[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.

Role of early hearing aid experience in speech recognition in patients with bilateral congenital microtia following Bonebridge implantation: a retrospective cohort study.

PURPOSE: To identify audiological and demographic variables that predict speech recognition abilities in patients with bilateral microtia who underwent Bonebridge (BB) implantation. METHODS: Fifty patients with bilateral microtia and bilateral conductive hearing loss (CHL) who underwent BB implantation were included. Demographic data, preoperative hearing aid use experience, and audiological outcomes (including pure-tone hearing threshold, sound field hearing threshold [SFHT], and speech recognition ability) for each participant were obtained. The Chinese-Mandarin Speech Test Materials were used to test speech recognition ability. The word recognition score (WRS) of disyllabic words at 65 dB SPL signals was measured before and after BB implantation in quiet and noisy conditions. RESULTS: The mean preoperative WRS under quiet and noisy conditions was 10.44 ± 12.73% and 5.90 ± 8.76%, which was significantly improved to 86.38 ± 9.03% and 80.70 ± 11.34%, respectively, following BB fitting. Multiple linear regression analysis revealed that lower preoperative SFHT suggested higher preoperative WRS under both quiet and noisy conditions. Higher age at implantation predicted higher preoperative WRS under quiet conditions. Furthermore, patients with more preoperative hearing aid experience and lower postoperative SFHT were more likely to have higher postoperative WRS under both quiet and noisy testing conditions. CONCLUSIONS: This study represents the first attempt to identify predictors of preoperative and postoperative speech recognition abilities in patients with bilateral microtia with BB implantation. These findings emphasize that early hearing intervention before implantation surgery, combined with appropriate postoperative fitting, contributes to optimal benefits in terms of postoperative speech recognition ability.

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.

[Management of microtia in children]. / Prise en charge de la microtie chez l'enfant.

Microtia is a malformation of the external ear with a prevalence of 1-17/10 000 births. It is mostly unilateral and is responsible for conductive hearing loss. It is present in some congenital syndromes but is most frequently isolated. It can be associated with atresia of the external auditory canal (EAC) as well as middle and inner ear malformations. While cosmetic issues seem to be of primary concern to parents at the time of diagnosis, functional audiological rehabilitation is essential for the development of stereophonic hearing in children. We present here a review of the current ENT management of microtia from birth to adulthood.

La microtie est une malformation concernant l'oreille externe et a une prévalence de 1 à 17/10000 naissances. Présente de manière majoritairement unilatérale, elle est responsable d'un déficit auditif de transmission. Elle est présente dans certains syndromes congénitaux, mais est le plus fréquemment isolée. Elle peut être associée à une atrésie du conduit auditif externe (CAE) ainsi que des atteintes de l'oreille moyenne et interne. Si l'atteinte esthétique semble être au premier plan dans la préoccupation des parents lors du diagnostic, la réadaptation fonctionnelle audiologique est importante pour le bon développement d'une audition stéréophonique chez les enfants. Nous présentons ici un rappel de la prise en charge actuelle ORL de la microtie, de la naissance jusqu'à l'âge adulte.

Experience of the 'Ear Glove' in children with microtia.

INTRODUCTION: Microtia is a congenital condition which can be found in isolation or as part of a syndrome. The key factors to consider when treating a child with microtia are hearing, speech and language development, cosmesis, and the psychological impact on the patient as well as the family. As children age and become more self-aware, the anxiety about transition from primary to secondary school can often be a trigger for carers and child to want a cosmetic solution at a younger age. Any form of cosmetic surgery ideally requires a child with an understanding of what is involved, as well as sufficient growth and anatomy to provide soft tissue resources for surgery. An additional issue for some children with microtia is the concern about adding to their already 'different' appearance by using a bone conduction solution/hearing implant. We present the outcomes of a novel non-surgical prosthesis 'Ear Glove' offered to pediatric patients with microtia. METHODS: Children with microtia are seen in the multidisciplinary outpatient clinic and reviewed by the team which includes an Otolaryngologist, Audiologist, Plastic surgeon and Maxillofacial prosthetist. When discussing cosmesis, all reconstruction options are explored. These include a 'no treatment' option, both adhesive and implant-retained prosthetic ears, and autologous and/or MedporⓇ ear reconstruction (age appropriate). All children who chose to undergo the adhesive non-surgical prosthetic option 'Ear Glove' for microtia were identified by our prosthetic department (n = 9), and their outcomes reviewed. RESULTS: Nine children have been fitted with the 'Ear Glove', with all 9 achieving excellent symmetry and aesthetics. Seven patients continue to successfully use their prostheses either daily or for special occasions. Two of these patients also decided to undergo bone anchored hearing implant surgery. One patient opted to change his treatment plans and chose 'no treatment' after feeling he preferred his 'little' ears. Finally, one patient reported the daily use of adhesive to be a deterrent. No skin reactions to the adhesive were reported in any patient. CONCLUSIONS: The 'Ear Glove' is increasingly being used by microtia patients in our department to good effect. This non-surgical alternative allows young patients to appreciate the cosmetic results of the surgical options before committing to an invasive procedure.

Ultrasonographic evaluation of costal cartilage for microtia reconstruction surgery.

PURPOSE: Autogenous costal cartilage grafts have gained the golden standard method in microtia reconstruction. Right now, there was no useful method to assess the quality of costal cartilage before microtia reconstruction surgery. The purpose of this study was to evaluate the role of ultrasonography in assessing costal cartilage in patients who were ready to do microtia reconstruction surgery. METHODS: A prospective controlled study was conducted to collect 65 patients who underwent microtia reconstruction and underwent ultrasonography of costal cartilage before operation. The results of costal cartilage calcification and honeycombed phenomenon measured by ultrasonography were compared with those during operation. The age-specific patterns in calcification and honeycombed phenomenon were explored. RESULTS: According to the results of ultrasonography, the positive rate of calcification was 10.9% in patients under 18 years old, while 80% in patients over 18 years old. The positive rate of honeycombed phenomenon was 2.8% in patients under 12 years old, 42.9% in patients between 12 and 18 years old, and 25% in patients over 18 years old. Compared with intraoperative results, the accuracy rate of ultrasonography for calcification was 100%. The accuracy rate for honeycombed phenomenon was 83.3%. CONCLUSION: Ultrasonography has high accuracy rate in assessing the calcification and honeycombed phenomenon of the costal cartilage, which was of vital importance for microtia reconstruction. The quality of costal cartilage changed with the age.

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.

Serial measurements of facial asymmetry using a wooden tongue depressor in patients with congenital microtia.

BACKGROUND AND OBJECTIVES: Congenital microtia may be associated with hemifacial microsomia, but little is known about their correlation and development with aging. Historically, facial asymmetry is easily assessed by observing the occlusal cant using a tongue depressor. We serially measured the occlusal cant in children with microtia to evaluate change in facial asymmetry with growth. METHODS: Since 2011, frontal photographs of patients with congenital microtia biting a tongue depressor were obtained and reviewed. The occlusal angle was compared between the baseline and final photographs, and the change was compared between cant-positive (>3° at baseline) and cant-negative (<3° at baseline) groups. Multivariate analysis was conducted to determine variables associated with the change in occlusal angle. RESULTS: Overall, 105 patients were enrolled. With a mean age of 5.4 years at baseline and a mean follow-up of 3.9 years, clinically significant aggravation was observed in 15.4% and 24.2% of cant-positive and cant-negative patients, respectively. Hemifacial microsomia (OR, 4.825; p = 0.005) and occlusal angle at baseline (OR, 0.821; p = 0.045) were associated with aggravation, but the severity of microtia showed no significant association. CONCLUSIONS: When hemifacial microsomia was present, the occlusal cant seemed to be aggravated in children with microtia at later ages. When the occlusal cant was present without noticeable hemifacial microsomia, some compensation in facial asymmetry was expected. The use of a wooden tongue depressor is a simple, non-invasive, and radiologic hazard-free aid to detect notable change in facial asymmetry in children with microtia.

Long-term Outcomes of Clip Coupler Implantation in Patients with Unilateral Congenital Aural Atresia.

OBJECTIVE: To evaluate the long-term safety and efficacy of the Clip coupler attached to the stapes head in patients with unilateral congenital aural atresia (CAA). METHODS: This single-center retrospective study included 16 Mandarin-speaking patients who had unilateral microtia accompanied by CAA. All patients were divided into two groups: the short-term follow-up group (n = 9) and the long-term follow-up group (n = 7). The floating mass transducer of the Vibrant Soundbridge (VSB) was positioned in the stapes head by the Clip coupler. The safety of the VSB was investigated by comparing preoperative and postoperative bone-conduction (BC) thresholds as well as by complications. The effectiveness was evaluated by functional gain (FG), word recognition score (WRS), speech reception threshold (SRT) and signal-to-noise ratio (SNR). RESULTS: Pre- and post-operative BC thresholds were no different in all patients. And no complications developed. VSB-aided thresholds in the free-field had improved significantly in both short- and long-term follow-up groups. The improvements of WRS were observed in two groups. The monosyllabic VSB-aided WRS in the long-term follow-up group was significantly higher than that in the short-term follow-up group. When speech was from the impaired ear and noise presented to the side of normal ear (SVSBNCL), lower SNRs were found in two groups after VSB implantation. However, there was no statistical difference in aided SNR between the two groups at SVSBNCL status. CONCLUSIONS: Our results show that the FMT connected to the stapes head is a secure and useful device for patients with unilateral CHL/MHL, not only in terms of improved hearing thresholds, but also improved speech intelligibility in quiet and noisy environments.

Acquisition limitations of bone conduction hearing devices in children with unilateral microtia and atresia.

OBJECTIVE: To characterize the use of bone conduction hearing devices (BCHD) for hearing management in children with unilateral congenital aural atresia (CAA) at a tertiary pediatric center's microtia clinic while assessing challenges in acquisition. BCHD generally provides better audiologic outcomes than atresiaplasty in pediatric CAA. BCHD is formally recommended for only bilateral CAA, although literature has begun to show benefit in unilateral CAA. METHODS: A retrospective case series of patients born between 2014 and 2018 with unilateral microtia at an urban tertiary care children's hospital collected information on demographics, CAA laterality, hearing loss (HL) severity, management, and acquisition. Statistical analysis aided characterization of BCHD use. RESULTS: 131 patients (65% males) were included with a mean age of 3.5 years. 108 (82%) patients with unilateral microtia were used for further analysis, and right sided microtia was most common (67.6%). Of patients with auditory brain response (ABR) testing available, 70% demonstrated conductive HL, 16% mixed HL, 1% sensorineural HL, 6% no HL secondary to grade 1 microtia, and 7% were pending evaluation. Overall, 46 (42.6%) patients with unilateral microtia obtained a BCHD. 68.7% of those offered a BCHD after ABR opted for acquisition. The average length of time from offer to fit was about 6 months. CONCLUSION: Even with thorough consultation and follow up, less than half of the patients with unilateral CAA received a BCHD. Missed initial ABR appointments that lead to BCHD recommendation seem to be the greatest limiting factor, while demographics did not play a confounding role. Family education and future research on obstacles preventing BCHD use in unilateral CAA will help standardize management and improve hearing within this critical auditory period.

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.

Comparative effects of unilateral and bilateral bone conduction hearing devices on functional hearing and sound localization abilities in patients with bilateral microtia-atresia.

Background: Various amplification options are available for patients with congenital bilateral conductive hearing loss. Unilateral bone conduction hearing device (BCHD) is widely used for these patients, whereas benefits of bilateral BCHDs in certain subgroups of patients require further exploration.Objectives: To evaluate functional and directional hearing in patients with unilateral Bonebridge (MEDEL) and contralateral ADHEAR (MEDEL) devices.Materials and methods: This study included 32 patients (20 males, 12 females), of mean age 11.8 years (range 7-27 years). Hearing thresholds, speech perception and sound localization were tested three months after activation of the Bonebridge under three conditions: unaided, unilateral BHCD (Bonebridge) and bilateral BHCDs (Bonebridge plus contralateral ADHEAR). Patient acceptance of these devices in daily life was evaluated by questionnaire.Results: Compared with unaided, the mean hearing thresholds (0.5, 1, 2, and 4 kHz) and speech perception with unilateral BCHD and bilateral BCHDs were improved significantly (p < .05 each). Markers of directional hearing ability, including percentages of accurate responses, bias angles and RMS errors, were significantly better with bilateral BCHDs than unilateral BHCD (p < .05 each). Questionnaire revealed high patient satisfaction with both unilateral and bilateral devices.Conclusions: Functional hearing and sound localization abilities were better with bilateral BCHDs than unilateral BCHD.

Utilization of Diagnostic Testing for Renal Anomalies and Congenital Heart Disease in Patients with Microtia.

OBJECTIVE: Congenital ear anomalies are associated with congenital cardiac and renal defects. Renal ultrasounds, electrocardiogram, and echocardiogram can be utilized for diagnosis of these concurrent defects. No standard of care exists for the workup of patients with microtia. The goals of this study were to describe the utilization of diagnostic testing for cardiac and renal anomalies and to identify their prevalence in patients with microtia. STUDY DESIGN: Case series with chart review. SETTING: Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center. SUBJECTS AND METHODS: This study is an Institutional Review Board-approved retrospective review of consecutive patients born between 2002 and 2016 who were diagnosed with microtia and seen in the otolaryngology clinic at a tertiary care children's hospital. Demographics, sidedness and grade of microtia, comorbid diagnoses, and details of renal and cardiovascular evaluations were recorded. Factors associated with retroperitoneal ultrasound and cardiac testing were assessed with logistic regression. RESULTS: Microtia was present in 102 patients, and 98 patients were included as they received follow-up. Microtia was associated with craniofacial syndrome in 34.7% of patients. Renal ultrasound was performed in 64.3% of patients, and 12.9% of patients with ultrasounds had renal aplasia. Cardiac workup (electrocardiogram or echocardiogram) was completed in 60.2% of patients, and of this subset, 54.2% had a congenital heart defect. CONCLUSION: Diagnostic testing revealed renal anomalies and cardiac defects in patients with isolated microtia at a higher rate than in the general population. This suggests the need for further evaluation of the role of routine screening in patients with microtia.

Long-term Outcomes of Bone Conduction Hearing Implants in Patients With Bilateral Microtia-atresia.

OBJECTIVES: To evaluate the long-term outcomes of three different types of bone conduction hearing implants (BCHI)-BAHA, Ponto, and Bonebridge-in Mandarin-speaking patients with bilateral microtia-atresia. METHODS: This cohort study enrolled 59 patients affected by bilateral microtia-atresia, with an upper bone conduction threshold limit of 30 dB HL at frequencies of 0.5 to 4 kHz. All subjects underwent unilateral BCHI surgery, including 26 (18 males, 8 females, of mean age 8.7 ±â€Š1.9 yr) implanted with BAHA devices; 10 (7 males, 3 females, of mean age 11.7 ±â€Š2.8 yr) implanted with Ponto devices; and 23 (14 males, 9 females, of mean age 9.0 ±â€Š1.8 yr) implanted with Bonebridge devices. The main outcome measures included long-term audiological benefits, patient satisfaction, and complications. Each subject acted as his or her own control. RESULTS: Two years after BCHI surgery, the mean hearing thresholds in the BAHA, Ponto, and Bonebridge groups had improved to 22.6 ±â€Š1.6 dB HL, 21.6 ±â€Š1.2 dB HL, and 22.5 ±â€Š1.5 dB HL, respectively. The mean percentages of subjects in these three groups recognizing speech at 65 dB SPL under quiet conditions were 97.7 ±â€Š4.2%, 96.3 ±â€Š1.1%, and 94.4 ±â€Š9.4%, respectively, whereas the mean percentages recognizing speech under noise conditions (signal:noise ratio +5) were 87.0 ±â€Š1.8%, 89.3 ±â€Š9.3%, and 85.3 ±â€Š4.7%, respectively. Questionnaires revealed patients' benefits and satisfaction with this surgery. Three (11.5%) of 26 patients in the BAHA group and 1 (10%) of 10 in the Ponto group experienced skin irritation, but all recovered after local treatment. Five (19.2%) patients in the BAHA group and two (20%) in the Ponto experienced abutment extrusion about 6 months postoperatively, with all achieving good results after revision surgery to replace the abutment. One (3.8%) patient in the BAHA group experienced local chronic inflammation and underwent surgery to replace the BAHA with a Bonebridge implant. One (4.3%) patient in the Bonebridge group developed a local infection 3 months postoperatively and underwent implant removal. CONCLUSIONS: All three BCHIs were well tolerated after long-term follow-up, and all improved audiometric thresholds and the intelligibility of speech in the presence of both quiet and noise. These implants should be considered valid and safe options for the functional rehabilitation of patients with bilateral microtia-atresia.

[Congenital microtia with aural atresia or stenosis accompany with first branchial cleft anomaly: report of 5 cases].

Objective: To summarize the experience of the diagnosis, treatment and effects of the cases with coexistence of first branchial cleft anomaly(FBCA) and microtia with congenital aural atresia or stenosis(external auditory canal stenosis, EACS). Method: This was a retrospective study. The clinical data of 5 patients with microtia and EACS in Beijing Tongren Hospital of Capital Medical University from October 2015 to March 2018 were collected, including 3 males and 2 females, aged from 5 to 28 years. The clinical characteristics, imaging findings, treatment methods and effects of 5 patients were analyzed. Result: The 5 cases were all coexistence of EACS and FBCA, three of who associated with cholesteatoma of external auditory canal. CT showed external auditory canal stenosis with soft tissue shadow, sometimes gas or bone septum found inside, filling in the external auditory canal, combined with canal bone destruction irregularly. All patients underwent surgical resection of FBCA, 3 patients accompanied by cholesteatoma resection and canalplasty. The postoperative follow-up ranged from 10 to 39 months, and no recurrence of infection was observed. Conclusions: EACS and FBCA both result from maldevelopment of the first branchial cleft. These two malformations, FBCA and EACS with or without cholesteatoma, can occur simultaneously, in which situation CT shows external auditory canal stenosis with soft tissue shadow inside. These patients underwent surgical resection of FBCA combined with cholesteatoma resection with good result.

Pyoderma gangrenosum: a presenting sign of myelodysplastic syndrome in undiagnosed Fanconi anemia.

A 26-year-old man with a history of congenital bilateral microtia, unilateral renal agenesis, left aural atresia, and right external auditory canal occlusion admitted for right rib cartilage graft harvest and left ear re-construction. Following surgery, an ulceration with violaceous borders and a yellow fibrinous base unresponsive to broad-spectrum antibiotics developed at the harvest site. The wound was expanding and not responsive to systemic broad-spectrum antibiotics. Biopsy revealed a dense dermal infiltrate of neutrophils with negative tissue cultures consistent with pyoderma gangrenosum (PG). He was treated with systemic, intralesional, and topical steroids, as well as doxycycline. Three weeks after the diagnosis of PG, he was found to have persistent anemia and leukopenia. Bone marrow aspiration analysis was consistent with hypocellular myelodysplastic syndrome and genetic testing was consistent with Fanconi anemia. There is a well-known association of PG with hematological disorders. Fanconi anemia is a rare genetic hematologic disorder with congenital defects leading to bone marrow failure and malignancy in long-standing disease. In our patient, we consider his development of PG a paraneoplastic sign associated with the onset of his hypocellular myelodysplastic syndrome.

Simultaneous auricular reconstruction and transcutaneous bone conduction device implantation in patients with microtia.

BACKGROUND/PURPOSE: The Bonebridge (BB) is a newly designed transcutaneous bone conduction hearing implant. We describe, for the first time, simultaneous BB implantation and different surgical techniques of auricular reconstruction for microtia patients with aural atresia/stenosis. METHODS: Ten patients with unilateral or bilateral microtia underwent BB implantation combined simultaneously with either total auricular reconstruction using bespoke hand-carved Medpor framework or second stage auricular projection using autologous costal cartilage framework. Auditory aided and unaided sound fields were evaluated using (1) a pure-tone average (PTA4), (2) a speech reception threshold (SRT), and (3) a Speech Discrimination Score (SDS) at a sound level of 65 dB SPL. RESULTS: All patients and their families were satisfied with the aesthetic outcome of their constructed ears with no requests for further revision. No major complications were encountered. One patient developed minor partial skin graft epidermolysis that healed uneventfully, and another patient had a three month period of auditory acclimatization to the BB device that resolved. Postoperatively, the mean aided PTA4 decreased by 35.35 dB, while the SRT was 54.5 dB HL unaided and 28 dB HL with use of a BB sound processor. The SDS increased by 16.4%-65 dB SPL. CONCLUSION: Simultaneous BB implantation during either total auricular reconstruction or framework projection for microtia patients who have aural atresia/stenosis is feasible and safe. This approach reduces operative stages, thereby minimizing schooling/occupational disruption and time to total microtia reconstruction and auditory rehabilitation.

Preferential Associated Malformation in Patients With Anotia and Microtia.

OBJECTIVE: Few epidemiological studies have investigated the external ear malformations anotia and microtia. The authors' study aimed to investigate the relationships between age, sex, type of microtia, and birth place and associated malformation as seen in 1 private Japanese clinic. METHODS: Cases of anotia/microtia that presented in Nagata Microtia and Reconstructive Plastic Surgery Clinic (Saitama, Japan) between 2005 and 2018 were included in the study. The authors identified 1896 patients with anotia/microtia with or without associated malformation. Most were primary cases, with some secondary reconstruction cases wherein the primary surgery was performed at another hospital. Cases were classified with Nagata classification; lobule type, small concha type, concha type, and anotia. Cryptotia was also observed in this study. RESULTS: Among the patients, 61.1% were male, 85.4% had unilateral defects (69.0% LB), and 59.1% had a right-sided defect. Most patients were less than 1 year old (15.0%) or 8 to 10 years old (5.5%-6.3%) on first examination, while 58% were from the Kanto region, including Tokyo. Regarding concomitant disorders, 32.7% had an accompanying malformation, while 13.3% had associated syndromes (eg, craniofacial microsomia, Treacher Collins syndrome). To conclude, in the authors' clinic, most cases of anotia/microtia were LB, unilateral, and seen in male patients. Information regarding several characteristic clinical features was obtained, especially that clefts and musculoskeletal deformities of the skull/face and jaw were the main accompanying malformations.