Epidemiology of Congenital Cholesteatoma: Surveys of the Last 17 Years in Japan.

(1) Background: The incidence of congenital cholesteatoma (CC) has rarely been discussed, particularly from a demographic viewpoint. Therefore, we conducted an epidemiological study of CC using local medical characteristics. (2) Methods: The participants were 100 patients (101 ears) who underwent initial surgical treatment at university hospitals in two rural prefectures between 2006 and 2022. A total of 68% of the patients were males and 32% were females, with a median age of 5 years. We reviewed the medical records for the date of birth, date of surgery, stage of disease, and first symptoms of the disease. (3) Results: The total incidence of CC was calculated to be 26.44 per 100,000 births and tended to increase. No significant difference was found between the incidences in the two prefectures. The number of surgeries performed was higher in the second half of the study period. No difference in the stage of progress was observed based on age. (4) Conclusions: The incidence of CC was estimated to be 26.44 per 100,000 newborn births. The number of patients with CC tended to increase; however, this can be attributed to an increase in the detection rate rather than the incidence.

Predicting residual cholesteatoma with the Potsic staging system still lacks evidence: a systematic review and meta-analysis.

PURPOSE: To investigate the rate of residual disease in the Potsic staging system for congenital cholesteatomas. METHODS: A protocol registration was published on PROSPERO (CRD42022383932), describing residual disease as a primary outcome and hearing improvement as secondary. A systematic search was performed in four databases (PubMed, Embase, Cochrane Library, Web of Science) on December 14, 2022. Articles were included if cholesteatomas were staged according to the Potsic system and follow-up duration was documented. Risk of bias was evaluated using the Quality In Prognosis Studies (QUIPS) tool. In the statistical synthesis a random effects model was used. Between-study heterogeneity was assessed using I2. RESULTS: Thirteen articles were found to be eligible for systematic review and seven were included in the meta-analysis section. All records were retrospective cohort studies with high risk of bias. Regarding the proportions of residual disease, analysis using the χ2 test showed no statistically significant difference between Potsic stages after a follow-up of minimum one year (stage I 0.06 (confidence interval (CI) 0.01-0.33); stage II 0.20 (CI 0.09-0.38); stage III 0.06 (CI 0.00-0.61); stage IV: 0.17 (CI 0.01-0.81)). Postoperative and preoperative hearing outcomes could not be analyzed due to varied reporting. Results on cholesteatoma location and mean age at staging were consistent with those previously published. CONCLUSION: No statistically significant difference was found in the proportions of residual disease between Potsic stages, thus the staging system's applicability for outcome prediction could not be proven based on the available data. Targeted studies are needed for a higher level of evidence.

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.

Study of Angiogenic, Pro-Apoptotic, and Pro-Inflammatory Factors in Congenital and Acquired Cholesteatomas.

OBJECTIVES: Despite recent advances in biomolecular research that have improved our knowledge of cholesteatoma pathogenesis, the reasons behind its highly variable clinical course are still not clarified. It has been proposed that biological signaling between peri-matrix and matrix cells could play a critical role in disease homeostasis. The aim of our study was to analyze the expression of inflammatory (IL-1ß), hyper-proliferative (STAT-3, TGF-ß), and angiogenic (VEGF-C, PDGFr) factors in congenital and acquired cholesteatomas (both in adults and children), which might correlate with the clinical features observed. We performed an experimental study on 37 patients (29 males and 8 females, ranging from 4 to 66 years of age) who were diagnosed with cholesteatoma between 2020 and 2021 in our institution. All patients underwent clinical, audiologic, and radiologic assessments. Bone erosion grading and staging of cholesteatoma growth were assessed through preoperative evaluation and intraoperative middle ear findings, according to the PTAM System proposed by the Japan Otological Society (2016). Retro-auricular skin specimens were intraoperatively collected in all patients. Skin and cholesteatoma samples were analyzed through histopathological, western blot, and immunohistochemical evaluations. The expression rate was measured to find out the differences between congenital and acquired cholesteatomas as well as between the adult and pediatric populations. Expression of angiogenic, inflammatory, and proliferative biomarkers is significantly increased in acquired cholesteatomas in children as compared to congenital and acquired forms in adults, in accordance with the higher stage of disease shown by imaging, surgical, and histological features. Our data suggest that pathways already supposed to be involved in the pathogenesis of cholesteatomas could be differently activated in more destructive forms, typically found in children. The identification of potential biomarkers of cholesteatoma aggressiveness could lead to more personalized management (timing of intervention, recurrence prevention) and the future identification of anti-growth/anti-proliferative agents as non-surgery therapeutic options.

The role of planned two-stage surgery in the management of congenital cholesteatoma.

OBJECTIVE: The purpose of this study is to verify the role of "planned two-stage surgery" in the management of advanced congenital cholesteatoma regarding disease recurrence rates, complications and the need for salvage surgery. METHOD: Retrospective review of all congenital cholesteatoma under the age of 18 years underwent surgery from October 2007 to December 2021 in a single tertiary referral center. Patients with Potsic stage I/II who had closed-type congenital cholesteatoma received one-stage surgery. Advanced cases or those with open-type infiltrative congenital cholesteatomas underwent planned two-stage surgery. The second stage of surgery was performed 6-10 months after the first stage of surgery. Ossiculoplasty would be performed in the second operation if a significant air-bone gap was detected in the preoperative pure-tone audiometry test. RESULTS: Twenty-four patients were included in the series. Six patients received one-stage surgery and no recurrence was noted in this group. The remaining 18 underwent planned two-stage surgery. Residual lesions found in the second operative phase were observed in 39% of patients who received planned two-stage surgery. Except for one patient whose ossicular replacement prosthesis protruded and two patients who had perforated tympanic membranes, none of the 24 patients required salvage surgery during follow-up (mean, 77 months after surgery), and no major complications occurred. CONCLUSIONS: Planned two-stage surgery for advanced-stage or open infiltrative congenital cholesteatoma could timely detect residual lesions to avoid extensive surgery and reduce complications.

A Case of a Congenital Cholesteatoma Without Growth for a Long Term.

Congenital cholesteatoma is typically an expanding cystic mass of keratinizing squamous epithelium located medial to the intact tympanic membrane in patients with no prior history of perforation, otorrhea and ear. It is generally thought to be a progressive disease and is usually surgically removed upon detection as the first-choice treatment. As such, it is rare to be observed for a long term without progression. Here we report a rare case of congenital cholesteatoma that remained in an undetectable size and did not deteriorate mild hearing loss for 12 years. A seven years old boy was referred to us with right hearing impairment. Pure-tone audiometry found conductive hearing loss with an air-bone gap of 25 dB and a high-resolution computed tomography (CT) scan found the eroded long process of incus but did not detect any soft tissue density indicating congenital cholesteatoma. He initially did not wish to undergo surgery. His hearing level and image finding remained virtually unchanged during the next 12 years of the follow-up period. Twelve years later, endoscopic ear surgery was performed, which revealed a very small cholesteatoma mass, an eroded long process of the incus and ossicular chain discontinuities. We suspect that the cholesteatoma was originally larger, partially eroded the incus, then regressed to a very small size, and remained small for at least 12 years under our observation.

Colesteatoma congénito: revisión de la literatura / Congenital cholesteatoma: literature review

El colesteatoma congénito es una entidad clínica única y desafiante, que se caracteriza por acumulación anormal de queratina en el oído medio, medial a la membrana timpánica. Se presenta, mayoritariamente, en el género masculino, con una incidencia estimada de 0.12 por 100.000 habitantes, representando el 4% a 24% de los colesteatomas en población pediátrica y un 2% a 5% del total de colesteatomas. Su origen aún es controversial, siendo la teoría más aceptada, la del arresto epitelial. Su diagnóstico es clínico, variando la sintomatología según severidad del compromiso, presentándose desde hallazgo incidental, hipoacusia de conducción, hasta presentar otalgia y perforación timpánica. Las imágenes se consideran un apoyo complementario preoperatorio. El tratamiento es quirúrgico, con diferentes técnicas disponibles, las cuales se deben definir de manera individual en el caso de cada paciente. Es fundamental su diagnóstico y manejo precoz, para lograr un tratamiento oportuno con menor tasa de complicaciones y compromiso a largo plazo. A continuación, se presenta una revisión de la literatura respecto de esta patología, para difusión en nuestro medio.

Congenital cholesteatoma (CC) is a unique and challenging clinical entity characterized by abnormal accumulation of keratin in the middle ear, medial to the tympanic membrane, being more frequent in the male gender, with an estimated incidence of 0.12 per 100,000 inhabitants. It represents 4% to 24% of cholesteatomas in the pediatric population and 2% to 5% of all cholesteatomas. Its cause is still controversial, the most accepted theory being epithelial arrest. The diagnosis is clinical, varying the symptoms according to the severity of the compromise, from incidental finding, through conduction hearing loss, to presenting otalgia and tympanic perforation. Images are considered additional preoperative support. Treatment is predominantly surgical, with different techniques available, which must be defined individually. Its early discovery and management are essential to achieve proper treatment with a lower rate of complications and long-term commitment. We present a review of the literature regarding CC to provide information relevant to our area of expertise.

Colesteatoma congénito: caso clínico y revisión de literatura / Congenital cholesteatoma: clinical case and literature review

El colesteatoma congénito (CC) es una lesión benigna de epitelio escamoso queratinizado que puede afectar diferentes aéreas del hueso temporal con predominio en el oído medio. El CC es una patología poco frecuente que se presenta en pacientes pediátricos y clínicamente se manifiesta como una lesión blanquecina detrás de un tímpano indemne. La mayoría de los pacientes no presenta historia de hipoacusia, otorrea, infección, perforación o cirugía otológica previa. Se analiza el caso de un prescolar con CC que consultó con trastorno de sueño sin sintomatología otológica, pero con otoscopía alterada como hallazgo clínico. En la resonancia magnética nuclear (RMN) con secuencia de difusión, se evidenciaron hallazgos sugerentes de lesión colesteatomatosa en oído medio. Se realizó tratamiento quirúrgico endoscópico combinado con remoción completa de la lesión compatible histológicamente con CC y reconstrucción funcional con prótesis inactiva con resultado auditivo satisfactorio. El CC requiere alta sospecha diagnóstica por pediatras y otorrinolaringólogos, siendo imprescindible realizar un examen físico acucioso que incluya otoscopía de rutina, aunque el paciente no manifieste síntomas otológicos. El tratamiento es quirúrgico y debe considerar uso de endoscópico para asegurar una extracción completa de la lesión. En algunos casos es requerido realizar una reconstrucción auditiva para asegurar un óptimo resultado funcional.

Congenital cholesteatoma (CC) is a benign lesion of keratinized squamous epithelium that can affect different areas of the temporal bone, predominantly in the middle ear. CC is a rare pathology that occurs in pediatric patients and clinically manifests as a white lesion behind an intact eardrum. Most patients do not have a history of hearing loss, otorrhea, infection, perforation, or previous otologic surgery. The following, is the case of an infant with CC who consulted with a sleep disorder without otological symptoms but with altered otoscopy as a clinical finding. Nuclear magnetic resonance (NMR) with diffusion sequence with findings compatible with a cholesteatomatous lesion in the middle ear. Endoscopic surgical treatment was performed combined with complete removal of the lesion histologically compatible with CC and functional reconstruction with inactive prosthesis with satisfactory hearing results. CC requires high diagnostic suspicion by paediatrics and otorhinolaryngologists, and it is essential to perform a thorough physical examination that includes routine otoscopy even if the patient does not show otological symptoms. Treatment is surgical and endoscopic use should be considered to ensure complete removal of the lesion. In some cases, hearing reconstruction is required to ensure optimal functional results.

Outcomes of transcanal endoscopic middle ear surgery for congenital cholesteatoma.

BACKGROUND: Transcanal endoscopic surgery offers advantages for congenital cholesteatoma (CC) which is expanding cystic mass located on an intact tympanic membrane. OBJECTIVES: To determine the efficacy of transcanal endoscopic ear surgery for the treatment of CC. MATERIAL AND METHODS: Eleven patients with CC confined to the middle ear underwent transcanal endoscopic ear surgery by surgeons who were skilled in otoscopic surgery. According to the Potsic classification, four, three, and four patients had lesions in stages 1, 2, and 3, respectively. The mean follow-up duration was 24.5 months. We retrospectively analyzed the recurrence rate, complications, and average hearing thresholds of the patients with CC. RESULTS: All CCs were successfully resected via the transcanal endoscopic approach, without any immediate or serious complications. The only complication was external auditory canal stenosis due to postoperative scarring, which occurred in a patient with a stage-3 cholesteatoma that recurred and required reoperation and second-stage ossicular chain reconstruction. Two more patients underwent one-stage ossicular chain reconstruction. All three patients received a total artificial ossicular replacement prosthesis, and two of them also received cartilage grafts. CONCLUSIONS AND SIGNIFICANCE: Transcanal endoscopic ear surgery is an effective method to treat CC belonging to Potsic stages 3 or below.

A Giant Congenital Mastoid Cholesteatoma With Extension to the Occipital Condyle.

A congenital mastoid cholesteatoma (CMC) is a keratinizing epithelium originating from embryological epithelial tissue of the mastoid. It is often not diagnosed until it becomes large because of its rarity and indolent nature. Although there are a few reports on giant CMC, its exact extensions have not been well described, and detailed information regarding surgical methods is lacking, especially in giant CMC involving the occipital condyle and the middle and posterior cranial fossae. In this article, we report a case involving a 70-year-old woman with a giant CMC that extended inferiorly to the occipital condyle. The CMC eroded the middle and posterior cranial fossae, sigmoid sinus plate, and fallopian canal of the facial nerve. For complete removal, we used a subtotal petrosectomy in conjunction with an exposure of the cranial cervical junction and a wide decompression of the suboccipit. The boundaries of exposure were similar to those of a petro-occipital transsigmoid approach which is usually used for management of tumor involving the jugular foramen. The wide exposure allowed for complete removal of the lesion without any complications. Thus, we recommend this surgical approach for management of the giant CMC involving the occipital condyle and the middle and posterior cranial fossae.

Congenital cholesteatoma clinical and surgical management.

OBJECTIVES: Congenital cholesteatoma (CC) is accompanied by hearing loss and an intact tympanic membrane. However, the hearing loss is usually associated with otitis media, and the diagnosis of CC is frequently delayed in patients with an intact tympanic membrane. This study aimed to describe the clinical characteristics, management and outcomes of patients with CC. METHODS: We reviewed patients with cholesteatoma from January 2011 to May 2020 and selected those meeting the congenital cholesteatoma criteria. The primary outcome measures included presenting symptoms, surgical findings, stage of disease, recurrence rate and hearing outcomes. RESULTS: We reviewed 1646 medical files of cholesteatoma patients and identified 18 patients with congenital cholesteatoma, the mean age at operation was 8.13 ± 1.36 years (range 3-18). The unilateral hearing loss included moderate 13 patients (72.2%), severe 4 patients (22.2%), and slight 1 (5.6%). There were 14 cases of conductive hearing loss (77.8%) and 4 cases of mixed hearing loss (22.2%). The mean course of disease was 1.41 ± 0.05 years (range 0.4-3). The surgical management was oto-endoscope exploratory tympanotomy in 1（5.6%）, canal wall up mastoidectomy in 12 (66.7%) and canal wall down in 5 (27.8%), with 17 (94.4%) ossicular replacements. Seventeen (94.4%) patients presented with Potsic stage III-IV. Recurrence occurred in 5.6% of patients in stage III and 11.1% of patients in stage IV. After surgery, patients achieved normal voice tone hearing. CONCLUSIONS: To diagnose it early, congenital cholesteatoma should be considered as a possible aetiology for hearing loss patients with an intact tympanic membrane. In our study, most patients were diagnosed at III and IV stage. This highlights the need to promote awareness of the disease among primary physicians in the community healthcare system. Surgical management with removal of the cholesteatoma and reconstruction of the tympanum and ossicular chain is an effective treatment.

Diagnosis and Treatment Modalities of Cholesteatomas: A Review.

A cholesteatoma is an abnormal, noncancerous skin growth that can appear beneath the eardrum in the center of your ear. Although it might be a congenital condition, frequent middle ear infections are the main culprit. A cholesteatoma frequently appears as a cyst or sac that exfoliates the skin's outer layers. As these dead skin cells build up, the growth can enlarge and penetrate intratemporal tissues, leading to various intracranial and extracranial difficulties that can compromise facial muscles, hearing, and balance. Cholesteatomas were recognized more than three centuries ago. A cholesteatoma can develop into a serious condition if it is not treated right away. Because of its fast development and invasive nature, it can cause several consequences, some of which can be fatal for people without access to advanced medical treatment. There are no effective nonsurgical treatments available right now. It will be necessary to have a thorough awareness of both previous developments and more current ones to develop an appropriate management approach for this condition. The background information on acquired middle ear cholesteatomas is briefly reviewed in this paper. We also take into account categorization-, epidemiology-, histology-, and pathogenesis-related difficulties, and we carefully review current management and diagnosis approaches.

Congenital cholesteatoma in identical twins.

Congenital cholesteatoma in identical twins has only been described once in Otolaryngology literature thus far. This report describes a case of monozygotic twins with a history of recurrent acute otitis media and bilateral middle ear effusions without tympanic membrane perforation. Upon myringotomy with pressure equalization tube insertion, both were found to have right-sided cholesteatoma with nearly identical location and pattern of progression. In the context of previous case series demonstrating familial clustering and reports of possible genetic associations of this condition, the authors present an important addition to the current understanding of congenital cholesteatoma disorder.

[Congenital cholesteatoma of the mastoid process: cases of diagnosis and surgical treatment]. / Vrozhdennaya kholesteatoma sostsevidnogo otrostka visochnoi kosti: sluchai diagnostiki i khirurgicheskogo lecheniya.

A brief review of the epidemiology and pathogenesis of congenital cholesteatoma (CC) of the temporal bone pyramid is presented, classifications reflecting the degree of spread or localization of the process are considered. Variants of local CCs of the mastoid process don't fit into these classifications, as well as there is no statistics on the frequency of their detection and treatment due to the rarity of this pathology. Clinical cases of diagnosis, surgical treatment of CC of the mastoid process and its results are described. Clinical examples indicate a long-term asymptomatic course of CC of the mastoid process, accompanied by a pronounced destructive process in the mastoid process. Early surgical treatment makes it possible to remove the neoplasm as radically as possible and preserve hearing function.

Particularidades del abordaje transcanal endoscópico de los tumores benignos de oído medio en adultos / Nuances in transcanal endoscopic approach to benign middle ear tumours in adult patients

Antecedentes y objetivo: La cirugía mínimamente invasiva de tumores benignos del oído medio es posible mediante el uso del endoscopio. La mejor iluminación y la visión más amplia que ofrece permiten el abordaje transcanal de estos raros tumores. El objetivo del presente trabajo es sintetizar los puntos clave de su diagnóstico y tratamiento, a través de una serie propia de casos.Materiales y métodosEstudio retrospectivo de tumores benignos de oído medio intervenidos exclusivamente por vía transcanal endoscópica en un hospital universitario de tercer nivel, de adultos, entre junio de 2018 y junio de 2020. El seguimiento postoperatorio se realizó mediante otoendoscopia y audiometría.ResultadosFueron intervenidos durante el periodo de estudio 6 pacientes; 5 fueron mujeres y uno fue hombre, con una edad media de 57,8 años (± 21,9). Cuatro tumores estaban en el oído izquierdo y 2 en el oído derecho. Estos incluían 4paragangliomas timpánicos (3 de tipo I y uno de tipo II), un neurinoma de cuerda de tímpano y un colesteatoma congénito. No hubo complicaciones postoperatorias graves. En el momento actual no se ha constatado recidiva tumoral en ninguno de los casos, con un seguimiento mínimo de 7 meses.ConclusionesEl presente estudio añade evidencia sobre la seguridad y eficacia de la cirugía transcanal endoscópica de oído, como técnica mínimamente invasiva, para el tratamiento de tumores benignos de oído medio confinados en la cavidad timpánica. (AU)

Background and objective: Minimally invasive surgery of benign middle ear tumours is possible by using the endoscope. The optimal lighting and the broadest vision it offers, allow a transcanal approach to these rare tumours. The objective of this work is to summarize its key points through a case series.Materials and methodsRetrospective study of benign middle ear tumours that underwent exclusive endoscopic surgery in a third-level adult university hospital between June 2018 and June 2020. Postoperative follow-up was performed by otoendoscopy and audiometry.ResultsSix patients underwent surgery during the study period. Five patients were female and one male, with an average age of 57.8 years (± 21.9). Four tumours were in the left ear and 2 in the right ear. These included 4tympanic paragangliomas (3 type I and one type II), a chorda tympani neuroma, and a congenital cholesteatoma. There were no serious postoperative complications. At present, no tumour recurrence has been found in either case, with a minimum follow-up of 7 months.ConclusionsThe present study adds evidence on the safety and efficacy of endoscopic transcanal ear surgery, as a minimally invasive technique, for the treatment of benign middle ear tumours confined to the tympanic cavity. (AU)

Nuances in transcanal endoscopic approach to benign middle ear tumours in adult patients.

BACKGROUND AND OBJECTIVE: Minimally invasive surgery of benign middle ear tumours is possible by using the endoscope. The optimal lighting and the broadest vision it offers, allow a transcanal approach to these rare tumours. The objective of this work is to summarise its key points through a case series. MATERIALS AND METHODS: Retrospective study of benign middle ear tumours that underwent exclusive endoscopic surgery in a third-level adult university hospital between June 2018 and June 2020. Postoperative follow-up was performed by otoendoscopy and audiometry. RESULTS: Six patients underwent surgery during the study period. Five patients were female and one male, with an average age of 57.8 years (±21.9). Four tumours were in the left ear and 2 in the right ear. These included four tympanic paragangliomas (three type I and one type II), a chorda tympani neuroma, and a congenital cholesteatoma. There were no serious postoperative complications. At present, no tumour recurrence has been found in either case, with a minimum follow-up of 7 months. CONCLUSIONS: The present study adds evidence on the safety and efficacy of endoscopic transcanal ear surgery, as a minimally invasive technique, for the treatment of benign middle ear tumours confined to the tympanic cavity.

Pearl Syndrome With an Unusual Association of Spina Bifida and Congenital Cholesteatoma.

We report a case of a one-day-old female with congenital facial nerve palsy, bilateral microtia, congenital heart disease, spina bifida, and congenital cholesteatoma. The newborn was brought by the mother with complaints of abnormally looking ear and a facial droop toward the left side, following which a two-dimensional echocardiography was done showing the atrial septal defect and ventricular septal defect. Computed tomography of the temporal bone showed the presence of congenital cholesteatoma in the left ear. MRI of the lumbosacral spine was suggestive of spina bifida occulta. Brainstem evoked response audiometry was suggestive of sensorineural hearing loss. Such a combination of symptoms is very rare, and therefore this case is being reported.

Congenital cholesteatoma: Clinical features and surgical outcomes.

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

RHINOGENIC MENINGITIS CAUSED BY CONGENITAL PETROUS APEX CHOLESTEATOMA: SIMULTANEOUS SURGICAL TREATMENT THROUGH TRANSOTIC AND TRANSSPHENOIDAL APPROACH.

A 66-year-old male patient was admitted due to high fever, severe headaches and disturbance of consciousness. Meningitis was confirmed by lumbar puncture and intravenous antimicrobial therapy was started. Since he had undergone radical tympanomastoidectomy 15 years before, otogenic meningitis was suspected, so the patient was referred to our department. Clinically, the patient manifested watery discharge from the right nostril. Microbiological analysis verified Staphylococcus aureus in a cerebrospinal fluid (CSF) sample acquired by lumbar puncture. Radiological work-up, including computed tomography and magnetic resonance imaging scans, showed an expanding lesion of the petrous apex of the right temporal bone disrupting the posterior bony wall of the right sphenoid sinus with radiological characteristics indicating cholesteatoma. Those findings confirmed rhinogenic meningitis caused by expansion of the petrous apex congenital cholesteatoma into the sphenoid sinus, allowing nasal bacteria to enter the cranial cavity. The cholesteatoma was removed completely by the simultaneous transotic and transsphenoidal approach. Since the right labyrinth was already non-functional, there was no surgical morbidity after labyrinthectomy. The facial nerve remained preserved and intact. The transsphenoidal approach enabled removal of the sphenoid portion of the cholesteatoma and two surgeons met together at the level of the retrocarotid segment of the cholesteatoma, completely removing the lesion. This case represents an extremely rare condition in which a petrous apex congenital cholesteatoma expanded through the petrous apex to the sphenoid sinus, causing CSF rhinorrhea and rhinogenic meningitis. According to available literature, this is the first case of petrous apex congenital cholesteatoma causing rhinogenic meningitis successfully treated with the simultaneous transotic and transsphenoidal approach.