatively common in individuals with cleft palate (Robinson et al., 1982; Ishikawa and Amitani, 1994). These conditions have historically been related to velopharyngeal insufficiency and reflux of pharyngeal contents into the nasal cavity (Robinson et al., 1982). Stasis of nasal secretions and rhinitis may develop if adenoid tissue or anatomic abnormalities obstruct the choanae. Rhinosinusitis in cleft patients has been attributed to mucociliary dysfunction (Ishikawa et al., 1989). Ishikawa and Amitani (1994) reported rhinosinusitis to be more common both in patients with cleft palate and in congenital (noncleft) velopharyngeal insufficiency relative to normal control subjects. Nasal obstruction and mucociliary dysfunction were significantly more common in cleft palate patients than in congenital velopharyngeal insufficiency subjects or normal controls. Hence, nasal obstruction, mucociliary dysfunction, and velopharyngeal insufficiency may all play a role in the pathogenesis of rhinosinusitis in individuals with cleft palate.
Throat
Recurrent tonsillitis and sleep apnea secondary to tonsillar hypertrophy are the most common indications for removal of the palatine tonsils in childhood. By virtue of their juxtaposition to the palate, tonsillar pathology can significantly impact palatal function and speech (Finkelstein et al., 1994). Though velopharyngeal insufficiency has been reported to be a complication of palatine tonsillectomy (Haapanen et al., 1994), D'Antonio and colleagues (1996) found no evidence of significant degradation of speech or velopharyngeal function following tonsillectomy in children with cleft palate. A number of reports have documented improvement in palatal function after removal of hypertrophied tonsils (MacKenzie-Stepner et al., 1987; Shprintzen et al., 1987; Kummer et al., 1993).
Palatine tonsillectomy may be necessary to accommodate other cleft palate procedures. Creation of a pharyngeal flap can seriously compromise the airway in the presence of tonsillar hypertrophy (Reath et al., 1987). Though tonsillectomy may be done prior to pharyngoplasty, they may be safely performed simultaneously (Reath et al., 1987; Eufinger and Eggeling, 1994; Eufinger et al., 1994).
Airway obstruction is the most serious of the otolaryngologic conditions involving individuals with oral clefts. Airway obstruction in individuals with cleft lip and palate, as discussed above, is commonly due to nasal abnormalities and adenotonsillar hypertrophy and may be aggravated following palatal or pharyngeal reconstruction. Obstructive sleep apnea may develop later in childhood, well after primary palatoplasty, as adenoid hypertrophy develops (Kiely et al., 1998; Nowak and Weider, 1998). Conservative management, such as continuous positive airway pressure ventilation, may be sufficient (Kiely et al., 1998), but adenotonsillectomy (Shapiro, 1982) or nasal reconstruction may be required (Lowenthal, 1981).
The pharyngeal airway is compromised in individuals with oral clefts (Smahel and Mullerova, 1992). Further compromise by associated anomalies leads to more serious airway compromise (Figueroa et al., 1991). Airway obstruction is of particular concern in patients with hypoplasia of the midface and mandible (Sculerati et al., 1998; Bath and Bull, 1997), as seen with Crouzon's, Treacher Collins, and Apert's syndromes as well as Robin sequence. Conservative measures, such as positioning and continuous positive airway pressure ventilation, may yield an adequate airway, but tracheotomy may be required (Olson et al., 1990; Caouette-Laberge et al., 1994; Lehman et al., 1995; Bath and Bull, 1997; Sculerati et al., 1998).
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