Expression of Periostin in Congenital and Acquired Middle Ear Cholesteatoma.

Type III-r ossiculoplasty (or incus reposition) is commonly used for transmastoid decompression of the facial nerve. Although this technique should also be applicable to cholesteatoma surgery with an intact ossicular chain, it has rarely been reported. The purpose of this study was to compare the postoperative outcomes of type III-r with other ossiculoplasty techniques in middle ear cholesteatoma surgery and to evaluate the efficacy of type III-r. A retrospective chart review was performed on 227 ears (227 patients) with middle ear cholesteatoma that underwent initial surgery during the period between April 2013 and August 2023, with special reference to the 65 ears (65 patients: 41 males, 24 females, mean age 42.2years) in which the ossicular chain was found to be continuous during surgery. Preoperative and postoperative air conduction threshold (AC) and air-bone gap (ABG) were compared between the ossiculoplasty types. Postopereative recurrence rate of cholesteatoma were compared between the ossiculoplasty types, as well. The types of ossiculoplasty performed included type III-r (8 ears), type I (36 ears), type III-i (6 ears), and type III-c (15 ears). The percentage of patients with successful hearing results (ABG < 20dB) was 100% for type III-r and type I, 100% for type III-i, and 85% for type III-c. Type III-r showed significant improvement in AC and ABG. During the follow-up period, there were no cholesteatoma recurrences in the type III-r, but 2 case recurrences (one residual and one reformation) after type I. In surgery for middle ear cholesteatoma with intact ossicular chain, the type III-r ossiculoplasty showed comparable hearing result with the type I. Therefore, it may be legitimate not to exclude the possibility of adopting type III-r in surgery for cholesteatoma. However, this study is preliminary and further study is necessary to reassure the lower residual recurrence of cholesteatoma after type III-r than type I.

Subtotal Petrosectomy in Middle Ear Surgery: Insights From 40 Years of Experience at "Gruppo Otologico".

Role of Epigenetics in the Pathogenesis of Acquired Cholesteatoma-A State of the Art Review.

Middle ear cholesteatoma (cholesteatoma), also known as a cholesteatomatous chronic otitis media, is concerning because it expands into the middle ear with bone destruction and causes irreversible hearing loss. Surgical resection is currently the only curative treatment, but the high recurrence rate remains a major problem, necessitating the development of novel therapies. In our previous study, we demonstrated that histone modifications are involved in the pathogenesis of cholesteatoma and that keratinocyte growth factor (KGF)-induced murine cholesteatoma is suppressed by administration of MI-503, a menin-MLL inhibitor. This study was designed to assess the therapeutic potential of menin-MLL inhibitors for the non-surgical management of cholesteatoma and to elucidate their mechanism of action. For the in vitro study, a growth inhibition assay was performed by administering menin-MLL inhibitors to mouse-derived primary tympanic membrane epithelial cells. For the in vivo study, menin-MLL inhibitors were topically administered into a KGF-induced murine cholesteatoma for seven consecutive days. The therapeutic effects on cholesteatoma were analyzed using micro-computed tomography imaging. The menin-MLL inhibitors reduced KGF-induced cholesteatoma in vivo (3/3, 100%). Among the menin-MLL inhibitors, BMF-219 (50 µM), a covalent menin inhibitor, showed the strongest inhibitory effect against cholesteatoma, with a 70.75 ± 8.92% rate of residual lesion. These findings show promising results for the therapeutic use of menin-MLL inhibitors in the non-surgical management of cholesteatoma.Supplementary InformationThe online version contains supplementary material available at 10.1038/s41598-025-34922-3.

Surgical management of chronic ear diseases, particularly middle ear cholesteatoma and chronic otitis media, often results in significant skin defects in the external auditory canal (EAC) and auricular region. To evaluate the clinical efficacy and safety of xenogeneic acellular dermal matrix (xeno-ADM) in repairing ear skin defects following surgical resection of middle ear pathologies. A prospective cohort study was conducted from March 2024 to April 2025 at a single tertiary care center. One hundred and twelve consecutive patients with ear skin defects underwent reconstruction using xeno-ADM. Primary outcomes included epithelialization time, functional recovery (ear canal patency, hearing improvement), and postoperative complications. Secondary outcomes included surgical difficulty, patient satisfaction, and physician evaluation. Air conduction threshold changes were measured pre- and postoperatively. Pain was assessed using Visual Analog Scale. Mean epithelialization time was 36.0 days. All patients achieved complete epithelialization with 100% ear canal patency. Hearing improved significantly with mean air conduction threshold reduction of -13.32 ± 14.67 dB, with 94% of patients showing improvement or stability. Postoperative pain was minimal. Complications were rare: mild exudate in 75.0%, local infection in 0.9%, partial membrane loss in 13.4% (successfully managed with reapplication). No rejection reactions occurred. Patient satisfaction was 100% for both appearance and function. Xeno-ADM demonstrates excellent efficacy and safety for ear skin defect repair, offering rapid epithelialization, significant hearing improvement, minimal pain, and high patient satisfaction during the mean follow-up of 6 months. However, the absence of a control group limits the strength of these conclusions, and long-term outcomes remain unknown due to the limited follow-up duration.

Cholesteatoma is a progressive disease characterized by abnormal proliferation of keratinizing squamous epithelium within the temporal bone. Although both congenital and acquired forms have been recognized, the molecular mechanisms underlying its development remain poorly understood. Although surgery is the only curative option, the high recurrence rates underscore the need to identify genetic risk factors. The rs17822931 (538G > A, G180R) polymorphism in the ABCC11 gene has been associated with cholesteatoma risk in the Japanese population. However, ethnic variation in the frequencies of SNPs other than rs17822931 raises the question of whether its involvement in cholesteatoma development is unique to the Japanese population or generalizable to other ethnic groups. To address this, we conducted a case-control study involving 818 individuals (510 controls and 308 cholesteatoma patients) who presented to the Heidelberg University Hospital (Kopfklinik) in Germany. Among 472 individuals aged ≥ 46 years (302 controls and 170 cholesteatoma patients), 164 matched pairs were selected using 1:1 caliper matching (width = 0.1). Conditional logistic regression was then performed on the resulting 328 individuals (164 controls and 164 cholesteatoma patients). We found that rs17822931 was significantly associated with cholesteatoma in the dominant (OR = 2.04; 95% CI: 1.23-3.41; p = 0.00594) and per-allele (OR = 1.80; 95% CI: 1.18-2.73; p = 0.00599) models. These findings from a German cohort replicate the association between the rs17822931 (538G > A, G180R) polymorphism in the ABCC11 gene and cholesteatoma risk in a non-Japanese population, suggesting a broader role of rs17822931 across ethnicities.

Objective:To explore the application value of high-resolution temporal bone CT and DW-MRI fusion technology in achieving precise diagnosis and anatomical localization of middle ear cholesteatoma during endoscopic surgery. Methods:Eighteen patients initially diagnosed with middle ear cholesteatoma in the Department of Otolaryngology Head and Neck Surgery, Shaanxi Provincial People's Hospital, from January to June 2024 were enrolled.Preoperative high-resolution temporal bone CT and DW-MRI were performed, and rtStation software was used for image fusion to construct CT-MRI fused images. The involvement of cholesteatoma in six anatomical subregions of the temporal bone was evaluated. Using surgical pathology as the gold standard, and combining surgical videos and anatomical records, the sensitivity, specificity, and accuracy of pure CT, pure DW-MRI, and CT-MRI fused images in evaluating middle ear cholesteatoma lesions were compared. Results:A total of 18 patients were included, and 17 cases were pathologically confirmed as middle ear cholesteatoma postoperatively. The sensitivity of the preoperative of preoperative CT was 100%, but the specificity was only 44.44%, with an overall accuracy of 72.22%; the sensitivity and specificity of DW-MRI evaluation were 81.46% and 85.19%, the accuracy was 83.33%, respectively. In contrast, the sensitivity and specificity of CT-MRI fusion image to the spatial localization of cholesteatoma were higher than that of DW-MRI alone（92.59% vs 81.46%; 98.15% vs 85.19%）, and the diagnostic accuracy was also significantly improved（95.37% vs 83.33%）. The Kappa values for the agreement between HRCT, DW-MRI, and CT-MRI segmentation localization and pathological results were 0.444, 0.667, and 0.907 respectively. The chi-square paired t-test confirmed statistically significant diagnostic differences between groups（P<0.001）. Results demonstrated that CT-MRI significantly outperformed HRCT and DW-MRI in diagnostic efficacy for segmental localization of primary posterior congenital middle ear cholesteatoma. Conclusion:High-resolution temporal bone CT combined with DW-MRI fusion technology demonstrates higher sensitivity, specificity, and accuracy in the diagnosis and spatial localization of middle ear cholesteatoma than single imaging modalities. It can provide more precise evaluation of lesion scope for endoscopic surgery, showing important clinical application value.

Effect of histone acetyltransferase inhibitor against middle ear cholesteatoma in mouse model.

Background and Objectives: To evaluate the surgical management and outcomes of lateral semicircular canal fistulas (LSCFs) in patients with middle ear cholesteatoma, focusing on hearing preservation and vestibular function. Materials and Methods: A retrospective study was conducted on nine adult patients diagnosed with LSCFs secondary to cholesteatoma who underwent surgery at a tertiary referral center between 2018 and 2024. The preoperative evaluation included otoscopy, audiometry, vestibular testing (HINTS), and high-resolution CT (HRCT) of the temporal bone. Surgical techniques included canal wall up (CWU) or canal wall down (CWD) mastoidectomy, depending on the disease extent. Cholesteatoma matrix removal from the fistula was performed carefully. Fistula closure involved layered grafts of temporalis fascia, temporalis muscle, and/or gelfoam. The postoperative follow-up included audiometry and vestibular assessments. Results: Nine patients with LSCFs were identified (one Type III, three Type IIb, and four Type I/IIa). Five patients were found to have additional disease complications intraoperatively, including facial nerve involvement and middle fossa dehiscence. Postoperatively, hearing outcomes varied, with some patients experiencing improvement, others demonstrating stable hearing, and some exhibiting further decline, particularly in cases with extensive disease. Vestibular symptoms, including vertigo, generally resolved postoperatively, although some patients required prolonged vestibular rehabilitation. Conclusions: LSCF management in cholesteatoma surgery requires a careful preoperative assessment, meticulous surgical technique, and individualized fistula closure based on the size and type. While hearing preservation remains a challenge, particularly in extensive cases, the “underwater technique” and layered grafting may contribute to minimizing further damage and promoting fistula closure. Vestibular rehabilitation plays a crucial role in managing postoperative balance issues. A long-term follow-up is essential to monitor for recurrence and assess both auditory and vestibular function.

Key Interventions Related to Middle Ear Cholesteatoma: A Systematic Umbrella Review.

The imaged "decisional line" for cholesteatoma surgical approach.

Risk Factors for Recidivism in Stage II Pars Flaccida Cholesteatoma: Impact of Disease Extent and Surgical Procedures.

Congenital Middle Ear Cholesteatoma in Children: A 12-Case Series

Dear Editor, We are sincerely grateful for the interest and valuable insights the writer has provided regarding the case that we presented. Prior to the patient’s consultation and admission to our institution, a number of general practitioners and ENT doctors who had seen the patient treated him with oral and topical antibiotics with a diagnosis of Chronic Otitis Media. Upon receiving the patient, we immediately thought of a different disease process present because of the unusual presentation. Diagnostics as well as surgical planning was mobilized. Intraoperatively, the tympanic membrane’s status was noted to be perforated because the mass that was seen obliterating the external ear canal was noted to have extended from the middle ear. About 40% of fibrous dysplasia presents with cholesteatoma and the majority of them are found in the external auditory canal.1 However, in our case, the cholesteatoma was seen in the middle ear. A similar case by Jethanamest and Roehm showed an unusual presentation of cholesteatoma in the middle ear.2 Another study by Ge et al. also observed middle ear cholesteatoma in their patient with fibrous dysplasia.3 There is a lack of literature identifying chronic otitis media in fibrous dysplasia. It has been pointed out that cholesteatoma in fibrous dysplasia is usually caused by the accumulation of keratin due to stenosis of the external auditory canal.4 Theoretically, cholesteatomas can further cause complications and can spread infection and products of inflammation like proteins, bacteria, and toxins.4 Their presence in the middle ear could cause infection and erosion of the ossicles. This is also seen in patients with cholesteatoma formed from the EAC stenosis of fibrous dysplasia. The similarity in effect could be diagnostically challenging for some otorhinolaryngologists. We completely agree with the important pointers and knowledge that the letter writer has imparted to us, and this is in line with the main purpose for writing the paper. We have highlighted that this case calls physicians, especially ENT physicians, to be vigilant of ear discharges. We have stressed that not all chronic ear discharges mean Chronic Otitis Media and that we have to be wary of other pathologies. The letter only shows that there is more to understand about this disease and that with the pointers, experience and expertise given by this letter and the previous article, the readers will understand the disease process better and will be able to identify and treat patients accordingly. This case was presented to share our experience on the diagnostic dilemma and outcome of the treatment done. We share the same sentiments that misdiagnosing would delay treatment, this is why we immediately took action when we received the referral. In the future, we are hoping that readers would be guided in planning for patients with fibrous dysplasia by the different literatures available and share their experiences of the outcomes. Thank you.

The article aimed to explore the differences in clinical features of facial paralysis secondary to petrous bone cholesteatoma and middle ear cholesteatoma. The clinical data of 156 patients (156 ears) aged (37.9±16.3) years with petrous apex cholesteatoma or middle ear cholesteatoma at the Eye & ENT Hospital of Fudan University from December 2014 to December 2024 were retrospectively collected, with 97 males and 59 females. There were 101 and 55 cases diagnosed with petrous apex cholesteatoma and middle ear cholesteatoma, respectively. The proportion of cases with facial paralysis as the initial symptom in petrous apex cholesteatoma [42.6% (43/101)] was statistically significantly higher than that of middle ear cholesteatoma [18.2% (10/55)] (P=0.002). The proportion of facial paralysis duration to the total disease course was significantly higher in petrous apex cholesteatoma [M (Q1, Q3)] [20.0% (0, 100%)] compared with middle ear cholesteatoma [0.4% (0, 23.3%)] (P=0.003). The main injury sites of facial nerve canal observed in patients of petrous apex cholesteatoma during surgery were the labyrinthine segment (58 ears, 57.4%), the geniculate ganglion (61 ears, 60.4%) and the tympanic segment (65 ears, 64.4%), while the tympanic segment (43 ears, 78.2%) was the most observed injury site in middle ear cholesteatoma, with a statistically significant difference between the two groups (P<0.001). The common lesion type of facial nerve seen in the surgery was compression (50 ears, 49.5%) inpetrous apex cholesteatoma and bareness (21 ears, 38.2%) in middle ear cholesteatoma, with a statistically significant difference between the two groups (P<0.001). The current study suggests that compared with middle ear cholesteatoma, petrous apex cholesteatoma, due to its deep location and insidious symptoms, often induces more severe damage to the facial nerve by the time of diagnosis.

ImportanceTrimming perforation margins (TFMs) is a classic procedure of myringoplasty.ObjectiveThe objective of this study was to evaluate the long-term graft outcomes and iatrogenic cholesteatoma of endoscopic cartilage myringoplasty with preserving perforation margins (PFMs) for repairing small- to medium-size perforation.Study designProspective semi-randomly control study.SettingTertiary university hospital.ParticipantsPatients with chronic small- to medium-size perforations.InterventionTFM group or the PFM group.Main outcome measuresComprehensive evaluations included graft success rate, hearing improvement, and imaging examinations at 3, 6, and 36 months after surgery and complications.ResultsAt postoperative 3 months, the graft success rate was 91.84% in the TFM group and 96.30% in the PFM group (P = .59). At postoperative 6 months, graft separation resulted in re-perforation in 3.70% patients in the PFM group, and the graft success rate was 91.84% in the TFM group and 92.59% in the PFM group (P = .82). At postoperative 3 years, the graft success rate was 93.88% in the TFM group and 94.44% in the PFM group (P = .77). Although postoperative air bone gaps (ABGs) were not significantly different between 2 groups, the PFM group had better hearing restoration compared with the TFM group in postoperative 3 months or 3 years. Additionally, although no significant differences were found in the ABGs between postoperative 3 months and postoperative 3 years in any group, postoperative 3 years showed better hearing restoration compared with postoperative 3 months. In addition, endoscopic morphology showed the graft stratification in .00% in the TFM group and 11.11% in the PFM group (P = .05). Postoperative high-resolution computed tomography (HRCT) revealed a few soft tissue shadow in 8.16% patients in the TFM group and in 5.56% patients in the PFM group (P = .89); middle ear cholesteatoma was excluded by magnetic resonance imaging. However, HRCT revealed that the middle ear and mastoid were well pneumatized in the remaining patients.Conclusions and relevanceThe long-term graft success rate and hearing improvement of cartilage underlay myringoplasty with PFMs are reliable for repairing small- to medium-size perforations; 3 years imaging examinations did not reveal iatrogenic cholesteatoma. However, it may cause graft stratification and graft separation.

CD9 downregulation activates EGFR/ERK/WAVE2 pathway to remodel F-actin and promote proliferation and migration of cholesteatoma epithelial cells.

Objective:To compare the preoperative presentation, intraoperative findings, and postoperative outcomes between middle ear cholesteatoma with tympanosclerosis （MECwTS） and middle ear cholesteatoma without tympanosclerosis （MECw/oTS）, thereby investigating the clinical characteristics of MECwTS. Methods:A retrospective analysis was conducted on the clinical data of 120 patients with middle ear cholesteatoma. Patients were divided into two groups based on the presence or absence of concomitant tympanosclerosis: the MECwTS group （n=49） and the MECw/oTS group （n=71）. All patients underwent preoperative evaluations including temporal bone CT, otoscopic examination, pure-tone audiometry, tympanometry, and assessment using the Zurich Chronic Middle Ear Inventory （ZCMEI-21） quality of life scale. All patients underwent canal wall down mastoidectomy with tympanoplasty. Concurrent ossicular chain reconstruction was performed: partial ossicular replacement prosthesis （PORP） in 83 cases and total ossicular replacement prosthesis （TORP） in 37 cases. Intraoperative disease severity was assessed using the Cholesteatoma Comprehensive Score Scale （CCSS）. Postoperative follow-up lasted at least one year and included pure-tone audiometry, otoscopic examination, and the ZCMEI-21 scale administered at ≥1 year post-surgery. Preoperative and postoperative air-bone gap （ABG） and ZCMEI-21 scores were compared between the MECwTS and MECw/oTS groups. Additionally, surgical efficacy was defined as a postoperative ABG ≤20 dB; the hearing improvement efficacy of PORP versus TORP was compared based on this criterion. Results: ①Preoperative ABG showed no significant difference between the MECw/oTS and MECwTS groups（P>0.05）. Postoperative ABG was （18.65±10.21） dB in the MECw/oTS group versus （22.55±9.53） dB in the MECwTS group, demonstrating a statistically significant intergroup difference （P<0.05）. ②Intraoperative CCSS scores were significantly higher in the MECwTS group （8.04±2.18） compared to the MECw/oTS group （5.93±1.44） （P<0.05）. ③Preoperative ZCMEI-21 scores showed no significant difference between groups （P>0.05）. Postoperative ZCMEI-21 scores were （22.24±8.11） in the MECw/oTS group versus （27.02±7.21） in the MECwTS group, indicating a statistically significant difference （P<0.05）. ④Postoperative ABG ≤20 dB was achieved in 54 patients （65.06%, 54/83） in the PORP group and 16 patients （43.24%, 16/37） in the TORP group. This difference in efficacy rates was statistically significant （P<0.05）. The overall efficacy rate for ossiculoplasty was 58.33% （70/120）. Conclusion: Patients with MECwTS exhibit more severe middle ear and mastoid pathology compared to those with MECw/oTS, resulting in poorer postoperative hearing levels and quality of life outcomes. Both PORP and TORP implantation can improve postoperative hearing to some extent; however, PORP appears to offer superior hearing improvement efficacy compared to TORP.

Rationale:Middle ear cholesteatoma (MEC) is a non-neoplastic cystic lesion that can cause severe complications if untreated. While subperiosteal abscesses, neck abscesses, and sigmoid sinus thrombophlebitis have been reported, an extensive deep neck abscess extending to the axillary fossa due to MEC has not been previously documented. This case highlights a deep neck abscess complicated by Proteus vulgaris infection during coronavirus disease 2019 (COVID-19) recovery, underscoring the importance of individualized management strategies based on pathogen characteristics.Patient concerns:A 27-year-old man with a history of left ear purulent otorrhea for > 20 years presented with neck swelling for 5 days during COVID-19 recovery.Diagnoses:Examination revealed: the left neck, up to the mastoid process, down to the ipsilateral armpit and lateral chest wall, and back to the trapezius muscle, was widely erythematous and swollen, with elevated skin temperature. Computed tomography revealed soft tissue density shadows in the left mastoid process, along with gas and pus accumulation in the left neck, pharynx, and axillary fossa. Culture results confirmed P vulgaris infection. The diagnosis was MEC complicated by a deep neck abscess and P vulgaris infection.Interventions:The patient underwent a modified radical mastoidectomy and incision and drainage of a left maxillofacial cervical multi-gap abscess. Postoperatively, the neck cavity was sutured, and negative pressure drainage tubes were placed. Due to subsequent parapharyngeal swelling and respiratory distress with a difficult airway, a tracheotomy was performed, followed by additional drainage of an abscess in the posterior oropharyngeal wall. Extensive necrosis of the cervical soft tissue necessitated reopening the neck incision and placement of Penrose drains for continued drainage.Outcomes:The patient recovered well following surgical interventions and tailored wound management, with no further complications.Lessons:This case underscores the importance of timely and appropriate abscess drainage and wound management tailored to the causative organism’s characteristics. It also highlights the need for aggressive treatment of the primary pathology (MEC) to prevent severe complications. Clinicians should be vigilant for unusual presentations of deep neck abscesses, especially in patients with recent infections such as COVID-19, which may complicate the clinical course.