Evaluation of significant gene expression changes in congenital and acquired cholesteatoma.

Etiopathogenesis of acquired and congenital cholesteatoma is still unclear. The clinical behavior of adult acquired, pediatric acquired and congenital cholesteatomas show differences. The scope of the this study was to detect the matrix metalloproteinase (MMP), tissue inhibitors of metalloproteinase (TIMP) and epidermal growth factor receptor (EGFR) gene expression changes in cholesteatoma perimatrix and to compare these changes among congenital cholesteatoma, adult acquired cholesteatoma and pediatric acquired cholesteatoma. A total of 16 genes including MMPs, TIMPs and EGFR were analyzed in the samples of 32 cholesteatoma tissues. Real-time PCR was used for detection of the gene expression levels. Data analyses were achieved by ΔΔCT method (Light Cycler 480 Quantification Software) and Statistical Package for Social Sciences (SPSS) version 22.0. The expression levels of MMP-2, -9, -10, -11, -13, -14, -15, -16 and EGFR genes were significantly higher in acquired cholesteatoma than healthy tissue (p < 0.05). There was a statistically significant decrease (3.34 times more) in the mean TIMP-2 gene expression level in acquired cholesteatoma compared to healthy tissue (p < 0.05). There was a significant increase in the mean expression level of MMP-7 gene and a decrease in the mean expression level of TIMP-1 gene (3.12 times more) in congenital cholesteatoma compared to healthy tissue (p < 0.05). This study indicates that increased expression levels of some particular MMP genes and EGFR gene and decreased expression levels of TIMP genes may play an important role in the development of cholesteatoma. Further, MMP-9, MMP-13 and MMP-14 genes may have a remarkable role in the development of more aggressive cholesteatoma forms. The authors concluded that overexpression of MMP-9, MMP-13 and MMP-14 may cause stronger inflammation associated with cholesteatoma.

Iatrogenic cholesteatoma originating from a misplaced tympanomeatal flap during tympanoplasty: a series of five patients.

PURPOSE: To report our experience of a sequence of events that resulted in an iatrogenic cholesteatoma originating from the external auditory canal (EAC) years after tympanoplasty that had included a tympanomeatal flap. METHODS: Data on the presentation and pathogenesis of iatrogenic cholesteatomas arising from misplaced tympanomeatal flaps during tympanoplasty without mastoidectomy were retrieved from the patients' medical records and analyzed. RESULTS: Five patients were identified with cholesteatomas involving the EAC. They all had recurrent ear infections and varying degrees of conductive hearing loss. Each patient's past surgical history included one or more tympanoplasties in which an ipsilateral tympanomeatal flap had been raised. None had undergone a mastoidectomy. Two patients presented with small cholesteatomas that had developed over an average of 6.5 years after surgery. Three patients had large cholesteatomas that had developed over an average of 33.7 years after surgery. Clinical presentations and imaging studies suggested a misplaced tympanomeatal flap as the most likely source of cholesteatoma. CONCLUSION: Tympanomeatal flap misplacement may cause iatrogenic cholesteatoma formation originating from the EAC during tympanoplasty even without mastoidectomy. These cholesteatomas can grow substantially before becoming symptomatic as they extend to and through the mastoid. They may not affect the sound conduction system until late in the course of the disease. Meticulous replacement of tympanomeatal flaps and exercising a high index of suspicion postoperatively can reduce the incidence of this complication.

Impact of the demographic and aetiological factors and intraoperative findings on postoperative outcomes in chronic otitis media surgery

Background/aim: Surgical success is related with many factors belonging to both the patient and the disease. This study aims to analyse the preoperative and intraoperative characteristics, the postoperative results, and the factors affecting the surgical success in different types of chronic otitis media (COM). Materials and methods: A total of 1510 ears of 1398 patients who underwent COM surgery were included in the study. Postoperative results were obtained from 376 ears of 356 patients who had been followed after surgery. The demographic characteristics of the patients, such as age and sex, operative findings, preoperative audiological examination results, and final audiometric and otoscopic examination findings, were retrospectively obtained from the archives of the department. Results: The most frequent diagnosis was simple COM (39.9%), and the most frequently performed surgery was tympanoplasty without mastoidectomy (46.6%). The overall hearing success rate was found to be 75.8%. Postoperative hearing success was significantly associated with the chronic otitis subgroup, ossicular pathologies, and the condition of the middle ear mucosa. Postoperative graft take rate was found to be 78.6%. Graft success was statistically significantly higher in patients with normal middle ear mucosa. Performing mastoidectomy, the presence of patency in aditus ad antrum, and being a paediatric case had no impact on graft success. Conclusion: Factors affecting the success of COM surgery include age, chronic otitis subgroup, location and size of perforation, the condition of the middle ear mucosa, and the level of the ossicular disease. These factors should be known and an appropriate treatment plan should be prepared.

The role of subtotal petrosectomy in cochlear implant recipients.

Describe the indications and outcome of subtotal petrosectomy for cochlear implant recipients. Tertiary care referral center. This is a retrospective study of all subtotal petrosectomies and cochlear implants performed between January 2012 and December 2014. We review the charts of these patients collecting the following data: age, gender, otologic history, audiologic tests, indication of subtotal petrosectomy, data of surgery, surgical and immediate complications, late complications and follow-up of for at least 2.5 years. 12 cases of subtotal petrosectomies with cochlear implantation in 11 patients were performed during this period; 2 children and 10 adults. The indication for a cochlear implant was in 10 cases bilateral severe to profound sensorineural hearing loss and in the remaining 2 cases was asymmetric hearing loss or unilateral hearing loss. The reason for performing a subtotal petrosectomy was chronic otitis media with or without cholesteatoma, radical cavities from previous surgeries or electrode extrusion of previously implanted devices. All cases were performed in one stage. One patient had an infectious complication that required revision surgery and finally an explantation. No other complications are described. Subtotal petrosectomy combined with cochlear implantation is a procedure required in certain situations. It is an effective and safe procedure for managing middle ear problems and creating a safe cavity to receive a cochlear implant either in adults and children.

Pediatric middle ear cholesteatoma: the comparative study of congenital cholesteatoma and acquired cholesteatoma.

This study examined the differences between congenital cholesteatoma (CC) and acquired cholesteatomas (AC) in children by comparing clinical features and treatment courses. This was a retrospective study which retrospectively evaluated 127 children with middle ear cholesteatomas using medical records from January 1999 to December 2012 in the Department of Otolaryngology, Niigata University Hospital. The study comprised 69 and 58 cases of CC and AC, respectively. The main outcome measures include patient backgrounds, the opportunities for consultations, mastoid cell development, intraoperative finding of stapes, surgical procedure and number of surgeries. The average age at operation was 6.4 and 9.8 years in CC and AC, respectively. AC was more prevalent in boys. Mastoid development was better in CC than in AC. We adopted a two-stage operation in 17 cases (25 %) of CC and in 22 cases (38 %) of AC. The repeat surgery rate was 11.6 % in CC and 27.6 % in AC. Three times as many operations were required for three cases (4.3 %) of CC and 10 cases (17.2 %) of AC. The lesions in AC were more difficult to control. In the treatment of pediatric middle ear cholesteatoma, we had to keep the outcome in mind.

[Criteria for the objective assessment of the dynamic state of the retraction pockets in the children presenting with excudative otitis media].

This publication continues the preceding paper entitled "Early diagnostics of the retraction pockets in the tympanic membrane of the children". The objective of the present work was to develop the criteria for the objective estimation of the dynamic state of the retraction pockets (RP) in the children at different stages of excudative otitis media. The secondary objective was to develop an approach to the early diagnostics of cholesteatoma of the middle ear. A group of 138 children at the age varying from 1 to 17 years was placed under dynamic observation for the purpose of drawing up the individual "photo-roentgenological passport of the retraction pockets". Special attention was given to RP-semiotics of different forms of the syndrome and its severity in accordance with the classification universally accepted by foreign researchers. The results of the dynamic observations were used to develop the indications for the early preserving surgical treatment. It was given to 16 children six of which presented with cholesteatoma. The original illustrative materials for all variants of the retraction pockets are presented.

Iatrogenic Cholesteatoma Presenting as Neck Mass.

We present a rare iatrogenic cholesteatoma of the neck in a ten year old male four years after tympanomastoidectomy, an entity that to our knowledge has not been published in the literature for over 30 years. Furthermore, we discuss the diagnostic uncertainty of typical magnetic resonance imaging protocols for pediatric neck lesions and the improved diagnostic specificity of diffusion weighted magnetic resonance imaging. En bloc surgical extirpation was performed. Laryngoscope, 131:E882-E884, 2021.

[Progress in middle ear dysventilation research].

Disfunction of Eustachian tube will cause negative pressure of middle ear, which may result in tympanic membrane retraction pocket. Severe pocket can consequently cause cholesteatoma. In clinical practice it is not uncommon to find a cholesteatoma limited to epitympanum, with an otherwise normal pars tensa and mesotympanum. This review explains the theory of "selective epitympanic dysventilation syndrome" developed by endoscopic technique. In the majority of the patients, the only ventilation pathway to the epitympanum is through the tympanic isthmus. Even if Eustachian tube function has recovered, an isthmus blockage with selective epitympanic dysventilation may lead to common attic cholesteatoma.

Bilateral Ear Canal Cholesteatoma with Underlying Type I First Branchial Cleft Anomalies.

OBJECTIVES:: To describe a case of bilateral ear canal cholesteatomas in the setting of underlying first branchial cleft cyst anomalies and to review the pathophysiology underlying the development of external auditory canal cholesteatomas from branchial cleft cyst abnormalities. METHODS AND RESULTS:: We present a case study of a 61-year-old man who presented with chronic right-sided hearing loss and left-sided postauricular drainage. Clinical evaluation, radiographic work-up, and pathologic analysis confirmed a diagnosis of bilateral ear canal cholesteatoma in the setting of underlying first branchial cleft cyst anomalies. The patient's clinical course, surgical treatment, and management considerations are discussed here. CONCLUSION:: Ear canal cholesteatoma represents a rare clinical disease entity deserving a thorough initial assessment. Careful consideration of underlying diseases that result in chronic inflammation, such as branchial cleft lesions, should be included in the differential diagnosis of idiopathic canal cholesteatoma in the absence of prior otologic surgery or trauma.

Primary cholesteatoma of the external auditory canal: is the epithelial migration defective?

OBJECTIVE: Primary cholesteatoma of the external auditory canal (EACC) is a rare disease, characterized by osteonecrosis with formation of sequesters and ingrowth of keratinizing squamous epithelium in the bony EAC. The aetiology and pathogenesis are unknown, but an earlier study has demonstrated abnormal epithelial migration in such ears. The present study explored whether this interesting result can be reproduced. STUDY DESIGN: The epithelial migration in 10 ears with EACC was studied using the ink-dot method. Two ears with minor lesions were studied before treatment, and 8 ears were studied after operation or conservative treatment. The results were compared with the migration in 15 normal ears examined in a previous study using the same method. SETTING: Tertiary referral center. MAIN OUTCOME MEASURES: 1) Presence or absence of epithelial migration; 2) change of the normal centrifugal pattern; and 3) estimated migration speed compared with normal ears. RESULTS: A qualitatively normal centrifugal migration was present on intact (unaffected or healed) skin in all 10 ears with EACC and was only missing directly on crust-covered lesions. The estimated migration speed was similar to the speed in normal ears, that is, approximately 150 mum/d. CONCLUSION: Ears with EACC seem to have qualitatively and quantitatively normal epithelial migration except directly on crust-covered lesions. It is unlikely that an abnormal epithelial migration is involved in the pathogenesis of this disease. These observations have implications for EAC disorders with similar clinical features that involve bony invasion, including osteoradionecrosis.

Fibrous dysplasia of the temporal bone complicated by cholesteatoma and thrombophlebitis of the transverse and sigmoid sinuses: a case report.

Fibrous dysplasia is a benign condition that can affect the skull and facial bones and cause a broad spectrum of otolaryngologic conditions. We present the case of a boy with polyostotic fibrous dysplasia with involvement of the temporal bone that was first diagnosed when he was 9 years old. His condition eventually became complicated by cholesteatoma and thrombophlebitis of the left transverse and sigmoid sinuses, and he died of his disease at the age of 19 years. We discuss these and other complications of fibrous dysplasia of the temporal bone and their management.

[Congestive optic disks in the brain massive lesions].

To study the specific features of development of papilledema (PE) in brain space-occupying lesions, the authors made a statistical analysis of neuroophthalmologic symptoms in 962 patients with brain tumors, colloid cysts of the third ventricle and cholesteatomas, who had been examined at the Research Institute of Neurosurgery during a calendar year. PE was identified in 30.8% of the patients. The frequency of PE was ascertained to depend on histological pattern, space-occupying lesion site, and age. Visual disturbances in PE were observed in more than a third of patients and manifested themselves as both diminished visual acuity and defects in the field of vision. The frequency and degree of visual deficit depended on the stage of PE.

Increased risk of cholesteatoma among patients with allergic rhinitis: A nationwide investigation.

OBJECTIVES: No large population-based studies have reported on the risk of cholesteatoma developing after allergic rhinitis (AR). This study used a nationwide population-based claims database to investigate the hypothesis that AR may increase the risk of cholesteatoma. STUDY DESIGN: Retrospective cohort study. METHODS: Data from Taiwan's Longitudinal Health Insurance Database were analyzed to compile the following: 1) 15,953 patients newly diagnosed with AR between 1997 and 2000, and 2) a comparison cohort of 63,812 matched non-AR enrollees (with a ratio of 1 to 4). Each patient was followed for 10 years to identify cases in which cholesteatoma subsequently developed. The Kaplan-Meier method was used to determine the cholesteatoma-free survival rate, and the log-rank test was used to compare survival curves. Cox proportional hazard regressions were performed to compute adjusted hazard ratios (HRs). RESULTS: Among the 79,765 patients enrolled in this study, 45 (159,364 person-years) from the AR cohort and 88 (638,130 person-years) from the comparison cohort were diagnosed with cholesteatoma during the follow-up period (incidence rates 0.28 and 0.14 of 1,000 person-years, respectively). Patients with AR were more likely to develop cholesteatoma compared to those without AR (adjusted HR 1.57, 95% confidence interval = 1.05-2.34, P < 0.05). Patients with AR presented a significantly lower 10-year cholesteatoma-free survival rate than did those in the comparison group (log-rank, P < 0.001). CONCLUSION: This is the first study to demonstrate a link between AR and the development of cholesteatoma. We suggest that clinicians keep this association in mind and carefully investigate the possibility of development of cholesteatoma among patients with AR. LEVEL OF EVIDENCE: 3b. Laryngoscope, 128:547-553, 2018.

Collaborative Establishment of Difficult Vascular Access for General Anesthetic Management of an Adult With Tetra-Amelia: A Case Report.

Tetra-amelia is a rare congenital disorder characterized by the absence of limbs. We describe the anesthetic management of a 29-year-old woman with tetra-amelia who underwent general anesthesia for tympanomastoidectomy with meatoplasty for an extensive right ear cholesteatoma. Anesthetic challenges related to tetra-amelia include difficult intravenous access, lack of sites for blood pressure monitoring, and possible difficult airway management due to craniofacial anomalies. Our case report focuses on the complex establishment of iliac artery access for invasive blood pressure monitoring by initially cannulating the carotid artery.

Preauricular Approach for Cholesteatoma Resection After Surgical Overclosure of the External Auditory Canal and Cochlear Implantation.

: Chronic suppurative otitis media can have long-term effects on hearing if not managed effectively. When combined with cholesteatoma the condition may require creation of an open mastoid cavity. Recurrence of cholesteatoma is a concern when cochlear implantation is performed with overclosure of the external auditory meatus. A 61-year-old female with recurrent cholesteatoma in this setting was treated using a preauricular approach to provide adequate visualization while preventing the need to remove the implant or risking injury to the internal components. This technique would be useful in similar patients to prevent morbidity from removal and reinsertion of a cochlear implant.

No evidence of cholesteatoma in untreated otitis media with effusion in children with primary ciliary dyskinesia.

INTRODUCTION: Primary Ciliary Dyskinesia (PCD) describes a group of inherited disorders that result in abnormal ciliary motion leading to mucous stasis. Clinical features include almost universally otitis media with effusion (OME), particularly in infants. PCD patients provide us with a cohort of patients with OME that is not treated with ventilatory tube (VT) insertion as these have been shown to result in frequent complications including chronic otorrhoea, early extrusion and persistent perforation without significant improvement to hearing in the long term. This cohort was used to investigate whether children with PCD and OME not treated with VT were predisposed to cholesteatoma formation in the setting of a paediatric quaternary referral centre. METHODS: A retrospective chart review was performed of all the children attending a multi-disciplinary PCD clinic at a national quaternary referral centre with a diagnosis of OME. We reviewed otoscopic findings, and audiometry and tympanometry results. We assessed the children in four groups: Watchful waiting, hearing aids, VT, and VT and hearing aids. RESULTS: One-hundred-and-one of 107 patients included in the study had a diagnosis of otitis media with effusion. No child with OME and PCD was diagnosed with a cholesteatoma during the follow up period. The only children who had insertion of a ventilatory tube were those who had the procedure prior to the formal diagnosis of PCD. We found a significant complication rate in the children with VT insertion. Hearing improved over time. The prevalence of retraction pockets in untreated OME was 1.72% (3 out of 174 ears). CONCLUSIONS: In children with PCD, OME is an almost universal finding in younger children, but not in adolescents. The study supports the current preference to avoid VT insertion in children with PCD as it confers a significantly higher rate of complications. No cases of cholesteatoma were found in this cohort of PCD children with OME managed without VTs.

[Frontal sinus pathology and epilepsy]. / Epilepsie et pathologie du sinus frontal.

OBJECTIVES: Because of its location and the fragility of its physiology, the frontal sinus is the first of the facial sinuses to cause complications. In this context, orbital sepsis, cranial vault osteitis, meningitis, cerebral abscess, longitudinal sinus thrombophlebitis can occur. A more uncommon consequence of frontal sinusitis is isolated epilepsy. METHODS: We report two cases of patients admitted in our department after a generalised epilepsy seizure with, on the CT-scan, an opacity of the frontal sinus with a posterior wall lysis. RESULTS: We operated on quickly both patients after the seizure via an eyebrow approach. The first one had a purulent collection of the frontal sinus, the second an infected cholesteatoma. Both had a stenosis of the nasofrontal canal and a lysis of the sinus posterior wall with a bare dura mater. The surgical treatment consisted in the cleaning of the sinus associated with an antibiotic treatment in one case and the cholesteatoma matrix removal in the other. The nasofrontal canal was calibrated for respectively four and two months. An antiepileptic treatment was administered for one year. Four years later the nasofrontal canal is pervious and the frontal sinus sound in both patients. CONCLUSION: An epilepsy seizure can follow a frontal sinusitis. It does not convey the existence of an endocranial complication but requires researching it. The posterior wall lysis of the sinus with a bare dura mater is sufficient to lead to a seizure in case of sinus infection.

Cholesteatoma as a complication of Langerhans Cell Histiocytosis of the temporal bone: A nationwide cross-sectional analysis.

OBJECTIVE: To determine if patients with Langerhans Cell Histiocytosis (LCH) of the temporal bone have a higher risk of developing cholesteatoma. METHODS: Review of literature and cross-sectional weighted analysis of patients under 19 with a diagnosis of LCH from the National Inpatient Sample (NIS) and Kids' Inpatient Database (KID) from 2000 to 2013. ICD-9 codes and demographics were analyzed; pairwise comparisons and multivariate analyses were performed. RESULTS: Only seven cases of cholesteatoma after the treatment for LCH of the temporal bone have been documented in the literature. No significant association between cholesteatoma and LCH was seen (OR 0.747 [0.149-3.751]). Patients with LCH did have a higher incidence of chronic otitis media, chronic otitis externa, chronic sinusitis, hearing loss, and otitis media with effusion. CONCLUSION: Our results show that patients with Langerhans Cell Histiocytosis do not appear to have a higher risk of developing cholesteatoma. However they are more likely to be diagnosed with chronic otitis externa which should be differentiated from cholesteatoma or recurrence of LCH.

Correlation between sensorineural hearing loss and chronic otorrhea.

Many studies have attempted to correlate chronic otorrhea in children and in adults with the sensorineural hearing loss in the affected ear, with contradictory results. This loss might be the result of the likely toxicity of the bacteria involved, effects of inflammatory cytokines, or constant use of ototoxic antibiotics. All studies evaluated to date compared the affected ear with the normal contralateral ear. From the digitized archive of otologic surgery files of the Department of Otorhinolaryngology at Santa Casa de São Paulo School of Medical Sciences, the ears of patients with chronic otorrhea were evaluated visually and compared with the normal contralateral ears. Ears with otorrhea were also compared with ears of other patients with dry tympanic perforation. Ears with suppuration were evaluated for cholesteatoma. The duration of otorrhea was considered. The sensorineural hearing threshold was evaluated for the frequencies of 500, 1,000, 2,000, and 4,000 Hz. A total of 98 patients with chronic otorrhea and 60 with dry tympanic membrane perforation were evaluated. A correlation between sensorineural hearing loss and chronic otorrhea was observed when compared with both contralateral normal ears and dry perforated ears of other patients. No relationship between hearing loss and the duration of suppuration or cholesteatoma was found. Sensorineural hearing loss occurs in ears with chronic otorrhea. The duration of otorrhea and the etiology of suppuration did not influence the hearing loss.