New strategies and methods for reconstruction of the outer ear canal (OEC).

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.

Primary anastomosis of segmental external auditory canal atresia in one dog.

This report describes a case of canine segmental external auditory canal atresia (EACA). The dog was managed medically with non-steroidal anti-inflammatory drugs until clinical deterioration, at which time a novel and successful end-to-end anastomosis surgical repair was performed. At the 30 day postoperative re-examination, the dog was clinically well and otoscopy confirmed that there was no evidence of auditory canal stenosis. The patient remained free of ongoing issues 20 months after the surgery. End-to-end anastomosis should be considered for treatment of developmental segmental EACA in the canine.

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.

Agujero Timpánico bilateral. Análisis de caso desde la literatura / Bilateral tympanic hole. Case analysis from the literature

La pared del conducto auditivo externo (CAE) parte de la formación del hueso timpánico; integrándose posteriormente a la porción petrosa del hueso temporal. El agujero timpánico o foramen de Huschke corresponde a un defecto en la osificación en donde existe fusión incompleta de porciones anteriores y posteriores del anillo timpánico dejando una abertura que comunica el CAE hacia anterior. Su presencia es normal hasta los 5 años de edad, tiempo en que se debiese obliterar. Su incidencia es baja (3-24%), pero la persistencia en adulto, conlleva sintomatología inespecífica caracterizada por otalgia, dolor en articulación temporomandibular (ATM), tinnitus, hipoacusia o manifestaciones complejas como descarga salival en CAE durante la masticación. Clínicamente puede complicar procedimientos de infiltración y artroscopias de ATM. Rara vez ocasiona, en pacientes mayores de 50 años, herniación de la cabeza del cóndilo mandibular. Su diagnóstico puede ser clínico por medio de otoscopia, donde se observa protuberancia de tejido en pared anterior del CAE, que aumenta de tamaño con la boca cerrada. También puede ser imagenológico con una tomografía computarizada. El tratamiento incluye desde medidas conservadoras para manejo del dolor e inflamación, hasta quirúrgicas con la implantación de injertos, placas o prótesis para cerrar la estructura o para reemplazar el cóndilo mandibular. El presente estudio pretende aportar incidencia dentro del área de estudio. Se analiza por observación directa, cráneo seco, completo, masculino, edad entre 12 a 15 años (según morfología del cóndilo mandibular y erupción dental). Se observa agujero de Huschke, bilateral, ambos permeables de diámetro 4 mm en ambos casos, determinados con regla milimetrada. La relevancia del defecto se asocia a la práctica clínica de otorrinolaringólogos, cirujanos maxilofaciales y odontólogos, ya sea como diagnóstico diferencial asociado a los síntomas inespecíficos, como para procedimientos más invasivos en la zona tales como infiltraciones o artroscopias de ATM

The wall of the external auditory canal (EAC) starts from the formation of the tympanic bone; later it is integrated to the petrous portion of the temporal bone. The tympanic foramen or foramen of Huschke corresponds to a defect in ossification where there is incomplete fusion of the anterior and posterior portions of the tympanic ring leaving an opening that communicates the EAC to its anterior aspect. Its presence is normal until 5 years of age, when it should be absolutely obliterated. Its incidence is low (3-24%), but its persistence in adults leads to non specific symptoms characterized by otalgia, pain in the temporomandibular joint (TMJ), tinnitus, hearing loss, or complex manifestations such as salivary discharge in the CAE during mastication. Clinically, it may complicate TMJ infiltration and arthroscopy procedures. It rarely causes herniation of the mandibular condyle head in patients older than 50 years. Its diagnosis can be clinical by means of otoscopy, where tissue protrusion is observed in the anterior wall of the CAE, which increases in size when the mouth is closed. It can also be imaging with computed tomography. Treatment includes from conservative measures to treat pain and inflammation, to surgical measures with the implantation of grafts, plates or prosthesis to close the structure or to replace the mandibular condyle. The present study aims to provide incidence within the study area. It is analyzed by direct observation, dry skull, complete, male, age between 12 to 15 years (according to mandibular condyle morphology and dental eruption). Huschke's foramen was observed, bilateral, both permeable, diameter 4mm in both cases, determined with a millimeter ruler. The relevance of the defect is associated with the clinical practice of otolaryngologists, maxillofacial surgeons and dentists, either as a differential diagnosis associated with nonspecific symptoms, or for more invasive procedures in the area such as infiltrations or TMJ arthroscopies.

Thiersch graft follow-up with narrow band imaging for acquired atresia of the external auditory canal: Canaloplasty with Thiersch graft versus vascularization evaluated with narrow band imaging.

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.

Imaging the External Ear: Practical Approach to Normal and Pathologic Conditions.

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.

Whole-Genome Sequencing Identifies Two Novel Rare Mutations in BMP5 and BMP2 in Monozygotic Twins With Microtia.

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.

Adults with unilateral congenital ear canal atresia - sound localization ability and recognition of speech in competing speech in unaided condition.

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.

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

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

3D Printing of a BAHA Protective Cap.

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.

Epithelial dynamics shed light on the mechanisms underlying ear canal defects.

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.

Measurement method for external auditory canal and clinical application in congenital aural stenosis.

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.

Unusual presentation of a first Branchial cleft cyst associated with an abnormal bony canal -a case report.

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.

Pleomorphic Adenoma of External Auditory Canal: Case Report of First Endoscopic Resection and Literature Review.

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.

Congenital soft tissue stenosis of the external auditory canal with canal cholesteatoma: Case report and literature review.

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.

[Surgical management of high-grade ear malformations in childhood]. / Chirurgische Therapie komplexer Fehlbildungen des kindlichen Ohrs.

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.

A Persistent Foramen of Huschke: A Small Road to Misery in Necrotizing External Otitis.

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.