Congenital cholesteatoma: what radiologists need to know.

Congenital cholesteatoma is a rare, non-neoplastic lesion that causes conductive hearing loss in children. It is underrecognized and often diagnosed only when there is an established hearing deficit. In the pediatric population, hearing deficiency is particularly detrimental because it can impede speech and language development and, in turn, the social and academic well-being of affected children. Delayed diagnosis leads to advanced disease that requires more extensive surgery and a greater chance of recurrence. A need to promote awareness and recognition of this condition has been advocated by clinicians and surgeons, but no comprehensive imaging review dedicated to this entity has been performed. This review aims to discuss the diagnostic utility of high-resolution computed tomography and magnetic resonance imaging in preoperative and postoperative settings in congenital cholesteatoma. Detailed emphasis is placed on the essential preoperative computed tomography findings that facilitate individualized surgical management and prognosis in the pediatric population.

Recurrent Cholesteatoma: Why it occurs?

A cholesteatoma is an expansion of keratinizing squamous epithelium that enters the middle ear cleft from the outer layer of the tympanic membrane or ear canal. Choleatomas are always treated surgically. Recurrence of the illness presents another challenge for the patient and the surgeon, though. There have been reports of recurrence rates as high as 30% in adults and as high as 70% in children. Here, we describe a case of persistent recurrent otorrhea following revision surgery, along with acquired recurrent cholesteatoma following canal wall down surgery. A 38-year -male with underlying Diabetes Mellitus and Hypertension presented with left scanty and foul-smelling ear discharge for 2 years and left reduced hearing. He was diagnosed with left chronic active otitis media with cholesteatoma for which he underwent left modified radical mastoidectomy, meatoplasty and tympanoplasty in 2017. Five months post operatively, he presented with left otorrhea. However, he defaulted followed up and presented in April 2018 for similar complaints. Otoscopy examination revealed left tympanic membrane perforation at poster superior quadrant of pars tensa and bluish discoloration behind pars flacida. He was diagnosed as recurrent left cholesteatoma and subsequently he underwent left mastoid exploration under general anesthesia in June 2018. Postsurgery, he developed recurrent ear discharge which was treated with topical antibiotics and ear toileting. We report a case of recurrent Cholesteatoma despite canal wall down procedure requiring a second redo procedure and with persistent recurrent otorrhea after the redo procedure.However, this case demonstrates the need for regular follow ups even after a canal wall down procedure for detecting recurrence of disease. Moreover, this case denotes some of the patient factors and surgeon factors involved in disease recurrence. Furthermore, importance of opting for an imaging study in case of high suspicion of the disease.

Is pre-operative audiometry a reliable predictor of ossicular chain condition?

OBJECTIVE: Investigation of ossicular chain (OC) status before surgery is important for preoperative patient consultation. This research aimed to investigate the relationship between pre-operative audiometric values and intra-operative OC condition in a relatively large population of chronic otitis media (COM) surgeries. METHODS: In this descriptive-analytic cross-sectional study, we evaluated 694 patients who underwent COM surgeries. We analyzed pre-operative audiometric data and intraoperative findings including ossicular anatomy, ossicular mobility, and the condition of middle ear mucosa. RESULTS: The optimal cut-off values of pre-operative speech reception threshold (SRT), mean air-conduction (AC), and mean air-bone gap (ABG) for predicting OC discontinuity were 37.5 dB, 37.2 dB, and 28.4 dB, respectively. For the prediction of OC fixation, the optimal cut-off points of SRT, mean AC, and mean ABG were 37.5 dB, 40.3 dB, and 32.8 dB, respectively. The computing of Cohen's d (95 % confidence interval) demonstrated the greater mean ABG in ears with OC discontinuity in comparison with ears with normal ossicles in all types of pathologies. There was a descending trend of Cohen's d from cholesteatoma to tympanosclerosis and then to granulation tissue and hypertrophic mucosa. There was a substantial relation between the type of pathology and OC status (P < 0.001). Ears with tympanosclerosis plaque had the most fixed OC among all types of pathologies (40 ears, 30.8 %), and ears with no pathology had the most normal OC (135 ears, 83.3 %). CONCLUSIONS: The results supported the view that pre-operative hearing is a key determining factor for the prediction of OC status.

[Anatomical factors of temporal bone residual cholesteatoma: our experience]. / Osobennosti stroeniya visochnoi kosti kak faktor retsidiva kholesteatomy: nash opyt.

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.

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

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

Comparison of endoscopic and microscopic management of attic cholesteatoma: A randomized controlled trial.

BACKGROUND: Attic cholesteatoma is a common disease encountered by otologists. OBJECTIVES: To compare the endoscopic approach to attic cholesteatoma with conventional microscopic technique. MATERIAL AND METHODS: A total of 190 patients (192 ears) diagnosed with attic cholesteatoma extending to the antrum area (stages Ib and II) were randomly assigned into two groups undergoing endoscopic approach and the other microscopic technique. The outcomes were preoperative and intraoperative findings, access to hidden areas expressed in MESVI, mean operative time from first incision to ear-packing, and postoperative findings. Statistical analysis was performed by SPSS version 24.0, and P ≤ 0.05 was considered statistically significant. RESULTS: The median Middle Ear Structural Visibility Index of the endoscopic group was better than the microscopic group (P < 0.05). The mean operating time by the endoscopic approach was less than the microscopic approach (P < 0.05). The median postoperative pain score in the endoscopic group was lower than the microscopic group (P < 0.05). In addition, there were no statistically significant differences in taste, hearing, vertigo, healing time and long term outcomes between the two groups. CONCLUSION AND SIGNIFICANCE: Endoscopic management of limited attic cholesteatoma showed definite advantages over the conventional microscopic approach, such as providing better visualization, requiring less postoperative time, subjecting the patients to less pain, and decreasing the incidence of complications.

Comparison of the EAONO/JOS, STAMCO and ChOLE cholesteatoma staging systems in the prognostic evaluation of acquired middle ear cholesteatoma in children.

OBJECTIVE: To compare the performance of the EAONO/JOS, STAMCO, and ChOLE Cholesteatoma Staging Systems in prognostic evaluation of children acquired middle ear cholesteatoma after primary surgery and identify the other factors that could predict cholesteatoma recidivism. And the correlation between the staging and the recidivism of cholesteatoma after grouping according to operation was evaluated. METHODS: A total of 123 ears of 118 patients that underwent surgery for primary cholesteatoma from November 2008 to May 2020 were included in this retrospective study, and then classified and staged according to the EAONO/JOS, STAMCO, and ChOLE cholesteatoma staging system, respectively. Each indicator involved in the system above was analyzed separately to evaluate its prognostic value for cholesteatoma recidivism. RESULTS: The type of surgical procedure performed (P = 0.020) was shown to be associated with cholesteatoma recidivism. Cholesteatoma location the supratubal recess (S1) (P = 0.026, HR = 3.614, 95% CI 1.137, 7.945), and the sinus tympani (S2) (P = 0.004, HR = 4.208, 95% CI 1.574, 11.250) were shown to be significantly associated with disease recidivism. When focusing on the CWU operation group, ossicular chain status in STAMCO stage (P = 0.043) and in the ChOLE stage (P = 0.018) were significantly associated with cholesteatoma recidivism. The results had shown no association between the three stages and cholesteatoma recidivism in the CWD and endoscopic surgery groups. CONCLUSIONS: Based on our study, the EAONO/JOS, STAMCO, and ChOLE Classifications have limited value in predicting cholesteatoma recidivism, in acquired middle ear cholesteatoma in children. Adding the pathological status of the ossicular chain may be useful for predicting the recidivism of cholesteatoma. Additional validation studies are entailed to definitively assess the clinical utility of these classifications.

[Case of diagnosis and surgical treatment of intracranial cholesteatoma]. / Sluchai diagnostiki i khirurgicheskogo lecheniya intrakranial'noi kholesteatomy.

The article provides a brief overview of the epidemiology of congenital and acquired intracranial cholesteatoma, the theory of its pathogenesis, classification, and clinical features. The most common localization of congenital and acquired intracranial cholesteatoma is described and various surgical approaches are presented that allow total removal of the formation. A clinical case of diagnosis and surgical treatment of acquired intracranial cholesteatoma caused by chronic suppurative otitis media is presented. A clinical example indicates the possibility of a long asymptomatic course of acquired intracranial cholesteatoma. The use of a combined (extradural subtemporal and transmastoid) surgical approach made it possible to completely remove the acquired cholesteatoma of the temporal lobe of the brain in a patient with chronic suppurative otitis media.

[Mastoid obliteration during last 5 years]. / Mastoidoplastika: tendentsii poslednikh 5 let.

The article presents modern approaches to mastoid obliteration in cases of chronic purulent otitis media with cholesteatoma, published in peer-reviewed russian and foreign journals from 2017 to 2021. The review tells us about effect of mastoid cavity obliteration on reducing the recurrence of cholesteatoma, improving the quality of life of patients according to the results of international questionnaires and the effect of surgery on hearing in the long-term postoperative period. An analysis of the use of autologous and biocompatible materials in middle ear surgery is presented. Their safety and ease of use during surgical intervention were evaluated.

[MIDDLE EAR CHOLESTEATOMA].

INTRODUCTION: Cholesteatoma is the most severe middle ear disease. In most cases cholesteatoma results from under-aeration of the middle ear cleft. It is characterized by otorrhea and hearing loss, and may lead to severe complications, intra- and extra-cranial. There is no non-surgical treatment for cholesteatoma. Surgical treatment is the only option. Cholesteatoma is diagnosed by micro-otoscopy. When cholesteatoma is suspected and otoscopy is inconclusive, imaging is mandatory. The goal of treatment in cholesteatoma is to achieve a stable and safe ear. In most cases, upon diagnosis, cholesteatoma is quite extensive and mastoidectomy is needed. Choice of surgery [Canal wall up mastoidectomy with tympanoplasty (the more conservative alternative), Canal wall down (radical) mastoidectomy with meatoplasty or Canal wall down (radical) mastoidectomy with reconstruction of external ear canal, tympanoplasty and mastoid obliteration], depends on the extent of the disease, the anatomic relationship between the sensitive structures that protrude into the walls of middle ear and mastoid cavities and the bony destruction that had already been caused by the disease. Hearing rehabilitation should lead to a stable, predictable, symmetric and as close as possible to normal hearing threshold. In many cases the solution is external or implantable hearing devices. Cholesteatoma has a high recidivism rate that leads to repeated surgery. In order to diagnose and treat cholesteatoma recidivism, a structured follow-up is needed after cholesteatoma surgery, with periodical otoscopy (every 6-12 months) and MRI (every 1-2 years). When cholesteatoma recidivism is diagnosed, surgery is indicated before the disease grows and becomes infected.

[Functional results of surgical treatment of patients with various types of acquired cholesteatoma]. / Funktsional'nye rezul'taty khirurgicheskogo lecheniya patsientov s razlichnymi tipami priobretennoi kholesteatomy.

OBJECTIVE: To study the long-term functional results of surgical treatment of patients with acquired cholesteatoma, their dependence on the age of patients, the type of cholesteatoma and the type of surgery. MATERIAL AND METHODS: The article presents the results of a dynamic comparison of the functional results of surgical treatment of 332 adults and 104 children with various forms of acquired middle ear cholesteatoma who underwent both closed and open surgical procedures. RESULTS: There were no statistically significant differences in hearing function into two age's groups depending on the type of cholesteatoma acquired. Average air conduction in adult patients for all types of cholesteatoma were ranked within 34.55-44.19 dB, air-bone gaps - 24.14-31.12 dB; in children - 33.72-39.16 dB and 27.33-34.95 dB, respectively. The exception was a group of adult patients with secondary cholesteatoma of the pars tensa. In the postoperative period, in patients of both age groups with all types of cholesteatoma, an improvement in the average values of air conduction by 18-20 dB and a decrease in the average values of the air-bone thresholds by 17-21 dB were noted. Analysis of the results of open surgical interventions showed a somewhat worse functional efficiency on 5-9 dB for air conduction average and 18-20 dB for air-bone gaps, both for adults and children. The stability of the achieved level of auditory function at all stages of postoperative observation was also noted. CONCLUSION: Surgical treatment of children and adult with all types of acquired cholesteatoma can improve hearing function and achieve stable results when using closed or open types of operations.

The evaluation of canal wall up cholesteatoma surgery with the Glasgow Benefit Inventory.

PURPOSE: The aim of the study was to investigate the change in health-related quality of life (HRQoL) after canal wall up cholesteatoma surgery, using the Glasgow Benefit Inventory (GBI). METHODS: Data from a consecutive group of 47 adults scheduled for primary cholesteatoma surgery using canal wall up (CWU) with obliteration, from January 2005 to December 2009, were analysed. Information was extracted from a medical database, and complementary data from patient files and audiograms were collected and recorded retrospectively. The GBI questionnaire was used for the assessment of HRQoL after surgery. RESULTS: There was no finding of residual or recurrent cholesteatomas in the study group. Hearing was improved at 1 and 3 years postoperatively. No patient suffered a total hearing loss. The overall GBI scores showed an improved HRQoL after surgery. Twenty-nine (85%) patients benefitted from surgery, 1 (3%) had no change, and 4 (12%) expressed deterioration. CONCLUSIONS: Cholesteatoma surgery using CWU with obliteration gives an improved HRQoL for the majority of patients. The GBI questionnaire provides complementary information to hearing and healing results after cholesteatoma surgery.

A tiny retraction of the pars flaccida may conceal an attic cholesteatoma.

OBJECTIVE: The purpose of this study was to evaluate the possibility of attic cholesteatomas concealed within a tiny retraction of the pars flaccida (classification of Tos and Poulsen type I or II attic retraction) in patients with an intact pars tensa of the tympanic membrane. METHODS: The clinical records of patients with a tiny retraction of the pars flaccida and an intact pars tensa of the tympanic membrane who presented to the ear clinic of a tertiary care medical center for the first time between March 2012 and February 2015 were retrospectively reviewed. All patients who had an abnormal pars flaccida of the tympanic membrane were recommended to undergo temporal bone computed tomography (CT) scans. In cases of a soft tissue density lesion within Prussak's space, an exploratory operation was recommended. RESULTS: Among 1320 adult patients, 146 patients (n = 168 ears) who had a tiny attic retraction with a normal pars tensa in unilateral or bilateral ears underwent temporal bone CT scans, and 18 ears had a soft tissue density lesion within Prussak's space. Among the ears with a tiny retraction of the pars flaccida and a normal pars tensa, an attic cholesteatoma was suspected in 10.7% (n = 18 ears) of cases based on the CT scans. After exploratory operations, 2% of patients who underwent CT scans (3 out of 146 patients) and 23% of patients who had a soft tissue density lesion within Prussak's space on CT scans (3 out of 13 operations) had an attic cholesteatoma. CONCLUSION: All attic retractions which are even in cases of Tos type I or II should be examined closely using endoscopy, microscopy, and, if necessary, temporal bone CT scan.

Middle-ear cholesteatoma co-existing with labyrinthine fistula and vestibular schwannoma.

BACKGROUND: Many conditions, among them vestibular schwannoma and middle ear cholesteatoma with lateral semicircular canal destruction, may be associated with asymmetrical sensorineural hearing loss (SNHL) and vertigo. However, the probability that these two distinct disease entities causing the same symptoms occur in a single patient is very low, approximately 1 per 28 billion per 1 year. METHODS: We present the case of a 40-year-old male admitted to our clinic because of chronic middle ear inflammation with concomitant tinnitus vertigo, and deafness in the right ear. The patient was diagnosed with lateral semicircular canal fistula caused by middle-ear cholesteatoma and concomitant vestibular schwannoma. Canal wall-down surgery was carried out to remove the cholesteatoma, followed by gamma knife radiosurgery for the vestibular schwannoma. RESULTS: Vertigo and tinnitus resolved within 3 days after the ear surgery, and gamma knife treatment resulted in the complete involution of the vestibular schwannoma. The patient presented with completely dry middle-ear cavity and no recurrence of the cholesteatoma was observed during a 3-year follow-up. CONCLUSION: As the hereby reported condition is very rare, the results cannot be compared with any similar report published previously. Nevertheless, based on the outcome, the treatment strategy seems to be both reasonable and effective.

[The experience of surgical treatment of a patient with a cholesteatoma of the temporal bone pyramid]. / Opyt khirurgicheskogo lecheniya patsienta s kholesteatomoi piramidy visochnoi kosti.

The article describes the clinical case of the cholesteatoma of the temporal bone pyramid, leading to a massive destruction of the surrounding structures. Attention is drawn to the importance of timely diagnosis of this pathology with the use of objective visualization methods to select the optimal surgical approach, which allows to completely remove this formation.

Bioactive glass granules for mastoid and epitympanic surgical obliteration: CT and MRI appearance.

PURPOSE: To evaluate the appearance of mastoid and epitympanic obliteration using S53P4 bioactive glass (BG) granules in high-resolution computed tomography (HRCT) and MRI. MATERIALS AND METHODS: Patients undergoing mastoid and epitympanic obliteration between May 2013 and December 2015 were prospectively included in an uncontrolled clinical study. All patients underwent a temporal HRCT scan 1 year after surgery, aimed at evaluating the attenuation, homogeneity, and osseointegration of the BG granules, as well as the ventilation of the middle ear and the volume of the obliterated paratympanic spaces. If a cholesteatoma was found during surgery, additional MRI, including at least pre- and post-contrast T1-weighted, T2-weighted, and axial non-echo-planar diffusion-weighted (DW) sequences, was performed 1 year after surgery, to study the normal signal of the BG granules and the presence of residual cholesteatoma and/or other temporal bone pathologies. RESULTS: Seventy cases were included. On 1-year HRCT, the mean attenuation of the BG granules was 888.34 ± 166.10 HU. The obliteration was found to be mostly homogeneous with partial osseointegration. The appearance of the BG granules having a low-intensity signal in T2-weighted imaging and DW MRI was always different from the appearance of cholesteatoma. A longer follow-up has shown no attenuation or signal modification of the BG granules compared with the 1-year imaging. CONCLUSION: Radiological follow-up of patients operated on with mastoid and epitympanic obliteration using BG granules is effective using both HRCT and MRI. A cholesteatoma and/or other potential complications could easily be detected due to the specific radiological appearance of the BG granules. KEY POINTS: • The appearance of mastoid and epitympanic obliteration by S53P4 bioactive glass (BG) granules on high-resolution computed tomography (HRCT) scans was homogeneous with an attenuation significantly higher than the attenuation of cholesteatoma and lower than mastoid bone attenuation. • The granules have a low-intensity signal on non-echo-planar diffusion-weighted sequences and on T2-weighted images and present contrast enhancement allowing the differential diagnosis with cholesteatoma and effective for the detection of other underlying temporal bone pathologies. • The volume and radiological appearance of the obliteration appear to be stable with time.

Three-dimensional reversed fast imaging with steady-state precession diffusion-weighted imaging for the detection of middle ear cholesteatoma.

AIM: To determine the usefulness of three-dimensional reversed fast imaging with steady-state precession diffusion-weighted imaging (3D-PSIF DWI) for the detection of middle ear cholesteatoma. MATERIALS AND METHODS: The study population consisted of 81 patients who underwent 3D-PSIF-DWI at 3 T. They included cholesteatoma in 73 cases, otitis media in five, and cholesterol granuloma in three. Two observers independently performed qualitative evaluations for the detection of cholesteatoma and measured apparent diffusion coefficient (ADC) values and ADC ratios of the lesions. Kappa (κ) statistics, the intraclass correlation coefficient (ICC), the independent t-test, and receiver operating characteristic (ROC) analysis were used for statistical analysis. Pair-wise comparison of the ROC curves was performed using the area under the ROC curve (AUC). RESULTS: Interobserver agreement and ICC for the qualitative and quantitative evaluations were excellent (κ=0.92 and ICC=0.90-0.92, respectively). The ADC value and the ADC ratio were significantly lower for cholesteatoma than non-cholesteatoma lesions (p<0.0001). In <5 mm cholesteatoma group, the diagnostic performance of the ADC value (AUC=0.97) and the ADC ratio (AUC=1) was significantly superior to qualitative 3D-PSIF-DWI (AUC=0.76; p=0.0001 and <0.0001, respectively). For ≥5 mm cholesteatoma group, there were no significant differences in diagnostic performance among the three parameters. CONCLUSION: 3D-PSIF-DWI sequence is useful for the detection of middle ear cholesteatomas, especially <5 mm lesions.

Facial canal dehiscence in patients with cholesteatoma: concordance between intraoperative inspection, computed tomography and neurophysiological findings.

OBJECTIVES: To assess the diagnostic capacity of intraoperative neurophysiological monitoring with respect to "gold standard" microscopic findings of facial canal dehiscence in middle ear cholesteatoma surgery. STUDY DESIGN, PATIENTS AND SETTING: We carried out a retrospective cohort study of 57 surgical interventions for cholesteatoma between 2008 and 2013 at Hospital Universitario de Canarias, Spain. DIAGNOSTIC INTERVENTIONS: Each patient underwent preoperative computed tomography (CT), intraoperative neurophysiological monitoring and intraoperative inspection of the facial nerve during microsurgery. Diagnostic concordance on the presence/absence of facial canal dehiscence was assessed in 54 surgical interventions. MAIN OUTCOME: Presence of facial canal dehiscence. RESULTS: Of 57 interventions, 39 were primary surgeries; 11 (28.2%) showed facial canal dehiscence. and 18 were revision surgeries; 6 (33.3%) showed facial canal dehiscence. The facial nerve was not damaged in any patient. Facial canal dehiscence was observed in 17 (29.82%) interventions. We used intraoperative microscopic findings as the gold standard. Neurophysiological study showed a sensitivity of 94.1, specificity 97.3, positive predictive value (PPV) 57.8 and negative predictive value of 97.2. CT showed a sensitivity of 64.7, specificity 78.4, PPV 57.8 and negative predictive value of 82. CONCLUSIONS: Our neurophysiological study showed greater sensitivity and higher PPV than CT for the detection of facial canal dehiscence. We found no relationship between disease progression time and the presence of facial canal dehiscence.

[Is routine histopathologic examination of middle ear cholesteatoma necessary?] / Ist die routinemäßige histologische Untersuchung von Mittelohrcholesteatomen nötig?

BACKGROUND: In the course of restoration of the middle ear, routine histological examinations are initiated in many cases, although a middle ear cholesteatoma can be clinically diagnosed in a normal case. AIM OF THE STUDY: To determine the correlation between the surgeons' diagnosis and that of the pathologist and comparison with literature data. To check the rate of unexpected diagnoses. Analysis of the costs and survey of German ENT clinics with regard to handling of the histological examination in suspected cholesteatoma. MATERIALS AND METHODS: Retrospective evaluation of cholesteatoma cases of the ENT Department of the University Medical Center of Mainz from 2010-2015. Germany-wide postal survey of ENT clinics. RESULTS: In all, 449 operations for the rehabilitation of a middle ear cholesteatoma, of which there were 312 (69.5%) first diagnoses and 137 (30.5%) recurrences. A histological examination was conducted in 78.6%. For first diagnoses, the sensitivity of the clinical assessment was 97.9%, specificity 10%, positive predictive value 96.3%, and negative predictive value 16.7%. Recurrences showed values of 100%, 40%, 97.1% and 100%. Unexpected malignant findings did not occur. This routine histological examination was performed by 58.6% of German ENT hospitals. A total of 80% of those questioned this to be useful. A histological examination cost an average of 14.06 €. CONCLUSION: Intraoperatively, there is a high degree of accuracy in diagnosing cholesteatoma. The cost factor of the histological examination is low and should not be the basis for the decision. The ear microscopy and the experience of the ear surgeon should be decisive for the decision for histological examination.

[Clinical and diagnostic observations of a complicated course of chronic otitis media, cholesteatoma in children]. / Kliniko-diagnosticheskie nabliudeniia oslozhnennogo techeniia khronicheskogo gnoinogo kholesteatomnogo srednego otita u detei.

Formation of labyrinth fistulas and destruction of the facial nerve canal in children with middle ear cholesteatoma is rare. Data of children with cholesteatoma of the middle ear, operated in the ENT department of MONIKI, is analyzed. Several clinical observations of multiple-aged patients with the presence of cholesteatoma intra-temporal complications are presented. The preoperative computer tomography made it possible to diagnose the presence of the labyrinth fistula and the destruction of the facial nerve channel, which was confirmed intraoperatively.