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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., (© 2024 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

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., (© 2021. The Author(s).)

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.)

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., (Copyright © 2019 Elsevier B.V. All rights reserved.)

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.

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.

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).

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.

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.

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 ± 2.23, 7.28 ± 0.10, and 4.52 ± 0.30, 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.

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., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

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.

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., (Copyright © 2016 by the American Academy of Pediatrics.)

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.)

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.

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.

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.

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.

Introduction: The exact aetiology of congenital cholesteatoma, the less common form of this destructive disease, is still under debate., Case Report: A two-year-old boy was referred to paediatric otolaryngology with persistent, bloody, left-sided otorrhoea refractory to oral and ototopical antibiotics. Prior to its onset at age 16 months, all ear examinations on the affected side were normal. Physical examination, imaging with computed tomography and eventual tympanomastoidectomy revealed extensive cholesteatoma. The extent of the disease, age at onset of symptoms and absence of otological disease before initial presentation suggested the diagnosis of congenital cholesteatoma. Review of the family history revealed that the patient's older brother had undergone tympanomastoidectomy for a small, well-encapsulated, mesotympanic congenital cholesteatoma at two years of age., Discussion: This case joins a single, previous report describing congenital cholesteatoma in multiple family members, suggesting that in some cases, hereditary factors may play a role in the formation of the disease.

Objective: Describe anatomical and radiological findings in 742 patients evaluated for congenital aural atresia and microtia by a multidisciplinary team. Develop a new classification method to enhance multidisciplinary communication regarding patients with congenital aural atresia and microtia., Methods: Retrospective chart review with descriptive analysis of findings arising from the evaluation of patients with congenital atresia and microtia between January 2008 and January 2012 at a multidisciplinary tertiary referral center., Results: We developed a classification method based on the acronym HEAR MAPS (Hearing, Ear [microtia], Atresia grade, Remnant earlobe, Mandible development, Asymmetry of soft tissue, Paralysis of the facial nerve and Syndromes). We used this method to evaluate 742 consecutive congenital atresia and microtia patients between 2008 and January of 2012. Grade 3 microtia was the most common external ear malformation (76%). Pre-operative Jahrsdoerfer scale was 9 (19%), 8 (39%), 7 (19%), and 6 or less (22%). Twenty three percent of patients had varying degrees of hypoplasia of the mandible. Less than 10% of patients had an identified associated syndrome., Conclusion: Patients with congenital aural atresia and microtia often require the intervention of audiology, otology, plastic surgery, craniofacial surgery and speech and language professionals to achieve optimal functional and esthetic reconstruction. Good communication between these disciplines is essential for coordination of care. We describe our use of a new classification method that efficiently describes the physical and radiologic findings in microtia/atresia patients to improve communication amongst care providers., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Conclusion: Active middle ear implant (AMEI) implantation in children and adolescents is safe and provides improved hearing results. No statistical difference in hearing outcomes was shown in the group of patients affected by chronic middle ear diseases versus aural atresia. Also, the transducer location (round window versus oval window placement) did not lead to different outcomes in hearing abilities., Objectives: (1) To assess the hearing outcomes with the active implant Vibrant Soundbridge (VSB) in children and adolescents. (2) To evaluate whether functional results of the subjects in the study could depend on the hearing loss etiology (chronic middle ear diseases versus aural atresia) or on transducer location (round window versus oval window placement)., Methods: The study was carried out with a retrospective, single-subject, repeated measures design, and included 22 children and adolescents with conductive or mixed hearing loss due to aural atresia or chronic middle ear diseases. Preoperative and postoperative pure tone air conduction (AC) and bone conduction (BC) thresholds were measured to demonstrate implantation safety. Free-field warble tone and speech audiometry were performed to assess postoperative hearing abilities with and without the VSB., Results: No significant changes in mean BC or AC thresholds between preoperative and postoperative conditions were seen in the 22 patients. Mean PTA4 functional gain was 30.7 dB. Averaged over all 22 patients, word recognition at 65 dB SPL changed from an average of 19% in the unaided postoperative condition to 97% in the VSB-aided condition. Functional results were independent of hearing loss etiology and transducer location.

Objectives: Hairy polyps are rare, congenital malformations of the oropharynx and nasopharynx. To date, approximately 145 cases have been reported. However, the histogenesis of these lesions remains unclear., Case Report: We report the case of a 2-day-old neonate presenting with a hairy polyp attached to the left palate, who re-presented aged 16 months with a discharging first branchial arch sinus., Conclusion: We propose this case as supporting evidence for the theory that hairy polyps are a malformation of the first branchial arch system.

Objective: Current strategies for functional rehabilitation of microtia-atresia patients with bone-anchored implants or surgical atresia repair have been extended by the feasibility of active middle ear implants. The aim of the present research is to evaluate a new flowchart of the treatment of these patients that considers active middle ear implants., Patients: Congenital aural atresia and microtia., Intervention(s): Bilateral cases are provided with a conductive hearing aid after birth and implanted with an active middle ear implant within the second year. Unilateral cases are provided with a conductive hearing aid and implanted with a middle ear or bone-conduction device in early childhood. Unilateral cases without amplification in the vulnerable time after birth are carefully selected for late implantation. At age 8 to 10, the auricular reconstruction is completed., Main Outcome Measure(s): Feasibility of implantation irrespective of age, functional gain in audiometry., Results: The results of early implantation are as good as those previously published for adolescents. Mean reaction threshold with the Vibrant Soundbridge was 21 dB. Mean functional gain was 48 dB. The local tissues are unaltered and ready for auricular reconstruction., Conclusion: Active middle ear implants allow early and selective stimulation of the auditory pathway in children with congenital conductive hearing loss and are expected to lead to the normal development of the binaural hearing functions. To date, it is the only option if the stimulation is to be started at the age of 12 to 18 months. This was implemented into a new flowchart for aural atresia-microtia.

Repair of complete congenital aural atresia (CAA) could be a challenging procedure due to complications reported with CAA surgery such as facial nerve palsy, canal stenosis, graft lateralization, sensorineural hearing loss or the difficulty involved in the surgical technique. From 2006 to 2009, we used a one stage-modified transmastoid approach for surgical repair of 33 ears with complete CAA via a non-randomized controlled clinical trial. Some modifications in the technique of mastoidectomy, ossiculoplasty, fascia and skin grafting and meatoplasty have been described. Patients were followed up for 12 months to assess audiometric results and post-operative complications. Changes in air-bone gap and need for revision surgery or hearing aids were assessed at follow-up. There were no cases of facial weakness, dead ear or bony canal stenosis. Hearing success in 2 months follow-up was achieved in 72.7% of all patients. Success rate increased to 92.3% in patients with Jahrsdoefer's scores of 8 and above. Overall success rate decreased to 63.6% at 12 months follow-up. There were no significant difference in Jahrsdoerfer score of patients with successful first surgical attempt and those who needed revision surgery (P value >0.056). Also patients of lower age (less than 5-years-old) did not have more need for revision surgery when compared with older patients (P value >0.36). However, being a syndromic patient did increase the need for revision surgery (P value <0.04). Age was not a predictor of meatal/canal stenosis and patients with lower Jahrsdoerfer scores could also achieve good results.

The pattern of congenital oral and craniofacial anomalies (CFAs) in the Kenyan population remains unknown. The objective of this study was to describe the pattern of occurrence of CFAs at two hospitals in Nairobi. A descriptive cross-sectional study at the Kenyatta National Hospital and Pumwani Maternity Hospital was carried out from November 2006 to March 2007. Mothers who delivered at the hospitals consented to an interview and physical examination of their babies within 48 h of delivery. The anomalies were classified for type and magnitude. Data were analysed to determine the association of these anomalies with ages of the mothers, gender, weight, birth order, mode of delivery and birth status of the babies. During the study period, 7989 babies were born. The CFAs manifested in 1.8% of the total births and were more common in female (1.4%) than in male (1.0%) live births. 12.8% of stillbirths had CFAs, with lesions manifesting more in males (16.7%) than in females (6.9%). The commonest CFA was preauricular sinus (4.3/1000) followed by hydrocephalus (1.9/1000) then preauricular tags and cleft lip and palate (1.5/1000 and 1.3/1000 total births, respectively)., (Copyright © 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Background: The possible association between otitis media in pregnancy (OMP) and structural birth defects, that is, congenital abnormalities (CAs), in their offspring has not been studied., Methods: The data set of the Hungarian Case-Control Surveillance of Congenital Abnormalities, 1980 and 1996, was evaluated., Results: There were 58 (0.25%) and 55 (0.14%) of 22 843 cases and 38 151 controls with mothers who had OMP, respectively. There was association of OMP and a higher risk of ear CA., Conclusions: A possible explanation for the association of OMP with higher risk of ear CA may be some morphological deviation of the inner ear., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Introduction: Preauricular fistulas are not uncommon congenital disorders in children. They are associated with imperfect auricle formation as a consequence of incomplete fusion of the auditory hillocks of the first and second branchial arches., Aim: The aim of this study was to present diagnostic methods with evaluation of treatment results of preauricular fistulas in children., Materials and Methods: We analysed retrospectively clinical and epidemiological features, diagnostic methods and treatment results of the patients with preauricular fistula attended in the Otolaryngology Department of Medical University in Gdańsk., Results: Between 1995 and 2009 in the Department of Otolaryngology MGUed 23 children (13 girls and 10 boys) were treated for preauricular fistulas. The age of the children ranged between 2 to 16 years (median 7.7). The fistula was recognized on the left side in 14 children, in 6 on the right and bilaterally in 3 children. The diagnosis was based on the presence of a cutaneous fistula in the region of the helix's anterior crus with inflamed surrounding and purulent discharged in physical examination and in radiological imaging (MRI). Patients were treated surgically in the classical way and using Prasada's method including resection of the cutaneous fistula and cyst with a part of the helix. Squamous epithelium in the resected fistulas was found in 91.3% of the children. No recurrence was observed, good cosmetic outcome was achieved in all children., Conclusions: Each patient with bilateral preauricular fistula needs to be diagnosed for congenital disorders of the middle ear and kidneys. MRI may be useful in establishing the location of the fistula. Surgical treatment is not necessary in preauricular, blind-ended sinuses. In the postoperative material of the sinus squamous epithelium is found.

A 2-year-old female child is reported with congenital cholesteatoma of the right external auditory canal. We describe the clinical features, computed tomography finding and surgical treatment. Congenital cholesteatomas can occur within the temporal bone. Congenital cholesteatoma of the external auditory canal is rare. Generally, it appears in the canal floor. Treatment consists of the resolution of granulation and removal of debris., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objectives: To define the prevalence of inner ear anomalies in aural atresia patients and to recognize patterns of developmental anomalies in aural atresia patients., Study Design: Retrospective review., Setting: Academic medical center., Intervention: Physical exam, audiometry, and temporal bone CT in selected patients., Patients: Pediatric patients with aural atresia., Main Outcome Measure: Prevalence of inner ear anomalies and coexisting facial paralysis or sensorineural hearing loss., Results: In this series of 118 patients with aural atresia, associated facial palsy was seen in 13%, whereas inner ear anomalies were present in 22%, including all patients with facial palsy. Interestingly, the inner ear anomalies often did not display a significant sensorineural hearing loss. Bilateral inner ear anomalies were frequently encountered despite unilateral atresia. Most anomalies involved the semicircular canals including several uncommon variants of posterior semicircular canal anatomy., Conclusion: Inner ear anomalies are common in the presence of aural atresia, especially when there is concurrent congenital facial palsy. The presence of inner ear anomalies should be recognized as a common feature of craniofacial microsomia.

Objective: To compare the results of radiofrequency thermal ablation versus 'cold steel' excision, for supra-auricular excision of preauricular sinus., Study Design: Prospective study. Observations included recurrence, intra-operative bleeding, operation time and ease of dissection., Setting: Tertiary referral hospital., Subjects and Methods: Sixty cases of preauricular sinus were assigned randomly to two groups (30 cases each). Group one patients underwent cold steel sinus excision, whereas group two patients underwent radiofrequency-assisted sinus excision., Results: Fifty patients were operated upon, with a mean age of 12.26 years. Most patients were aged between zero and five years (40 per cent). The average patient follow-up time was 15 months (range, six to 24 months). Recurrence rates were 23 per cent in group one and 3 per cent in group two; this difference was statistically significant (p = 0.016). Dissection of the sinus tract was reported to be much easier in the radiofrequency group, compared with the cold steel group (p = 0.023), with minimal bleeding., Conclusion: From our observations, radiofrequency-assisted, local, wide excision appears to be preferable for treatment of preauricular sinus, compared with cold steel excision, as it allows better peri-operative visualisation, easier dissection, minimal bleeding and a lower subsequent recurrence rate (3.3 per cent).

Complete deficiency of a member of the type II transmembrane serine protease family, tmprss1 (also known as hepsin), is associated with severe to profound hearing loss in mice and a gross enlargement of the tectorial membrane in the cochlea. Levels of thyroxine in these mice have been shown to be significantly lower when compared with wild-type controls. As thyroxine is critical for inner ear development, we delivered thyroxine to these mice during the prenatal or postnatal stage of development. Both the treatments could not ameliorate hearing loss or correct deformities in the tectorial membrane of these mutant mice, suggesting that a deficiency in tmprss1 affects thyroxine responsiveness in the inner ear in vivo.

Background: Polyotia is an extremely rare type of congenital external ear malformation, which is defined as an accessory ear that is large enough to resemble an additional pinna. The terms 'mirror ear' or 'accessory ear' are sometime used. We present our methods in correcting this malformation and summarise the aetiology., Methods: The posterior part of the polyotia may presents with a normal ear, a constricted ear or a microtic ear. Free auricular composite tissue transplantation was used to correct the constricted ear. Ear reconstruction was applied in cases of microtia. The anterior auricle was mainly used to form the tragus., Results: 7 cases polyotia were treated between 2004 and 2008. After free auricular composite tissue transplantation the size of the constricted ear and the contralateral ear was similar. In microtia cases the reconstructed ears were natural looking and had a satisfactory three-dimensional contour. The extra tissue of the anterior ear was excised and the tragus was reconstructed., Conclusions: Through operative intervention tailored to the individual case natural-looking and symmetric ears were acquired. The aetiology of polyotia probably relates to abnormal migration of neural crest cell., (Copyright 2009 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Objective: To develop a quality of life scale for patients with congenital external and middle ear malformation, and to explore its reliability and validity., Methods: The initial quality of life scale for patients with congenital external and middle ear malformation was constructed based on quality of life scales from home and abroad. A total of 140 patients with congenital external and middle ear malformation had been recruited in the study. After pretest and item sifting, the quality of life scale was constructed, and its reliability and validity were evaluated., Results: Eighteen-item quality of life scale for patients with congenital external and middle ear malformation was constructed, which included three parts: physiological function, psychological status and social interaction. The retest reliability was 0.878; split-half reliability coefficient and Cronbach's alpha coefficient were 0.927 and 0.899, respectively. The results of factor analysis showed satisfactory construct validity. The reliability and validity of this scale was consistent with the demands of psychometrics., Conclusion: Congenital external and middle ear malformation quality of life scale is believable and effective, which can be used for clinical practice.

Objective: To explore the potential value of knowing the relationship between congenital auricular deformities and middle ear malformations., Methods: A total of 86 patients with congenital auricular deformities and middle ear malformations, including 51 males and 35 females, were admitted from January 2008 to December 2009 to the Eye Ear Nose and Throat Hospital of Fudan University. Fifty-eight patients had unilateral deformities (R:L = 34:24), while 28 were bilateral. One hundred and fourteen ears with congenital auricular deformities were included. High-resolution CT (HRCT) data was obtained from each patient. The auricular deformities were classified into three grades using the Marx H classification system. The modified Jahrsdoerfer grading system was used to score the malformations using HRCT data. The correlation between the grades of auricular deformities and scores of middle ear malformations was analyzed using Spearman rank correlation analysis., Results: The Marx H grades of congenital auricular deformities were 12 patients with grade I, 25 patients with grade II and 77 patients with grade III, while their corresponding Jahrsdoerfer scores were 7.8 ± 2.4, 6.8 ± 2.6 and 6.0 ± 2.8, respectively. The statistical analysis suggested a trend of negative correlation between the Marx H grades of auricular deformities and the Jahrsdoerfer scores of middle ear malformations (r = -0.2386, P = 0.0106)., Conclusion: There was a trend to a negative correlation between congenital auricular deformities and middle ear malformations.

Background: Ear reconstruction for congenital microtia is a challenge for the plastic and reconstructive surgeon. Ten percent of microtia cases are bilateral. However, the published literature contains relatively little information about auricular reconstruction in bilateral microtia. Some authorities prefer to reconstruct each side at different stages. In this article, we introduce an operative method to reconstruct both sides simultaneously. This is completely feasible, and saves time and cost. Furthermore, this method allows comparison between sides during surgery, and facilitates carving of bilateral ear frameworks of equal size and shape., Methods: From March 2007 to June 2008, 21 cases of congenital bilateral microtia were treated by post-auricular skin flap expansion, autogenous rib cartilage framework implantation, post-auricular fascial flap lifting, followed by split-thickness free skin grafting to reconstruct bilateral external ears during the same stage., Results: With a follow-up duration of 6 months to 1 year, two cases in a total of 21 showed different levels of absorption and cartilage deformation. The rest (19 cases) of the bilateral reconstructed ears showed good symmetry in size, shape and location. The bilateral reconstructed ears looked symmetrical and similar in outline, with well-defined structures., Conclusion: Simultaneous bilateral congenital microtia reconstruction is feasible and effective. The authors recommend it as the treatment of choice for bilateral microtia reconstruction., (Crown Copyright 2009. Published by Elsevier Ltd. All rights reserved.)

In microtia reconstruction, maintaining a healthy contact between the skin and the fabricated cartilage framework is essential to attaining a smooth and accentuated contour of the reconstructed auricle. Conventional means to achieve this include bolster sutures and continuous suction drains, both of which have associated shortcomings. A new dressing method was developed and applied in 10 consecutive patients who underwent the first of a two-stage microtia reconstruction using the Nagata technique. A small catheter was introduced into the space between the skin and the cartilage framework. Negative pressure was applied through the catheter, drawing the skin onto the cartilage framework. This evens out the skin, accentuates the contour of the framework and concurrently eliminates potential dead space. Skin contact on the framework is maintained whilst the catheter is removed and an occlusive transparent dressing is applied to the ear. Of the 10 cases in which this manoeuvre was performed, one had to be converted to the bolster suture technique due to a persistent air leak from the wound. Overall results of the nine cases in which this technique was carried out successfully demonstrate smooth skin contour and excellent definition of the fabricated framework. This negative pressure manoeuvre provides a simple, safe and consistent approach to achieving a smooth and accentuated contour in auricular reconstruction., (Copyright 2009 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Background: Pinna deformities, combined with congenital aural atresia, have been a matter of serious debate in the literature as they are associated with major aesthetic and functional problems that are difficult to manage. These problems have been described as early as 2000 BC. The aim of the present article is to approach the whole problem as one (pinna malformation and aural atresia) and present the history as well as the current approaches in reconstruction., Methods: Extensive literature search and medical history books were used as scientific sources., Results: For many centuries, the prevalent view was that any surgical attempts to reconstruct the pinna and the ear canal were of little value. In addition, the aesthetic result of these early surgical procedures was mostly unacceptable. Over time, new surgical techniques and synthetic materials were used, leading to satisfactory and lasting aesthetic and functional results in selected patients, improving their quality of life, while reducing the complication rate. However, many cases are still challenging for plastic surgeons and ENT surgeons alike., Conclusions: Despite significant progress in the field, surgery for pinna deformities combined with congenital aural atresia still remains one of the most challenging and risky procedures. Accurate audiological evaluation of newborns as well as assessment of their craniofacial development is necessary and can help the plastic surgeons and otologists choose proper candidates for surgical repair and a suitable and age-appropriate therapeutic plan. History and repeated failures have taught us that close multidisciplinary approach is of paramount importance., (Copyright (c) 2009 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)