Electrocardiographic abnormalities in patients with microtia.

The main objective of this study was to investigate the incidence and characteristics of electrocardiographic abnormalities in patients with microtia, and to explore cardiac maldevelopment associated with microtia. This retrospective study analyzed a large cohort of microtia patients admitted to Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, from September 2017 to August 2022. The routine electrocardiographic reports of these patients were reviewed to assess the incidence and characteristics of abnormalities. The study included a total of 10,151 patients (5598 in the microtia group and 4553 in the control group) who were admitted to the Plastic Surgery Hospital of Peking Union Medical College. The microtia group had a significantly higher incidence of abnormal electrocardiographies compared to the control group (18.3% vs. 13.6%, P < 0.01), even when excluding sinus irregularity (6.1% vs. 4.4%, P < 0.01). Among the 1025 cases of abnormal electrocardiographies in the microtia group, 686 cases were reported with simple sinus irregularity. After excluding sinus irregularity as abnormal, the most prevalent abnormalities was right bundle branch block (37.5%), followed by sinus bradycardia (17.4%), ST-T wave abnormalities (13.3%), atrial rhythm (9.1%), sinus tachycardia (8.3%), and ventricular high voltage (4.7%). Less common ECG abnormalities included atrial tachycardia (2.1%), ventricular premature contraction (2.4%), and ectopic atrial rhythm (1.8%). atrioventricular block and junctional rhythm were present in 1.2% and 0.9% of the cases, respectively. Wolff Parkinson White syndrome and dextrocardia had a lower prevalence, at 0.6% and 0.9%, respectively. The occurrence of electrocardiographic abnormalities in microtia patients was found to be higher compared to the control group. These findings highlight the potential congenital defect in cardiac electrophysiology beyond the presence of congenital heart defect that coincide with microtia.

A clinical epidemiological study on congenital ear malformation (CEM).

BACKGROUND: There is no report about the definition, classification and clinical epidemiological study of congenital ear malformation (CEM).Aims/Objectives: To investigate the definition, clinical classification and distribution of a large number of CEM cases, along with the clinical and epidemiological characteristics associated with congenital malformation of the middle and outer ear (CMMOE). MATERIAL AND METHODS: A total of 3231 cases (4714 ears) with CEM and related malformations were retrospectively analyzed, including 2,658 cases (4,064 ears) CEM and 573 cases (650 ears) preauricular fistulas and accessory ears, specifically related malformations to CEM. RESULTS: Among the 2,658 cases (4,064 ears) CEM, 64.9% cases were male and 35.1% were female. 52.9% cases with bilateral, 29.1% with right and 18.0% with left CEM. A subgroup of 1,050 cases (1,331 ears) was identified as CMMOE out of 1,090 cases (1,379 ears) microtia. In the CMMOE subgroup, 74.0% cases were male, 46.3% on the right side, 26.8% showed bilateral involvement. Out of the CMMOE with available images, there were 947 cases (1,105 ears). Additionally, related malformations including preauricular fistulas and accessory ears, were solely occured in 573 cases (650 ears), except for their occurrence simultaneously with CEM. The classification and distribution of CEM are as follows:1. 2658 cases of CEM (1) 69.9% of them were confined to the ear, including: ① the outer ear 2.6%, the middle ear 17.0% and the inner ear 32.4%, ② two parts simultaneous malformations of the outer ear, the middle ear and the inner ear 17.2%, ③ three parts simultaneous malformations of the outer ear, the middle ear and the inner ear 0.6%, (2) Multiple malformations including in CEM 29.1%; (3) syndromes related to CEM 1.0%.2. 1050 cases of CMMOE (1) 36.3% of them were confined to the middle and outer ears; (2) Multiple malformations associated with CMMOE 61.4%, including hemifacial microsomia 50.5%, accessory ears 9.8%, preauricular fistulas 9.2%, heart malformation 7.1%, and kidney malformation 2.0%; and (3) syndromes related to CMMOE 2.3%, (4) Complications of the ear 16.9%, mainly including otitis media 7.9%, outer ear canal cholesteatoma 6.3%, retroauricular abscess 1.1%, and nasal and pharyngeal complications 18.6%. The proportions of microcia grades I-Ⅴ based on the images of 1105 ears CMMOE were 5.2%, 10.5%, 74.1%, 6.9%, and 3.3%, respectively. Among the 1331 ears CMMOE, the incidence rates of outer ear canal atresia, stenosis, normal, and ossicles malformation were 86.5%, 9.9%, 3.6%, and 98.3%, respectively.Conclusion and Significance: Approximately 69.9% of CEM cases were confined to the ear itself, with the highest proportion being inner ear malformation alone. Multiple malformations were predominantly associated with CMMOE. Syndromes related to CEM were relatively rare. Among cases of microtia, 96.3% were classified as CMMOE. These cases were predominantly observed in males, right ear and exhibited characteristics such as grade III microtia, atresia of the outer ear canal, ossicles malformation, and multiple malformations. The multiple malformations frequently included hemifacial microsomia, accessory ear, preauricular fistula, as well as heart and kidney deformities. CMMOE often coexisted with ear infections, cholesteatoma, and complications in the nasal and pharyngeal regions.

Inheritance analysis and family history for microtia: A hospital-based study in China.

OBJECTIVES: Microtia is a congenital anomaly of the outer ear. Although genetic and environmental factors could play a role, no consensus has been established on the pathogenesis and cause of this condition. In this study, we surveyed the frequency and pattern of family history in patients with microtia in a Chinese specialty clinic population. METHODS: We evaluated data from 672 patients (mean age = 9.2, male-to-female ratio = 2.6:1) with microtia admitted to the Department of Auricular Reconstruction at the Plastic Surgery Hospital of Peking Union Medical College from December 2014 to February 2016. Family history of congenital ear anomalies across three generations was recorded. Pearson chi-square test or Fisher exact test was used to test the associations between the characteristics of microtia and hereditary features. RESULTS: A family history of auricle anomalies was identified in 202 patients (30.1%), of whom, 95 families showed vertical transmission, 14 families skipped a generation, and 120 families showed family aggregations. The incidence of family history varied with grades of microtia (P = 0.001). Patients with preauricular tags or pits (38.3%) had a higher familial incidence of microtia than those with simple microtia (24.1%) (P < 0.001). CONCLUSION: Patients with a lower grade of microtia demonstrated a higher incidence of family history. Patients with microtia had significantly more relatives with preauricular tags or pits. Microtia and preauricular tags or pits are different manifestations of the same defect, and their significant concurrency among relatives suggests that a considerable proportion of microtia is inherited and could recur with varying degrees of severity in other family members.

Vertebral Anomalies in Microtia Patients at a Tertiary Pediatric Care Center.

OBJECTIVE: Microtia is a congenital condition known to be associated with vertebral anomalies and congenital syndromes, most prominently hemifacial microsomia. There is controversy, however, on whether to screen with spinal imaging. Additionally, microtia ear reconstruction utilizes rib harvesting that could potentially worsen pre-existing vertebral and rib anomalies, specifically scoliosis. We report on the prevalence and characteristics of vertebral anomalies among microtia patients at a tertiary pediatric center. STUDY DESIGN: Retrospective case review with literature review. SETTING: Tertiary pediatric referral center. METHODS: A review of 425 children with microtia was conducted, characterized as either syndromic or nonsyndromic. Data included demographics, spinal imaging performed, indications, anomalies detected, and microtia repair. RESULTS: Among 425 microtia patients, 24.5% were syndromic with an average age of 9.7 years. Only 18.4% of all patients had spinal imaging performed (50% syndromic vs 8.1% nonsyndromic). Overall, 10.6% had a vertebral anomaly with a 57.7% detection rate (67.3% syndromic vs 38.5% nonsyndromic). The most common anomaly was scoliosis, with a prevalence of 7.8%. Fusion defects and rib deformities were the next most prominent. Microtia repair, most commonly with an autologous rib graft, was performed in 21.6% of the cohort. However, only 19.2% had spinal imaging and 16.7% with a vertebral anomaly. CONCLUSION: Children with microtia are at a greater risk of vertebral abnormalities. Scoliosis prevalence in isolated microtia is comparable to the general population (2%-3%) but greatly increased with genetic syndromes. Screening for vertebral anomalies should be considered when planning microtia reconstructions, especially in the syndromic population.

Prevalence and characteristics of microtia in Oman: 37 Years analysis.

BACKGROUND: Prevalence of microtia varies in different parts of the world and between ethnic groups. This article reviews the prevalence of Microtia in Oman. METHODS: A retrospective study of all cases of Microtia between the period from January 1988 to March 2020. RESULTS: 152 patients fulfilled the study criteria. The prevalence of microtia was 0.83 per 10,000 births. Analysis of the affected cases showed 56% males and 44% females. 66% of the cases were of the lobular type, and 30% were of the conchal type. Anotia cases were only 4%. Unilateral cases were predominant consisting 86% of the cases, of which 67% were on the right side. 70% of the cases had hearing problems and 35% of them required hearing aids. 14% were syndromic. Goldenhar and Treacher-Collins syndromes were the most common syndromes. Family history was present in 18% of the cases. 49% of them had parents' consanguinity. CONCLUSION: Omani population has low prevalence of microtia but the characteristics does not differ from other reports.

Patterns of co-occurring birth defects in children with anotia and microtia.

Many infants with anotia or microtia (A/M) have co-occurring birth defects, although few receive syndromic diagnoses in the perinatal period. Evaluation of co-occurring birth defects in children with A/M could identify patterns indicative of undiagnosed/unrecognized syndromes. We obtained information on co-occurring birth defects among infants with A/M for delivery years 1999-2014 from the Texas Birth Defects Registry. We calculated observed-to-expected ratios (OER) to identify birth defect combinations that occurred more often than expected by chance. We excluded children diagnosed with genetic or chromosomal syndromes from analyses. Birth defects and syndromes/associations diagnosed ≤1 year of age were considered. We identified 1310 infants with non-syndromic A/M, of whom 38% (N = 492) were diagnosed with co-occurring major defects. Top combinations included: hydrocephalus, ventricular septal defect, and spinal anomalies (OER 58.4); microphthalmia and anomalies of the aorta (OER 55.4); and cleft lip with or without cleft palate and rib or sternum anomalies (OER 32.8). Some combinations observed in our study may represent undiagnosed/atypical presentations of known A/M associations or syndromes, or novel syndromes yet to be described in the literature. Careful evaluation of infants with multiple birth defects including A/M is warranted to identify individuals with potential genetic or chromosomal syndromes.

Epidemiological Study of Neonatal Congenital Microtia in Shandong Province, China, 2011-2020.

OBJECTIVE: Congenital microtia is a common congenital disease in newborns. The specific cause of congenital microtia is currently unknown. The main objective of this study is to elucidate the epidemiological characteristics of congenital microtia and explore the possible etiology of congenital microtia. METHODS: Part of the newborn data from 2011 to 2020 in Shandong Province Birth Defects Monitoring Hospitals were randomly selected. The software GraphPad Prism 9 was used to analyze the data and draw figures. RESULTS: A total of 4247 infants were diagnosed with congenital malformation among 149,525 newborns randomly selected from the Shandong Province Birth Defects Monitoring Hospitals. Among them, a total of 115 infants were diagnosed with congenital microtia. The mean incidence of microtia during 10 years was 76.14±21.93 per 100,000 infants. The mean incidence of microtia in infants with congenital malformation was 2.67±0.75%. The average incidence of male and female infants with microtia during 10 years were 86.93±23.22 and 64.18±32.71 per 100,000 infants, respectively. In terms of maternal age, the older the mother, the higher the incidence of microtia. In terms of the place of residence, rural infants have a higher incidence of microtia than urban infants. CONCLUSIONS: The average incidence of microtia was 76.14±21.93 per 100,000 infants in Shandong Province, China, 2011-2020. The female-to-male incidence ratio was 1.45:1. The authors recommend that women choose to give birth at the age of 25 to 29. They hope that the government will take measures to improve the medical and health conditions in rural areas and improve parenting knowledge in rural areas. This can effectively reduce the prevalence of microtia in infants.

Maternal occupation as a nail technician or hairdresser during pregnancy and birth defects, National Birth Defects Prevention Study, 1997-2011.

OBJECTIVE: Nail technicians and hairdressers may be exposed to chemicals with potential reproductive effects. While studies have examined birth defects in children of hairdressers, those in children of nail technicians have not been evaluated. We investigated associations between selected birth defects and maternal occupation as a nail technician or hairdresser versus a non-cosmetology occupation during pregnancy. METHODS: We analysed population-based case-control data from the multisite National Birth Defects Prevention Study, 1997-2011. Cases were fetuses or infants with major structural birth defects; controls were live-born infants without major birth defects. Expert raters classified self-reported maternal jobs as nail technician, combination nail technician-hairdresser, hairdresser, other cosmetology work or non-cosmetology work. We used logistic regression to calculate adjusted ORs and 95% CIs for associations between occupation during pregnancy and birth defects, controlling for age, smoking, education and race/ethnicity. RESULTS: Sixty-one mothers worked as nail technicians, 196 as hairdressers, 39 as combination nail technician-hairdressers and 42 810 as non-cosmetologists. The strongest associations among nail technicians included seven congenital heart defect (CHD) groups (ORs ranging from 2.7 to 3.5) and neural tube defects (OR=2.6, CI=0.8 to 8.4). Birth defects most strongly associated with hairdressing included anotia/microtia (OR=2.1, CI=0.6 to 6.9) and cleft lip with cleft palate (OR=2.0, CI=1.1 to 3.7). All oral cleft groups were associated with combination nail technician-hairdresser work (ORs ranging from 4.2 to 5.3). CONCLUSIONS: Small samples resulted in wide CIs. Still, results suggest associations between maternal nail technician work during pregnancy and CHDs and between hairdressing work and oral clefts.

Ten-year trends in the incidence of microtia: a nationwide population-based study from Taiwan.

PURPOSE: Few studies have explored population-based incidence rates of microtia using nationwide data. The aim of this study was to analyze the 10-year secular trends in the incidence of microtia and/or anotia in Taiwan from 2008 to 2017 using nationwide population-based data. METHODS: Patient data were retrieved from Taiwan's National Health Insurance Dataset, after identifying 1152 children aged ≤ 1 year with a first-time diagnosis of microtia or anotia between January 2008 and December 2017. The annual microtia-anotia incidence rate was the sum of new microtia-anotia cases in a year divided by total infant population in the year. Furthermore, we used the annual percent change (APC) to study the secular trend in microtia-anotia incidence rate. RESULTS: The annual incidence rate of microtia-anotia averaged across the 10-year period was 57.7 per 100,000 infants (standard deviation = 8.6). The annual incidence rates of microtia and anotia were 53.3 and 4.4 per 100,000 infants, respectively, during this period. Furthermore, female infants had a higher incidence than males (63.3 vs. 52.4 per 100,000). The incidence of microtia-anotia gradually decreased between 2008 and 2017 with an APC of - 5.64% (95% CI - 9.31 ~ - 1.18%, p = 0.004). Since 2011, females had a significantly higher annual incidence rate of microtia-anotia than males. CONCLUSIONS: The incidence of microtia-anotia was 57.7 per 100,000 infants in Taiwan, which declined during the study period 2008-2017. The female-to-male incidence ratio was 1.21:1.

Evaluation of respiratory system anomalies associated with microtia in a Chinese specialty clinic population.

BACKGROUND: Microtia is a congenital malformation of the external ear often with one or more associated congenital anomalies. The purpose of this study was to identify the characteristics and prevalence of respiratory anomalies in patients with microtia, and clarify the importance of this association in the perioperative period of patients' external ear reconstruction surgery. METHODS: Data were collected from 923 microtia patients between August 2017 and December 2020 in the Department of Auricular Reconstruction at the Plastic Surgery Hospital of Peking Union Medical College. Co-occurring respiratory anomalies were detected using chest computed tomography plus three-dimensional reconstruction and Chest X-ray. Physical examination was performed to assess the severity and type of microtia by trained clinicians. Fisher's exact test was used to analyze the relation between laterality of pulmonary underdevelopment and microtia type. RESULTS: Among the 923 participants enrolled in the study, we identified 21 cases (2.3%) having respiratory system anomalies, consisting of 6 cases with pulmonary underdevelopment (28.6% of all anomalies of respiratory system detected), 2 cases with tracheal bronchus (9.5%), 1 case with tracheal diverticula (4.8%), 11 cases with lung bullae(52.4%), and 1 case with pulmonary azygos lobe (4.8%). The laterality of pulmonary underdevelopment was related to the type of microtia (difference between types, p < 0.05), as patients with concha-type remnant ear had pulmonary underdevelopment ipsilaterally. CONCLUSIONS: This study represents the first detailed and thematic study of a association featured by microtia and respiratory anomalies. Characteristics and prevalence of respiratory anomalies was observed in a Chinese clinical microtia population. Early diagnosis of associated respiratory malformations had practical clinical significance for microtia patients, plastic surgeons and anesthesiologists. Future studies are required to improve understanding of this association and its cause.

Congenital abnormalities associated with microtia: A 10-YEARS retrospective study.

OBJECTIVE: Microtia is a congenital auricular malformation, often part of a syndromic form (35%-55% of cases). The accurate prevalence of associated malformations remains to be determined with regard to the heterogeneous results of the previous studies. This study aims to describe in a large population cohort the abnormalities associated with microtia and to determine the most suitable assessment for these children. METHODS: This is a retrospective and observational cohort study collecting data from the medical records of children affected by microtia, diagnosed or followed-up between 2007 and 2017. Data were collected via a computer database. Clinical data, as well as imaging or genetic results, were noted. RESULTS: Six hundred ninety four children were included, 587 (84.6%) with unilateral and 107 (15.4%) with bilateral microtia. Inner ear malformations were observed in 14.1% of the ears. The main associated anomalies were hemifacial microsomia (29%), velopharyngeal insufficiency (9%), ophthalmologic (6.2%), vertebral (5.9%), cardiac (5.5%) and kidney (3%) abnormalities. Main identified entities were Goldenhar, Treacher-Collins and Guion-Almeida syndromes. CONCLUSION: A comprehensive clinical assessment must be completed when microtia is diagnosed. Besides screening well-known oculo-auriculo-vertebral spectrum malformations, velopharyngeal insufficiency should be systematically sought. Specialized care must be provided to the very frequently associated hemifacial macrosomia. Mild forms of this last malformation may correspond to Guion-Almeida syndrome, especially in cases of learning disability.

Epidemiological Analysis and the Nomogram for Possible Risk Factors for Severe Microtia.

BACKGROUND: Microtia is a severe congenital malformation of the external ear. This study aimed to explore the epidemiologic characteristics and the possible risk factors in patients with severe microtia in China, and integrate significant variables into a predictive nomogram. METHODS: A total of 965 patients with microtia were included. This retrospective case study was conducted from July 2014 to July 2019 at Plastic Surgery Hospital in China. The detailed questionnaires concerning potential risk factors were completed and data were gathered. Chi-Square and Fisher tests were used to analyze the variables, and a multivariate logistic regression model was used to select variables related to severe microtia, and then construct a nomogram. The nomogram model was evaluated by the concordance index (C-index), calibration plot, and receiver operating characteristics (ROCs) curve. Bootstraps with 1000 resamples were applied to these analyses. RESULTS: Of the 965 microtia patients, 629 (65.2%) were male and 867 (89.8%) were sporadic. The cases were observed more commonly in unilateral (83.1%) and right-sided (52.0%). And multiple malformations were observed in 392 (40.6%) cases. Multivariate logistic regression analysis showed that maternal age, miscarriage frequency, virus infection, anemia, using progesterone, paternal alcohol intake, and topography of living areas were associated with a higher risk of severe microtia. All the significant variables were combined into a predictive nomogram (C-index = 0.755,95% CI = 0.703-0.807). Higher prediction accuracy (adjusted C-index = 0.749) was further verified via bootstrap validation. The calibration plot showed good performance, and the ROCs curve analysis demonstrated high sensitivity and specificity. CONCLUSIONS: Most microtia patients are male, sporadic, and accompanied by other malformations, which are similar to the phenotypic analysis results of other studies. A nomogram predicting severe microtia was constructed to provide scientific guidance for individualized prevention in clinical practice.

Epidemiological Research of Microtia Combined With Congenital Heart Disease.

SUMMARY: Congenital heart disease (CHD) is one of the most common combined malformations of microtia. There is currently no specific study that investigates the relationship between microtia and CHD. METHODS: This study collected microtia inpatients admitted from May 1, 2015 to July 31, 2016. The diagnosis of CHD was based on patient's symptoms, past history, and echocardiography. Pearson χ2 test was used to analyze the correlation between CHD and microtia. RESULTS: A total of 30 cases (3.35%) were documented with CHD, including atrial septal defect (12/40.00%), ventricular septal defect (7/23.30%), patent ductus arteriosus (2/6.70%), complex congenital heart disease (3/10.00%), combined CHD (2/6.70%) and other malformations (4/13.30%). Analysis showed no statistically significant relation between CHD and the side of affected ear or gender. CONCLUSIONS: The occurrence of CHD in microtia patients was higher than that in the general population. The relationship between them was explored mainly from the etiological perspective. Microtia and CHD were often combined in syndromes such as Goldenhar syndrome, 22q11 deletion syndrome, and CHARGE syndrome. Absence of genes or abnormal embryo development associated with these syndromes leads to the occurrence of both.

Congenital heart defects in patients with isolated microtia: evaluation using colour Doppler echocardiographic image.

BACKGROUND: The objective of this study was to delineate the characteristics and incidence of congenital heart disease (CHD) in patients with isolated microtia and to determine whether the prevalence of CHD among patients with isolated microtia increases with the severity of microtia. METHODS: A total of 804 consecutive patients had a pre-operative colour Doppler echocardiographic examination. A retrospective study was performed with the clinical and imaging data from November, 2017 to January, 2019. The χ2 test was performed to analyse the interaction between isolated microtia and CHD. RESULTS: With the colour Doppler echocardiographic examination's data from 804 consecutive isolated microtia patients, we found CHD, including atrial septal defect, ventricular septal defect, tetralogy of Fallot, patent ductus arteriosus, and others, occurred in 52 of 804 patients (6.5%). Atrial septal defect prevalence in patients with isolated microtia was significantly higher than ventricular septal defect (24/804 versus 11/804, p < 0.05) and patent ductus arteriosus (24/804 versus 2/804, p < 0.001). Ventricular septal defect prevalence in patients with isolated microtia was significantly higher than patent ductus arteriosus (11/804 versus 2/804, p < 0.05). All four types of microtia (concha-type microtia, small concha-type microtia, lobule-type microtia, and anotia) had similar incidences of CHD with no difference in the incidences among these types (p > 0.05 respectively). Furthermore, there was no significant difference in the incidence of the atrial septal defect among the four subtypes (p > 0.05 respectively). Similarly, ventricular septal defect and patent ductus arteriosus also showed no differences (p > 0.05 respectively). CONCLUSIONS: The overall incidences of CHD and three most common CHD subtypes (atrial septal defect, ventricular septal defect, and patent ductus arteriosus) in patients with isolated microtia are higher than general population. The prevalence of CHD among patients with isolated microtia does not increase with the severity of microtia. According to our experience in this study, we suggest colour Doppler echocardiographic imaging should be performed for isolated microtia patients soon after birth if possible. Furthermore, for the plastic surgeon and anaesthesiologist, it is important to take pre-operative colour Doppler echocardiographic images which can help evaluate heart function to ensure the safety of the peri-operative period. Future studies when investigating CHDs associated with isolated microtia could focus on genetic and molecular mechanisms.

Cardiac anomalies in microtia patients at a tertiary pediatric care center.

OBJECTIVE: Microtia is a congenital condition that is known to be associated with cardiac abnormalities. Current guidelines suggest performing an echocardiogram or other cardiac work-up in the presence of ear anomalies with dysmorphic features but not isolated microtia. We report on the prevalence and characteristics of cardiac anomalies among microtia patients at a tertiary pediatric center. METHODS: A review of 428 children with microtia was conducted. Patients were identified as syndromic or non-syndromic. Data included echocardiograms performed, anomalies detected, need for cardiology follow-up, and need for surgical intervention. RESULTS: In the 428 patients with microtia, 77 patients (18%) were syndromic, the most common being Goldenhar (26%). 23.5% (101) of patients overall had documented echocardiograms, with structural anomalies found in 75.9% of patients screened and 18.5% overall, including disorders ranging from minor septal defects to Tetralogy of Fallot. The most common anomalies were left-right shunts in 77.2% of anomalies. Syndromic patients had a greater percentage of echocardiograms performed, cardiac anomalies, and cardiology follow-up compared to non-syndromic microtia patients. CONCLUSION: Children with microtia are at significant risk for cardiac abnormalities. Many patients with lesions required treatment and cardiology follow-up. Anomalies may have been missed in those who did not receive an echocardiogram. Given the risk of cardiac anomalies going unnoticed at the time of birth, we recommend a thorough cardiac physical exam for each microtia patient and the consideration of screening echocardiogram in syndromic children born with microtia.

Ear mold for congenital ear malformation: A randomized controlled trial.

BACKGROUND: Congenital auricular deformities (CAD) are prevalent worldwide. The objective of this study is to investigate the effectiveness and safety of ear molding for children with CAD at their early days. METHODS: One hundred and nighty children (under 3 days) with CAD will be included in the study. Participants will be randomly allocated to treatment or waiting list group (n = 95). The treatment group will receive ear molding within 3 days after birth for 2 weeks. The control group will receive usual care and receive the same ear molding at 6th week if spontaneously recover is not occur. Physician and parent assessment of improvement, parent's anxiety, depression, and quality of life and adverse events will be measured at baseline, 3rd and 6th week of initial treatment. The primary outcome recovery rate will be compared between groups using Chi square test. Secondary continuous outcomes will be compared using analysis of variance. DISCUSSION: This study is the first randomized controlled trial to examine the effectiveness, safety and cost-effectiveness of ear molding for CAD comparing with waiting list, to inform clinical decision of CAD treatments and relevant guideline development.

Hearing Aid Uptake in Children with Unilateral Microtia and Canal Atresia: A Comparison between a Tertiary Center and Peripheral Centers.

OBJECTIVES: To review the trialing and uptake of hearing aids in children with unilateral microtia or canal atresia, known collectively as congenital unilateral conductive hearing loss (CUCHL), observed in a tertiary hospital and local peripheral services. MATERIALS AND METHODS: A retrospective review of medical records for patients with CUCHL was conducted using data from a shared audiology database at a tertiary children's hospital. RESULTS: We identified 45 patients with CUCHL and excluded seven of them due to missing data. Of the 38 patients, 16 (16/38, 42%) did not have any subjective hearing complaints. Furthermore, 32% (12/38) of patients attended audiology at a tertiary centre and 83% (10/12) from this group trialled a hearing aid. In comparison, 46% (12/46) whose audiology care was delivered peripherally trialled aiding. Of the patients from the tertiary center, 58% (7/12) are still using a hearing aid compared to 27% (7/26) of patients from peripheral centers. CONCLUSION: Our analysis shows that patients with CUCHL are more likely to try hearing aids and continue using them if their audiology care is in a tertiary center. Allowing for a small sample size, this may indicate a health inequality. Agreeing on minimum standards for the management of patients with CUCHL or managing them in a designated center could increase consistency.

Renal anomalies and microtia: Determining the clinical utility of screening affected children.

OBJECTIVES: Microtia is a congenital ear anomaly that hinders quality of life. Microtia patients, both syndromic and in isolation, may be at-risk for significant renal anomalies that can go undetected at the time of birth. The goal of this study was to characterize the prevalence of renal anomalies among microtia patients at our institution in order to guide optimal patient management. Current guidelines suggest performing a renal ultrasound when there is presence of preauricular pits and ear anomalies in association with dysmorphic features, but not in cases of isolated microtia and atresia. DESIGN: A retrospective review of 237 children with microtia was conducted from 2001 through 2018 at our tertiary-care pediatric institution, of which 98 also had a documented renal ultrasound. Patients were identified as syndromic or non-syndromic. Data endpoints included renal ultrasounds performed, structural anomalies found, and follow-up. RESULTS: Among the 237 patients, 98 had received renal ultrasounds. 12% of the total cohort was found to be syndromic, the most common being Goldenhar. Structural anomalies were detected in 24% of the 98 patients that underwent ultrasound and included disorders such as pelviectasis, renal ectopia, duplicated collecting systems, and renal agenesis. A third of patients with anomalies required follow-up with nephrology for chronic kidney disease or renal failure. Of note, 21% of non-syndromic patients and 43% of syndromic patients screened had an abnormality on ultrasonography. CONCLUSIONS: Children with microtia are at a significant risk of structural renal abnormalities, even when isolated outside of a genetic syndrome. We recommend the strong consideration of performing a screening renal ultrasound in all patients with microtia. Prospective data would be helpful in developing future clinical guidelines regarding the utility of screening ultrasonography.