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.

Intracochlear Trauma and Local Ossification Patterns Differ Between Straight and Precurved Cochlear Implant Electrodes.

HYPOTHESIS: Trauma to the osseous spiral lamina (OSL) or spiral ligament (SL) during cochlear implant (CI) insertion segregates with electrode type and induces localized intracochlear ossification and fibrosis. BACKGROUND: The goal of atraumatic CI insertion is to preserve intracochlear structures, limit reactive intracochlear tissue formation, and preserve residual hearing. Previous qualitative studies hypothesized a localized effect of trauma on intracochlear tissue formation; however, quantitative studies failed to confirm this. METHODS: Insertional trauma beyond the immediate insertion site was histologically assessed in 21 human temporal bones with a CI. Three-dimensional reconstructions were generated and virtually resectioned perpendicular to the cochlear spiral at high resolution. The cochlear volume occupied by ossification or fibrosis was determined at the midpoint of the trauma and compared with regions proximal and distal to this point. RESULTS: Seven cases, all implanted with precurved electrodes, showed an OSL fracture beyond the immediate insertion site. Significantly more intracochlear ossification was observed at the midpoint of the OSL fracture, compared with the -26 to -18 degrees proximal and 28 to 56 degrees distal to the center. No such pattern was observed for fibrosis. In the 12 cases with a perforation of the SL (9 straight and 3 precurved electrodes), no localized pattern of ossification or fibrosis was observed around these perforations. CONCLUSION: OSL fractures were observed exclusively with precurved electrodes in this study and may serve as a nidus for localized intracochlear ossification. Perforation of the SL, in contrast, predominantly occurred with straight electrodes and was not associated with localized ossification.

Elongated styloid process of an autopsied skull from the Cemetery of Santa Maria Maggiore in Vercelli, 18th-19th century (Piedmont, northern Italy).

We discuss the coexistence of a postmortem cut and a pathological alteration, recorded on a skeleton belonging to an adult man that was discovered during the archaeological investigations of the cemetery of the Church of Santa Maria Maggiore in Vercelli (northern Italy, 18th-19th century). The skull presents an oblique cleft, which from the top of the frontal bone bends towards the occipital, and the left styloid process is elongated compared to normal values (48 mm). The elongated styloid process is due to the ossification of the styloid ligament which has several possible causes. To increase the knowledge about this pathological condition in the past, it was necessary to compare all the data present in the literature today and consider the few cases published in the paleopathological field. In this paper, our main goals are: i) to investigate the reasons for which the craniotomy was performed; ii) to examine the possible cause of the ossification of the styloid process, described as Eagle's syndrome; iii) to enrich the archaeological literature of elongated styloid process cases and iv) to investigate the presence of a hypothetical relationship between the autopsy cut and the diagnosed Eagle's syndrome on this skull.

Evaluation of Eustachian tube dimensions by temporal bone computed tomography in patients with chronic otitis media.

OBJECTIVE: To clarify the relationship between Eustachian tube dimensions and chronic otitis media aetiology using temporal bone computed tomography. METHODS: The data of 231 adults who had undergone surgery for unilateral chronic otitis media were reviewed retrospectively. Diseased and healthy ears were enrolled in groups 1 and 2, respectively. Group 1A included chronic otitis media with cholesteatoma (n = 28) and group 1B included chronic otitis media without cholesteatoma (n = 203). The Eustachian tube dimensions of groups 1 and 2 were compared, to clarify the relationship between the Eustachian tube dimensions and chronic otitis media aetiology. Groups 1A and 1B were compared to assess the effect of Eustachian tube dimensions on cholesteatoma development. RESULTS: The Eustachian tube was shorter, narrower and located more horizontally in ears with chronic otitis media. No significant difference was found between groups 1A and 1B. CONCLUSION: Eustachian tube dimensions are closely related to chronic otitis media aetiopathology, but are not related to cholesteatoma development.

The clinical availability of facial nerve enhancement in temporal bone MRI for the patients of idiopathic acute peripheral facial palsy.

PURPOSE: This study is to evaluate the duration of facial nerve enhancement in gadolinium-enhanced temporal bone MRI after the onset of acute facial palsy. METHODS: Gd-enhanced MRI imagines were examined in 13 patients with idiopathic acute facial palsy within 14 days after the onset. The degree of facial nerve function was measured according to the House-Brackmann (H-B) grading system at their first visit at outpatient clinic. The follow-up MRI was taken about 16.5 months (7-24 months) after onset of disease. The degree of facial nerve enhancement was measured with signal intensity (SI) which was quantitatively analyzed using the region-of-interest (ROI) measurements for each segment of the facial nerve. SI was statistically analyzed by comparing SI values of contralateral site and ipsilateral site using the paired t test with SPSS program. RESULTS: The gadolinium enhancement was statistically increased at labyrinthine segment and geniculate ganglion area of facial nerve at initial temporal bone MRI. The gadolinium enhancement was statistically decreased at all the segments of facial nerve except tympanic segment (p < 0.05) at follow-up MRI. CONCLUSIONS: The facial nerve enhancement in Gd-enhanced MRI images prolonged more than 21 months of the onset. The newly developed pathologic lesions of acute facial palsy especially occur at the site of labyrinthine and geniculate ganglion.

Imaging of Pathologies of the Temporal Bone and Middle Ear: Inflammatory Diseases, Their Mimics and Potential Complications-Pictorial Review.

Imaging of the temporal bone and middle ear is challenging for radiologists due to the abundance of distinct anatomical structures and the plethora of possible pathologies. The basis for a precise diagnosis is knowledge of the underlying anatomy as well as the clinical presentation and the individual patient's otological status. In this article, we aimed to summarize the most common inflammatory lesions of the temporal bone and middle ear, describe their specific imaging characteristics, and highlight their differential diagnoses. First, we introduce anatomical and imaging fundamentals. Additionally, a point-to-point comparison of the radiological and histological features of the wide spectrum of inflammatory diseases of the temporal bone and middle ear in context with a review of the current literature and current trends is given.

Is the promontory a promising site to diagnose otitis media in paleopathology? A search for evidence.

OBJECTIVE: The promontory of the middle ear was recently suggested to be an appropriate site for diagnosing otitis media (OM) in archaeological bones by endoscopic inspection. The present study scrutinized the underlying assumption that a bulgy, irregular promontorial surface represents a pathological condition. MATERIALS: We compared an allegedly healthy individual and an allegedly diseased individual in skeletal remains of two human individuals from the early Medieval period in Germany. METHODS: The specimens were studied using microscopic analyses of thin ground sections. RESULTS: The osseous architecture of the three-layered promontorial wall was the same in both specimens. Both the contour of the resorption front of the middle layer and the thickness of the overlying outer periosteal layer showed some variation, resulting in an either smooth or a bulgy promontorial surface, while signs of resorptive or proliferative changes at the periosteal surface were missing in both cases. CONCLUSIONS: Our results suggest that an irregular promontorial surface represents normal variation in the development of the otic capsule rather than a pathological condition. We therefore conclude that the promontory is not an appropriate site for diagnosing OM in archaeological bone. SIGNIFICANCE: The study contributes to evidence-based diagnoses in paleo-otological studies. Our assumption is in line with clinical and experimental findings indicating that the otic capsule is protected against bone remodeling. LIMITATIONS: Only two specimens were studied. SUGGESTIONS FOR FURTHER RESEARCH: SEM-studies to detect more subtle changes to the promontorial surface.

Prevalence of zygomatic air cell defects in a North American population.

Zygomatic air cell defects (ZACDs) are asymptomatic defects present in the zygomatic process and articular eminence of the temporal bone. This defect is considered a normal anatomical variant and can be detected on routine panoramic images. The objective of this study was to determine the prevalence, dominant type, and location of ZACDs in a North American population. A total of 1724 panoramic images of adult patients were analyzed by a third-year dental student in conjunction with an oral and maxillofacial radiologist. Patient demographics (age and sex) as well as ZACD location (unilateral right/left or bilateral) and appearance (unilocular or multilocular) were evaluated. Statistical analyses included the Clopper-Pearson method to calculate the 95% CI, Student t test for independent samples, and Fisher exact test. The prevalence of ZACDs in this study population was 2.1%. A total of 24 women (2.3%) and 12 men (1.7%) were affected, with no statistically significant difference between the sexes. The mean (SD) age of patients presenting with a ZACD was 56.6 (17.7) years, while that of patients without a ZACD was 53.0 (16.3) years, with no statistically significant difference between groups. Among the 36 patients with ZACDs, 28 (1.6%) had unilateral and 8 (0.5%) had bilateral lesions. One patient with bilateral ZACDs had a unilocular lesion on the right side and a multilocular lesion on the left, so there were a total of 31 unilocular lesions (1.8%) and 6 multilocular lesions (0.3%). The results of this study indicate that the prevalence of ZACDs in the study population was 2.1%, without any statistically significant differences based on the sex or age of the patient or laterality or appearance of the lesions.

Dedifferentiated Primary Parosteal Osteosarcoma of the Temporal Bone in a 19-Year-Old Patient: A Case Report.

Osteosarcoma is the most common primary malignant tumor affecting the bone but is a rare occurrence in the head and neck region. Complete surgical resection with wide surgical margins is currently the main treatment strategy for osteosarcoma but can be hard to achieve due to the complex anatomy of the head and neck. We report the first case of primary high-grade dedifferentiated parosteal osteosarcoma arising from the temporal bone in published literature. The 19-year-old patient presented with a left retroauricular lesion measuring 3 cm in diameter. Radiographic imaging and biopsy suggested the diagnosis of intermediate-grade chondrosarcoma, but definitive histopathology confirmed a diagnosis of dedifferentiated parosteal osteosarcoma. The tumor was resected with wide margins, removing the underlying temporal bone, periosteum and overlying soft tissue through a lateral temporal bone resection. The middle ear was reconstructed with cartilage grafting, and the dura of the posterior and middle cranial fossa was covered using temporal fascia grafts and local transpositional flaps. The patient is recurrence free 10 months after treatment. This report was assembled following CARE [The CARE guidelines (for Case Reports)] guidelines and describes clinical, histological, and radiological manifestations of our patient's rare clinical entity and may provide more data in treating patients with osteosarcoma affecting the anatomically complex head-and-neck region.

Emerging Mechanisms in the Pathogenesis of Menière's Disease: Evidence for the Involvement of Ion Homeostatic or Blood-Labyrinthine Barrier Dysfunction in Human Temporal Bones.

HYPOTHESIS: Analysis of human temporal bone specimens of patients with Menière's disease (MD) may demonstrate altered expression of gene products related to barrier formation and ionic homeostasis within cochlear structures compared with control specimens. BACKGROUND: MD represents a challenging otologic disorder for investigation. Despite attempts to define the pathogenesis of MD, there remain many gaps in our understanding, including differences in protein expression within the inner ear. Understanding these changes may facilitate the identification of more targeted therapies for MD. METHODS: Human temporal bones from patients with MD (n = 8) and age-matched control patients (n = 8) were processed with immunohistochemistry stains to detect known protein expression related to ionic homeostasis and barrier function in the cochlea, including CLDN11, CLU, KCNJ10, and SLC12A2. Immunofluorescence intensity analysis was performed to quantify protein expression in the stria vascularis, organ of Corti, and spiral ganglion neuron (SGN). RESULTS: Expression of KCNJ10 was significantly reduced in all cochlear regions, including the stria vascularis (9.23 vs 17.52, p = 0.011), OC (14.93 vs 29.16, p = 0.014), and SGN (7.69 vs 18.85, p = 0.0048) in human temporal bone specimens from patients with MD compared with control, respectively. CLDN11 (7.40 vs 10.88, p = 0.049) and CLU (7.80 vs 17.51, p = 0.0051) expression was significantly reduced in the SGN. CONCLUSION: The results of this study support that there may be differences in the expression of proteins related to ionic homeostasis and barrier function within the cochlea, potentially supporting the role of targeted therapies to treat MD.

Patterns of spread and anatomical prognostic factors of pre-auricular cutaneous squamous cell carcinoma extending to the temporal bone.

BACKGROUND: Improvements can be made in the management and staging of advanced pre-auricular cutaneous squamous cell carcinoma (cSCC). We aimed to analyze radiological patterns of spread and clinico-anatomical prognostic factors. METHODS: Retrospective review of 54 patients with pre-auricular cSCC (cutaneous/nodal) who underwent temporal bone resection with curative intent. RESULTS: Involvement of the cartilaginous external auditory canal (EAC) (79.6%) and retromandibular space (63.0%) was common. Styloid process/anterior carotid sheath (ACS) (11.1%) and bony EAC (7.4%) involvement were rare. ACS involvement resulted in high rates of involved surgical margins (100%) and poor outcomes on univariable analysis. Negative prognostic factors on multivariable analysis included salvage surgery and invasion of the bony EAC, mandible, pterygoid muscle(s), and dura. CONCLUSION: The bony EAC and ACS can form temporary barriers to tumor spread, with the latter representing a potential limit of resectability. Prognostic factors revealed can lead to the development of a more appropriate staging tool.

Natural history of facial paraganglioma with 2 decades of follow-up: A case report and literature review.

OBJECTIVE: To report 20 years of natural history data for a facial paraganglioma and provide a comprehensive review of the existing literature. PATIENT: 81-year-old female with a remote history of cardiac arrest while under anesthesia who elected to observe her facial paraganglioma for 20 years. INTERVENTIONS: Observation, clinical documentation, radiographic surveillance. MAIN OUTCOME MEASURES: Tumor progression, patient symptomatology, and review of management options. RESULTS: The initial presentation of the facial paraganglioma was facial spasm. Over the course of observation, symptoms progressed to include complete facial nerve paralysis, pulsatile tinnitus, and otalgia on the affected side. Radiologic surveillance demonstrated incremental growth and erosion of surrounding structures, including the posterior external auditory canal, stylomastoid foramen, and lateral semicircular canal with near-dehiscence. Twenty-four cases of facial paraganglioma were identified in the extended literature search and are summarized herein. CONCLUSIONS: This unique case contributes to the scarce literature surrounding facial paragangliomas by reporting the extended natural history of this disease.

[Diagnosis and treatment of rare malignant temporal bone tumors].

Objective:To analyze the diagnosis, treatment and prognosis of patients with rare malignant tumors of the temporal bone. Methods:Four cases of rare temporal bone malignant tumors in our hospital between March 2014 and December 2020 were reviewed, including two cases of chondrosarcoma, one case of fibrosarcoma and one case of endolymphatic cystic papillary adenocarcinoma. There were three males and one female, ages between 28 and 56 years at the time of surgery. Common symptoms included hearing loss, facioplegia, tinnitus, and headache. All patients underwent imaging examinations to evaluate the extent of the lesions. Tumors were removed by subtotal temporal bone resection or infratemporal fossa approach, and postoperative adjuvant radiotherapy was applied if necessary. Results:One of the two chondrosarcoma patients was cured by complete resection of the tumor for 75 months, the other one recurred after the first excision of the tumor and underwent infratemporal fossa approach resection of skull base mass again with no recurrence found yet for 112 months. One patient with fibrosarcoma survived for 28 months after surgery with a positive margin and post-operative radiotherapy. One patient with endolymphatic cystic papillary adenocarcinoma recurred 12 months after subtotal lithotomy, and underwent subtotal temporal bone resection again, combined with radiotherapy. No recurrence was found for 63 months. Conclusion:The incidence of rare temporal bone malignant tumors is extremely low, the location is hidden, and the symptoms are atypical. Attention should be paid for early detection and early treatment. Surgical resection is the main treatment, and radiotherapy can be supplemented in the advanced stage or with a positive margin.

Giant Cell Tumor of the Temporal Bone and Skull Base.

Giant cell tumor (GCT) is a benign tumor that originates from undifferentiated mesenchymal cells of the bone marrow. The craniums as well as temporal bone are extremely rare locations for GCTs. Clinical, radiological, and anatomical diagnosis of this locally aggressive disease poses a major challenge in clinical practice. In this article, we present a clinical study for a 35-year-old female who was found to have left-sided temporal bone GCT with extension to middle cranial fossa and temporomandibular joint (TMJ) with its clinical features and management.

An inter-observer assessment of mastoid pneumatization and degree classification using sigmoid sinus: comparing two levels of temporal bone computed tomograms.

BACKGROUND: The degree of mastoid pneumatization of the temporal bone (TB) has been implicated in the pathogenesis of TB diseases and surgical implications, and planning of a few otologic surgeries. However, there is lack of consensus in the classification of the degree of pneumatization. This study aimed to suggest a simple, quick, and less-burden classification system for assessing and rating the degree of pneumatization by comparing two levels of TB computed tomographs (CTs) using the SS as a reference in an inter-observer assessment among otologists. METHODS: This was a randomized pilot survey among otologists. A questionnaire consisting of different axial CTs of TB taken at two levels: the level of malleoincudal junction (MIJ) and the level of lateral semicircular canal (LSCC), with different pneumatization patterns, was used to assess participants' impressions of the degree of pneumatization. The terms "hypo-," "moderate," "good," and "hyper-" pneumatization were listed as options to rate their impressions on the degree of mastoid pneumatization of the TB images using the SS as a reference structure. Likert scale was used to assess their level of agreement or disagreement with using SS as a reference in evaluating mastoid pneumatization. RESULTS: Participants who correctly rated images taken at the level of LSCC according to their respective degree of pneumatization were significantly higher (p < 0.05) regardless of their year of experience compared to those that correctly rated corresponding images taken at the level of MIJ. A 76% positivity in their level of agreement with the use of sigmoid sinus in evaluating mastoid pneumatization was observed on the Likert-scale chart. CONCLUSION: Findings from this study suggest that evaluating air cells around the SS at the level of LSCC on CTs could be easier in assessing and classifying the degree of mastoid pneumatization.

A clinical conundrum: Temporal bone metastases from lung adenocarcinoma.

BACKGROUND: Metastatic disease to the temporal bone is rare. Even more uncommonly, it can be the first manifestation of an underlying malignancy. Patients typically present late in the disease process with non-specific symptoms of hearing loss, facial nerve palsy and otorrhea. CASE: A 62-year-old Chinese female presented with right facial weakness, which had near-complete improvement in response to pulse prednisolone. On examination, she had a right temporal swelling and right mild-severe conductive hearing loss. A computed tomography scan showed a destructive lesion centred in the squamous temporal bone, with an associated soft tissue component. Positron emission tomography scan revealed bony and lung metastases, but no distinct hypermetabolic primary site. An incisional biopsy unexpectedly returned as metastatic lung adenocarcinoma. CONCLUSION: Although rare, it is important for otolaryngologists to be aware of the insidious nature of temporal bone metastases and possible atypical clinical and radiological features, to facilitate timely workup and initiation of treatment.

Anton de Haen (1704-1776) and his extraordinary "portentosum infundibulum" case: the futile skull cauterization of a blind patient with a craniopharyngioma.

Anton de Haen (1704-1776) became one of the most influential physicians in the Habsburg Empire as a reformer of clinical instruction at Vienna Citizen's Hospital (Bürgerspital), where he introduced the bedside teaching method he had learned from Herman Boerhaave in Leyden, Holland. He also promoted the meticulous recording of clinical observations and the use of postmortem studies to identify the cause of death in hospitalized patients. Among the numerous clinicopathological reports compiled in his monumental 18-volume work Rationis Medendi in Nosocomio Practico, published in 1761, was the first documented patient with amenorrhea caused by a pituitary tumor, appearing in the 6th volume. This 20-year-old amaurotic woman, who had suffered from chronic excruciating headache, died after the unsuccessful application of a cauterizing iron to her temporal bone. At the autopsy, a large solid-cystic and calcified tumor with gross characteristics typical of adamantinomatous craniopharyngioma was found encroaching on the infundibulum and third ventricle. This is the first known account of an infundibulo-tuberal lesion associated with the impairment of sexual functions, predating by 140 years the pathological evidence for a sexual brain center sited at the basal hypothalamus. In this paper, the authors analyze the historical importance and impact of de Haen's foundational report on the fields of neuroendocrinology and neurosurgery.

Neoadjuvant Immunotherapy for Head and Neck Squamous Cell Carcinoma: Expecting Its Application in Temporal Bone Squamous Cell Carcinoma.

Temporal bone malignant tumors are characterized by atypical clinical symptoms, and easy recurrence and metastasis. They account for 0.2% of head and neck tumors, and the most common pathological type is squamous cell carcinoma. Patients with squamous cell carcinoma of the temporal bone are often at advanced stages when diagnosed, and lose the chance for surgery. Neoadjuvant immunotherapy has recently been approved as the first-line treatment for refractory recurrent/metastatic squamous cell carcinoma of the head and neck. However, it remains to be determined whether neoadjuvant immunotherapy can be used as the first-line treatment for temporal bone squamous cell carcinoma to reduce the tumor stage before surgery, or as a palliative treatment for patients with unresectable advanced stage carcinoma. The present study reviews the development of immunotherapy and its clinical application in head and neck squamous cell carcinoma, summarizes the treatment of temporal bone squamous cell carcinoma, and prospects the neoadjuvant immunotherapy as the first-line treatment for temporal bone squamous cell carcinoma.

Cochlear implantation in a patient with congenital microtia, cochlear hypoplasia, venous anomalies of the temporal bone and laryngomalacia: Challenges and surgical considerations.

RATIONALE AND PATIENT CONCERNS: Congenital hearing loss is often caused by an inner ear malformation, in such cases, the presence of other anomalies, such as microtia, and venous anomalies of the temporal bone and laryngomalacia makes it challenging to perform cochlear implantation surgery. DIAGNOSES: This study reports the case of a 28-month-old girl with congenital profound hearing loss, laryngomalacia, and malformed inner ear, who received cochlear implantation surgery. The bony structure, vessels and nerves were first assessed through magnetic resonance imaging and computed tomography before exploring the genetic basis of the condition using trio-based whole exome sequencing. Perioperative evaluation and management of the airway was then performed by experienced anesthesiologist, with the surgical challenges as well as problems encountered fully evaluated. INTERVENTIONS: Cochlear implantation was eventually performed using a trans-mastoid approach under uneventful general anesthesia. OUTCOMES: Due to the small size of the cochlea, a short electrode FLEX24 was inserted through the cochleostomy. LESSONS: Considering the high risk of facial nerve injury and limited access to the cochlea when patients present significant bony and venous anomalies, cochlear implantation in such patients require careful preoperative evaluation and thoughtful planning. In these cases, airway assessment, magnetic resonance venography, magnetic resonance arteriography, and magnetic resonance imaging and computed tomography can be useful to minimize the risks. Intraoperative facial nerve monitoring is also recommended to assist in the safe location of facial nerve.

Sex and Race Representation in Temporal Bone Histopathology Studies in the United States: A Systematic Review.

OBJECTIVES: The author's objective was to evaluate sex and race representation in temporal bone histopathology studies. DESIGN: PubMed, Embase, Cochrane, Web of Science, and Scopus were searched for studies written in English examining temporal bone histopathology specimens from U.S.-based institutions from January 1, 1947, to September 1, 2021. Two authors then performed "snowballing" by reviewing references from the initial search and included the studies that fulfilled the inclusion criteria. For each study, the following information was collected: publication details, study design, funding, institution from where temporal bone specimens were procured, number of study specimens, and donor demographical information. RESULTS: The authors found that out of 300 studies, 166 (55%) report sex while only 15 (5%) reported race information. Over the past 70 years, the ratio of studies reporting sex to those that do not has increased from 1.00 to 2.19 and the number of female temporal bone histopathology subjects relative to male has increased from 0.67 to 0.75. Over 90% of studies that do report this information feature participant racial compositions that do not reflect the diversity of the U.S. population. CONCLUSIONS: Studies of temporal bone histopathology often do not report participant sex or race. The reporting of participant sex and the inclusion of specimens from female donors have both increased over time. However, temporal bone histopathology study cohorts are not representative of the racial diversity of the U.S. population. The otolaryngology community must strive to build temporal bone histopathology libraries that are representative of the diverse U.S. population.