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Congenital cholesteatoma is a cyst of keratinizing squamous cell epithelium in the setting of an intact tympanic membrane, in a patient without a history of otorrhea, tympanic membrane perforation, or otologic surgery. The most common presentation of a congenital cholesteatoma is that of an asymptomatic pearly white mass in the anterosuperior quadrant of the tympanic cavity. The etiology of congenital cholesteatoma has been debated at length, with the leading theory being the epithelial rest theory. Treatment for congenital cholesteatoma is surgical, with advances in endoscopic ear surgery allowing for improved intraoperative visualization and postoperative lowered recidivism rates., Competing Interests: Disclosure The author has nothing relevant to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Petrous bone cholesteatoma, or cholesteatoma that extends beyond the middle ear and mastoid, represents a rare but destructive pathology. Diagnosis can be difficult before substantial morbidity is incurred, and patients can present with life-threatening complications. Determination of disease extent and the functional status of the facial nerve and cochleovestibular system are critical in surgical planning. Typically, surgery involves ablative procedures with the goal of complete disease resection given the low likelihood of preserved inner ear function. In experienced hands, disease control and facial nerve outcomes are favorable; however, disease recidivism is not uncommon and, thus, these patients require lifelong surveillance., Competing Interests: Disclosure Financial material & support: This study was performed without commercial sponsorship or support. Relevant conflicts of interest to declare: no relevant financial disclosures relating to this article., (Published by Elsevier Inc.)

Bilateral congenital cholesteatoma (BCC) is rare among congenital cholesteatoma (CC) cases, and bilateral surgery poses a significant psychophysical burden on pediatric patients. Here, we present 2 pediatric cases of BCC that were successfully managed using simultaneous bilateral transcanal endoscopic ear surgery (TEES). The surgical approach provided clear visualization of the middle ear structures, enabling successful cholesteatoma removal with minimal invasiveness and a short operative time. In both cases, follow-up computed tomography showed no recurrence, and pure-tone audiometry results demonstrated good outcomes. Simultaneous bilateral TEES can be a viable option for pediatric BCC, as it reduces patient burden due to its less invasive procedure and short operative time.

Pneumocephalus refers to air inside the cranium; however, otogenic pneumocephalus is rarely reported in the literature. The neurological presentations of pneumocephalus include headache, lethargy, confusion, disorientation, and seizure. Here, we have reported a case of a 42-year-old woman with extensive pneumocephalus and cerebrospinal fluid leak secondary to petrous bone cholesteatoma. She presented to the emergency department with sudden headache and left ear discharge. Physical examination revealed watery otorrhea through a hole in the tympanic membrane. Radiological studies demonstrated extensive soft tissue in the left middle ear and mastoid extending to the internal auditory canal. Free intracranial air was observed, and bony destruction was seen in the cochlea, vestibule, and semicircular canals. The patient was managed surgically via the transotic approach and fully recovered. Although otogenic pneumocephalus is rarely encountered in clinical practice, early diagnosis and urgent management are important to prevent fatal complications., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: Cholesteatoma is a rare disease characterized by the accumulation of keratinized squamous epithelial cells in the middle ear or mastoid cavity. Vertigo and facial palsy, which are rare complications, may indicate erosion into the semicircular canals or the fallopian canal., Patient Concerns: A 40-year-old woman presented to our clinic with progressive right-sided hearing loss over 5 years (primary concern). Approximately 10 years ago, the patient had developed acute right-sided facial weakness with no additional symptoms. A neurologist at another hospital had diagnosed her condition as Bell's palsy and treated it accordingly., Diagnosis: Adult-onset congenital cholesteatoma in the hypotympanum., Intervention: Combined endoscopic and microscopic removal of the cholesteatoma., Outcomes: Physical examination revealed slight improvement in right-sided peripheral facial palsy., Lesson: Routine eardrum examination is recommended for patients presenting with isolated peripheral facial palsy. If necessary, a patient should be referred to an otologist for further evaluation and treatment., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

External Auditory Canal Cholesteatomas (EACC), is an exceptionally rare condition with a prevalence of only 0.1-0.5% among new patients1. EACC are known to possess bone eroding properties, causing a variety of complications, similar to the better-known attic cholesteatomas. We describe here the novel surgical management of a case of EACC. She is 38-year-old female who presented with otorrhea for 6 months. Clinical examination and radiological investigations suggested the diagnosis of an external auditory canal cholesteatoma. The patient underwent modified radical mastoidectomy with type 1 tympanoplasty with meatoplasty. Post-operatively, the patient showed marked clinical improvement.

A 48-year-old man presented with a chief complaint of intermittent right ear otorrhea of several-month duration, occasional otalgia and progressive unilateral hearing impairment. He also reported frequent episodes of headache and pressure in the sinuses and maxilla. Previous systemic treatment with antibiotics failed to alleviate the symptoms. A head/neck CT showed completely normal mastoid, middle ear and external auditory canal regions without any evidence of opacification or bone erosion. Otoscopic examination of the right ear disclosed aggregates of dried, brown, fibrillar material and debris occluding the external auditory canal and obstructing the otherwise intact tympanic membrane. Dilation of the external auditory canal or thickening of the tympanic membrane were not appreciated. The canal was debrided and the fibrillar material was placed in formalin. Histopathologic examination revealed numerous branching, septated fungal hyphae organized in densely-packed clusters. In other areas, the fungal hyphae abutted or were attached to lamellated collections of orthokeratin. As highlighted by GMS staining, the fungi were morphologically compatible with Aspergillus species. The clinicopathologic findings supported a diagnosis of fungal otitis externa, while the numerous anucleate squamous cells were compatible with colonization of an underlying, probably developing, cholesteatoma. Culture of material isolated from the external auditory canal confirmed the presence of Aspergillus flavus. In this illustrative case, we present the main clinical and microscopic characteristics of Aspergillus-related otomycosis developing in the setting of a tautochronous cholesteatoma., (© 2024. The Author(s).)

Background: The optimal timing for surgery to promote postoperative recovery in children with congenital stenosis of the external auditory canal with external auditory canal cholesteatoma, who are susceptible to exudative inflammation of middle ear and mastoid process, is still uncertain., Objectives: To investigate the treatment of congenital stenosis of external auditory canal with external auditory canal cholesteatoma complicated with exudative inflammation., Material and Methods: A retrospective analysis of 45 patients with congenital stenosis of external auditory canal with external auditory canal cholesteatoma complicated with mastoiditis. Based on whether effusion had resolved at the time of surgery, the patients were divided into two groups. Pure-tone audiometry tests were performed before and after surgery for both groups., Results: The average postoperative duration of dry ear was 36.38 days in Group 1 and 47.90 days in Group 2 ( p  < 0.05). Among patients who underwent hearing reconstruction, the average air conduction threshold decreased by 15-36 dBHL in Group 1 and by 7-22 dBHL in Group 2 ( p  < 0.05)., Conclusions and Significance: The postoperative outcomes of dry ear and hearing improvement can be enhanced by performing operation after effusion resolution in cases of congenital external auditory canal stenosis with external auditory canal cholesteatoma exudative inflammation.

Background: This study aimed to discuss 3 cases of congenital cholesteatoma located posterior to the sigmoid sinus, with no/minimal involvement of mastoid, and compare them with cases presented in the literature to better define this rare entity., Methods: Retrospective chart analysis of 3 congenital cholesteatomas located posterior to the sigmoid sinus treated surgically in 2 skull-base centers and literature review. Though congenital cholesteatoma can arise outside the middle ear, only a few cases presenting in the retrosigmoid occipital bone have been described earlier., Results: In all 3 patients, there was a delay in the presentation, as symptoms were nonspecific or lacking, leading in 1 case to severe complications. Computed tomography and magnetic resonance imaging, especially diffusion-weighted imaging scans, allowed accurate diagnosis and surgical planning. Surgery happened to be challenging due to the tight adherence of the cholesteatoma to the thinned dural surface. Complete excision was achieved in all the cases., Conclusion: Congenital cholesteatoma located posterior to the sigmoid sinus is a rare entity and is even more exceptional after a critical review of the literature. Complete excision is quintessential to prevent intradural extension or infection. The most important surgical issue is the management of the posterior fossa dura and the sigmoid sinus. We recommend meticulous dissection with slow peeling of the epithelial lining from the dura. Bipolar coagulation of the dura may help in avoiding recidivism. Moreover, cerebrospinal fluid (CSF) leak during dissection has to be avoided as long as possible, because the loss of tension of the already thinned dura makes its peeling particularly difficult.

Keratosis obturans (KO) and external auditory canal cholesteatoma (EACC) are two distinct keratinaceous lesions of the external ear. This article reviews the signs, symptoms, pathophysiology, workup, and treatment of each. Patients with either pathology can often be managed in the clinic with debridement; however, EACC is more likely to involve osteonecrosis and require more extensive operative management if disease is not confined to the canal on account of the bony erosion characteristic of cholesteatoma. If required for extensive disease, surgical approaches to both pathologies are similar., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Congenital temporal bone cholesteatoma is a rare lesion in otolaryngology.The disease is locally invasive and may lead to significant complications,including hearing loss（conductive or sensorineural）, temporal bone destruction and intracranial invasion. This article reviews the characteristic symptoms of congenital temporal bone cholesteatoma, testing and imaging of the disease, stage and the current treatment options in order to promote awareness to this rare disease entity and perform early surgical treatment, effectively avoid the destruction of the temporal bone and its surrounding structures, thereby reducing the occurrence of complications. By improving the understanding of the disease and performing early surgical treatment, the destruction of the temporal bone and its surrounding structures can be effectively avoided, thereby reducing the occurrence of complications., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Introduction: External auditory canal cholesteatoma (EACC) is a rare disease, with an estimated incidence of approximately 1:1,000 adult and 1.6:1,000 pediatric otologic patients. Systematic studies of chronic ear disease and taste alteration prior to surgery are rare; in fact, there are no such studies for EACCs. Therefore, we describe chorda tympani nerve (CTN) dysfunction and the related clinical consequences in EACC patients., Methods/study Design: Between 1992 and 2021, we retrospectively analyzed the symptoms, signs, and radiological and intraoperative descriptions of CTN involvement in 73 patients. Liquid taste tests and, since 2009, Taste StripsTM as well as an olfactory screening test (Smell DiskettesTM) have been performed for all symptomatic patients and, when feasible, all other EACC patients., Results: Ten of 73 patients complained subjectively of dysfunction, and 8 showed abnormal taste test results. Four patients complained of olfactory dysfunction (3 cases with pathological taste tests). Gustatory dysfunction was most frequent in radiogenic EACC cases (n = 4), followed by postoperative EACC (n = 3). Two postoperative patients were asymptomatic despite abnormal test results. Rarely, patients with idiopathic (n = 2) and posttraumatic (n = 1) EACC showed acute taste dysfunction that was confirmed in each with abnormal test results., Discussion/conclusion: CTN dysfunction often developed asymptomatically in chronic ears, except for idiopathic and posttraumatic EACCs under previous healthy middle ear conditions. Taste disturbance is not a cardinal symptom of EACC, but objective testing suggests that up to one out of 10 EACC patients with advanced disease may experience regional gustatory dysfunction prior to surgery. Especially in context of a new and acute presentation, regional taste dysfunction may alert the clinician of potential progressive EACC invasion and danger to the facial nerve., (© 2023 S. Karger AG, Basel.)

The article describes clinical cases of invasive cholesteatoma of the temporal bone pyramid, leading to a massive destruction of the surrounding structures. Paying attention to the importance of knowing the microsurgical anatomy of critical structures of temporal bone (the anterior bony plate (cog), the tensor tympani fold and the tendon, the tympanic orifice of the eustachian tube, the pretympanic recess, tympanic sinuses) bearing a significance and helping to improve the removal of the invasive cholesteatoma.

Langerhans cell histiocytosis is a rare condition affecting the temporal bone in up to 60% of cases. Symptoms are non-specific and the differential diagnosis includes infection, benign lesions such as cholesteatoma, and malignant lesions of the skull base. Here, we report the case of a 14-yearold child referred with chronic ear discharge, and background of multifocal Langerhans cell histiocytosis 9 years prior. Recurrence of Langerhans cell histiocytosis was initially suspected and systemic treatment was considered. Further imaging workup and surgical exploration of the mastoid showed a secondary acquired cholesteatoma arising from a dehiscent posterior ear canal wall. Surgical removal of the cholesteatoma was performed with a canal wall down procedure. We review the presentation and management of temporal bone Langerhans cell histiocytosis. We recommend that cholesteatoma should be considered in case of recurrence of otological symptoms in patients with a background of Langerhans cell histiocytosis.

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

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.

Background: Congenital cholesteatomas account for just up to 5% of all cholesteatomas and most commonly arise in the petrous apex and middle ear. Congenital cholesteatomas arising in the mastoid are rare and typically present late., Methods: In this study, we report a case series of 3 cases managed in our department between 2006 and 2021 and present a summary of the current literature., Results: Congenital cholesteatomas arising in the mastoid is a rare finding and even among reported cases, not all are clearly mastoid in origin. Their location allows for considerable growth before symptoms develop. Pain and localized swelling in the temporal area are the most common presenting symptoms which can lead to diagnostic challenges. Our cases show that although surgery is often appropriate, conservative manage- ment may be suitable in certain situations., Conclusion: Congenital cholesteatoma of mastoid origin is rare and can present a diagnostic challenge. Greater awareness is important to facilitate early detection. A high index of suspicion is needed in those presenting with retro-auricular pain and swelling in the context of a normal ontological examination.

Objective: To evaluate the effectiveness of surgical treatment of patients with petrous bone cholesteatoma (PBC) depending on the localization of the pathological process., Material and Methods: The analysis of surgical treatment using various surgical approaches and its results in 32 patients with PBC, depending on the type, localization in petrous bone and intraoperative findings, is presented. Patients with supralabirint PBC underwent extended atticoantromastoidotomy with tympanoplasty and mastoidoplasty with automaterials ( n =19), labyrinthectomy ( n =4), subtotal petrozectomy with labyrinthectomy and suturing of the external auditory meatus (EAM) ( n =2). In infralabirint and infralabirint-apical PBC, a transotic approach was used with Rambo suturing of EAM ( n =9). The pre-sigmoid approach was performed in 1 patient. With an extradural subtemporal approach, PBC of apical localization was removed in 1 case., Results: After surgical treatment, hearing remained at the same level in 15 (47%) patients, 14 of them had deafness. In the early postoperative period, a temporary increase in bone conduction hearing thresholds by 10-20 dB was detected in 14 (44%) patients with their gradual recovery over 3 months. Deafness in the postoperative period developed in 3 (9%) patients after removal of supralabirint cholesteatoma. In the early postoperative period, 3 (9%) patients developed systemic dizziness, which was stopped after 3 months. In 25 (78%) patients, the function of the facial nerve in the early postoperative period remained at the same level, of which 14 (44%) were normal, and 11 (34%) had the same degree according to the House-Brackmann (HB) classification. Improvement of function by one degree of HB classification was observed in 4 (12.5%) patients on average 5 months after surgery., Conclusion: An adequate personalized choice of surgical treatment methods allowed mainly to preserve the function of hearing and facial nerve. In cases of deterioration of facial nerve function in the postoperative period, gradual improvement was observed for 3-10.5 months with further positive dynamics.

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.

Objectives: To analyze the clinical characteristics of congenital cholesteatoma (CC), to explore the risk factors related to recurrence of the disease, and to clarify the importance of staging for treatment., Methods: A total of 87 patients were followed up for more than 5 years, who had undergone surgical procedures for CC from September 2010 to January 2017 in Beijing Children's Hospital, Capital Medical University. Patients with CC were identified in accordance with the following Levenson's criteria. The clinical characteristics of CC on different stage and risk factors related to recurrence of the disease were analyzed., Results: Canal wall up mastoidectomy and tympanoplasty (n = 45), transcanal endoscopic approach (n = 29), and canal wall down mastoidectomy and tympanoplasty (n = 13) was, respectively, performed depending on cholesteatoma extension. Between 2010 and 2013, 20.93% of patients had stage I-II disease, whereas 61.26% had stage I-II disease from 2014 to 2017. Meanwhile, the proportion with stage III-IV disease decreased from 79.07% to 38.64% between these 2 time periods. The preoperative air conduction threshold in patients with stage I, II, III, and IV was, respectively, 23.36 ± 8.20, 45.40 ± 12.82, 47.49 ± 12.03, and 50.37 ± 11.80 dB. The stage of disease was a significant risk factor regarding recurrence ( P = .02). Surgery on patients with stage III-IV disease was performed with the aid of a microscope from 2010 to 2013 and with a microscope and endoscope from 2014 to 2017, which reduced the recurrence rate from 26.92% in the former period to 8.33% in the latter period., Conclusion: Early detection of CC is crucial regarding the facilitation of minimally invasive surgery and reducing complication and recurrence rates. The stage of the disease is a significant risk factor regarding recurrence. The surgery shows us the possibility of reducing the recurrence rate of CC, which is performed under a microscope and an endoscope.

Background: To compare cholesteatoma care internationally and to evaluate outcomes, ear surgeons must use the same terminology. However, a clear universal definition on how to describe the extension, destruction and accompanying morbidity caused by the cholesteatoma is lacking. The practical applicability by means of interrater agreement is assessed for the STAMCO and the ChOLE classification., Methods: A total of 134 adult patients derived from the nationwide multicentre study in the Netherlands, entitled Dutch Cholesteatoma Data (DCD) were included. Retrospective analysis of 134 surgical reports according to the STAMCO and ChOLE classification for localisation/extension of the cholesteatoma, complication status and ossicular chain status. Both the percentage agreement and the interrater agreement were determined for each item of the classifications and interrater agreement was compared between the classifications as a whole., Results: Differences in interrater agreement were found for both the localisation/extension of the cholesteatoma and ossicular chain status. STAMCO classification derived from the surgical report scored better on the localisation/extension of the cholesteatoma, whereas the ChOLE classification derived from the surgical report scored better on the status of the ossicular chain. In both classifications, complication status had a low agreement level but was also poorly registered in the surgical reports., Conclusion: Both STAMCO and ChOLE will be beneficial in uniform registration of cholesteatoma pathology in practice. Modifications proposed for both classifications may make them even more practical applicable in the future. A common denominator obtained from these two classifications may be incorporated in a standardised surgical report to facilitate evaluation which make outcomes transferable towards both classifications., (© 2020. The Author(s).)

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., (© 2020 The American Laryngological, Rhinological and Otological Society, Inc.)

The report presents modern views on the origin and pathomorphological characteristics of the cholesterol granuloma of the petrous apex of temporal bone, its clinical comparison with congenital cholesteatoma of the same localization. The differential diagnostic features in CT and MRI scans are analyzed in detail, and the principles of surgical treatment, indications and contraindications are discussed. Also the case of atypical course of cholesterol granuloma is presented, which was difficult in the diagnostics but with a successful treatment result.

Introduction: chronic otitis media with cholesteatoma (COMC) is a serious condition that may cause severe complications. According to the literature, in pediatric patients this entity can have a more aggressive behavior, leading to higher rates of morbidity. Consensus regarding the definition of aggressive and extensive cholesteatoma was lacking until recently. European Academy of Otology and Neurotology/Japan Otologic Society (EAONO/JOS) proposed a classification and staging system which was highly accepted by a group of otology experts., Objective: to compare cholesteatoma characteristics between pediatric and adult patients using the EAONO/JOS stating system., Material and Methods: a retrospective analysis of the patients who underwent surgery for cholesteatoma treatment in a tertiary hospital was performed using EAONO/JOS classification. Congenital cholesteatoma, revision surgery, and follow-up losses were excluded. The patients were further divided into two cohorts: a pediatric group (<16 years old) and an adult group (>16 years old). The results were compared between them., Results: 134 patients fulfilled the inclusion criteria, 27 were pediatric, and 107 adult patients. We found a significant difference in supratubal recess, with higher rates of extension to this location in the pediatric group. Also in the pediatric cohort, COMC was accompanied by malleus erosion in a higher frequency, with a significant difference from adults; it also presented in higher stages of the disease according to EAONO/JOS classification. We found no differences in hearing gain post-operatively. Moreover, we found that age younger than 16 years was an independent risk factor for relapse with time., Discussion and Conclusions: according to our data, cholesteatoma in pediatric age has a more aggressive and extensive nature than in adults, and age younger than 16-years-old is an independent risk factor for relapse., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Middle ear cholesteatoma is a destructive disease in which inflammation plays an important role in development and progression, and there are currently no biomarkers predicting prognosis or recurrence. Cylindromatosis (CYLD), a tumor suppressor deubiquitinase, serves as a negative regulator of inflammation expressed in tissues including the middle ear. To determine the clinical significance of CYLD in acquired cholesteatoma, we evaluated CYLD expression in acquired cholesteatoma tissue by immunostaining and analyzed its correlation with clinicopathological characteristics. Our immunohistochemical analysis revealed that CYLD expression levels were varied in the tissues of acquired cholesteatoma patients. The relative expression levels of CYLD in cholesteatoma exhibited a significant correlation with the grade of otorrhea (R = 0.532, p = 0.039). Moreover, the period of epithelialization was also significantly associated with the relative expression levels of CYLD (R = 0.720, p = 0.002). In addition, CYLD expression tended to be lower in the group with recurrence. These results suggest that low CYLD expression correlates with postoperative recovery of acquired cholesteatoma, while potentially affecting the induction of recurrence. This is the first report showing that low CYLD expression correlates with accelerated disease recovery, and suggests a new aspect of CYLD as a prognostic predictor of acquired cholesteatoma., Competing Interests: The authors have declared that no competing interests exist.

A 17-year-old female patient who came to the Otorhinolaryngology and Head and Neck Surgery department with chief complaints of bilateral ear discharge and hearing loss Since 1 year. She was diagnosed as a case of Turner syndrome based on Cardic-CT, USG-pelvis, CT-MRI Pelvis with Abdomen Screening, and Chromosomal analysis revealed 45, X pattern in all the metaphases. In the Preoperative investigations, Pure tone audiometry shows left ear (83dB HL) severe mixed hearing loss and right ear has (63dB HL) with moderately severe mixed hearing loss and in HRCT-Temporal bone features suggesting the most possibility of bilateral Cholesteatoma. Pre operatively Left ear soft tissue Histopathological examination was showed Cholesteatoma with active inflammation. Tympanomastoidectomy was planned for left ear and performed the procedure was Canal wall down mastoidectomy with type 3 tympanoplasty. Intraoperatively findings were thick KORNER'S septum, full of pale non bleeding granulations in antrum, aditus and around ossicles were observed in the middle ear. After surgery, Postoperative histopathology report showed necrotizing granulomatous inflammation consistent with tuberculosis (special stain for AFB is positive). In conclusion primarily this is a very rare case of Tuberculous Otitis Media. In such type of cases there are more chances of misdiagnosing by many clinicians as Tuberculosis was rarely occur in the ear and most of the findings are also absent as seen in our case. It is difficult to identify and diagnose the case at initial stages of a disease. In such type of cases outcome of patients is poor due to delayed diagnosis., Competing Interests: Conflicts of interest The authors have none to declare., (Copyright © 2020 Tuberculosis Association of India. Published by Elsevier B.V. All rights reserved.)

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

Objective: The clinical symptoms, imaging features and surgical treatment of congenital cholesteatoma are reported. Method: The clinical data of 20 patients with congenital cholesteatoma diagnosed and treated in our hospital from January 2016 to May 2018 were retrospectively analyzed, including the age of onset, clinical symptoms, signs, audiology, HRCT of temporal bone, surgical methods and so on. Result: In 20 patients with congenital cholesteatoma, the clinical manifestations were hearing loss in the affected ears, including 5 cases with ear fullness and 4 cases with tinnitus. There were 13 cases of local yellow-white shadow of tympanic membrane, 5 cases of normal tympanic membrane and 2 cases of tympanic membrane bombe. Pure tone audiometry showed conductive deafness in 14 cases and mixed deafness in 6 cases. CT scan of temporal bone showed that 15 cases had pneumatic type of mastoid, 4 cases had diploectic type and 1 case had sclerostic type. There were 15 cases of interspersed flocculent shadow and 5 cases of mass shadow. Among the 20 cases of chronic otitis media with cholesteatoma, 6 have cholesteatoma in epitympanum, 4 in tympanic cavity, 3 in tympanic cavity and epitympanum, 2 in mastoid and tympanic antrum, 1 in mastoid, tympanic antrum and tympanic cavity, 1 in tympanic antrum, tympanic cavity and petrous apex, 1 in tympanic cavity, hypotympanum, posterior tympanum and ostium tympanicum tubae auditivae, 1 in aditus ad antrum, epitympanum and posterior tympanum, 1 in tympanic cavity, epitympanum and posterior tympanum. Intact canal wall mastoidectomy and tympanoplasty were performed in 7 cases, open mastoidectomy and tympanoplasty in 5 cases, middle ear exploration and tympanoplasty in 4 cases, atticotomy with reconstruction and tympanoplasty in 3 cases and subtotal temporal bone resection in 1 case. Conclusion: For patients with intact tympanic membrane presenting with hearing loss, the tympanic membranes should be carefully examined, and thin-section CT and MRI of temporal bone should be performed in time to avoid missed diagnosis of congenital cholesteatoma. Once diagnosed, surgery should be performed as soon as possible., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Aim: To explore the value of the applying the apparent diffusion coefficient (ADC) to the quantitative diagnosis of temporal bone cholesteatoma., Materials and Methods: Seventy-one patients clinically suspected of temporal bone cholesteatoma were enrolled prospectively. These patients underwent ear magnetic resonance imaging (MRI) and turbo spin-echo diffusion-weighted imaging (TSE-DWI) using a Philips Ingenia 3 T superconductive MRI system, and their ADCs were measured. Subsequently, all enrolled patients underwent surgery within 15 days of the MRI. Using receiver operating characteristic (ROC) curve analysis, the optimal threshold and diagnostic performance for diagnosing temporal bone cholesteatoma were determined. Logistic regression modelling was used to combine ADCs and T1-weighted imaging (T1WI) sequences and calculate the combined diagnostic performance., Results: Based on the pathology results, patients were categorised into the cholesteatoma group (CS group, n=43) and the non-cholesteatoma group (NCS group, n=28). In the CS group, ADCs were significantly lower (mean, 0.87±0.31×10 -3 mm 2 /s) than in the NCS group (mean, 1.87±0.49×10 -3 mm 2 /s; p<0.001); the area under the ROC curve (AUC) was 0.915. Based on the optimal threshold of ADC, ≤1.23×10 -3 mm 2 /s, the diagnostic performance was high; the sensitivity and specificity for diagnosing cholesteatoma were 95.35% and 85.71%, respectively. By combining the ADC and T1WI sequence, AUC increased to 0.953, and the sensitivity and specificity were 90.7% and 96.43%, respectively., Conclusion: ADC quantitative analysis has a high value in the preoperative diagnosis of cholesteatoma, and the combination of ADC and T1WI sequences can improve the diagnostic accuracy and specificity and thereby facilitate clinically effective diagnosis., (Copyright © 2019 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

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.

Objectives: To investigate the potential use of Ki-67 and pronuclear cell antigen (PCNA) as indicators of recurrent cholesteatoma., Material and Methods: Patients who had been diagnosed with cholesteatoma and who had undergone canal wall-down mastoidectomy were included in this study. Subjects were divided into two groups: recurrent and non-recurrent (i.e., cases without recurrence for at least 2 years). Ossicular pathologies were recorded. Histopathologic specimens were stained for Ki-67 and PCNA and the percentages of stained cells were calculated., Results: Neither group demonstrated a significant difference in terms of total Ki-67 per cell, Ki-67-stained cell counts, Ki-67-staining percentages, total PCNA per cell, PCNA-stained cell counts, or PCNA-staining percentages (p>0.05). No significant relationship was noted between the staining percentages for either Ki-67 or PCNA and the incudostapedial involvement (p>0.05); however, a significant relationship was noted between Ki-67 staining and malleus involvement (p<0.05)., Conclusion: Although the recurrent and non-recurrent cholesteatoma groups showed no significant differences in terms of the percentages of stained cells for either Ki-67 or PCNA, we detected high Ki-67 staining in the malleus involvement group. We concluded that cell-proliferation markers could not be defined as indicators of recurrence of cholesteatoma, but they could be defined as indicators of destructive patterns of this disease.

Objective:: To describe an observed case of spontaneous regression of cholesteatoma in a patient on chronic anti-tumor necrosis factor-alpha (TNF-a) therapy and inspire further research into the role of TNF-a in cholesteatoma., Methods:: Clinical assessment of disease in a single-patient case report., Results:: A 49-year-old woman suffered a severe case of Stevens-Johnson syndrome when she was 12 years old, leaving her with bilateral corneal opacification and tympanic membrane perforations with extensive cholesteatoma. For her corneal opacification, a corneal prosthesis was placed, which was complicated by a foreign body reaction necessitating long-term therapy with infliximab, a monoclonal antibody against TNF-a that is therapeutic in some chronic inflammatory diseases. She was otherwise healthy and took no other medications. While on infliximab, the patient had spontaneous and complete resolution of her cholesteatoma without any surgical intervention., Conclusions:: This surprising case suggests that there may be a prominent role of TNF-a in cholesteatoma pathophysiology and that TNF-a may be an effective target for nonsurgical therapy.

Objective: Large-scale otoscopic and audiometric assessment of populations is difficult due to logistic impracticalities, particularly in low- and middle-income countries (LMIC). We report a novel assessment methodology based on training local field workers, advances in audiometric testing equipment and cloud-based technology., Methods: Prospective observational study in Bohol, Philippines. A U.S. otolaryngologist/audiologist team trained 5 local nurses on all procedures in a didactic and hands-on process. An operating otoscope (Welch-Allyn R ) was used to clear cerumen and view the tympanic membrane, images of which were recorded using a video otoscope (JedMed R ). Subjects underwent tympanometry and distortion product otoacoustic emission (DPOAE) (Path Sentiero R ), and underwent screening audiometry using noise cancelling headphones and a handheld Android device (HearScreen R ). Sound-booth audiometry was reserved for failed subjects. Data were uploaded to a REDCap database. Teenage children previously enrolled in a 2000-2004 Phase 3 pneumococcal conjugate vaccine trial, were the subjects of the trainees., Results: During 4 days of training, 47 Filipino children (M/F = 28/19; mean/median age = 14.6/14.6 years) were the subjects of the trainee nurses. After the training, all nurses could perform all procedures independently. Otoscopic findings by ears included: normal (N = 77), otitis media with effusion (N = 2), myringosclerosis (N = 5), healed perforation (N = 6), perforation (N = 2) and retraction pocket/cholesteatoma (N = 2). Abnormal audiometric findings included: tympanogram (N = 4), DPOAE (N = 4) and screening audiometry (N = 0)., Conclusion: Training of local nurses has been shown to be robust and this methodology overcomes challenges of distant large-scale population otologic/audiometric assessment., (Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.)

Objective: To describe a case of middle ear lipoma, review the current literature, and discuss the surgical approach., Methods: Published case reports in the English literature of lipomas restricted to the middle ear were reviewed. The presentation, location, and management of the middle ear lipomas were analyzed., Results: Histological examination of the resected middle ear lesion was compatible with lipoma. Review of the literature suggests middle ear lipomas are rare and involve the epitympanum., Conclusion: Lipomas should be included in the differential diagnosis for middle ear lesions. Adequate surgical exposure can be achieved through a transcanal approach, with particular attention to carefully elevate the tympanic membrane off the malleus, preserving the integrity of the ossicular chain., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: An aural cholesteatoma, more appropriately named tympanokeratoma, is an epidermoid cyst of the middle ear described in several species, including dogs, humans and Mongolian gerbils. The cyst lining consists of stratified, keratinizing squamous epithelium with central accumulation of a keratin debris. This case report describes vestibular ganglioneuritis and perineuritis in a dog with chronic otitis, bilateral tympanokeratomas and presumed extension of otic infection to the central nervous system., Case Presentation: An 11-year-old intact male Dalmatian dog with chronic bilateral otitis externa and sudden development of symptoms of vestibular disease was examined. Due to the dog's old age the owner opted for euthanasia without any further examination or treatment and the dog was submitted for necropsy. Transection of the ears revealed grey soft material in the external ear canals and pearly white, dry material consistent with keratin in the tympanic bullae bilaterally. The brain and meninges were grossly unremarkable. Microscopical findings included bilateral otitis externa and media, unilateral otitis interna, ganglioneuritis and perineuritis of the spiral ganglion of the vestibulocochlear nerve and multifocal to coalescing, purulent meningitis. A keratinizing squamous epithelial layer continuous with the external acoustic meatus lined the middle ear compartments, consistent with bilateral tympanokeratomas. Focal bony erosion of the petrous portion of the temporal bone and squamous epithelium and Gram-positive bacterial cocci were evident in the left cochlea. The findings suggest that meningitis developed secondary to erosion of the temporal bone and ganglioneuritis and/or perineuritis of the vestibulocochlear nerve., Conclusions: Middle ear tympanokeratoma is an important and potentially life-threatening otic condition in the dog. Once a tympanokeratoma has developed expansion of the cyst can lead to erosion of bone and extension of otic infection to the inner ear, vestibulocochlear ganglion and nerve potentially leading to bacterial infection of the central nervous system.

Background: Congenital cholesteatomas (CC) arise from epithelial remnants around the petrous bone. They enlarge gradually causing progressive destruction and functional damage to the ear and surrounding structures. Because of their insidious course, most patients are misdiagnosed and present late with complications., Methods: This is a retrospective study of 41 cases diagnosed as CC and followed up for 2 years. All patients underwent full audiological evaluation and a radiological study (CT, MRI)., Results: The male:female ratio was 2.3: 1, and the mean age was 30.89 years. The mean delay before presentation was 13 months. The commonest presentations in order of frequency were: discharge (60.9%), hearing loss (51.2%), facial paralysis (31.7%) and intracranial complications (31.7%). Forty patients were treated surgically by a variety of approaches depending on the extensions and functional status. The facial nerve was decompressed in 9 cases and cable grafted in 4. Two cases underwent VII-XII anastomosis later on., Conclusion: CC present with a myriad of clinical manifestations and should be suspected in all patients with unexplained otological signs and symptoms. They must be completely excised and the functional status of the ear rehabilitated either immediately or later. Follow-up must be indefinite as they have a high recurrence rate., (© 2018 S. Karger AG, Basel.)

Tympanic paragangliomas are common benign tumors of the middle ear, made up of neuroendocrine cells dispersed along the major vascular axes of the head, the neck and the vertebral column. The revealing symptoms are hearing loss and a pulsatile tinnitus. Otoscopy often shows pulsatile retrotympanic reddish mass. MRI and somatostatin analogue scintigraphy (OctreoScan) are the imaging modality of choice. OctreoScan allows physicians to detect other tumor locations. The dosage of urinary methoxyl derivatives is determined, complemented by the screening of other components of multiple endocrine neoplasias (MEN). Biopsy is contraindicated because of the risk of hemorrhage. Surgery is the treatment of choice. We report the case of F. A., a 75-year old patient, admitted with tympanic paraganglioma revealed by hearing loss in his right ear (occurred 4 years before) associated with pulsatile tinnitus concomitant with the heart beating in his ear. He underwnt otoscopy showing retrotympanic reddish pulsatile mass in his right ear (A). (B) MRI of the brain showed total involvement of the middle ear and of the mastoid cells, without identifiable tumoral process, with distinct hyperintensity on diffusion-weighted imaging and strong contrast-enhancement. MEN assessment was without abnormalities, including 24h methoxyl derivatives, parathyroid hormone and calcitonin. The diagnosis of paraganglioma was retained because of the strong contrast-enhancement. OctreoScan was indicated before surgery.

Ear, nose and throat (ENT) presentations to primary care are common and frequently affect military patients. Many patients can be managed in primary care with appropriate treatment, but some presentations require appropriate, timely, and occasionally emergency onward referral for hospital management. This paper discusses the management of common otological presentations including otitis externa (OE), acute otitis media, chronic suppurative otitis media (including cholesteatoma), tympanic membrane (TM) perforations and pinna haematoma.

Cholesteatoma is a destructive and expanding growth benign tumor, mainly consisting of stratified squamous epithelium. It possesses the capacity of eroding bone and can exist in a nonaggressive state, remaining undetected for years. Once cholesteatoma appeared, it grows relentlessly and threats to invade intra-temporal structures. Most cholesteatomas occurred in the petrous bone and also affected the labyrinth and middle cranial fossa. Here, we report a preliminary wrongly-diagnosed case. The patient was wrongly diagnosed as "sebaceous cyst with infection" by local hospital. The definite diagnosis was made after the surgery performed by our department, which was confirmed by pathological result. So far, there were few reports of giant cholesteatoma which occurs in fronto-parietal part of skull with bone defect.

The congenital cholesteatoma is a rare benign tumor whose diagnosis is mainly made during childhood. Otoscopic examination shows a white retrotympanic collection with or without hearing loss. In case of a late diagnosis, the disease will spread to the surrounding structures, leading to ossicules destruction, facial palsy, sensorineural hearing loss and vestibular dysfunction. Surgery is the therapeutic gold-standard, with different techniques chosen based on the cholesteatoma's extension. Patient should benefit from a long term medical and radiological (MRI) follow-up., Competing Interests: Les auteurs déclarent n’avoir aucun conflit d’intérêts en relation avec cet article.

Objectives: Cholesteatoma is a chronic inflammatory clinical disorder, and it may cause hearing loss and various complications due to its destructive nature. Mean platelet volume (MPV) is a prothrombotic and proinflammatory marker. The aim of this study is to investigate predictive values of MPV, neutrophil-to-lymphocyte ratio, erythrocyte distribution width, and platelet lymphocyte ratio in cholesteatomatous or noncholesteatomatous pediatric chronic otitis media., Methods: In this study, the authors retrospectively analyzed 24 patients with cholesteatomatous chronic otitis media, 9 patients with noncholesteatomatous chronic otitis media, and 36 age- and sex-matched healthy controls. Cholesteatomatous and noncholesteatomatous chronic otitis media groups were compared with each other, and with controls for WBC, MPV, erythrocyte distribution width, neutrophil-to-lymphocyte ratio, and platelet lymphocyte ratio., Results: Mean platelet volume was significantly lower in patients with cholesteatoma (8.17 ± 1.22 fL) compared with the control group (10.42 ± 0.87 fL) (P <0.001). Region of conversion curve analysis showed that the cutoff level of 9.5 fL for MPV was an independent predictor for cholesteatoma with a high sensitivity (86%) and specificity (84%). Cholesteatomatous and noncholesteatomous chronic otitis media patients were similar for other parameters studied., Conclusion: Mean platelet volume may be used as a fast-to-obtain, reliable, and inexpensive marker with a high predictive level to indicate cholesteatoma in children.

External auditory canal cholesteatoma is defined as an accumulation of keratin leading to osteolytic bony erosion. It is a rare or underdiagnosed otologic entity. Our study aims to illustrate one case of external auditory canal cholesteatoma. Hypertensive diabetic patient, 65 years of age, under therapy suffering from severe right earache, insomnia, hearing loss, small purulent otorrhea and right facial paralysis grade V since 3 months. Initially the diagnosis was malignant external otitis. The patient underwent antibiotic therapy without improvement. Otologic examination showed burgeoning ulcerative lesion of the posterior wall of the right external auditory meatus. Systematic lesion biopsy was performed and confirmed the diagnosis of cholesteatoma. The patient underwent open technique tympanoplasty. External auditory canal cholesteatoma is rare, clinical symptoms are not specific, coronal CT scan makes a major contribution to a positive diagnosis showing bony crater of external auditory meatus. Treatment depends on the extent of the lesions. It can be based on simple local cares or on open technique tympanoplasty. External auditory canal cholesteatoma may have multiple clinical aspects and lend to confusion with other pathologies of the external auditory meatus., Competing Interests: Conflits d’intérêts Les auteurs ne déclarent aucun conflit d'intérêts.

One case of Eosinophilic granuloma of the temporal bone was reported which had been misdiagnosed as cholesteatoma.This disease is a rare benign tumour that is lack of specific feature in clinical performances and easy to be misdiagnosed., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Congenital cholesteatoma is one of the more common causes of the onset of childhood conductive hearing loss unrelated to middle ear effusion. If undiagnosed, the disease can progress to irreversibly destroy the conductive hearing architecture, as well as the surrounding skull base of the lateral temporal bone. When diagnosed early, the growth can be removed and the conductive hearing mechanism preserved in the vast majority of patients. Because most children are asymptomatic, the burden falls on primary care providers to perform pneumatic otoscopy and visualize all quadrants of the tympanic membrane even in young children who frequently resist attempts to conduct a thorough examination to rule out suspicious lesions. [Pediatr Ann. 2016;45(5):e167-e170.]., (Copyright 2016, SLACK Incorporated.)

Clinical presatation: right ear tinnitus, hearing loss, headache for 1 year with intermittent vertigo for 9 months. Specialized examination: right ear tympanum hyperemia and edema, right ear severe conduction deafness, left ear mild hearing loss. The average threshold: right ear 65 dB, the left ear 35 dB. Imaging examination: CT showed the soft tissue density mass in the right ear tympanum, part of the ear bone obscurity, superior wall of the tympanic cavity attenuation, tympanic sinus entry is not expanding. Imaging diagnosis: chronic otitis media with cholesteatoma formation. Postoperative diagnosis: meningioma., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Clinically significant sellar cysts unrelated to pituitary adenomas are uncommon. Intracranial cholesteatomas are also rare and are most common in the middle ear and mastoid region. We report an even rarer case of cholesteatoma in the sellar region-a challenging diagnosis guided by clinical presentations, radiological signs, and biopsy, aiming at emphasize the importance of considering cholesteatoma when making differential diagnoses of sellar lesions.We present a case of cholesteatoma in the sellar region in a 56-year-old man with hypopituitarism, diabetes insipidus, and cystic imaging findings. It was difficult to make an accurate diagnosis before surgery. We present detailed analysis of the patient's disease course and review pertinent literature.The patient underwent a surgical exploration and tumor resection through a transsphenoidal approach. Pathologic results revealed a cholesteatoma. The patient's symptoms improved a lot after surgery, and the postoperative period was uneventful. Taken together, the lesion's imaging appearance, pathological characteristics, and clinical features were all unique features that lead to a diagnosis of cholesteatoma.As we did not see such reports by Pubmed and EMBASE, we believe this is the first reported case of sellar cholesteatoma presenting in this manner. This article emphasized that cholesteatomas, although rare, should be considered part of the differential diagnosis of sellar lesions., Competing Interests: The authors have no conflicts of interest to disclose.