[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.

[Contemporary diagnosis and management of congenital microtia and aural atresia : Part 2: Overview of therapeutic approaches]. / Aktuelle Diagnostik und Therapie bei Ohrmuscheldysplasien und Gehörgangsfehlbildungen : Teil 2: Übersicht der verschiedenen Therapieansätze.

Congenital malformations of the pinna and aural atresia can result in major aesthetic and functional deficits. Knowledge about embryologic developments and established classification systems is an essential requirement when dealing with affected patients. Early detection of deficiencies and introduction of appropriate diagnostic measures is vital to initiate adequate therapies and prevent long-term disabilities. Treatment for malformations of the pinna-if requested-is mostly surgical, infrequently an epithesis is applied. As in other surgical fields, tissue engineering will likely play a crucial role in the future. Treatment of aural stenosis and atresia aims at improvement of hearing levels and prevention of secondary complications like cholesteatoma and chronic otorrhea. Auditory rehabilitation comprises a spectrum from conventional hearing aids to invasive hearing implants, the latter being favored in recent years.

Bioengineered human tissue regeneration and repair using endogenous stem cells.

We describe a general approach to produce bone and cartilaginous structures utilizing the self-regenerative capacity of the intercostal rib space to treat a deformed metacarpophalangeal joint and microtia. Anatomically precise 3D molds were positioned on the perichondro-periosteal or perichondral flap of the intercostal rib without any other exogenous elements. We find anatomically precise metacarpal head and auricle constructs within the implanted molds after 6 months. The regenerated metacarpal head was used successfully to surgically repair the deformed metacarpophalangeal joint. Auricle reconstructive surgery in five unilateral microtia patients yielded good aesthetic and functional results. Long-term follow-up revealed the auricle constructs were safe and stable. Single-cell RNA sequencing analysis reveal early infiltration of a cell population consistent with mesenchymal stem cells, followed by IL-8-stimulated differentiation into chondrocytes. Our results demonstrate the repair and regeneration of tissues using only endogenous factors and a viable treatment strategy for bone and tissue structural defects.

Effectiveness of Bone Conduction Hearing Aids in Young Children with Congenital Aural Atresia and Microtia.

BACKGROUND The aim of this study was to assess the effectiveness of bone conduction hearing aids in children under 2 years old who have congenital microtia and atresia. MATERIAL AND METHODS This prospective study involved 42 children under 2 years old with congenital microtia and atresia who were divided into 2 groups: 21 with unilateral defect and 21 with bilateral defect. All children were provided with bone conduction hearing aids on a softband. Air and bone auditory thresholds were assessed by auditory brainstem responses (ABRs). The LittlEARS questionnaire was used to evaluate auditory development at baseline and after 6 months of hearing aids use. Behavioral observation audiometry (BOA) was used to assess auditory thresholds and compare aided and unaided hearing. RESULTS After 6 months of hearing aid use, the total score of the LittlEARS questionnaire in children with unilateral defect was 24±5.60, while children with bilateral defect achieved a result of 26.29±6.17. Hearing thresholds in both groups with bone conduction hearing aids improved significantly and approached the normal level. CONCLUSIONS Our results confirm that bone conduction hearing aids provide an effective method of auditory rehabilitation for children with conductive and mixed hearing loss caused by microtia and atresia. Using bone conduction hearing aids in such children is crucial for proper hearing, speech, and language development.

Long-term evaluation of development in patients with bilateral microtia using softband bone conducted hearing devices.

OBJECTIVE: The ability of early intervention with softband bone conducted hearing device (BCHD) to ensure normal development of speech, language and psychosocial situations remains undetermined. We aimed to evaluate auditory and speech development, as well as psychosocial situations of children with bilateral microtia fitted with a softband BCHD for 3-5 years. METHODS: The study included 53 patients with bilateral microtia and 53 sex- and age-matched children with normal hearing. Auditory development was evaluated using the Meaningful Auditory Integration Scale (MAIS) and Categories of Auditory Performance (CAP). Speech Intelligibility Rating (SIR) and Meaningful Use of Speech Scale (MUSS) were used to assess speech development. The psychometric properties of these patients were evaluated using Achenbach's Child Behavior Checklist (CBCL), and subjective benefits were measured using the Glasgow Children's Benefit Inventory (GCBI) questionnaire. RESULTS: The average unaided and aided hearing thresholds measured using VRA were 73.8 ± 5.1 dB HL and 30.5 ± 6.0 dB HL, respectively. The total MAIS scores of the patients were 89.6 ± 9.6% and 93.0 ± 8.8% of normal hearing children at the last follow-up. The CAP scores of the two groups were 6.5 ± 1.3 and 6.9 ± 0.3, respectively. The mean MUSS score of the patients and the control group were 31.9 ± 7.0 and 34.3 ± 6.0, respectively. The mean SIR score of the two groups were 4.6 ± 0.7 and 4.8 ± 0.4. CBCL found that only two patients could be considered problematic psychosocially. The average benefit score on the GCBI was 32.9 ± 29.3. CONCLUSIONS: Softband BCHD significantly improved auditory development in patients with bilateral microtia, with speech development reaching the level of normal hearing peers. No significant behavioral problems were found in the patients, with subjective evaluations showing that softband BCHD improved patient quality of life.

The effect of anatomical variables and use of the Lifts system on hearing outcomes after implantation of an active transcutaneous bone conduction device in bilateral congenital conductive hearing loss.

BACKGROUND: Malformations of the temporal bone present different challenges to the implantation of a transcutaneous active bone conduction device, such as Bonebridge (Med-el, Innsbruck, Austria). This study aims to describe the benefits of high-resolution computed tomography (HRCT) in preoperative assessment and to analyze whether characteristics of the mastoid process, intraoperative compression of the dura or sigmoid sinus, and the use of the Lifts system, lead to differences in audiological performance after implantation. METHODS: We examined 110 cases of congenital microtia. The structure of the temporal bone was examined using HRCT and a 3D simulation software program. The mean anteroposterior mastoid bone thickness from the external auditory canal to the sigmoid sinus was measured (a measurement referred to as "AP", hereafter). Sound field threshold (SFT), speech reception threshold (SRT) in noise, and word recognition score (WRS) in quiet, before and after implantation, were also measured. Independent variables were recorded in all patients: mastoid type (well pneumatized or poorly pneumatized), the presence of dural or sigmoid sinus compression, and the use of the Lifts system. RESULTS: We found that the mean AP in the non-compression group was 16.2 ± 2.3 mm and in the compression group, 13.1 ± 2.9 mm (p < 0.001). We analyzed the hearing improvement of patients grouped by mastoid development, dural or sigmoid sinus compression, and use of the Lifts system, and found that these factors did not interact and that they had no influence on the hearing outcomes (p > 0.05). CONCLUSIONS: The AP dimension in the non-compression group was significantly larger than that in the compression group. This finding combined with the ROC curve analysis revealed the AP dimension was a high-accuracy predictor of potential surgical complications involving the dura and sigmoid sinus compression. Further analysis revealed that there was no interaction between the chosen variables: mastoid type, dural or sigmoid sinus compression, and the use of the Lifts system, and that all of these factors had no significant impact on hearing performance. Bonebridge was shown to produce effective and stable bone conduction and to improve patients' hearing performance.

Lattice Boltzmann simulation of swelling of an implant for microtia manufactured with IPN hydrogel.

The swelling characteristics of an implant for microtia treatment manufactured with IPN hydrogel was achieved using a three-dimensional lattice Boltzmann model. Dimensionless units of length and mass were employed. Comparisons between computational data and experimental values obtained by an adsorption isotherm test of a hydrogel implant immersed in Ringer's solution at a constant temperature of 310.15 K achieve coefficients of determination between 0.98782 and 0.99669. The results demonstrate that the thickness of the sample has a great impact on the time required to achieve thermodynamic equilibrium. The method presented here is suitable to simulate the swelling of hydrogels of arbitrary geometries.

Tissue engineering the human auricle by auricular chondrocyte-mesenchymal stem cell co-implantation.

Children suffering from microtia have few options for auricular reconstruction. Tissue engineering approaches attempt to replicate the complex anatomy and structure of the ear with autologous cartilage but have been limited by access to clinically accessible cell sources. Here we present a full-scale, patient-based human ear generated by implantation of human auricular chondrocytes and human mesenchymal stem cells in a 1:1 ratio. Additional disc construct surrogates were generated with 1:0, 1:1, and 0:1 combinations of auricular chondrocytes and mesenchymal stem cells. After 3 months in vivo, monocellular auricular chondrocyte discs and 1:1 disc and ear constructs displayed bundled collagen fibers in a perichondrial layer, rich proteoglycan deposition, and elastin fiber network formation similar to native human auricular cartilage, with the protein composition and mechanical stiffness of native tissue. Full ear constructs with a 1:1 cell combination maintained gross ear structure and developed a cartilaginous appearance following implantation. These studies demonstrate the successful engineering of a patient-specific human auricle using exclusively human cell sources without extensive in vitro tissue culture prior to implantation, a critical step towards the clinical application of tissue engineering for auricular reconstruction.

Active Transcutaneous Bone Conduction Implant: Middle Fossa Placement Technique in Children With Bilateral Microtia and External Auditory Canal Atresia.

AIM: The aim of this study is to present the middle fossa technique (MFT) as an alternative for patients who cannot undergo traditional surgery for active transcutaneous bone conduction implants (ATBCI) due to their altered anatomy or desire for future aesthetic reconstruction. DESIGN: A case series descriptive study was designed. The MFT was developed. Preoperative and postoperative information from 24 patients with external auditory canal atresia (EACA) and implanted with ATBCI was reviewed. RESULTS: A total of 24 children with bilateral EACA received implants in the middle cranial fossa. Their average age was 12. Of these patients, eight had an associated congenital disorder: Goldenhar Syndrome, Treacher Collins Syndrome or the Pierre Robin Sequence. The average follow-up was at 17 months (ranging from between 2- and 36 mo) and there were no major complications. Four patients showed skin erythema at the processor site after turn on, which was solved by lowering the magnet strength. One patient had a scalp hematoma that required puncture drainage. The hearing thresholds went down on average from 66.5 to 25.2 dB 1 month after turn on. Speech recognition improved respectively from 29.4% without and 78.9% with a bone conduction hearing aid to 96.4%. CONCLUSION: MFT placement of the ATBCI was proven to be safe and effective and a viable option for treating pediatric patients with EACA who cannot receive implants at the sinodural angle or in the retrosigmoidal position because of their altered anatomy and/or desire for future aesthetic reconstruction.

Parental preferences for the first consultation for microtia.

OBJECTIVES: The aim of our study was to investigate subjective information concerning parental experiences and preferences with regard to the initial information that is provided right after the birth of a child with microtia. The analysis of these data is intended to help professionals improve the way in which such conversations are conducted. As a result, future parents may feel better informed and, hence, better fit to cope with challenges they may encounter having a child with microtia. METHODS: A self-administered questionnaire was sent to 106 parents and caretakers of children with microtia who visited the annual International Microtia and Atresia Conference in the Netherlands, May 2016. RESULTS: Eighty-seven questionnaires were returned (response rate 82%). Results revealed that 26% of the participants did not receive any information about the condition in the perinatal period. Participants who did receive information right after birth were informed by pediatricians and otolaryngolosits in most of the cases. Plastic surgeons and maxillofacial surgeons were the less commonly serving specialties in this role. A majority of the participants experienced their informing consultation as either being "terrible", or "bad". Parents desired more information about all domains regarding microtia. Development, clothing and appearance, and psychology were believed to be the least important domains. CONCLUSIONS: Parents are dissatisfied with several aspects of the initial information that is provided right after the birth of a child with microtia. We believe that there is a need for correct information and patient- and family centered care administered by multiple disciplines.

In Vitro Regeneration of Patient-specific Ear-shaped Cartilage and Its First Clinical Application for Auricular Reconstruction.

Microtia is a congenital external ear malformation that can seriously influence the psychological and physiological well-being of affected children. The successful regeneration of human ear-shaped cartilage using a tissue engineering approach in a nude mouse represents a promising approach for auricular reconstruction. However, owing to technical issues in cell source, shape control, mechanical strength, biosafety, and long-term stability of the regenerated cartilage, human tissue engineered ear-shaped cartilage is yet to be applied clinically. Using expanded microtia chondrocytes, compound biodegradable scaffold, and in vitro culture technique, we engineered patient-specific ear-shaped cartilage in vitro. Moreover, the cartilage was used for auricle reconstruction of five microtia patients and achieved satisfactory aesthetical outcome with mature cartilage formation during 2.5years follow-up in the first conducted case. Different surgical procedures were also employed to find the optimal approach for handling tissue engineered grafts. In conclusion, the results represent a significant breakthrough in clinical translation of tissue engineered human ear-shaped cartilage given the established in vitro engineering technique and suitable surgical procedure. This study was registered in Chinese Clinical Trial Registry (ChiCTR-ICN-14005469).

Microtia: definición, clasificación y tratamiento: revisión de la literatura / Microtia: definition, classification and treatment: Literature review

El pabellón auricular es una importante unidad estética en el ser humano. Existe un grado variable de malformaciones, en Chile tiene una incidencia de 1 por cada 5600 nacidos vivos. Anatómicamente el pabellón auricular está compuesto por tres estructuras principales: complejo hélix antihélix, concha y lóbulo. Es según la anomalía que se presente en la anatomía externa la forma en que se clasificará, existiendo diversos sistemas para esto, algunos de ellos con utilidad anátomo quirúrgica.El eje principal del tratamiento de estas anomalías es la reconstrucción quirúrgica, teniendo disponible en la literatura variadas técnicas, como son el uso de implantes, reconstrucción protésica y la reconstrucción con injerto autólogo de cartílago costal, entre otras. Reinisch ha sido pionero en el uso de implantes para la reconstrucción, mientras que diversos autores han mostrado resultados satisfactorios con el uso de prótesis osteointegradas. Sin embargo, la técnica más aceptada y utilizada es la reconstrucción con injerto autólogo de cartílago costal, la cual fue descrita en sus inicios por Tanzer, Walton y Beahm, siendo perfeccionada posteriormente por Brent, quien propuso las bases para su desarrollo moderno. En la actualidad los métodos quirúrgicos más aceptados y utilizados son los descritos por Nagata y Firmin, ambos con excelentes resultados.

The ear is an important aesthetic unity in humans. There is a varying degree of malformations, Chile has an incidence of 1 per 5600 live births. Anatomically the pinna is composed of three main structures: helix antihelix complex, shell and lobe. It is according to the anomaly present in the external anatomy how it is classified, various systems exist for this, some of them with anatomic surgical utility. The main focus of treatment of these anomalies is the surgical reconstruction, taking various techniques available in the literature, such as the use of implants, prosthetic reconstruction and reconstruction with autologous rib cartilage graft, among others. Reinisch has pioneered the use of implants for reconstruction, while several authors have shown satisfactory results with the use of bone anchored prosthesis. However, the most accepted and used technique is the reconstruction with autologous rib cartilage graft, which was described at the beginning by Tanzer, Walton and Beahm and was later perfected by Brent, who proposed the foundation for modern development. Currently the most accepted and used surgical methods are described by Nagata and Firmin, both with excellent results.

Meier-Gorlin syndrome.

Meier-Gorlin syndrome (MGS) is a rare autosomal recessive primordial dwarfism disorder, characterized by microtia, patellar applasia/hypoplasia, and a proportionate short stature. Associated clinical features encompass feeding problems, congenital pulmonary emphysema, mammary hypoplasia in females and urogenital anomalies, such as cryptorchidism and hypoplastic labia minora and majora. Typical facial characteristics during childhood comprise a small mouth with full lips and micro-retrognathia. During ageing, a narrow, convex nose becomes more prominent. The diagnosis MGS should be considered in patients with at least two of the three features of the clinical triad of microtia, patellar anomalies, and pre- and postnatal growth retardation. In patients with short stature and/or microtia, the patellae should be assessed with care by ultrasonography before age 6 or radiography thereafter. Mutations in one of five genes (ORC1, ORC4, ORC6, CDT1, and CDC6) of the pre-replication complex, involved in DNA-replication, are detected in approximately 67-78% of patients with MGS. Patients with ORC1 and ORC4 mutations appear to have the most severe short stature and microcephaly. Management should be directed towards in-depth investigation, treatment and prevention of associated problems, such as growth retardation, feeding problems, hearing loss, luxating patellae, knee pain, gonarthrosis, and possible pulmonary complications due to congenital pulmonary emphysema with or without broncho- or laryngomalacia. Growth hormone treatment is ineffective in most patients with MGS, but may be effective in patients in whom growth continues to decrease after the first year of life (usually growth velocity normalizes after the first year) and with low levels of IGF1. At present, few data is available about reproduction of females with MGS, but the risk of premature labor might be increased. Here, we propose experience-based guidelines for the regular care and treatment of MGS patients.

The formation of human auricular cartilage from microtic tissue: An in vivo study.

OBJECTIVES: This study aimed to isolate, culture-expand and characterize the chondrocytes isolated from microtic cartilage and evaluate its potential as a cell source for ear cartilage reconstruction. Specific attention was to construct the auricular cartilage tissue by using fibrin as scaffold. STUDY DESIGN: Cell culture experiment with the use of microtic chondrocytes. DESIGN: Cell culture experiment with the use of microtic chondrocytes. METHODS: After ear reconstructive surgery at the Universiti Kebangsaan Malaysia Medical Center, chondrocytes were isolated from microtic cartilage. Chondrocytes isolated from the tissue were cultured expanded until passage 4 (P4). Upon confluency at P4, chondrocytes were harvested and tissue engineered constructs were made with human plasma polymerized to fibrin. Constructs formed later is implanted at the dorsal part of nude mice for 8 weeks, followed by post-implantation evaluation with histology staining (Hematoxylin and Eosin (H&E) and Safranin O), immunohistochemistry and RT-PCR for chondrogenic associated genes expression level. RESULTS: Under gross assessment, the construct after 8 weeks of implantation showed similar physical characteristics that of cartilage. Histological staining showed abundant lacunae cells embedded in extracellular matrix similar to that of native cartilage. Safranin O staining showed positive staining which indicates the presence of proteoglycan-rich matrix. Immunohistochemistry analysis showed the strong positive staining for collagen type II, the specific collagen type in the cartilage. Gene expression quantification showed no significant differences in the expression of chondrogenic gene used which is collagen type I, collagen type II, aggrecan core protein (ACP), elastin and sox9 genes when compared to construct formed from normal auricular tissue. CONCLUSION: Chondrocytes isolated from microtia cartilage has the potential to be used as an alternative cell source for external ear reconstruction in future clinical application.

[Efficacy of softband Ponto in young children with bilateral congenital microtia with aural atresia].

OBJECTIVE: To evaluate auditory developments and the effectiveness of children with congenital bilateral aural atresia after using softband Ponto and to compare them with children with normal hearing. METHOD: Twenty patients (age ranging from 3 months to 21 months ) with bilateral aural atresia were studied. The air and bone auditory thresholds were assessed by auditory brain stem response (ABR). The infant-todder meaning auditory integration scale (IT-MAIS) was used to evaluate the auditory development at three time levels: baseline, 3 months and 6 months. RESULT: The average unaided bone conduction hearing thresholds of patients is (17.5 ± 5.9)dB nHL,and the average air conduction hearing thresholds is (72.5 ± 9.3)dB nHL. The average VRA hearing thresholds of 5 patients is (30.5 ± 5.9) dB HL. The IT-MAIS total, detection and perception scores are improved specifically after wearing softband Ponto and approaching the normal level. CONCLUSION: Softband Ponto is suitable for infants with bilateral atresia. Results from these auditory development testing are encouraging. Softband Ponto should be used as a bridge for surgical implantations when temporal bone is thick enough.

A tutorial on implantable hearing amplification options for adults with unilateral microtia and atresia.

BACKGROUND: Patients with unilateral atresia and microtia encounter problems in sound localization and speech understanding in noise. Although there are four implantable hearing devices available, there is little discussion and evidence on the application of these devices on patients with unilateral atresia and microtia problems. OBJECTIVE: This paper will review the details of these four implantable hearing devices for the treatment of unilateral atresia. They are percuteaneous osseointegrated bone anchored hearing aid, Vibrant Soundbridge middle ear implant, Bonebridge bone conduction system, and Carina fully implantable hearing device. METHODS: Four implantable hearing devices were reviewed and compared. The clinical decision process that led to the recommendation of a device was illustrated by using a case study. CONCLUSIONS: The selection of appropriate implantable hearing devices should be based on various factors, including radiological findings and patient preferences, possible surgical complications, whether the device is Food and Drug Administration- (FDA-)/CE-approved, and the finances. To ensure the accurate evaluation of candidacy and outcomes, the evaluation methods should be adapted to suite the type of hearing device.