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.

Development of Air Cell System Following Canal Wall Up Mastoidectomy for Pediatric Cholesteatoma.

Background: The development of temporal bone pneumatization is related to the postnatal middle ear environment, where the development of air cells is suppressed with otitis media in early childhood. However, whether air cell formation restarts when mastoidectomy is performed during temporal bone pneumatization remains unclear. Herein, we evaluated temporal bone pneumatization after canal wall up (CWU) tympanomastoidectomy for middle ear cholesteatoma in children. Methods: In total, 63 patients, including 29 patients with congenital cholesteatoma (CC) and 34 patients with acquired cholesteatoma (AC), were assessed using a set of pre- and postoperative computed tomography images. The air cells of the temporal bone were divided into five areas: periantral (anterior), periantral (posterior), periantral (medial), peritubal, and petrous apex. The number of areas with air cells before and after surgery was compared to evaluate temporal bone pneumatization after surgery. Results: A total of 63 patients, comprising 29 with CC and 34 with AC (pars flaccida; 23, pars tensa; 7, unclassified; 4), were evaluated. The median age of patients (18 males and 11 females) with CC was 5.0 (range, 2-15 years), while that of the AC group (23 males and 11 females) was 8 (range, 2-15 years). A significant difference in air cell presence was identified in the CC and AC groups after surgery (Mann-Whitney U, p < 0.001 and p = 0.003, respectively). Between the two groups, considerably better postoperative pneumatization was observed in the CC group. A correlation between age at surgery and gain of postoperative air cell area development was identified in the CC group (Spearman's rank-order correlation coefficient, r = -0.584, p < 0.001). In comparison with the postoperative pneumatization rate of each classified area, the petrous apex area was the lowest in the CC and AC groups. Conclusions: Newly developed air cells were identified in the temporal bones after CWU mastoidectomy for pediatric cholesteatoma. These findings may justify CWU tympanomastoidectomy, at least for younger children and CC patients, who may subsequently develop air cell systems after surgery.

Canal-wall up cholesteatoma surgery with mastoid obliteration leads to lower rates of disease recurrence without affecting hearing outcomes.

Objectives: The primary objective was to determine whether obliteration of the epitympanic area and mastoid cavity during canal wall up (CWU) cholesteatoma surgery reduces the rate of recurrent and residual cholesteatoma compared to not obliterating the same area. The secondary objective was to compare postoperative hearing outcomes between both techniques. Methods: A retrospective cohort study was conducted in a tertiary referral center. One-hundred-fourty-three ears were included of patients (≥18y) who underwent a CWU tympanomastoidectomy for cholesteatoma with or without bony obliteration between January 2015 and March 2020 in the University Medical Center Utrecht. The median follow-up was respectively 1.4 (IQR 1.1-2.2) vs. 2.0 years (IQR 1.2-3.1) (p = 0.013). Interventions: All patients underwent CWU tympanomastoidectomy for cholesteatoma. For 73 ears bone dust, Bonalive® or a combination was used for obliteration of the mastoid and epitympanic area, the rest of the ears (n = 70) were not obliterated. In accordance with the Dutch protocol, included patients are planned to undergo an MRI scan with diffusion-weighted imaging (DWI) one, three and five years after surgery to detect recurrent or residual cholesteatoma. Main outcome measures: The primary outcome measure was recurrent and residual cholesteatoma as evaluated by MRI-DWI and/or micro-otoscopy and confirmed by micro-otoscopy and/or revision surgery. The secondary outcome measure was the postoperative hearing. Results: In this cohort, the group treated with canal wall up tympanomastoidectomy with subsequent bony obliteration (73 ears, 51.0%) had significantly lower recurrent (4.1%) and residual (6.8%) cholesteatoma rates than the group without obliteration (70 ears, 25.7% and 20.0%, respectively; p < 0.001). There was no significant difference between both groups in postoperative bone conduction thresholds (mean difference 2.7 dB, p = 0.221) as well as the mean air-bone gap closure 6 weeks after surgery (2.3 dB in the non-obliteration and 1.5 dB in the obliteration group, p = 0.903). Conclusions: Based on our results, a canal wall up tympanomastoidectomy with bony obliteration is the treatment of choice, since the recurrent and residual disease rate is lower compared to the group without obliteration. The bony obliteration technique does not seem to affect the perceptive or conductive hearing results, as these are similar between both groups.

Quality of life after mastoid surgery. Is there a real benefit to preserving the posterior wall of the external auditory canal? A systematic review and meta-analysis.

OBJECTIVE: Canal wall up (CWU) and canal wall down (CWD) mastoidectomies represent the most common cholesteatoma surgical techniques. In this meta-analysis, we compare the postoperative quality of life (QoL) in patients treated with either CWU or CWD mastoidectomy. METHODS: A literature search was conducted in the following three electronic databases: Medline/PubMed, ScienceDirect, and Google Scholar. Articles were assessed for eligibility in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement and data were extracted independently by two authors. Biases assessment was conducted for each study according to the Methodological Items for Non-Randomized Studies (MINORS) tool. Meta-analysis was performed for postoperative QoL following CWU versus CWD mastoidectomy. RESULTS: Our systematic review included four studies that met the inclusion criteria, three prospective cohort studies, and one retrospective cohort study. The meta-analysis did not favor treatment with one of the two surgical techniques. Postoperative QoL did not show a statistically significant difference between CWU and CWD mastoidectomies (p>0.05). CONCLUSION: Our systematic review and meta-analysis results indicate that QoL is not statistically significantly better among patients who underwent CWU mastoidectomies when compared with CWD. The trend of selecting CWU over CWD mastoidectomies in selected cases - for QoL purposes - is not always based on evidence-based data. The statistically insignificant difference between the two surgical techniques suggests that an initial more radical approach might prevent patients from further surgeries, without affecting postoperative QoL.

Efficiency of intraoperative endoscopic inspection in reducing residuals in canal-wall-up surgery for pediatric cholesteatoma involving the mastoid.

OBJECTIVE: To investigate the efficiency of additional intraoperative endoscopic inspection in reducing residual cholesteatoma in pediatric cholesteatoma involving the mastoid treated with classic canal-wall-up mastoidectomy and tympanoplasty. MATERIALS AND METHODS: 32 cases of pediatric cholesteatoma involving the mastoid were enrolled in this perspective study and treated with classic canal-wall-up mastoidectomy and tympanoplasty. Transmastoid posterior tympanotomy, atticotomy and transecting tendon of tympani tensor were conducted to achieve adequate visualization of hidden spaces in the middle ear. After complete removal of cholesteatoma, endoscopic inspection was additionally performed to check residual cholesteatoma. All cases had at least a 2-year follow-up by routine otoscopy examination, CT scan or MR imaging. Residual rates of both intraoperative and follow-up findings were used to evaluate the efficiency of the endoscopic inspection in reducing residual cholesteatoma and compared with published reports. RESULTS: The additional intraoperative endoscopic inspection did not find any residual in this case series. In the 2-year follow-up, 2 cases (2/32, 6.3%) with residual cholesteatoma and 3 cases with recurrence (3/32, 9.4%) were found. The mean duration of endoscopic inspection and microscopic procedure were 17.9 min and 93.6 min, respectively. CONCLUSIONS: This study suggested that the additional intraoperative endoscopic inspection in microscopic CWU surgery for pediatric cholesteatoma involving the mastoid had no obvious value in reducing residual cholesteatoma but took extra time.

Mastoid obliteration versus canal wall down or canal wall up mastoidectomy for cholesteatoma: Systematic review and meta-analysis.

BACKGROUND: There is ongoing debate over the optimal surgical approach for cholesteatoma treatment1-5. The aim of this review was to determine whether canal-wall down with mastoid obliteration is associated with favourable outcomes compared to either canal-wall down without obliteration or canal-wall up. The primary outcome was cholesteatoma reoccurrence with secondary outcomes including otorrhoea, reoperation and patient-reported outcome measures. METHODS: Medline, Embase, Cochrane databases and clinicaltrials.gov were searched for studies reporting outcomes of patients undergoing primary CWD-MO for cholesteatoma and directly compared with CWD and CWU mastoidectomies. Data were collated and meta-analysis performed. RESULTS: 2379 patients were included from 12 studies that met the inclusion criteria. CWD-MO was found to be associated with significantly less risk of recurrence when compared to CWU (OR = 0.330 (95 % CI 0.191-0.570, p < 0.001). When compared to CWD, CWD-MO was associate with significantly lower incidence of persistent otorrhoea (OR 0.405 (95 % CI 0.232-0.706, p < 0.001) while the rates of recurrence were comparable (OR: 1.231 [95 % CI: 0.550-2.757] p = 0.613). Otorrhoea rates between CWD-MO and CWU were equivalent as was reoperation. Quality of life and hearing outcomes were variably reported. CONCLUSIONS: This systematic review demonstrates that CWD-MO technique achieves lower recurrence rates comparable to CWD approaches and superior to CWU, while offering improved long-term control of otorrhoea. The final procedural choice should be decided based on surgeon experience, skill set and individual patient factors. To address the risk of selection bias, a randomised controlled trial is needed to answer this important research question.

Change in chorda tympani nerve function after two-stage tympanoplasty for cholesteatoma.

Objectives: Chorda tympani nerve (CTN) function may be damaged more by two-stage than by one-stage surgery for middle ear cholesteatoma. However, few studies have reported the relationship between two-stage cholesteatoma surgery and CTN function. This study aimed to investigate CTN function after two-stage surgery for cholesteatoma. Methods: In this prospective study, 35 patients underwent two-stage canal wall up tympanoplasty (CWUT). Perioperative CTN function was assessed using questionnaires and electrogustometry (EGM). Participants were categorized into minor, major, and section groups, based on the degree of CTN manipulation during surgery. Results: In the first-stage surgery, posterior tympanotomy with an intact canal wall reduced the degree of CTN manipulation. The incidence of taste disorder after the first-stage surgery was 71.4%. Postoperative taste disorder and the EGM threshold improved early in the minor manipulation group. In the second-stage surgery, no new CTN damage occurred, even if this surgery involved removal of residual cholesteatoma. The incidence of taste disorder after second-stage surgery was less than that after first-stage surgery, independent of CTN preservation. However, the recovery rate of the EGM threshold after second-stage surgery was significantly lower in the section group than in those with CTN preservation. Conclusion: CTN function, including symptoms and EGM threshold, can be preserved during two-stage cholesteatoma surgery if care is taken to preserve the CTN in both the first- and second-stage surgeries. A two-stage CWUT, ensuring an intact bony annulus, may be effective to facilitate CTN preservation. Level of Evidence: 2b.

Mastoid obliteration and external auditory canal reconstruction using 3D printed bioactive glass S53P4 /polycaprolactone scaffold loaded with bone morphogenetic protein-2: A simulation clinical study in rabbits.

Introduction: The lack of good prosthetic materials and objective standards has limited the promotion of mastoid obliteration and external auditory canal reconstruction, and the quality of the surgery varies. In this study, bioactive glass S53P4 (S53P4), the most popular artificial prosthetic material, was modified and combined with polycaprolactone (PCL) and bone morphogenetic protein-2 (BMP-2) to produce an individualized biological scaffold using 3D printing technology to explore a better material and method for mastoid obliteration and external auditory canal reconstruction. Methods: 3D-printed S53P4/PCL scaffolds were fabricated from 3D reconstruction data of bone defect areas in New Zealand rabbits simulating "Canal Wall Down Mastoidectomy". The water absorption, swelling rate, porosity, and Young's modulus of the scaffold were measured, and the morphology and pore size of the scaffold were observed using scanning electron microscopy. The cytotoxicity of the S53P4/PCL scaffolds was detected using the CCK8 assay, and the in vitro antibacterial activity of the S53P4/PCL scaffolds was detected using the inhibition circle method. The BMP-2-loaded S53P4/PCL scaffolds were prepared using the drop-in lyophilization method and implanted into animal models. The biocompatibility, osteogenic activity, and external auditory canal repair of the scaffolds were observed using endoscopy, micro-CT, and histological examination. Results: The S53P4/PCL scaffold was highly compatible with the defective area of the animal model, and its physicochemical properties met the requirements of bone tissue engineering. In vitro experiments showed that the S53P4/PCL scaffold was non-cytotoxic and exhibited better antibacterial activity than the same volume of the S53P4 powder. In vivo experiments showed that the S53P4/PCL scaffold had good biocompatibility and osteogenic activity, and could effectively repair bone defects and reconstruct the normal morphology of the external auditory canal in animal models. Furthermore, its osteogenic activity and repair ability were significantly improved after loading with BMP-2. Conclusions: The 3D printed S53P4/PCL scaffold has great potential for clinical mastoid obliteration and external auditory canal reconstruction.

Ears with Cholesteatoma: Outcomes of Canal Wall Up and Down Tympano-Mastoidectomies-A Comparative Prospective Study.

This prospective study has been carried out to observe the outcomes of canal wall up (CWU) and canal wall down (CWD) Tympano-mastoidectomies in ears with cholesteatoma. Outcomes of the procedures have been done in terms of recurrence of cholesteatoma, complications, graft uptake rates and post-operative hearing gain. This study was carried out in the Department of Otorhinolaryngology of a reputed tertiary teaching hospital of North India from January 2016 to June 2020, with a mean follow-up of 32 months. The study included 100 patients of otitis media with cholesteatoma. In Canal Wall Up Tympano-mastoidectomy (CWUT) group, the number of males and females were 22 each. On the other hand, 36 males and 20 females underwent Canal Wall Down Tympano-mastoidectomy (CWDT). Each surgery was done as a single-staged and at the end of the surgical procedure angled oto-endoscopes were used for ensuring complete removal of the disease. Though there is no statistically significant difference in recurrence of cholesteatoma and complications rate in CWU and CWD Tympano-mastoidectomy techniques, but CWUT is superior to CWDT in terms of better graft uptake (p = 0.0156), and better average audiological gain (8.56 ± 0.93 dB) with p value = 0.0315. A diligent post-operative follow-up is must for assessing the outcomes of different types of mastoidectomies. This study shows no statistical difference in the disease recurrence and complication rates between Canal Wall Up and Down Tympano-mastoidectomies, though the anatomical changes and creation of the mastoid cavity in canal wall down procedure may affect the graft uptake and post-operative hearing gain. Use of Oto-endoscopes in cholesteatoma surgery helps in improving the outcomes of both techniques and thus recommended if the facility for the same is available.

Hearing improvement among children with cholesteatoma who underwent canal wall up and canal wall down surgical management.

Objectives: The most frequently used surgical methods for treating cholesteatoma include canal wall up and canal wall down procedures. The objective of the study was to compare the hearing improvement among children with cholesteatoma who underwent canal wall up and canal wall down surgical management. Methods: The cross-sectional analytical study design was used. The study was conducted at the ENT Department of Nishtar Medical University & Hospital Multan from 15th June to 15th Nov 2020.. Forty six patients with cholesteatoma were enrolled in the study after taking informed consent. Inclusion and exclusion criteria were followed. The participants were categorized into two groups. Group-A was treated with canal wall-up surgery while Group-B was treated with canal wall down Mastoidectomy. A 12-month post-operative follow-up and the audiometry assessment were compared with pre-surgical values. Additionally, a COMOT-15 survey was administered to analyze self-perceived hearing functions. The Chi-square test was used for comparative analysis of the surgical outcome and hearing improvement among the two groups. P-value (p value<0.05) was considered statistically significant. Results: Forty six patients were included in the study with 23 participants in each group. Among 46, 26 were male and 20 were female. The pre and post-operative mean Pure-tone average values were significantly different in (Group-A) who underwent canal wall up Mastoidectomy (p<0.05) than in Group-B, who underwent canal wall down Mastoidectomy. Similarly, hearing sub-section responses of the COMOT-15 survey favored the Canal wall technique. However, the survey showed no significant differences in the mental health status of the two groups (p<0.05). Conclusion: Our data collected after a one-year follow-up of patients suggests canal wall up as a preferred technique for hearing improvement than canal wall down technique.

Cholesteatoma: Influence of surgical technique and EAONO/JOS stage on audiological results.

INTRODUCTION AND OBJECTIVE: The treatment of cholesteatoma is surgical in most cases. When it is indicated, it is preferable to choose a reconstructive surgical technique with the dual purpose of eradicating the disease and preserving or improving the patient's hearing. In 2017, the European Academy of Otology and Neuro-Otology/Japanese Otological Society (EAONO/JOS) published a new cholesteatoma classification. The aims of this study were to determine the influence of the surgical technique used and this classification on patient's hearing outcomes. METHODS: A retrospective study that included patients who underwent reconstructive surgery of cholesteatoma between 2012 and 2017 was carried out. Based on pre-surgical computed tomography (CT) images, disease was staged according to the EAONO/JOS classification. Hearing outcomes obtained by pre and postoperative pure tone audiometry were analysed according to the surgical technique used and according to the stage of the disease. RESULTS: 143 patients with no statistically significant differences in hearing thresholds before surgery were included. One year after surgery, all the patients' (P = .01 and P = .001) airpure tone average (PTA) and mean differential auditory threshold had improved significantly. Those patients who underwent tympanoplasty with two-stage canal wall up mastoidectomy presented better postsurgical air PTA and postsurgical mean differential auditory threshold outcomes (P = .007 and P = .014) than those patients who underwent tympanoplasty with canal wall down mastoidectomy. Moreover, the patients who underwent tympanoplasty with two-stage canal wall up mastoidectomy had improved air PTA and mean differential auditory threshold one year after the surgery with statistical significance (P = .001, P = .013). The mean differential auditory threshold was also better (P = .008) in the patients who underwent tympanoplasty with canal wall down mastoidectomy one year after the procedure. CONCLUSIONS: Reconstructive surgical techniques improve hearing one year after surgery. In our study, this improvement was significantly greater with tympanoplasty with two-stage canal wall up mastoidectomy.

Use of bone pate and bioactive glass granules for mastoid obliteration in cholesteatoma surgery / Mastoidobliterációhoz használt csontpor és bioaktív üveggranulátum a cholesteatoma sebészetében

Introduction and objective: Mastoid obliteration technique combines the advantages of canal wall-up (CWU) and canal wall-down (CWD) approaches in the surgery of chronic suppurative otitis media with cholesteatoma. We aim to demonstrate our experience with mastoid obliteration technique using bone dust and BonAlive® (S53P4) bioactive glass granule in a comparative prospective clinical study. Patients and methods: Between 1st of March 2012 and 31st of November 2021, mastoid obliteration surgery was performed in 14 patients using bone dust (7 cases) and BonAlive® granule (7 cases). Prior to these interventions, the patients had undergone more than three ear surgeries (CWU and CWD) generally in both groups. Changes of complaints, audiological results, and changes in quality of life were analysed in both groups, postoperatively. Results: Having performed the mastoid obliteration technique, cochlear damage did not occur in either patient group. Long-term ear discharge and vertigo were occasionally observed after performing obliteration with bone dust. However, these complaints disappeared after a while. Complications were not reported in the case of obliteration with BonAlive®. Outstanding improvement was experienced in both groups. Conclusion: In our practice, mastoid obliteration surgery, using either bone dust or BonAlive® granule, has proved to

Colesteatoma: influencia de la técnica quirúrgica y el estadio EAONO/JOS en los resultados audiológicos / Cholesteatoma: Influence of surgical technique and EAONO/JOS stage on audiological results

Antecedentes y objetivo: El tratamiento del colesteatoma es quirúrgico en la mayoría de los casos. Cuando está indicado, es preferible optar por una técnica quirúrgica reconstructiva con el doble propósito de eliminar la enfermedad y preservar o mejorar la audición del paciente. En 2017 se publicó una nueva clasificación del colesteatoma por parte de la European Academy of Otology and Neuro-Otology/Japanese Otological Society (EAONO/JOS). El objetivo de este estudio es determinar la influencia que tanto la técnica quirúrgica empleada como la clasificación de la enfermedad tienen en los resultados auditivos de los pacientes. Materiales y métodos: Se realiza un estudio retrospectivo que incluye pacientes intervenidos de colesteatoma mediante una técnica reconstructiva entre 2012 y 2017. A partir de la tomografía computarizada (TC) prequirúrgica de cada paciente se estadifica la enfermedad según la clasificación de la EAONO/JOS. Los datos audiológicos obtenidos mediante audiometría tonal liminar (ATL) pre y postoperatoria son analizados en función de la técnica quirúrgica empleada y según el estadio de la enfermedad. Resultados: Se incluyen 143 pacientes con umbrales auditivos sin diferencias significativas antes de la cirugía. Al año de la intervención, el umbral medio auditivo (pure tone average [PTA]) aéreo y el umbral diferencial auditivo (UDA) medio mejoran significativamente (p=0,01 y p=0,001) en toda la muestra. Los pacientes intervenidos con técnica cerrada en dos tiempos presentan mejores resultados en el PTA aéreo postoperatorio y UDA medio posquirúrgico que los operados mediante técnica abierta (p=0,007 y p=0,014). El PTA aéreo postoperatorio y el UDA medio postoperatorio mejoran en los pacientes intervenidos con técnica cerrada en dos tiempos quirúrgicos (p=0,001 y p=0,013), en comparación con la audición previa que presentaban. El UDA medio postoperatorio también mejora significativamente en los pacientes operados mediante técnica abierta (p=0,008). Conclusiones: Todas las técnicas quirúrgicas reconstructivas producen una mejora significativa de la audición al año de la intervención. En nuestro estudio esta mejora es significativamente superior con una timpanoplastia con mastoidectomía cerrada en dos tiempos quirúrgicos.(AU)

Introduction and objective: The treatment of cholesteatoma is surgical in most cases. When it is indicated, it is preferable to choose a reconstructive surgical technique with the dual purpose of eradicating the disease and preserving or improving the patient's hearing. In 2017, the European Academy of Otology and Neuro-Otology/Japanese Otological Society (EAONO/JOS) published a new cholesteatoma classification. The aims of this study are to determine the influence of the surgical technique use and this classification on patient's hearing outcomes. Methods: A retrospective study that include patients who underwent reconstructive surgery of cholesteatoma between 2012 and 2017 is carried out. Based on pre-surgical computed tomography (CT) images, disease is staged according to the EAONO/JOS classification. Hearing outcomes obtained by pre and postoperative pure tone audiometry are analysed according to the surgical technique used and according to the stage of the disease. Results: 143 patients with no statistically significant differences in hearing thresholds before surgery are included. One year after surgery, all the patients’ airbone pure tone average (PTA) and mean differential auditory threshold have improved significantly (P = .01 and P = .001). Those patients who undergo tympanoplasty with two-stage canal wall up mastoidectomy presente better postsurgical air PTA and postsurgical mean differential auditory threshold outcomes (P=.007 and P=.014) than those patients who underwent tympanoplasty with canal wall down mastoidectomy. Moreover, the patients who underwent tympanoplasty with two-stage canal wall up mastoidectomy had improved airbone PTA and mean differential auditory threshold one year after the surgery with statistical significance (P=.001 and P=.013). The mean differential auditory threshold is also better (P=.008) in the patients who undergo tympanoplasty with canal wall down mastoidectomy one year after the procedure. Conclusions: Reconstructive surgical techniques improve hearing one year after surgery. In our study, this improvement is significantly greater with tympanoplasty with two-stage canal wall up mastoidectomy.(AU)

Prevalence and surgical management of cholesteatoma in Down Syndrome children.

OBJECTIVE: The objective was to report the outcomes of surgical management of cholesteatoma in Down Syndrome (DS) children and to determine the prevalence of cholesteatoma in DS children at three academic tertiary care centers. METHODS: A retrospective chart review was performed of all DS children diagnosed with cholesteatoma from three tertiary care academic centers. Demographic data, comorbidities, imaging findings, surgical management, and pre- and postoperative pure tone averages were collected. RESULTS: The prevalence of cholesteatoma in DS patients at these three institutions was calculated to be 8/802 (0.998%), 4/448 (0.893%), and 4/1016 (0.394%), with an average prevalence of 0.762%. Of the 16 patients with cholesteatoma identified, the average age of diagnosis was 10.8 years, and 5/16 (31%) had at least three sets of pressure equalizing tubes (PETs) placed. Of the 13 patients with preoperative CTs, 4/13 (30.8%) had blunting of the scutum, 4/13 (30.8%) had tegmen involvement, 1/13 (7.7%) had erosion of the semicircular canal, 2/13 had facial nerve dehiscence, and 7/13 (53.8%) had ossicular chain erosion. For surgical management, disease control was attained in 4/16 children with tympanoplasty only and canal wall up (CWU) tympanomastoidectomy in 11/16 patients. Available pre- and postoperative audiograms were evaluated; however, there was insufficient audiological data to contribute to outcomes. CONCLUSION: Cholesteatoma occurs in nearly 1% of DS patients, and the disease tends to be advanced with tegmen involvement in nearly a third of patients, and ossicular erosion seen in more than half the patients. A CWU procedure can be successful in managing DS patients with cholesteatoma when consistent long term follow-up is planned. Close monitoring and a high index of suspicion for cholesteatoma are essential to prevent delays in diagnosis and treatment of DS patients with a history of multiple sets of PET placement.

A retrospective analysis of hearing after cholesteatoma surgery: the bony obliteration tympanoplasty versus canal wall up and canal wall down without mastoid obliteration.

OBJECTIVE: To evaluate and compare the hearing outcome after the bony obliteration tympanoplasty (BOT), canal wall up (CWU) without mastoid obliteration and canal wall down (CWD) without mastoid obliteration in a large patient cohort. As the aeration of the middle ear is associated with hearing outcome, we hypothesized that the post-operative hearing after the BOT may be better compared to CWU and CWD without obliteration. METHODS: This is a retrospective cohort study on all adult patients who underwent the BOT, CWU without obliteration or CWD without obliteration for primary or revision cholesteatoma between January 2003 and March 2019 with audiological follow-up at our institution. Pre-operative, short-term post-operative and long-term post-operative hearing tests were analyzed and potential factors influencing post-operative hearing were assessed. RESULTS: 626 ears were included. We found no significant differences between the short-term and long-term post-operative audiometry. The pre-operative air-bone gap (ABG) was the factor with the largest effect size on change in air-bone gap (ABG) between pre- and post-operative. When stratifying for this factor along with the type of ossicular chain reconstruction to account for differences at baseline, no significant differences in post-operative ABG were found between BOT and non-obliteration CWU and CWD. CONCLUSION: In this large retrospective cohort study, we found no significant differences in post-operative ABG between the BOT and the non-obliteration CWU and CWD. A solid comparison of hearing between groups remains very challenging as hearing outcome seems to be dependent on many different factors. Hearing outcome seems to be no additional argument to choose for BOT over non-obliteration surgery.

Cost-effectiveness of Canal Wall-Up vs Canal Wall-Down Mastoidectomy: A Modeling Study.

OBJECTIVE: To assess the relative lifetime costs, benefits, and cost-effectiveness between the 2 approaches, canal wall-up (CWU) and canal wall-down (CWD) tympanomastoidectomy, used in the treatment of cholesteatomas. STUDY DESIGN: Markov state transition model. SETTING: Tertiary academic health system. METHODS: A Markov state transition model was used to simulate outcomes across the patient lifetime. Outcome and complication probabilities were obtained from the existing literature. Costs were calculated from the payer perspective, with procedure, hospital, clinic, and physician cost derived from Medicare reimbursement. Quality-adjusted life years (QALYs) were used to represent effectiveness and utility. One-way and probability sensitivity analyses (PSAs) were conducted. RESULTS: The base case analysis, assuming a 40-year-old patient, yielded a lifetime cost of $14,214 for a patient treated with the CWU approach assuming second-look surgery and $22,290 with a CWD approach. CWU and CWD generated a benefit of 17.11 and 17.30 QALYs, respectively. The incremental cost-effectiveness ratio for CWU was $43,237 per QALY. The Monte Carlo PSA validated the base case scenario. Using a standard $50,000 willingness-to-pay threshold, CWD was the more cost-effective approach and was selected 54.8% of the time by the simulation. CONCLUSION: Both CWU and CWD were found to be cost-effective, with CWD being cost-effective 54.8% of the time at a WTP threshold of $50,000. The assumptions used in the analysis were validated by the results of 1-way and PSA.

Silastic sheeting in staged ear surgery: Is there still a role for this procedure?

OBJECTIVE: To review long-term outcomes for chronic otitis media with and without cholesteatoma in staged canal-wall-up tympanoplasty with temporary silastic sheeting and to compare hearing and recurrence results with the literature. METHODS: Retrospective data analysis of all patients suffering from chronic otitis media with or without cholesteatoma (COMC/COM) and treated by staged canal-wall-up (CWU) technique with silastic insertion between 1992 and 2012. Literature analysis in PubMed 1990-2017. RESULTS: 74 cases were included in the analysis. In COMC (n = 47) a total of 2 (4%) recurrent and 14 (30%) residual cholesteatoma were documented. The postoperative hearing test showed a pure-tone-average (PTA) of 36 dB hearing level (HL) and an air-bone-gap (ABG) of 21 dB HL. A significant improvement was only observed for stage I disease (PTA 8 dB HL and ABG 9 dB HL). In COM (n = 27) postoperative PTA and ABG were significantly improved by 33 dB HL and 23 dB HL, respectively. Mean postoperative follow-up was 47 months (12-173) for COMC and 22 months (2-120) for COM. CONCLUSIONS: The cholesteatoma recurrence rate in this study reflects contemporary published rates. Assessment of hearing outcome is difficult due to the low number of cases and very high heterogeneity of published data. Still, the staged CWU procedure with temporary silastic sheeting seems to bear some advantages in regard to hearing. The role of additional factors such as Eustachian Tube function to assess outcome should be considered. An internationally agreed upon reporting system should be followed, if various surgical approaches are to be compared. LEVEL OF EVIDENCE: 3.

Long term outcomes of canal wall up and canal wall down tympanomastoidectomies in pediatric cholesteatoma.

Cholesteatomas in children have a more aggressive growth pattern compared to adults, which leads to a higher incidence of both residual and recurrent disease. A staged canal wall-up or a canal wall-down tympanomastoidectomy (CWUT and CWDT, respectively) is selected depending on the extent of the disease and condition of the middle ear (ME) cleft and mastoid. Endoscopic ear surgery (EES) has been recently introduced as an adjuvant tool for the treatment of this pathology even in the pediatric population. OBJECTIVES: To analyze long term outcomes of CWUT and CWDT in the pediatric population, focusing on residual and recurrence rates of cholesteatoma and hearing results. A literature review including cases treated with EES were discussed. MATERIAL AND METHODS: Pediatric patients treated for cholesteatoma involving both the ME and mastoid with a follow-up (FU) of at least 4 years were retrospectively analyzed in a quaternary referral center for otology and lateral skull base surgery. Patients were grouped according to the surgical technique (CWUT versus CWDT). Rates of residual and recurrent cholesteatoma after each surgical technique were reported and compared. Mean Air-Bone Gap (ABG) of 0.5-1-2-4 KHz was measured and reported before the first surgery and at the last post-operative FU. RESULTS: Two-hundred and thirty-six cases fulfilled our inclusion criteria. The mean FU was 100.4 ± 44.2 months (median 89 months). One-hundred and five (44.5%) cases underwent a CWUT, whereas 131 (55.5%) a CWDT. A second stage surgery was performed in 73.5% of CWUT and 58.7% of CWDT. Among the CWUT group, residual cholesteatoma occurred in 22 (21%) ears and recurrence in 24 (22.9%). Patients undergoing CWDT showed lower rates of both residual and recurrent cholesteatoma (7.6% and 2.3%, respectively). ABG improvement was noted for both groups, even though CWUT showed better post-operative hearing results. CONCLUSIONS: The CWDT technique offers a definite surgical therapy, with minimal residual and recurrence rates and audiological results comparable to the CWUT technique. EES must still prove its added benefit or equivalence to pure microscopic approaches.

Cholesteatoma: Influence of surgical technique and EAONO/JOS stage on audiological results. / Colesteatoma: influencia de la técnica quirúrgica y el estadio EAONO/JOS en los resultados audiológicos.

INTRODUCTION AND OBJECTIVE: The treatment of cholesteatoma is surgical in most cases. When it is indicated, it is preferable to choose a reconstructive surgical technique with the dual purpose of eradicating the disease and preserving or improving the patient's hearing. In 2017, the European Academy of Otology and Neuro-Otology/Japanese Otological Society (EAONO/JOS) published a new cholesteatoma classification. The aims of this study are to determine the influence of the surgical technique use and this classification on patient's hearing outcomes. METHODS: A retrospective study that include patients who underwent reconstructive surgery of cholesteatoma between 2012 and 2017 is carried out. Based on pre-surgical computed tomography (CT) images, disease is staged according to the EAONO/JOS classification. Hearing outcomes obtained by pre and postoperative pure tone audiometry are analysed according to the surgical technique used and according to the stage of the disease. RESULTS: 143 patients with no statistically significant differences in hearing thresholds before surgery are included. One year after surgery, all the patients' airbone pure tone average (PTA) and mean differential auditory threshold have improved significantly (P = .01 and P = .001). Those patients who undergo tympanoplasty with two-stage canal wall up mastoidectomy presente better postsurgical air PTA and postsurgical mean differential auditory threshold outcomes (P=.007 and P=.014) than those patients who underwent tympanoplasty with canal wall down mastoidectomy. Moreover, the patients who underwent tympanoplasty with two-stage canal wall up mastoidectomy had improved airbone PTA and mean differential auditory threshold one year after the surgery with statistical significance (P=.001 and P=.013). The mean differential auditory threshold is also better (P=.008) in the patients who undergo tympanoplasty with canal wall down mastoidectomy one year after the procedure. CONCLUSIONS: Reconstructive surgical techniques improve hearing one year after surgery. In our study, this improvement is significantly greater with tympanoplasty with two-stage canal wall up mastoidectomy.

[Clinical analysis of 21 cases of cholesteatoma recidivism after canal-wall-up mastoidectomy with tympanoplasty].

Objective:To discuss the possible reasons for cholesteatoma recidivism after canal-wall-up mastoidectomy with tympanoplasty by analyzing clinical characteristics of patients. Methods:Data of 21 cases who suffered from cholesteatoma recidivism after canal-wall-up surgery were retrospectively reviewed, including preoperative examination, high resolution temporal bone CT, and intraoperative findings. Results:90.5%（19/21） cases had recurrent cholesteatoma with retraction pockets. Among 12 cases with previous operative notes, 66.7%（8/12） had extensive cholesteatoma which was not limited to attic in the original surgery. The intraoperative features of revision surgery in 21 patients including the destruction of reconstructive lateral attic wall and scutumwere found in 19.0%（4/21） cases, the head of malleus left in 19.0%（4/21） cases, the cholesteatoma found in hidden part in 14.3%（3/21） cases, the hadeustachian tube dysfunction in 38.1%（8/21）cases. the sclerotic mastoid in 42.9%（9/21） cases. hadanatomic variations of the temporal bone in 14.3%（3/21） cases and atresia of external auditory canal in 4.8%（1/21） cases. Conclusion:In this group of recidivism cases, most patients had extensive cholesteatoma, which may lead to excessive mucosa loss during lesion clearance, poor ventilation of tympanic isthmus after surgery, and promote the formation of retraction pocket. In addition, some cases had eustachian tube dysfunction, unstable reconstruction of attic lateral wall, and improper selection of the indications, which may also increase the risk of recurrence. Therefore, in order to reduce cholesteatoma recidivism after canal-wall-up surgery, attention should be paid to the striction of surgical indications, comprehensive preoperative evaluation, thorough clearance of lesions and firm reconstruction.