A new active bone-conduction implant: surgical experiences and audiological outcomes in patients with bilateral congenital microtia.

PURPOSE: First-generation bone bridges (BBs) have demonstrated favorable safety and audiological benefits in patients with conductive hearing loss. However, studies on the effects of second-generation BBs are limited, especially among children. In this study, we aimed to explore the surgical and audiological effects of second-generation BBs in patients with bilateral congenital microtia. METHODS: This single-center prospective study included nine Mandarin-speaking patients with bilateral microtia. All the patients underwent BCI Generation 602 (BCI602; MED-EL, Innsbruck, Austria) implant surgery between September 2021 and June 2023. Audiological and sound localization tests were performed under unaided and BB-aided conditions. RESULTS: The transmastoid and retrosigmoid sinus approaches were implemented in three and six patients, respectively. No patient underwent preoperative planning, lifts were unnecessary, and no sigmoid sinus or dural compression occurred. The mean function gain at 0.5-4.0 kHz was 28.06 ± 4.55-dB HL. The word recognition scores improved significantly in quiet under the BB aided condition. Signal-to-noise ratio reduction by 10.56 ± 2.30 dB improved the speech reception threshold in noise. Patients fitted with a unilateral BB demonstrated inferior sound source localization after the initial activation. CONCLUSIONS: Second-generation BBs are safe and effective for patients with bilateral congenital microtia and may be suitable for children with mastoid hypoplasia without preoperative three-dimensional reconstruction.

Analysis of predictive factors related to long-term hearing and temporal bone development in congenital microtia.

OBJECTIVE: To investigate the predictive factors of long-term hearing threshold and temporal bone development in children with congenital microtia (CM). METHODS: 74 patients (92 ears) with CM enrolled, which all had auditory brainstem response (ABR) results during infancy or toddlerhood, pure tone audiometry (PTA) and high-resolution computed tomography (HRCT) results during childhood or adolescence, and had not undergone any surgery. We compared the relationship between ABR, auditory steady-state response (ASSR), the affected side, auricular morphology, presence of external auditory canal stenosis or atresia, PTA average, mastoid pneumatization, Jarhsdoerfer scores, and wether cholesteatoma exists. RESULTS: The average age of ABR in 92 ears was 2.72 ± 3.52 years old, PTA was 7.26 ± 2.51 and HRCT was 6.91 ± 2.76 years old. ABR-AC was related to PTA average, mastoid pneumatization, Jarhsdoerfer scores, and wether cholesteatoma exists in CM. While ABR-ABG was related to all of these factors except Jarhsdoerfer score, and ABR-BC had no relationship with any of them. ASSR only showed correlation with frequencies of 1, 2 kHz and was related to Jarhsdoerfer score, with no other correlations observed. The impaired ear side showed no relevance. However, auricular morphology was related to all of these factors except wether cholesteatoma exist. External auditory canal stenosis or atresia was related to PTA average, but unrelated to mastoid pneumatization. CONCLUSION: The ABR examination in the infant stage plays a crucial role in predicting the long-term hearing and temporal bone development in patients with CM.

Maxillofacial prosthetics and digital technologies: Cross-sectional study of healthcare service provision, patient attitudes, and opinions.

PURPOSE: Digital technologies are continuously improving the accuracy and quality of maxillofacial prosthetics, but their impact on patients remains unclear. This cross-sectional study aimed to analyze the impact of facial prosthetics service provision, patients' perception, and digital technology on prostheses construction. MATERIALS AND METHODS: All patients who presented for evaluation and management of facial defects between January 2021 and December 2021 at the ENT clinic were eligible for study enrollment. Patients requiring prosthetic reconstruction of their missing facial parts were included in the study. Forty-five questionnaires were delivered, inquiring about the patients' prosthetic demographics, prosthesis manufacture using 3D technologies, and their perceptions and attitudes. RESULTS: A total of 37 patients responded (29 males, eight females; mean age 20.50 years). The congenital cause was the highest among other causes (p = 0.001) with auricular defects being the highest (p = 0.001). A total of 38 prostheses were constructed and 17 prostheses were retained by 36 craniofacial implants (p = 0.014). The auricular and orbital implants success rates were 97% and 25%, respectively. The implant locations were digitally planned pre-operatively. Digital 3D technologies of defect capture, data designing, and 3D modeling were used and perceived as helpful and comfortable (p = 0.001). Patients perceived their prosthesis as easy to handle, suited them, and they felt confident with it (p = 0.001). They wore it for more than 12 h daily (p = 0.001). They were not worried that it would be noticed, and found it comfortable and stable during various activities (p = 0.001). Implant-retained prosthesis patients were more satisfied with it, and found it easy to handle and stable (p = 0.001). CONCLUSIONS: Congenital defects are the main cause of facial defects in the study country. The overall acceptance of maxillofacial prostheses was good, showing high patient perception and satisfaction. Ocular and implant-retained silicone prostheses are better handled, more stable, and the latter is more satisfying than traditional adhesive prostheses. Digital technologies save time and effort invested in manufacturing facial prostheses.

Vascular variation of temporoparietal fascia in microtia associated with hemifacial microsomia.

The study analyzed vascular variations in microtia associated with hemifacial microsomia (HFM). A retrospective analysis was conducted on 47 patients with microtia and HFM, who underwent computed tomography angiography between November 2011 and May 2022. The vascular course and branching supplying the TPF were analyzed. Craniometric measurements were conducted to determine the horizontal distance from the porion and fronto-zygomatic suture (F-Z suture) to the vessels. On the affected side, the TPF was primarily supplied by either the superficial temporal artery (STA) or the postauricular artery-originated STA (Po-STA). The Po-STA (n = 29) was more prevalent than the STA (n = 18), and mostly exhibited a single frontal branch (n = 20). Craniometric analysis revealed that the Po-STA was closer to the porion, ear vestige, and F-Z suture than the STA on the non-affected side. Furthermore, a significant correlation was observed between the severity of mandibular hypoplasia and presence of Po-STA variation (Cramer's V = 0.498, p = 0.005). Microtia associated with HFM exhibits vascular variations in the TPF - in particular, a unique Po-STA variation. The Po-STA is prone to injury during ear reconstruction because of its proximity to the external auditory canal and ear vestige. Surgeons should be cautious of these anatomical variations for safer ear reconstruction procedures, and utilize preoperative imaging for meticulous planning.

An ear-shaped film with a "leg".

Background: The reconstructed auricle projection is an essential element of ear reconstruction. The novel use of an ear-shaped film with one or two "legs" can successfully provide a healthy auricular contour, length, and width, hence improving the three-dimensional (3D) contour of the reconstructed auricle. Methods: Sixty-one patients (31 men and 30 women) with unilateral ear reconstruction (22 on the left and 39 on the right side) who underwent auricular reconstruction using the novel ear-shaped film at the Plastic Surgery Hospital of the Chinese Academy of Medical Sciences between February 2021 and June 2022 were enrolled in this retrospective study. Results: Using the Jarque-Bera and paired t-test, we found no statistically significant differences between the reconstructive and healthy ears in terms of length (5.93±0.56 vs. 5.89±0.49 cm, P=0.208), width (3.15±0.31 vs. 3.13±0.30 cm, P=0.224), height (2.48±0.33 vs. 2.51±0.36 cm, P=0.079), and perimeter (10.83±1.06 vs. 10.69±0.95 cm, P=0.164), using the novel ear-shaped film. The reconstructed auricle location was deemed satisfactory for all patients and their families. Conclusions: The novel ear-shaped film may reflect the structure and height of the auricle during ear reconstruction surgery. Implementing this method is easy, and its impact is significant. This technique can be widely used in all types of otoplasties.

The role of MRI in the prenatal diagnosis and classification of fetal microtia.

OBJECTIVE: To investigate the role of MRI in the diagnosis and classification of fetal microtia. METHODS: Ninety-five fetuses with suspected microtia based on ultrasound and MRI performed within 1 week were enrolled in this study. The diagnosis based on MRI was compared with postnatal diagnosis. Among the microtia cases suspected on the basis of MRI, mild and severe cases were further classified. In addition, external auditory canal (EAC) atresia was evaluated by MRI in 29 fetuses with a gestational age > 28 weeks, and the accuracy of MRI in the diagnosis and classification of microtia was determined. RESULTS: Of 95 fetuses, 83 were considered to have microtia on the basis of MRI, 81 were confirmed to have microtia, and 14 were found to be normal according to postnatal diagnosis. Among 190 external ears in 95 fetuses, 40 ears were suspected to have mild microtia, and 52 ears were suspected to have severe microtia on the basis of MRI. According to the postnatal diagnosis, mild and severe microtia were confirmed in 43 and 49 ears, respectively. Among the 29 fetuses with a gestational age > 28 weeks, 23 ears were suspected to have EAC atresia according to MRI and 21 ears were ultimately confirmed to have EAC atresia. The accuracy of MRI in diagnosing microtia and EAC atresia was 93.68% and 93.10%, respectively. CONCLUSION: MRI shows good performance in diagnosing fetal microtia and has the potential to evaluate its severity on the basis of classification and EAC status. CLINICAL RELEVANCE STATEMENT: This study was aimed at investigating the role of MRI in the diagnosis and classification of fetal microtia. MRI shows good performance and can help evaluate microtia severity and EAC atresia, thus allowing for better clinical management. KEY POINTS: • MRI is a useful adjunct to prenatal ultrasound. • MRI has a higher accuracy rate than ultrasound in diagnosing fetal microtia. • The accurate classification of fetal microtia and the diagnosis of external auditory canal atresia through MRI may help guide clinical management.

Efectividad de la reconstrucción de la oreja con cartílago costal en el tratamiento de microtias / Effectiveness of Auricle Reconstruction with Costal Cartilage for the Treatment of Microtias

Introducción: La reconstrucción de la oreja en la actualidad es un problema difícil de solucionar. Se han propuesto varias técnicas quirúrgicas y solo las que se basan en el uso de cartílago costal autólogo son las que se aceptan a nivel mundial. Objetivo: Evaluar la efectividad de la técnica de reconstrucción del pabellón auricular descrita por Burt Brent. Métodos: Se realizó un estudio cuantitativo con diseño descriptivo de corte transversal con 41 pacientes que presentaron microtia congénita o perdida adquirida de la oreja; a quienes se les realizó reconstrucción auricular con cartílago costal autólogo tratados en el Hospital "William Soler", el Centro de Investigaciones Médico-Quirúrgica y Clínica Central "Cira García" en el Servicio de Cirugía Plástica desde 1994 hasta 2019. Se describió y se documentó la técnica empleada descrita por Burt Brent. Se utilizó una escala de 10 puntos que se basó en la anatomía auricular normal para la valoración de los resultados. Resultados: Se encontraron 32 pacientes portadores de microtia congénitas (78,04 %) y 9 pérdidas traumáticas (21,95 %), predominó el sexo femenino (56,09 %). La incidencia fue mayor en el lado derecho (68,75 %) en pacientes con microtia congénita. En el 95 % de los casos se alcanzaron resultados favorables y satisfactorios. Conclusiones: La reconstrucción del pabellón auricular requiere el empleo de un fragmento de cartílago costal de suficiente tamaño, forma y proyección. La clave consiste en esculpir un marco cartilaginoso de la oreja y mantener estos detalles a través de la piel lo más semejante a la oreja normal. Para un resultado satisfactorio se requiere una alta especialización.

Introduction: Auricle reconstruction is, nowadays, a difficult problem to solve. Several surgical techniques have been proposed and only those based on the use of autologous costal cartilage are accepted worldwide. Objective: To evaluate the effectiveness of the auricle reconstruction technique described by Burt Brent. Methods: A quantitative study with a cross-sectional descriptive design was carried out with 41 patients who presented congenital microtia or acquired loss of the ear and who were performed auricle reconstruction with autologous costal cartilage in the plastic surgery service at Hospital "William Soler", Centro de Investigaciones Médico-Quirúrgicas and Clínica Central "Cira García", from 1994 to 2019. The used technique described by Burt Brent was, in turn, described and documented. A 10-point scale based on the normal atrial anatomy was used to assess the outcomes. Results: Thirty-two patients with congenital microtia (78.04 %) and nine traumatic losses (21.95 %) were found, with a predominance of the female sex (56.09 %). The incidence was higher on the right side (68.75 %) in patients with congenital microtia. Favorable and satisfactory outcomes were achieved in 95 % of cases. Conclusions: Auricle reconstruction requires the use of a costal cartilage piece of enough size, shape and projection. The key is to sculpt a cartilaginous framework of the ear and to maintain these details through the skin as close as possible to the normal ear. A high level of specialization is required for a satisfactory outcome.

Simultaneous bilateral transcutaneous bone conduction device implantation: sound localisation and speech perception in children with bilateral conductive hearing loss.

OBJECTIVE: This study investigated the audiometric and sound localisation results in patients with conductive hearing loss after bilateral Bonebridge implantation. METHOD: Eight patients with congenital microtia and atresia supplied with bilateral Bonebridge devices were enrolled in this study. Hearing tests and sound localisation were tested under unaided, unilateral and bilateral aided conditions. RESULTS: Mean functional gain was higher with a bilateral fitting than with a unilateral fitting, especially at 1.0-4.0 kHz (p < 0.05, both). The improvement in speech reception threshold in noise with a bilateral fitting was a 2.3 dB higher signal-to-noise ratio compared with unilateral fitting (p < 0.05). Bilateral fitting had better sound localisation than unilateral fitting (p <0.001). Four participants who attended follow up showed improved sound localisation ability after one year. CONCLUSION: Patients demonstrated better hearing threshold, speech reception thresholds in noise and directional hearing with bilateral Bonebridge devices than with a unilateral Bonebridge device. Sound localisation ability with bilateral Bonebridge devices can be improved through long-term training.

A novel method to accurately locate the reconstructed auricle.

Background: Congenital microtia is a common congenital disease in children, the cause of which is still unclear. At present, the main treatment for congenital microtia is ear reconstruction. Accurately locating of the reconstructed ear on the affected side before ear reconstruction surgery is difficult, while it is the key of successful operation. Our ear reconstruction team has developed a novel method to accurately locate the reconstructed auricle. This novel method has achieved good results in clinical practice. Methods: Thirty patients with unilateral ear reconstruction, who underwent auricle reconstruction using our invented auricle reconstruction positioning method in the Plastic Surgery Hospital of Chinese Academy of Medical Sciences from January 2020 to July 2021, were enrolled in this study. Results: Through Wilcoxon signed rank test, we found that there was no statistical difference between the mean distance from the highest point of the patient's normal ear to the central axis of the nose and that from the highest point of the reconstructed ear to the central axis of the nose (P>0.05). Meanwhile, there was no statistical difference between the mean distance from the lowest point of the patient's normal ear to the central axis of the nose and that from the lowest point of the reconstructed ear to the central axis of the nose (P>0.05). The satisfaction rate of patients and their families to the location of the reconstructed auricle was 100%. Conclusions: The novel method of locating the reconstructed auricle employs simple materials. The implementation process is easy, and the effect is significant. To a certain extent, it solves the difficulty of locating the reconstructed auricle in ear reconstruction operation. Although this method can only be applied to patients with unilateral microtia, we recommend it for locating the reconstructed auricle by every plastic surgeon.

Hearing impairment and ear anomalies in craniofacial microsomia: a systematic review.

The aim of this systematic review was to review the literature on hearing impairment and ear anomalies in patients with craniofacial microsomia and to determine their prevalence. Sixty-two records including 5122 patients were included. Ear anomalies were present in 52-100% of patients. The most reported external ear malformations were microtia, pre-auricular tags, and atresia of the external auditory canal. Ossicular anomalies were the most reported middle ear malformations, whereas the most reported inner ear malformations included oval window anomalies, cochlear anomalies, and anomalies of the semicircular canals. Hearing loss in general was reported in 29-100% of patients, which comprised conductive hearing loss, mixed hearing loss, and sensorineural hearing loss. Between 21% and 51% of patients used hearing aids, and 58% underwent a surgical intervention to improve hearing. The relationship between different phenotypes of craniofacial microsomia and the type and severity of hearing loss is mostly unclear. In conclusion, the high prevalence of ear and hearing anomalies in patients with craniofacial microsomia underlines the importance of audiological screening in order to facilitate individual treatment.

Primer tiempo de reconstrucción auricular en microtia: tallado de la maqueta de cartílago / First stage microtia reconstruction: carving of the cartilage framework

Introducción y objetivo: El secreto del éxito en la reconstrucción auricular se basa en dos pilares fundamentales: la forma y la cobertura de la nueva oreja. El motivo principal por el cual el cartílago costal es el material de elección para la reconstrucción, es porque al ser tejido autólogo, puede colocarse inmediatamente bajo la piel del remanente micrótico y la región mastoidea adyacente. Otras ventajas del cartílago costal son que existe en cantidad adecuada para tallar una oreja tridimensional con secuelas mínimas, y que es flexible, pero con la rigidez necesaria para resistir las fuerzas de retracción cicatricial, manteniendo su forma a largo plazo. Para utilizar este material es imprescindible realizar un entrenamiento intensivo en el arte de la talla, ya que el resultado final dependerá de la calidad y el detalle de la maqueta. El objetivo de este artículo es explicar la técnica quirúrgica de confección del armazón paso a paso, para facilitar la tarea al cirujano que se inicia, y aportar detalle al más experimentado, a fin de brindarle a la futura oreja la mejor cobertura posible: la piel local, que jamás se debe resecar ni comprometer para brindar una cobertura de inferior calidad en pos de la colocación de un material aloplástico. Material y método: Describimos la edad adecuada para la cirugía, los estudios preoperatorios necesarios, los tipos de armazones auriculares, la técnica quirúrgica paso a paso: preparación de los moldes, toma del cartílago costal, tallado de la maqueta y cuidados postoperatorios. Además, presentamos nuestra casuística entre los años 2011 a 2020. (AU)

Background and objective: The key to the success in auricular reconstruction relies in two equally important pillars: coverage and structure of the new ear. Costal cartilage is the material of choice due to its autologous nature. It can be placed directly under the vascularized and supple native skin of the microtic remnant and adjacent mastoid region and it´s available in large quantities to reproduce the complex spatial structure of an ear, with minimal sequelae at the donor site. It is flexible, yet rigid enough to withstand the forces of contraction during healing, maintaining its shape in the long term. The surgeon must practice exhaustively to learn the framework carving, because the end result will rely mostly in the quality and details of the cartilage ear. In this paper we describe a step- by- step surgical technique, in the aim to simplify the task for surgeons who are learning their first steps in auricular reconstruction, and explain some technical details for more experienced surgeons, in order to give the future ear the best coverage possible: the native skin, which should never be discarded and replaced for a coverage of less quality to favor the placement of an alloplastic material. Methods: We describe the appropriate age for surgery, the necessary preoperative studies, the types of auricular frames, the surgical technique step by step: preparation of the molds, taking of the rib cartilage, carving of the model and postoperative cares.In addition, we present our casuistry between 2011 to 2020. (AU)

Metabolomic characterization of congenital microtia: a possible analysis for early diagnosis.

Background: Although metabolic abnormalities have been deemed one of the essential risk factors for growth and development, the relationship between metabolic abnormalities and microtia is still unclear. In this study, we aimed to establish a cell model of microtia and the changes of serum metabolites in patients with microtia. Methods: After constructing a cell model of microtia with low expression of BMP5, we performed integrative metabolomics analysis. For the altered metabolites, the content of glycerophosphocholine (PC), triacylglycerol (TG), and choline in the serum of 28 patients (15 patients with microtia and 13 controls) with microtia was verified by enzyme-linked immunosorbent assay (ELISA). Results: Detailed metabolomic evaluation showed distinct clusters of metabolites between BMP5-low expressing cells and normal control (NC) cells. The cell model of microtia had significantly higher levels of TG, PC, glycerophosphoethanolamine (PE), sphingomyelin, sulfatide, glycerophosphoglycerol, diacylglycerol, and glycosphingolipid. The main abnormal metabolites were mainly concentrated in the glycerophospholipid metabolism pathway, and PC and choline were closely related. In the serum of patients with microtia, the contents of PC, TG, and choline were significantly increased. Conclusions: The individual serum samples confirmed the different metabolites between patients with microtia and controls. In particular, we showed that a newly developed metabolic biomarker panel has a high sensitivity and specificity for separating patients with microtia from controls.

An Unusual Delayed Complication of Rib Graft Microtia Reconstruction After Two Decades: A Case Report.

Microtia comprises a spectrum of congenital malformation characterized by partial deformities of the auricular components to complete absence of pinna. Surgical reconstructions of this anomaly are often complex and technically challenging. Infection, hematoma, and skin necrosis with resultant exposed cartilage graft tend to occur in the early phase of post-operative care. Herein, we report a case of a spontaneous auricular abscess with exposed cartilage framework 20 years following rib cartilage reconstruction. To our knowledge, this is the first case of such an occurrence. The treatment options are discussed, along with the review of the literature.

Application of digital technology and three-dimensional silicone cartilage models in auricular reconstruction surgery / 中华医学美学美容杂志

Objective:To establish silicone cartilage models of donor-sites for the microtia patients by using digital technology, and to explore the application of surgical simulation in auricular reconstruction.Methods:From June 2018 to October 2019, 19 congenital microtia patients underwent thoracic CT scans and following three-dimensional costal cartilage imaging with Mimics software at the Nanfang Hospital, Southern Medical University. Among these patients, 16 were males and 3 were females. The mean age of patients was 16 years (range 8 to 35 years). Silicon cartilage models were produced by 3D printing and used for surgical planning and preoperative simulation in ear framework fabrication. Cartilaginous framework was sculptured according to the simulation during operation. Patients were followed up for a minimum of six months to evaluate the size, outline, height and auriculocephalic angle of the reconstructed ear. The satisfactory outcomes of the patients were scored according to a 5-point Likert scale.Results:All the patients received the surgical simulation and sculpture training with silicone cartilage models before operation. Auricular reconstruction was completed successfully according to the simulation. The duration of sculpture was shortened to 1-1.5 hours. There were no serious complications, such as hematoma, inflammation, skin necrosis and framework exposure. The contour of reconstructed ear was natural and clear over a 6 months follow-up, and all the patients were satisfied with their surgical outcomes.Conclusions:With the application of digital technology and silicone cartilage models by 3D printing to the surgical planning and training in microtia patients, patient-specific framework is fabricated with precisely assembling, which not only shortens the operation time, but also provides the unexperienced surgeons with a safe and effective training of ear framework fabrication.

[Clinical analysis of congenital microtia complicated with retroauricular subperiosteal abscess in 7 cases].

Objective:To dissect the etiology and clinical features of congenital microtia with retroauricular subperiosteal abscess, and to explore its pathogenesis and prognosis. Methods:Among 178 patients with congenital microtia, 7 cases concomitant with " retroauricular subperiosteal abscess" were collected in this retrospective study . All of the 7 patients underwent mastoidotomy, the lesions were cleared, and secretions were sent to the bacterial culture test. According to the middle ear lesions, we performedopen mastoidectomy + tympanoplasty in 3 cases， wall mastoidectomy + tympanoplasty in 3 cases of andradical mastoidectomy in 1 case.The wound healing was observed subsequently， and the patients were followed up 3 months later. Results:The patients were mainly young. The average of air-bone gap before operation was （57.14±9.51） dB. The average ofair-bone gap after operation was （40.00±11.54） dB, which was lower than that before （17.14±11.12） dB. The operation can effectively control the flow of pus and improve hearing. Postoperative ear abscess was effectively controlled, and there is no recurrence, after half a year of follow-up. Conclusion:Early diagnosis of mastoiditis is of great significance for congenital microtia complicated with retroauricular subperiosteal abscess. It should be diagnosed and intervened as soon as possible. Once it develops into abscess, tympanoplasty should be performed as soon as possible, for cleaning lesions and draining obstruction, subsequently as to provide favorable conditions for the repair of auricular malformation.

Reconstruction of congenital microtia after ear canaloplasty using V-Y advancement of a temporal triangular flap.

BACKGROUND: Reconstruction of congenital microtia remains challenging, particularly in patients with a history of ear canaloplasty due to insufficient regional soft tissue. The insertion of a tissue expander prior to implantation of the cartilage framework has traditionally been employed. However, this procedure could induce additional morbidity. Herein, we present a method using V-Y advancement of a temporal triangular flap to gain additional soft tissue in these challenging cases. METHODS: Congenital microtia patients with a history of ear canaloplasty who underwent auricular reconstruction using the Nagata technique between 2016 and 2020 were reviewed. To obtain additional soft tissue, V-Y advancement of a temporal triangular flap was performed concurrently with implantation of the costal cartilage framework, without prior insertion of a tissue expander. The outcomes of these patients with respect to postoperative complications and esthetics were evaluated. RESULTS: Eight patients with bilateral lesions were included. No specific complications developed after the first-stage surgery. However, one patient experienced complications after the second stage (auricular elevation). An analysis of the esthetic results showed most patients had excellent outcomes, achieving a satisfactory convolution. The median number of operations needed to complete reconstruction was 2, which was fewer than required using the conventional method with prior insertion of a tissue expander. CONCLUSIONS: In patients with a history of previous canaloplasty, V-Y advancement of a temporal triangular flap could serve as an alternative to tissue expansion for microtia reconstruction. This technique provided reliable and satisfactory results with a reduced number of surgical stages.

Craniofacial microsomia: Reflections on diagnosis and severity assessment based on a series of cases.

This study aims to discuss diagnostic criteria and severity assessment for craniofacial microsomia (CFM). A series of 61 patients with diverse CFM phenotypes had their clinical data collected by experienced dysmorphologists using a single protocol. Genetic abnormalities were searched through karyotype and chromosomal microarray analysis. Sex ratio, prenatal risk factors, and recurrence rate corroborated the literature. Despite the wide variability of clinical findings, ear disruption was universal. Eight patients were assigned as syndromic, four of whom had demonstrable genetic alterations. The majority of patients (67.2%) fulfilled four known diagnostic criteria, while 9.8% fulfilled one of them. Data strengthened disruptions of the ear and deafness as a semiotically valuable sign in CFM. Facial impairment should consider asymmetry as a mild expression of microsomia. Spinal and cardiac anomalies, microcephaly, and developmental delay were prevalent among extra craniofacial features and should be screened before planning treatment and follow up. The severity index was able to recognize the less and the most affected patients. However, it was not useful to support therapeutic decisions and prognosis in the clinical scenario due to syndromic and non-syndromic phenotypes overlapping. These issues make contemporary the debate on diagnostic methods and disease severity assessment for CFM. They also impact care and etiopathogenetic studies.

Auricular reconstruction using Medpor combined with different hearing rehabilitation approaches for microtia.

BACKGROUND: Congenital microtia-atresia affects patients in two specific ways: severe conductive hearing loss and difficulty in integrating into social environments due to auricle malformation. AIMS/OBJECTIVES: To investigate the safety and efficacy of single-stage auricular reconstruction and hearing rehabilitation in children with microtia and external auditory canal atresia. MATERIAL AND METHODS: From January 2016 to December 2019, we included 32 patients with microtia and external canal atresia who received auricle reconstruction with high-density polyethylene (Medpor) framework and three different hearing rehabilitation approaches at the Ninth People's Hospital affiliated to Shanghai Jiao Tong University School of Medicine. Twenty patients underwent the traditional external auditory canal and middle ear repair (EACR), eight patients were implanted with Bonebridge (BB) devices, and four patients were implanted with bone-anchored hearing aid (BAHA) in one stage. Postoperative changes in auricle morphology and hearing and speech recognition and occurrence of complications were evaluated. RESULTS: After 6-24 months of follow-up, the auricle shape recovered well in all three groups, and the average score of 14 fine structures in the auricle was 9.43 (EACR), 10.67 (BB), and 9.75 (BAHA) points. The average score of auricle symmetry was 6.83 (EACR), 6.00 (BB), and 6.44 (BAHA) points. No significant differences in auricle shape were observed among the three groups (p > .05). After surgery, the average hearing improvement in the BB group was 43.33 dB, and the average speech recognition threshold declined to 42.28 dB. In the BAHA group, the average hearing improvement was 35 dB, and the average speech recognition threshold declined to 33.5 dB, similar to that of the BB group. However, in the EACR group, the average hearing improvement was only 4.13 dB, and the average speech recognition threshold declined to 11.36 dB. No vertigo, tinnitus, cerebrospinal fluid leakage, facial nerve paralysis, osseointegration failure, and other complications occurred in all the patients. In the EACR group, auricle stent fracture, ear canal restenosis, and canal atresia occurred in one patient each. In the BAHA group, two patients developed local ear infections. CONCLUSIONS AND SIGNIFICANCE: The procedure of single-stage auricular reconstruction and hearing rehabilitation for microtia is feasible and effective. The appropriate method of hearing reconstruction should be determined by evaluating the development of the inner and middle ear of the patients. For those patients with poor development of the mastoid and ossicular chain, hearing aid devices are recommended to achieve a stable and significant hearing effect.