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Mutation screening of IRF6 among families with non-syndromic oral clefts and identification of two novel variants: review of the literature

Salahshourifar I, Wan Sulaiman WA, Halim AS, Zilfalil BA

Eur J Med Genet, 2012 Jun;55(6-7):389-93.
PMID: 22440537 DOI: 10.1016/j.ejmg.2012.02.006

Non-syndromic oral clefts share the main clinical features of Van der Woude Syndrome (VWS), with the exception of the lower lip pit. Thus, about 15% of VWS cases are indistinguishable from cases with non-syndromic oral clefts. IRF6 mutations are the major cause of VWS; however, variants in this gene show strong association with non-syndromic oral clefts, with a higher increased risk among cases with cleft lip only (CLO). A total of 39 individuals, including 16 patients with CLO and 23 patients with a family history of cleft, were examined for IRF6 mutations in the present study. Seven variants, including five known (c.-75-4 A>; G, c.-73T>; C, c.459G>; T 5, c.820G>; A, and c.1060 + 37C>; T) and two novel (c.-75-23G>; C and c.1380G>; T), were found. Both novel variants were inherited from non-affected parents and we did not find also in the 120 control chromosomes. In silico analysis revealed that both c.1380G>; T and c.-75-23G>; C variants may disrupts a putative exonic splicing enhancer and intronic splicing binding site for SC35, respectively. Taken together, the presence of deleterious IRF6 variants in patients with non-syndromic oral clefts could be most likely an evidence for VWS. While, IRF6 variants could, at best, contribute to clefting as part of a complex inheritance pattern, with both additional genes and environmental factors having a role.

Matched MeSH terms: Cleft Palate/genetics*
Multivariate analysis on unilateral cleft lip and palate treatment outcome by EUROCRAN index: A retrospective study

Yew CC, Alam MK, Rahman SA

Int J Pediatr Otorhinolaryngol, 2016 Oct;89:42-9.
PMID: 27619027 DOI: 10.1016/j.ijporl.2016.07.026

OBJECTIVES: This study is to evaluate the dental arch relationship and palatal morphology of unilateral cleft lip and palate patients by using EUROCRAN index, and to assess the factors that affect them using multivariate statistical analysis.
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.

Matched MeSH terms: Cleft Palate/surgery*
Multiple milia in a newborn with congenital malformations: oral-facial-digital syndrome type 1

Nanda A, Sharaf A, Alsaleh QA

Pediatr Dermatol, 2011 4 22;27(6):669-70.
PMID: 21510010

Oral-facial-digital syndrome type 1 (OMIM #311200) is an X-linked dominant, developmental disorder. Among the 13 described clinical variants of oral-facial-digital syndrome, oral-facial-digital syndrome type 1 is of significance to dermatologists due to presence of congenital milia and hypotrichosis, not described in other variants. Since oral-facial-digital syndrome type 1 is genetically a distinct entity, awareness of these features help to clinically delineate this from other variants.

Matched MeSH terms: Cleft Palate/pathology*
Modified Activation Technique for Nasal Stent of Nasoalveolar Molding Appliance for Columellar Lengthening in Bilateral Cleft Lip/Palate

Patil PG, Nimbalkar-Patil SP

J Prosthodont, 2018 Jan;27(1):94-97.
PMID: 27002917 DOI: 10.1111/jopr.12464

Bilateral cleft lip/cleft palate is associated with nasal deformities typified by a short columella. The presurgical nasoalveolar molding (NAM) therapy approach includes reduction of the size of the intraoral alveolar cleft as well as positioning of the surrounding deformed soft tissues and cartilages. In a bilateral cleft patient, NAM, along with columellar elongation, eliminates the need for columellar lengthening surgery. Thus the frequent surgical intervention to achieve the desired esthetic results can be avoided. This article proposes a modified activation technique of the nasal stent for a NAM appliance for columellar lengthening in bilateral cleft lip/palate patients. The design highlights relining of the columellar portion of the nasal stent and the wire-bending of the nasal stent to achieve desirable results within the limited span of plasticity of the nasal cartilages. With this technique the vertical taping of the premaxilla to the oral plate can be avoided.

Matched MeSH terms: Cleft Palate/surgery*
Maternal uniparental heterodisomy of chromosome 6 in a boy with an isolated cleft lip and palate

Salahshourifar I, Halim AS, Sulaiman WA, Zilfalil BA

Am J Med Genet A, 2010 Jul;152A(7):1818-21.
PMID: 20583164 DOI: 10.1002/ajmg.a.33526

We describe a chromosome 6 uniparental disomy (UPD6) in a boy, discovered during a screening for the genetic cause of cleft lip and palate. In the medical literature, almost all documented cases of UPD6 are paternal in origin, and only four were maternal. We present here a report of complete maternal chromosome 6 uniparental heterodisomy. Haplotype analysis was performed using highly polymorphic short tandem repeat (STR) markers that span both arms of chromosome 6. Analysis of these markers revealed the presence of two maternal alleles but no paternal allele, indicating an instance of maternal uniparental heterodisomy. Chromosome analysis of peripheral blood lymphocytes confirmed a normal male karyotype. Advanced maternal age at the time of the infant's birth and heterodisomy of markers around the centromere favors a meiosis-I error. No specific phenotype has been reported for maternal UPD6. Therefore, the cleft lip and palate in the present case probably occurred due to other risk factors. This report provides further evidence that maternal UPD6 has no specific clinical consequences and adds to the collective knowledge of this rare chromosomal finding.

Matched MeSH terms: Cleft Palate/genetics*
Fulltext Mandibular advancement by distraction Osteogenesis for obstructive sleep apnea in patient with Pierre Robin Sequence: a case report

Mohamed Hisham Mohamed Jali @ Yunos, Shaifulizan Abdul Rahman, Ramizu Shaari

International Medical Journal Malaysia, 2017;16(21):28-28.
MyJurnal

Pierre Robin Sequence (PRS) is a condition consists of set of anomalies, which are
cleft palate, micrognathia and glossoptopsis. Management of patients with PRS addresses two main
problems, namely airway obstruction and feeding difficulties. Airway obstruction may lead to
obstructive sleep apnea (OSA). Treatment modalities for OSA are based on the causes. There are
surgical and non-surgical methods. Non-surgical methods such as diet, medication, oral appliances
and continuous positive airway pressure (CPAP) can only be employed in moderate cases. Surgical
method such as maxillo-mandibular advancement or expansion can be achieved by orthognathic
surgery or distraction osteogenesis. (Copied from article).

Matched MeSH terms: Cleft Palate
Management of velopharyngeal insufficiency by modified Furlow palatoplasty with pharyngeal flap: a retrospective outcome review

Wong LS, Lim E, Lu TC, Chen PKT

Int J Oral Maxillofac Surg, 2019 Jun;48(6):703-707.
PMID: 30755357 DOI: 10.1016/j.ijom.2019.01.010

The surgical approach for the correction of residual velopharyngeal insufficiency requiring secondary surgery at Chang Gung Memorial Hospital is the modified Furlow palatoplasty with pharyngeal flap (mFP-PF). The aim of this study was to describe the mFP-PF technique and to determine the results obtained with regard to improvements in velopharyngeal function in patients undergoing this surgery. This retrospective analysis included 58 non-syndromic patients treated during the period 1992-2015 who complained of hypernasal speech after primary cleft palate repair and failed postoperative speech therapy. All of them underwent mFP-PF surgery. Preoperative and postoperative perceptual speech assessment results were obtained. The male to female ratio in the study group was 1.2:1, and the mean patient age at the time of surgery was 8.27 years. The patients underwent nasoendoscopic examination and the velar closing ratio was categorized as 0.1-0.4 in 53.4% and 0.5-0.7 in 46.6%. The assessment of speech after mFP-PF showed statistically significant changes for all perceptual speech outcomes. The incidence of repeat surgery was 3.4%. This study revealed that 96.6% of patients did not require second surgery for velopharyngeal insufficiency. Further studies on obstructive sleep apnoea in post-mFP-PF patients and improvements to the surgical technique should be considered.

Matched MeSH terms: Cleft Palate*
Fulltext Management of otitis media with effusion in children

Zulkiflee S, Siti Sabzah MH, Philip R, Md Aminuddin MY

Malays Fam Physician, 2013;8(2):32-35.
PMID: 25606279 MyJurnal

Otitis media with effusion (OME) is a condition characterised by a collection of fluid within the middle ear without signs of acute inflammation. It is common in young children, with a bimodal peak at two and five years of age. Eighty percent of children have at least one episode of OME by the age of 10 years. This disease is a common ear problem among children with craniofacial anomalies including cleft palate and Down syndrome (DS).

Matched MeSH terms: Cleft Palate
Inhibitive behaviour of corrosion of aluminium alloy in NaCl by mangrove tannin

Solhan Yahya, Afidah Abdul Rahim, Affaizza Mohd Shah, Rohana Adnan

Sains Malaysiana, 2011;40.

Anticorrosion potential of mangrove tannins on aluminium alloys AA6061 in NaCl solution has been studied using potentiodynamic polarisation method and scanning electron microscopy (SEM). The study was carried out in different pH of corrosive medium in the absence and presence of various concentrations of tannin. The corrosion inhibition behaviour of the mangrove tannin on AA6061 aluminium alloy corrosion was found to be dependant on the pH of NaCl solution. Our results showed that the inhibition efficiency increased with increasing tannins concentration in chloride solution at pH 6. Treatment of aluminium alloy 6061 with all concentrations of mangrove tannins reduced the current density, thus decreased the corrosion rate. Tannins behaved as mixed inhibitors at pH 6 and reduction in current density predominantly affected in cathodic reaction. Meanwhile, at pH 12, addition of tannins shifted the corrosion potential to more cathodic potentials and a passivating effect was observed in anodic potentials. SEM studies have shown that the addition of tannins in chloride solution at pH 12 reduced the surface degradation and the formation of pits.

Matched MeSH terms: Cleft Palate
Fulltext Incidence and management of middle ear effusion in cleft palate patients

Lokman S, Loh T, Said H, Omar I

Med J Malaysia, 1992 Mar;47(1):51-5.
PMID: 1387450

For a complete overall rehabilitation of cleft palate patients a multi-disciplinary approach should be adopted. Plastic and Head and Neck Surgeons in whom most of the treatment are entrusted should be concerned not only at achieving palatal function and cosmetic acceptability but also the various other problems associated with cleft palate especially hearing loss. In this study, 66 patients with repaired and unrepaired cleft palates were examined for the presence of hearing loss due to middle ear effusion. The incidence of middle ear effusion was high (57.6%). It was also found that only eight of these patients (12.1%) ever complained of hearing loss or any associated symptoms and repair of the cleft palate did not influence the incidence of middle ear effusion. As such, screening should be done in all cleft palates and otolaryngologists should therefore play an important role in the multi-disciplinary team which should comprise the paediatrician, plastic surgeon, speech therapist, orthodontist and dental specialist.

Matched MeSH terms: Cleft Palate/complications*
Incidence and management of cleft lip and palate in Pakistan

Sharif F, Mahmood F, Azhar MJ, Asif A, Zahid M, Muhammad N, et al.

J Pak Med Assoc, 2019 May;69(5):632-639.
PMID: 31105281

OBJECTIVE: To compare the occurrence, distribution and management of clefts of lip and palate in local patients with the available data from India and China.
METHODS: The retrospective study was conducted at the Interdisciplinary Research Centre in Biomedical Materials, COMSATS University Islamabad, Lahore Campus, Lahore, Pakistan, and comprised data related to a three-month period from January to March 2015 at two medical centres in Lahore. Data from Pakistani centres was analysed based on province, gender, age and clefts of lip and palate conditions and Spearman's correlation matrix.
RESULTS: Of the 1574 cases, 1061(67.4%) were from Punjab, 361(23%) Khyber Pakhtunkhwa, 85(5%) Sindh and 67(4.2%) were from Azad Jammu and Kashmir. The incidence of clefts of lip and palate was higher in males than females. There was higher awareness of the need for timely management in new borns with clefts of lip and palate. Some patients seeking secondary treatment were also being surgically corrected. There is no national registry of children born with cleft defect, making it difficult to assess the full scale of the problem..
CONCLUSIONS: Based on available data, it is likely that there are many adults who have not been treated when younger..

Matched MeSH terms: Cleft Palate/epidemiology*; Cleft Palate/surgery
Identification of Copy Number Variation Among Nonsyndromic Cleft Lip and or Without Cleft Palate With Hypodontia: A Genome-Wide Association Study

Ghazali N, Abd Rahman N, Ahmad A, Sulong S, Kannan TP

Front Physiol, 2021;12:637306.
PMID: 33732167 DOI: 10.3389/fphys.2021.637306

Nonsyndromic cleft lip and or without cleft palate (NSCL/P) with the hypodontia is a common developmental abnormality in humans and animals. This study identified the genetic aberration involved in both NSCL/P and hypodontia pathogenesis. A cross-sectional study using genome-wide study copy number variation-targeted CytoScan 750K array carried out on salivary samples from 61 NSCL/P and 20 noncleft with and without hypodontia Malay subjects aged 7-13 years old. Copy number variations (CNVs) of SKI and fragile histidine triad (FHIT) were identified in NSCL/P and noncleft children using quantitative polymerase chain reaction (qPCR) as a validation analysis. Copy number calculated (CNC) for each gene determined with Applied Biosystems CopyCaller Software v2.0. The six significant CNVs included gains (12q14.3, 15q26.3, 1p36.32, and 1p36.33) and losses (3p14.2 and 4q13.2) in NSCL/P with hypodontia patients compared with the NSCL/P only. The genes located in these regions encoded LEMD3, IGF1R, TP73, SKI, FHIT, and UGT2β15. There were a significant gain and loss of both SKI and FHIT copy number in NSCL/P with hypodontia compared with the noncleft group (p < 0.05). The results supported that CNVs significantly furnish to the development of NSCL/P with hypodontia.

Matched MeSH terms: Cleft Palate
Hypernasality in singing among children with cleft palate: a preliminary study

Peter S, Abdul Rahman ZA, Pillai S

Int J Oral Maxillofac Surg, 2019 Oct;48(10):1317-1322.
PMID: 31014926 DOI: 10.1016/j.ijom.2019.03.896

The aim of this study was to document differences in hypernasality during speaking and singing among children with cleft palate and to compare nasality score ratings of trained and untrained listeners. Twenty subjects with cleft palate aged between 7 and 12 years participated in this study. Audio recordings were made of the children reading a passage and singing a common local song, both in the Malay language. The degree of hypernasality was judged through perceptual assessment. Three trained listeners (a speech therapist, a classical singer, and a linguistic expert - all academicians) and two untrained listeners (a cleft volunteer worker and a national high school teacher) assessed the recordings using a visual analogue scale (VAS). Inter-rater and intra-rater reliability for hypernasality in both speaking and singing were verified using the intra-class correlation coefficient (ICC). A significant reduction in hypernasality was observed during singing as compared to speaking, indicating that hypernasality reduces when a child with cleft palate sings. The act of singing significantly reduces hypernasality. The outcome of this study suggests that children with cleft palate would benefit from singing exercises to ultimately reduce hypernasality. However, future research is needed to objectively measure nasality in singing compared to speaking.

Matched MeSH terms: Cleft Palate*
Holoprosencephaly in neonates

Ram SP, Noor AR, Mahbar Z, Krishna TN

Int J Pediatr Otorhinolaryngol, 1994 Mar;29(1):65-71.
PMID: 8169049

A single nostril associated with alobar holoprosencephaly is a rare congenital lesion. This paper reports two female term neonates with holoprosencephaly. The first neonate asphyxiated at birth had a single nostril, hypotelorism, posterior cleft palate, inferonasal coloboma of the iris and disc and persistent tunica vasculosa lentis. The other neonate had cleft lip and palate and recurrent convulsions. Both neonates had gross motor and developmental delay. Cranial sonography and CT scan showed features consistent with alobar holoprosencephaly. Karyotyping for the first neonate and her family members was normal. Both of them were treated conservatively and supervised.

Matched MeSH terms: Cleft Palate/pathology
Hearing status and behavioural patterns among school aged children with cleft lip and/or palate

Goh BS, Tang CL, Hashim ND, Annamalay T, Abd Rahman FN

Int J Pediatr Otorhinolaryngol, 2019 Mar;118:1-5.
PMID: 30578988 DOI: 10.1016/j.ijporl.2018.12.010

OBJECTIVE: There is a dearth of studies on long term hearing status and behavioural patterns among cleft lip and/or palate children after their primary lip and palate closure in Malaysia. This study describes the audiology status and behavioural patterns in a group of school aged children with cleft lip and/or palate.
METHOD: A cross sectional study was carried out where caretakers of cleft lip and/or palate were asked to complete the translated Malay language version of Strength Difficulties Questionnaire. The hearing status of the children was analyzed based on recent pure tone audiometric and tympanogram results. The patients' age, gender, type of cleft pathology, age of palatal surgery and behavioural patterns were examined for their potential relationship with hearing status.
RESULTS: A total of 74 children (148 ears) aged between 7 and 17 years with cleft lip and/or palate were recruited. The result showed 37 ears (25.0%) had hearing loss with majority suffered from mild conductive hearing loss. There were 16 ears (10.8%) that had persistent middle ear effusion. Hearing improvement occurred when palatal repair was performed at the age of less than 1 year old. (p = 0.015) There was no significant relationship between patients' gender, age, type of cleft and history of myringotomy with their hearing status. In terms of behavioural patterns, 16.3% were abnormal for total behavioural score, 39.2% for peer problem and 17.6% for conduct problem. For prosocial behaviour, 16.3% were rated low and very low. There was fair correlation between age and hyperactivity problems (r = 0.44). Patients' gender, type of cleft pathology, had been teased apart and hearing status was found not related to behavioural problems.
CONCLUSION: Cleft lip and/or palate patients have a good longterm hearing outcome. Majority had normal hearing and if there is hearing impairment, it is only a mild loss. Early palatal repair surgery before the age of 1 year can significantly reduce the risk of hearing loss. Cleft lip and/or palate patients experienced peer problems. There was no significant correlation between behavioural difficulty and hearing status among school-aged children with cleft lip and palate.

Matched MeSH terms: Cleft Palate/surgery*
Haplotype diversity in 11 candidate genes across four populations

Beaty TH, Fallin MD, Hetmanski JB, McIntosh I, Chong SS, Ingersoll R, et al.

Genetics, 2005 Sep;171(1):259-67.
PMID: 15965248

Analysis of haplotypes based on multiple single-nucleotide polymorphisms (SNP) is becoming common for both candidate gene and fine-mapping studies. Before embarking on studies of haplotypes from genetically distinct populations, however, it is important to consider variation both in linkage disequilibrium (LD) and in haplotype frequencies within and across populations, as both vary. Such diversity will influence the choice of "tagging" SNPs for candidate gene or whole-genome association studies because some markers will not be polymorphic in all samples and some haplotypes will be poorly represented or completely absent. Here we analyze 11 genes, originally chosen as candidate genes for oral clefts, where multiple markers were genotyped on individuals from four populations. Estimated haplotype frequencies, measures of pairwise LD, and genetic diversity were computed for 135 European-Americans, 57 Chinese-Singaporeans, 45 Malay-Singaporeans, and 46 Indian-Singaporeans. Patterns of pairwise LD were compared across these four populations and haplotype frequencies were used to assess genetic variation. Although these populations are fairly similar in allele frequencies and overall patterns of LD, both haplotype frequencies and genetic diversity varied significantly across populations. Such haplotype diversity has implications for designing studies of association involving samples from genetically distinct populations.

Matched MeSH terms: Cleft Palate/ethnology; Cleft Palate/genetics
Fulltext Genetics of cleft lip and palate

ROSLINA R., ZAINUL AHMAD R., ZILFALIL BA, WAN AZMAN WS, AHMAD SUKARI H, SAIDI J.

Buletin Persatuan Genetik Malaysia, 2011;18(1):22-25.
MyJurnal

Orofacial clefts are one of the most common congenital malformations among newborns. The two main types of oral clefts are cleft lip with or without cleft (CLP) and cleft palate alone (CP). Cleft is an abnormal ssure in an anatomical structure that is normally fused. Cleft lip is the congenital failure of the maxillary and medial nasal processes to fuse, forming a ssure in the lip. Cleft palate is the congenital failure of the palate to fuse properly, forming a ssure in the roof of the mouth (Mossey, 2009).clefts are one of the most common congenital malformations among newborns. The two main types of oral clefts are cleft lip with or without cleft (CLP) and cleft palate alone (CP). Cleft is an abnormal ssure in an anatomical structure that is normally fused. Cleft lip is the congenital failure of the maxillary and medial nasal processes to fuse, forming a ssure in the lip. Cleft palate is the congenital failure of the palate to fuse properly, forming a ssure in the roof of the mouth (Mossey, 2009).

Matched MeSH terms: Cleft Palate
Fixed presurgical orthopaedics for bilateral cleft lip and palate

Abu-Rub N, Samsudin AR, Abdullah AB, Abdullah N

Aust Orthod J, 2005 May;21(1):39-43.
PMID: 16433080

Presurgical orthopaedics has been employed since the 1950s as an adjunctive neonatal therapy for the correction of cleft lip and palate. It is accepted that presurgical orthopaedic plates facilitate lip repair and balanced orofacial growth.

Matched MeSH terms: Cleft Palate/surgery; Cleft Palate/therapy*
Fulltext Factors influencing the perception of mothers toward their children with cleft defect. [Faktor-faktor yang mempengaruhi persepsi ibu terhadap kanak-kanak dengan kecacatan klef]

Mohd Rohaizat Hassan, Mohd Hafiz Yahya, Normala Basiron, Zairizam Zakaria, Mohd Rizam Abdul Rahman, Hazlina Mohd Miskam, et al.

Int J Public Health Res, 2017;7(2):836-844.
MyJurnal

Introduction A study was conducted to determine the level of mothers' perception on children with cleft deformity and its associated factors
Methods This was a cross sectional study involving 110 mothers with children attending the Plastic And Reconstructive Surgery Clinic. Data was gathered from a face to face interview based on a questionnaire that assesses the level of perception and its associated factors. The level of perception was evaluated from a scoring method and divided into four domains namely psychosocial, care management, treatment and education.
Results The level of perception was high in all four domains ranged from 62% for treatment and 80% for education. Factors that were significantly associated with the level of perception were ethnicity, religion, type of cleft deformity and level of information.
Conclusions Information regarding cleft deformity must be tailored toward mothers’ personal characteristics and type of cleft deformity to improve mothers' perception on this problem.
Study site: Plastic And Reconstructive Surgery Clinic, Hospital Kuala Lumpur, Malaysia

Matched MeSH terms: Cleft Palate*
Factors contributing to hearing impairment in patients with cleft lip/palate in Malaysia: A prospective study of 346 ears

Cheong JP, Soo SS, Manuel AM

Int J Pediatr Otorhinolaryngol, 2016 Sep;88:94-7.
PMID: 27497393 DOI: 10.1016/j.ijporl.2016.06.045

OBJECTIVE: To determine the factors contributing towards hearing impairment in patients with cleft lip/palate.
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 cleft groups and the outcome of hearing tests. However, hearing improvement occurred when palatal repair was performed at the age of <1year old (OR = 2.37, CI 1.2 = 4.6, p = 0.01).
CONCLUSION: Majority of the cleft patients had normal hearing (70%). Hearing threshold varied significantly between the different types of cleft pathology. Surgery conferred no significant impact on the hearing outcome unless surgery was performed at the age of <1 year old.

Matched MeSH terms: Cleft Palate/epidemiology*; Cleft Palate/pathology; Cleft Palate/surgery

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