Reijo Ranta

The first neurophysiological evidence for cognitive brain dysfunctions in children with Catch

CATCH syndrome, caused by a microdelection in chromosome 22, is characterized by cleft palate and cardiac anomalies. The majority of these children also have learning difficulties or speech and language deficits. These problems are often due to the dysmorphology of the articulatory system. In the present study, the duration of auditory sensory memory, which is of central importance to speech perception and understanding, was investigated. As a research method we used mismatch negativity (MMN), an attention independent eventrelated potential, which provides an objective electrical index of auditory sensory memory. The present data suggest that the duration of this memory span is considerably shorter in 6–10-year-old children with CATCH than in healthy controls. Thus, the language-related problems encountered in children suffering from CATCH syndrome are likely to be caused also by CNS dysfunctions.

Cortical auditory dysfunction in children with oral clefts: relation with cleft type

OBJECTIVEnUp to 46% of individuals with oral clefts suffer from language-learning disabilities. The degree of these disabilities varies according to cleft type. The pathogenesis of cognitive malfunctioning or its relationship with cleft type is not known. We investigated persistence of auditory short-term memory (STM) that is implicitly involved in language-specific perception in children with clefts, grouped using fine-graded cleft classification.nnnMETHODSnCortical evoked potentials were recorded in 78 children with non-syndromic oral clefts and in 32 healthy peers. A mismatch negativity (MMN) potential that indexes preattentive detection of change in auditory input was obtained in response to tone sounds. In order to test durability of short-term memory traces, sounds were presented with three stimulation rates.nnnRESULTSnWith slowest stimulation, MMN amplitudes were reduced in cleft children as compared to the healthy peers (P < 0.00065). Only cleft-lip children did not significantly differ from controls. Among isolated palatal clefts, the more posteriorly delimited the cleft was, the smaller was the amplitude of MMN. MMNs of smallest amplitudes were obtained in the subgroup of complete unilateral cleft of lip and palate.nnnCONCLUSIONSnReduced MMN amplitudes, found in cleft children, imply deficiency in auditory STM trace maintenance. This dysfunction is likely to contribute to their language and learning disabilities. The MMN diminution with shorter/more posterior clefts suggests that differences in auditory cortex function are one of the underlying mechanisms of the cleft type-malcogniton association.

Cephalometric Measurements in Patients with Pierre Robin Syndrome and Isolated Cleft Palate

To compare craniofacial structure and growth, two standard lateral cephalograms of 35 children with Pierre Robin syndrome and 30 age and sex matched children with isolated cleft palate were taken at two different ages. The mean (SD) age of the children with Pierre Robin syndrome was 9.5 (3.0) years and of the children with isolated cleft palate 7.1 (1.1) years at the start of the follow up period. The follow up time in the first group was 4.3 (1.6) and in the second group 3.8 (2.7) years. There were no significant differences between the groups in the shape of the cranial base or the maxilla. The main differences were in the mandible, which showed more recession among those with Pierre Robin syndrome. There was a significant correlation between the shape of the maxilla and that of the mandible in that group, but not in the group with isolated cleft palate. During the follow up period there were no significant differences between the two groups in the rate of growth of the jaws. The association between the maxilla and the mandible (ss-n-sm) remained unchanged, or increased, or decreased with equal frequency in both groups. There was no catch up mandibular growth among the children with Pierre Robin syndrome.

Dental abnormalities in permanent dentition in children with submucous cleft palate.

Seventy‐three children with submucous cleft palate (38 girls and 35 boys), mean age 8.2 years (range 7.7–9.5), were studied retrospectively from orthopantomograms. Dental abnormalities in permanent dentition were found in 26 patients (36%). Missing teeth, mainly lower 2nd premolars, upper lateral incisors, and upper 2nd premolars, were found in 12 patients (16%). Most of the patients had 1 or 2 missing teeth, 2 had 3 missing teeth. In 5 patients hypodontia was associated with another dental abnormality. Other dental abnormalities included peg‐shaped lateral incisors in 7 patients (10%), ectopic eruption of upper 1st molars in 6 patients (8%), transposition of upper canines and 1st premolars in 3 patients (4%), supernumerary teeth in 2 patients (3%), and palatally displaced upper canines in 1 patient (1%). As children with submucous cleft palate have a tendency towards increased frequency of missing teeth and other dental abnormalities, the need for thorough clinical and radiological dental examination is emphasized.

Velopharyngeal function from the age of three to eight years in cleft palate patients

The purpose of this investigation was to study changes of velopharyngeal function between the ages of 3 and 8 years. The subjects were 65 (30 girls and 35 boys) Finnish-speaking non-syndromic children with isolated cleft palate (CP, n = 35) and with unilateral cleft lip and palate (UCLP, n = 30) operated primarily at the age of 1.0–2.0 years. Before the age of 8 years, 16 children required velopharyngoplasty (VPP, ad modum Hoenig). The children were followed up for speech at the age of 3, 6 and 8 years. The perceptual speech characteristics nasal air emission, hypernasality, weakness of pressure consonants and compensatory articulations were registered. Indications for a velopharyngeal flap (by VPP) were identified on the basis of perceptual speech characteristics and confirmed by instrumental examinations. The results indicated that the method and timing of primary palatoplasty and sex did not correlate with the quality of velopharyngeal function. It was good both in children treated conservatively or with VPP at the age of 8 years. The children with a flap required speech therapy significantly more often than other children. No child with VPP and only 12% of the children without VPP had simultaneous nasal air emissions and hypernasality. Compensatory articulation was completely eliminated and weakness of pressure consonants was diagnosed only in 1 child without VPP. The CP children required significantly more often a velopharyngeal flap than the UCLP children. In conclusion, the CP and UCLP children develop a similar velopharyngeal function but in a different way.

Velopharyngeal Function in Cleft Patients Undergoing Maxillary Advancement

Maxillary advancement may result in movement of the posterior border of the hard palate with its soft palate attachment, which may cause impairment of velopharyngeal (VP) function. We examined VP function before and after Le Fort I osteotomy in 15 cleft lip and palate patients. The extent of maxillary advancement was measured by means of standard cephalometric radiographs taken before and after the operation. VP function was evaluated in terms of perceptual speech assessments, pressure-flow data and nasalance scores preoperatively and 2, 6 and 12 months after the operation. The results showed that maxillary advancement resulted in impairment of VP function in 4 (27%) of the patients.

Cephalometric pharyngeal changes after Le Fort I osteotomy in patients with unilateral cleft lip and palate

Pharyngeal changes after Le Fort I osteotomy were evaluated cephalometrically in 37 patients (27 M, 10 F; mean age 23.8 years) with unilateral cleft lip and palate (UCLP). Seven patients had previously undergone velopharyngeal (VPP) flap surgery to improve speech. One year postoperatively the patients without previous VPP showed a significant change (55%-85% of the surgical change) in the upper and lower sagittal depth of the nasopharyngeal airway, but not in the depth of oropharyngeal airway, length of soft palate, or position of hyoid bone. No significant changes were observed between 6 months and 1 year postoperatively. Mean surgical horizontal advancement was 4.7 mm and the mean vertical lengthening 4.7 mm anteriorly and 1 mm posteriorly. There was a correlation between the amount of horizontal advancement and the amount of change in the nasopharyngeal airway. The patients with previous VPP showed significant postoperative change (85% of the surgical change) only in the lower nasopharyngeal airway, although their surgical advancement was similar to that without previous VPP. Patients with previous VPP showed significantly smaller depths of upper nasopharyngeal airway postoperatively than the patients without previous VPP. Five patients (13%) needed VPP after the osteotomy. There was no difference in the nasopharyngeal airway in the patients with VPP after the osteotomy when compared to those without, but they seemed to have shortest maxillary lengths and largest mean surgical changes vertically both anteriorly (5.5 mm) and posteriorly (2.3 mm). Moderate maxillary advancement in UCLP patients results in significant changes in the nasopharyngeal airway.

Occurrence of Dental Consonant Misarticulations in Different Cleft Types

To study the occurrence and type of misarticulations in dental consonants /r/, /s/ and /l/ 280 (115 girls, 165 boys) 6-year-old cleft children were examined by 1 of the 2 experienced speech pathologists of the cleft team. The patients included 82 children with isolated cleft palate (CP), 82 with cleft lip with (34) or without (48) cleft alveolus [CL(A)], 85 with unilateral cleft lip and palate (UCLP) and 31 with bilateral cleft lip and palate (BCLP). CP children were first divided into subgroups; there were 17 children with soft palate cleft, 49 with partial and 16 with complete hard palate cleft. All patients were native Finnish speakers, and had normal hearing, no known syndrome or associated anomalies possibly affecting speech or psychomotor retardation. The results showed that the occurrence and severity as well as the number of errors of all studied sounds separately or grouped increased with the severity of the cleft being constantly greatest in the BCLP group and lowest in the CL(A) group. Altogether 44% of the patients misarticulated at least one studied sound; 41% distorted and 5% substituted, and 2% both distorted and substituted. The /r/ sound was misarticulated by 36%, the /s/ sound by 23%, and the /l/ sound by 18% of the patients. Boys tend to have more problems in producing the studied sounds correctly.

One-Stage Closure of Isolated Cleft Palate with the Veau-Wardill-Kilner v to y pushback Procedure or the Cronin Modification I. Comparison of Operative Results

The long term operative results of one-stage closure of isolated cleft palate with either the Veau-Wardill-Kilner V to Y pushback procedure or the Cronin modification were evaluated and compared retrospectively. A total of 116 consecutive patients with isolated cleft palate who had been operated on at the age of 18-24 months were followed up until 17-20 years of age. Twenty-four of the patients needed one or more additional operations on the palate, mainly pharyngeal flaps (20%) and repair of fistulas (10%). There was no significant difference in the number of patients who needed further operations, either between the two different operations or between the sexes, but the patients who presented with the most extensive clefts required the most operations.

Minimal cleft lip: Comparison of associated abnormalities

The literature relevant to minimal forms of cleft lip (CL(M)) has been reviewed. CL(M) has been defined as a cleft which does not extend past the vermilion. 20 patients with CL(M) (18 unilateral, 2 bilateral) were studied. Clinical and roentgenological findings from the lip, nose, alveolar ridge, teeth and palate were compared. All patients had some degree of nasal deformity; 13 of 15 patients had dental and 6 of 20 alveolar ridge deformities. Among 20 patients, there was 1 bifid uvula, 2 submucous cleft palate, and 1 cleft of the soft palate. 3 patients had several other abnormalities. A positive family history of clefts in relatives was noted in 2 cases. The severity of the dento-alveolar deformity did not seem to correlate with the severity of the nasal or the lip deformity, but the nasal deformity directly correlated with the lip deformity. Thus, the present CL(M) patients can be regarded as having actual clefts, although some may be normal variations.