Open-type cholesteatoma is the predictive factor for residual disease in congenital cholesteatoma treated with TEES.

OBJECTIVE: To determine the predictive factors for residual disease occurring after surgical removal of congenital cholesteatomas and whether these predictive factors differ between microscopic ear surgery (MES) using data from the literature and transcanal endoscopic ear surgery (TEES) using data from our own institution. METHODS: Twenty-three patients with a congenital cholesteatoma who underwent surgical treatment at Yamagata University Hospital between December 2011 and December 2017 were retrospectively investigated. We divide TEES into three different approaches: non-powered TEES, powered TEES and dual MES/TEES. Main outcome measures were Potsic stage, closed or open congenital cholesteatoma type, TEES surgical approach, appearance of residual disease, tympanoplasty type and hearing outcome. RESULTS: A logistic regression analysis was conducted on the Potsic stage, closed or open type, TEES surgical approach and age to obtain the odds ratio for residual disease. The chance of residual disease significantly increased in the presence of an open-type congenital cholesteatoma (odds ratio: 30.82; 95 % confidence interval: 1.456-652.3; p = 0.0277), but not for any of the other factors including Potsic stage. The timing of the confirmation of residual disease after ossicular chain reconstruction was analyzed using a Kaplan-Meier analysis. The residual disease rate was significantly higher with an open-type congenital cholesteatoma (log-rank test, p < 0.05). In addition, all residual disease occurred within three years after surgery. CONCLUSIONS: Our results showed that an open-type congenital cholesteatoma is the strongest predictive factor for residual disease when removing a congenital cholesteatoma by TEES.

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.

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.

Cholesteatoma in the pediatric aerodigestive population.

OBJECTIVES: To report the prevalence of cholesteatoma and related comorbidities in pediatric aerodigestive patients requiring tracheostomy or airway reconstruction procedures. To use study findings to inform clinical management of these complex patients. METHODS: A repository of clinical data drawn from our institution's electronic medical records was queried to identify airway reconstruction (airway) and complex hospital control (control) patient cohorts. Retrospective chart review was then performed to investigate the occurrence of cholesteatoma and related pathologies in these patients, as well as clinical management. RESULTS: The prevalence of cholesteatoma in airway and control patients was 6/374 (1.60 %) and 35/30,565 (0.11 %), respectively. The relative risk of cholesteatoma diagnosis in airway patients was 14.01 (95 % CI 6.06-32.14). Airway patients were more likely than control patients to have pressure equalization tube history (relative risk 3.25, 95 % CI 2.73-3.82). Age at cholesteatoma diagnosis and first surgical intervention was younger in airway compared to control patients (5.43 vs. 8.33, p = 0.0182, and 6.07 vs. 8.82, p = 0.0236). However, time from diagnosis to intervention and extent of surgery were similar between the groups. CONCLUSION: This is the first study to investigate the prevalence of cholesteatoma in the pediatric aerodigestive population. The relative risk of cholesteatoma diagnosis was found to be 14 times higher in patients with tracheostomy or airway reconstruction history. Underlying eustachian tube and palatal dysfunction are likely contributing factors to the elevated risk. Additionally, cholesteatoma in this population was diagnosed and required surgical intervention at a younger age, which may suggest a more aggressive disease course. Providers should maintain a high degree of suspicion for cholesteatoma in this complex population.

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.

Adult-onset congenital cholesteatoma in the hypotympanum initially presenting as Bell's palsy: A case report.

INTRODUCTION: Cholesteatoma is a rare disease characterized by the accumulation of keratinized squamous epithelial cells in the middle ear or mastoid cavity. Vertigo and facial palsy, which are rare complications, may indicate erosion into the semicircular canals or the fallopian canal. PATIENT CONCERNS: A 40-year-old woman presented to our clinic with progressive right-sided hearing loss over 5 years (primary concern). Approximately 10 years ago, the patient had developed acute right-sided facial weakness with no additional symptoms. A neurologist at another hospital had diagnosed her condition as Bell's palsy and treated it accordingly. DIAGNOSIS: Adult-onset congenital cholesteatoma in the hypotympanum. INTERVENTION: Combined endoscopic and microscopic removal of the cholesteatoma. OUTCOMES: Physical examination revealed slight improvement in right-sided peripheral facial palsy. LESSON: Routine eardrum examination is recommended for patients presenting with isolated peripheral facial palsy. If necessary, a patient should be referred to an otologist for further evaluation and treatment.

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.

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.

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.

Risk factors for occurrence and progression of external auditory canal cholesteatoma.

OBJECTIVE: External auditory canal cholesteatoma (EACC) is characterized by retained squamous debris within the external canal and variable amounts of localized bone destruction. The etiology of primary EACC remains incompletely understood. This study was conducted to analyze the clinical features and backgrounds of patients with primary EACC and to clarify the risk factors for the occurrence and progression of EACC. METHODS: Sixty-nine ears of 62 patients diagnosed with primary EACC were included in this study (EACC group). Additionally, 74 ears of 60 patients with chronic otitis media (COM) with perforation who underwent tympanoplasty or myringoplasty were included as controls (COM group). We retrospectively investigated the clinical features, life history, and medical history of the patients in both groups. In addition, to investigate the risk factors for progression of EACC, we compared the clinical features and medical history of patients with stage IV (advanced) disease versus stage I + II (mild) disease. RESULTS: The inferior wall of the bony canal was the main structure affected in patients with primary EACC of all stages. The following factors were significantly more common in the EACC than COM group: older age, female sex, left-sided disease, osteoporosis, renal dysfunction, anemia, and treatment with bisphosphonates. Among these, the most significant factor associated with EACC was renal dysfunction (odds ratio, 11.4; 95 % confidence interval, 2.32-55.9). The significant factors observed in patients with stage IV disease were younger age, male sex, posterior wall involvement, and otorrhea. Surgical treatment was required for more than half of the patients with stage III and IV EACC. CONCLUSION: Patients with renal dysfunction are at risk of primary EACC. In particular, younger patients and relatively younger elderly patients with posterior wall involvement have a risk of progression to advanced-stage EACC. Canalplasty should be considered in patients with EACC who have these risk factors to prevent progression to advanced-stage disease.

Comparison of temporalis fascia and cartilage graft over-under myringoplasty for repairing large perforations.

OBJECTIVE: The objective of this study was to compare the operation time, graft success, audiometric outcomes and complications of over-under technique using a temporalis fascia (TMF) and cartilage grafts for the repair of large perforations. STUDY DESIGN: Randomized controlled trial. MATERIALS AND METHODS: 80 large perforations >2 quadrants of eardrum were prospectively randomized to undergo TMF over-under technique group (TFON, n = 40) or cartilage-perichondrium over-under technique group (CPON, n = 40). The graft success rate, audiometric outcomes, and complications were compared among two groups at 12 months. RESULTS: The mean operation time was 56.8 ± 4.2 (range:52-71) min in the TFON group and 37.9 ± 2.8 (range: 31-47) min in the CPON group (P < 0.001). The lost follow-up rate was 3 (7.5 %) patients in the TFON group and 2 (5.0 %) patient in the CPON group (P = 0.644). Finally, 37 patients in the TFON group and 38 patients in the CPON group were included in this study. The graft infection rate was 2 (5.4 %) patients in the TFON group and 2 (5.3 %) patient in the CPON group (P = 0.626), all the graft infection resulted in the residual perforation. The remaining residual perforation was 2 (5.4 %) patients in the TFON group and 1 (2.6 %) patient in the CPON group; the re-perforation was 3 (8.1 %) patients in the TFON group and 0 (0.0 %) patient in the CPON group. The graft success rate was 81.1 % (30/37) patients in the TFON group and 92.1 % (35/38) patient in the CPON group. The mean preoperative and 12-month postoperative ABGs were significantly different in any group (P < 0.01). However, there were no significant difference among two groups regardless of pre-or post-ABGs or ABG closure. No lateralization of the graft or blunting was noted in any group. Four (10.8 %)patients developed atelectasis and one (2.7 %) developed the EAC scarring in the TFON group. Graft cholesteatomas was found in 2 (5.4 %) patients in the TFON group and in 5 (13.2 %) patients in the CPON group (P = 0.449). Three (8.1 %) patients had temporary hypogeusia in the TFON group. CONCLUSION: Although temporalis fascia graft over-under technique obtained similar graft success rates and hearing outcomes for large chronic perforations to the cartilage-perichondrium over-under technique, temporalis fascia graft technique prolonged the operation time and increased the re-perforation and graft atelectasis. Nevertheless, the graft cholesteatomas were comparable among two techniques.

Attic reconstruction techniques in cholesteatoma surgery: a comparative analysis of cartilage versus bone pate.

BACKGROUND: Cholesteatoma surgery involves canal wall down (CWD) and canal wall up (CWU) mastoidectomy. CWU is associated with higher cholesteatoma recurrence, often linked to attic retraction pockets. Attic reconstruction with cartilage or bone pate lacks comparative evidence. AIMS/OBJECTIVES: To compare the effectiveness of cartilage and bone pate in attic reconstruction during CWU mastoidectomy for cholesteatoma. MATERIAL AND METHODS: We conducted a retrospective study at King Abdullah University Hospital (KAUH) in Jordan, analyzing surgeries performed from 2011 to 2021. Patients who underwent CWU mastoidectomy with attic reconstruction using tragal cartilage with perichondrium or bone pate were included. RESULTS: Of 48 patients analyzed, 26 had cartilage graft attic reconstruction, and 22 received bone pate. Recurrent cholesteatoma occurred in 19.23% of the cartilage group but none in the bone pate group (p = .001). Ear discharge was observed in 19.23% of the cartilage group and 18.18% of the bone pate group, while tympanic membrane perforations and external auditory canal cholesteatoma were more prevalent in the cartilage group. CONCLUSIONS AND SIGNIFICANCE: Our study indicates that bone pate results in significantly lower cholesteatoma recurrence than cartilage grafting in CWU mastoidectomy attic reconstruction. Bone pate offers stability and favorable long-term outcomes for outer attic wall repair.

Analysis of Paparella Type 1 tympanostomy tubes in pediatric patients: A single-center retrospective review.

OBJECTIVE: This study aims to evaluate the demographic characteristics, indications for surgery, clinical follow-up results and complication rates of pediatric patients who have received a Paparella Type 1 tympanostomy tube (TT) insertion. METHODS: Retropective review of 816 ears of 442 pediatric patients who received Paparella type 1 tympanostomy tube insertions was performed. The patients' age, indication for surgery, middle ear effusion, time to extrusion and postoperative complications were analyzed retrospectively. Ears operated for chronic otitis media with effusion (COME) and recurrent acute otitis media (RAOM) were included in the study. Ears that underwent tympanostomy tube insertion for middle ear atelectasis and suppurative complications of acute otitis media were excluded from the study. Ears with middle ear effusion mucoid and serous were included. Ears without middle ear effusion or with purulent effusion were excluded from the study. Patients with a cleft palate, Down syndrome, craniofacial anomalies and those without regular follow-up until their tubes were extruded, were excluded from the study. RESULTS: The mean age of surgery was 5.11 years. 54.3 % of the patients were male and 45.7 % were female. 734 (90 %) tube insertions were performed for patients with COME and 82 (10 %) for those with RAOM. Mucoid middle ear effusion was observed in 86.9 % and serous in 13.1 %. The mean extrusion time of the tubes was 7.16 months. 93.1 % of the tubes were extruded spontaneously within 1 year and 99.9 % within 2 years. Postoperative complications of patients that were included were 8.7 % with otorrhea, 7.7 % premature extrusion, 8.2 % tube occlusion, 0.2 % displacement into the middle ear, 8.2 % tympanic membrane changes (5.4 % sclerosis, 2.3 % retraction and 0.5 % atrophy), 1.2 % permanent perforation, 0.1 % cholesteatoma and 0.1 % retained their tube. Premature extrusion was found to be significantly higher in the RAOM group compared with the COME group (p = 0.042). Tube extrusion time did not affect tympanic membrane changes (p = 0.061). CONCLUSIONS: Complication rates after Paparella Type 1 tube insertion are low. The incidence of complications such as otorrhea and tube occlusion were not significantly different between the indication and middle ear effusion groups. Compared to COME group, premature extrusion were found more frequently in the RAOM group. Complications of displacement into the middle ear, permanent perforation, cholesteatoma and retained tube were much rarer.

Diagnosis and management of bilateral lipoma of the middle ear.

INTRODUCTION: Middle ear lipomas are rare, accounting for less than 1% of all middle ear tumours. To our knowledge there have been only 16 reported patients with middle ear lipoma. Only one of these had bilateral congenital middle ear lipomas. The aim of this review is to comprehensive overview the diagnosis and management of congenital lipomas of the middle ear. METHODS: A literature review was performed searching multiple data bases, using the key words "middle ear", AND "lipoma", AND "congenital". A total of 116 articles were identified. One hundred were excluded on various criteria, leaving 16 articles for detailed review. Additionally, we report the second case of bilateral congenital middle ear lipoma. RESULTS: Based on this case report and literature review, the incidence of middle ear lipomas is greater in paediatric patients than in adult patients. Middle ear lipomas are distributed evenly between males and females. Three patients had recognised genetic syndromes, while five had prior ventilation tube placement. Clinically patients present with conductive hearing loss and middle ear effusions. In some cases, presence of a white or yellow mass behind the anterior aspect of the tympanic membrane is recognised. The most common location of the masses is in the anterosuperior quadrant of the middle ear, the site of predilection for small congenital cholesteatomas. An MRI scan is most useful in distinguishing between lipoma and cholesteatoma. All cases reported surgical excision of the middle ear lipoma. There was no evidence of recurrence noted up to 4 years follow up. CONCLUSION: Though rare, congenital lipomas of the middle ear should be a differential diagnosis of a middle ear masses. Early identification can allow surgical lipoma resection with minimal disruption to the ossicular chain. Though there is currently no consensus regarding management, surgical removal and appropriate reconstruction of the ossicular chain if needed, is appropriate.

Congenital Retrosigmoid Cholesteatoma: Case Series and Literature Review.

BACKGROUND: This study aimed to discuss 3 cases of congenital cholesteatoma located posterior to the sigmoid sinus, with no/minimal involvement of mastoid, and compare them with cases presented in the literature to better define this rare entity. METHODS: Retrospective chart analysis of 3 congenital cholesteatomas located posterior to the sigmoid sinus treated surgically in 2 skull-base centers and literature review. Though congenital cholesteatoma can arise outside the middle ear, only a few cases presenting in the retrosigmoid occipital bone have been described earlier. RESULTS: In all 3 patients, there was a delay in the presentation, as symptoms were nonspecific or lacking, leading in 1 case to severe complications. Computed tomography and magnetic resonance imaging, especially diffusion-weighted imaging scans, allowed accurate diagnosis and surgical planning. Surgery happened to be challenging due to the tight adherence of the cholesteatoma to the thinned dural surface. Complete excision was achieved in all the cases. CONCLUSION: Congenital cholesteatoma located posterior to the sigmoid sinus is a rare entity and is even more exceptional after a critical review of the literature. Complete excision is quintessential to prevent intradural extension or infection. The most important surgical issue is the management of the posterior fossa dura and the sigmoid sinus. We recommend meticulous dissection with slow peeling of the epithelial lining from the dura. Bipolar coagulation of the dura may help in avoiding recidivism. Moreover, cerebrospinal fluid (CSF) leak during dissection has to be avoided as long as possible, because the loss of tension of the already thinned dura makes its peeling particularly difficult.

Canal wall up versus canal wall down mastoidectomy techniques in the pediatric population with cholesteatoma: A systematic review and meta-analysis of comparative studies.

IMPORTANCE: The optimal surgical management of cholesteatoma remains controversial. Within pediatric otolaryngology, one of the most vital points of contention is the selection of canal wall-up (CWU) versus canal wall-down (CWD) procedures. Pediatric cholesteatoma has high rates of recurrence (16%-54%). In adults, there is evidence that the selection of surgical techniques affects recurrence rates. This has not been shown in children. OBJECTIVES: 1. To systematically review the literature on recurrent and residual cholesteatoma after CWU and CWD in children and perform a meta-analysis of the data. 2. To assess the rates of recurrent and residual cholesteatoma between CWU and CWD techniques in pediatric patients. 3. To assess hearing outcomes by evaluating postoperative differences in the air-bone gap (ABG) between CWU and CWD techniques. DATA SOURCES: A systematic search of PubMed, Embase, Scopus, and Cochrane Collaboration was performed from inception to May 1st, 2020, to identify studies that compared CWU and CWD procedures for acquired cholesteatoma in children. STUDY SELECTION: Search records were screened in duplicate by four reviewers. Inclusion criteria consisted of comparative randomized clinical trials and observational studies assessing outcomes of CWU and CWD techniques in the pediatric population. Studies involving patients with congenital cholesteatoma were excluded. DATA EXTRACTION AND SYNTHESIS: Four reviewers working independently and in duplicate systematically reviewed and extracted study data. Dichotomous variables were analyzed as risk ratios (RR), while continuous variables were compared using weighted mean differences (MD). The risk of bias was assessed using the CLARITY Scale. PRIMARY OUTCOMES AND MEASURES: The outcomes were recurrence, residual disease, air-bone gap (ABG), and air conductive (AC) thresholds. RESULTS: After screening 1036 publications, 17 retrospective cohort studies were selected. 1333 children were included; the overall mean age was ten years (SD 7.9), and the overall mean follow-up time was 5.9 years (SD 6.6). CWU and CWD techniques were performed in 60% (796) and 40% (537) cases. We did not find differences in cholesteatoma recurrence (RR: 1.50, 95% CI 0.94; 2.40; n = 544; I2 0%; Tau [2]: 0.00), or rates of residual cholesteatoma (RR 1.51, 95% CI 0.96; 2.38, n = 506; I2: 0%; Tau [2]: 0.00) in patients who underwent CWU and CWD mastoidectomy. The mean air-bone gap was lower with CWU than CWD (mean difference: 7.60, 95% CI -10.65; -4.54; n = 242; I2: 71%; Tau [2]: 5.98). CONCLUSION: and relevance: We show similar rates of recurrence and residual disease after either CWU or CWD tympanoplasty. Our results challenge the fundamental principle of CWD surgery as a standard technique, as there is no difference in rates of recurrence and residual disease in CWU and CWD. Moreover, audiometric results support CWU with improved hearing outcomes. TRIAL REGISTRATION: PROSPERO identifier: CRD42020184029.

External Ear Disease: Keratinaceous Lesions of the External Auditory Canal.

Keratosis obturans (KO) and external auditory canal cholesteatoma (EACC) are two distinct keratinaceous lesions of the external ear. This article reviews the signs, symptoms, pathophysiology, workup, and treatment of each. Patients with either pathology can often be managed in the clinic with debridement; however, EACC is more likely to involve osteonecrosis and require more extensive operative management if disease is not confined to the canal on account of the bony erosion characteristic of cholesteatoma. If required for extensive disease, surgical approaches to both pathologies are similar.

[Partial sequestration of the labyrinth in a child with chronic suppurative otitis media with cholesteatoma]. / Chastichnaya sekvestratsiya labirinta u rebenka s khronicheskim gnoinym srednim otitom s kholesteatomoi.

A clinical case of a 16-year-old child with chronic suppurative otitis media with cholesteatoma complicated by partial labyrinth sequestration is presented. The case describes is a rare disorder. Computed tomography of temporal bones consistent with intraoperative data was decisive in establishing the diagnosis and the surgery extent.

The role of planned two-stage surgery in the management of congenital cholesteatoma.

OBJECTIVE: The purpose of this study is to verify the role of "planned two-stage surgery" in the management of advanced congenital cholesteatoma regarding disease recurrence rates, complications and the need for salvage surgery. METHOD: Retrospective review of all congenital cholesteatoma under the age of 18 years underwent surgery from October 2007 to December 2021 in a single tertiary referral center. Patients with Potsic stage I/II who had closed-type congenital cholesteatoma received one-stage surgery. Advanced cases or those with open-type infiltrative congenital cholesteatomas underwent planned two-stage surgery. The second stage of surgery was performed 6-10 months after the first stage of surgery. Ossiculoplasty would be performed in the second operation if a significant air-bone gap was detected in the preoperative pure-tone audiometry test. RESULTS: Twenty-four patients were included in the series. Six patients received one-stage surgery and no recurrence was noted in this group. The remaining 18 underwent planned two-stage surgery. Residual lesions found in the second operative phase were observed in 39% of patients who received planned two-stage surgery. Except for one patient whose ossicular replacement prosthesis protruded and two patients who had perforated tympanic membranes, none of the 24 patients required salvage surgery during follow-up (mean, 77 months after surgery), and no major complications occurred. CONCLUSIONS: Planned two-stage surgery for advanced-stage or open infiltrative congenital cholesteatoma could timely detect residual lesions to avoid extensive surgery and reduce complications.