Congenital Middle Ear Cholesteatoma Research Articles

Diagnosis and Management of Unexplained Conductive Hearing Loss With Intact Tympanic Membrane: A Systematic Review.

Objective: To analyze the etiology, diagnosis, and treatment of unexplained conductive hearing loss (UCHL) with intact tympanic membrane. Methods: A systematic review was conducted based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 642 articles were retrieved from databases such as PubMed, Embase, Web of Science, and Cochrane. Fifty-four research articles and 21 case reports were screened out according to the inclusion and exclusion criteria for analysis of the etiology of UCHL. Seven research articles with UCHL who underwent exploratory tympanotomy were selected for data extraction and analysis of clinical characteristics. Results: UCHL is a common manifestation of various diseases, including congenital ossicular anomalies (COA), otosclerosis (OTS), congenital middle ear cholesteatoma (CMEC), oval window atresia, superior semicircular-canal dehiscence, congenital stapedial footplate fixation, middle ear osteoma or adenoma, congenital ossification of stapedial tendon, and so on. A total of 522 patients were included in the 7 articles; among whom OTS showed a tendency to increase with age. The main symptoms were hearing loss, followed by tinnitus, dizziness, ear fullness, ear pain, facial paralysis. A total of 87.5% to 93.0% patients with COA manifested as nonprogressive deafness that occurred since childhood, with tinnitus incidence of 15.6% to 30.2%, and 86.4% to 96.4% patients with OTS presented with progressive hearing loss, with tinnitus incidence of 60.1% to 90.9%. The diagnosis positive rate of high-resolution computed tomography (HRCT) was 33.8% to 87.1%, and CMEC was higher than that of COA (83.3%-100% vs 28.6%-64%). All the articles reported good hearing recovery. The most common surgical complications included taste abnormalities, tinnitus, and dizziness. Conclusion: UCHL presents with similar clinical manifestations and poses challenges in preoperative diagnosis. Exploratory tympanotomy is the primary method for diagnosis and treatment, with good prognosis after removing the lesion and reconstructing hearing during the operation. Children can also safely undergo the surgery.

Treatment of congenital middle ear cholesteatoma in children using endoscopic and microscopic ear surgeries: a case series

IntroductionSurgical removal is widely employed in children with congenital middle ear cholesteatoma (CMEC). Here, we report the surgical outcomes of CMEC removal via endoscopic ear surgery (EES) and microscopic ear surgery (MES) in children.MethodsChildren with CMEC who underwent preoperative medical history inquiry, hearing test, endoscopic evaluation, and radiology imaging before receiving EES or MES were included. Postoperative audiological outcomes and recurrence rates were collected.ResultsSeventeen children (20 ears) with stage II-IV CMEC were included. Of those, 11 ears (55.0%) underwent EES, and 9 ears (45.0%) underwent MES. The follow-up time was 35 ± 13.5 months. One child in the EES group with stage III CMEC had a recurrence during the follow-up period. In the EES group, the average minimum diameter of the external auditory canal on the affected side was 5.8 mm (4.3–8.0 mm). No linear association was found between age and the minimum diameter of the external auditory canal.DiscussionEES is a promising treatment option for children with early-stage CMEC because of its low recurrence rate and minimally invasive nature. The minimum diameter of the external auditory canal on the affected side should be meticulously examined when performing EES in children.

Fusion computed tomography-magnetic resonance imaging scans for pre-operative staging of congenital middle-ear cholesteatoma.

To evaluate if fusion computed tomography-diffusion-weighted magnetic resonance imaging may have a role in the pre-operative assessment of congenital middle-ear cholesteatoma. A retrospective chart review of surgically treated congenital middle-ear cholesteatoma patients over a 2-year timespan was conducted. Pre-operative staging was performed on computed tomography and fusion computed tomography-diffusion-weighted magnetic resonance imaging based on extension of the disease according to the ChOLE classification system and the Potsic classification system. Intra-operative staging was compared to imaging findings to evaluate accuracy of the two imaging modalities in predicting congenital middle-ear cholesteatoma extent. Computed tomography was able to correctly predict congenital middle-ear cholesteatoma extent in three out of six cases according to the ChOLE classification system, all of which were staged as Ch1a and Ch1b on pre-operative computed tomography. Cases in which computed tomography was not able correctly to determine congenital middle-ear cholesteatoma extent were staged as Ch3 on pre-operative computed tomography. Fusion scans correctly determined congenital middle-ear cholesteatoma extent in all cases according to the ChOLE classification. Fusion computed tomography-diffusion-weighted magnetic resonance imaging may be helpful in cases of congenital middle-ear cholesteatoma where pre-operative computed tomography shows mastoid and antrum opacification, in which computed tomography alone may overestimate cholesteatoma extension beyond the level of the lateral semi-circular canal.

Etiology analysis and diagnosis of noninflammatory conductive hearing loss in children

Objective:To investigate the etiology, diagnosis and treatment of noninflammatory conductive hearing loss in children. Methods:The clinical data of children patients admitted to the Eye & ENT Hospital of Fudan University from January 2019 to November 2022 were retrospectively analyzed. Results:A total of 179 cases（189 ears） were analyzed. The main symptoms from high to low were: ear tightness, hearing loss, earache, and facial paralysis. The degree of hearing loss was mild in 34 ears（19.5%）, moderate in 70 ears（40.2%）, moderate-severe in 52 ears（29.9%）, severe in 18 ears（10.3%）. The mean hearing threshold of otosclerosis was the highest（63.5±7.8） dB HL, and the mean air-bone gap of ossicular chain malformation was the largest（35.4±9.8） dB HL. The mean hearing threshold of the affected ear was（50.4±14.5） dB HL, and the mean air bone gap was（30.3±10.4） dB HL. After operation, the results were（36.1± 14.5） dB HL and（20.0±8.6） dB HL, respectively. Distribution of surgical methods for ossicular chain reconstruction: 88 ears（46.6%） of TORP, 49 ears（25.9%） of PORP, 8（4.2%） ears of Piston, 9 ears（4.8%） of autogenous ossicular reconstruction, and 35 ears（18.5%） of ossicular chain relaxation. CT diagnostic rate showed more sensitivity to malleus and incus abnormalities, the diagnosis rate of congenital middle ear cholesteatoma was the highest. The mean duration time of diagnosis was（2.2±2.9） years, while the diagnosis of ossicular chain malformation（[5.2±4.2]years）, otosclerosis（[4.4±4.1]years）, tympanosclerosis（[5.4±0.9]years） took longer. Conclusion:In the diagnosis and treatment of noninflammatory conductive hearing loss in children, the combination of detailed medical history, specialized examination and imaging examination can maximize the accuracy of diagnosis and achieve the purpose of personalized comprehensive treatment. Surgical intervention with appropriate timing is important to remove lesions and improve hearing.

Transcanal endoscopic treatment for congenital middle ear cholesteatoma in children.

To investigate the feasibility and efficacy of transcanal endoscopic treatment for congenital middle ear cholesteatoma in children.Eleven children diagnosed with congenital middle ear cholesteatoma, who underwent total ear endoscopic surgery under general anesthesia, were included from the Huazhong University of Science and Technology Union Shenzhen Hospital between January 2016 and December 2020. We retrospectively analyzed their operation process and surgical complications through the surgical video; moreover, we compared the pre- and postoperative hearing outcomes.One child underwent a planned second operation to reconstruct the ossicular chain. At 6 postoperative months, all 11 children underwent reexamination. There was no significant change and a significant decrease in the mean bone and air conduction hearing thresholds, respectively (P > .05 and P < .05); moreover, there was a significant reduction in the air–bone conduction difference (P < .05). Further, the air–bone conduction difference was reduced to >20 dB and >10 dB in 11 and 7 children, respectively. Follow-up of the children did not reveal sensorineural deafness, facial paralysis, and other serious complications; further, there were no cases of recurrence.Transcanal endoscopic treatment for congenital middle ear cholesteatoma in children is feasible, minimally invasive, and functional.

Immunohistochemical localization of d-β-aspartic acid in congenital and acquired middle ear cholesteatoma.

Objective/HypothesisMiddle ear cholesteatoma is characterized by abnormal growth of the keratinizing squamous epithelium of the temporal bone. d‐β‐aspartic acid is the major isomer of d‐aspartic acid found in elderly tissue. We assessed the immunoreactivity to k‐β‐aspartic acid of congenital and acquired middle ear cholesteatomas.Study DesignCase–control studies.Material and MethodsTissue samples were collected from 21 patients comprising 21 ears with congenital middle ear cholesteatoma and 26 patients comprising 29 ears with acquired type. Their clinical and histopathological features were investigated. We divided the middle ear cholesteatoma samples into three layers: the perimatrix, matrix, and cystic contents. The patterns of immunoreactivity to d‐β‐aspartic acid expression were then assessed immunohistochemically.ResultsTwo patterns of immunoreactivity to d‐β‐aspartic acid were detected in middle ear cholesteatoma: infiltrative and diffuse. In congenital middle ear cholesteatoma, d‐β‐aspartic acid expression was observed throughout all the layers (perimatrix, matrix, and cystic contents), and immunoreactivity to d‐β‐aspartic acid was dramatically strong in all layers. The expression levels of d‐β‐aspartic acid to the cystic content and perimatrix were significantly higher in congenital middle ear cholesteatoma than in the acquired type.ConclusionsThis study showed the expression levels of d‐β‐aspartic acid in middle ear cholesteatoma to differ significantly between congenital and acquired middle ear cholesteatoma. Our results indicate that overexpression of d‐β‐aspartic acid is likely to be involved in the pathogenesis of cholesteatoma, and we speculate that d‐β‐aspartic acid could be a novel biomarker for, and a therapeutic target in, congenital and acquired middle ear cholesteatoma.Level of Evidence4

Surgical Treatment Of Congenital Cholesteatoma of Middle Ear at Children

Surgical Treatment Of Congenital Cholesteatoma of Middle Ear at Children

Clinical Effect of Ear Endoscopic Intervention on CMEC Patients and Analysis of the Relationship between ROS, P-Akt, and HIF-1α Expression and the Degree of Bone Destruction.

The clinical efficacy of ear endoscopic intervention in patients with congenital middle ear cholesteatoma (CMEC) is explored, and the relationship between the expression of reactive oxygen species (ROS), phosphorylated protein kinase B (P-Akt), hypoxia-inducible factor-1 α (HIF-1α) and the degree of bone damage are analyzed. A total of 72 CMEC patients admitted to the otolaryngology department of our hospital from 2019 to January 2021 for surgical treatment are selected. According to the different intervention methods, the microscope group and the otolaryngology intervention group are established, respectively, with 36 patients in each group. The patients in the microscope group are treated with a microscope for middle ear cholesteatoma surgery, and the patients in the otoscope intervention group are treated with an otoscope for middle ear cholesteatoma surgery. The experimental results show that ear endoscopic intervention has better clinical efficacy for CMEC patients, which can effectively shorten the operation time, reduce the incidence of postoperative complications, and effectively improve the hearing of patients.

The Role of Otoscopy in the Diagnosis of Rare Diseases in Pediatrics. Congenital Middle Ear Cholesteatoma in the Child: Clinical Case

The clinical recommendations “Acute respiratory viral infection (ARVI) in children" indicate that otoscopy should be a part of routine pediatric examination of each patient along with auscultation, percussion, etc. Nowadays, there are no legal regulations on which specialists can perform otoscopy. Thus, there is significant pediatricians’ interest in otoscopy, especially in diagnosis of acute otitis media (AOM) during primary examination for timely antibacterial management. Moreover, pediatricians could reveal such rare and very aggressive middle ear disease as cholesteatoma, its early diagnosis can prevent the development of any complications and determines the range and quality of rehabilitation actions.

The Tensor Tympani Tendon: A Hypothetical Site of Origin of Congenital Cholesteatoma.

Multiple theories have been discussed about the etiopathogenesis of congenital middle ear cholesteatoma (CMEC) and its specific site of origin. The intraoperative identification of the precise location of the keratinous mass is important to guarantee its complete removal, in order to reduce the risk of recurrence. This study proposes the tensor tympani tendon (TTT) as a possible site of origin of CMEC. All CMECs treated between 2013 and 2019 were reviewed. Only Potsic stage I lesions were included. Preoperative radiologic images were compared to intraoperative findings. Three removed TTT were sent for histologic evaluation. Seven patients were included (M:F = 3:4). Preoperative CT images were classified as type A in 2 cases (28.6%) and type B in 5 cases (71.4%). At intraoperative evaluation all CMEC sacs were found pedunculated on the TTT. The histologic examinations confirmed the connection between the cholesteatomatous sac and the TTT. According to the correlation of imaging, intraoperative findings and histology, we proposed that the TTT could be the primary site from which CMEC originates.

Suggestion of a Modified Classification for Congenital Middle Ear Cholesteatoma: Based on the Clinical Characteristics and Staging of Fifty-Seven Patients.

Objective: To explore more refined classification methods of congenital middle ear cholesteatoma (CMEC) based on two existing staging systems. Subjects and Methods: This study involved a retrospective data review of 57 patients (61 ears involved) with CMEC requiring the surgical treatment. Patients were classified into different stages according to Nelson, Potsic, and Modified Nelson staging system. Preoperative data and intraoperative findings were recorded. Results: The mean age at operation was 15 ± 15.04 years with a median of 10 years. The main clinical manifestation was hearing loss (72.13%). CMEC mass was mainly located in the posterior portion of the tympanic cavity (65.57%). No patient was classified into Potsic stage II. The erosion of incus happened in all cases. Patients with Nelson type 2 and type 3 had erosions to the structures out of middle ear, such as dura mater, lateral semicircle canal, and facial canal. Postoperative follow-up time was more than 24 months. Recurrence occurred in four patients (6.56%), all of them in Nelson type 2, who had received canal wall down mastoidectomy (three cases) and canal wall up mastoidectomy (one case). Conclusions: Nelson staging system was more suitable for advanced CMEC patients than Potsic staging system. The rare case of Potsic stage II restricted the application of Potsic staging system. Moreover, since both of two staging systems do not distinguish the type of involved ossicles, the authors recommended to subdivide Nelson type 2 into type 2a and type 2b based on the erosion of the ossicular chain, as well as subdivide Nelson type 3 into type 3a and 3b based on the erosion of structures out of middle ear, which was named as Modified Nelson staging system.

Characteristics of diagnosis and surgical treatment of various forms of congenital temporal bone cholesteatoma

To conduct a retro- and prospective analysis of the clinical characteristics and results of surgical treatment of patients with congenital temporal bone cholesteatoma. On the basis of the SPb Scientific Research Institute of ENT of the Ministry of Health of Russia 23 patients were diagnosed with congenital temporal bone cholesteatoma for the period from 2011 to 2018. There were 11 men (47.8%), 12 women (52.2%), the age of patients - from 2 to 44 years. The M.J. Levenson criteria were used to verify congenital middle ear cholesteatoma. (1986). According to intrasurgery findings, we identified congenital cholesteatoma of the middle ear stages according to the ChOLE classification. Other types of congenital temporal bone cholesteatoma included the patients with congenital atresia of the external auditory opening, combined with congenital temporal bone cholesteatoma; we found a patient with exostoses obstructing the external auditory canal combined with congenital cholesteatoma of the middle ear; and a patient with congenital cholesteatoma of the temporal bone pyramid. An increase in the incidence of congenital temporal bone cholesteatoma was noted, which is probably due to increased awareness of specialists and the wider use of imaging research methods. A relationship was revealed between the stage of congenital cholesteatoma according to the ChOLE classification and the necessary volume of surgical intervention. Of the total number of cases of congenital temporal bone cholesteatoma, 26.1% were the patients with anomalies in the development of the outer and middle ear. All patients with suspected congenital temporal bone cholesteatoma require a temporal bone MSCT and middle ear MRI in DWI mode.

Is transcanal tympanoplasty an appropriate surgical treatment for congenital middle ear cholesteatoma with ossicular involvement?

ObjectivesThe aims of this study are to analyze the clinical characteristics of congenital middle ear cholesteatoma (CMC), to evaluate the treatment results according to the types of surgical approach, and to suggest the appropriate surgical treatment option in each stage. MethodsOne hundred fifteen children (≤15 years old) with surgically confirmed CMC, who underwent surgery at a tertiary hospital during 1994–2012 and were followed up more than 2 years, were enrolled in this study. CMC was classified into four stages by the staging system proposed by Potsic. Clinical characteristics of CMC were analyzed and its association with the rate of residual disease was evaluated. ResultsBased on the location of the lesion, posterior-origin CMCs were detected significantly later (5.5 years old, P = 0.018) and more likely to be in the Stage 3 or 4 (P < 0.001). Residual CMCs were observed in 10 out of 115 cases (8.7%) and were more frequent in the Stage 3 or 4 patients (P = 0.007). However, the rate of the residual disease was not statistically different between anterior- and posterior-origin CMCs (P = 0.101). All 58 cases of CMC in the Stage 1 or 2 were successfully removed by transcanal tympanoplasty with only one residual case (1.7%). In the Stage 3 cases, transcanal tympanoplasty was conducted in 24 out of 29 patients, and the residual disease rate was 12.5% (3 out of 24). ConclusionsThe early stages of CMC were likely to be diagnosed at younger age, and the early diagnosis seemed to show better surgical outcomes with less invasive techniques. Transcanal tympanoplasty can be an effective surgical option for CMCs in the Stage 3.

Clinical treatment of congenital middle ear cholesteatoma in children

Objective:To summarize the clinical characteristics and clinical treatment of congenital cholesteatoma (CC) of the middle ear in children, provide early diagnosis methods and explore standardized diagnosis and treatment plan.Method:A retrospective chart review of 94 patients with a diagnosis of middle ear cholesteatoma, in Beijing Children's Hospital, between 2009 and 2015 was carried on. 14 patients with CC were identified using the criteria proposed by Levenson, and were divided into two groups according to the course of disease. The course of disease in group A was less than 3 months, and group B was more than 3 months. The main complains, diagnostic methods and Potsic's stage of temporal bone CT findings were recorded.Result:①The age of 14 cases of congenital cholesteatoma of the middle ear ranged from 3.33 to 10.17 years, with the median age of 7.20 years. ②Hearing loss (13/14, 92.86%) was the most common complain. Finding methods included hearing screening and CT scan (11/14, 78.57%), tympanotomy (2/14, 14.28%) and otoscopic examination (1/14, 7.14%). ③There were 6 people in group A and 8 in group B. According to Potsic's grading standard, the difference between the two groups was statistically significant (P=0.043). ④The preoperative Air-Bone conduction threshold Gap (ABG) in A and B two groups were (38.10±7.43) dB and (42.09±9.96) dB, respectively, and there was no significant difference in analysis (P=0.427). ⑤The difference between pre-ABG and post-ABG ［(36.26±5.56）dB and （21.70±3.80）dB, P=0.004］ was significant. Canal wall up mastoidectomy was the preferred procedure and 11/14 (78.57%) patients had this surgery done.Conclusion:The shorter the course of disease, the lower the stage of cholesteatoma of the middle ear indicates the importance of early detection. But congenital cholesteatoma is more occult, and even within 3 months, cholesteatoma can cause severe damage to the hearing and middle ear structure. Early screening programs can recommend hearing screening and CT scan to facilitate early intervention.

Congenital middle ear cholesteatoma in childhood - Α case report

We describe a case of an 11-year-old patient suffering from left side conductive hearing loss and persistent earache in the last five months. The imaging study with MRI revealed soft tissue mass between the left middle ear, Atticus dome and the apex of the petrosal bone, compatible with congenital cholesteatoma. The purpose of this study is to describe the MRI characteristics of congenital cholesteatoma, as well as to point out the necessity of the use of MRI for the early detection of the disease, that can lead to furher extension and development of complications. Πeριγράφουμe την πeρίπτωση μιας ασθeνούς ηλικίας 11 eτών που προσήλθe στο ιατρeίο μe βα- ρηκοΐα αγωγιμότητας και eμμένουσα ωταλγία από πeνταμήνου. Η απeικονιστική μeλέτη μe μαγνητική τομογραφία ανέδeιξe μeταξύ του αριστeρού μέσου ωτός, του Αττικού θόλου και της κορυφής του λι- θοeιδούς οστού πρόσθeτο ιστό, eικόνα συμβατή μe συγγeνές χολοστeάτωμα του μέσου ωτός. Σκοπός eίναι να eπισημάνουμe την αναγκαιότητα της χρήσης της μαγνητικής τομογραφίας ως προς την έγκαι- ρη αναγνώριση των καταστάσeων που μπορeί να οδηγήσουν στην ανάπτυξη του χολοστeατώματος και την αντιμeτώπισή τους πριν eξeλιχθούν.

Congenital Cholesteatoma of middle ear in children (14 cases experience)

Congenital Cholesteatoma of middle ear in children (14 cases experience)

Surgical management of congenital middle ear cholesteatoma

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Temporal bone HRCT features of the congenital middle ear cholesteatoma

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先天性真珠腫にかかわる諸問題

先天性真珠腫にかかわる諸問題

Risk Factors of Post-Operative Recidivism in Congenital Cholesteatoma of the Middle Ear

Received May 7, 2014 Revised June 23, 2014 Accepted June 26, 2014 Address for correspondence Hyung-Jong Kim, MD, PhD Department of OtorhinolaryngologyHead and Neck Surgery, Hallym University College of Medicine, 22 Gwanpyeong-ro 170beon-gil, Dongan-gu, Anyang 431-796, Korea Tel +82-31-380-3841 Fax +82-31-386-3860 E-mail hjk1000@hallym.ac.kr Background and ObjectivesZZThere is no general consensus on the risk factors affecting the recurrence or residual disease (recidivism) after surgery of congenital cholesteatoma of the middle ear. In this study, we analyzed clinical characteristics of recidivistic cases after the surgery and compared those of non-recurrent cases to investigate the risk factors regarding the post-operative recidivism of the disease. Subjects and MethodZZData were collected from retrospective chart reviews and computerized database of patients who have underwent surgeries at Kangdong and Hallym University Sacred Heart Hospital during the last 24 years. All surgeries, consisting of 95 primary cases and 24 revision cases, were performed by the same surgeon. ResultsZZRecurrence was detected in 24 cases of the 95 patients (including 4 cases, of which primary surgeries were done at other hospitals). Among the risk factors affecting post-operative recidivism such as sex, age, symptom, disease duration, type of cholesteatoma, mastoid pneumatization, ossicle status, and stage of disease, factors of longer symptom duration, poor mastoid pneumatization, older age, poor mastoid pneumatization, advanced or recurrent cholesteatoma were significantly related to the recidivism of disease. ConclusionZZLonger symptom duration, poor mastoid pneumatization, and advanced or recurrent cholesteatoma were revealed as high risk factors for post-operative recidivism in this study. Korean J Otorhinolaryngol-Head Neck Surg 2014;57(10):664-70