Auricular spoon-shaped crus malformation including variants: Clinical features and a novel surgical approach.

BACKGROUND: Certain auricular malformations are uncommon and lack generally accepted diagnostic names. This study investigates an uncommon complex auricular malformation known as auricular spoon-shaped crus malformation providing a detailed description of its external characteristics. Additionally, an effective surgical approach is proposed. METHODS: Between 1991 and 2023, 12 auricles in 11 patients with auricular spoon-shaped crus malformation including variants were surgically treated at our center. Patient medical records and photographic data were retrospectively reviewed. RESULTS: Each auricle exhibited 2 to 4 major structural deformities within the 5 areas of the superior crus, inferior crus, and stem of the antihelix, helical crus, and earlobe. These deformities resulted in depression between the antihelix and antitragus, vertical shortening, horizontal elongation of the auricle, and/or drooping of the ear. Three patients displayed a low positioning of the malformed ear, and 8 patients exhibited mild to moderate hemifacial microsomia. In corrections we conducted earlier, we utilized various methods with variable aesthetic outcomes. Recently, an improved corrective method we implemented yielded consistently promising aesthetic results. We have confidence that adopting the surgical approach we suggest can lead to promising aesthetic results when addressing this malformation. Furthermore, we hope that the presented malformation will be recognized as a primary auricular malformation by auricular surgeons in the future.

Efficiency of ear molding for treating constricted ears of different severity.

PURPOSE: To investigate the treatment time and efficiency of constricted ears of different severity after correction. MATERIALS AND METHODS: We included the patients with constricted ear presented to our hospital for treatment between December 2021 and December 2023 in this retrospective analysis. The patients were divided into class I, II and III groups based on the severity of the constriction. Then we collected the data on classification of severity from each patient, together with sex, family history, age at initial correction, being informed upon diagnosis after birth, as well as utilization of auricle correction system. Logistic regression analysis was performed to identify the factors associated with the treatment time and efficiency. RESULTS: The correction system yielded a high effective rate in the constricted ears. The treatment time in class II was significantly longer compared with those of class I after adjusting these parameters. Compared with the cases of class I, those with a class III showed significant attenuation in the symptoms and conditions (95 % CI: 0.034, 0.365; P < 0.001), after adjusting the age at initial correction, being informed upon diagnosis after birth, and utilization of auricle correction system. There were no statistical differences between class II and III in the treatment efficiency after correction. CONCLUSIONS: The Amazing Ear Correction System was effective in treating constricted ear, yielding satisfactory treatment efficiency. Patients with class II constriction required longer treatment time compared with those of class I. The treatment outcome in the class I constriction was better than that of class III.

One-Year Results of Ear Reconstruction with 3D Printed Implants.

PURPOSE: External ear reconstruction has been a challenging subject for plastic surgeons for decades. Popular methods using autologous costal cartilage or polyethylene still have their drawbacks. With the advance of three-dimensional (3D) printing technique, bioscaffold engineering using synthetic polymer draws attention as an alternative. This is a clinical trial of ear reconstruction using 3D printed scaffold, presented with clinical results after 1 year. MATERIALS AND METHODS: From 2021 to 2022, five adult patients with unilateral microtia underwent two-staged total ear reconstruction using 3D printed implants. For each patient, a patient-specific 3D printed scaffold was designed and produced with polycaprolactone (PCL) based on computed tomography images, using fused deposition modeling. Computed tomography scan was obtained preoperatively, within 2 weeks following the surgery and after 1 year, to compare the volume of the normal side and the reconstructed ear. At 1-year visit, clinical photo was taken for scoring by two surgeons and patients themselves. RESULTS: All five patients had completely healed reconstructed ear at 1-year follow-up. On average, the volume of reconstructed ear was 161.54% of that of the normal side ear. In a range of 0 to 10, objective assessors gave scores 3 to 6, whereas patients gave scores 8 to 10. CONCLUSION: External ear reconstruction using 3D printed PCL implant showed durable, safe results reflected by excellent volume restoration and patient satisfaction at 1 year postoperatively. Further clinical follow-up with more cases and refinement of scaffold with advancing bioprinting technique is anticipated. The study's plan and results have been registered with the Clinical Research Information Service (CRIS No. 3-2019-0306) and the Ministry of Food and Drug Safety (MFDS No. 1182).

Effect of External Ear Deformity on Hearing in Wrestlers.

PURPOSE: Cauliflower ear in wrestlers can lead to hearing impairment. This study primarily aims to assess the hearing of wrestlers with bilateral cauliflower ears and determine their external ear canal (EEC) resonance frequencies. Our second aim is to evaluate their hearing quality, speech, and spatial perception. METHOD: This study included 28 male wrestlers aged 18-35 years with bilateral cauliflower ears, as well as 27 male participants in the control group with no wrestling history. The participants' hearing thresholds were determined across the frequency range of 125-16000 Hz for air-conduction and 500-4000 Hz for bone conduction. EEC resonance frequencies were measured. Additionally, all participants completed the Turkish version of the Speech, Spatial, and Qualities of Hearing Scale (SSQ) questionnaire. RESULTS: Wrestlers with cauliflower ears exhibited significantly higher hearing thresholds, particularly at frequencies above 4000 Hz (p < .05). Analysis of EEC resonance showed a shift to higher frequencies in the second resonance peak of the right ear (p < .001) and the first resonance peak of the left ear (p = .045). SSQ scores revealed that wrestlers had higher spatial perception (p = .046), hearing quality (p = .004), and general scores (p = .042) in comparison to the control group. CONCLUSIONS: Blunt traumas in wrestlers, leading to cauliflower ear, can result in hearing loss. Moreover, deformities in the external ear affect the resonance frequencies of the EEC. Therefore, it is crucial to advocate for the use of ear protection equipment among wrestlers. When fitting hearing aids, attention should be given to changes in the EEC resonance frequency.

[Management of patients with complex ear deformities]. / Prise en charge des patients présentant des déformations complexes du pavillon de l'oreille.

Complex ear reconstruction requires specialized multidisciplinary care. Most patients present with microtia, often associated with hearing disorders. The management of these disorders is a priority, and reconstruction of the external ear remains optional. Nowadays, auricular reconstruction is based on the subcutaneous implantation of either autologous cartilage or an allogeneic implant. Autologous reconstruction requires highly specialized surgical expertise and involves harvesting rib cartilage but carries a lower risk of exposure compared to allogeneic implants. Both techniques yield good results with a high success rate and have a positive impact on the social functioning and daily life of patients.

La reconstruction complexe du pavillon auriculaire nécessite une prise en charge multidisciplinaire spécialisée. La majorité des patients nécessitant ce geste présentent une microtie, souvent associée à des troubles de l'audition. La prise en charge de ceux-ci est prioritaire et la reconstruction du pavillon reste facultative. Aujourd'hui, la reconstruction du pavillon se base sur l'implantation sous-cutanée d'une maquette de cartilage autologue ou d'un implant allogène. La reconstruction autologue demande une expertise chirurgicale hautement spécialisée et nécessite un prélèvement de cartilage costal mais présente un risque d'exposition inférieur par rapport à l'implant allogène. Les deux techniques permettent d'atteindre de bons résultats avec un taux de réussite élevé et un effet positif sur le fonctionnement social et le quotidien des patients.

Early Non-Surgical Treatment For Microtia Types 1 and 2.

Esthetic concerns and psychosocial distress often accompany auricular deformities and malformations in both children and their parents. Approximately 30% of newborns are affected by auricular anomalies, with 15% to 20% resulting in permanent defects. While surgical intervention is typically considered the gold standard for malformations, a non-surgical approach, such as splinting, molding, or other non-invasive techniques, can effectively address deformations if promptly administered by a specialist. Microtia, classified into 4 types, presents challenges ranging from fundamental structural anomalies in types 1 and 2 to severe defects in type 3 and complete absence of the external ear in type 4 (anotia). This study introduces a novel non-invasive treatment modality for microtia types 1 and 2. The cohort consisted of 5 newborns treated for microtia types 1 or 2 between 2022 and 2023. Utilizing the EarWell system, treatment was initiated before 3 weeks of age (mean age: 2 weeks), with an average treatment duration of 6.6 weeks, supplemented by molding treatment as needed. Minor adverse effects, such as simple dermatitis, were observed in 2 patients. All parents expressed high satisfaction with the esthetic outcomes, with 60% reporting extreme satisfaction. The prompt initiation of the treatment protocol for microtia types 1 and 2 led to outstanding and timely outcomes in infants, enhancing the quality of life for both parents and their children. Early intervention for subsequent treatment may improve the condition and, in certain cases, serve as a satisfactory alternative for parents hesitant about further surgical intervention for their children.

New Method Using Local Affected Cartilage and Flap for the Cleft in Question Mark Ear.

BACKGROUND: As a rare auricular deformity, despite numerous surgical procedures for correcting moderate-to-severe question mark ears described in past studies, there remains a need to explore a more cost-effective approach. The optimal utilization of ear cartilage and surrounding skin while achieving superior outcomes continues to pose a significant challenge. METHODS: From 2018 to 2023, twenty-four patients with unilateral question mark ear were enrolled in this study. Seven of them were severe type deformities (absence of lower part of auricle), and seventeen were moderate (only cleft between helix and lobule). All patients were treated with new method using local cartilage and flap without damage in unaffected area. RESULTS: All patients were satisfied with significant improvement of question mark ear and the overall symmetrical appearance. The surgical scar was not obvious. No complications were observed. The follow-up period revealed that the corrective procedure kept producing the symmetrical and cosmetic results. CONCLUSION: Our new method enables optimal utilization of deformed tissue and surrounding skin, rendering this method effective and reliable for correcting moderate-to-severe question mark ears. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Identification of a novel phenotype of external ear deformity related to Coffin-Siris syndrome-9 and literature review.

De novo germline variants of the SRY-related HMG-box 11 gene (SOX11) have been reported to cause Coffin-Siris syndrome-9 (CSS-9), a rare congenital disorder associated with multiple organ malformations, including ear anomalies. Previous clinical and animal studies have found that intragenic pathogenic variant or haploinsufficiency in the SOX11 gene could cause inner ear malformation, but no studies to date have documented the external ear malformation caused by SOX11 deficiency. Here, we reported a Chinese male with unilateral microtia and bilateral sensorineural deafness who showed CSS-like manifestations, including dysmorphic facial features, impaired neurodevelopment, and fingers/toes malformations. Using trio-based whole-exome sequencing, a de novo missense variant in SOX11 (NM_003108.4: c.347A>G, p.Y116C) was identified and classified as pathogenic variant as per American College of Medical Genetics guidelines. Moreover, a systematic search of the literature yielded 12 publications that provided data of 55 SOX11 intragenic variants affecting various protein-coding regions of SOX11 protein. By quantitatively analyzing phenotypic spectrum information related to these 56 SOX11 variants (including our case), we found variants affecting different regions of SOX11 protein (high-mobility group [HMG] domain and non-HMG regions) appear to influence the phenotypic spectrum of organ malformations in CSS-9; variants altering the HMG domain were more likely to cause the widest range of organ anomalies. In summary, this is the first report of CSS with external ear malformation caused by pathogenic variant in SOX11, indicating that the SOX11 gene may be not only essential for the development of the inner ear but also critical for the morphogenesis of the external ear. In addition, thorough clinical examination is recommended for patients who carry pathogenic SOX11 variants that affect the HMG domain, as these variants may cause the widest range of organ anomalies underlying this condition.

The non-operative correction of ear anomalies in infants using the EarWell infant corrective system in the Netherlands.

BACKGROUND: Congenital ear anomalies are regular but often overlooked occurrences. The golden standard of treatment has been to surgically correct these anomalies at a minimum age of 5 to 7 years. As of the last century, ear molding has developed to be a safe, reliable, and effective treatment method. Different treatment methods are still under investigation. This study aims to investigate the use of the EarWell Infant Corrective System in the Dutch population. METHODS: Children aged 0-12 weeks were included in the Zuyderland Medical Center to be treated with the EarWell Infant Corrective System in case of ear deformations. Every 2 weeks, the system was replaced and correction was evaluated by both physician and parents. RESULTS: Seventy-three participants were included, of whom 123 ears in total were treated. Age at initiation was 35.5 days on average; treatment lasted an average of 59 days. Parents and physicians both reported an amelioration of all ear anomalies after treatment, scoring the correction grade an 8.8. Overall satisfaction with the treatment method was 9 or higher for both groups. CONCLUSIONS: The EarWell Infant Corrective System is a safe, reliable, and effective treatment method for the correction of ear anomalies in infants.

Retrospective study on unilateral polyotia combined with microtia utilizing the technique of preserving residual ear tissue.

BACKGROUND: The presence of polyotia in individuals with microtia is a rare deformity. Due to the intricate structure of the auricle, uncertain etiology, and challenging corrective techniques, it has always been a focal point in the field of plastic surgery. The present study presents a technique for correcting the combination of polyotia and microtia by utilizing residual ear tissue as graft material. METHODS: The retrospective study included 23 patients with polyotia and microtia from 2018 to 2022. The residual ear tissue was used to rectify auricular deformities in all patients. The patients were instructed to evaluate the satisfaction of the auricle shape using a visual analog scale (VAS) both before and 6 months after the surgical procedure. The esthetic outcomes of auricle subunits were simultaneously assessed by a senior physician pre- and postoperatively. RESULTS: The mean duration of follow-up in this study was 8.73 months. The preoperative VAS satisfaction score was recorded as 2.26 ± 0.86, while the post-operative VAS score significantly increased to 7.86 ± 0.86. The preoperative auricle esthetic outcomes score was recorded as 9.95 ± 1.74, while the post-operative score significantly increased to 24.04 ± 2.16. The follow-up period did not present any cases of flap necrosis, hematoma, infection, or wound dehiscence. CONCLUSION: The study demonstrates that comprehensive utilization of residual auricular tissue can lead to optimal outcomes in correcting polyotia with concha-type microtia. The utilization of residual ear tissue can be maximized to streamline the operation, minimize bodily harm, and enhance patient satisfaction.

Loeys-Dietz syndrome and Goldenhar syndrome unveiled together.

Haemifacial microsomia is an asymmetrical congenital tissue malformation developed from the first and second branchial arches with or without multi-system involvement. Alternatively recognised as Goldenhar syndrome or oculoauriculovertebral spectrum (OAVS), it is an aetiologically heterogeneous group of disorders showing dominant trends in inheritable form.We present a case of a boy in early childhood with concomitant craniofacial features of craniofacial microsomia with Loeys-Dietz syndrome. He had a unilateral hypoplastic face, asymmetrical ear malformations and multiple preauricular tags with epibulbar dermoid (features suggestive of Goldenhar syndrome). On detailed clinical evaluation, he met Beighton's criteria and was diagnosed with arterial tortuosity. Further molecular testing confirmed the diagnosis of Loeys-Dietz syndrome type II.Loeys-Dietz syndrome is characterised by aortic root enlargement or type A dissection with or without other vascular malformations and facial midline defects. Molecular testing is required to establish the diagnosis because of overlapping features with other connective tissue disorders.

Modified Valente Technique for Cauliflower Ear: Outcomes in Children at Two-Year Follow-Up : New Surgical Technique for Cauliflower Ear.

BACKGROUND: Cauliflower ear deformity, a common sequela of auricular trauma, presents an esthetic and reconstructive challenge. Existing surgical techniques have limitations, including complexity, donor site morbidity, and variable long-term outcomes. MATERIALS AND METHODS: In this case series, we present a novel and minimally invasive surgical approach for the correction of cauliflower ear deformity that adapts the Valente otoplasty technique; it combines cartilage debulking with helical rim release and Mustardé mattress stitches to restore ear contour and reduce the risk of recurrence. The procedural steps include bielliptic post-auricular skin and soft tissue incision, release of the cartilaginous spring, removal of excess fibrocartilaginous tissue, cartilage reshaping with suture to restore contour, and tissue redistribution to promote adherence of skin to the cartilage framework. RESULTS: Outcomes were evaluated in 7 patients (9 ears) with cauliflower ear deformity, assessing surgical duration, complications, patient satisfaction, and esthetic outcomes at two years after surgery. The mean surgical duration per patient was 52 ± 17 minutes, including 2 bilateral procedures. Follow-up at 24 months showed favorable esthetic outcome in all patients with sustained improvements in auricular contour and symmetry with neither loss of the shape nor recurrence of deformity. Patients reported high satisfaction and improved quality of life, with mean Glasgow Children Benefit Questionnaire scores of 99.3 ± 6.3. CONCLUSIONS: This technique thus demonstrated lasting correction of cauliflower ear with favorable cosmetic outcomes, low risk of complications, and high patient satisfaction. Further investigations and longer-term follow-up are warranted to validate the technique's durability and expand its application to older and more diverse patient populations. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these evidence-based medicine ratings, please refer to the Table of contents or the online Instructions to Authors www.springer.com/00266 .

Prograde transposition for anteriorly low-set earlobe in lobule-type microtia reconstruction.

BACKGROUND: Lobule transposition, a common procedure in auricle reconstruction, has been successfully performed over the past few decades. However, the transposition methods for unilateral microtia with evident asymmetry of bilateral earlobe positions still remain a challenge. The objective of this study was to investigate the application of prograde transposition for anteriorly low-set earlobes. METHOD: A total of 25 patients with lobule-type microtia with anteriorly low-set residual earlobe underwent prograde transposition during auricle reconstruction between 2020 and 2022. The post-operative earlobe aesthetic assessment and patient satisfaction were evaluated, and the data on any complications that occurred when followed-up were collected. This study provides a comprehensive analysis and summary of the techniques used in earlobe transposition for auricular reconstruction. RESULTS: The patients with evident asymmetry between the residual and healthy earlobes were usually concomitant with hemifacial microsomia and the residual ear was located in the anterior and lower region. No instances of flap necrosis, hematoma, or wound dehiscence were observed following auricular reconstruction. The mean aesthetic score of the auricle was 3.52, with 23 patients attaining good or excellent aesthetic outcomes. The mean Visual Analog Scale satisfaction score was 3.68, with 24 patients reporting relative satisfaction or satisfaction. CONCLUSION: The prograde transposition of anteriorly low-set earlobe in lobule-type microtia reconstruction can effectively ensure adequate blood supply, enhance aesthetic appearance, and significantly improve patient satisfaction.

Analysis of the effect and influencing factors of EarWell auricle orthosis in the treatment of congenital auricle deformity in children.

OBJECTIVE: To observe the efficacy of EarWell ear orthosis in treating children with different types of ear deformities. METHODS: We selected 80 children aged <6 weeks with ear deformities (110 ears: 15, 30, 21, 25, and 19 ears with prominent ear, lop ear, cup ear, cryptotia, and helical rim deformity, respectively). Differences in effectiveness rate, treatment time, and incidence of complications among children with different types of auricular deformities were compared. Recurrence rates at 1 and 3 months after the treatment were compared. RESULTS: The overall success rate was 92.73 %, and the treatment effectiveness rate did not differ significantly among the children with different types of auricular malformations (P > 0.05). The correction time of the helical rim deformity was the shortest, and the correction times of the prominent and cup ears were significantly longer than those of the other groups (P < 0.05). The incidence of complications associated with helical rim deformity and lop ear was lower, and the incidence of prominent and cup ear complications was significantly higher than that in the other groups (P < 0.05). The recurrence rate in children with prominent and cup ears was higher at 1 and 3 months after correction, and children with a lop ear and cryptotia showed no recurrence at 1 and 3 months after treatment, which correlated with the correction time, incidence of complications, and recurrence rate (P < 0.05) CONCLUSION: The EarWell auricle orthosis is an effective treatment in children with auricular morphological malformations. Correction time, complication rate, and recurrence rate were related to the malformation type.

[Analysis of risk factors for congenital auriclar deformity and its different types].

Objective:To Explore the clinical characteristics,risk factors,and differences in risk factors for different types of congenital auricular deformities,in order to provide theoretical basis for precise prevention and control of congenital auriclar deformity. Methods:Full-term newborns born in the Second Affiliated Hospital of Zhengzhou University from May 2022 to January 2023 were screened for auricle malformation, general information and data were collected,,and high-risk factors were investigated withself-made questionnaire.Using a case-control study method,newborns with auriclar deformities were selected as the case group and those without auriclar deformities during the same period were selected as the control group.A case-control study was conducted to analyze the incidence rate,high-risk factors,and differences in high-risk factors for different types of auricle deformities. Results:A total of 1 758 newborns （3 516 ears） were included in this study,including 562 newborns（927 ears） with auriclar deformities,the incidence of congenital malformations of the auricle is 26.37%.Among them,289 ears （8.22%） were helical rim deformity,244 ears （6.94%） were lidding/lop ear,166 ears （4.72%） were mixed deformities,131 ears （3.73%） were prominent/cup ear,79 ears （2.25%） were Stahl's ears,16 ears （0.46%） were abnormal conchal crus,and 2 ears （0.06%） were cryptotia.Maternal history of infection in early pregnancy（OR=1.513，95%CI 1.119-2.045）,previous miscarriage history（OR=1.300，95%CI 1.049-1.613）,and abnormal pregnancy（OR=1.278，95%CI 1.032-1.582） are risk factors for congenital auricular malformations.There was no statistically significant difference in the history of infection（χ²=1.877，P=0.391）,previous miscarriage（χ²=4.706，P=0.095）,and abnormal pregnancy（χ²=5.026，P=0.081） among mothers with helical rim deformity,lidding/lop ear,and mixed deformities. Conclusion:The incidence rate of congenital auricle deformity is high, with common malformations such as helical rim deformity, lidding/lop ear,and mixed deformities. Congenital auricular deformity is caused by various factors, the same risk factor has roughly the same impact on different types of morphological abnormalities.

A publicly available newborn ear shape dataset for medical diagnosis of auricular deformities.

Early and accurate diagnosis of ear deformities in newborns is crucial for an effective non-surgical correction treatment, since this commonly seen ear anomalies would affect aesthetics and cause mental problems if untreated. It is not easy even for experienced physicians to diagnose the auricular deformities of newborns and the classification of the sub-types, because of the rich bio-metric features embedded in the ear shape. Machine learning has already been introduced to analyze the auricular shape. However, there is little publicly available datasets of ear images from newborns. We released a dataset that contains quality-controlled photos of 3,852 ears from 1,926 newborns. The dataset also contains medical diagnosis of the ear shape, and the health data of each newborn and its mother. Our aim is to provide a freely accessible dataset, which would facilitate researches related with ear anatomies, such as the AI-aided detection and classification of auricular deformities and medical risk analysis.

Incidence of audiologic or otolaryngologic evaluation in patients with external ear anomalies.

PURPOSE: The relationship between specific external ear anomalies (EEA) and hearing loss has been previously described. However, there is no literature regarding the appropriate evaluation of patients with EEA by audiology or otolaryngology. The objective of this study was to determine the incidence of audiologic or otolaryngologic evaluation of patients with EEA. MATERIALS AND METHODS: A retrospective review of charts was conducted following approval from the institutional review board at Boston Medical Center. Charts of patients younger than 18 years old with EEA, identified using International Classification of Diseases (ICD)-9 codes 380-380.99, 744, and 744.4 and ICD-10 codes H61.90-92, Q16.0-16.9, Q17.0-17.9, Q18.0-18.2, from January 2012 to January 2019 were reviewed. Primary variables included incidence of audiologic and otolaryngologic evaluation, newborn hearing screen and audiometry results, and completion of surgical intervention. Binary logistic regressions were conducted for each group for diagnostic, procedural, and demographic characteristics. RESULTS: A total of 723 patients were diagnosed with EEA from January 2012 to January 2019. Of these patients, 327 (45.2 %) were evaluated by audiology and 327 (45.2 %) were evaluated by otolaryngology. Of the 364 patients who obtained audiograms, 63 (17.3 %) demonstrated hearing loss. Surgical procedures were performed on 119 (16.5 %) patients, with the most common procedure being excision of the EEA (n = 79, 66.4 %). A total of 468 patients had a documented newborn hearing screen. Failure of newborn hearing screen and presence of microtia were associated with increased otolaryngologic and audiologic evaluation. CONCLUSIONS: A majority of patients with EEAs do not obtain audiologic or otolaryngologic evaluation.