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Hypothesis: To evaluate the effectiveness of the menin-MLL inhibitor, MI503, as a conservative treatment of middle ear cholesteatoma (cholesteatoma) in a mouse model and to confirm its safety profile regarding auditory function in vivo., Background: Cholesteatoma is a mass formed by the keratinizing squamous epithelium in the tympanic cavity and/or mastoid and subepithelial connective tissue and by the progressive accumulation of keratin debris with/without a surrounding inflammatory reaction. Although the main treatment is surgical therapy, the techniques to prevent recurrence remain a critical area of research. Recently, the use of MI503 in experiments resulted in the inhibition of the growth of cholesteatoma in vivo under histone modification., Methods: After cholesteatoma was induced in ICR mice (n = 7) by keratinocyte growth factor expression vector transfection, MI503 (50 μM) or phosphate-buffered saline was topically injected for 14 days. The effects of MI503 against cholesteatoma were analyzed by micro-computed tomography images. For the in vivo ototoxicity study, a single intratympanic injection of MI503 (50 or 500 μM) or phosphate-buffered saline (n = 4 each) was done in the ICR mice. An auditory brainstem response was performed at days 0, 1, and 14. For morphological analysis, immunostaining for Phalloidin/F-actin and Myo7a was performed., Results: MI503 reduced keratinocyte growth factor-induced cholesteatoma in vivo (4 of 4 [100%]). No difference was found in the mean variation of the average of the auditory brainstem response thresholds between the three groups in the in vivo ototoxicity study, thus confirming its safety profile regarding auditory function. MI503 does not demonstrate any deleterious effects on murine hair cells when assessed by immunostaining., Conclusion: These findings demonstrate an encouraging safety profile for the use of menin-MLL inhibitor for the conservative treatment of cholesteatoma., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Background: Intratympanic membrane cholesteatoma presents as an asymptomatic, white, round mass on the tympanic membrane, and is usually detected incidentally in children., Case Presentation: A 12-year-old Korean boy visited our otorhinolaryngology clinic for a whitish mass on the right tympanic membrane. He had a history of traumatic tympanic membrane perforation in the right ear that had occurred 1 year prior, which had healed well with a paper patch placement. The mass was completely removed under local anesthesia during surgery with a microscope. The mass was on the outer epithelial layer of the right tympanic membrane and did not invade the middle fibrous and inner mucosal layers. Cholesteatoma was diagnosed on the basis of histopathology., Conclusion: Intratympanic membrane cholesteatoma may not induce symptoms or invade the middle ear because it can grow outwards into the external auditory canal. However, intratympanic membrane cholesteatoma can grow over time, and then after growth, it can compress the tympanic membrane and advance into the middle ear, which can cause symptoms such as hearing loss. Intratympanic membrane cholesteatoma in children should be carefully evaluated and followed, and surgical removal should be considered, even for asymptomatic cases, to minimize potential damage and hearing loss., (© 2023. The Author(s).)

Purpose: To relate the creation and expert validation (face and content validity) of an affordable three-dimensional (3-D) printed model of temporal bones with chronic otitis media with cholesteatoma (COMC) as a simulator for mastoidectomy., Methods: We performed computed tomography (CT) of the temporal bones of a patient with COMC followed at the University of São Paulo (USP) Hospital with 3-D Slicer to create a 3-D model of the affected bone using light-curing resin and silicone (cholesteatoma). The final 3-D printed images were scored by 10 otologists using a customized version of the Michigan Standard Simulation Scale Experience (MiSSES). Internal consistency and inter-rater reliability were assessed using Cronbach's α and intraclass correlations., Results: Otologists consistently scored the model positively for fidelity, educational value, reactions, and the overall model quality. Nine otologists agreed that the model was a good educational device for surgical training of COMC. All experts deemed the model ready-or nearly ready-for use. The final cost of the model, including raw materials and manufacturing, was 120 USD., Conclusions: Using 3-D printing technology, we created the first anatomically accurate, low-cost, disease-reproducing 3-D model of temporal bones for mastoidectomy training for cholesteatoma., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: This study aimed to investigate the variability of the retrotympanum in patients undergoing surgical treatment for cholesteatoma., Methods: We included 59 ears of patients undergoing middle ear surgery for cholesteatoma who had preoperative computed tomography scans. A retrospective analysis of the medical records was conducted. The sinus tympani (ST), subtympanic sinus (STS) and facial recess (FR) were classified into types A-C based on the relationship of their extension to the facial nerve. The mastoid and petrous apex were assessed and categorized as normal pneumatized or sclerotic., Results: Type A extension was the most frequently found in all sinuses (ST 64%, FR 77%, STS 69%), Type B extension was found more often in ST (34%) and STS (24%) than in FR (15%). A very deep extension was found only rarely (ST 2%, FR 8%, STS 7%). A sclerotic mastoid was found in 67% of cases. Those cases showed a statistically significant difference regarding retrotympanum pneumatization when compared with normal mastoid., Conclusion: The most frequent variant of retrotympanic pneumatization in relation to the facial nerve was type A in all subsites in cholesteatoma patients. The variability among patients with cholesteatoma is different to previously published results in healthy subjects. Moreover, the pneumatization of the retrotympanum is associated with mastoid pneumatization., (© 2022. The Author(s).)

The article describes clinical cases of invasive cholesteatoma of the temporal bone pyramid, leading to a massive destruction of the surrounding structures. Paying attention to the importance of knowing the microsurgical anatomy of critical structures of temporal bone (the anterior bony plate (cog), the tensor tympani fold and the tendon, the tympanic orifice of the eustachian tube, the pretympanic recess, tympanic sinuses) bearing a significance and helping to improve the removal of the invasive cholesteatoma.

Purpose: Few data are available regarding subjective complaints and quality of life (QoL) after subtotal petrosectomy (STP). The purpose of our study was to assess long-term surgical results after STP, and to evaluate disease-specific, patient-reported outcomes including QoL and subjective hearing., Methods: A retrospective cohort study, including a postal survey, was performed in the Amsterdam University Medical Centers (Amsterdam UMC) location Academic Medical Centre (AMC). All patients who underwent a STP between 1990 and 2018 were included. Patient characteristics, indication for surgery, adverse events, disease recidivism, and patient-reported health outcomes were evaluated., Results: 181 patients (183 ears) underwent a STP for several indications. The main indication was chronic otitis media (COM) with or without cholesteatoma (69%). In the total cohort, 8 residual cholesteatoma (5%) and 6 inclusion cholesteatoma 4% were detected. Postoperative (functional) health outcomes showed a significant negative impact on QoL in the STP cohort compared to normative data. Compared to patients without ear problems, the postoperative STP patients scored worse on almost all domains of the Chronic Ear Survey (CES) (p < 0.001). SF-36 scores of postoperative STP data showed negative Z-scores in almost all subscales, indicating lower levels of QoL compared to Dutch reference values. Most subscales of the Amsterdam Inventory for Auditory Disability and Handicap (AIADH) demonstrate a large-effect size in disadvantage of the STP cohort when compared to Dutch reference values., Conclusion: STP is a suitable technique to tackle severe ear disease. Despite its favourable surgical results, STP has a negative impact on several domains of patient's QoL., (© 2022. The Author(s).)

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.

Introduction: External auditory canal cholesteatoma (EACC) is a rare disease, with an estimated incidence of approximately 1:1,000 adult and 1.6:1,000 pediatric otologic patients. Systematic studies of chronic ear disease and taste alteration prior to surgery are rare; in fact, there are no such studies for EACCs. Therefore, we describe chorda tympani nerve (CTN) dysfunction and the related clinical consequences in EACC patients., Methods/study Design: Between 1992 and 2021, we retrospectively analyzed the symptoms, signs, and radiological and intraoperative descriptions of CTN involvement in 73 patients. Liquid taste tests and, since 2009, Taste StripsTM as well as an olfactory screening test (Smell DiskettesTM) have been performed for all symptomatic patients and, when feasible, all other EACC patients., Results: Ten of 73 patients complained subjectively of dysfunction, and 8 showed abnormal taste test results. Four patients complained of olfactory dysfunction (3 cases with pathological taste tests). Gustatory dysfunction was most frequent in radiogenic EACC cases (n = 4), followed by postoperative EACC (n = 3). Two postoperative patients were asymptomatic despite abnormal test results. Rarely, patients with idiopathic (n = 2) and posttraumatic (n = 1) EACC showed acute taste dysfunction that was confirmed in each with abnormal test results., Discussion/conclusion: CTN dysfunction often developed asymptomatically in chronic ears, except for idiopathic and posttraumatic EACCs under previous healthy middle ear conditions. Taste disturbance is not a cardinal symptom of EACC, but objective testing suggests that up to one out of 10 EACC patients with advanced disease may experience regional gustatory dysfunction prior to surgery. Especially in context of a new and acute presentation, regional taste dysfunction may alert the clinician of potential progressive EACC invasion and danger to the facial nerve., (© 2023 S. Karger AG, Basel.)

Purpose: To evaluate the morphology of the sinus tympani (ST) based on computed tomography (CT) scans (axial view), describing the findings in a cohort of 148 patients (296 ears), and classifying the prevalence according to our ST classification. To evaluate the surgical prevalence based on the type of ST. To calculate the sensibility and positive predictive value (PPV) of high-resolution computed tomography (HRCT) scans for ST involvement by cholesteatoma. Methods: Retrospective review of the radiologic database and surgical reports. Results: In total, 98/296 (33.1 %) middle ears presented a radiologic morphology Type A; 185/296 (62.5 %) middle ears presented a radiologic morphology Type B; 13/296 (4.4 %) middle ears presented a radiologic morphology Type C; HRCT showed a sensibility of 91 %, specificity of 65 %, PPV of 68 % and negative predictive value (NPV) of 90 %. Conclusions: ST shape and depth can influence surgical preference in cholesteatoma surgery. In the case of a shallower ST, an exclusive endoscopic exploration is chosen; whereas in the case of a deeper ST, a retrofacial approach is usually preferred. HRCT scans demonstrated high sensibility and NPV for ST involvement by cholesteatoma. [ABSTRACT FROM AUTHOR]

ntroductionIn all otologic surgeries, the facial nerve can be considered the most important structure that must be protected. The surgical damage risk of the facial nerve will be increased in presence of facial canal dehiscence (FCD).AimIn this study, we aimed to evaluate the FCD and its associated situations in patients with chronic otitis media undergoing mastoidectomy.Materials and Methods850 patients who performed ear surgery were reviewed. Patients who did not perform mastoidectomy were excluded. In patients, the ratios, localization of FCD, and association of facial canal dehiscence with cholesteatoma were researched. Association between FCD and closed-cavity tympanomastoidectomy, open-cavity tympanomastoidectomy, and radical mastoidectomy procedures, and also an association between FCD and primary or revision surgery was evaluated. ResultsFacial canal dehiscence was found in 8.4% of patients. The relationship between dehiscence and cholesteatoma was found significant (p&lt;0.001). In the comparison of radical mastoidectomy and closed-cavity tympanomastoidectomy, dehiscence was higher in the radical mastoidectomy group (p&lt;0.03). In revision cases, dehiscence was seen more frequently (p&lt;0.003). FCD incidence was higher in adult patients than in pediatric patients (p&lt;0.001). Twenty of 468 patients who were operated on for cholesteatoma had congenital cholesteatoma. ConclusionFacial canal dehiscence can be seen especially in cases with cholesteatoma and when performing radical mastoidectomy and revision surgeries. Therefore, a detailed evaluation of the status of facial nerve and determination of situations in which occurrence of FCD is more likely both preoperatively and intraoperatively will minimalize potential complications.Keywords: Cholesteatoma, tympanic, radical mastoidectomy, oval window, tympanomastoidectomy.

Cholesteatomas are non-neoplastic, invasive lesions created by the accumulation of keratinized squamous epithelium in the temporal bone. If left untreated, its expansion may cause local destruction of the surrounding structures, eventually leading to inner ear fistula, dehiscence of tegmen and possible intracranial pathology, and facial nerve paralysis. Surgical resection is the mainstay of curative treatment. We hereby present a case of a giant cholesteatoma in a 62-year-old patient who presented with a right-sided hemifacial spasm with later paralysis who was treated with botulinum toxin injection by a neurologist. CT scan imaging showed a very large cholesteatoma with involvement of the carotid canal, cochlea, and geniculate ganglion. The patient underwent transmastoid and subarcuate approach for resection which led to marked improvement of her symptoms.

Congenital cholesteatoma is a whitish mass in the middle ear medial to an intact tympanic membrane. It is often without symptoms and therefore incidentally diagnosed. Pediatric congenital cholesteatoma generally starts as a small pearl-like mass in the middle ear cavity that eventually expands to involve the ossicles, epitympanum, and mastoid. The location, size, histopathological type, and extent of the mass must be evaluated to select the appropriate surgical method. Although microscopic ear surgery has traditionally been performed to remove congenital cholesteatoma, a recently introduced alternative is endoscopic surgery, which allows a minimally invasive approach and has better visualization. Here, we report the first known case of a patient with congenital cholesteatoma in the anterior epitympanic recess and discuss the utility of an endoscopic approach in the removal of a congenital cholesteatoma in the hidden area within the middle ear.

Objective: The current study systematically reviewed the literature to compare auditory outcomes of patients treated for labyrinthine fistula (LF) based on characteristics of disease and surgical management., Databases Reviewed: PubMed, Scopus, Web of Science., Methods: Original series (at least five cases) published from 2000 reporting management and hearing results of LF secondary to cholesteatoma were included. Proportion and odds-ratio (OR) meta-analyses were conducted through inverse variance random-effects models based on logit transformation., Results: The prevalence of LF is estimated to be 7% (95% confidence interval [CI], 5-9%). Fistulae involving the lateral semicircular canal (90%; 95% CI, 87-93%) and larger than 2 mm (53%; 95% CI, 43-64%) were common, whereas membranous involvement was less frequent (20%; 95% CI, 12-30%). Complete removal of the cholesteatoma matrix overlying the LF was mostly applied. Bone conduction (BC) preservation was frequently achieved (81%; 95% CI, 76-85%); new-onset postoperative anacusis was rarely reported (5%; 95% CI, 4-8%). A higher chance of BC preservation was associated with sparing the perilymphatic space (OR, 4.67; 95% CI, 1.26-17.37) or membranous labyrinth (OR, 4.56; 95% CI, 2.33-8.93), exclusive lateral semicircular canal involvement (OR, 3.52; 95% CI, 1.32-9.38), smaller size (<2 mm; OR, 3.03; 95% CI, 1.24-7.40), and intravenous steroid infusion (OR, 7.87; 95% CI, 2.34-26.42)., Conclusion: LF occurs in a significant proportion of patients with cholesteatoma. In the past two decades, complete removal of the cholesteatoma matrix followed by immediate sealing has been favored, supported by the high proportion of BC preservation. Hearing preservation depends primarily on characteristics of the LF, and specific surgical strategies should be pursued. Intraoperative and postoperative intravenous steroid infusion is recommended., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objectives: The aim of this study was to evaluate, by means of the Chronic Ear Survey (CES), the quality of life of patients who had undergone either intact-canal wall tympanoplasty (ICWT) or canal wall-down tympanoplasty (CWDT) with mastoid obliteration. Methods: This was a retrospective case review study performed at a tertiary referral center. Among 379 patients affected by middle ear and mastoid cholesteatoma operated on between November 2000 and December 2009, 50 patients who underwent ICWT and 50 who underwent CWDT with mastoid obliteration were randomly selected. The CES scores were analyzed for both groups. Results: The mean scores on the CES were 6.5 ±2.1 in patients who underwent CWDT and 6.9 ± 2.2 in patients treated with ICWT (t = -0.93; p > 0.05). No significant differences between the two groups were found on the activity restriction, symptom, or medical resource subscales (p > 0.05). Conclusions: The results of this study demonstrate that CWDT with mastoid obliteration resulted in a quality of life comparable with that after ICWT. Postoperative hearing loss is the most frequently reported problem for both techniques. [ABSTRACT FROM AUTHOR]

Objective: To investigate the clinical characteristics, surgical experience, and surgical outcomes of external auditory canal cholesteatoma (EACC) surgery under endoscopic otolaryngoscopy., Methods: A retrospective analysis of 85 EACC cases admitted to the Department of Otolaryngology, Renji Hospital, Shanghai Jiaotong University School of Medicine, from January 2016 to February 2021 was performed, followed by retrospective analysis of clinical data to explore the feasibility and clinical characteristics of all-oral endoscopic EACC surgery. A total of 85 EACC patients (90 ears) with a mean age of 49.93 ± 14.87 years were included in the study. According to Udayabhanu staging, 43 ears (47.78%) were stage I, 40 ears (44.44%) were stage II, and 7 ears (7.78%) were stage III. All patients underwent transendoscopic surgery., Results: 79 ears (87.78%) underwent endoscopic EACC resection alone (+external auditory canal tumor resection/tympanostomy tube insertion), 9 ears (10%) underwent endoscopic EACC resection+tympanostomy+tympanoplasty, 1 ear (1.11%) underwent endoscopic EACC resection+tympanoplasty, and 2 ears (2.22%) underwent EACC resection+otolaryngotomy+tympanoplasty+auditory chain reconstruction endoscopically. Of these, 7 ears (7.78%) underwent auricular cartilage-chondroplasty and 2 ears (2.22%) underwent auricular cartilage membrane repair. All patients were reviewed at 1 week, 2 weeks, 1 month, 3 months, 6 months, and 1 year postoperatively. One patient with stage II external auditory atresia had a recurrence after 6 months and underwent endoscopic ear surgery (ESS) again. One patient with stage 2 atresia recurred after 1 year and again underwent endoscopic ear surgery. The rest of the patients recovered well after the surgery, and the grafts healed well., Conclusion: EACC surgery through the external ear canal under a dedicated endoscope is a safe, reliable, and effective method. Patients with stage I and II external auditory canal cholesteatoma surgery under endoscopy have a rapid postoperative recovery with significant hearing improvement, and stage IIIA patients can also achieve good results under strict evaluation of indications., Competing Interests: The authors declare that they have no conflict of interest., (Copyright © 2022 Yanan Guo et al.)

Objective: Recurrent otitis media and persistent otitis media with effusion in early childhood may cause an atelectatic eardrum and adhesive otitis media, which sometimes progress to pars tensa cholesteatoma. When and how children with adhesive otitis media should be operated on remain controversial. Therefore, this study aimed to analyze the clinical characteristics of children with adhesive otitis media and pars tensa cholesteatoma, and to determine the risk factors of progression to cholesteatoma., Methods: Seventeen ears of 15 children with adhesive otitis media (adhesive group) and 14 ears of 13 children with pars tensa cholesteatoma (tensa cholesteatoma group) who underwent tympanoplasty were included in this study. We analyzed the following clinical characteristics of children in both groups: medical and life history, associated diseases, sites of the adhesion, and development and aeration of mastoid air cells as shown by temporal bone computed tomography., Results: Most of the children in both groups had a history of recurrent otitis media and/or persistent otitis media with effusion. They showed a male predominance and a frequent association of allergic rhinitis. The number of ears showing undeveloped mastoid air cells in the tensa cholesteatoma group was significantly larger than that in the adhesive otitis media group (P=0.0068). A lack of aeration of the middle ear, including the eustachian tube, was more frequently found in ears with pars tensa cholesteatoma than in ears with adhesive otitis media (P=0.0012). Using multivariate logistic regression, the presence of otorrhea (odds ratio [OR], 14.847; 95% confidence interval [CI], 0.834-264.184), total adhesion (OR, 28.550; 95% CI, 0.962-847.508), and undeveloped mastoid air cells (OR, 19.357; 95% CI, 1.022-366.589) were related to pars tensa cholesteatoma., Conclusion: Children with adhesive otitis media should be carefully followed up in the outpatient setting. Ears with poor mastoid development may develop pars tensa cholesteatoma. Additionally, ears with middle ear effusion, total adhesion, and the presence of otorrhea tend to be at risk of pars tensa cholesteatoma. Tympanoplasty or tympanostomy tube insertion should be considered for children with adhesive otitis media who have these risk factors to prevent progression to pars tensa cholesteatoma., Competing Interests: Declaration of Competing Interest The authors declare no competing interest., (Copyright © 2022. Published by Elsevier B.V.)

Objective: To assess outcomes of transcanal endoscopic ear surgery (TEES) for congenital cholesteatoma., Study Design: Case series with chart review of children who underwent TEES for congenital cholesteatoma over a 10-year period., Setting: Three tertiary referral centers., Methods: Cholesteatoma extent was classified according to Potsic stage; cases with mastoid extension (Potsic IV) were excluded. Disease characteristics, surgical approach, and outcomes were compared among stages. Outcomes measures included residual or recurrent cholesteatoma and audiometric data., Results: Sixty-five cases of congenital cholesteatoma were included. The mean age was 6.5 years (range, 1.2-16), and the mean follow-up was 3.9 years (range, 0.75-9.1). There were 19 cases (29%) of Potsic stage I disease, 10 (15%) stage II, and 36 (55%) stage III. Overall, 24 (37%) patients underwent a second-stage procedure, including 1 with Potsic stage II disease (10%) and 21 (58%) with Potsic stage III disease. Eight cases (12%) of residual cholesteatoma occurred. One patient (2%) developed retraction-type ("recurrent") cholesteatoma. Recidivism occurred only among Potsic stage III cases. Postoperative air conduction hearing thresholds were normal (<25 dB HL) in 93% of Potsic stage I, 88% of stage II, and 36% of stage III cases., Conclusion: TEES is feasible and effective for removal of congenital cholesteatoma not extending into the mastoid. Recidivism rates were lower with the TEES approach in this large series than in previously reported studies. Advanced-stage disease was the primary risk factor for recidivism and worse hearing result. As minimally invasive TEES is possible in the youngest cases, children benefit from early identification and intervention.

Purpose: After radical surgery for chronic cholesteatoma (CWD mastoidectomy), patients have the option to have the posterior wall of their external auditory canal surgically reconstructed with S53P4 bioactive glass. The procedure eliminates some of the restrictions related to having a postoperative cavity and extends the options for a hearing prosthesis. If classic reconstruction is not possible and a hearing aid is not used, we suggest use of a Bonebridge implant., Methods: This study describes, over 18 months of follow-up, 16 patients after a two-stage surgical procedure: obliteration of the mastoid cavity with bioactive glass followed by Bonebridge implantation. There were 7 patients who received the first generation implant (BCI 601) and 9 who used the second (BCI 602). Before and after implantation, pure tone audiometry, sound field thresholds, and free-field audiometry were performed. Speech reception thresholds in noise were assessed using the Polish Sentence Matrix Test. Subjective assessment of benefits was done using the APHAB (Abbreviated Profile of Hearing Aid Benefit) questionnaire., Results: During the observation period, no serious complications were found. The study demonstrated the safety and validity of the procedures and confirmed the safety of using S53P4 bioactive glass in otosurgery (antibacterial effect, nonrecurrence of cholesteatoma, and no effect on the inner ear). The audiological benefits expected from using the Bonebridge implant processor were also confirmed., Conclusion: It is concluded that, after reconstructing the posterior wall of the external auditory canal with bioactive glass, two-stage implantation of a Bonebridge implant in a typical site is a safe solution for patients who have difficult anatomical conditions following their CWD mastoidectomy., Competing Interests: Declaration of competing interest None., (Copyright © 2022. Published by Elsevier Inc.)

Objective: This study aimed to compare surgical and audiometric outcomes of tympanoplasty alone (T) to tympanoplasty and mastoidectomy (T&M) in patients without cholesteatoma., Databases Reviewed: According to PRISMA guidelines, English articles in PubMed, Scopus, CINAHL, and Cochrane Library databases from inception to 7/29/2021 were searched., Methods: Studies describing a comparison of patients who underwent T to patients who underwent T&M were included. Studies describing patients with cholesteatoma were excluded. Patient demographics, graft failure rates, and preoperative and postoperative audiological findings were collected. Mean differences (MD) and risk difference (RD) were calculated using RevMan 5.4. Heterogeneity was assessed using Q test and I2 statistic. Risk of bias was assessed using both version 2 of the Cochrane risk-of-bias tool for randomized trials and Risk of Bias in Non-randomized Studies of Interventions., Results: A total of 27 studies fulfilled eligibility with T (n = 1,711) and T&M (n = 1,186). When pooling the data, mean differences between T versus T&M for air bone gap (-0.3 dB: 95% CI = -1.9 to 1.3, p = 0.730) and pure tone average (1.9 dB: 95% CI = -0.3 to 4.2, p = 0.090) were not statistically significant. Graft failure was higher with T only (16.4% versus 14.2%) than T&M (RD = -0.04, 95% CI = -0.07 to -0.00, p = 0.030, I2 = 35%]., Conclusion: This study endorses clinically similar audiological outcomes and a reduced risk difference of graft failure with mastoidectomy. Although these data suggest that adding a mastoidectomy could decrease the risk of graft failure, the risk reduction is minimal. More research on the cost-effectiveness and the specific patient clinical characteristics and comorbidities that would benefit from adding a mastoidectomy is warranted., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Purpose: Transcanal endoscopic ear surgery for congenital cholesteatoma provides a smaller incision and better visibility than conventional surgical microscope ear surgery. We aimed to evaluate the treatment results of transcanal endoscopic ear surgery in ameliorating the recurrence and post-operative complications of pediatric congenital cholesteatoma., Materials and Methods: A retrospective chart review was conducted on patients with congenital cholesteatoma who underwent transcanal endoscopic ear surgery at a tertiary referral medical center from January 2012 to December 2020. We categorized the patients into two groups according to the presence of remnant cholesteatoma and compared their characteristics., Results: This study included 46 patients (32 males and 14 females; 46 ears). The mean age was 3.0 ± 2.6 (1-17) years. Congenital cholesteatoma was predominantly located in the anterior-superior quadrant (63.0 %), and ossicular involvement was confirmed in six cases. The mean cholesteatoma size identified by temporal bone computed tomography was 3.9 ± 2.0 (1.2-13) mm. Seven cases had remnant cholesteatoma lesions (15.2 %); four improved after revision surgery, and three were followed up without immediate further intervention. Postoperative complications (e.g., tympanic membrane perforation, retraction) were observed in 10 cases. Comparative evaluations of various characteristics after categorizing the participants into residual and non-residual lesion groups revealed no significant differences., Conclusions: Consequently, transcanal endoscopic ear surgery can be considered an effective and safe operation for treating congenital cholesteatoma in the pediatric population with no serious side effects. Further large-scale research with hearing test results should be conducted meticulously as a follow-up to this study., Competing Interests: Declaration of competing interest The authors declare that they have no conflicts of interest., (Copyright © 2022. Published by Elsevier Inc.)

Conclusion: Chronically discharging mastoid cavities had well-defined technical faults from previous surgery. Identifying and eliminating these errors during revision canal wall-down (CWD) tympanomastoidectomy converted troublesome mastoid cavities into safe ears in 84% of cases. Objective: To assess intraoperative findings of revision CWD tympanomastoidectomy to determine the most common causes of failure of previous surgery. The short-term results of revision surgery after a 12-month follow-up period were analyzed. Methods: The study group consisted of 50 consecutive patients, 27 males and 23 females, aged 7-63 years (mean 34 ± 17 years) undergoing revision CWD tympanomastoidectomy due to persistently discharging mastoid cavities. The outcome of revision surgery was measured semi-quantitatively using a grading system. Results: The most common causes for failure of previous surgery were persistent mastoid cells in the tegmental, sinodural angle, and the retrolabyrinthine, retrofacial, and mastoid tip regions (100%); the bony overhangs at the edges of the mastoid cavity and the high facial ridge (98%); inadequately narrow meatus (84%); recurrent or residual cholesteatoma (46%); and remaining malleus head (40%). Revision surgery included elimination of all encountered limitations followed by mastoid cavity obliteration in about two-thirds of cases. Hearing restoration was done in only 34% of ears. In the remaining cases, intact ossicular chain (6%), existing sufficient previous reconstruction (22%) or impossible situation for reconstruction (38%) was found. [ABSTRACT FROM AUTHOR]

Objectives: We sought to identify factors that would predict hearing preservation in the treatment of semicircular canal labyrinthine fistulas. Methods: Between 1990 and 2010, 97 patients with semicircular canal fistulas were operated on and enrolled in this retrospective study. In 62 patients the matrix was removed and the fistula was sealed, whereas in 35 patients the canal was drilled, the matrix was detached, and the canal was occluded. Perioperative corticosteroids were administered in 51 patients. The main outcome measures were the bone conduction thresholds evaluated at 1 year after the operation. Factors considered for possible association with hearing preservation included age, gender, site and size of the fistula, primary versus revision surgery, surgeon, perioperative corticosteroid treatment, and surgical management of the fistula. Results: The bone conduction hearing level was improved in 16 patients, remained unchanged in 73 patients, and had worsened in 11 patients. On the univariate analysis, good hearing was predicted by grade II fistula, canal plugging, and corticosteroid treatment. However, none of these factors attained significance in the logistic regression model. Conclusions: In surgery of semicircular canal fistulas, good hearing outcomes are to be expected if perioperative corticosteroids are administered, matrix removal and fistula sealing is performed in grade II fistulas, and canal occlusion is performed in grade III and IV fistulas. [ABSTRACT FROM AUTHOR]

Abstract: Objective: To present the anatomical and functional results of the inside-out technique applied in pediatric cholestetaoma surgery and to evaluate functionality with good hearing results against radicality with lower recurrence rate. Methods: Retrospective analysis and evaluation of the postoperative outcome in a consecutive series of 126 children or 130 ears operated between 1992 and 2008. With the inside-out technique, cholesteatoma is eradicated from the epitympanum toward the mastoid and, as a single stage procedure, functional reconstruction of the middle ear is achieved by tympanoossiculoplasty. Results: In 89.2% of all cases, the ear was dry postoperatively. 80.9% of the ears reached a postoperative air-bone gap of 30dB or less and the median air conduction hearing threshold was 29dB; in 60.9% of all cases, hearing was postoperatively improved. The recurrence rate was 16.2% in a mean postoperative follow-up 8.5 years. Altogether, 48 ears (36.9%) underwent revision surgery. The complication rate was 3.1% and involved only minor complications. Conclusion: The inside-out technique allows a safe removal of cholesteatoma from the epitympanum toward the mastoid with a single-stage reconstruction of the ossicular chain. For this reason we support our individual approach, which allows creation of the smallest possible cavity for the size of the cholesteatoma. Our results confirm that the inside-out technique is effective in the treatment of pediatric cholesteatoma. [Copyright &y& Elsevier]

Conclusions: A total of 330 cases of adult cholesteatoma were operated with canal-wall down (CWD) and total reconstruction procedure (TRP) without staging. Independent of preoperative middle ear conditions, cholesteatoma extent and localization, long-term improvement of hearing with a low incidence of residual and recurrent disease were achieved. Objectives: To evaluate long-term surgical and hearing results using a well-defined surgical technique without staging in adult cholesteatoma. Methods: The same CWD surgical technique, including obliteration of the mastoid cavity, reconstruction of the canal wall, and ossiculoplasty with autologous bone, was used by three senior surgeons (1982-2004). Preoperative and postoperative pure tone average (PTA) for air conduction (AC), bone conduction (BC), and air-bone gap (ABG) were assessed and compared 1, 3, and 6 years after surgery. Various prognostic factors with potential influence on long-term hearing outcome were evaluated. Results: Recurrence of AC occurred in 10%, residual disease in 3%. Six years after surgery all patients except one had a dry ear and over 92% of all cases were water resistant. Three patients developed complete deafness. Long-lasting improvement and/or preservation of hearing, with maintenance of PTA-ABG closure in 68% of all cases within 20 dB, were obtained. Sixty-four (19%) ossicular revisions were performed. [ABSTRACT FROM AUTHOR]

A petrous bone cholesteatoma (PBC) is a rare epidermoid cyst of the petrous portion of the temporal bone. The main treatment is subtotal petrosectomy (SP), which generally involves sacrificing the chorda tympani. We report a case of extensive supralabyrinthine PBC in an elderly patient undergoing hemodialysis that was treated by SP with anatomical preservation of the chorda tympani. To the best of our knowledge, preservation of the chorda tympani during SP has not been previously reported. For maintenance of postoperative taste and appetite, preservation of the chorda tympani is a meaningful maneuver whenever possible.

Background: The aggressiveness of paediatric cholesteatoma has long been a matter of debate. While much of the evidence is substantiated by data from the Western world, it is further limited by the retrospective nature of most studies. Therefore, this paper presents a comparative analysis of various characteristics of cholesteatoma between paediatric and adult populations seen at our centre., Methods: A total of 50 patients (25 adults and 25 paediatric) with clinical diagnosis of chronic suppurative otitis media with cholesteatoma underwent canal wall down mastoidectomy over a period of two years. The intra-operative findings were noted and patients were followed up for six months., Results: There was more extensive spread and ossicular erosion in paediatric cases. However, complications such as facial canal dehiscence and lateral semicircular canal dehiscence were more common in adults., Conclusion: Paediatric cholesteatoma is more aggressive and invasive than adult cholesteatoma, and the clinical behaviour is consistent with findings from other parts of the world.

Objective: To analyze the clinical features and surgical outcomes of petrous bone cholesteatomas (PBCs). Methods: Data from 39 PBCs patients treated in the Department of Otorhinolaryngology, Xijing Hospital from September 2011 to December 2017 were reviewed retrospectively, including 23 males, 16 femals, aged 12-71 years old, with the median age of 37. Clinical classifications, surgical methods, facial and hearing function, and intraoperative and postoperative complications were made summary analysis. Results: In this study, five patients were congenital PBCs and 34 patients were acquired PBCs. The common clinical symptoms were hearing loss (100%, 39/39), ear discharge/pus (89.7%, 35/39) and facial paralysis (46.2%, 18/39). According to Sanna's classification, 14 cases were supralabyrinthine, including three cases underwent transcochlear (TC) approach, six cases underwent transotic (TO) approach and five underwent translabyrinthine (TL) approach. 10 cases were infralabyrinthine, including eight cases underwent subtotal petrosectomy, one case underwent TO approach and one underwent TL approach.10 cases were massive, including seven cases underwent TC approach, three cases underwent TO approach. Five cases were infralabyrinthine-apical, including two cases underwent TC approach, two cases underwent TO approach, and one case underwent endoscope assisted infratemporal fossa type B. The degree of facial nerve (FN) dysfunction from high to low was massive (6/10), supralabyrinthine (8/14), infralabyrinthine-apical (2/5) and infralabyrinthine (2/10). 19 cases involved in facial nerve operation, three cases underwent FN decompression, four cases underwent FN rerouting, four cases underwent nerve grafting, and one case underwent facial-hypoglossal anastomosis. Preoperative FN involvement in 18 cases, and the FN function was improved in 14 cases after surgery. The improved rate of postoperative FN function was 77.8%. The bone conducted hearing retained 50.0% (14/28) postoperatively. Five cases with cerebrospinal fluid leak were managed by inserting free muscle plugs and cavity obliteration. Two cases with the cholesteatomas matrix involved the sigmoid sinus and the jugular bulb, and occlusion of the sigmoid sinus was performed. Postoperatively, two patients presented with synkinesis. The patients were followed up for 40 to 115 months, and there was no recurrence. Conclusions: There are no specific clinical manifestations for PBCs, thus, it is difficult in early diagnosis and treatment. According to Sanna's classification, preoperative FN and hearing function, the best surgical approach should be selected with minimal recurrences and perioperative morbidity.

Background: Congenital cholesteatomas account for just up to 5% of all cholesteatomas and most commonly arise in the petrous apex and middle ear. Congenital cholesteatomas arising in the mastoid are rare and typically present late., Methods: In this study, we report a case series of 3 cases managed in our department between 2006 and 2021 and present a summary of the current literature., Results: Congenital cholesteatomas arising in the mastoid is a rare finding and even among reported cases, not all are clearly mastoid in origin. Their location allows for considerable growth before symptoms develop. Pain and localized swelling in the temporal area are the most common presenting symptoms which can lead to diagnostic challenges. Our cases show that although surgery is often appropriate, conservative manage- ment may be suitable in certain situations., Conclusion: Congenital cholesteatoma of mastoid origin is rare and can present a diagnostic challenge. Greater awareness is important to facilitate early detection. A high index of suspicion is needed in those presenting with retro-auricular pain and swelling in the context of a normal ontological examination.

Background: The aim of this study was to classify congenital cholesteatoma along an entire spectrum of involvement ranging from the middle ear to petrous apex., Methods: A total of 131 patients (85 adults and 46 children) underwent operations for congenital cholesteatoma over the duration of 27 years., Results: For most cases, middle ear mucosa was normal, the first ossicle eroded by the mass was the stapes, and the mastoid air cell system was well-pneumatized on intraoperative and radiographic views. Totally 34% of patients presented with facial nerve weakness and 45% of these cholesteatomas arose from the supralabyrinthine area (32.8%) and from the petrous apex (12.2%)., Conclusion: In this unified classification system, the otologist sees congenital cholesteatoma as a continuum, with facial nerve involvement and anacusis as part of the picture. This system of congenital cholesteatoma accommodates the supralabyrinthine and petrous bone locations of the disease.

Objective: Cholesteatoma is occasionally accompanied by excessive calcification and presumably has different osteogenic dynamics compared to noncholesteatomous chronic otitis media. Cholesteatoma-related sclerotic lesions through the temporal bone can pose manipulatory difficulties, possibly leading to worse surgical outcomes or complications. Therefore, we compared surgical outcomes of cholesteatomas with and without sclerotic lesions., Study Design: Retrospective cohort study., Setting: Tertiary academic medical center., Patients: Consecutive ears with acquired cholesteatomas requiring primary resection between January 2009 and December 2019. The ears followed up for <1 year were excluded., Interventions: Tympanoplasty with/without mastoidectomy., Main Outcome Measures: Prevalence and location of sclerotic lesion, comorbid fistulae, postoperative air-bone gap, recidivism, and complications., Results: Overall, 19 (6.4%) of 299 ears had sclerotic lesions. More than two-thirds (68.4%) of sclerotic lesions were located adjacent to the lateral semicircular canal (LSC). Among ears with sclerotic lesions adjacent to the LSC, 46.2% had a comorbid fistula in the LSC. The prevalence of labyrinth fistula was significantly greater in ears with sclerotic lesions (42.1%) than in ears without sclerotic lesions (5.0%) (p < 0.0001, Fisher's exact test). Sensorineural hearing deterioration was significantly higher in ears with sclerotic lesions than in ears without (p = 0.0004, Fisher's exact test). Multivariate logistic regression analysis demonstrated that the presence of sclerotic lesions was a significant prognostic factor for residual disease (odds ratio [95% confidence interval]: 6.820 [2.055-22.633], p = 0.0017)., Conclusions: Surgeons should be conscious of preoperative identification of sclerotic lesions adjacent to one of the semicircular canals, possibly leading to postoperative sensorineural hearing deterioration or residual cholesteatoma., Competing Interests: The authors disclose no conflicts of interest. The authors alone are responsible for the content and writing of the manuscript., (Copyright © 2022, Otology & Neurotology, Inc.)

Background: Results and success measures of cholesteatoma surgery are generally described using objective data whereas subjective data are mostly lacking. Patients experiences and complaints are becoming more important alongside clinical and audiometric outcome measures in cholesteatoma care., Objective: To investigate the course of patient-reported complaints, the impact of complaints, audiometric measures and the stability of audiometric measures, and complaints over time after primary and recurrent/residual cholesteatoma surgery., Methods: Postoperative patients were prospectively included and divided into primary acquired and recurrent/residual cholesteatoma. The EuroQol 5D (EQ-5D-3L), Otology Questionnaire Amsterdam (OQUA), and the Speech Spatial Questionnaire (SSQ) were completed by 144 patients up to 2 years postoperative. Patient-reported complaints divided in eight separate domains, postoperative hearing and impact on daily life were longitudinally assessed by means of linear mixed models., Results: Hearing loss and tinnitus are the most reported postoperative complaints over time. Patient-reported loss of taste and the impact of all complaints decline over time. All other patient-reported complaints remain stable over time, only itch complaints fluctuate. Primary cholesteatoma patients score significantly higher on hearing loss complaints compared with recurrent/residual patients although they have comparable mean audiometric hearing loss. Furthermore, pure-tone hearing threshold, instead of asymmetric hearing loss, is correlated with the localization domain of the SSQ., Conclusion: This study provides important insights in the course of complaints and its impact on daily life after cholesteatoma surgery. Overall, the postoperative patient-reported complaints after cholesteatoma surgery are generally low in the studied population., Competing Interests: Conflicts of Interest and Source of Funding: F.A.t.T., S.E.K., R.J.P., and P.M. were financially supported by The Netherlands Organisation for Health Research and Development (ZonMw) (grant 80-83700-9816504), Zorgverzekeraars Nederland (ZN), and Stichting Het Heinsius- Houbolt foundation. For the remaining authors none were declared., (Copyright © 2022, Otology & Neurotology, Inc.)

Purpose: To demonstrate our experience in the treatment of petrous bone cholesteatoma (PBC)., Methods: Data of PBC patients in our hospital from January 2000 to December 2019 were collected. Surgical approaches and facial function were mainly discussed and compared with the literature. The management of 2 giant PBC cases affecting rhinopharynx has been demonstrated., Results: The supralabyrinthine type was the most frequent type followed by the massive type. There were 5 cases with cholesteatoma extending into the clivus (2 cases), sphenoid (1 case) and rhinopharynx (2 cases). The translabyrinthine approach (40%) was our most frequently used approach followed by the middle fossa approach (36%) and the transmastoid approach (11%). There were 10 cases managed with the assistance of endoscope, including 3 cases with cholesteatoma extending into clivus, sphenoid and rhinopharynx separately. Obliteration of the cavity was performed in 70.3% (135/192) cases; 3 of them recurred. For the 2 giant PBC cases affecting rhinopharynx, traditional microscopic surgery assisted with transnasal endoscope was performed. The reduced exposure was beneficial for postoperative recovery, and the approach in the nasal cavity provided a permanent drainage for postoperative examination., Conclusion: Otologic endoscope combined with traditional microscopic surgery could reduce the exposure in surgery. For extremely extended cases of PBC, supplementary transnasal endoscopic approach deserves to be considered for the traditional temporal bone approach., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Abstract: Objective: This study was designed to analyse long-term results after surgery of acquired (ACH) and congenital cholesteatoma (CCH) of the middle ear in children and compare these with adults. Methods: Computer-based analysis of consecutively operated paediatric patients for ACH and CCH in a tertiary referral centre was made in 57 cases under the age of 12 operated 1983–2004 by three surgeons using identical technique. A canal wall down and total reconstruction procedure (TRP) with obliteration of the mastoid cavity, canal wall reconstruction, ossiculoplasty with consistent use of autologous bone and an “aeration enhancement procedure” (AEP) with silicon sheet in selected cases were used. Pre- and post-operative PTA (0.5–3kHz) and pure-tone average air-bone gap (PTA-ABG) together with surgical parameters were assessed 1, 3 and 6 years following surgery. Results: Results showed stable hearing over 6 years with low incidence of persistent and recurrent disease comparable with results from adult patients. In nearly half of the cases, silastic sheeting was used. In 21 cases, stapes was eroded. Bone conduction thresholds levels remained unaffected 6 years after surgery. No deaf ears, postoperative facial dysfunction or other lesions related to surgery were observed. Six years after surgery every evaluated ear was found to be water-resistant and infection -free. Conclusion: Our results suggest that one-stage eradication of ACH and CCH in children using total reconstruction procedure (TRP) provide long-term improvement or preservation of hearing, with a low incidence of persistent or recurrent disease. No difference in surgical outcome between children and adults was found. [Copyright &y& Elsevier]

Summary: Objective: The aim of this prospective study is to evaluate the differences in the ability to view structures in the middle ear between Tos modified canal wall-up mastoidectomy and classic canal wall-up (CWU) and canal wall-down (CWD) mastoidectomies. Methods: Eighteen temporal bones of 10 cadaver heads were used. Six bones were randomly selected for each surgical technique (Tos’ technique, CWU and CWD mastoidectomies) and fixed on an operating table. In the Tos’ technique: (1) transmeatal posterior tympanotomy with drilling of the supero-posterior bony annulus, (2) transmeatal atticotomy with preservation of the bony bridge and (3) cortical mastoidectomy were performed. In the classic CWU mastoidectomy, cortical mastoidectomy and posterior atticotympanotomy (facial recess approach) were performed. Incus and posterior half of the tympanic membrane were removed from all temporal bones. An independent specialist performing otologic surgery evaluated all of the temporal bones and approved the proper extent of dissections. Two another independent observers blinded to the purpose of the study were asked to point five anatomic structures and locations (sinus tympani, anterior attic, posterior crus, lateral attic and the Eustachian tube orifice) in two different sessions. Observers were allowed to rotate the operating table as it has been done during otologic surgery, and the line of view for the observers was both transmastoid and transcanal. The ability to view these structures was recorded as “correct/visible” or “incorrect/invisible”. These records were also verified after removing the posterior and superior walls of the outer ear canal in temporal bones of the Tos and CWU groups at the end of the study. Results: Significant differences were found in the ability to view middle ear structures between the Tos’ technique and CWU mastoidectomy as well as between CWU and CWD mastoidectomies, with the Tos’ and CWD techniques almost equally showing superiority to CWU (p <.0001). Of the five locations, sinus tympani and anterior attic were observed more frequently with the Tos’ technique versus CWU (p =.001). Sinus tympani (p <.005), anterior attic (p =.001) and posterior crus (p <.05) were observed more frequently with CWD versus CWU. There was no significant difference in the ability to observe lateral attic and the Eustachian tube orifice among the three techniques. In addition, there was no significant difference in the ability to visualize all the five locations between the Tos’ and CWD technique. Conclusions: Statistical analysis showed good reproducibility of this randomised, blinded study. Tos’ modified CWU mastoidectomy, in which most part of the canal wall is intact, showed similar advantage of the CWD in viewing structures in the middle ear. The Tos’ technique and CWD mastoidectomy provided better visualization of sinus tympani and anterior attic that are the most frequent places of residual cholesteatoma in the classic CWU mastoidectomy. [Copyright &y& Elsevier]

This survey investigates fallopian canal dehiscences in order to assess the risk of encountering an unprotected facial nerve during routine ear surgery. In a prospective non-randomized study, the intraoperative appearance of the facial canal in 357 routine ear operations was compared with 300 temporal bone specimens from 150 autopsies. Intraoperatively, a dehiscence was detected in 6.4% (23/357) of the operations, most frequently at the oval niche region (16/23 cases). The incidence increased with the number of operations (P<0.0002). Cholesteatoma surgery had the highest relative risk (RR 4.6) of exposing an unprotected facial nerve. Postoperatively, no persistent facial paralysis was observed. In four of five cases with a transient facial palsy due to local anesthetics, a bony dehiscence could be found. The anatomical study revealed fallopian canal dehiscences in 29.3% (44/150) of the autopsies. One-third (15/44) of the individuals affected displayed bilateral findings, thus resulting in 19.7% (59/300) of temporal bones affected. A total of 17/59 bones showed microdehiscences, and most (55/59) were located at the oval niche. The actual prevalence of fallopian canal dehiscences is significantly higher than intraoperative findings suggest. The oval niche is the most affected region. High-resolution computed tomography is of diagnostic value only in selected cases. Facial paralysis following local anesthesia is the most significant clinical sign. Vigilance in acute facial palsy after local anesthetics and in cholesteatoma surgery and adequate intraoperative exposure help to prevent iatrogenic injury of the uncovered nerve. In unclear cases, nerve monitoring can facilitate a safe outcome. [ABSTRACT FROM AUTHOR]

Objectives: A typical presentation of congenital cholesteatoma (CC) is asymmetric conductive hearing loss (CHL). As CHL is usually associated with middle ear effusion, diagnosis of CC is frequently delayed. This study aimed to describe the clinical characteristics, treatment and outcomes of children with CC., Methods: The medical files of children diagnosed with CC at a large tertiary pediatric medical center during 2000-2019 were reviewed. The primary outcome measures were: presenting symptoms, surgical findings, stage of disease, recurrence rate and hearing outcome. Imaging findings and the size of mastoid air cells were assessed in CT scans., Results: Thirty-nine children were diagnosed with CC. The presenting symptom was unilateral CHL in 85%, with an average speech reception threshold of 41.5 ± 13.7 dB in the affected ear. The mean time from first symptoms to diagnosis was 1.3 years. The surgical approach was exploratory tympanotomy in 25% and canal wall up mastoidectomy in 69%. Seventy percent of the children presented with Potsic stage III-IV. The mean postoperative speech reception threshold was 26.4 ± 12.2 dB (P = 0.002). Recurrence of cholesteatoma occurred in 38% of the patients, mostly in stage III-IV. Mastoid air cell size was significantly smaller on the affected than the unaffected side., Conclusions: In children with persistent unilateral or asymmetric conductive hearing loss, CC should be suspected. Late diagnosis of CC is associated with a high recurrence rate. This highlights the need to promote awareness to the disease among primary physicians in the community health care system., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Objective: Cholesteatoma is an aggressive form of chronic otitis media (COM). For this reason, it is important to distinguish between COM with and without cholesteatoma. In this study, the role of artificial intelligence modelling in differentiating COM with and without cholesteatoma on computed tomography images was evaluated., Methods: The files of 200 patients who underwent mastoidectomy and/or tympanoplasty for COM in our clinic between January 2016 and January 2021 were retrospectively reviewed. According to the presence of cholesteatoma, the patients were divided into two groups as chronic otitis with cholesteatoma (n = 100) and chronic otitis without cholesteatoma (n = 100). The control group (n = 100) consisted of patients who did not have any previous ear disease and did not have any active complaints about the ear. Temporal bone computed tomography (CT) images of all patients were analyzed. The distinction between cholesteatoma and COM was evaluated by using 80% of the CT images obtained for the training of artificial intelligence modelling and the remaining 20% for testing purposes., Results: The accuracy rate obtained in the hybrid model we used in our study was 95.4%. The proposed model correctly predicted 2952 out of 3093 CT images, while it predicted 141 incorrectly. It correctly predicted 936 (93.78%) of 998 images in the COM group with cholesteatoma, 835 (92.77%) of 900 images in the COM group without cholesteatoma, and 1181 (98.82%) of 1195 images in the normal group., Conclusion: In our study, it has been shown that the differentiation of COM with and without cholesteatoma with artificial intelligence modelling can be made with highly accurate diagnosis rates by using CT images. With the deep learning modelling we proposed, the highest correct diagnosis rate in the literature was obtained. According to the results of our study, we think that with the use of artificial intelligence in practice, the diagnosis of cholesteatoma can be made earlier, it will help in the selection of the most appropriate treatment approach, and the complications can be reduced., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: To investigate the clinical features, diagnosis, and treatment experience of congenital stenosis of an external auditory canal with external auditory canal cholesteatoma. Methods: The clinical data of 152 patients（153 ears） with congenital external auditory canal stenosis complicated with external auditory canal cholesteatoma treated in the Department of Otorhinolaryngology Head and Neck Surgery of Beijing Tongren Hospital affiliated to Capital Medical University from January 2009 to December 2019 were analyzed retrospectively, including the age of onset, clinical manifestations, signs, audiology, high-resolution computed tomography（HRCT） of the temporal bone, mode of operation and so on. According to the preoperative imaging findings, intraoperative findings,and pathological results, 152 patients with congenital external auditory canal stenosis with external auditory canal cholesteatoma were treated with canaloplasty and tympanoplasty while clearing the cholesteatoma. Results: All patients were followed up for 2-2.5 years, there was no recurrence of cholesteatoma, and the reconstructed external auditory canal was spacious. The hearing levels of 108 ears who underwent hearing reconstruction were significantly improved, and the average hearing threshold was reduced by 20-35 dB. Conclusion: The stenosis of the external auditory meatus is easy to be complicated with cholesteatoma of the external auditory canal,and the occurrence of cholesteatoma of the external auditory canal is directly related to the diameter of the external auditory canal meatus.But the time of occurrence of the cholesteatoma is not directly related to the diameter of the external auditory canal. Severe congenital stenosis of the external auditory canal with auricle deformity is easy to be missed and misdiagnosed due to retroauricular redness, swelling, and ulceration. For this kind of patient, cholesteatoma should be treated first, and then plastic surgery such as auricle reconstruction should be performed. Retroauricular incisions should be avoided to create conditions for auricle reconstruction in the future., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

A total of 49 patients with cerebellopontine angle cholesteatoma from the Department of Neurosurgery, Affiliated Hospital of Xuzhou Medical University between January 2013 and January 2021 were recruited. All patients were evaluated by MRI scan before surgery and tumor resection was performed under microscope via retrosigmoid sinus approach. Then residual tumor was searched with 0°and 30°neuroendoscopy, and tumor resection was performed.Residual tumors were foundand resectedin 38 cases under theneuroendoscopy after routine microsurgery.Total and subtotalresections were performed in 44 cases and 5 cases, respectively. Complications included aseptic meningitis ( n =8), cerebrospinal fluid leakage ( n =1) and intracranial hematoma ( n =2). Follow-up[42±3(6-72)months] was available in all patients. During follow-up, 45 cases (91.8%) had a Kar-nofsky Performance Status (KPS)score ≥80.Neuroendoscopy-assisted microsurgery for cerebellopontine angle cholesteatomas helps enhance the total resection rate and decrease the operative risk.

Purpose: Limited literature exists on primary external auditory canal (EAC) cholesteatoma (EACC). Here, we focus on the clinical features of this rare disease, especially the invasive patterns of lesion progression, through a large population study and present simple and practical staging., Methods: In all, 276 patients (male 99; female 177; mean age 41.3 ± 21 years; ears 301) with primary EACC were retrospectively analyzed. Stage I indicated EACC without bony lesions, stage II indicated invasion confined within EAC, stage III indicated invasion beyond the EAC involving mastoid air cells or tympanic cavity, but within the temporal bone, and stage IV indicated invasion beyond the temporal bone., Results: In all, 41, 219, 40, and 1 ear with Stage I, II, III, and IV lesions were found, respectively. Common clinical symptoms were hearing loss (237 ears, 78.7%), otalgia (221 ears, 73.4%), and otorrhea (85 ears, 28.2%). The mean air conduction and air-bone gaps were 45.4 ± 17.9 dB HL and 24.6 ± 15 dB HL, respectively. EACCs were found to invade in all directions of the EAC, with the inferior wall (224 ears, 74.4%) > posterior wall (207 ears, 68.8%) > anterior wall (186 ears, 61.8%) > superior wall (86 ears, 28.6%) invasion; multiwall invasions (207 ears) were common; however, inward invasions into the tympanic cavity were rare., Conclusion: Primary EACCs occurred mostly in women and often unilaterally invaded multiple bony walls in the lower half of the EAC. The present staging reflects the patterns and severity of lesion progression and may be beneficial in treatment planning., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Compare surgical and audiological outcomes of patients with congenital aural stenosis (CAS) with cholesteatoma to patients with CAS without cholesteatoma and patients with complete congenital aural atresia (CCAA)., Study Design: Retrospective case series., Setting: Tertiary care hospital., Patients: Patients with CAS (with and without cholesteatoma) and CCAA., Intervention: Surgery for CAS/CAA., Main Outcome Measures: Patients with CAS and CAA undergoing surgical repair from June 2004 to July 2020 were identified from an institutional database. Included patients were divided by presence of a canal cholesteatoma. Clinical history, pre- and postoperative audiometric data, and clinical outcomes were compared., Results: Of the 283 patients (300 ears), 18 (19 ears) had a canal cholesteatoma. When compared to ears without cholesteatoma (CCAA ears plus CAS ears without cholesteatoma), ears with cholesteatoma were more likely to be younger (9.2 ± 6.6 vs. 11.5 ± 9.2; p = 0.015), female (66.7% vs. 38.1%; p = 0.02; OR 3.2, 95% CI 1.18-8.9), and have normal/Grade I microtia (47.4% vs. 9.6%; p < 0.0001; OR 0.12, 95% CI 0.044-0.32), but not a history of draining ear (5.3% vs. 0%; p = 0.05; OR 0.06, 95% CI 0.004-0.999). Preoperative audiometric data demonstrated a lower mean air-bone gap (45.8 dB vs. 52.3 dB; p = 0.009) and better speech reception threshold (48.7 dB vs. 57.4 dB; p = 0.0004) in cholesteatoma ears. Postoperatively, ears with cholesteatoma were more likely to close the ABG within 20 dB (p = 0.001; OR 0.19, 95% CI 0.072-0.52). No patient in the cholesteatoma group developed post-operative bony/soft-tissue stenosis (0% vs. 9.7%; p = 0.65; OR 1.61; 0.21-12.6) or required revision surgery (0% vs. 11%; p = 0.38; OR 2.46, 0.32-19)., Conclusions: Patients with CAS and cholesteatoma have better audiometric outcomes and likely a more durable repair with a decreased need for revision possibly secondary to greater embryologic development of the meatus, ear canal, and middle ear space despite the cholesteatoma., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

In this paper, a case of middle ear cholesteatoma with sigmoid sinus thrombophlebitis and retroauricular subperiosteal abscess was reported. The female patient was hospitalized with bilateral ear abscess for more than 20 days and fever with vomiting for 14 days. Anti-infection treatment after admission, emergency right mastoid radical resection and tympanoplasty were performed under general anesthesia. The patient recovered well after surgery, and there was no recurrence after in the follow-up for more than 2 years.The clinical manifestations, imaging features and prognosis of this disease were discussed and analyzed in the paper., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)