A Study on the Nonsurgical Correction Treatment Age Window and Long-Term Follow-Up of Infants With Congenital Ear Anomalies in China.

OBJECTIVES: To evaluate the efficacy of ear molding across various initial ages and analyze challenges encountered by infants beyond the optimal treatment age window. METHODS: A retrospective review of 331 infants (527 ears) treated with EarWell was conducted over 5 years from January 2017 to March 2022 at a single center. The treatment duration of the ear molding, success rate, recurrence rate, and complication rate were analyzed among the 3 age groups. Concentrate on evaluating treatment outcomes for infants with an initial age exceeding 42 days. RESULTS: The mean age at initial treatment was 25±28 days. In addition, it includes a child with cryptotia who is 3.5 years old (1278 d). The mean duration of treatment was 7±5 weeks. In the long-term follow-up, the overall treatment success rate was 92%, with 467 ears (88.6%) showing improvement without recurrence, 30 ears (5.7%) experiencing varying degrees of recurrence, and 30 ears (5.7%) showing no improvement or complete recurrence. A total of 20 infants (3%) developed mild skin complications during treatment. CONCLUSIONS: Ear molding is a safe and effective option for the treatment of congenital ear anomalies, with a low recurrence rate during long-term follow-up. For infants with congenital auricular anomalies aged over 42 days, ear molding remains a viable option. Treatment success may be influenced by the age at treatment, the subtype of anomalies, and relies on the assessment of a specialized otologist, expert procedural techniques, as well as thorough understanding and cooperation from parents.

[Clinical features of congenitally enlarged bony portion of Eustachian tube].

Objective:To investigate the clinical features of patients with congenitally enlarged bony portions of the Eustachian tube（ET）. Methods:The medical history, physical examination, hearing test, temporal bone high resolution computed tomography（HRCT） of six patients（nine ears） with congenitally enlarged bony portion of the ET were retrospectively analyzed. Results:Four patients were men and two were women. The minimum, maximum, and average ages were 5, 21, and（14.7±6.4） years, respectively. Three malformations were bilateral and three were left-sided. Three ears had conductive hearing loss（average bone and air conduction thresholds were 13.7 dB and 71.3 dB）, three had mixed hearing loss（average bone and air conduction thresholds were 27.7 dB and 83.7 dB）, and one had extremely severe sensorineural hearing loss. The average maximum length and width of the enlarged bony ET on temporal bone HRCT were（22.61±2.94） mm and（6.50±2.33） mm, respectively. The enlargement was combined with an external auditory canal malformation in six ears, narrow tympanic cavity in six, tympanic antrum malformation in five, ossicular chain malformation in seven, cochlear malformation in six, helicotrema malformation in three, vestibule widening in two, semicircular canal malformation in three, vestibular window malformation in six, facial nerve abnormality in five, internal auditory meatus malformation in two, low middle cranial fossa in eight, and severe internal carotid artery malformation in one. Conclusion:Bony ET enlargement is a rare congenital middle ear malformation which could combined with other ear malformations. Patients can have no ET dysfunction but different patterns of hearing loss. The defect is usually found unintentionally during imaging, and the HRCT of temporal bone is significant.

Clinical experience of an adhesive bone conduction hearing system in children with congenital single-sided deafness.

OBJECTIVES: This study aimed to investigate the effects of an adhesive bone conduction device (aBCD) in children with congenital single-sided deafness (SSD). Specifically, we examined whether the aBCD elicits improvement in the speech perception ability of children with congenital SSD and whether using this device would adversely affect the horizontal localisation abilities of these children. METHODS: Thirteen school-aged children with SSD and seven children with Normal Hearing (NH) were included in this study. Speech perception in noise was measured using the Mandarin Speech Test Materials and sound localisation performance was evaluated using broadband noise stimuli (0.5-20 kHz), randomly played from seven loudspeakers at different stimulus levels (65-, 70-, and 75-dB SPL). RESULTS: All children with SSD showed inferior speech perception and sound localisation performance compared with children with NH. The aBCD use remarkably improved the speech perception abilities of these children under quiet and noise conditions; however, their sound localisation abilities neither improved nor deteriorated. CONCLUSION: This study reveals the effectiveness and safety of a non-surgical aBCD in paediatric patients with SSD. Our results provide a theoretical basis for early hearing intervention with an aBCD in children with congenital SSD who are temporarily unable to undergo ear surgery. LEVEL OF EVIDENCE: Level 3.

[Summary of the clinical characteristics of tuberculous otitis media].

Objective:The purpose of this study was to analyze and summarize the clinical characteristics and diagnostic methods of tuberculous otitis media（TOM）, to enrich clinical experience in diagnosis and treatment of tuberculous otitis media, so as to reduce missed diagnosis and misdiagnosis, and facilitate timely and effective therapy for better prognosis. Methods:This study retrospectively analyzed the clinical data of patients with tuberculous otitis media who were hospitalized in the Ear ward of our hospital and received surgical treatment from 2008 to 2022. The data of patients' clinical characteristics, radiological examination, intraoperative findings and therapeutic strategies were recorded and summarized. Results:A total of 23 cases （26 ears） of tuberculous otitis media were included in this retrospective study. The most common clinical symptoms were otorrhea（thin odorless fluid）（100%） and conductive hearing loss（100%）, with a high incidence of facial paralysis（23.1%）. It was not sensitive to traditional antibiotic treatment, eg. Levofloxacin （50% effective rate only）, and relapsed soon after drug withdrawal. It was revealed that all the surgical views had gray and white tough granulation tissue hyperplasia（100%）, and 23.1% with caseous necrosis. The purpose of surgery was to clear the lesion, reduce the recurrence rate of suppurative infection, and repair the function （hearing reconstruction or facial nerve decompression） as appropriate. The paraffin pathology of granulation tissue were reported as typical granulomatous inflammation and caseous necrosis with positive acid-fast staining, which was consistent with tuberculosis. Conclusion:It was easily confused by the clinical manifestations of tuberculous otitis media and common chronic suppurative otitis media. When met with the following conditions, we should pay highly attention to suspect tuberculous otitis media: The severity of local manifestations did not match with the length of the disease; with poor tympanic membrane at the early stage with no obvious cholesteatomas, with facial paralysis or hearing loss early onset; insensitive to traditional antibiotic treatment; with extensive granulation appeared in the tympanum and or mastoid cavity, with or without caseous necrosis or dead bone in the early days. The diagnosis should be confirmed based on the acid-fast staining of the histopathological section to detect positive acid-fast bacilli. Meanwhile, multiple laboratory examination methods（such as T-spot and PCR） should be integrated synchronously to help support the diagnosis.

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.

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.

FOXI3 pathogenic variants cause one form of craniofacial microsomia.

Craniofacial microsomia (CFM; also known as Goldenhar syndrome), is a craniofacial developmental disorder of variable expressivity and severity with a recognizable set of abnormalities. These birth defects are associated with structures derived from the first and second pharyngeal arches, can occur unilaterally and include ear dysplasia, microtia, preauricular tags and pits, facial asymmetry and other malformations. The inheritance pattern is controversial, and the molecular etiology of this syndrome is largely unknown. A total of 670 patients belonging to unrelated pedigrees with European and Chinese ancestry with CFM, are investigated. We identify 18 likely pathogenic variants in 21 probands (3.1%) in FOXI3. Biochemical experiments on transcriptional activity and subcellular localization of the likely pathogenic FOXI3 variants, and knock-in mouse studies strongly support the involvement of FOXI3 in CFM. Our findings indicate autosomal dominant inheritance with reduced penetrance, and/or autosomal recessive inheritance. The phenotypic expression of the FOXI3 variants is variable. The penetrance of the likely pathogenic variants in the seemingly dominant form is reduced, since a considerable number of such variants in affected individuals were inherited from non-affected parents. Here we provide suggestive evidence that common variation in the FOXI3 allele in trans with the pathogenic variant could modify the phenotypic severity and accounts for the incomplete penetrance.

Clinical manifestations and treatment strategies for congenital aural atresia with temporomandibular joint retroposition: a retrospective study of 30 patients.

BACKGROUND: Patients with congenital aural atresia (CAA) can present with concomitant temporomandibular joint (TMJ) retroposition, implying that even with a high Jahrsdoerfer score, canaloplasty and tympanoplasty cannot be performed. Therefore, this study aimed to summarize the clinical manifestations and share our diagnostic and treatment experience of this rare entity, which has not been described previously. METHODS: Thirty patients (30 ears) with CAA and TMJ retroposition without maxillofacial dysplasia were included. Diagnosis was based on patient history, physical examination, pure-tone average test results, and temporal bone high-resolution computed tomography (HRCT) findings. Their Jahrsdoerfer scores and interventions were also recorded. RESULTS: Twenty-four and six patients among the 30 patients (males, n = 15) had CAA and TMJ retroposition on the right and left sides, respectively. Seventeen ears had a normal auricle; most had an enlarged cavum conchae and a large tragus. Twelve ears had an accessory auricle, and two had a preauricular fistula. All external auditory canals had complete atresia, including four with a shallow concavity and four with a small orifice in the cavum conchae. Temporal bone HRCT revealed poor or undeveloped tympanic temporal bone in the diseased ears, atresia in the external auditory canals, and partial/complete occupation of the mandibular condyle with or without soft tissue. The average Jahrsdoerfer score was 8.17. Thirteen patients opted for different surgeries, three wore a bone-conduction hearing aid, and fourteen chose no intervention. CONCLUSIONS: CAA with TMJ retroposition was often unilateral, typically on the right side. Most patients had normal auricles, with an enlarged cavum conchae and a large tragus ("mirror ear"). Even with a high Jahrsdoerfer score, traditional hearing reconstruction surgery could not be performed. Patients can undergo Vibrant Soundbridge or Bonebridge implantation or wear bone-conduction hearing aids to improve hearing levels, or refuse intervention because of mild hearing loss. The TMJ location can be used as a Jahrsdoerfer Grading System supplement for preoperative evaluation.

One-Year Outcomes of Ear Molding for Infants with Constricted Ear.

BACKGROUND: Ear molding is a noninvasive treatment that shows promising results for neonatal ear deformations. Little research has been reported evaluating 1-year outcomes or relapse after ear molding for ear malformations. METHODS: One-year molding efficacy for constricted ear, a common malformation that affects the aesthetic appearance of the auricle, was assessed during a single-center, prospective study conducted over a 3-year period (from May of 2017 to April of 2020). Infants with constricted ears were recruited and treated with the EarWell Infant Ear Correction System. Constricted ear classification, age at treatment application, duration of treatment, complications, and parental satisfaction were analyzed. Photographic documentation of the ears was performed before treatment, at treatment termination, and 12 months after treatment to evaluate treatment efficacy and relapse. RESULTS: Sixty patients with 91 constricted ears were recruited. The EarWell Infant Ear Correction System was initiated before 2 weeks of age for 75.0% of these patients. Successful correction was achieved in 85.8% of patients. Early molding initiation (before 14 days of age) resulted in a significantly higher success rate ( P = 0.017). Class 1 and class 2 deformities achieved better outcomes than class 3 deformities ( P = 0.001). Among the 91 auricles, 37 ears (40.7%) relapsed: 36.3% had mild relapse, 4.4% had moderate relapse, and 0% had severe relapse. The treatment duration for patients with relapse was shorter than for patients without relapse ( P = 0.035). CONCLUSION: Early ear molding is an effective treatment for constricted ear. Sufficient molding duration and consolidation periods are crucial in maintaining treatment effects. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Diagnosis and treatment of congenital stenosis of external auditory canal with external auditory canal cholesteatoma complicated with exudative effusion in the middle ear and mastoid process.

BACKGROUND: The optimal timing for surgery to promote postoperative recovery in children with congenital stenosis of the external auditory canal with external auditory canal cholesteatoma, who are susceptible to exudative inflammation of middle ear and mastoid process, is still uncertain. OBJECTIVES: To investigate the treatment of congenital stenosis of external auditory canal with external auditory canal cholesteatoma complicated with exudative inflammation. MATERIAL AND METHODS: A retrospective analysis of 45 patients with congenital stenosis of external auditory canal with external auditory canal cholesteatoma complicated with mastoiditis. Based on whether effusion had resolved at the time of surgery, the patients were divided into two groups. Pure-tone audiometry tests were performed before and after surgery for both groups. RESULTS: The average postoperative duration of dry ear was 36.38 days in Group 1 and 47.90 days in Group 2 (p < 0.05). Among patients who underwent hearing reconstruction, the average air conduction threshold decreased by 15-36 dBHL in Group 1 and by 7-22 dBHL in Group 2 (p < 0.05). CONCLUSIONS AND SIGNIFICANCE: The postoperative outcomes of dry ear and hearing improvement can be enhanced by performing operation after effusion resolution in cases of congenital external auditory canal stenosis with external auditory canal cholesteatoma exudative inflammation.

Morphological and pathological changes of Eustachian tube mucosa in an animal model of eosinophilic otitis media / Alterações morfológicas e patológicas da mucosa da tuba auditiva em um modelo animal de otite média eosinofílica

Abstract Introduction Eosinophilic otitis media is an intractable otitis media and a fairly common middle ear disease. However, the pathogenesis of eosinophilic otitis media is obscure. Objective To observe the pathological and ultrastructural changes of the Eustachian tube mucosal epithelium in rats with eosinophilic otitis media and further explore the pathogenesis of eosinophilic otitis media. Methods Animals were intraperitoneally injected with 2000 mg ovalbumin and 100 mg aluminum hydroxide (alum) on day 0, followed by 100 mg ovalbumin and 100 mg alum injection on days 7 and 14. Next they were topically boosted by daily application of 100 mg ovalbumin solution via nasal drip and intratympanic injection of 0.1 mL ovalbumin (1000 mg/mL) in the right ear (group A, n = 80) and 0.1 mL saline in the left ear as control (group B, n = 80) starting on day 21 and continuing for 14 days. The temporal bones were dissected on the 35th, 38th, 41st and 43rd day separately under anesthesia. Scanning electron microscopy, hematoxylin-eosin and toluidine blue staining were used to observe the pathological and morphological changes of Eustachian tube mucosa stained samples. Moreover, inflammatory cells and cilia were counted. Results The epithelium of the Eustachian tube in group A was swollen and thickened. The cilia were arranged in a disorderly manner and partially detached. Eosinophils infiltrated the submucosal layer of the Eustachian tube, and their number increased significantly compared with that in group B (p< 0.05). Simultaneously, mast cell degranulation was observed in group A. Scanning electron microscopy revealed that the cilia were lodged and gathered along the whole length of Eustachian tube in group A. Ciliated cell density was significantly lower than that in Group B (p< 0.01). Conclusion In the eosinophilic otitis media model, allergy caused significant changes in pathology and morphology of the Eustachian tube mucosa, affecting the normal function of the Eustachian tube which played an important role in the occurrence and development of eosinophilic otitis media.

Resumo Introdução A otite média eosinofílica é uma doença relativamente comum de orelha média; entretanto, sua patogênese é ainda obscura, assim como o tratamento. Objetivo Observar as alterações histopatológicas e ultraestruturais do epitélio da mucosa da tuba auditiva em ratos com otite média eosinofílica e investigar a sua patogênese. Método Os animais foram injetados intraperitonealmente com 2.000 mg de ovalbumina e 100 mg de hidróxido de alumínio (alúmen) no dia 0, seguido por 100 mg de ovalbumina e 100 mg de injeção de alúmen nos dias 7 e 14. Em seguida, receberam um reforço tópico através de uma aplicação diária de 100 mg da solução por gotejamento nasal e injeção intratimpânica de 0,1 mL de ovalbumina (1000 mg/mL) na orelha direita (grupo A, n = 80) e 0,1 mL de solução salina na orelha esquerda como controle (grupo B, n = 80), começou no dia 21 e continuou por 14 dias. Os ossos temporais foram dissecados nos dias 35, 38, 41 e 43 separadamente sob anestesia. Foram usadas microscopia eletrônica de varredura e coloração com hematoxilina-eosina e azul de toluidina para observar as alterações histopatológicas e morfológicas das amostras coradas de mucosa da tuba auditiva. Além disso, células inflamatórias e cílios foram contados. Resultados O epitélio da tuba auditiva no grupo A estava edematoso e espessado. Os cílios estavam dispostos de forma desordenada e parcialmente destacados. Os eosinófilos infiltraram a camada submucosa da tuba auditiva e seu número aumentou significantemente em comparação ao grupo B (p < 0,05). Simultaneamente, degranulação dos mastócitos foi observado no grupo A. A microscopia eletrônica de varredura mostrou que os cílios estavam depositados e reunidos ao longo de todo o comprimento da tuba auditiva no grupo A. A densidade das células ciliadas foi significantemente menor do que no grupo B (p < 0,01). Conclusão No modelo de otite média eosinofílica, a alergia causou alterações significativas à histopatologia e na morfologia da mucosa da tuba auditiva, afetou a função normal dela, o que desempenhou um papel importante na ocorrência e no desenvolvimento da otite média eosinofílica.

Comparing of anteroinferior and posteroinferior tympanostomy tube insertion: a cohort study.

Background: Tympanostomy tube insertion (TTI) is one of the primary treatments for Otitis media with effusion (OME) in children, a common condition in children. However, there is still no conclusion on the better choice of tube position, and no relevant research has been found in the literature. This study analyzed the results of the two commonly selected tube insert positions, anteroinferior quadrant (AQ) and posteroinferior quadrant (PQ), in order to determine which is the better choice. Methods: Children with bilateral OME who received TTI in Beijing Tongren Hospital from May 2020 to January 2021 were selected as subjects. one side on AQ and the other side on PQ randomly. Follow-up visits were arranged at 1 week, 1 month, 6 months and 12 months after surgery. The results of audiology and the tube status of the two positions were recorded and compared statistically. Results: A total of 90 patients with bilateral OME were selected. There was no difference in preoperative hearing between the 2 groups. The AQ group's average air conduction threshold was significantly higher than that of the PQ group 1 week after surgery. Although there was no significant difference in tube fall-off rate between the two groups within 6 months, it was significantly higher in AQ group than that in PQ group at 12 months after surgery. Conclusions: Compared to TTI on AQ, TTI on PQ is more stable and better improves hearing, and the operation is also more convenient for surgeons. This study provides reference for a better position of TTI in OME therapy.

Total auricular reconstruction concomitant with BONEBRIDGE implantation using a retrosigmoid sinus approach.

BACKGROUND: Retrosigmoid sinus (RS) approach was not the dominant choice for BONEBRIDGE implantation. OBJECTIVE: To investigate the efficacy and safety of auricular reconstruction concomitant with BONEBRIDGE implantation using retrosigmoid approach for aural atresia patients. MATERIALS AND METHODS: A retrospective analysis was conducted of 15 children (28 ears) who underwent auricular reconstruction using a completely expanded postauricular flap with a skin expander concomitant with retrosigmoid BONEBRIDGE implantation from July 2019 to September 2020. RESULTS: All 15 patients healed well and had no bone conduction shift. Average SFT improvement was 27.1 dB HL (p < .001). Comparison between aided and unaided speech recognition rate in quiet and in noise showed significant improvements, respectively (p < .001). After a follow-up of 21-35 months, the hearing results were stable and the aesthetic outcomes were satisfying. CONCLUSIONS AND SIGNIFICANCE: For patients who undergo auricle reconstruction using expanded postauricular flap, retrosigmoid approach can avoid interfering the flap of reconstructed auricular without increasing the surgical risk or impact the clinical efficacy, and without significantly prolonging the total anesthesia time. The integrated surgical approach is a safe, and convenient option for patients who require simultaneous auricular reconstruction with BONEBRIDGE implantation.

[Clinical analysis and surgical treatment of congenital external auditory canal stenosis complicated with external auditory canal cholesteatoma].

Objective:To investigate the clinical features, diagnosis, and treatment experience of congenital stenosis of an external auditory canal with external auditory canal cholesteatoma. Methods:The clinical data of 152 patients（153 ears） with congenital external auditory canal stenosis complicated with external auditory canal cholesteatoma treated in the Department of Otorhinolaryngology Head and Neck Surgery of Beijing Tongren Hospital affiliated to Capital Medical University from January 2009 to December 2019 were analyzed retrospectively, including the age of onset, clinical manifestations, signs, audiology, high-resolution computed tomography（HRCT） of the temporal bone, mode of operation and so on. According to the preoperative imaging findings, intraoperative findings,and pathological results, 152 patients with congenital external auditory canal stenosis with external auditory canal cholesteatoma were treated with canaloplasty and tympanoplasty while clearing the cholesteatoma. Results:All patients were followed up for 2-2.5 years, there was no recurrence of cholesteatoma, and the reconstructed external auditory canal was spacious. The hearing levels of 108 ears who underwent hearing reconstruction were significantly improved, and the average hearing threshold was reduced by 20-35 dB. Conclusion:The stenosis of the external auditory meatus is easy to be complicated with cholesteatoma of the external auditory canal,and the occurrence of cholesteatoma of the external auditory canal is directly related to the diameter of the external auditory canal meatus.But the time of occurrence of the cholesteatoma is not directly related to the diameter of the external auditory canal. Severe congenital stenosis of the external auditory canal with auricle deformity is easy to be missed and misdiagnosed due to retroauricular redness, swelling, and ulceration. For this kind of patient, cholesteatoma should be treated first, and then plastic surgery such as auricle reconstruction should be performed. Retroauricular incisions should be avoided to create conditions for auricle reconstruction in the future.

Audiological and subjective benefit with a new adhesive bone conduction hearing aid in children with congenital unilateral microtia and atresia.

PURPOSE: This study aimed to evaluate the audiological benefits and subjective satisfaction in using a new adhesive bone conduction hearing aid in children with congenital unilateral microtia and atresia. METHODS: We evaluated the effectiveness of the hearing aid using the sound field hearing threshold, speech recognition ability under quiet and noise, and subjective questionnaires in 13 children (5-15 years old). RESULTS: The mean sound field hearing threshold significantly improved with a gain of 25.4 ± 4.9 dB HL. Mean word recognition scores were ameliorated in quiet and noise by 1.9 ± 2.5% and 7.3 ± 5.3%, respectively. Speech recognition ability results in noise varied; when the speech signal and noise were presented from the front, the mean speech recognition ability improved by 2.5 ± 1.6 dB signal-to-noise ratios (SNR). When noise was presented towards the normal hearing side, speech understanding was improved by 2.9 ± 1.6 dB SNR. When the speech signal was presented from the atretic side with noise from the normal hearing side, an improvement of 5.7 ± 3.4 dB SNR (p < 0.001) was noted. However, when noise was presented towards the newly aided atretic ear, no statistical significance was found. The questionnaire results indicated that the hearing device provided benefits in speech recognition ability in different complex situations, with high satisfaction rates. CONCLUSION: The adhesive bone conduction hearing aid investigated here offers a concealed and aesthetic method to improve hearing in children with congenital unilateral microtia and atresia during their early years, with high subjective satisfaction.

[Round window implantation of vibrant soundbridge through a retrofacial approach].

Objective:To investigate the specific retrofacial approach for application of vibrant soundbridge （VSB） to the round window （RW） in patients with complex congenital ear malformation. Methods:The clinical information of four patients with congenital ear malformation were retrospectively analyzed. VSB were implanted unilaterally in these four patients via a retrofacial approach. During the operation, it is difficult to reach the RW via a classic posterior tympanotomy pathway, due to the mastoid portion of FN positioned anteriorly severely. So the round window niche was identified through a retrofacial pathway and the round window vibroplasty was completed. Results:All four patients received the round window vibroplasty successfully via retrofacial approach. Satisfied auditory benefit were confirmed after surgery. Compared with the preoperative data, clear improvements in both hearing threshold and the speech discrimination score were confirmed after VSB activation. Neither intraoperative nor postoperative surgical complications was observed in all patients. Conclusion:During the round window vibroplasty, when it was unable to access the RW through the classic facial recess pathway due to the aberrant facial nerve， the retrofacial approach could be a valid and feasible alternative approach.

Novel risk factors for craniofacial microsomia and assessment of their utility in clinic diagnosis.

Craniofacial microsomia (CFM, OMIM%164 210) is one of the most common congenital facial abnormalities worldwide, but it's genetic risk factors and environmental threats are poorly investigated, as well as their interaction, making the diagnosis and prenatal screening of CFM impossible. We perform a comprehensive association study on the largest CFM cohort of 6074 samples. We identify 15 significant (P < 5 × 10-8) associated genomic loci (including eight previously reported) and decipher 107 candidates based on multi-omics data. Gene Ontology term enrichment found that these candidates are mainly enriched in neural crest cell (NCC) development and hypoxic environment. Single-cell RNA-seq data of mouse embryo demonstrate that nine of them show dramatic expression change during early cranial NCC development whose dysplasia is involved in pathogeny of CFM. Furthermore, we construct a well-performed CFM risk-predicting model based on polygenic risk score (PRS) method and estimate seven environmental risk factors that interacting with PRS. Single-nucleotide polymorphism-based PRS is significantly associated with CFM [P = 7.22 × 10-58, odds ratio = 3.15, 95% confidence interval (CI) 2.74-3.63], and the top fifth percentile has a 6.8-fold CFM risk comparing with the 10th percentile. Father's smoking increases CFM risk as evidenced by interaction parameter of -0.324 (95% CI -0.578 to -0.070, P = 0.011) with PRS. In conclusion, the newly identified risk loci will significantly improve our understandings of genetics contribution to CFM. The risk prediction model is promising for CFM prediction, and father's smoking is a key environmental risk factor for CFM through interacting with genetic factors.