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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.

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Ear molding therapy is a nonsurgical technique to correct certain congenital auricular deformities. While the advantages of nonsurgical treatments over otoplasty are well-described, few studies have assessed aesthetic outcomes. In this study, we compared assessments of outcomes of ear molding therapy for 283 ears by experienced healthcare providers and a previously developed deep learning CNN model. 2D photographs of ears were obtained as a standard of care in our onsite photography studio. Physician assistants (PAs) rated the photographs using a 5-point Likert scale ranging from 1(poor) to 5(excellent) and the CNN assessment was categorical, classifying each photo as either "normal" or "deformed". On average, the PAs classified 75.6% of photographs as good to excellent outcomes (scores 4 and 5). Similarly, the CNN classified 75.3% of the photographs as normal. The inter-rater agreement between the PAs ranged between 72 and 81%, while there was a 69.6% agreement between the machine model and the inter-rater majority agreement between at least two PAs (i.e., when at least two PAs gave a simultaneous score < 4 or ≥ 4). This study shows that noninvasive ear molding therapy has excellent outcomes in general. In addition, it indicates that with further training and validation, machine learning techniques, like CNN, have the capability to accurately mimic provider assessment while removing the subjectivity of human evaluation making it a robust tool for ear deformity identification and outcome evaluation.

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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.

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OBJECTIVE: The deficits in the cochlea which is at the one end of the ear sound transfer system, may effect middle ear functions. Wideband typanometry (WBT) is frequently used to evaluate these transfer functions which play a crucial role in setting the impedance matching between the external ear and the cochlea. To this end, the aim of this study was to investigate the ear transfer functions in inner ear malformations via WBT, and to question whether these functions change depending on the types of inner ear malformation. METHODS: This prospective case-control study was conducted in a university hospital. One hundered-fifty-seven ears (aged 3-37 years) under the groups of cochlear hypoplasia, incomplete partition I, incomplete partition II, cochlear aplasia and complete labyrinthine aplasia were evaluated. In the control group, 30 ears with normal hearing were enrolled and WBT was carried out. Tympanometric peak pressure, equivalent middle ear volume, static admittance, tympanogram width, resonance frequency, average wideband tympanometry and absorbance measurements were analyzed. RESULTS: The inner ear malformation groups demonstrated statistically significant differences than the control group and from each other in terms of traditional tympanometric parameters and WBT test parameters (p<0.05). The most remarkable difference was between the group of complete labyrinthine aplasia and the control group, most probably because of complete labyrinthine aplasia's structural effects. However, on some parameters, incomplete partition II and the control group showed similarities. In absorbance measurements, there was significant difference between all patient groups and the control group, especially at high frequencies (p<0.05). The largest difference was between the control group and the group of complete labyrinthine aplasia which has revealed the lowest absorbance values (p<0.05). In averaged-wideband tympanogram analysis, all patient groups obtained a lower amplitude peak than the control group; complete labyrinthine aplasia group had the flattest peaked amplitude, while the incomplete partition II group had a near-normal curve. CONCLUSION: The results of the study revealed the distinctive effects of inner ear malformations in middle ear transfer functions. It is concluded that the absence of inner ear structures causes negative effects on energy absorbance and the other transfer functions of the middle ear. WBT may provide additional information on diagnosis of patients with inner ear malformations.

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Quantifying ear deformity using linear measurements and mathematical modeling is difficult due to the ear's complex shape. Machine learning techniques, such as convolutional neural networks (CNNs), are well-suited for this role. CNNs are deep learning methods capable of finding complex patterns from medical images, automatically building solution models capable of machine diagnosis. In this study, we applied CNN to automatically identify ear deformity from 2D photographs. Institutional review board (IRB) approval was obtained for this retrospective study to train and test the CNNs. Photographs of patients with and without ear deformity were obtained as standard of care in our photography studio. Profile photographs were obtained for one or both ears. A total of 671 profile pictures were used in this study including: 457 photographs of patients with ear deformity and 214 photographs of patients with normal ears. Photographs were cropped to the ear boundary and randomly divided into training (60%), validation (20%), and testing (20%) datasets. We modified the softmax classifier in the last layer in GoogLeNet, a deep CNN, to generate an ear deformity detection model in Matlab. All images were deemed of high quality and usable for training and testing. It took about 2 hours to train the system and the training accuracy reached almost 100%. The test accuracy was about 94.1%. We demonstrate that deep learning has a great potential in identifying ear deformity. These machine learning techniques hold the promise in being used in the future to evaluate treatment outcomes.

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OBJECTIVES: To make otolaryngologists aware of the variant types of auricular sinus, we have performed a systematic review of patient diagnoses and presented our operative experiences. METHODS: From 2009 to 2013 in Sun Yat-Sen Memorial Hospital, there was a total of 20 children with the variant type of auricular sinuses including the comprehensive group. Postauricular sinuses have pits located posterior to the imaginary vertical line that is tangent to the external auditory canal. Sinuses that penetrate the cartilage and cause postauricular swelling or skin defects characterize type 1 of the variant type, while sinuses that adhere to the cartilage and cause preauricular or auricular swelling or skin defects characterize type 2. Patients with pits both anterior to and posterior to the imaginary vertical line comprise the comprehensive group. The patients who had infected underwent auricular sinusectomy using a dual approach, with accurate fistula tracing and proper cartilage removal. RESULTS: Sixteen children who had infected sinus underwent surgery, while the other four were asymptomatic. Ten children (62.5%) of 16 patients were diagnosed as type 1 of the variant type, 2 (12.5%) as type 2. Four children (25%) were diagnosis as the comprehensive group. The asymptomatic could not be defined as the sinuses location were unknown. Sixteen children (100%) of 16 patients who underwent surgery had a history of misdiagnosis and treatment. These patients did not experience recurrence over a 5-year follow-up period. CONCLUSION: The locations of pits and sinuses help to categorize the different types of auricular sinus. The effective method that we have described should be considered a viable way to reduce recurrence.

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Rehabilitation of the patients suffering from with congenital malformations of external and middle ear is a specific area of medicine requiring special knowledge and skills of the audiologists and ear surgeons. This article highlights the issues of epidemiology, classification and methods for the rehabilitation of patients presenting with this pathology that reflect our own experience and the methodology based on it for all phases of both functional and aesthetic surgical inerventions. Special attention is given to the assessment of the effectiveness of the treatment, as part of the evaluation of the quality of medical services. The results were demonstrated in 56 patients aged from 5 to 17 years. 32 of them underwent reconstructive, hearing improving surgery (meatotympanoplasty), 24 patients were managed by means of the implantation of the bone-anchored hearing systems. The assessment of the effectiveness of the treatment was conducted according to the algorithm developed by the authors, including free field pure tone audiometry and validated questionnaires for the assessment of the quality of life (the Glasgow Children's Benefit Inventory).

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Despite reported success of early nonsurgical splinting of congenital ear deformities, the technique has not been widely utilized due to difficulty with application and positional maintenance of these devices. Delay in treatment can result in unnecessary surgery for the child later in life. In response to this need, we developed a simplified system consisting of Velcro, conformers, and polysiloxane gel that can be applied in 3 steps. Before and after photos after application of the device were graded by a plastic surgeon independent of the study. A total of 33 ear deformities were treated. In all, 92.3% of ears achieved significant improvement and normalization of their ears after therapy. This device allows for a more straightforward and individualized application than current products available. Our hope is that practitioners involved early in the infant's care will utilize this technology resulting in improved outcomes and avoiding unnecessary surgery.

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OBJECTIVES: 1) To describe the technique for transcanal endoscopic management of congenital ossicular chain fixation. 2) To highlight the utility and outcomes of the endoscopic approach for management of congenital ossicular fixation. STUDY DESIGN: Retrospective patient series. SETTING: Academic tertiary pediatric hospital. PATIENTS: Pediatric patients (age 6-12) undergoing transcanal endoscopic management of congenital ossicular fixation from May 2014 to December 2014. INTERVENTIONS: A transcanal endoscopic approach was used in eight procedures. Ossicular chain pathology was managed with either mobilization, ossiculoplasty with a stapes prosthesis, or incus interposition. MAIN OUTCOME MEASURES: Pure-tone averages, speech reception thresholds, and speech discrimination scores were recorded pre- and postoperatively for each subject. Preoperative computed tomography evaluations were compared to intraoperative findings for each subject. RESULTS: An improvement in the pure-tone average, as well as air-bone gap, was noted after six of eight procedures. No patients experienced a complication or a reduction in their bone conduction hearing. The chorda tympani nerve was preserved in all eight patients. Conversion to open approach was not necessary for any of the eight procedures performed. CONCLUSION: The transcanal endoscopic approach was successful in improving hearing in pediatric patients with congenital ossicular fixation that involves any of the three ossicles. An endoscopic transcanal approach provides an alternative method to manage congenital ossicular pathology with the advantage of providing excellent visualization and the avoidance of a postauricular incision.

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OBJECTIVES/HYPOTHESIS: Preauricular sinuses are benign congenital malformations of preauricular soft tissues. Complete excision using either sinectomy or supra-auricular approach is advised to prevent recurrence. Reported recurrence varies between 0 and 42%. We evaluated which surgical technique resulted in lowest complication and recurrence rates. STUDY DESIGN: PubMed, Embase, Scopus, Web of Science. METHODS: Two authors appraised studies on directness of evidence and risk of bias. Original data were extracted and pooled when I(2) was smaller than 50%. Results are reported according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. RESULTS: Fourteen high directness of evidence studies were included. Reported complication rates were similar: sinectomy [0-31.4%] and supra-auricular approach (SAA) [0-18.2%]. Pooled recurrence rates showed that sinectomy resulted in significantly (P = .04) more recurrence 5.5% (95% confidence interval [CI] 3.6-8.3%) than SAA 2.2% (95% CI 0.7-7.0). Sinectomy using the microscope resulted in the lowest sinectomy recurrence rates (1.9%). SAA in combination with a Penrose drain resulted in 0% recurrence in revision cases. Drain use resulted in the lowest SAA recurrence rates; however, drain application was not advised due to higher complication rates (frequent wound infection [P = .003] and more [P = .002] and longer [P = .001] compression dressing use). CONCLUSION: SAA could be the preferable technique for preauricular sinus removal. If despite evidence, sinectomy is elected over SAA, microscope use can further decrease recurrence rates comparable to SAA levels. Level of included evidence (Ib-IV) indicates the need for a prospective study comparing surgical outcomes between techniques. Laryngoscope, 126:1535-1544, 2016.

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OBJECTIVE: To investigate the relationship between the severity of ear anomaly and mandibular dysplasia in congenital microtia. STUDY DESIGN: Retrospective case review. SETTING: Sapporo Medical University Hospital. PATIENTS: Congenital microtia: 44 patients over a period of 4 years. INTERVENTIONS: The height of the condylar process of the mandible was assessed by three-dimensional computed tomography (CT), and the patients were divided into three groups based on the ratio of the condylar process height on the affected side to that on the unaffected side: Group A, ≥1.00; Group B, 0.99 to 0.85; Group C, <0.85. Developmental abnormalities of the ear were evaluated using Jahrsdoerfer's scoring system on high-resolution CT scans. MAIN OUTCOME MEASURES: Nonparametric statistical tests were used to determine correlations between the height of the condylar process of the mandible and Jahrsdoerfer's score. RESULTS: The total Jahrsdoerfer's score for each group was 7.36 ±â€Š2.23, 7.28 ±â€Š0.10, and 4.52 ±â€Š0.30, respectively; this value was significantly lower in Group C than in the other groups. In terms of subtotal points, oval window open, middle ear aeration, and mastoid pneumatization correlated significantly with mandibular dysplasia. Patients in Group C tended to have grade III microtia, by Marx's classification. Facial nerve weakness was not significantly correlated with mandibular dysplasia. CONCLUSIONS: In congenital microtia, mandibular development correlated significantly with aeration of the middle ear space, pneumatization of the mastoid, and formation of the oval window, but not with the presence of a bony part of the external auditory canal or with ossicular development.

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The incidence of auricular deformities is believed to be ∼11.5 per 10,000 births, excluding children with microtia. Although not life-threatening, auricular deformities can cause undue distress for patients and their families. Although surgical procedures have traditionally been used to reconstruct congenital auricular deformities, ear molding has been gaining acceptance as an efficacious, noninvasive alternative for the treatment of newborns with ear deformations. We present the successful correction of bilateral Stahl's ear deformity in a newborn through a straightforward, nonsurgical method implemented on the first day of life. The aim of this report is to make pediatric practitioners aware of an effective and simple molding technique appropriate for correction of congenital auricular anomalies. In addition, it stresses the importance of very early initiation of ear cartilage molding for achieving the desired outcome.

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To investigate whether differences existing in the distance between facial nerve (FN) and round window niche opening among congenital aural atresia (CAA), congenital aural stenosis (CAS) and a normal control group and to assess its effect on the round window implantation of vibrant soundbridge, CT images of 10 normal subjects (20 ears), 27 CAS patients (30 ears) and 25 CAA patients (30 ears) were analyzed. The distances from the central point of round window niche opening to the terminal point of the horizontal segment, the salient point of pyramidal segment, the beginning point of the vertical segment, and the vertical segment of the facial nerve (abbreviate as OA, OB, OC, OE, respectively) were calculated based on three-dimensional reconstruction using mimics software. The results suggested that the pyramidal segment of the FN was positioned more closely to round window niche opening in patients with both CAA and CAS groups than that in control group, whereas there was no significant difference between CAA and CAS group (P < 0.05). The vertical portion of the FN was positioned more closely to round window niche opening in the CAA group than those in both the CAS and control groups with statistical significance (P < 0.05). Furthermore, the vertical portion of the FN was positioned more closely to round window niche opening in the CAS group than that in control group (P < 0.05). In conclusion, the dislocation between facial nerve and round window niche in patients with congenital auditory canal malformations could have significant effects on the round window implantation of vibrant soundbridge. Moreover, three-dimensional measurements and assessments before surgery might be helpful for a safer surgical approach and implantation of vibrant soundbridge.

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Coils are typically utilized as one of the most popular embolization agents. Coil migration to the pulmonary artery rarely occurs and is associated with a high tendency of severe pulmonary or cardiovascular complications. We herein present a 25-year-old man with arteriovenous malformations in the right ear. Two coils 4-mm in diameter and 3-mm in length migrated to the pulmonary artery during embolization. No further damage related to the coil migration was found during a 2-year follow-up period. In addition, the possible mechanisms of coil migration are discussed.

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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.

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Congenital earlobe clefts (CELC) may be annoying for the plastic surgeons. Many classifications and techniques were described for CELC. The previously described techniques are useful in some conditions, but not proper in all. We present a simple, effective method for a rare CELC 'triple lobe' in this article.

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PURPOSE: Prominent ear is one of the most common congenital deformities in the head and neck region. More than 100 techniques have been described for correction of prominent ears in literature. Mustarde technique is one of the most widely accepted procedures of otoplasty. In our study, we used a surgical method using a drill for correction of prominent ears in combination with the Mustarde technique. Patients with only lack of antihelical fold were included the study. METHODS: Thirty patients were operated on during 2007-2010. The angle of prominent ear from the mastoid tip to the tip of the posterior surface of the auricle was measured preoperatively and postoperatively (3 months after operation) using jib and protractor. Patient satisfaction was evaluated using a questionnaire. RESULTS: The angulation of prominent ear was 45.2 ± 8.6 degrees preoperatively. Postoperatively, the angulation was 25.6 ± 6.3 degrees. The difference was statistically significant (P < 0.001). Fifteen patients (50%) reported that the operation was very satisfactory, 13 patients (43.4%) reported that the operation was satisfactory, and 2 patients (6.6%) reported that the operation was not satisfactory cosmetically. CONCLUSION: The described technique of otoplasty using a drill to thin the auricular cartilage is a safe and easy-to-learn procedure. In Mustarde technique, permanent sutures are used to reshape the cartilage structure in fixed position. Over time, structural strength elasticity of cartilage can weaken the force of sewing forces. Combining the technique with permanent sutures, results were satisfactory. This technique can be used to recreate the antihelical fold. We will follow the cases long term to further show more results.

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OBJECTIVES: To estimate the prevalence of external auditory canal (EAC) stenosis in patients with congenital aural atresia (CAA) and the prevalence of acquired ear canal cholesteatoma in patients with EAC stenosis and to identify risk factors that may predict the presence of ear canal cholesteatoma in those patients. STUDY DESIGN: Retrospective chart review. SETTING: University tertiary referral center. MATERIALS AND METHODS: Patients with EAC stenosis with and without acquired ear canal cholesteatoma were identified from the medical records of 673 patients (770 ears) with CAA. Demographic data, symptoms at presentation, and audiometric data were compared between those with and those without cholesteatoma to identify the risk factors for the presence of ear canal cholesteatoma. RESULTS: Of the 770 ears evaluated, 101 (13.1%) were found to have at least 1 stenotic ear canal. Of this group of 101 ears with canal stenosis, 18 of 94 ears (7 ears were missing data; 19.1%) had a concurrent cholesteatoma, with 1 patient having bilateral cholesteatomas. Demographic, clinical, and audiometric parameters showed that only female sex was associated with a higher rate of ear canal cholesteatoma. Within the cholesteatoma group, right ears in female patients and left ears in male patients predominated. CONCLUSION: Approximately 1 in 5 patients with congenital aural stenosis were found to have ear canal cholesteatoma. Female sex is a risk factor; basic audiometric parameters provide no diagnostic utility in distinguishing ears with cholesteatoma from those without cholesteatoma.

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