The Hyperproliferation Mechanism of Cholesteatoma Based on Proteomics: SNCA Promotes Autophagy-Mediated Cell Proliferation Through the PI3K/AKT/CyclinD1 Signaling Pathway.

Cholesteatoma is a chronic inflammatory ear disease with abnormal keratinized epithelium proliferation and tissue damage. However, the mechanism of keratinized epithelium hyperproliferation in cholesteatoma remains unknown. Hence, our study sought to shed light on mechanisms affecting the pathology and development of cholesteatoma, which could help develop adjunctive treatments. To investigate molecular changes in cholesteatoma pathogenesis, we analyzed clinical cholesteatoma specimens and paired ear canal skin with mass spectrometry-based proteomics and bioinformatics. From our screen, alpha-synuclein (SNCA) was overexpressed in middle ear cholesteatoma and might be a key hub protein associated with inflammation, proliferation, and autophagy in cholesteatoma. SNCA was more sensitive to lipopolysaccharide-induced inflammation, and autophagy marker increase was accompanied by autophagy activation in middle ear cholesteatoma tissues. Overexpression of SNCA activated autophagy and promoted cell proliferation and migration, especially under lipopolysaccharide inflammatory stimulation. Moreover, inhibiting autophagy impaired SNCA-mediated keratinocyte proliferation and corresponded with inhibition of the PI3K/AKT/CyclinD1 pathways. Also, 740Y-P, a PI3K activator reversed the suppression of autophagy and PI3K signaling by siATG5 in SNCA-overexpressing cells, which restored proliferative activity. Besides, knockdown of SNCA in RHEK-1 and HaCaT cells or knockdown of PI3K in RHEK-1 and HaCaT cells overexpressing SNCA both resulted in attenuated cell proliferation. Our studies indicated that SNCA overexpression in cholesteatoma might maintain the proliferative ability of cholesteatoma keratinocytes by promoting autophagy under inflammatory conditions. This suggests that dual inhibition of SNCA and autophagy may be a promising new target for treating cholesteatoma.

Improved reconstruction for highly accelerated propeller diffusion 1.5 T clinical MRI.

PURPOSE: Propeller fast-spin-echo diffusion magnetic resonance imaging (FSE-dMRI) is essential for the diagnosis of Cholesteatoma. However, at clinical 1.5 T MRI, its signal-to-noise ratio (SNR) remains relatively low. To gain sufficient SNR, signal averaging (number of excitations, NEX) is usually used with the cost of prolonged scan time. In this work, we leveraged the benefits of Locally Low Rank (LLR) constrained reconstruction to enhance the SNR. Furthermore, we enhanced both the speed and SNR by employing Convolutional Neural Networks (CNNs) for the accelerated PROPELLER FSE-dMRI on a 1.5 T clinical scanner. METHODS: Residual U-Net (RU-Net) was found to be efficient for propeller FSE-dMRI data. It was trained to predict 2-NEX images obtained by Locally Low Rank (LLR) constrained reconstruction and used 1-NEX images obtained via simplified reconstruction as the inputs. The brain scans from healthy volunteers and patients with cholesteatoma were performed for model training and testing. The performance of trained networks was evaluated with normalized root-mean-square-error (NRMSE), structural similarity index measure (SSIM), and peak SNR (PSNR). RESULTS: For 4 × under-sampled with 7 blades data, online reconstruction appears to provide suboptimal images-some small details are missing due to high noise interferences. Offline LLR enables suppression of noises and discovering some small structures. RU-Net demonstrated further improvement compared to LLR by increasing 18.87% of PSNR, 2.11% of SSIM, and reducing 53.84% of NRMSE. Moreover, RU-Net is about 1500 × faster than LLR (0.03 vs. 47.59 s/slice). CONCLUSION: The LLR remarkably enhances the SNR compared to online reconstruction. Moreover, RU-Net improves propeller FSE-dMRI as reflected in PSNR, SSIM, and NRMSE. It requires only 1-NEX data, which allows a 2 × scan time reduction. In addition, its speed is approximately 1500 times faster than that of LLR-constrained reconstruction.

Congenital cholesteatoma: what radiologists need to know.

Congenital cholesteatoma is a rare, non-neoplastic lesion that causes conductive hearing loss in children. It is underrecognized and often diagnosed only when there is an established hearing deficit. In the pediatric population, hearing deficiency is particularly detrimental because it can impede speech and language development and, in turn, the social and academic well-being of affected children. Delayed diagnosis leads to advanced disease that requires more extensive surgery and a greater chance of recurrence. A need to promote awareness and recognition of this condition has been advocated by clinicians and surgeons, but no comprehensive imaging review dedicated to this entity has been performed. This review aims to discuss the diagnostic utility of high-resolution computed tomography and magnetic resonance imaging in preoperative and postoperative settings in congenital cholesteatoma. Detailed emphasis is placed on the essential preoperative computed tomography findings that facilitate individualized surgical management and prognosis in the pediatric population.

Outcomes of perichondrium graft covering the epithelium of the tympanic membrane for large-sized perforations: A 3-5-year follow-up study.

OBJECTIVE: The objective of this study was to evaluate the graft outcomes and iatrogenic cholesteatomas for 3 years following cartilage-perichondrium over-underlay technique with perichondrial graft covering the epithelium for large-sized tympanic membrane (TM) perforations. MATERIALS AND METHODS: This prospective case series enrolled patients with large-sized perforation who underwent endoscopic cartilage-perichondrium over-underlay technique. The graft success rate, hearing outcomes, and development of iatrogenic middle ear cholesteatomas and graft cholesteatomas were assessed at 3 years postoperatively. RESULTS: This study included 62 ears of 62 patients. The mean follow-up duration was 47.3 ± 10.8 (38-64) months. Neovascularization was observed in the lateral perichondrium graft in 55 (88.7 %) patients, which inosculated into the TM remnant at 4-5 weeks. However, graft neovascularization was not observed in the four patients with excessive perichondrium graft that migrated into the external auditory canal and the three patients with middle ear infections. The graft failure rate was 6.5 % at 6 months, 11.3 % at 12 months, 6.5 % at 24 months, and the overall graft success rate was 91.8 % at the last follow-up. Granular myringitis developed in 11.3 % (7/62) of the patients. High-resolution computed tomography revealed well-pneumatized mastoids and middle ear at the final follow-up. However, graft cholesteatomas were observed in 3 (4.8 %) patients at 7-24 months postoperatively. CONCLUSIONS: The cartilage-perichondrium over-underlay technique with perichondrial graft covering TM epithelium is safe and effective for the repair of large perforations, with good short- and long-term graft outcomes, minimal risk of graft cholesteatoma development, and no risk of iatrogenic middle ear cholesteatomas.

Hypertrophic pachymeningitis of the internal auditory canal and cerebellopontine angle from a large recurrent attic cholesteatoma.

OBJECTIVES: To describe a rare complication of cholesteatoma. METHODS: Case report with literature review. RESULTS: We report a case of a 37-year-old male who presented for evaluation of otorrhea, headache, and progressive left sensorineural hearing loss. Clinical and radiologic evaluation demonstrated a large recurrent attic cholesteatoma with erosion into the lateral and superior semicircular canals, and diffuse enhancement of the internal auditory canal and cerebellopontine angle suggestive of hypertrophic pachymeningitis secondary to cholesteatoma. After treatment with a course of antibiotics and canal wall down mastoidectomy surgery for cholesteatoma exteriorization, he experienced improvement of his symptoms and resolution of hypertrophic pachymeningitis. CONCLUSION: Hypertrophic pachymeningitis is a rarely described complication of cholesteatoma. In the context of cholesteatoma, treatment with antibiotics and surgical removal or exteriorization of cholesteatoma are effective treatments for HP.

3D printed temporal bones for preoperative simulation and planning.

OBJECTIVE: Demonstrate the utility of 3D printed temporal bone models in individual patient preoperative planning and simulation. METHODS: 3D models of the temporal bone were made from 5 pediatric and adult patients at a tertiary academic hospital with challenging surgical anatomy planned for cochlear implantation or exteriorization of cholesteatoma with complex labyrinthine fistula. The 3D models were created from CT scan used for preoperative planning, simulation and intraoperative reference. The utility of models was assessed for ease of segmentation and production and impact on surgery in regard to reducing intraoperative time and costs, improving safety and efficacy. RESULTS: Three patients received cochlear implants, two exteriorization of advanced cholesteatoma with fistulas (1 internal auditory canal/cochlea, 1 all three semicircular canals). Surgical planning and intraoperative referencing to the simulations by the attending surgeon and trainees significantly altered original surgical plans. In a case of X-linked hereditary deafness, optimal angles and rotation maneuvers for cochlear implant insertion reduced operating time by 93 min compared to the previous contralateral side surgery. Two cochlear implant cases planned for subtotal petrosectomy approach due to aberrant anatomy were successfully approached through routine mastoidectomy. The cholesteatoma cases were successfully exteriorized without necessitating partial labyrinthectomy or labyrinthine injury. There were no complications. CONCLUSION: 3D printed models for simulation training, surgical planning and use intraoperatively in temporal bone surgery demonstrated significant benefits in designing approaches, development of patient-specific techniques, avoidance of potential or actual complications encountered in previous or current surgery, and reduced surgical time and costs.

Predicting residual cholesteatoma with the Potsic staging system still lacks evidence: a systematic review and meta-analysis.

PURPOSE: To investigate the rate of residual disease in the Potsic staging system for congenital cholesteatomas. METHODS: A protocol registration was published on PROSPERO (CRD42022383932), describing residual disease as a primary outcome and hearing improvement as secondary. A systematic search was performed in four databases (PubMed, Embase, Cochrane Library, Web of Science) on December 14, 2022. Articles were included if cholesteatomas were staged according to the Potsic system and follow-up duration was documented. Risk of bias was evaluated using the Quality In Prognosis Studies (QUIPS) tool. In the statistical synthesis a random effects model was used. Between-study heterogeneity was assessed using I2. RESULTS: Thirteen articles were found to be eligible for systematic review and seven were included in the meta-analysis section. All records were retrospective cohort studies with high risk of bias. Regarding the proportions of residual disease, analysis using the χ2 test showed no statistically significant difference between Potsic stages after a follow-up of minimum one year (stage I 0.06 (confidence interval (CI) 0.01-0.33); stage II 0.20 (CI 0.09-0.38); stage III 0.06 (CI 0.00-0.61); stage IV: 0.17 (CI 0.01-0.81)). Postoperative and preoperative hearing outcomes could not be analyzed due to varied reporting. Results on cholesteatoma location and mean age at staging were consistent with those previously published. CONCLUSION: No statistically significant difference was found in the proportions of residual disease between Potsic stages, thus the staging system's applicability for outcome prediction could not be proven based on the available data. Targeted studies are needed for a higher level of evidence.

Reconstructive ossiculoplasty options in primary cholesteatoma surgeries with an intact stapes.

PURPOSE: In primary cholesteatoma patients, incus destruction with an intact and mobile stapes is a frequent finding. Different techniques have been described to restore the ossicular chain, including incus interposition, stapes augmentation and type III tympanoplasty. Controversy about postoperative hearing results in open versus closed surgical techniques exist. METHODS: We performed a retrospective analysis of clinical, surgical and audiometric data of patients with primary cholesteatoma surgery operated between 2010 and 2020, and a mobile stapes and one-stage ossicular reconstruction. Pre- and post-operative audiograms were compared for the different surgical groups, mainly focusing on postoperative air-bone gap. Mastoid pneumatization and ventilation was also considered. RESULTS: The mean postoperative air-bone gap (0.5-4 kHz) of the 126 included patients was 20 dB. Hearing after type III tympanoplasty (26 dB) was worse than incus interposition (19 dB) and stapes augmentation (20 dB). Hearing after an open (23 dB) versus closed (19 dB) surgical technique was significantly different. No improvement in air-bone gap was observed for the higher frequencies. CONCLUSION: A residual postoperative air-bone gap should be considered after primary cholesteatoma surgery with intact and mobile stapes. Incus interposition in closed cavity operation is the optimal situation, but open cavity surgery should not be avoided because of hearing. Extent of the disease is prioritized and poorer ventilation before and after surgery may affect postoperative hearing.

Acquired cholesteatoma after cochlear implants: case series and literature review.

PURPOSE: To assess the prevalence and management of acquired cholesteatoma after cochlear implantation in pediatric and adult patients. METHODS: Retrospective case review of pediatric and adult cochlear implants (CI) followed at a tertiary referral center and literature review of acquired cholesteatoma after CI surgery, to identify its prevalence, cause, and treatment. RESULTS: Nine pediatric CIs were diagnosed with cholesteatoma in seven patients after 6.4 ± 4 years from CI surgery, and two adults after 11.3 and 21.7 years from CI surgery. Thirty-four pediatric cases and 26 adult cases are described in the literature. Cholesteatoma has a prevalence of 0.54% in pediatric CIs, and 1.79% in adult CIs (case series and literature). Adult cases were diagnosed significantly later compared to pediatric cases (Mann-Whitney test, p = 0.0460). Three pediatric cholesteatomas were treated with conservative surgery and preservation of the CI; they all developed recurrent disease. The remaining pediatric cases underwent subtotal petrosectomy with simultaneous CI explantation and staged reimplantation. Only one case recurred. The adult cases underwent simultaneous subtotal petrosectomy, explantation, and reimplantation. Similarly, 33.3% of cases treated with conservative/reconstructive surgery in the literature required revision surgery or conversion to subtotal petrosectomy against 6.2% of subtotal petrosectomies in the literature. CONCLUSIONS: Cholesteatoma after CI is a rare and late-onset complication of CIs. It is more prevalent in the adult CI population, although it affects children significantly earlier. The treatment of choice is subtotal petrosectomy and CI explantation with simultaneous or staged reimplantation.

Long-term clinical and radiological results for fat graft obliteration in subtotal petrosectomy and cochlear implant surgery: a retrospective clinical study.

PURPOSE: The study aimed to evaluate the long-term clinical, radiological, and functional results for subtotal petrosectomy and cochlear implant surgery with closure of the external auditory canal and fat obliteration. METHODS: We retrospectively included all consecutive cases of simultaneous subtotal petrosectomy and cochlear implant surgery performed at a tertiary referral center between 2009 and 2016 using the same surgical technique. All patients underwent postoperative high-resolution computed tomography (HRCT) and annual audiological assessments. A 5-year minimum clinical, radiological, and audiological follow-up was performed. The early and late postoperative results were compared. The main outcome measures were complications, postauricular retraction, fat graft reabsorption, and audiological outcomes. RESULTS: Twenty-nine procedures performed in 23 patients (six bilateral) met the inclusion criteria. The mean age of the patients was 67 ± 13.4 years and mean follow-up duration was 7.5 ± 2 years. At follow-up, postauricular retraction was detected in 24 cases (82.8%), including five cases (17.1%) with subcutaneous protrusion of implant and array. Fat graft volume was significantly reduced at late-HRCT in terms of maximum diameter (2.24 ± 1.0 cm vs 3.69 ± 0.7 cm; p < 0.0005) and surface area (1.88 ± 1.2 vs 4.24 ± 1.6 cm2, p < 0.0005). Six patients had extracochlear electrodes at late-HRCT (3/6 had an increased number of extracochlear electrodes), with a lowering of this group's performance of - 15% (p < 0.005) in the follow-up speech comprehension test. CONCLUSIONS: Subtotal petrosectomy with cochlear implantation is an effective long-term technique in selected cases. Fat grafts showed significant reabsorption at long-term follow-up with reaeration of the middle ear spaces. Prolonged clinical and radiological follow-up is recommended for monitoring implant performances and late complications.

Occurrence of mucosa-affecting diseases of the upper airways in middle ear cholesteatoma patients: a nationwide case-control study.

PURPOSE: Exploring a possible link between upper airway inflammation and the development of cholesteatoma by studying the association between mucosa-affecting diseases of the upper airways and cholesteatoma surgery. METHODS: This is a nationwide case-control study of 10,618 patients who underwent surgery for cholesteatoma in Sweden between 1987 and 2018. The cases were identified in the National Patient Register and 21,235 controls matched by age, sex and place of residency were included from national population registers. Odds ratios (OR) and corresponding 95% confidence intervals were used to assess the association between six types of mucosa-affecting diseases of the upper airways and cholesteatoma surgery. RESULTS: Chronic rhinitis, chronic sinusitis and nasal polyposis were more common in cholesteatoma patients than in controls (OR 1.5 to 2.5) as were both adenoid and tonsil surgery (OR > 4) where the strongest association was seen for adenoid surgery. No association was seen between allergic rhinitis and cholesteatoma. CONCLUSION: This study supports an association between mucosa-affecting diseases of the upper airways and cholesteatoma. Future studies should aim to investigate the mechanisms connecting mucosa-affecting diseases of the upper airways and cholesteatoma formation regarding genetic, anatomical, inflammatory and mucosa properties.

Exploring trends and developments in cholesteatoma research: a bibliometric analysis.

PURPOSE: Examination of the scientific literature on cholesteatoma from past to present using bibliometric methods. METHODS: A total of 2353 articles on cholesteatoma between 1980 and 2023 were obtained from the Web of Science database and statistically analysed using bibliometric techniques. RESULTS: The journals with the highest number of contributions to the literature were Otology & Neurotology (n = 192), Acta Oto-Laryngologica (156) and Laryngoscope (149). The most impactful journals based on h-index and total citation (TC) counts were Laryngoscope (h:36, TC: 4241), Otology & Neurotology (29, 3141), and American Journal of Otology (26, 2071) respectively. The most prolific author was Kojima H. (n = 49). According to the findings of the Reference Publication Year Spectroscopy (RPYS) analysis, there has been a significant increase in academic interest in Cholesteatoma since its first description in 1959, particularly following the expansion of its definition in 1977. Subsequently, the trend of increased interest has risen rapidly in later years, with notable peaks in research intensity observed in 1989, 2006, and 2011. The highest academic trend occurred in 2006. In recent years, trending topics have included innovative approaches such as diagnostic and imaging methods particularly alongside technological developments, endoscopic surgery, recurrence and complications, prognosis and quality of life, artificial intelligence, and obliteration. CONCLUSION: In conclusion, the evolution of cholesteatoma and its research priorities reveal temporal shifts and emerging areas of interest over time. Primarily, diagnosis, treatment, and surgical approaches remain central. Additionally, research has expanded to encompass developments in surgical techniques, imaging modalities, cellular biology, pathology, and molecular mechanisms.

Endoscopic approach to geniculate ganglion: a multicentric experience.

PURPOSE: A variety of lesions could arise from the GG area, or extend into this region from adjacent sites. The management of perigeniculate lesions includes observation, surgery, and radiation, according to the nature, the size of the lesion, and the accompanying symptoms. Preliminary experiences on the exclusive transcanal endoscopic approach to the GG area have shown safety and feasibility avoiding of any postauricular incision, or brain manipulation. The experience from two referral centers on patients treated for a GG lesion with a totally endoscopic approach is herein reported. METHODS: Data about patients who underwent exclusive endoscopic approach to the GG area at the Otolaryngology Departments of the University Hospitals of Modena and Bologna between May 2017 and February 2022 were retrospectively collected. RESULTS: The total number of patients included in our study was 11. 10 patients (91%) had progressive unilateral facial paralysis and 1 patient (11%) presented with chronic otorrhea. The mean largest diameter of the treated lesions was of 8 mm. The resection was extended to the fundus of the IAC in 2 patients (expanded approach). The remaining 9 patients (82%) underwent partial ossicular replacement prosthesis (PORP). No major complications occurred. Facial nerve outcomes were good in all patients and the mean ABG worsened from 12 dB pre-operatively to 22 dB post-operatively. CONCLUSIONS: The exclusively endoscopic approach to GG lesions represents a viable alternative to traditional microscopic approaches and may be included in the armamentarium of ear surgeons.

A tailored approach in cholesteatoma surgery.

PURPOSE: In recent years, new techniques have been added to cholesteatoma surgery, and established microsurgical approaches are being reconsidered. This study aims to present the importance of individualized decision-making for the selection of an intact canal wall (CWU) or canal wall down (CWD) surgical procedure for each patient. METHODS: Using the "ChOLE" classification we categorized 264 operations retrospectively. 162 CWU and 102 CWD surgeries were performed. We focus to determine why a CWD procedure was chosen quite frequently despite some low-stage cases. Furthermore, we evaluated recidivism and hearing outcomes. RESULTS: Smaller cholesteatomas (Ch-stage 1a, 1b & 2a) were found in 182 patients (70%), ossicular chain status feasible for straightforward reconstruction (O-stage 0, 1 & 2) was present in 186 patients (70%), minor complications due to the cholesteatoma (L-stage 1) were infrequent with 28 cases (11%) and a well-pneumatized mastoid was found in 144 cases (55%). Recidivism rates were low (7%) without any difference in both groups and a mean follow-up time of 4 years and 8 months. In primary surgeries there was a significant difference (p < 0.05) in postoperative mean air-bone gap (ABG) between CWU (17dB) and CWD (27dB). CONCLUSION: The main goals of cholesteatoma surgery remain the avoidance of recidivism and optimal hearing rehabilitation. We recommend a tailored approach in the treatment of cholesteatomas and not a dogmatic one. Surgeons should not hesitate to perform a CWD procedure if required. Performed correctly it results in a dry ear and CWD surgery should remain in the skill set of the otologic surgeon.

A study on the efficacy of combined surgery in advanced-stage congenital cholesteatoma with canal-wall-up surgery.

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

Combined microscope-endoscopy resection of petrous bone cholesteatoma with temporary facial nerve transposition versus nontransposition.

PURPOSE: The narrow supralabyrinthine space affects surgical procedures. To study the effect of temporary transposition of geniculate ganglion of facial nerve versus nontransposition on lesion recurrence and facial nerve function in patients with petrous bone cholesteatoma. METHODS: A total of 18 patients with petrous bone cholesteatoma involving the facial nerve were treated in our hospital from November 2016 to March 2023. The main surgical method is the extended supralabyrinthine approach assisted by a microscope and an endoscope. We collected and retrospectively analyzed their medical records. RESULTS: Temporary facial nerve transposition was performed in five patients, and nontransposition was performed in 13 patients. Cholesteatoma recurred in three patients with facial nerve nontransposition, whereas none in patients with facial nerve transposition. In this study, except for one case with a second operation, postoperative facial paralysis in other cases was improved to varying degrees, and there was no significant difference between the two groups. CONCLUSION: Temporary transposition of geniculate ganglion of facial nerve will not affect the postoperative nerve function of patients and can reduce the possibility of cholesteatoma recurrence of the petrous bone.

Management of Petrous Bone Cholesteatoma: The Gruppo Otologico Experience.

BACKGROUND: The term petrous bone cholesteatoma (PBC) refers to lesions extending deep to the bony labyrinth via superior, inferior, and posterior cell tracts. PBC is a rare incidence accounting for only 4-9% of petrous bone lesions. Lesions of petrous bone represent a real surgical challenge due to its complex relationship with critical neurovascular structures. OBJECTIVE: To demonstrate our 40-plus years' experience in the management of PBC, depict the clinical features of PBC according to Sanna's Classification, evaluate the postoperative follow-up of surgically treated PBC patients, and determine the recurrence rate. STUDY DESIGN: Retrospective medical record review. MATERIAL AND METHODS: Medical records of 298 PBC cases operated from the year 1983 to 2024 were thoroughly evaluated. RESULTS: A total of 298 PBC cases were surgically treated at our center. The average age at presentation in this series was 47 years. Males are more affected than females with a male-to-female ratio of 2.2:1. The most common presenting symptoms were hearing loss (84%), tinnitus (48%), and facial nerve paralysis (45%). Mixed hearing loss (41%) was the commonest audiometric pattern of hearing loss followed by conductive hearing loss (26%) and profound sensorineural hearing loss (4%) and a total of 86 (29%) had anacusis at presentation. On preoperative facial nerve function examination, 133(45%) of patients had various degrees of paresis and complete paralysis whereas 55% had normal HB-I function. The commonest degree of paresis noted was HB-III (18%) followed by HB-VI (5%). A total of 150 (50%) patients had previous otologic surgery and two-thirds of these cases had two or more prior otologic surgeries. According to Sanna's PBC Classification system, we identified that the supralabyrinthine class (44%) is the commonest of all classes followed by massive (33%), infralabyrinthine-apical (9%), infralabyrinthine(8%), and apical (5%) classes in that order. However, only ten patients had congenital type of PBC. Extension to clivus, sphenoid, nasopharynx, intradural space, and occipital condyle was found in 8, 2, 1, and 2 cases respectively. The most commonly used surgical approaches at our center were TO, MTCA with rerouting of the facial nerve, and TLAB with external auditory canal (EAC) closure. Postoperative complications were minimal and the duration of follow-up ranged from one to 458 months with a mean duration of 65 months. Residual lesions were evident in 11 cases (3.7%), with the surgical cavity, middle and posterior fossa dura, and jugular bulb being the commonest sites. CONCLUSION: Petrous bone cholesteatoma represents diagnostically and surgically challenging lesions of temporal bone which are usually frustrating to the treating surgeon. A high index of clinical suspicion, thorough clinical evaluation examination, and preoperative radiologic evaluation make the diagnosis easier. Preoperative anatomic classification of the lesion enables the physician to choose the appropriate surgical approach. Sanna's classification is widely used to classify PBC in relation to the labyrinthine block. Radical disease removal should always come before hearing preservation. Cavity obliteration is the solution to the problems related to a large cavity. Finally, advancements in lateral skull base approaches create adequate surgical access for the complete removal of the lesion with excellent control of critical neurovascular structures.

[Contemporary diagnosis and management of congenital microtia and aural atresia : Part 2: Overview of therapeutic approaches]. / Aktuelle Diagnostik und Therapie bei Ohrmuscheldysplasien und Gehörgangsfehlbildungen : Teil 2: Übersicht der verschiedenen Therapieansätze.

Congenital malformations of the pinna and aural atresia can result in major aesthetic and functional deficits. Knowledge about embryologic developments and established classification systems is an essential requirement when dealing with affected patients. Early detection of deficiencies and introduction of appropriate diagnostic measures is vital to initiate adequate therapies and prevent long-term disabilities. Treatment for malformations of the pinna-if requested-is mostly surgical, infrequently an epithesis is applied. As in other surgical fields, tissue engineering will likely play a crucial role in the future. Treatment of aural stenosis and atresia aims at improvement of hearing levels and prevention of secondary complications like cholesteatoma and chronic otorrhea. Auditory rehabilitation comprises a spectrum from conventional hearing aids to invasive hearing implants, the latter being favored in recent years.

Surgical Option for External Auditory Canal Cholesteatoma: A Case Report.

External Auditory Canal Cholesteatomas (EACC), is an exceptionally rare condition with a prevalence of only 0.1-0.5% among new patients1. EACC are known to possess bone eroding properties, causing a variety of complications, similar to the better-known attic cholesteatomas. We describe here the novel surgical management of a case of EACC. She is 38-year-old female who presented with otorrhea for 6 months. Clinical examination and radiological investigations suggested the diagnosis of an external auditory canal cholesteatoma. The patient underwent modified radical mastoidectomy with type 1 tympanoplasty with meatoplasty. Post-operatively, the patient showed marked clinical improvement.

Recurrent Cholesteatoma: Why it occurs?

A cholesteatoma is an expansion of keratinizing squamous epithelium that enters the middle ear cleft from the outer layer of the tympanic membrane or ear canal. Choleatomas are always treated surgically. Recurrence of the illness presents another challenge for the patient and the surgeon, though. There have been reports of recurrence rates as high as 30% in adults and as high as 70% in children. Here, we describe a case of persistent recurrent otorrhea following revision surgery, along with acquired recurrent cholesteatoma following canal wall down surgery. A 38-year -male with underlying Diabetes Mellitus and Hypertension presented with left scanty and foul-smelling ear discharge for 2 years and left reduced hearing. He was diagnosed with left chronic active otitis media with cholesteatoma for which he underwent left modified radical mastoidectomy, meatoplasty and tympanoplasty in 2017. Five months post operatively, he presented with left otorrhea. However, he defaulted followed up and presented in April 2018 for similar complaints. Otoscopy examination revealed left tympanic membrane perforation at poster superior quadrant of pars tensa and bluish discoloration behind pars flacida. He was diagnosed as recurrent left cholesteatoma and subsequently he underwent left mastoid exploration under general anesthesia in June 2018. Postsurgery, he developed recurrent ear discharge which was treated with topical antibiotics and ear toileting. We report a case of recurrent Cholesteatoma despite canal wall down procedure requiring a second redo procedure and with persistent recurrent otorrhea after the redo procedure.However, this case demonstrates the need for regular follow ups even after a canal wall down procedure for detecting recurrence of disease. Moreover, this case denotes some of the patient factors and surgeon factors involved in disease recurrence. Furthermore, importance of opting for an imaging study in case of high suspicion of the disease.