Clinical evaluation of temporal bone anatomical abnormalities and surgical method selection in patients with congenital aural atresia.

OBJECTIVES: To analyze anatomic variations of the temporal bone in congenital aural atresia (CAA) and their correlation with the Jahrsdoerfer score, in order to guide clinical selection of surgical treatment methods. MATERIAL AND METHODS: We retrospectively studied 53 patients (72 ears) with unilateral or bilateral CAA, including 34 ears with normal hearing as controls. Audiological and imaging data were collected and analyzed. We evaluated the Jahrsdoerfer score and anatomical variations, including tegmen mastoideum position, anterior sigmoid sinus displacement, and elevated jugular bulb. RESULTS: The average air conduction hearing threshold (PTA4) ranged from 0.5 to 4 kHz was 65.48 ± 8.19 dBHL, with an average Jahrsdoerfer score of 4.93 ± 2.78. In CAA group, there was a higher prevalence and severity of anterior sigmoid sinus and low position of the tegmen mastoideum (P < 0.01). However, there was no significant difference in incidence rates among groups with high jugular bulb (P > 0.05). Anterior sigmoid sinus and high jugular bulb showed no correlation with the Jahrsdoerfer score, while the low position of the tegmen mastoideum had a weak correlation. The Jahrsdoerfer score did not adequately predict temporal bone anatomical abnormalities in CAA patients. CONCLUSION: CAA exhibit a higher incidence and greater severity of temporal bone anatomical abnormalities compared to the control group, and the Jahrsdoerfer score inadequately assesses these abnormalities. Anomalies like low position of the tegmen mastoideum, anterior sigmoid sinus, and high jugular bulb should also be considered as independent factors influencing surgical decisions for atresiaplasty.

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.

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.

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.

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.

Optimal Choice for Improving the Hearing in Children with Unilateral Microtia and Atresia: Softband or Adhesive Adapter?

INTRODUCTION: A nonsurgical bone conduction hearing aid (BCHA) is a well-established treatment for children with congenital unilateral microtia and atresia (UMA). To date, limited studies have evaluated the audiological characteristics of the different wearing modes in the same nonsurgical BCHA. METHODS: Eighteen patients with UMA aged 5-24 years were included. Warble tones at frequencies of 0.5, 1, 2, and 4 kHz were presented to determine functional hearing gain (FHG) of hearing thresholds (in dB HL) in the sound field. The speech perception abilities were assessed by the speech discrimination score (SDS, in %) of monosyllables, disyllables, and sentences in quiet and noise using the Chinese Mandarin speech test materials. Hearing outcomes were evaluated with the ADHEAR™ worn on a softband and with an adhesive adapter. A correlational analysis was conducted to analyze the correlations between variables (e.g., age, height, weight, body mass index [BMI], bone conduction pure-tone threshold, and air conduction pure-tone threshold) and the differences in the two wearing modes. RESULTS: The mean FHG (standard deviation, SD) at 0.5-4 kHz was 20.63 (3.94) dB HL with the adhesive adapter and 26.39 (3.15) dB HL with the softband. When aided with the BCHA, significant improvements in SDS were revealed in all Mandarin speech test material lists either in quiet or noise for both wearing modes. Compared with the adapter mode, the softband provided higher aided SDS values. Correctional analyses revealed that higher BMI values were positively associated with larger delta outcomes between the two coupling methods of the softband and adhesive adapter in patients with UMA. Furthermore, a larger delta average FHG of 0.5-4 kHz was consistently associated with larger delta monosyllabic SDS in quiet, disyllabic SDS in quiet, and disyllabic SDS in noise. DISCUSSION: To the best of our knowledge, this is the first study to compare the hearing benefits of coupling methods using novel adhesive adapters and conventional softbands with the same audio processor (ADHEAR™). Under uniform internal settings, softband integration provided more hearing benefits than adhesive adapter integration, and the differences were more obvious in patients with higher BMI values. Besides, a brief measurement of FHG can be utilized to predict individualized speech perception levels.

Bilateral adhesive bone conduction devices in patients with congenital bilateral conductive hearing loss.

PURPOSE: This study aims to characterize the hearing benefits and sound localization accuracy of bilateral adhesive bone conduction devices (aBCDs) compared to unilateral devices in patients with congenital bilateral conductive hearing loss (BCHL). METHODS: Sixteen children and adolescents with congenital BCHL were enrolled and tested under four listening conditions: (1) unaided, (2) R aided: aided with a right-side aBCD, (3) L aided: aided with a left-side aBCD, and (4) B aided: aided with aBCDs on both sides. The sound field hearing threshold (SFHT, in dB hearing level [HL]) and the word recognition score (WRS) were measured. The mean absolute error (MAE) of sound source localization was calculated to assess the sound localization accuracy. RESULTS: The performance in SFHT and WRS was significantly higher in the B aided condition than that in the unaided, R and L aided conditions; moreover, no significant difference was observed between the R and L aided conditions. Concerning sound source localization, the accuracy of localization exhibited a sharp decline when using a single aBCD, while the application of bilateral aBCDs (B aided condition) resulted in a significantly improved localization accuracy as compared to the unilaterally aided conditions (both R and L); however, no significant difference was found between the unaided and B aided condition. CONCLUSION: Patients with congenital BCHL experienced suboptimal hearing benefits and manifested significant challenges in sound source localization when utilizing a single aBCD, as compared to the utilization of bilateral aBCDs.

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.

Sound-localisation performance in patients with congenital unilateral microtia and atresia fitted with an active middle ear implant.

OBJECTIVE: This study assessed the safety and sound-localisation ability of the Vibrant Soundbridge (VSB) (Med-EL, Innsbruck, Austria) in patients with unilateral microtia and atresia (MA). METHODS: This was a single-centre retrospective research study. Twelve subjects with unilateral conductive hearing loss (UCHL) caused by ipsilateral MA were recruited, each of whom underwent VSB implantation and auricular reconstruction. The bone-conduction (BC) threshold was measured postoperatively, and the accuracy of sound localisation was evaluated at least 6 months after surgery. Horizontal sound-localisation performance was investigated with the VSB activated and inactivated, at varying sound stimuli levels (65, 70 and 75 dB SPL). Localisation benefit was analysed via the mean absolute error (MAE). RESULTS: There was no statistical difference in mean BC threshold of impaired ears measured preoperatively and postoperatively. When compared with VSB-inactivated condition, the MAE increased significantly in unilateral MA patients in the VSB-activated condition. Besides, sound-localisation performance worsened remarkably when sound was presented at 70 dB SPL and 75 dB SPL. Regarding the side of signal location, the average MAE with the VSB device was much higher than that without the VSB when sound was from the normal-hearing ear. However, no significant difference was observed when sound was located from the impaired ear. CONCLUSION: This study demonstrates that in patients with unilateral MA, the VSB device does not affect inner-ear function. Sound-localisation ability is not improved, but deteriorated at follow-up. Our results suggest that the VSB-aided localisation abilities may be related to the thresholds between the ears, plasticity of auditory system and duration of use of VSB.

Audiological effectiveness of Bonebridge implantation for bilateral congenital malformation of the external and middle ear.

PURPOSE: To evaluate the audiological effectiveness of Bonebridge implantation in patients with bilateral congenital malformation of the external and middle ear. METHODS: Twenty-eight cases [17 boys and 11 girls; median age, 12 years (range 8-36 years)] had unilateral Bonebridge implantation. Audiological tests were performed preoperatively and postoperatively, and included the pure-tone audiometry test, speech discrimination score (SDS), and evaluation of the unaided and aided hearing thresholds in sound fields. For the group of patients aged > 12 years, Mandarin Speech Test Materials were used to determine the SDS. For the other cases, the Mandarin Lexical Neighborhood Test was used. The daily life efficacy was assessed using the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire postoperatively. The t and t' tests were used in the statistical analyses. RESULTS: The hearing threshold with the Bonebridge improved by 25-35 dB HL, compared to that of the unaided condition. The SDS of patients aged > 12 years improved by about 50%; the SDS also improved by 10-20% in the three patients aged < 12 years. According to the APHAB, the mean Bonebridge scores of ease of communication, background noise, and reverberation decreased by 60.3 ± 15.7, 50.2 ± 11.1, and 59.4 ± 7.8, respectively, compared to the preoperative scores, and the Bonebridge score of aversiveness was higher (69.8 ± 10.8) than the unaided score. CONCLUSIONS: The audiological effectiveness of the Bonebridge was significant and noticeable in bilateral congenital malformation of the external and middle ear. The Bonebridge can be an alternative and effective solution for those patients to improve hearing.

Audiological features in congenital bony atresia of external auditory canal with temporal-mandibular joint retroposition.

OBJECTIVES: To facilitate the diagnosis, treatment and surgical options for congenital bony atresia of external auditory canal (EAC) with temporal-mandibular joint (TMJ) retroposition by analyzing its audiological features and the morphology of temporal bone on CT scan. MATERIALS AND METHODS: Two cohorts of patients with congenital EAC bony atresia with (n=23) or without (n=21) TMJ retroposition were recruited from September 2012 to July 2014 at Beijing Tongren Hospital, Capital Medical University. The patients with TMJ retroposition were set as the group A and those without as group B. Based on the degree of TMJ retroposition, group A was further divided into two sub-groups A1 (n=13) and A2 (n=10). The temporal bone CT scan, pure tone average (PTA) and air-bone gap (ABG) were obtained for the main outcome measurements. SPSS 17.0 was used for the statistics analysis with t and t test. RESULTS: For group A, the average air conduction (AC) was 55.22±12.53dBHL, the average bone conduction (BC) was 7.07±3.34dBHL, and the average ABG was 50.69±8.60dBHL. For the sub-groups A1 and A2, the average AC was respectively 45.77±8.43dBHL and 59.50±7.43dBHL, BC 7.07±3.34dBHL and 6.89±4.37dBHL, and ABG 47.31±7.92dBHL and 53.00±7.91dBHL. For group B, the average AC was 70.24±5.63dBHL, BC 6.78±4.37dBHL, and ABG 60.19±6.09dBHL. CONCLUSIONS: The degree of TMJ retroposition is negatively related to the severity of hearing loss among patients with congenital EAC bony atresia, and those with TMJ have suffered less severe hearing loss than those without. Although TMJ retroposition might be a disadvantage for patients undergoing EAC plasty and tympanoplasty, it must be considered for its influence on hearing loss severity and auditory canal abnormality when planning the surgical treatment. Different from normal surgical protocol for congenital EAC bony atresia, we commend other hearing reconstruction methods such as BAHA and VSB, even without intervention.

[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.

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.

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.

[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.

Bonebridge implants versus atresiaplasty in children with unilateral congenital aural atresia: A comparison study of audiological outcomes.

OBJECTIVES: To evaluate and compare audiological outcomes of atresiaplasty and Bonebridge (BB) implantation in patients with unilateral congenital aural atresia (UCAA), to guide clinical decision-making. METHODS: Twenty-seven subjects diagnosed with UCAA were included in the study. Thirteen were implanted with the BB, while 14 undergone atresiaplasty. All patients underwent pre-and post-surgery examinations, including pure-tone audiometry, sound field threshold (SFT), speech reception threshold (SRT), word recognition score (WRS), and horizontal sound source localization tests. RESULTS: (1) Postoperatively, the average SFT decreased by 11.79 ± 5.93 dB HL in the atresiaplasty group and by 24.46 ± 9.36 dB HL in the BB group, with a significantly greater decrease in the BB group compared to the atresiaplasty group (P < 0.05). (2) Both groups demonstrated a significant improvement in average disyllabic WRS postoperatively under normal ear-masking conditions, with the BB group showing a significantly higher improvement than the atresiaplasty group. (3) When the speech signal was presented from the CAA side with noise from the normal hearing side, both surgical groups exhibited a significant decrease in postoperative signal-to-noise ratio compared to preoperative levels, with improvements of 2.14 ± 2.95 dB SNR in the atresiaplasty group and 4.92 ± 5.83 dB SNR in the BB group (P < 0.05). (4) The average minimum audible angle preoperative in the atresiaplasty group was 29.71 ± 18.42°, which decreased to 18.1 ± 10.07° at 6 months postoperatively, showing a statistically significant improvement (P < 0.05). CONCLUSION: We concluded that both atresiaplasty and Bonebridge implantation can significantly improve speech perception under both quiet and noisy conditions in children with UCAA. BoneBridge implantation appears to provide better audiological outcomes than atresiaplasty. Atresiaplasty can significantly improve the accuracy of sound localization. No significant improvement in sound localization accuracy was observed in the short period after Bonebridge implantation. Further research should be conducted with a larger sample size and longer follow-up time.

[Expression and significance of IFN-γ and IL-4 in the effusion and peripheral blood of chronic otitis media with effusion patients].

OBJECTIVE: To explore the shift of Th2/Th1 type cytokine profiles in middle ear and peripheral blood of chronic otitis media with effusion (COME) patients. METHODS: Middle ear effusions (MEEs) and peripheral bloods were collected from 30 COME patients between April 2011 and July 2012 at Department of Otolaryngology, Beijing Tongren Hospital, Capital Medical University. Peripheral blood samples were collected from 20 healthy controls. The expressions of IFN-γ (Th1 cytokine) and IL-4 (Th2 cytokine) in peripheral blood and middle ear fluid were assessed by flow cytometry and cytometric bead array. The expression levels of cytokines in peripheral blood were compared between patient and control groups. Moreover, the levels of cytokines in the Middle ear fluid and the supernatant of COME patients were also compared. RESULTS: Compared to the control group, the level of IL-4 in peripheral blood of COME patients significantly increased (0.7% ± 0.8% vs 2.4% ± 4.5%, P < 0.05) . The ratio of IFN-γ positive cells/IL-4 positive cells was significantly lower in peripheral blood of COME patients than that of control group (17 ± 13 vs 35 ± 19, P < 0.05) . However, the level of IFN-γ showed no significant difference between two groups (12% ± 8% vs 13% ± 8%, P > 0.05) . The levels of IL-4 and IFN-γ in middle ear fluid and the supernatant of the case group showed no significant difference. CONCLUSIONS: COME may be a Th2 predominant disease. Th1/Th2 imbalance is probably involved in the pathogenesis of COME. The role of the allergic reaction in middle ear microenvironment remains uncertain.

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.