OBJECTIVE: To determine the clinical course and outcome of familial congenital laryngotracheal stenosis (FCLS).
METHODS: A literature search was conducted over a period of one month (September 2023) by searching several databases to identify studies published from inception to 31st August 2023.
RESULTS: Of 256 papers identified, five articles met the inclusion criteria. A total of 17 patients with slight female predominance (59 %) were identified. Familial congenital tracheal stenosis was reported in female twins (100 %). A variety of clinical presentations were listed. An endoscopic airway study was performed on all patients. 64.8 % of the included children were managed surgically. Genetic studies performed on 41 % of children could not locate genetic abnormalities.
CONCLUSION: Consanguinity, twin births, and female gender could be predisposing factors for FCLS, although the quality of evidence is low due to the rarity of the condition.
METHOD: Data was collected on 1085 CI recipients of as part of a prospective, longitudinal, observational, international, multi-centre, paediatric registry, initiated by Cochlear Ltd (Sydney, NSW, Australia). Outcome data from children (≤10 years old) implanted in routine practice was voluntarily entered into a central, externally hosted, e-platform. Collection occurred prior to initial device activation (baseline) and at six monthly follow-up intervals up to 24 months and then at 3 years post activation. Clinician reported baseline and follow up questionnaires and Categories of Auditory Performance version II (CAP-II) outcomes were collated. Self-reported evaluation forms and patient information were provided by the parent/caregiver/patient via the implant recipient baseline and follow up, Children Using Hearing Implants Quality of Life (CuHIQoL) and Speech Spatial Qualities (SSQ-P) Parents Version questionnaires.
RESULTS: Children were mainly bilaterally profoundly deaf, unilaterally implanted and used a contralateral hearing aid. Prior to implant 60% used signing or total communication as their main mode of communication. Mean age at implant was 3.2 ± 2.2 years (range 0-10 years). At baseline 8.6% were in mainstream education with no additional support and 82% had not yet entered school. After three years of implant use, 52% had entered mainstream education with no additional support and 38% had not yet entered school. In the sub-group of 141 children who were implanted at or after three years of age and were thus old enough to be in mainstream school at the three-year follow up, an even higher proportion (73%) were in mainstream education with no support. Quality of life scores for the child improved statistically significantly post implant compared to baseline and continued to improve significantly at each interval up to 3 years (p
AIMS: To determine the (a) diagnostic accuracy, interobserver reliability and reliable oxygen desaturation index of 4% (ODI4) score at diagnosing obstructive sleep apnoea syndrome in children and (b) correlation between the apnoea hypopnoea index (AHI) with ODI4 and oxygen nadir between both PSG and oximetry.
METHODS: This cross-sectional study included children aged 1-18 years old, undergoing a fully attended overnight PSG for suspected obstructive sleep apnoea syndrome. The Nonin 3150 WristOx2 ™ [Fig. 2] was worn simultaneously during the PSG. Poor oximetry recordings were excluded. Pulse oximetry was scored using the McGill Oximetry Score (MOS) whereby a score of 2-4 was positive for OSAS. Specificity, sensitivity, positive predictive values (PPV), negative predictive values (NPV) and interobserver reliability of the WristOx2 were calculated.
RESULTS: One hundred and sixty-two children with a mean (SD) age of 9.3 (±3.5) years (range 2 years 6 months old - 17 years old) were included after excluding 18 children (poor oximetry data [n = 16] and incomplete PSG [n = 2]). Interobserver agreement of the WristOx2 was 0.8763 (95% CI:0.80, 0.95). WristOx2 had a sensitivity 50%, specificity 96.7%, PPV 96% and NPV 53% at diagnosing OSAS. ODI4 ≥ 2 events/hour in oximetry had a sensitivity of 97.6% and negative predictive value of 85.7% at diagnosing OSA.
CONCLUSION: Overnight pulse oximetry with the Nonin 3150 WristOx2 ™ is an accurate and reliable tool in diagnosing significant OSAS in children.
METHOD: The study utilized a semi-structured interview with open-ended questions to obtain information about parents' experiences during the diagnosis period and their challenges when going through that process. In this study, a total of 16 parents of children who were diagnosed with moderate to profound sensorineural hearing loss and received intervention within three years at the time of the study participated. Ten of the children were cochlear implant users, and six were hearing aid users.
RESULTS: Thematic analysis was used to analyse themes generated from the data according to the study objective. Four main themes and 17 subthemes were identified from this study. The four main themes were 1) Parents' emotion; 2) Parental knowledge; 3) Others; 4) Profesional services. Challenges that parents faced often include emotional behaviours such as feeling guilty and devastated during the diagnosis, lack of information-sharing from healthcare givers, lack of knowledge on childhood hearing loss among parents, support from families, seek for a second opinion, worry about others' acceptance, longer time for diagnosis to confirm, late referral to other related profesionals and no priority for the appointment.
CONCLUSION: Emotion is identified as the biggest challenge faced by parents in the process of diagnosis for their children with hearing loss. Hence, management of parental emotion needs to be emphasized by health profesionals as it influences the acceptance of parents towards their child's diagnosis.
MATERIAL AND METHODS: A literature search was conducted for the period from January 2000 to December 2019 by using a number of medical literature data bases including Scopus, PubMed and Embase. The following search words were used either individually or in combination: drug-induced sleep endoscopy, sleep endoscopy directed surgery, paediatrics sleep apnoea. The search was conducted over a month period (December 2019). Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and the Cochrane Handbook for Systematic Reviews of Interventions were followed when possible.
RESULTS: Seven clinical research articles were selected based on our objective and selection criteria. Seven studies were of level III evidence: retrospective, case-control and prospective series. Altogether, there were 996 patients with male predominance; 61%. Over 10% of patients (133 patients) were found to have comorbidities or were syndromic. The mean age of patient was 6 years and majority (87.6%) of our patients were found to be surgically naïve, that is, no previous surgical procedures were performed for OSA. Surgical decision was changed in 295 patients (30%) following DISE. Post intervention outcomes were objectively revealed in 4 studies. Most of our patients underwent a multilevel surgery based on DISE (86%). Complications were documented in 3 studies.
CONCLUSIONS: Analysis of the results indicated that DISE directed surgery was an effective, safe therapeutic approach to treating paediatrics obstructive sleep apnoea. DISE directed surgery has shown to have changed surgical management in most studies.
DATA SOURCES: PubMed, Scopus, Embase.
REVIEW METHODS: A literature search was conducted over a period from January 1990 to March 2020. The following search words were used either individually or in combination: voice disorders, laryngopharyngeal reflux, and gastroesophageal reflux. The search was conducted over a period of a month: April 2020.
RESULTS: Five clinical research were selected based on our objectives and selection criteria. Four studies were of level III evidence. Altogether, a total of 606 patients were pooled with male predominance of 63%. In all studies, reflux was suggested to have strong relation with dysphonia. Majority of cases used 24-h pH monitoring to confirm reflux which yielded positive results in 69%. The top three most common endoscopic findings include: interarytenoid erythema and edema (32/38), vocal cord erythema and edema (160/231) and postglottic edema (141/337). Vocal cord nodules were found in 28% of our patients. Acoustic analysis and perceptual assessment of voice was performed in only 1 study. No complication from any procedure was mentioned in any of the studies. Outcome of treatment was mentioned in 1 study, whereby after 4.5 months of follow-up, 68% of children showed improvement in symptoms.
CONCLUSION: Current evidence shows that there is strong relation between reflux and dysphonia in children. Most common laryngoscopic findings suggestive of reflux includes interarytenoid erythema and edema, vocal cord erythema and edema and postglottic edema.
OBJECTIVES: To analyze the presence of DPOAE across frequencies and DP amplitude in infants with and without IDA.
METHOD: DPOAE were recorded on 40 infants with IDA and 40 infants without IDA in the age range of 6-24 months. Cubic DPOAEs (2f1-f2) were measured at six f2 frequencies (1500 Hz, 2000 Hz, 3000 Hz, 4500 Hz, 6000 Hz & 8000 Hz) with primary tone stimulus of intensity L1 equal to 65 dBSPL and L2 equal to 55 dBSPL. Immittance audiometry was performed using 226 Hz probe tone prior to DPOAE recording to ascertain normal middle ear functioning.
RESULTS: DPOAEs were present in all infants with and without IDA across frequencies tested. DP amplitude across the frequencies did not show any statistically significant difference (p
METHOD: A prospective cross-sectional otoscopic and audiological analysis was conducted on 102 cleft palate children (204 ears) aged 1-18 years old who were treated for middle ear effusion at our centre. Retrospective chart review was done to determine patient characteristics and prior patient management. The aim was to assess the effect of ventilation tube insertion (VTI) on hearing, speech and chronic otitis media; comparing the timing and number of ventilation tubes per ear and determining other factors affecting the short-term and long-term outcome.
RESULTS: 68 children or 130 ears (63% of all cases) were selectively treated with ventilation tube insertion. Repeat procedures (more than 2) were performed in 41 ears. Among children with VTI performed, the incidence of chronic otitis media in children after the age of 4 was 17%. Overall, abnormal tympanic membrane findings and hearing loss were detected in nearly half the cases who were previously treated with VTI. Early ventilation tube insertion at less than 1 year of age, resulted in a better middle ear and hearing outcome in children less than 4 years old (p
METHODS: A group of 33 healthy children, aged from 5 years 9 months-12 years 4 months (mean ± SD = 8.83 ± 1.92 years), was recruited. Their otolith saccular function was assessed using 750 Hz tone burst for cVEMPs (with ER3A insert phone), while their utricular function was assessed using Brüel & Kjaer Mini-shaker Type 4810 (Naerum, Denmark) for oVEMPs.
RESULTS: For cVEMPs, the mean value of P13 latency, N23 latency, P13-N23 interamplitude and asymmetry ratio were 12.62 ± 1.38 ms, 19.85 ± 1.95 ms, 92.47 ± 50.35 μV and 14.03 ± 9.75%, respectively. For oVEMPs, the mean value of N10 latency, P15 latency, N10-P15 interamplitude and asymmetry ratio were 9.23 ± 1.07 ms, 14.41 ± 1.04 ms, 10.32 ± 5.65 μV and 15.84 ± 11.49%, respectively. Two-way ANOVA analysis found that ear laterality and gender had no significant effect on all cVEMPs and oVEMPs parameters. No significant correlation was found between age and all VEMPs parameters.
CONCLUSIONS: The normative data for cVEMPs and oVEMPs obtained in this study can be used as a guide by health professionals to assess saccular and utricular functions among children age from 5 to 12 years of age.
METHODS: In this study that employed a comparative design, fifty-nine typically developing Malaysian children (24 boys, aged from 8 years, 0 months to 8 years, 11 months) were enrolled. They were chosen from three different classrooms (consisting of high achieving, medium achieving and low achieving students, respectively) in a primary school. Their ORF and SRF skills were assessed according to the established methods.
RESULTS: As revealed by two-way ANOVA results, both ORF and SRF scores were found to be comparable between boys and girls (p > 0.05). In contrast, ORF and SRF results between the three classrooms were significantly different from each other (p
OBJECTIVE: To evaluate and compare the treatment effect of Reverse Twin-Block (RTB) and Reverse Pull Face Mask (RPFM) on Pharyngeal Airway Space (PAS) in early and late mixed dentition Class III samples.
METHODS: Ninety-five mixed dentition Malay children with Class III malocclusion were included in this study. Data consists of 190 pre- and post-treatment lateral cephalograms of early (8-9 years) and late (10-11 years) mixed dentition Class III samples. forty-nine samples were under RTB and forty-six samples were under RPFM. Treatment changes were evaluated by McNamara airway analysis and changes in tongue and hyoid bone position.
RESULTS: Both upper and lower pharyngeal airway width were increased after treatment with RTB and RPFM ruling out the chance of airway constriction. There was also posterior positioning of the tongue and hyoid bone indicating mandibular retrusion. No significant treatment changes were found on the Pharyngeal Airway Space by the factor age, gender or type of appliance.
CONCLUSIONS: Both RTB and RPFM increased the Pharyngeal Airway Space and produced similar treatment effect. As age does not affect the treatment outcome significantly, treatment can be delayed until late mixed dentition stage.
METHOD: A cross-sectional survey was conducted on 362 mothers and mothers-to-be (mean age: 31.9 ± 4.9 years, range: 20-48 years old) recruited from Hospital Universiti Kebangsaan Malaysia in Cheras, Kuala Lumpur and two health clinics in Lenggeng, Negeri Sembilan and Beranang, Selangor representing the urban and rural areas respectively. All participants were interviewed in person using a newly developed and reliable questionnaire that tested their knowledge and attitude on childhood hearing loss.
RESULTS: Generally, the majority of mothers had a moderate level of knowledge and positive attitude towards childhood hearing loss. Urban mothers had a significantly higher knowledge than those of the rural area (p
OBJECTIVE: To study the effectiveness of distortion product otoacoustic emission (DPOAE) and automated auditory brainstem response (AABR) as first screening tool among non-risk newborns in a hospital with high delivery rate.
METHOD: A total of 722 non-risk newborns (1444 ears) were screened with both DPOAE and AABR prior to discharge within one month. Babies who failed AABR were rescreened with AABR ± diagnostic auditory brainstem response tests within one month of age.
RESULTS: The pass rate for AABR (67.9%) was higher than DPOAE (50.1%). Both DPOAE and AABR pass rates improved significantly with increasing age (p-value<0.001). The highest pass rate for both DPOAE and AABR were between the age of 36-48 h, 73.1% and 84.2% respectively. The mean testing time for AABR (13.54 min ± 7.47) was significantly longer than DPOAE (3.52 min ± 1.87), with a p-value of <0.001.
CONCLUSIONS: OAE test is faster and easier than AABR, but with higher false positive rate. The most ideal hearing screening protocol should be tailored according to different centre.
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.
METHODS: Via cross sectional approach, the current study collected data using a validated questionnaire to obtain information on the awareness of UNHS program among the health practitioners and to test the formulated hypotheses. 51, representing 81% response rate, out of 63 questionnaires distributed to the health professionals were returned and usable for statistical analysis. The survey instruments involving healthcare practitioners' awareness, human resource, program layout, screening instrument, and screening techniques instruments were adapted and scaled with 7-point Likert scale ranging from 1 (little) to 7 (many). Partial Least Squares (PLS) algorithm and bootstrapping techniques were employed to test the hypotheses of the study.
RESULTS: With the result involving beta values, t-values and p-values (i.e. β=0.478, t=1.904, p<0.10; β=0.809, t=3.921, p<0.01; β= -0.436, t=1.870, p<0.10), human resource, measured with training, functional equipment and program layout, are held to be significant predictors of enhanced knowledge of health practitioners. Likewise, program layout, human resource, screening technique and screening instrument explain 71% variance in health practitioners' awareness. Health practitioners' awareness is explained by program layout, human resource, and screening instrument with effect size (f2) of 0.065, 0.621, and 0.211 respectively, indicating that program layout, human resource, and screening instrument have small, large and medium effect size on health practitioners' awareness respectively. However, screening technique has zero effect on health practitioners' awareness, indicating the reason why T-statistics is not significant.
CONCLUSION: Having started the UNHS program in 2003, non-public hospitals have more experienced and well-trained employees dealing with the screening tools and instrument, and the program layout is well structured in the hospitals. Yet, the issue of homogeneity exists. Non-public hospitals charge for the service they render, and, in turn, they would ensure quality service, given that they are profit-driven and/or profit-making establishments, and that they would have no option other than provision of value-added and innovative services. The employees in the non-public hospitals have less screening to carry out, given the low number of babies delivered in the private hospitals. In addition, non-significant relationship between screening techniques and healthcare practitioners' awareness of UNHS program is connected with the fact that the techniques that are practiced among public and non-public hospital are similar and standardized. Limitations and suggestions were discussed.
METHODS: A retrospective descriptive cohort study on the audiological findings detected during the first hearing assessment done on a child with craniosynostosis using otoacoustic emissions, pure tone audiometry or auditory brainstem response examination. The main aim of this study was to evaluate the type and severity of hearing loss when compared between syndromic and non-sydromic craniosynostosis, and other associated contributory factors.
RESULTS: A total of 31 patients with 62 ears consisting of 14 male patients and 17 female patients were evaluated. Twenty two patients (71%) were syndromic and 9 (29%) were non-syndromic craniosynostosis. Amongst the syndromic craniosynostosis, 9 (41%) had Apert syndrome, 7 (32%) had Crouzon syndrome, 5 (23%) had Pfieffer syndrome and 1 (4%) had Shaethre Chotzen syndrome. Patients with syndromic craniosynostosis were more likely to present with all types and severity of hearing loss, including severe to profound sensorineural hearing loss while children with non-syndromic craniosynostosis were likely to present with normal hearing (p
METHODS: A total of 11 parents and 8 teachers of the 6-year old CI children participated and rated the children using the School Readiness Scale to Year One. Data from 207 6-year old NH children from five states in Malaysia were also collected using the same scale which has nine domains. Results from the NH children were categorized into the 25th and 75th percentile scores to be the reference cut-offs for below average (below the 25th percentile), average (25th to 75th percentile) and above average (above 75th percentile).
RESULTS: The school readiness skills of the CI children were lower than the NH group as rated by teachers especially in the civic and language and communication domains. Comparisons between parents' and teachers' ratings for 8 CI children indicated that teachers tended to rate the CI children's school readiness poorer than that of parents especially in the academic domain. Intra-class correlation analysis revealed poor inter-rater agreement.
CONCLUSIONS: The results suggest that our CI children, generally, need an intervention 'bridging' program to improve their school readiness skills. Parents and teachers had different views on the readiness of the CI children at school entry level.