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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.

Purpose Congenital cholesteatoma (CC) presents as a white pearl-like lesion behind a normal tympanic membrane (TM), without a history of otorrhea, infection, perforation or previous otologic surgery. Several recent studies provided new data improving this pathology characterization. The aim of this paper is to expand the knowledge about CC and to provide new insights on its pathogenesis. Methods The study consisted of two main research parts: (1) systematic review and meta-analysis; (2) medical literature review englobing anatomy, histology, embryology and congenital pathology of the ear. Results The search strategy identified a total of 636 papers. Seventy retrospective studies were included. A total of 1497 cases were studied and the mean age was 6.58 years, with a male–female ratio of 3:1, 34% were asymptomatic, 26% had hearing loss and 2% had facial dysfunction/paralysis. The overall estimate for antero-superior quadrant involvement was 0.70 [95% confident interval (CI) 0.64–0.76], in the postero-superior quadrant was 0.60 (95% CI 0.52–0.69), in the antero-inferior quadrant was 0.32 (95% CI 0.23–0.41), in the postero-inferior quadrant was 0.38 (95% CI 0.29–0.47), in the attic was 0.53 (95% CI 0.43–0.63) and in the mastoid was 0.33 (95% CI 0.26–0.41). More advanced Potsic stages were present in older patients. The most likely inclusion place seems to be between the pars flaccida and the upper quadrants of the pars tensa. Conclusions During the last decades, a substantial improvement in CC diagnosis and management had been achieved. The presented mechanism seems to explain most of middle ear CC.

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.

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.

Background Human ear growth continues beyond puberty, resulting in ears that are typically approximately 10 millimeters (mm) larger in older individuals compared to their younger counterparts. Not only is the overall growth of the ear an indicator of age, but also the disproportionate growth of its parts. The largest part, the concha, shows the slowest growth rate, while the smallest part, the earlobe, shows the greatest growth rate. Thus, over time, the net increases in the scapha, concha, and earlobe become nearly equal. Therefore, it is appropriate to make approximately equal reductions from each unit in order to restore a youthful balance to the ear. Method Aged and large, but otherwise normal, ears were selected for use of the “rule of threes” technique. This technique involves reducing the height of each of the three units (scapha, concha, and earlobe) by approximately 3 mm. Depending on individual needs, reductions were also applied to two units or just one unit as necessary. Adjustments in reduction amounts were made for previously disproportionate ears. Patients and Results A total of 32 ears from 16 patients were included in the study. Scapha reduction was performed on six patients, scapha and concha reduction on five patients, combined (including earlobe) reduction on two patients, and isolated earlobe reduction on three patients. All patients were followed up for at least 6 months. The planned reduction target was achieved in all cases, resulting in balanced youthful ears. Two skin sloughs in the earlobe, one requiring revision, and bilateral hypertrophic scarring behind the earlobe were observed in one case. Conclusion The described ear reduction technique yields satisfactory results when performed with precision. Each ear should be assessed individually, allowing for some flexibility rather than strict adherence to the proposed amounts of reduction. Care must be taken not to disrupt the blood supply to the ear structures. Achieving harmony at junction lines requires careful planning and meticulous technique. Level of Evidence IV This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266

Lymphoepithelioma-like carcinoma typically arises in the nasopharynx. Primary occurrence in the middle ear with subsequent intracranial extension remains exceedingly rare, and its diagnosis is often challenging owing to the paucity of detailed clinicoradiological data. Herein, we describe a case of an 82-year-old male presenting with a 2-year history of left-sided hearing loss, a 3-year history of left facial asymmetry (including mouth deviation and eyelid insufficiency), and a 10-day history of nausea/vomiting. His medical history included chronic otitis media. Imaging studies revealed a left cerebellopontine angle mass and chronic left otomastoiditis. Subtotal tumor resection was performed, and pathological analysis confirmed the diagnosis of lymphoepithelioma-like carcinoma. The patient received only supportive care and survived for 23 months. This case report aimed to emphasize the need for clinicians consider atypical cerebellopontine angle tumors in patients with chronic otitis media from Epstein-Barr virus–endemic regions and highlight the importance of a comprehensive diagnostic and therapeutic approach.

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.

ABSTRACT Few studies have evaluated the efficacy of proton beam therapy (PBT) for external auditory canal cancer (EACC) or middle ear cancer (MEC). Therefore, the present study aimed to evaluate the efficacy and toxicity of PBT for EACC and MEC. Between December 2009 and August 2018, 15 patients (seven males and eight females) underwent PBT for EACC or MEC. The median patient age was 64 years (range: 40–82 years). Ten patients had EACC, and five patients had MEC. PBT was administered using the passive scattering method. The median total dose of the BED10 was 88.8 Gy relative biological effectiveness (RBE) (range = 85.6–99.3 Gy [RBE]), administered in 2 Gy fractions (EQD2) (α/β = 10). Chemotherapy was conducted in 14 patients, systemic chemotherapy in five and intra-arterial infusion chemotherapy in nine. The median follow-up period for all patients was 60 months (range: 7–136 months). The 3-year overall survival, local control and progression free survival rates were 65%, 66.7% and 53.3%, respectively. Four patients had grade 3 hearing impairment owing to late toxicity. However, these patients had hearing impairment before treatment as well. No grade 4 or higher late toxicity was observed during the follow-up period. PBT is an effective treatment for EACC and MEC with tolerable toxicity, including in the setting of combined chemotherapy.