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Purpose: This study aimed to compare the outcomes of patients with advanced congenital cholesteatoma who underwent microscopic or endoscope-combined Canal Wall Up Tympanomastoidectomy (CWUT) in our clinic and to determine the contribution of endoscope use in reducing recurrence/residual rates., Methods: In this retrospective study, the data of individuals who underwent microscopic or combined endoscopic surgery between 2008 and 2022 in our clinic were scanned from the database. Demographic data, preoperative computed tomography (CT) findings, preoperative and postoperative hearing results, operation and intraoperative status of the ossicles, duration of surgery, postoperative follow-up period, recurrence and residual disease status during follow-up were investigated., Results: The data of 37 pediatric cases operated in our clinic were included in the study. All of the included cases were Potsic Stage 4 patients who underwent CWUT. The mean age of the operated individuals was 8.7 years (5-12 years) and the mean follow-up period was 47.3 months (12-112 months). 19 cases were performed microscopically only, 2 recurrences and 5 residuals were detected. 18 cases were performed combined and 1 recurrence and 1 residual was found., Conclusion: In this study, it was determined that using an endoscope together with a microscope in congenital cholesteatoma cases, decreased the rate of recurrence and residual disease by protecting the external auditory canal in patients with advanced mastoid invasion., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Total joint reconstruction (TJR) has become the most preferred method of reconstruction in recent years for hemifacial microsomia (HFM). This requires meticulous planning for the glenoid fossa and ramus prosthesis along with the arch in certain indications. TJR in a type V HFM is extremely challenging due to agenesis/hypolasia of the zygoma and arch which compromises anchorage of the glenoid prosthesis. Conventional options used for such indications incorporate extended designs for the fossa. However, the two designs used commonly are associated with limitations; (i) the temporal extension is overtly large and cannot be anchored in patients with thin temporal bone and (ii) the glenoid fossa incorporated into the arch prosthesis, where the fossa is not positioned on stable bone and occlusal loading is non-physiological; transmitted onto the arch prosthesis rather than the skull base. The authors propose the use of the mastoid process as an alternative for anchorage of fossa prosthesis. This technique offers stable anchorage while facilitating ideal positioning on the skull base for optimal masticatory load transmission. The case illustration depicts reconstruction of the TMJ, zygoma and the zygomatic arch in a 31-year-old man with type V HFM, with a three-year follow-up, with good clinical outcomes sans complications., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Objective: To make surgeons aware of the differing types of preauricular sinuses (PAS), we summarize our experience with diagnosis and treatment of varying types of PAS., Methods: We retrospectively reviewed clinical data from patients who had undergone preauricular fistulectomy between March 2015 and March 2020. These patients were categorized into two groups according to locations of congenital fistula pit., Results: Twelve patients with variant PAS accounted for 6.8% (12/177) of all patients. The variant types of PAS could be classified into three types (from type 1 to type 3), based on the location of the fistula pit. Type 1 (seven patients; eight ears) patients had pits located on the ascending helix crus, whereas type 2 (four patients, four ears) and type 3 (one patient, one ear) patients had pits located on the external auditory canal (EAC) and lobule, respectively. Fistular tracts penetrated the cartilage of the helix crus in seven of the type 1 variant ears. Swelling and discharge were located at the ascending helix crus (in four ears), cavum concha (in two ears), and posterior to the auricle (in one ear). In four of the type 2 ears, the fistular tracts were located at the anterior margin of the ascending limb of the helix., Conclusion: Fistula tracts where fistula pit occurred on the ascending helix crus were more likely to penetrates through the cartilage, and fistula tracts with fistula pits that occurred on the EAC were adjacent to the cartilage of the ascending helix and tragus. Meticulous dissection and complete removal of fistula tissue are critical to avoid postoperative recurrence., Level of Evidence: 4 Laryngoscope, 134:3839-3845, 2024., (© 2024 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Background: Foramen tympanicum (FT) is a defect located anterior-inferior to the external acoustic meatus. We evaluated its prevalence, location, size, and relationship with temporomandibular joint (TMJ) disorders., Methods: Cone beam computed tomography was performed for 200 patients who presented to the Karamanoğlu Mehmetbey University Ahmet Keleşoğlu Faculty of Dentistry Hospital. The location and size of the FT in the axial and sagittal planes were evaluated. Descriptive statistics were used to compare the study parameters among age and sex groups. Patients with FT were reevaluated by two maxillofacial surgeons at the study centre., Results: In total, 200 images from 400 joints were examined. Unilateral and bilateral FT (19 [9.5%] and 8 [4%], respectively) was detected in 35 (17.5%) images from 27 (13.5%) patients. Examinations were performed for TMJ disorders in 24 patients. Participants with bilateral defects had the highest rates of presence of sounds and ear pain on the left and right sides (p < .05)., Conclusion: Foramen tympanicum can lead to TMJ disorders and spread of tumours or infections from the external auditory canal to the infratemporal fossa. The increased prevalence of such disorders in patients with bilateral FT suggests an association between them., (© 2024 The Authors. Journal of Oral Rehabilitation published by John Wiley & Sons Ltd.)

Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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.

Background: The incidence of re-stenosis or re-atresia after reconstruction of the Outer Ear Canal (OEC) in patients with Congenital Malformation of the Middle and Outer Ear (CMMOE) is very high (up to 48%), and it has been a difficult problem for otologists not being able to solve. Aims/Objectives: To explore new strategies and methods to improve re-stenosis or re-atresia after reconstruction of the OEC in patients with CMMOE. Material and Methods: According to the characteristics of reconstructed OEC (r-OEC) re-stenosis or re-atresia summarized by us, a number of new prevention strategies and methods have been proposed and related patent products have been designed, including the improvement of covering epithelium types and skin grafting methods (7 types), simulated drum ring function to prevent the formation of negative pressure in the cavity, and strengthen postoperative support to reduce skin shrinkage and bone hyperplasia. The postoperative effects of different ages and preoperative OEC malformations are statistically analyzed. Results: The incidence of re-stenosis/re-atresia is 14.3% (5/35) in the thin sectional skin of the temporal scalp overlap splicing skin grafting, which was significantly better than 45.5% (15/33) in the whole piece mosaic splicing and barrel skin grafting from the inner thin sectional thigh skin and overlay splicing other methods, including the inner thigh thin sectional skin, chest medium thick skin and subcutaneous pedicle + chest medium thick skin ( p <0.05). The patent artificial drum ring and the model stent of the OEC have obvious effects. The mean operation age of postoperative atresia, stenosis, and good groups are 9.3, 13.1, and 12.5 years old, respectively. The proportion of preoperative atresia is 91.3%, 85.7%, and 57.7%, respectively. The total incidence of re-atresia and re-stenosis of r-OEC for two groups of atresia and stenosis of OEC before surgery is 40.5% (49/121) and 13.3% (8/60), respectively. Conclusions and Significance: The best result is found in overlapping the splicing thin sectional skin of the temporal scalp, combined with artificial drum ring implantation, effective support of postoperative model stent of OEC and post-pubertal surgery selection are new and effective strategies and methods to prevent re-stenosis or re-atresia of r-OEC. Atresia or stenosis of the OEC before the operation is the influence factor of the postoperative effect.

Acquired atresia of the external ear canal is a narrowing of the external ear canal (EAC) that appears obstructed by fibrous tissue or bone tissue. Acquired atresia has two different phases: wet and dry stage. Computed tomography (CT) scan may show a scan where soft tissue fills EAC. Treatment may be medical and/or surgical. The surgical treatment of choice is represented by canaloplasty with a skin-free flap. To our knowledge, no article has reported data on the analysis of vascularization of acquired atresia of the external ear canal and vascularization of skin flap during follow-up with narrow-band imaging. This study evaluated patients suffering from acquired atresia of the external auditory duct, treated surgically in our Department of Organi di Senso of Sapienza University, from 2017 to 2020. All patients underwent: anamnestic collection, physical examination, CT. Preoperative and postoperative otoendoscopic evaluation (1,3,6 and12 months) was performed with both cold white light endoscopic vision (CWL) and narrowband imaging (NBI). 17 patients were enrolled in the study. Preoperative otoendoscopic examination of WL showed stenosis with a diameter <75% and a tympanic membrane not viewable in all patients. At 12 months of follow-up, 94% of patients had no recurrence of external ear canal stenosis. 88% of patients had normal NBI light vascularization. Our study evaluated how NBI can be a superior method, compared to CWL, to assess the state of the flap and can be relevant in the decision-making process of a re-intervention.

Abstract: Microtia is a rare congenital anomaly of the ear; it is regulated by both genetic and environmental factors. However, the mechanisms underlying its pathogenesis are unknown. In this study, the genomes of 2-year-old twin sisters with right microtia were sequenced using human genome-wide sequencing, an approach useful for identifying mutations in genes responsible for congenital microtia. The phenotypes of the twin sisters included congenital microtia on the right side, abnormal auricle shape in the right external ear, a peanut shape for the residual ear, and complete atresia of the right external auditory canal. In the twin sisters, we identified a previously unknown mutation in BMP5(exon4:c.833- 4C>G), as well as a new mutation (exon2:c.G332T:p.S111I) in BMP2, both of which were confirmed using polymerase chain reaction-based amplification of the corresponding genome regions, followed by first-generation sequencing. The exon4:c.833-4C>G mutation in human BMP5 may be the main cause of microtia in the twin sisters. A pathogenic mutation in human BMP2 (exon2:c.G332T:p.S111I) may be responsible for the facial deformity in the twin sisters. Thus, our study demonstrates the potential of genome-wide sequencing for identifying novel mutations associated with microtia on the whole-genome scale and extends the mutation spectrum of BMP5. Additionally, our data suggest that BMP2 is another pathogenic gene associated with microtia., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

The external ear (EE) is an osseous-cartilaginous structure that extends from the auricle to the tympanic membrane. It is divided into two parts: the auricle (or pinna) and the external auditory canal (EAC). Given the ease of access to the EE, imaging studies are not always needed to make a diagnosis. However, when lesions block visual access to areas deep to the EE abnormality, complications are suspected, or there is lack of response to treatment, imaging becomes essential. A basic understanding of the embryologic development and knowledge of the anatomy of the auricle and EAC are useful for accurate diagnosis of EE lesions. Congenital, traumatic, inflammatory, neoplastic, and vascular conditions can affect the EE. An overview of the anatomy and embryologic development of the EE is presented, with discussion and illustrations of common and uncommon conditions that affect EE structures and a focus on the CT and MRI features that are of interest to radiologists. CT is usually the first diagnostic modality used to evaluate the EAC and is the superior method for demonstrating bone changes. MRI provides excellent tissue characterization and enables one to better define lesion extension and perineural tumor spread. In addition, a flowchart to facilitate the differential diagnosis of EE abnormalities is provided. Online supplemental material is available for this article. © RSNA, 2022.

Background: Individuals with unilateral hearing loss show poor spatial hearing, but individual variability is high., Aims/objectives: To investigate if the degree of hearing loss in unilateral ear canal atresia affects horizontal sound localization and speech recognition., Materials and Methods: Twelve subjects with unilateral ear canal atresia without childhood hearing intervention. Previously published data from eight normal-hearing subjects in normal binaural as well as experimentally induced unilateral hearing loss served as a reference. Horizontal sound localization and recognition of speech in spatially separate competing speech were assessed., Results: Linear regression analysis demonstrated a relationship between sound localization accuracy (SLA) and the air conduction pure tone average of the atretic ear ( r  = 0.85, p =.007). The large proportion of variability in SLA (72%) explained by the degree of hearing loss of the atretic ear indicates that binaural processing is possible. SLA was worse than for normal hearing individuals ( p <.0001), and comparable to moderate simulated unilateral hearing loss ( p =.13). Speech discrimination was significantly worse than normal ( p <.0001) and not dependent on degree of hearing loss of the atretic ear., Conclusions and Significance: Individuals with congenital unilateral ear canal atresia show impaired horizontal SLA and recognition of speech in competing speech.

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

Mechanical feedback is one of the most common difficulties encountered when fitting hearing aids for toddlers and young children. We described the use of 3D printing to tailor a protective cap for a toddler with bilateral microtia/canal atresia to facilitate bone-anchoring hearing aid use.

Defects in ear canal development can cause severe hearing loss as sound waves fail to reach the middle ear. Here, we reveal new mechanisms that control human canal development and highlight for the first time the complex system of canal closure and reopening. These processes can be perturbed in mutant mice and in explant culture, mimicking the defects associated with canal atresia. The more superficial part of the canal forms from an open primary canal that closes and then reopens. In contrast, the deeper part of the canal forms from an extending solid meatal plate that opens later. Closure and fusion of the primary canal was linked to loss of periderm, with failure in periderm formation in Grhl3 mutant mice associated with premature closure of the canal. Conversely, inhibition of cell death in the periderm resulted in an arrest of closure. Once closed, re-opening of the canal occurred in a wave, triggered by terminal differentiation of the epithelium. Understanding these complex processes involved in canal development sheds light on the underlying causes of canal atresia., Competing Interests: Competing interestsThe authors declare no competing or financial interests., (© 2020. Published by The Company of Biologists Ltd.)

Objective: To describe a measurement method for external auditory canal (EAC), especially in congenital aural stenosis (CAS)., Methods: High-resolution CT (HRCT) datasets of CAS were imported into the MIMICS 15.0 software for image processing. We used two methods to evaluate the degree of CAS. One is sagittal reconstruction measurement method, the minor axis of the bony ear canal was measured in each layer using sagittal reconstruction. The other is Matlab procedure calculation, we calculated the midpoint axis values of the EAC in each Frankfurt plane. Finally, we compared the minimum value of each method, and verified the sagittal reconstruction measurement method retrospectively in the CAS cases without cholesteatoma who had undergone meatoplasty., Results: Twenty CAS cases were selected using the sagittal reconstruction measurement method and Matlab procedure calculation to evaluate the degree of CAS. The mean age was 9.55 ± 2.85 years old (range: 6-15). The mean degree of CAS was 2.09 ± 0.50 mm by sagittal reconstruction measurement method and 2.03 ± 0.58 mm by Matlab procedure calculation. There were no significant differences in the two groups (t = -1.371, p = 0.186). A total of 94 HRCT datasets were imported into MIMICS for verification. The mean age was 11.66 ± 6.56 years old (range: 6-39). Mean stenosis of EAC at surgery was 2.88 ± 0.93 mm (range: 1-4) by using sagittal reconstruction measurement method., Conclusions: Matlab procedure calculation is precise but relatively complicated and is only for scientific research. The sagittal reconstruction measurement method to calculate the most stenotic part of the bony ear canal is relatively simple and accurate to evaluate the degree of CAS. In cases of CAS without cholesteatoma, the precise measurement was critical for the next consultation., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Congenital external auditory canal stenosis (EACS) is a spectrum of abnormalities affecting the external and middle ear. We report a 6 year-old patient with EACS affecting the lateral fibrocartilaginous canal that was successfully repaired. This patient highlights a variant of EACS characterized by lateral soft tissue narrowing with normal osseous development. Most previous studies of CAA have described severe forms associated with complete atresia, bony stenosis, and middle ear malformations. Stenosis affecting only the fibrocartilaginous canal is a milder form resulting from premature arrest of the canalization process during embryologic development, and may predispose to cholesteatoma formation., Competing Interests: Declaration of competing interest None., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Malformations of the ear are the most common dysplasias in childhood. Ear malformations are classified into dysplasias of the auricle, of the ear canal, and of the middle and inner ear. The latter types are fortunately relatively rare. Most common is a low-grade dysplasia of the auricle (otapostasis, cup ear). Higher-grade dysplasia of the auricle (microtia II-III) is often associated with syndromes and/or malformations of the ear canal and the middle ear (e.g., congenital aural atresia with high-grade microtia and dysplasia of the middle ear). Functional aspects of hearing and acquisition of language as well as aesthetic aspects emphasize the importance of surgical therapy. Due to the importance of correctly scheduling surgical treatment in early childhood, this article gives an overview of the possibilities for aesthetic reconstruction of the auricle and the frequently associated functional rehabilitation of hearing.

Background: First branchial cleft anomalies are rare, accounting for only 10% of all branchial cleft anomalies. We report an even more rare and unique case of a branchial cleft cyst with features of both first and second arch derivatives., Case Presentation: A 6-year-old boy presented to us with a left conductive hearing loss associated with pre-tympanic keratin debris and an ipsilateral painful cervical mass. He had a past medical history of left ear surgery for presumed cholesteatoma 2 years prior and left neck abscess drainage 6 months prior. CT and MRI revealed a lesion originating in the external auditory canal and extending cervically through a bony canal located medial to the facial nerve and terminating as a parapharyngeal cyst. The complete removal was accomplished in one surgical stage consisting of three distinct steps: robotic assisted transoral resection of the pharyngeal cyst, an endaural approach and a parotidectomy approach., Conclusion: We believe that our detailed description of this rare first branchial cleft cyst with pharyngeal extension, possibly a hybrid case between a first and second branchial cyst, can serve as a valuable tool to Otolaryngologists - Head and Neck Surgeons who come across a similar unusual presentations.

Ceruminous pleomorphic adenoma is a very rare, mostly benign tumor originating from the ceruminal glands in the external auditory canal. Histologically, it is a mixed tumor with epithelial and stromal parts of different proportions, and is recognized today by the World Health Organization (WHO) as a ceruminous adenoma. Similar to the pleomorphic adenoma of salivary glands, recurrence or malignant degeneration with cellular atypia and metastasis can occur on rare occasions. Here, we describe an 87-year old female patient with a growing spherical mass in the right external auditory canal. After exclusive endoscopic tumor resection, a ceruminous pleomorphic adenoma was histologically diagnosed. Due to the absence of nuclear pleomorphism, no increased mitotic rate, no perineural invasion and no fusion transcripts of the MYB or MYBL1 gene loci, an adenoid cystic carcinoma could be excluded. The postoperative course was without any evidence of complications. A literature review identified 44 articles with 49 patients that were considered. Hearing loss and ear sensations were the most commonly reported symptoms. Most cases underwent an excision via an endaural or retroauricular approach. Recurrences were described in four patients, three of which had a malignant transformation.

Emanuel syndrome is a rare inherited chromosomal abnormality caused by an unbalanced translocation of chromosomes 11 and 22. Clinically, Emanuel syndrome is characterized by a wide spectrum of congenital anomalies, dysmorphisms, and developmental disability often confused with other similar syndromes. Outside of genetic testing, diagnosis remains challenging and current literature on typical radiologic findings is limited. We present classic neuroimaging findings of Emanuel syndrome consistent with prior literature including microcephaly, microretrognathia, external auditory canal stenosis, and cleft palate; and also introduce the additional maxillofacial anomaly of dysplastic middle ear ossicles, to our knowledge not previously described in the literature. Recognition of findings leading to earlier diagnosis of Emanuel syndrome may improve outcomes and quality of life for patients and their families., (Copyright Journal of Radiology Case Reports.)

Background and Purpose: Necrotizing external otitis is a serious complication of external otitis with different spreading patterns. A persistent foramen of Huschke is a dehiscence located antero-inferior in the osseous external ear canal and posterior-medial to the temporomandibular joint. This dehiscence can facilitate extension of infection in an anterior pattern next to classic spread along the fissures of Santorini. The aim of this study was to define the prevalence and size of a persistent foramen of Huschke in patients with necrotizing external otitis., Materials and Methods: We retrospectively examined 78 CT temporal bone studies (39 patients with necrotizing external otitis, 39 control subjects). The side and presence of the foramen were noted, and its prevalence was calculated. The maximal width of the foramen of Huschke was measured in the axial plane and classified as subtle, mild, moderate, or extensive., Results: A persistent foramen of Huschke was present in 21 patients (26 ears) and 7 control subjects (9 ears). Prevalence was 50% (20/40) and 11.5% (9/78) in affected ears of patients with necrotizing external otitis and control subjects, respectively. Almost all affected ears showed an anterior distribution pattern of necrotizing external otitis. The extensive dehiscence was most common in affected ears., Conclusions: An anterior necrotizing external otitis spreading pattern is associated with the presence and increased size of a persistent foramen of Huschke. These findings facilitate the theory that a persistent foramen of Huschke is an additional risk factor the development of necrotizing external otitis., (© 2019 by American Journal of Neuroradiology.)

Purpose: The foramen tympanicum (FT) represents a developmental anomaly that forms due to incomplete fusion of processes of the tympanic ring. Its presence in the population is controversial and it has been associated with a number of otologic complications. The aim of this study was to systematically analyze the prevalence, anatomical characteristics, and ethnic variations of the FT and compare these parameters in cadaveric and radiologic studies., Methods: An extensive search was conducted through the major electronic databases, and identified articles were separated into 2 groups based on their methodology: cadaveric and radiologic studies. Data extracted included study modality, prevalence data, ethnicity, gender, side, laterality, and diameter., Results: A total of 8 studies (n = 2671 patients) were included into our meta-analysis. The main findings revealed that the prevalence of the FT in the population is 14.9%, it is more often unilateral (62.5%) than bilateral (37.5%), it is most often reported in Asia (21.4%), and it is more prevalent in cadavers (21.2%) than in radiologic studies (8.8%) (not statistically significant)., Conclusion: As the FT is present in more than one-tenth of the population, it is important to consider the possibilities of its presence when undertaking surgical procedures in the temporomandibular joint and ear region and plan accordingly to avoid injuries. Clinicians should keep it as one of the possible diagnoses while confronted with patients presenting with otologic complications. The use of newer imaging techniques was recommended, such as cone-beam computer tomography to detect the FT prior to surgery.

The interface between cells and inorganic surfaces represents one of the key elements for bioelectronics experiments and applications ranging from cell cultures and bioelectronics devices to medical implants. In the present paper, we describe a way to tailor the biocompatibility of substrates in terms of cell growth and to significantly improve cell-chip communication, and we also demonstrate the reusability of the substrates for cell experiments. All these improvements are achieved by coating the substrates or chips with a self-assembled monolayer (SAM) consisting of a mixture of organic molecules, (3-aminopropyl)-triethoxysilane and (3-glycidyloxypropyl)-trimethoxysilane. By varying the ratio of these molecules, we are able to tune the cell density and live/dead ratios of rat cortical neurons cultured directly on the mixed SAM as well as neurons cultured on protein-coated SAMs. Furthermore, the use of the SAM leads to a significant improvement in cell-chip communications. Action potential signals of up to 9.4 ± 0.6 mV (signal-to-noise ratio up to 47) are obtained for HL-1 cells on microelectrode arrays. Finally, we demonstrate that the SAMs facilitate a reusability of the samples for all cell experiments with little re-processing.

Objective: To describe, in terms of functional gain and word recognition, the audiological results of patients under 18 years of age implanted with the active bone conduction implant, Bonebridge™., Design: Retrospective case studies conducted by reviewing the medical records of patients receiving implants between 2014 and 2016 in the public health sector in Chile., Study Sample: All patients implanted with the Bonebridge were included (N = 15). Individuals who had bilateral conductive hearing loss, secondary to external ear malformations, were considered as candidates., Results: The average hearing threshold one month after switch on was 25.2 dB (95%CI 23.5-26.9). Hearing thresholds between 0.5 and 4 kHz were better when compared with bone conduction hearing aids. Best performance was observed at 4 kHz, where improvements to hearing were observed throughout the adaptation process. There was evidence of a significant increase in the recognition of monosyllables., Conclusions: The Bonebridge implant showed improvements to hearing thresholds and word recognition in paediatric patients with congenital conductive hearing loss.

We conducted a retrospective study to evaluate the relationship between external auditory canal (EAC) anomaly, temporal bone abnormality, and hearing levels using objective scoring systems in Chinese patients with microtia. The study population consisted of 106 ears of 94 Chinese patients (67 male and 27 female) aged 5 to 45 years (mean: 12.6) with microtia. The EAC abnormalities were classified into 4 types according to Schuknecht's criteria: type A, type B, type C, and type D. Developmental anomalies of the temporal bone were evaluated by Jahrsdoerfer computed tomography (CT) scoring system using high-resolution CT scans of the temporal bone. Temporal bone malformation parameters were divided into 4 subgroups: ossicular chain development, windows connected to the cochlea, aeration development of the middle ear, and facial nerve aberration. Hearing levels (air conduction and bone conduction) were examined. Outcomes parameters included correlation coefficients (r) and a number of other variables. The total points (10 points) and subtotal points related to ossicles (4 points), windows (2 points), aeration (2 points), and facial nerve (1 point) correlated inversely with the EAC abnormalities. The hearing levels (air conduction, r = 0.396, p <0.01; bone conduction, r = 0.21, p = 0.03) correlated significantly with the EAC abnormalities of Schuknecht's classification. We conclude that the better developed the external auditory canal, the better developed the temporal bone and the better developed the external auditory canal, the better hearing level. The hearing level also can serve as an indicator to determine whether a patient will be suitable for reconstructive surgery.

Objective: To date, the impact of conductive hearing loss on the auditory pathway at brainstem level has only been investigated in animal studies, which showed a species-specific delay of maturation. In this study, the functional maturation of auditory brainstem response (ABR) parameters in humans with unilateral atresia of the external auditory canal was investigated., Methods: 42 newborns and toddlers ranging in age from 13 days to 11 months were included. The click-evoked ABR interpeak latencies (IPL) of the atretic ears and the contralateral ears with normal hearing were evaluated. The children had no comorbidities and had never been fitted with any kind of hearing aid. The absolute latencies (AL) and IPL of a matched control group were compared to the contralateral normally hearing ears of the children with unilateral atresia., Results: The mean air-bone gap in the ears with atresia was 44 dB HL. Despite this partial acoustic deprivation, no significant difference between the IPLs of normal ears and ears with atresia could be detected. Both for AL and IPL, the differences between the normal ears and the control group were all within 1 standard deviation to the mean., Conclusion: The data showed that the monaural acoustic deprivation by a block of sound conduction does not produce any delay of functional maturation at brainstem level in this group of patients. With regard to the AL and IPL on brainstem level, no differences between the normal ears of children with unilateral atresia and children with bilateral normal hearing could be detected., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Conclusion: Each of eight patients with bilateral microtia and atresia underwent bilateral reconstruction of the auricles and external auditory canals and were fitted bilateral canal-type hearing aids in the operated ears to replace a bone conduction hearing aid. The ability to discriminate inter-aural intensity difference (IID) and even inter-aural time difference (ITD) was retained in all these patients., Objective: This study studied the post-operative sound lateralization ability of patients with bilateral microtia and atresia after total reconstruction of both auricles and external auditory canals, followed by fitting of bilateral canal-type hearing aids., Methods: Eight patients with bilateral microtia and atresia ranging in age from 13-43 years were recruited in this study. Each of them underwent bilateral reconstruction of the auricles and external auditory canals and were fitted canal-type hearing aids in both the operated ears to replace a bone conduction hearing aid. A sound lateralization test was conducted to determine IID and ITD discrimination ability thresholds., Results: In all the patients, the IID discrimination ability thresholds of the patients were more than 3-fold those of the controls, the ITD discrimination ability threshold was more than 5-fold those of controls, and binaural hearing was retained.

A six-month-old baby with congenital patent ductus arteriosus (PDA), bilateral microtia and canal atresia was referred for hearing assessment. The audiology assessment revealed bilateral profound hearing loss, which is atypical for a case of pure canal atresia. Imaging was performed much earlier than usual and, as suspected, the patient also had bilateral severe inner ear anomaly. It is extremely rare for a person to have both external and inner ear anomaly because of the different embryological origin. The only suitable hearing rehabilitation option for this kind of patients is brainstem implant. However, the parents had opted for sign language as a form of communication.

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., (Copyright © 2016 Elsevier Inc. All rights reserved.)

We report a case of a nine-year-old male who presented with facial nerve stimulation four years after cochlear implantation. Computed tomography was performed revealing a dilated internal auditory meatus and the cochlear implant electrode was found to be protruding into the fallopian canal at the level of the geniculate ganglion. Subsequent genetic analysis demonstrated X-linked deafness type 2 (DFNX2) caused by a novel c.769C > T nucleotide change in the POU domain, class 3, transcription factor 4 gene (POU3F4). Inactivation of electrodes 1 and 19-21 successfully abated facial nerve stimulation., (Copyright © 2016. Published by Elsevier Ireland Ltd.)

Objectives: To determine whether a canal wall down mastoidectomy can provide long-term benefit for children with aural stenosis., Methods: Retrospective case series of children with congenital aural stenosis having undergone a canal wall down mastoidectomy over a twelve-year period at a tertiary children's hospital., Results: Data from thirteen children who underwent a total of twenty canal wall down mastoidectomies for aural stenosis were reviewed. The mean age at surgery was 7.1 years (range, 3.3-12.3 years). All patients had genetic syndromes including Trisomy 21 (n = 7), Trisomy 21 and Pierre Robin sequence (n = 1), Angelmann (n = 1), Cri-du-chat (n = 1), Branchio-oto-renal syndrome (n = 1), Spina bifida (n = 1) and Nager syndrome (n = 1). Seven (54%) children underwent bilateral canal wall down mastoidectomies. All thirteen ears that could not be visualized preoperatively had improved ease of office examination following surgery. Only one patient required revision surgery and all canals were patent at the last clinic visit. The mean follow-up was 4.9 years. There were no cases of facial nerve injury or cerebrospinal fluid leak., Conclusion: Syndromic children with congenital aural stenosis with poorly pneumatized mastoids may benefit from canal wall down mastoidectomy to improve ease of office examinations., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Polycomb Group (PcG) proteins assemble a chromatin state that maintains developmental gene repression. A new study combining structure and in vivo analysis details a molecular network from DNA recognition to PcG recruitment, highlighting the essential role of Sterile Alpha Motifs., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

The aim of the present study was to comprehensively evaluate the clinical features and long-term outcomes of congenital aural stenosis (CAS). This study presents a retrospective review of patients who underwent meatoplasty for CAS at a tertiary referral hospital from 2008 to 2015. A total of 246 meatoplasty procedures were performed on 232 patients in the present study. We performed multivariate regression analysis. Except in the age < 6 years group, no significant difference was observed among different age groups for cholesteatoma formation, p > 0.05. Except for the stenosis of the external auditory canal (EAC) (>4 mm) group, the other stenosis of EAC groups were not associated with cholesteatoma formation, p > 0.05. Postoperative air-bone gaps (ABG) less than 30 dB occurred in 77.3% (99/128) of the patients, and the Jahrsdoerfer score was associated with postoperative ABG, p < 0.001. The complication rate of CAS was 13.8% (20/144), and males showed a higher risk for postoperative complications (OR, 6.563; 95% CI, 1.268-33.966, p = 0.025). These results indicate that meatoplasty was an effective surgical intervention for CAS, showing a stable hearing outcome with prolonged follow-up. There was no significant difference between the cholesteatoma and no cholesteatoma groups for hearing outcomes, p > 0.05.

Objective: To investigate the histopathology of inner ear malformations (IEMs) in order to explain their pathophysiology., Method: Light microscopy was used to study 33 specimens exhibiting various IEMs in the collection of the Otopathology Laboratory at Harvard University's Massachusetts Eye and Ear Infirmary., Results: The investigation found 18 incidences of cochlear hypoplasia (CH) (3 CH-I, 10 CH-II, 5 CH-III), 11 incomplete partitions (IPs) (5 IP-I, 6 IP-II), 2 vestibular dilatations, and 2 cases of cochlear nerve aplasia. The IP-I cases had characteristic defective endosteums, while the IP-II cases showed hydropic changes in the scala vestibuli. The CH cases were small in size externally, with normal or defective internal architecture., Conclusion: In combination with embryological data, these findings suggest that cases of CH-III and CH-IV are most probably genetically predetermined to be small in size, and that development of the membranous labyrinth stops at a point earlier than normal, so that it is shorter. At the time of complete ossification, this results in a cochlea with small external dimensions and normal internal architecture. In CH-I and CH-II cases, there is arrested development of the internal architecture, in addition to a small cochlea; it is most likely that in these cases, there is a severely defective vascular supply from the internal auditory canal (IAC). IP-I may be the result of a defective vascular supply from the blood vessels of the IAC. In IP-II, an enlarged endolymphatic sac (EES) appears to be the genetic abnormality that causes the other abnormalities, as it allows high pressure to be transmitted into the cochlea and vestibule. In IP-III, the pathophysiology appears to be an abnormal vascular supply from the middle ear mucosa, caused by a genetic abnormality and resulting in a thinner otic capsule and the absence of the modiolus.

Conductive hearing loss (CHL), far more common than sensorineural hearing loss in children, can be acquired or congenital, can range from mild to moderately severe, and can be caused by a simple cerumen impaction, middle ear fluid, or complex middle ear abnormalities with or without the absence of the ear canal (congenital aural atresia). This article presents evidence-based recommendations for the evaluation and management of the child with both acquired and congenital CHL., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Few studies have investigated ear involvement in nonsyndromic autosomal recessive congenital ichthyosis (ARCI)., Objectives: To assess the type and frequency of otologic manifestations of ARCI in patients under follow-up at the pediatric dermatology department of our hospital., Materials and Methods: We prospectively studied the presence of ear pain, ear itching, tinnitus, otitis, cerumen impaction, accumulation of epithelial debris, and hearing loss. Daily hygiene measures, topical treatments, medical-surgical interventions, and frequency of visits to an ear, nose, and throat (ENT) specialist were noted in the patients' medical records. Ear examination and hearing tests were performed in all cases., Results: Ten patients were studied: 2 had a self-healing collodion baby phenotype and 8 had ichthyosis. There was mention of otologic manifestations in the records of all 8 patients with ichthyosis (100%); 6 of these patients (75%) had abnormalities in the external auditory canal examination and 2 (25%) had conductive hearing loss. Our findings are limited by the small number of patients studied, all of whom were younger than 19 years., Conclusions: The involvement of both dermatologists and ENT specialists in the management of patients with ichthyosis is crucial to ensure the application of the best therapeutic and preventive measures. More studies are needed to assess the prevalence and impact on quality of life of ear involvement in patients with ichthyosis and to determine the optimal interval between ENT visits for these patients., (Copyright © 2015 Elsevier España, S.L.U. and AEDV. All rights reserved.)

Syngnathia is a rare congenital disorder of jaw fusion with a paucity of literature from developed countries. We present a case of an infant noted to have multiple anomalies at birth including syngnathia, microcephaly with a variant of brain abnormality between holoprosencephaly and syntelencephaly, optic nerve hypoplasia, ear canal anomalies, hemi-vertebrae, and suspected hypomelanosis of Ito. To our knowledge, this patient with syngnathia and multiple anomalies is the first to be reported, but whether they are a coincidence, a pathogenetic association, or a new syndrome remains unknown. This case is discussed with a brief review of the literature.

Purpose: There currently is no guideline regarding amplification or verification for children with aural atresia. This population, with the absence of the ear canal, has obvious congenital hearing loss. Research suggests that delayed treatment for hearing loss can result in speech and language impairments, including poor performance in school. In this study we examined the relationship between amplification and emerging speech and language development in children with aural atresia., Method: Subjects included children, 3 to 6 years of age, with conductive hearing loss due to atresia. Clinical evaluations were used to determine relationships between amplification and speech and language development. Subjects underwent an audiology exam, including pure-tone or warble-tone air and bone conduction using play audiometry techniques, and a speech and language evaluation assessing language and vocabulary skills., Results: Subjects who were fitted before 1 year of age showed better compliance with aid use and exhibited fewer delays in speech and language development. Children with right-sided atresia had significantly greater speech and language delays when compared with age norms from standardized tests., Conclusion: Our findings highlight a relationship between side of atresia, time of first amplification, compliance in aid use, and speech and language abilities.

The differential diagnostics between the common positional posterior plagiocephaly and relatively rare lambdoid synostosis is important due to the differences in their treatment plan and clinical management. However, the clinical criteria for the diagnosis of lambdoid synostosis are not clear since there is a considerable overlap in the features of positional posterior plagiocephaly and unilateral lambdoid synostosis. To systematically evaluate the clinical findings in these 2 patient groups, we quantitatively compared the characteristics of endocranial skull base and ectocranial calvarium in 3D computed tomography, in 9 children (mean age 2.9 years) with unilateral lambdoid synostosis and 9 children with positional posterior plagiocephaly. The groups were sex and age matched. Our results show that the skull bases in the lambdoid synostosis are posteriorly shorter and more twisted than in positional posterior plagiocephaly. Anterior twisting was mild in both skull types. Our study confirmed earlier suggested diagnostic feature: prominent ipsilateral mastoidal bossing downward and laterally in all lambdoid skulls. In positional posterior plagiocephaly the bossing was typically not detected. Interestingly, there was a great variation in the position of the ipsilateral ear and external auditory meatus in both patient groups. Thus, neither antero-posterior nor vertical position of ear is a reliable differential diagnostic feature between lambdoid synostosis or positional posterior plagiocephaly.

Ear abnormalities in oculo-auricular-vertebral spectrum commonly present with varying degrees of external and middle ear atresias, usually in the expected locations of the temporal bone and associated soft tissues, without ectopia of the external auditory canal. We present the unique imaging of a 4-year-old girl with right hemifacial microsomia and ectopic location of an atretic external auditory canal, terminating in a hypoplastic temporomandibular joint containing bony structures with the appearance of auditory ossicles. This finding suggests an early embryological dysfunction involving Meckel's cartilage of the first branchial arch.

Purpose: First aim of the study was to define normal shape and diameter of the internal auditory canal (IAC). In the second part the clinical relevance of IAC-enlargement was analyzed, considering also lesions of the subtle structures at the fundus of the internal auditory canal., Materials and Methods: 440 high resolution CT-scans of the temporal bone were used for retrospective analysis of the internal auditory canal and its fundus region., Results: The mean value of the IAC diameter in axial and coronal plane was determined. In 20 of 440 patients IAC enlargement was found. In the group with pronounced enlargement (3fold SD) nearly all patients suffered from hearing impairment. In some of them we found structural abnormalities near the IAC fundus in the CSF/perilymph border zone., Conclusion: A new CT-based definition of normal shape and diameter of the internal auditory canal is presented. There is some evidence that a pathologic transmission of CSF-pressure in case of IAC-enlargement and/or abnormal fistulous communications could play an important role in the pathophysilogy of hearing loss., Key Points: • New CT-based definition of normal internal auditory canal.• Nearly all patients showing pronounced IAC-enlargement suffer from hearing impairment.• Possible pathomechanism: Transmission of CSF-pressure on the inner ear., (© Georg Thieme Verlag KG Stuttgart · New York.)

The aim of this study was to observe the effect of a titanium tube on external auditory canal reconstruction in congenital aural atresia and to assess the tube's effectiveness in preventing external canal stenosis or atresia after reconstruction. Reconstruction of the external ear canal with a titanium mesh tube was performed in 16 patients (16 ears) with congenital aural atresia at the First People's Hospital of Foshan. The titanium mesh tube was removed 1 year after surgery. The patients were followed up for 2 years (2 ± 0.3 years), and all of the patients had formed a new external ear canal. There was no local infection, granulation tissue, re-stenosis, or atresia in any of the patients after surgery. All of the patients were content with their newly formed external ear canal. Titanium mesh tubing is safe and effective for reconstruction of the external ear canal during surgery for congenital aural atresia.

We report the case of a no. 4 Tessier cleft in association with an unknown cleft of the mandible extending to the external auditory meatus. This has not been previously published in the literature and its underlying pathology remains undetermined. The nature of the cleft, possible classifications, and potential embryologic origins will be discussed. Amniotic band syndrome is the most likely cause of the cleft., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)