Diagnostic and Imaging Findings in Inflammatory Opacifications of the Middle Ear: A Review of the Literature.

Opacification in the middle ear and mastoid region can stem from a wide range of factors. In terms of diagnostic imaging, CT is the primary tool due to its exceptional spatial resolution, particularly for examining the temporal bone and ossicles. MRI complements this by offering detailed soft tissue lesion characterization and assessing involvement in the inner ear and cranial nerves. This study focuses on inflammatory causes of opacification in the middle ear and mastoid, with an emphasis on the utility of CT and MRI. This comprehensive review aimed to provide a practical framework for considering potential differential diagnoses.

[Specialist consensus on endoscopic surgery of the middle ear mastoid].

This article discusses otoscopic middle ear mastoid surgery from multiple perspectives. Firstly, it discusses the indications and contraindications for surgery from the nature of the lesion and the imaging manifestations; secondly, it recommends the applicable equipment and describes the surgical approach in detail; finally, it summarizes the principles of the management of the operative cavity of the mastoid process in the middle ear from the perspectives of function and reconstruction. The purpose of this article is to illustrate otoscopic middle ear mastoid surgery with the aim of providing reference or guidance for performing related surgeries.

Congenital Retrosigmoid Cholesteatoma: Case Series and Literature Review.

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.

The Mastoid Skin Graft: A Fast, Efficient Donor Site for Auricle Reconstruction After Mohs Surgery.

BACKGROUND: Mohs micrographic surgery has been considered the gold standard for treating nonmelanoma skin cancers. Approximately 8% to 10% of skin cancers requiring Mohs surgery occur on the auricle. Skin grafts can be used to achieve optimal functional and cosmetic results. OBJECTIVES: To describe a skin graft technique using the mastoid area as a donor site for auricular reconstruction following Mohs micrographic surgery, to achieve optimal esthetic results in functionally important areas. METHODS: This retrospective study assessed the outcomes of patients who underwent MMS in an out-patient Mohs surgery clinic from 2010 to 2021. All patients had MMS of the auricle with reconstruction using a split thickness skin graft harvested from the mastoid area. RESULTS: A total of 154 patients were included. The average lesion diameter was 13.7 mm (range 5-30 mm), excised in an average of 1.9 rounds of MMS. Skin graft success rate was 96%. Complications included one patient with a hypertrophic scar. The cosmetic appearance of the recipient site was rated as excellent by 87.5% of patients. All patients rated the cosmetic appearance of the donor area as excellent and well-concealed. CONCLUSIONS: Split thickness skin grafts harvested from the mastoid area are a quick and efficient reconstruction method for post-Mohs auricular defects.

Temporal bone CT-based anatomical parameters associated with the development of cholesteatoma.

BACKGROUND: Cholesteatoma is caused by disorders of the middle ear ventilation that trigger a progressive series of events responsible for its formation. The aim of this study was to identify possible radiological CT-derived parameters predisposing to ventilation disorders and cholesteatoma. METHODS: In this retrospective study, patients diagnosed with cholesteatomatous chronic otitis media who underwent temporal bone CT and open tympanoplasty surgery have been included, as well as control patients with clinical examination negative for organic otological pathology who underwent temporal bone CT for other reasons. For each patient, the following parameters have been extracted from CT volumes: degree of mastoid pneumatization, prominence of the cog, patency of the Eustachian tube, antrum width, aditus width, anterior and posterior epitympanic widths, and epitympanic height. RESULTS: Sixty patients have been included, thirty of whom belonged to the group of patients with cholesteatoma and the remaining part to the group of patients without organic otological pathology. The prevalence of a low degree of mastoid pneumatization was significantly higher among patients with cholesteatoma, as well as for the prevalence of cog prominence (p < 0.001). All the continuous variables were found to have statistical significance (p < 0.05) in the comparison between groups except for the width of the antrum. CONCLUSION: Mastoid pneumatization degree, prominence of the cog and epitympanic measures based on temporal bone CT could be good radiological correlates of the ventilatory capabilities of the epitympanum which, if compromised, can facilitate the development of cholesteatoma.

Management of a Complex Case of a Recurrent Paraganglioma.

Paragangliomas are rare, slow-growing, hypervascular, catecholamine-secreting neuroendocrine tumors arising from the paraganglia. Paragangliomas are rarely found in the head and neck and are typically benign, presenting as a painless, slow-growing mass. Surgical extirpation in combination with long-term surveillance has been long regarded as the standard of care; however, the advances in imaging, radiation therapy, and embolization techniques have improved diagnostic and therapeutic modalities. We present a case of an 87-year-old female who had previously undergone resection of a paraganglioma in 1998, with no evidence of disease in 2002. Eighteen years later, the patient presented to the clinic with otogenic complaints. Imaging showed an expansive mass from the jugular foramen with bone destruction and opacification within the ear canal. The patient opted for observation. The patient eventually presented to the emergency room with neurologic manifestations. Imaging showed a cerebellar abscess prompting emergency drainage. Intraoperative cultures grew Prevotella loescheii and S. intermedius, and the patient was started on 6 weeks of IV antibiotic therapy. Debulking of the paraganglioma was performed followed several months by mastoid and ear canal obliteration; however, the patient experienced complications, including dehiscence of the external auditory canal and Clostridium difficile infection. The patient was eventually treated successfully, marked by a reduction in complaints, a return to baseline activities, and imaging showing no increase in tumor size.

Imaging guidance for cholesteatoma surgery using tissue autofluorescence.

Significance: Cholesteatoma is an expansile destructive lesion of the middle ear and mastoid, which can result in significant complications by eroding adjacent bony structures. Currently, there is an inability to accurately distinguish cholesteatoma tissue margins from middle ear mucosa tissue, causing a high recidivism rate. Accurately differentiating cholesteatoma and mucosa will enable a more complete removal of the tissue. Aim: Develop an imaging system to enhance the visibility of cholesteatoma tissue and margins during surgery. Approach: Cholesteatoma and mucosa tissue samples were excised from the inner ear of patients and illuminated with 405, 450, and 520 nm narrowband lights. Measurements were made with a spectroradiometer equipped with a series of different longpass filters. Images were obtained using a red-green-blue (RGB) digital camera equipped with a long pass filter to block reflected light. Results: Cholesteatoma tissue fluoresced under 405 and 450 nm illumination. Middle ear mucosa tissue did not fluoresce under the same illumination and measurement conditions. All measurements were negligible under 520 nm illumination conditions. All spectroradiometric measurements of cholesteatoma tissue fluorescence can be predicted by a linear combination of emissions from keratin and flavin adenine dinucleotide. We built a prototype of a fluorescence imaging system using a 495 nm longpass filter in combination with an RGB camera. The system was used to capture calibrated digital camera images of cholesteatoma and mucosa tissue samples. The results confirm that cholesteatoma emits light when it is illuminated with 405 and 450 nm, whereas mucosa tissue does not. Conclusions: We prototyped an imaging system that is capable of measuring cholesteatoma tissue autofluorescence.

Cholesteatoma Masquerading as Recurrent Langerhans Cell Histiocytosis.

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.

Desmoid tumour of the external ear and mastoid following middle-ear surgery.

BACKGROUND: Desmoid tumours (aggressive fibromatosis) are rare, locally invasive, benign tumours. The following case represented a diagnostic challenge, because of the uncommon nature of the lesion. CASE REPORT: A 26-year-old woman, who had previously undergone middle-ear surgery for cholesteatoma, presented with a painful swelling involving the post-auricular area and the conchal bowl. Initially, it was believed to be an infective process related to the surgery or an unusual cholesteatoma recurrence. Following investigations, which involved imaging and histology, the swelling was diagnosed as a desmoid tumour, and the patient received chemotherapy. CONCLUSION: Two incidences of paediatric desmoid tumours affecting the ear have been described in the literature, but there is no previous report of a desmoid tumour related to ear surgery. Desmoid tumours have, however, been reported following trauma, including surgery.

Risk factors for posttreatment mastoid cellulitis in patients with oral malignancy.

OBJECTIVES: To investigate the possible risk factors for mastoid cellulitis after treatment in patients with oral cancer. METHODS: Patients with oral cancer who underwent preoperative and postoperative contrast-enhanced computed tomography (CECT) between January 2017 and March 2020 were retrospectively evaluated. Using an image reporting system, cases with reports containing the keyword "mastoid cellulitis" were registered in the mastoid cellulitis group. Fifty patients with oral cancer who did not develop mastoid cellulitis were randomly selected as the control group. Information concerning age, sex, location, and histopathological type of the primary tumor, presence or absence of cervical lymph node metastasis, and treatment methods were obtained from the hospital's electronic medical records to investigate the association between these factors and the occurrence of mastoid cellulitis. RESULTS: Statistically significant differences were observed in the following factors: age (p = 0.004), primary tumor site (p < 0.001), histopathological type (p = 0.032), radiotherapy (p < 0.001), and chemotherapy (p < 0.001). Logistic regression analysis revealed that maxillary tumors (p < 0.001), histopathological type (p = 0.047), and radiotherapy (p = 0.0301) were significantly associated with the occurrence of mastoid cellulitis. CONCLUSIONS: In oral cancer, the risk of postoperative mastoid cellulitis was higher in patients with a maxillary tumor, histopathology other than squamous cell carcinoma, and history of radiotherapy.

A unique presentation of IgG4 disease with ocular, neurologic and mastoid involvement.

A man in his 20s presented with headache and acute deterioration in visual acuity. He was found to have panuveitis and raised intracranial pressure with papilloedema. MRI and F-fluorodeoxyglucose positron emission tomography confirmed a subclinical, but active, inflammatory mastoid process. Histology of the mastoid showed immunoglobulin G4 (IgG4) cells, plasma cells and storiform fibrosis.This presentation of IgG4 disease has not been previously described.Treatment with high-dose steroids was initiated, followed by long-term immunosuppressive therapy. The patient's symptoms improved, although he remains dependent on azathioprine and low dose oral steroids for symptom control. To date, there has been no progression of the disease.

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

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

Clinical characteristics of atelectatic eardrums and adhesive otitis media in children.

OBJECTIVES: Atelectatic eardrum and adhesive otitis media in children are related to persistent otitis media with effusion (OME) and eustachian tube dysfunction in infancy. However, the pathogenesis of these diseases is not fully understood, and some cases even progress to pars tensa cholesteatoma. This study analyzed the clinical characteristics of children with atelectatic eardrum and adhesive otitis media to clarify associated causes of and risk factors for progression of these middle ear pathologies in children. PATIENTS AND METHODS: This retroactive study included 38 ears of 31 children with atelectatic eardrums (atelectasis group), and 19 ears of 17 children with adhesive otitis media (adhesive OM group). Thirty-two contralateral non-cholesteatoma ears of 32 children with congenital cholesteatoma were also examined as a control group. Participants were aged 15 or younger. Life history (obtained via questionnaire), associated diseases, hearing acuity, aeration and development of the temporal bone were investigated. RESULTS: All children in the atelectasis and adhesive OM groups had a history of recurrent acute otitis media (rAOM) and/or OME. The prevalence of perennial allergic rhinitis was 18 cases (58%) in the atelectasis group and 16 cases (94%) in the adhesive OM group. The prevalence of perennial allergic rhinitis was significantly higher in the adhesive OM group compared to the control group (P < 0.01). The development of mastoid air cells, categorized by MC classification, showed MC0 + MC1 (poor pneumatization) in 19 ears (50.0%) with atelectatic eardrums and 12 ears (63.2%) with adhesive OM. Poor pneumatized mastoid was more frequently observed in the ears of the atelectasis and adhesive OM groups compared to the control group (P < 0.01). No significant difference was found in regards to aeration of the middle ear between the two groups; however, aeration was significantly poorer in both groups compared to the control group (P < 0.01). Three characteristics were analyzed using multivariate logistic regression: perennial allergic rhinitis (odds ratio [OR] 4.319, P = 0.013), poor mastoid pneumatization (OR 8.457, P = 0.012), and pars flaccida retraction pocket (OR 20.897, P = 0.006). These characteristics were shown to be significant risk factors for atelectatic eardrums and adhesive OM. In addition, the predisposition to perennial allergic rhinitis was shown to be the most important factor in the progression from atelectatic eardrum to adhesive otitis media (OR 16.615, P = 0.012). CONCLUSION: Children with perennial allergic rhinitis, poor development of mastoid air cells, poor aeration of the temporal bone, and with pars flaccida retraction pocket were at an increased risk of developing an atelectatic eardrum and adhesive OM. In particular, perennial allergic rhinitis was shown to be a significant risk factor in the progression from atelectatic eardrum to adhesive otitis media. Allergic inflammation may affect not only the nasal passages but also the eustachian tube, resulting in persistent middle ear dysfunction. Therefore, children with rAOM/OME who have these risk factors should be carefully monitored and treat over time in effort to prevent progression of pathology.

Posterior-Anterior Cephalometric Study of Neurofibromatosis Type 1 Patients With Facial Plexiform Neurofibroma: Analysis of Skeletal Symmetry Concerning Midfacial and Skull Base Reference Points (Zygomatic Arch, Mastoid, and Juga).

BACKGROUND/AIM: Neurofibromatosis type 1 (NF1) is an autosomal dominant tumor predisposition syndrome that is also characterized by skeletal abnormalities. In the cranial region, skeletal dysplasia is observed that is associated with a characteristic peripheral nerve sheath tumor, the plexiform neurofibroma (PNF). The aim of the study was to determine PNF-associated skeletal asymmetries of the mid-skull and skull base as an indicator of local tumor development. PATIENTS AND METHODS: The distances of the zygomatic arch, mastoid, and juga measurement points from the interorbital horizontal plane and median sagittal plane were examined on anterior-posterior cephalograms of 168 NF1 patients (females: 82, males: 86) and compared with the findings of a control group. A distinction was made as to whether the patients had developed a facial PNF. RESULTS: The distances of the measurement points from the reference planes differed between the patient and control group (p<0.05). Within the NF1 patients, differences between certain distances were noted when comparing patients with PNF and without PNF (p<0.05). In PNF patients, discrete changes in the skull contour were also detectable on the non-affected side (p<0.05). The caudal measurement point of the skull base showed no changes in the group comparison. CONCLUSION: The individually very variable facial PNF in NF1 is associated with deformations and misalignments of midfacial bones that follow a pattern quantifiable with cephalometric analysis.

Recurrent Primary Inverted Papilloma of the Mastoid with Intracranial Invasion: A 7-Year Follow-Up.

A 55-year-old man presented to the otolaryngology department complaining of aural fullness in his left ear after an episode of probable otitis 3 months before. magnetic resonance imaging revealed a soft tissue mass within the mastoid cavity that had destroyed the posterior wall of the middle ear with no apparent middle ear or sinonasal origin. The patient underwent a left canal wall-up tympanomastoidectomy, and the pathology report confirmed an inverted papilloma. Inverted papillomas are uncommon benign epithelial tumors related to a high recurrence rate and high risk of secondary malignant transformation after multiple surgeries. The patient has undergone 2 additional surgical interventions involving the neurosurgery team due to recurrent inverted papilloma that exerted a mass effect over the left cerebellar hemisphere. Despite no signs of recurrence on magnetic resonance imaging 5 years after the last surgery, at least 1 radiologic study per year is granted. Clinical multidisciplinary follow-up including nasal endoscopy and head and neck examination as a part of a stringent follow-up is essential to rule out synchronous nasosinusal inverted papillomas.

Experience with three-dimensional exoscope-assisted surgery of giant mastoid process osteoma.

BACKGROUND: The excision of a giant mastoid process osteoma can be challenging. In such situations, the three-dimensional exoscope intra-operative optic may be a promising tool. CASE REPORT: This paper provides a technical description of a giant mastoid osteoma excised under three-dimensional exoscope magnification. A fragmented excision by intralesional curettage was adopted to prevent peri-tumoural damage. This technique had been previously described for the excision of large osteomas of the sinus. CONCLUSION: The three-dimensional exoscope magnification tool had excellent applicability during surgery of a rare mastoid osteoma. In complex anatomical areas, the cavitation and fragmented excision of the tumour may prevent complications and is therefore recommended.

Mastoiditis Masquerade.

We report a case of primary temporal bone diffuse B-cell non-Hodgkin lymphoma, which is a rare entity. A 71-year-old male with a history of dementia and hemicraniectomy presented due to 1 month of a pronounced left ulcerative mastoid lesion. Strikingly, there were no cranial nerve deficits which was unexpected due to the degree of the lesion. Initially, infectious mastoiditis was suspected based on physical examination alone. Due to the patient being a poor historian, it was difficult to determine whether this was an acute or chronic issue. Temporal bone squamous cell carcinoma, infectious mastoiditis, and actinomycosis were on the differential, but biopsies revealed non-Hodgkin lymphoma.

Primary external auditory canal cholesteatoma of 301 ears: a single-center study.

PURPOSE: Limited literature exists on primary external auditory canal (EAC) cholesteatoma (EACC). Here, we focus on the clinical features of this rare disease, especially the invasive patterns of lesion progression, through a large population study and present simple and practical staging. METHODS: In all, 276 patients (male 99; female 177; mean age 41.3 ± 21 years; ears 301) with primary EACC were retrospectively analyzed. Stage I indicated EACC without bony lesions, stage II indicated invasion confined within EAC, stage III indicated invasion beyond the EAC involving mastoid air cells or tympanic cavity, but within the temporal bone, and stage IV indicated invasion beyond the temporal bone. RESULTS: In all, 41, 219, 40, and 1 ear with Stage I, II, III, and IV lesions were found, respectively. Common clinical symptoms were hearing loss (237 ears, 78.7%), otalgia (221 ears, 73.4%), and otorrhea (85 ears, 28.2%). The mean air conduction and air-bone gaps were 45.4 ± 17.9 dB HL and 24.6 ± 15 dB HL, respectively. EACCs were found to invade in all directions of the EAC, with the inferior wall (224 ears, 74.4%) > posterior wall (207 ears, 68.8%) > anterior wall (186 ears, 61.8%) > superior wall (86 ears, 28.6%) invasion; multiwall invasions (207 ears) were common; however, inward invasions into the tympanic cavity were rare. CONCLUSION: Primary EACCs occurred mostly in women and often unilaterally invaded multiple bony walls in the lower half of the EAC. The present staging reflects the patterns and severity of lesion progression and may be beneficial in treatment planning.

A 10-year review of malignant otitis externa: a new insight.

OBJECTIVES: This study aims to assess the clinical trends of malignant otitis externa (MOE) and classify MOE based on the findings related to high-resolution computed tomography (HRCT) of the temporal bone and 99-Tech3-Phase Bone Scintigraphy (TPBS). We also reconstruct a treatment algorithm for MOE in our institution. METHODOLOGY: A 10-year retrospective review was carried out on MOE in a single otology institution from January 2011 to December 2020. The MOE was classified based on proposed Tengku's radiological stratification according to HRCT and TBPS findings. Phase I is defined as inflammation limited to the soft tissue in the external auditory canal, without involvement of the bone. Phase II is the inflammation beyond the soft tissue, involving bone, but limited to the mastoid. Phase III is when the inflammation extends medially, involving the petrous temporal bone or temporomandibular joint, with or without parapharyngeal soft tissue involvement. Phase IV refers to inflammation extending medially to involve the nasopharynx, with or without abscess formation. Finally, Phase V is inflammation that further extends to the contralateral base of the skull. RESULTS: A sample of 49 patients was involved in this study. Majority of the patients were having Phase III (36.7%) of the disease, followed by Phase V (24.5%), Phase II (18.4%), Phase IV (16.3%), and Phase I (4.1%). A comprehensive treatment algorithm was drafted based on our institution's experience in managing MOE. The mortality rate was low (8.2%), mainly involving patients in advanced phase of the disease (Phases IV and V). CONCLUSION: This study has revealed the evidence of progression of MOE based on the proposed radiological stratification. This stratification is simple and practically applicable in clinical settings. We suggest the use of our proposed treatment algorithm as a standard diagnostic and treatment protocol for MOE.