OBJECTIVES: This study examined the speech and hearing status of Malay-speaking children with CLP residing in Kuala Lumpur.
METHODS: Parents whose children were between the age of 5 and 7 years were recruited via the Cleft Lip and Palate Association of Malaysia (CLAPAM) registry. Parents completed a survey and the children completed a speech and hearing assessment at the Audiology and Speech Sciences Clinic, Universiti Kebangsaan Malaysia.
OUTCOMES: Speech measures include nasality rating, nasalance scores, articulation errors and speech intelligibility rating, while hearing measures include hearing thresholds and tympanometry results for each child.
RESULTS: Out of 118 registered members who fulfilled the inclusion criteria, 21 agreed to participate in the study. The overall speech and hearing status of children in this sample were poor. Only four (19%) participants had normal speech intelligibility rating and normal hearing bilaterally. In terms of overall cleft management, only four (19%) participants were seen by a cleft team while seven (33%) had never had their hearing tested prior to this study.
CONCLUSION: Participants in this sample had poor outcomes in speech and hearing and received uncoordinated and fragmented cleft care. This finding calls for further large scale research and collaborative efforts into improving and providing centralised, multidisciplinary care for children born with CLP.
RESULTS: A significant nonparametric linkage (NPL) score was detected in family 100. Other suggestive NPL and logarithm of the odds (LOD) scores were attained from families 50, 58, 99 and 100 under autosomal recessive mode. Heterogeneity LOD (HLOD) score ≥ 1 was determined for all families, confirming genetic heterogeneity of the population and indicating that a proportion of families might be linked to each other. Several candidate genes in linkage intervals were determined; LPHN2 at 1p31, SATB2 at 2q33.1-q35, PVRL3 at 3q13.3, COL21A1 at 6p12.1, FOXP2 at 7q22.3-q33, FOXG1 and HECTD1 at 14q12 and TOX3 at 16q12.1.
CONCLUSIONS: We have identified several novel and known candidate genes for nonsyndromic cleft lip and/or palate through genome-wide linkage analysis. Further analysis of the involvement of these genes in the condition will shed light on the disease mechanism. Comprehensive genetic testing of the candidate genes is warranted.
METHOD: A prospective analysis was conducted on 173 patients (346 ears) with cleft lip and palate (CL/P) who presented to the combined cleft clinic at University Malaya Medical Centre (UMMC) over 12 months. The patients' hearing status was determined using otoacoustic emission (OAE), pure tone audiometry (PTA) and auditory brainstem response (ABR). These results were analysed against several parameters, which included age, gender, race, types of cleft pathology, impact and timing of repair surgery.
RESULTS: The patients' age ranged from 1-26 years old. They comprised 30% with unilateral cleft lip and palate (UCLP), 28% with bilateral cleft lip and palate (BCLP), 28% with isolated cleft palate (ICP) and 14% with isolated cleft lip (ICL). Majority of the patients (68.2%) had normal otoscopic findings. Out of the 346 ears, 241 ears (70%) ears had passed the hearing tests. There was no significant relationship between patients' gender and ethnicity with their hearing status. The types of cleft pathology significantly influenced the outcome of PTA and ABR screening results (p
METHOD: A total of one hundred and seven patients from age five to twelve years old with non-syndromic unilateral cleft lip and palate were included in the study. These patients have received cheiloplasty and one stage palatoplasty surgery but yet to receive alveolar bone grafting procedure. Five assessors trained in the use of the EUROCRAN index underwent calibration exercise and ranked the dental arch relationships and palatal morphology of the patients' study models. For intra-rater agreement, the examiners scored the models twice, with two weeks interval in between sessions. Variable factors of the patients were collected and they included gender, site, type and, family history of unilateral cleft lip and palate; absence of lateral incisor on cleft side, cheiloplasty and palatoplasty technique used. Associations between various factors and dental arch relationships were assessed using logistic regression analysis.
RESULT: Dental arch relationship among unilateral cleft lip and palate in local population had relatively worse scoring than other parts of the world. Crude logistics regression analysis did not demonstrate any significant associations among the various socio-demographic factors, cheiloplasty and palatoplasty techniques used with the dental arch relationship outcome.
CONCLUSIONS: This study has limitations that might have affected the results, example: having multiple operators performing the surgeries and the inability to access the influence of underlying genetic predisposed cranio-facial variability. These may have substantial influence on the treatment outcome. The factors that can affect unilateral cleft lip and palate treatment outcome is multifactorial in nature and remained controversial in general.
MATERIALS AND METHODS: One hundred and one sets of dental models of patients having CUCLP were assessed in this retrospective study. Five examiners that were blinded to case-specific information scored the dental models at two instances with an interval of two weeks to ensure memory bias elimination (5 × 101 × 2 = 1010 observations). Calibration courses were conducted prior to scoring and each examiner was provided with scoring sheets, pictures of GOSLON reference models and flowcharts explaining the scoring method.
RESULTS: According to GOSLON index, a mean (SD) GOSLON score of 3.04 (1.25) was determined. Based on treatment outcome groups, 62 patients had favorable (grade 1, 2, and 3) and 39 cases had unfavorable (grade 4 and 5) treatment outcome. Chi-square tests revealed a significant association of gender (P = 0.002), cheiloplasty (P = 0.001) and palatoplasty (P
MATERIALS AND METHODS: This study is conducted at Hospital Universiti Sains Malaysia. UCLP group comprised 48 patients with nonsyndromic UCLP who have had the lip and palate repaired, whereas the control group comprised 48 healthy noncleft cases. The lateral cephalometrics measurements were used to determine the vertical height, sagittal depth of the face, and cranial base length and angle. Maxillary arch dimensions were measured on the study cast including arch width, depth, and length.
RESULTS: Vertical facial height and sagittal depth measurements showed a significant decrease (P < 0.05) in the mean growth pattern in UCLP group. The anterior cranial base length (S-N) was shorter in UCLP children (P < 0.001), while Ba-N length had no significant difference (P = 0.639). Nasion-Sella Tursica-Basion angle was significantly higher in the UCLP group (P = 0.016). Dental arch width with reference to canine-to-canine and first premolar-to- first premolar distance was significantly larger in control (P = 0.001).
CONCLUSION: Mean vertical and sagittal facial dimensions in the UCLP children who do not undergo orthodontic treatment are significantly lesser in all directions of growth than healthy noncleft children. The maxillary dental arch had a normal depth but constricted in width and arch length.
METHODS: Dental models of 84 subjects were taken before orthodontic treatment and alveolar bone grafting. The mean age was 7.69 (SD 2.46) years. The DAR and PM were assessed blindly by five raters using the EUROCRAN index (EI). Kappa statistics was used to evaluate the intra- and inter-examiner agreement, chi square was used to assess the associations, and logistic regression analysis was used to explore the responsible factors that affect DAR and PM.
RESULTS: The mean EUROCRAN scores were 2.44 and 1.93 for DAR and PM, respectively. Intra- and inter-examiner agreement was moderate to very good. Using crude and stepwise backward regression analyses, significant associations were found between the modified Millard technique (P = 0.047, P = 0.034 respectively) of cheiloplasty and unfavorable DAR. Complete UCLP (P = 0.017) was also significantly correlated with unfavorable DAR. The PM showed a significant association with the type of cleft, type of cheiloplasty and type of palatoplasty.
CONCLUSION: This multivariate study determined that the complete type of UCLP and the modified Millard technique of cheiloplasty had significantly unfavorable effects on both the DAR and PM.
MATERIALS AND METHODS: It is a retrospective cross sectional study. Dental models were collected from archives of two cleft referral centers in Pakistan. Five blinded examiners scored 101 models twice at two week interval. The primary outcome was mean EUROCRAN scores based on dental arch relationships and palatal surface morphology.
RESULTS: A mean(SD) score of 2.72 (0.76) and 2.20 (0.73) was determined based on dental arch relationships and palatal surface morphology, respectively. According to the final logistic regression model, modified Millard technique (cheiloplasty) and Veau-Wardill-Kilners' method (palatoplasty) had higher odds of producing unfavorable treatment outcome.
CONCLUSIONS: Present study determined a fair and a fair to poor treatment outcome based on dental arch relationships and palatal surface morphology, respectively. Our study suggests a significant association between treatment outcome and primary surgical techniques for lip and palate. These findings could warrant a modification of management protocols to ensure improvement in future cleft outcomes.