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External auditory canal carcinoma (EACC) is a rare and aggressive malignancy with substantial variability in prognosis depending on tumor stage and adjacent structure involvement. We retrospectively reviewed 56 patients with histologically confirmed squamous cell carcinoma of the external auditory canal treated at a tertiary referral center between 2000 and 2022. Clinical data including demographics, tumor stage, treatment modalities, surgical approach, and survival outcomes were analyzed. Kaplan–Meier survival curves and Cox proportional hazards regression were used to identify prognostic factors for overall survival (OS) and disease-specific survival (DSS). Of the 56 patients (mean age 61.6 years; 46.4% female), 30 had early-stage (T1–T2) and 26 had advanced-stage (T3–T4) tumors. The 5-year OS rates were 100.0% for early-stage, 60.0% for T3, and 42.0% for T4 disease. Advanced T-stage, nodal metastasis, and abutment to vascular structures such as the carotid artery or jugular bulb were significantly associated with worse outcomes. In multivariate analysis, younger age, vascular abutment, and nodal metastasis were independent negative prognostic factors. En bloc resection with clear margins was associated with improved survival. These findings emphasize the importance of early diagnosis and meticulous surgical planning to achieve complete resection and optimize outcomes in patients with EACC.

Cholesteatoma is a prevalent disease affecting both children and adults. In this review, we present the recent findings related to the molecular mechanisms involved in cholesteatoma and discuss how researchers can target new molecules to treat this disease. These new approaches illustrate the paradigm shift from a primarily surgical solution to a biological “control and prevent” strategy.

Porcine ear necrosis (PEN) (also referred to as ear-tip necrosis, ETN) is a syndrome of global presence and unclear aetiology. Initially reported in the 1950s, many different infectious and non-infectious causes have been suggested as the causative(s) agent(s), but none has been confirmed in controlled studies. Here, we investigated the aetiology of PEN using pure culture of bacteria associated with lesions in controlled animal trials. A commercial farm with no history of ear-tip necrosis was identified and used as the source for 5-week-old pigs. Two independent trials were initially executed with identical designs. Piglets (=12/trial) were intradermally inoculated with either pure cultures of Staphylococcus hyicus or Fusobacterium necrophorum (left ear, n = 10) or sterile media (right ear, n = 10). Two pigs in each trial were not inoculated, serving as sentinels. A third trial used F. necrophorum as the inoculum, 3 pigs as sentinels and 9 as inoculated. All animals were clinically monitored daily following challenge, and an ear score was used to follow disease progression. All ears inoculated with S. hyicus remained lesion free. Four out of ten and 7/9 pigs challenged with F. necrophorum developed lesions undistinguishable from PEN, including necrosis and loss of portions of the ear pinna (P < 0.001). F. necrophorum was isolated from 4/10 and 7/9 pigs that developed necrotic lesions. Histopathology after resolution of necrosis revealed granulomatous tissue. Evidence presented here suggests that F. necrophorum causes PEN-like lesions, as seen in commercial barns. It is therefore suggested as the etiological agent of this syndrome.

The existence of acquired cholesteatoma has been recognized for more than three centuries; however, the nature of the disorder has yet to be determined. Without timely detection and intervention, cholesteatomas can become dangerously large and invade intratemporal structures, resulting in numerous intra- and extracranial complications. Due to its aggressive growth, invasive nature, and the potentially fatal consequences of intracranial complications, acquired cholesteatoma remains a cause of morbidity and death for those who lack access to advanced medical care. Currently, no viable nonsurgical therapies are available. Developing an effective management strategy for this disorder will require a comprehensive understanding of past progress and recent advances. This paper presents a brief review of background issues related to acquired middle ear cholesteatoma and deals with practical considerations regarding the history and etymology of the disorder. We also consider issues related to the classification, epidemiology, histopathology, clinical presentation, and complications of acquired cholesteatoma and examine current diagnosis and management strategies in detail.

High-resolution computed tomography (HRCT) has become a widely used tool for studying the inner ear morphology of vertebrates. Amphisbaenians are one of the most specialized groups of fossorial reptiles but are poorly understood relative to other squamate reptile. In this paper we survey the anatomy of the inner and middle ear of these fossorial reptiles using HRCT models and we describe qualitatively and quantitatively (using 3D morphometrics) the anatomy of the inner ear. Amphisbaenians are diverse in skull anatomy, especially in the configuration of the snout, which correlates with digging modes. We demonstrate that the ear also exhibits a diversity of configurations, which are independent of phylogenetic relationships. Results from morphological analyses also allow us to describe 11 new potentially informative phylogenetic characters including some that help to diagnose amphisbaenians, such as: 1) the globular vestibule, ii) semicircular canals arranged in a circular trajectory, and iii) an extensive area of interaction between the columella footplate and the lagenar recess. Among extant amphisbaenians, Rhineura floridana has the most unusual inner ear configuration, including a horizontal semicircular canal that is in the same orientation as the inclined snout. The new morphological information helps us to better understand the morphology of headfirst-burrowing fossorial reptiles and contributes new data for resolution of phylogenetic relationships among amphisbaenians.

To investigate the differences in computed tomography (CT) features between closed-type congenital cholesteatoma (CCC) and open-type congenital cholesteatoma (OCC) of the middle ear and to evaluate the usefulness of preoperative CT examination for staging workup of congenital cholesteatoma (CC) in correlation with the surgical findings. We retrospectively reviewed the preoperative CT scans of the temporal bone obtained from 80 patients with surgically confirmed CC of the middle ear. All patients had a solitary lesion, except for one patient with two lesions, resulting in 81 CCs, which formed the basis of this study. We compared the CT features between CCCs and OCCs, focusing on their morphological characteristics, such as size, shape, location, and bone change. Based on the Potsic classification, the stage of CCs was determined at CT and surgery, and the results were compared between CCCs and OCCs. Of the 81 CCs, surgery revealed 43 CCCs and 38 OCCs. On CT scans, CCC was frequently seen as a small (median: 3.15 mm), round to oval (65.1%) mass, most commonly located in the anterosuperior quadrant (74.4%) of the middle ear with less frequent ossicular erosion (14.0%). In contrast, OCC was frequently seen as a large (median: 6.70 mm), irregular (94.7%) mass, most commonly located in the posterosuperior quadrant (68.4%) of the middle ear with frequent ossicular erosion (55.3%). The size, shape, location, and presence of ossicular erosion were significantly different between the two types. Overall, the CT and surgical stages of CCs demonstrated good agreement (kappa value: 0.77) and the CT and surgical stages of OCCs were statistically significantly higher than those of CCCs ( < 0.001 in both). CT is useful for preoperative determination of the types and staging of CC of the middle ear. Preoperative differentiation between CCC and OCC is important to avoid reoperation and prevent an extensive surgery. By providing valuable information on the morphology and extent of the lesions, CT is useful for not only the accurate preoperative determination of the type of CCs but also the accurate prediction of staging of the lesion, which should be important to preparing optimal treatment plans.

The prognosis of patients with advanced squamous cell carcinoma of the external auditory canal and middle ear has been improved by advances in skull base surgery and multidrug chemoradiotherapy during the last two decades. Ninety-five patients with squamous cell carcinoma of the external auditory canal and middle ear who were treated between 1998 and 2017 were enrolled. The number of patients with tumour stages T1, T2, T3 and T4 was 15, 22, 24 and 34, respectively. Oncological outcomes and prognostic factors were retrospectively investigated. Among patients with T4 disease, invasion of the brain (p = 0.024), carotid artery (p = 0.049) and/or jugular vein (p = 0.040) were significant predictors of poor prognosis. The five-year overall survival rate of patients with at least one of these factors (T4b) was significantly lower than that of patients without these factors (T4a) (25.5 vs 65.5 per cent, p = 0.049). It is proposed that stage T4 be subclassified into T4a and T4b according to the prognostic factors.

Human pluripotent stem cells are differentiated into inner ear organoids containing cells similar to hair cells and sensory neurons. The derivation of human inner ear tissue from pluripotent stem cells would enable in vitro screening of drug candidates for the treatment of hearing and balance dysfunction and may provide a source of cells for cell-based therapies of the inner ear. Here we report a method for differentiating human pluripotent stem cells to inner ear organoids that harbor functional hair cells. Using a three-dimensional culture system, we modulate TGF, BMP, FGF, and WNT signaling to generate multiple otic-vesicle-like structures from a single stem-cell aggregate. Over 2 months, the vesicles develop into inner ear organoids with sensory epithelia that are innervated by sensory neurons. Additionally, using CRISPR–Cas9, we generate an ATOH1-2A-eGFP cell line to detect hair cell induction and demonstrate that derived hair cells exhibit electrophysiological properties similar to those of native sensory hair cells. Our culture system should facilitate the study of human inner ear development and research on therapies for diseases of the inner ear.

Children suffering from microtia have few options for auricular reconstruction. Tissue engineering approaches attempt to replicate the complex anatomy and structure of the ear with autologous cartilage but have been limited by access to clinically accessible cell sources. Here we present a full-scale, patient-based human ear generated by implantation of human auricular chondrocytes and human mesenchymal stem cells in a 1:1 ratio. Additional disc construct surrogates were generated with 1:0, 1:1, and 0:1 combinations of auricular chondrocytes and mesenchymal stem cells. After 3 months in vivo, monocellular auricular chondrocyte discs and 1:1 disc and ear constructs displayed bundled collagen fibers in a perichondrial layer, rich proteoglycan deposition, and elastin fiber network formation similar to native human auricular cartilage, with the protein composition and mechanical stiffness of native tissue. Full ear constructs with a 1:1 cell combination maintained gross ear structure and developed a cartilaginous appearance following implantation. These studies demonstrate the successful engineering of a patient-specific human auricle using exclusively human cell sources without extensive in vitro tissue culture prior to implantation, a critical step towards the clinical application of tissue engineering for auricular reconstruction.