Colesteatoma del conducto auditivo externo: Experiencia de 5 años Hospital del Salvador / External ear canal cholesteatoma: 5-year experience Hospital del Salvador

Introducción: El colesteatoma del conducto auditivo externo (CCAE) es una estructura quística revestida por epitelio escamoso estratificado queratinizado que tiene la capacidad de invadir y erosionar localmente al hueso temporal. Su incidencia es de 0,19 a 0,3/100.000 habitantes siendo 60 veces menos frecuente que el de oído medio. Objetivo: Describir las características epidemiológicas, clínicas, imagenológicas y tratamiento de los pacientes diagnosticados con CCAE en el Servicio de Otorrinolaringología Hospital del Salvador. Material y Método: Se presenta una serie de ocho casos clínicos recopilados durante el período 2017 y 2021. Se realizó revisión de fichas clínicas, biopsias y tomografías computadas de oídos (TC oídos). Se describen los hallazgos y tratamiento efectuado. Resultados: El promedio de edad fue de 65,6 años, correspondiente a 5 mujeres y 3 hombres con presencia de tabaquismo y diabetes en la mitad de los casos. Los síntomas y signos más frecuentes fueron otalgia e hipoacusia seguido de otorrea. 7 pacientes se presentaron con tímpano íntegro y el compromiso de la pared inferior del conducto se evidenció en 6 de 8 pacientes. La TC oídos mostró erosión ósea del conducto, con o sin compromiso de estructuras adyacentes, en todos los casos y el diagnóstico histológico fue efectuado en el 100% de los pacientes. Se privilegió el tratamiento conservador mediante curaciones óticas periódicas asociado a ácido salicílico al 3% y/o antibióticos tópicos en 6/8 pacientes. Conclusiones: El CCAE es una entidad poco frecuente sin signos ni síntomas patognomónicos por lo que el diagnóstico histológico junto con el estudio imagenológico es perentorio. El tratamiento conservador es una alternativa terapéutica válida que ofrece buenos resultados en pacientes con adecuada adherencia al tratamiento y posibilidad de seguimiento estricto.

Introduction: External ear canal cholesteatoma (EECC) is a cystic structure lined by keratinized stratified squamous epithelium that has the ability to locally invade and erode the temporal bone. Its incidence is 0.19 to 0.3 / 100,000 habitants, being 60 times less frequent than that of the middle ear. Aim: To describe the epidemiological, clinical, imaging and treatment characteristics of patients diagnosed with EECC in the Hospital del Salvador ENT department. Material and Methods: A series of eight clinical cases collected during the period 2017 and 2021 is presented. A review of clinical records, biopsies and computed tomography of the ear (ear CT) was carried out. The findings and treatment carried out are described. Results: The average age was 65.6 years corresponding to 5 women and 3 men with the presence of smoking and diabetes in half of the cases. The most frequent symptoms and signs were earache and hearing loss followed by otorrhea. 7 patients presented with an intact eardrum and compromise of the inferior wall of the canal was evidenced in 6 of 8 patients. Ears CT showed bone erosion of the canal with or without compromise of adjacent structures in all cases and the histological diagnosis was made in 100% of the patients. Conservative treatment with periodic ear dressings associated with 3% salicylic acid and / or topical antibiotics was favored in 6/8 patients. Conclusion: EECC is a rare entity without pathognomonic signs or symptoms, therefore the histological diagnosis together with the imaging study is peremptory. Conservative treatment is a valid therapeutic alternative that offers good results in patients with adequate adherence to treatment and the possibility of strict follow-up.

Colesteatoma congénito: revisión de la literatura

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.

Presentación simultánea de quiste epidermoide del ángulo pontocerebeloso y colesteatoma infiltrante del oído medio. Reporte de caso y revisión de literatura / Simultaneous presentation of an epidermoid cyst of the cerebellopontine angle and infiltrating cholesteatoma of the middle ear. Case report and literature review

Introducción: los quistes epidermoides son el tercer tumor más común del ángulo pontocerebeloso (APC). Es infrecuente detectar simultáneamente un colesteatoma infiltrativo del oído medio (OM). Caso clínico: paciente de 51 años acude a urgencias por cefalea hemicraneal intensa, pulsátil secundaria a hidrocefalia aguda, requirió ventriculostomía. En la resonancia magnética nuclear (RMN) cerebral contrastada se reporta una masa en el APC sugestivo de quiste epidermoide y simultáneamente un colesteatoma infiltrativo del OM. La paciente fue intervenida primero con resección de colesteatoma del OM; en un segundo tiempo resección del quiste epidermoide del APC por vía translaberíntica. El posoperatorio la evolución clínica fue satisfactoria. Discusión: los quistes epidermoides del APC son histopatológicamente idénticos al colesteatoma del OM y pueden ser secundarios a estos. Conclusión: se debe individualizar el manejo sin descartar la posibilidad de tener las dos enfermedades de manera simultánea.

Introduction: cysts are the third most common tumor of the cerebellopontine angle (CPA). It is rare to simultaneously detect an infiltrative cholesteatoma of the middle ear (OM). Clinical case: a 51-year-old patient attended the emergency department due to intense throbbing hemicranial headache secondary to acute hydrocephalus, requiring ventriculostomy. Contrast-enhanced cerebral magnetic resonance imaging (MRI) reported a mass in the APC suggestive of an epidermoid cyst and simultaneously an infiltrative cholesteatoma of the OM. The patient underwent first surgery with resection of the OM cholesteatoma; in a second stage, resection of the epidermoid cyst of the APC through a translabyrinthine approach. The postoperative clinical evolution was satisfactory. Discussion: APC epidermoid cysts are histopathologically identical to OM cholesteatoma and may be secondary to them. Conclusion: management must be individualized without ruling out the possibility of having both diseases simultaneously.

Inside-Out Transcanal Endoscopic Mastoidectomy: Literature Revision

Abstract Introduction Recently, there have been significant advancements in transcanal endoscopic ear surgery (TEES). The combination of rigid and thin otoendoscopes with highdefinition cameras enabled a less invasive transcanal access to the middle ear and a clearer view of the surgical field. Several surgeons have recently published studies about cholesteatoma resection via transcanal endoscopic surgery, even in cases where the disease has extended to the mastoid, requiring transcanal endoscopic mastoidectomy. Objectives To analyze the currently available literature on transcanal endoscopic inside-out mastoidectomy, and to determine its efficacy as a surgical technique by evaluating the disease's relapse/recurrence rate. Data Synthesis Initially, the titles and abstracts of articles identified were analyzed. At this stage, 117 articles were analyzed, 97 of which were excluded for not meeting the inclusion criteria. The 20 remaining articles were further evaluated. The articles were classified on the basis of five levels of scientific evidence. Final Comments The analysis of the studies showed that the transcanal endoscopic approach is effective in providing access to the attic or antrum, especially in cases of sclerotic mastoids. There was only one study with grade A recommendation, which showed the efficacy of endoscopic ear surgery in the treatment of cholesteatoma. Furthermore, there were three studies with grade B recommendation, showing less relapse/recurrence after TEES. More studies with grade A and B recommendations are needed to better evaluate the effectiveness of TEES, especially compared with that of traditional microscopic surgery.

Colesteatoma congénito: caso clínico y revisión de literatura

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.

Complications-of-chronic-cholesteatomatous-otitis-media-at-the-university-hospital-center-of-andohatapenaka-antananarivo-madagascar

Chronic cholesteatomatous otitis media is the particularly aggressive form of chronic otitis media. This ear infection can lead to functional impairment of the ear and exposes you to serious complications that can be life-threatening. The purpose of this work was to list the complications of cholesteatomatous otitis in our hospital center. Patients And Method: This is a descriptive retrospective study of cases of complicated cholesteatomatous otitis. The study was carried out within the university hospital center Andohatapenaka from January 2015 to December 2019 (5 years). Complications of chronic not cholesteatomatous otitis media have been ruled out. The frequency ofResults: cholesteatomatous otitis media is 2.57%. It mainly affects patients between 10 and 20 years old with a male predominance. Extra-cranial complications are more frequent (78.94%) compared to intracranial complications. The circumstances of discovery are dominated by otorrhea (31.57%) and retro-auricular swelling (21.05%). The clinical signs are variable and depend on the complications. The otological history badly or not treated (60.52%) remains a predictive factor of these complications. Computed tomography has a very important place in the diagnostic component. Cholesteatomatous otitis media is always at risk of serious complications in the absence ofConclusion : well-adapted treatments. Complications can be functional which blames the hearing or vital following the endocranial extension.

Experimental cholesteatoma: a comparison between spontaneous and induced models

Abstract Objective: To compare the incidence and the histopathological aspect of spontaneous and two induced Mongolian gerbils' models of cholesteatoma: External Auditory Canal (EAC) obliteration model and the Auditory Tube (AT) cauterization model. Methods: Fifty-four ears of 27 animals were divided into EAC obliteration, AT cauterization, and control groups and histologically assessed for cholesteatoma incidence and classification at intervals of 2, 4, 8, and 16 weeks. Results: Cholesteatoma was diagnosed in 30 of the 53 ears evaluated with a significantly higher incidence in groups that received some type of intervention (p < 0.0001). It was not possible to histologically distinguish cholesteatomas of the same stage between the study groups. Conclusion: Although we observed a significant increase in cholesteatoma incidence with the two methods used when compared to the control group, all developed cholesteatomas were apparently identical from a histological point of view.

Perforación timpánica y colesteatoma, una patología de niños y adultos / Tympanic perforation and cholesteatoma, a pathology of children and adults

INTRODUCCIÓN. La patología de oído es una enfermedad frecuente en nuestro medio, asociada a infecciones a repetición del oído, con la presencia de perforación timpánica y colesteatoma, que determinará la presencia de lesiones mucho más acentuadas en cuanto a la evolución auditiva o complicaciones locales o sistémicas. OBJETIVO. Determinar la asociación existente entre la presencia de colesteatoma y perforación timpánica en pacientes con otitis media crónica. MATERIALES Y MÉTODOS. Estudio epidemiológico analítico retrospectivo. Población de 4 733 y muestra de 75 pacientes para casos y 75 para controles basados en historias clínicas tomadas del sistema informático AS 400, que acudieron a la consulta externa de torrinolaringología del Hospital de Especialidades Carlos Andrade Marín en el periodo de enero de 2018 a diciembre de 2019; Criterios de inclusión para grupo de casos: Hombres y mujeres de 20 a 65 años de edad, diagnóstico de otitis media crónica, diagnóstico de colesteatoma ótico. Criterios de inclusión para grupo controles: Hombres y mujeres de 20 a 65 años de edad, no presentar diagnóstico de colesteatoma. RESULTADOS. Se observó una relación fuerte entre el poseer perforación timpánica y el desarrollo de colesteatoma con un valor de OR 33,14 con un IC al 95% de 31,94 ­ 34,34, con lo que se comprobó la hipótesis del estudio. Se determinó que la perforación timpánica es un factor de riesgo asociado con el desarrollo de colesteatoma en pacientes con otitis media crónica, la prevalencia de colesteatoma en relación a la edad estuvo en un 72% en pacientes de 41 a 65 años, con mayor predominancia en mujeres en un 57,3%. DISCUSIÓN. La presencia de perforación timpánica de acuerdo a lo observado es un factor de riesgo para el desarrollo de colesteatoma, ligado en su mayoría a cuadros de Otitis Media Crónica. CONCLUSIONES. Se confirmó que la perforación timpánica, es un factor de riesgo en el desarrollo del colesteatoma en los pacientes que tienen otitis media crónica, lo que demuestra la necesidad de manejo actualizado y continuo en pacientes con esta patología de oído. Se requieren estudios con muestras más amplias para determinar otros factores de riesgo como sexo, nivel de educación y edad que podrían influir en el desarrollo de colesteatoma.

INTRODUCTION. Ear pathology is a frequent disease in our environment, associated with repeated ear infections, with the presence of tympanic perforation and cholesteatoma, which will determine the presence of much more accentuated lesions in terms of auditory evolution or local or systemic complications. OBJECTIVE. To determine the association between the presence of cholesteatoma and tympanic perforation in patients with chronic otitis media. MATERIALS AND METHODS. Retrospective analytical epidemiological study. Population of 4 733 and sample of 75 patients for cases and 75 for controls based on clinical histories taken from the AS 400 computer system, who attended the Otorhinolaryngology outpatient clinic of the Carlos Andrade Marín Specialties Hospital in the period from January 2018 to December 2019; Inclusion criteria for case group: Men and women aged 20 to 65 years, diagnosis of chronic otitis media, diagnosis of otic cholesteatoma. Inclusion criteria for controls group: men and women aged 20 to 65 years, no diagnosis of cholesteatoma. RESULTS. A strong relationship was observed between having tympanic perforation and the development of cholesteatoma with an OR value of 33,14 with a 95% CI of 31,94 - 34,34, thus proving the study hypothesis. It was determined that tympanic perforation is a risk factor associated with the development of cholesteatoma in patients with chronic otitis media, the prevalence of cholesteatoma in relation to age was 72% in patients aged 41 to 65 years, with greater predominance in women in 57,3%. DISCUSSION. The presence of tympanic perforation according to what was observed is a risk factor for the development of cholesteatoma, mostly linked to Chronic Otitis Media. CONCLUSIONS. It was confirmed that tympanic perforation is a risk factor in the development of cholesteatoma in patients with chronic otitis media, which demonstrates the need for updated and continuous management in patients with this ear pathology. Studies with larger samples are required to determine other risk factors such as sex, education level and age that could influence the development of cholesteatoma.

Concordancia entre hallazgos radiológicos y quirúrgicos en pacientes sometidos a mastoidectomía

Introducción: Existe gran controversia acerca de los signos radiológicos de la patología de oído medio y su relación con la extensión real de la patología que nos sugiera decidir un comportamiento determinado. Objetivo: establecer la concordancia entre los hallazgos radiológicos y quirúrgicos encontrados en pacientes sometidos a mastoidectomía. Material y Método: Diseño observacional descriptivo de corte transversal de prueba diagnóstica. Se revisó fichas clínicas y se especificó el tipo de cirugía junto con los hallazgos intra-quirúrgicos. Resultados: se observó erosión de la cadena osicular intraoperatoria en 75 pacientes, 63 predichos correctamente por tomografía computada (TC), con sensibilidad del 84% y especificidad del 94% (k = 0,625). Se detectó erosión del tegmen tympani en nueve pacientes por TC de los 10, con un VPP = 90% y un VPN = 95% (k = 0,809). Hubo sospecha de erosión del canal semicircular lateral en cinco pacientes y se encontró dehiscencias intraoperatorias en 12, sin falsos positivos (k = 0,554). La TC detectó dehiscencia del canal del nervio facial con sensibilidad del 55% y especificidad del 98% (k = 0,636). Conclusión: Los resultados sugieren que la TC preoperatoria con evidencia radiológica de erosión osicular tiene una buena a muy buena concordancia con los hallazgos intraoperatorios.

Introduction: There is a great controversy about the radiological signs of middle ear pathology and its relation with the real extension of the pathology that suggests us to decide a certain medical choice. Aim: To establish concordance between radiological and surgical findings in patients submitted to mastoidectomy. Material and Method: An observational and descriptive design of retrospective cross-sectional diagnostic test. Clinical records were reviewed to specify the type of surgery and intraoperative findings. Results: Intraoperative ossicular chain erosion was observed in 75 patients, 63 were correctly predicted by computed tomography (CT), with a sensitivity of 84% and specificity of 94% (k = 0.625). Tegmen tympani erosion was detected by CT in 9 patients out of 10, with a PPV = 90% and NPV = 95% (k = 0.809). There was suspected lateral semicircular canal erosion in 5 patients and intraoperative dehiscence was found in 12, with no false positives (k = 0.554). CT detected facial nerve canal dehiscence with sensitivity of 55% and specificity of 98% (k = 0.636). Conclusion: The results suggest that preoperative CT with radiological evidence of ossicular erosion has good to very good agreement with intraoperative findings.

Implante coclear en otitis media crónica: Revisión de literatura

La otitis media crónica (OMC) activa y colesteatomatosa se consideraban una contraindicación para el uso de implante coclear (IC) en el pasado. Actualmente, se han propuesto múltiples opciones quirúrgicas para el IC, de acuerdo con las características clínicas del paciente y el tipo de patología otológica prexistente. La cirugía del IC se puede realizar en un tiempo o en etapas, siendo fundamental intervenir un oído seco y estable previo a la instalación definitiva del electrodo para reducir complicaciones y obtener resultados auditivos satisfactorios. El IC es un tratamiento seguro y eficaz en pacientes con OMC. Múltiples reportes internacionales han evidenciado baja tasa de complicaciones mayores y óptimos resultados auditivos funcionales en pacientes con hipoacusia y OMC.

Supurative and cholesteatomatous chronic otitis media (COM) were considered a contraindication to cochlear implant (CI) use in the past. Currently, multiple surgical options have been proposed for CI according to the clinical characteristics of the patient and the type of pre-existing otological pathology. Cochlear implant surgery can be performed in a single time or in stages, being essential to intervene a dry and stable ear prior to the definitive installation of the electrode to reduce complications and obtain satisfactory hearing results. CI is a safe and effective treatment in patients with COM. Multiple international reports have shown a low rate of major complications and optimal functional hearing results in patients with hearing loss and COM.

Mongolian Gerbils as a Model for the Study of Cholesteatoma: Otoendoscopic as a Diagnostic Tool

Abstract Introduction Cholesteatoma is a disease with significant clinical impact but is incompletely understood. The challenge of performing studies with long-term follow-up in humans is a factor that has restricted the advance of knowledge in this field. Thus, the use of animal models is highly pertinent, and the Mongolian gerbil model has emerged as one of the most useful. Objective The present study aims to evaluate, through serial otoendoscopies, the development and characteristics of pars flaccida retraction pocket and cholesteatoma in Mongolian gerbils after obliteration of the eustachian tube and compare it with the control group. Methods Forty Mongolian gerbils were divided into two groups of 20 animals each. In the intervention group, the animals were followed with serial otoendoscopies after eustachian tube obliteration. In the control group, the animals were only followed through serial otoendoscopies. Results At the end of the 16-week follow-up, cholesteatoma was present in 13 of 38 (34.2%) ears in the intervention group, and in 7 of 34 (20.6%) in the control group (p = 0.197). When we considered cholesteatoma and its potential precursor, pars flaccida retraction pocket, in a combined way, we verified it in 23 of 38 (60.8%) in the intervention group and in 11 of 34 (32.3%) in the control group (p = 0.016). Conclusions Over the 16 weeks of follow-up, serial otoendoscopies enabled us to evaluate the formation and development of pars flaccida retraction pockets and cholesteatomas in Mongolian gerbils and proved to be an excellent diagnostic tool.

Are Hounsfield Unit Measurements of Bony Absorption Changes in Cholesteatoma Helpful?

Abstract Introduction High-resolution computed tomography (HRCT) scans of the temporal bone are used to assess the bony erosion of the middle-ear structures whenever cholesteatoma is suspected. Objective To study the differences in HRCT Hounsfield unit (HU) index measurements of middle-ear bony structures between an ears with and without cholesteatoma. Methods A retrospective study of 59 patients who underwent surgery due to unilateral cholesteatoma. The HRCT HU index of the scutum, of three middle-ear ossicles, of the lateral semicircular canal (LSCC), and of the fallopian canal was measured in both ears. A comparison was made between the cholesteatoma and the non-cholesteatomatous ear (control). All measurements were conducted by an otolaryngologist. To assess the interobserver bias, 10% of the samples were randomly and independently assessed by another otolaryngologist and a neuroradiologist who were blinded. Results The average HU index was lower in the ear with cholesteatoma when compared with the non-cholesteatomatous ear. While the differences were statistically significant regarding the measurements of the scutum (516.02 ± 311.693 versus 855.64 ± 389.999; p = 0.001), the malleus (1049.44 ± 481.765 versus 1413.47 ± 313.376; p = 0.01), and the incus (498.03 ± 264.184 versus 714.25 ± 405.631; p = 0.001), the differences in the measurements of the LSCC (1042.34 ± 301.066 versus 1154.53 ± 359.609; p = 0.69) and of the fallopian canal (467.19 ± 221.556 versus 543.51 ± 263.573; p = 0.108) were not significantly different between both groups. The stapes was immeasurable in both groups due to its small size. Conclusion Hounsfield unit index measurements are a useful tool that may aid in the diagnosis of early-stage cholesteatoma.

Endoscopic-Assisted Canal Wall-up Tympanomastoidectomy for Reduction of Residual Cholesteatoma

Abstract Introduction The treatment of cholesteatoma is generally surgical, and the major obstacle is the high prevalence of recidivism. The endoscopic ear surgery technique is proposed to minimize this problem. Objectives To utilize endoscopes to visualize and manipulate cholesteatoma residues after microscopic removal Methods Cross-sectional study. Thirty-two patients with cholesteatoma underwent microscopic wall-up mastoidectomy combined with the endoscopic approach. The subjects were assessed for the presence and location of covert disease. Results Of the 32 cases, 17 (53.12%) had residual cholesteatoma in the endoscopic phase. Minimal disease was found, usually fragments of the cholesteatoma matrix. Pars tensa cholesteatomas had more covert disease than pars flaccida cholesteatomas (62.50% vs 43.75%). Posterior recesses (47.05%) and tegmen tympani (41.17%) were the locations with more covert disease (p< 0.05). Conclusion Cholesteatomas of the pars tensa presented more residual disease and were significantly more common in the posterior recesses and tegmen tympani.

External Auditory Canal Cholesteatoma: Clinical and Radiological Features

Abstract Introduction External auditory canal cholesteatoma (EACC) is often misdiagnosed. Objectives To outline the clinical presentation of EACC, and to describe its radiological findings on high-resolution computed tomography (HRCT) of the temporal bone. Methods The clinical records of all patients diagnosed with EACC from April 2017 to March 2020 in a tertiary care center were retrospectively reviewed. The clinical presentation, the findings on the HRCT of the temporal bone, and the treatment provided were analyzed. Results A total of 9 patients, 7 males and 2 females, with a mean age of 30 years, were diagnosed with primary EACC. Six patients presented with otorrhoea, three, with otalgia, three. with hearing loss, and one with facial palsy. Some patients had multiple symptoms. The most common findings on otomicroscopy were destruction of the posterior and inferior canal walls, with cholesteatoma and intact tympanic membrane (six patients). Two patients had aural polyp, and one had a narrow ear canal due to sagging of the posterior canal wall. On HRCT, all nine patients showed soft-tissue density in the external auditory canal with erosion of the canal wall. The disease extended to the mastoid in eight cases, and to the cavity of the middle ear in one. There were three cases of dehiscence of the facial canal. Dehiscence of the dural and sinus plates was observed in two cases each. Eight patients underwent mastoidectomy, and one underwent debridement with canalplasty. Conclusion Review of the clinical and radiological findings is essential to reduce the rate of misdiagnosis.

Fibrous dysplasia: rare manifestation in the temporal bone / Displasia fibrosa: manifestação rara no osso temporal

Abstract Introduction: Fibrous dysplasia is a benign disorder, in which normal bone is replaced by fibrosis and immature bone trabeculae, showing a similar distribution between the genders, and being more prevalent in the earlier decades of life. Fibrous dysplasia of the temporal bone is a rare condition, and there is no consensus as to whether it is more common in monostotic or polyostotic forms. External auditory meatus stenosis and conductive dysacusis are the most common manifestations, with cholesteatoma being a common complication, whereas the involvement of the otic capsule is an unusual one. Surgical treatment is indicated to control pain or dysacusis, otorrhea, cholesteatoma, and deformity. Objectives: To describe the clinical experience of a tertiary referral hospital with cases of fibrous dysplasia of the temporal bone. Methods: Sampling of patients diagnosed with fibrous dysplasia of the temporal bone, confirmed by tomography, treated at the pediatric otology and otorhinolaryngology outpatient clinics, between 2015 and 2018. The assessed variables were age, gender, laterality, external auditory meatus stenosis, deformity, hearing loss, presence of secondary cholesteatoma of the external auditory meatus, lesion extension and management. Results: Five patients were included, four females and one male, with age ranging from 13 to 34 years. Three had the polyostotic form and two the monostotic form of fibrous dysplasia of the temporal bone. Four patients had local deformity and external auditory meatus stenosis, two of which progressed to cholesteatoma. All patients showed some degree of hearing impairment. All had preserved otic capsule at the tomography. Two patients are currently undergoing clinical observation; two were submitted to tympanomastoidectomy due to secondary cholesteatoma; one was submitted to lesion resection, aiming to control the dysacusis progression. Conclusion: Five cases of fibrous dysplasia of the temporal bone were described, a rare disorder of which the otologist should be aware.

Resumo Introdução: Displasia fibrosa é uma desordem benigna, na qual o osso é substituído por fibrose e trabeculado ósseo imaturo, com distribuição semelhante entre sexos, mais comum nas primeiras décadas de vida. O acometimento do osso temporal pela displasia fibrosa é raro, não há consenso se é mais comum nas formas monostóticas ou poliostóticas. Estenose do meato acústico externo e disacusia condutiva são as manifestações mais comuns. Colesteatoma é também uma complicação comum e o acometimento da cápsula ótica incomum. O tratamento cirúrgico está indicado para controle de dor ou disacusia, otorreia, colesteatoma, deformidade. Objetivos: Descrever a experiência clínica de hospital terciário de referência com casos de displasia fibrosa do osso temporal. Método: Amostragem dos pacientes com diagnóstico de displasia fibrosa do osso temporal, confirmado pela tomografia, atendidos nos ambulatórios de otologia e otorrinolaringologia pediátrica, entre 2015 e 2018. As variáveis avaliadas foram idade, gênero, lateralidade, estenose do meato acústico externo, deformidade, perda auditiva, presença de colesteatoma secundário de meato acústico externo, extensão da lesão e conduta adotada. Resultados: Foram incluídos cinco pacientes, quatro do sexo feminino e um masculino, de 13-34 anos. Três apresentaram a forma poliostótica da displasia fibrosa do osso temporal e dois a forma monostótica. Quatro apresentaram deformidade local e estenose do meato acústico externo, dois desses evoluíram com colesteatoma. Todos manifestaram algum grau de comprometimento auditivo. Todos apresentaram cápsula ótica preservada na tomografia. Duas pacientes estão em observação clínica; duas foram submetidas a timpanomastoidectomia devido a colesteatoma secundário; um foi submetido a ressecção da lesão para controle de progressão da disacusia. Conclusão: Foram descritos cinco casos de displasia fibrosa do osso temporal, desordem rara para a qual o otologista deve estar atento.

Absceso mastoideo como presentación de colesteatoma congénito en preescolar ¿cuál es el origen? / Mastoid abscess as congenital cholesteatoma presentation in a child what is the origin?

Resumen El colesteatoma congénito es una entidad que puede manifestarse con una amplia variedad de síntomas o ser silente durante largo tiempo y constituir un hallazgo incidental. Una vez diagnosticada es importante valorar su extensión y el compromiso de estructuras adyacentes, para lograr una adecuada planificación quirúrgica, eliminando la enfermedad y manteniendo la mejor funcionalidad posible. Se presenta un caso de colesteatoma congénito infantil.

Abstract Congenital cholesteatoma is an entity that can manifest with a wide variety of symptoms or be silent for a long time and constitute an incidental finding. Once diagnosed, it is important to assess the extension to apply the most efficient treatment, eliminating the disease and providing functionality if possible. A case of congenital cholesteatoma in a child is presented.

O uso do retalho de fáscia parietotemporal no tratamento do colesteatoma recidivado: caso clínico / The use of parietotemporal fascia flap in the treatment of recurrent cholesteatoma: clinical case

O colesteatoma consiste em um processo inflamatório que resulta na migração do epitélio escamoso queratinizado para o ouvido médio. Embora considerada uma entidade histopatologicamente benigna, pode se comportar de forma bastante agressiva sendo uma importante causa de surdez em todos os países. Descarga, dor, ruptura do tímpano com extensão para o ouvido interno levando à surdez e vertigem, são as manifestações clínicas mais comuns. O tratamento consiste na excisão cirúrgica de todo o epitélio estranho da orelha média. As recorrências podem chegar a 50% e são um desafio para os médicos de ouvido, nariz e garganta. Neste relato de caso descrevemos um caso de colesteatoma recorrente adquirido em um hospital terciário em Portugal, tratado com ablação radical de ouvido médio e cavidade mastóide pelos médicos otorrinolaringologistas. A opção reconstrutiva escolhida foi a obliteração do espaço morto com retalho fascial temporo-parietal pelo Serviço de Cirurgia Plástica.

Cholesteatoma consists of an inflmmatory process that results in the migration of squamous keratinized epithelium into the middle ear. Although regarded as a histopathologically benign entity it can behave quite aggressively being an important cause of deafness in all countries. Ear discharge, pain, ear drum rupture with extension into the inner ear leading to deafness and vertigo, are the most common clinical manifestations. Treatment consists of surgically excising all the foreign epithelium from the middle ear. Recurrences can be as high as 50% and are a challenge to Ear, Nose and Throat doctors. In this case report we describe a case of an acquired recurrent cholesteatoma in a tertiary hospital in Portugal, treated with radical ablation of middle ear and mastoid cavity by the otolaryngologists. The chosen reconstructive option was obliteration of the dead space using a temporo-parietal fascial flap by the Plastic Surgery Department

Colesteatoma complicado en un niño con síndrome de Down: un caso clínico / Complicated cholesteatoma in a child with Down syndrome: a case report

El colesteatoma adquirido en niños es una enfermedad agresiva debido a su rápido crecimiento y la alta tasa de recurrencia. Las complicaciones se dividen en dos grandes grupos: las relacionadas con el hueso temporal (dentro o fuera de él) y las complicaciones intracraneales. El absceso subperióstico es la complicación extratemporal más común y es más frecuente en los niños más pequeños. Los pacientes que padecen síndrome de Down tienen una prevalencia elevada (superior al 80 %) de otitis media con efusión, que puede estar determinada anatómicamente por la hipoplasia mediofacial con una nasofaringe estrecha y adenoides hipertrófica, junto a trastornos funcionales y mecánicos de la trompa auditiva. Se presenta un niño de 8 años con síndrome de Down que desarrolló un absceso subperióstico como complicación de un colesteatoma que requirió abordaje quirúrgico inmediato para su resolución.

Acquired cholesteatoma in children is an aggressive disease due to its rapid growth and high recurrence rate. The complications are divided into intra-and extratemporal complications or intracranial complication. Subperiosteal abscess is the most common extratemporal complication. It is most frequent in young children. However, there are also other associated complications described in the literature. Down syndrome patients have anatomical and functional predisposing factors that contribute to chronic cholesteatomatous otitis media. The prevalence is greater than 80 %. In this report, we present a case of subperiosteal abscess in an 8-year-old child with Down's syndrome. This abscess presented as a complication of an extended cholesteatoma and required inmediate surgery for resolution.

Audiological Outcome in Myringoplasties with an Intact Ossicular Chain: Is there a Difference between Chronic Otitis with or without Cholesteatoma?

Abstract Introduction Chronic otitis media (COM) with a central perforation or a concomitant cholesteatoma are both inflammatory lesions, however, with different etiologies. Both entities may present with an intact chain, and the final reconstruction is quite similar. Does it also apply for the hearing outcome? Objectives In a retrospective analysis, we investigated the preoperative hearing and the final hearing outcome of two groups of patients: those with COM and those with cholesteatoma, and compared various factors. Methods Patients operated between 2010 and 2019 were entered prospectively into a research database, and the integrity of the ossicular chain, the extent of the cholesteatoma, and the findings on computed tomography (CT) scans were retrospectively analyzed and correlated to the final hearing outcome. Results Out of 210 tympanoplasties for COM, 162 (80%) presented with an intact chain, and 85 (40%) ears could be analyzed. Out of 283 cholesteatoma surgeries, 53 (19%) ears presented with an intact chain. The preoperative air-bone gap (ABG) was worse in the COM group, but the postoperative ABG over the frequencies of 0.5 kHz and 4 kHz was the same (10 dB to 12 dB) in both groups, and remained within 20 dB in 90% (40 and 78 patients, respectively). The extension of the disease was rather limited in the cholesteatoma group (stages Ch1a and 1b), and better pneumatization and ventilation were beneficial for a good result. Postoperatively, the frequency of 4 kHz had the largest ABG (14 dB and 18 dB). Conclusion Overall, 80% of the patients with COM and less than 20% of those with cholesteatoma had an intact and mobile chain at surgery. Using equivalent surgical techniques for the tympanoplasty, the final outcome was almost the same for both groups, with a mean ABG of 10 dB to 12 dB.

Intracranial epidermoid cyst in a cat / Cisto epidermoide intracraniano em um gato

ABSTRACT: Epidermoid cysts, also known as epidermal and keratin cysts, or congenital cholesteatomas are benign congenital non-neoplastic tumors, rarely observed in the brain of humans and some animal species (dogs, horses, mice, and rats). Histologically, they are composed of laminated, concentrically arranged keratin surrounded by a thin layer of stratified squamous epithelium. We describe a case of intracranial epidermoid cyst in a 6-year-old mixed-breed male cat in southern Brazil. The patient presented central vestibular syndrome. Given the poor prognosis and the fact that it belonged to a shelter with many dogs and cats, the owner requested euthanasia, and a thorough post-mortem examination was conducted immediately after death. The definitive diagnosis was based on histopathological findings. To the best of our knowledge, this is the first report of an intracranial epidermoid cyst in a cat.

RESUMO: Cisto epidermoide, também denominado cisto epidermal, cisto de queratina ou colesteatoma congênito é um tumor não neoplásico, benigno e congênito raramente encontrado no encéfalo de humanos e de algumas poucas espécies animais (cães, equinos, camundongos e ratos). Histologicamente, esse tumor é composto por queratina laminada concentricamente arranjada e circundada por uma fina parede de epitélio escamoso estratificado. Descreve-se um caso de cisto epidermoide intracraniano em um gato, macho, sem raça definida, de seis anos de idade, no sul do Brasil. O paciente foi encaminhado para atendimento veterinário por apresentar sinais de síndrome vestibular central. Devido ao mau prognóstico e por pertencer a um abrigo com muitos cães e gatos, o proprietário optou pela eutanásia e o paciente foi encaminhado para a realização de necropsia. O diagnóstico definitivo foi baseado nos achados histopatológicos. Pelo conhecimento dos autores, este é o primeiro relato de um cisto epidermoide intracraniano em um gato.