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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.

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.

In cholesteatoma surgery, precise tissue resection is important, as the diseased tissue needs to be completely removed while preserving the ossicular chain to prevent recurrence and hearing loss. The ability to precisely ablate tissue using a pulsed diode laser operating in single-trigger mode was investigated. Single laser pulses with a duration of were applied with a power ranging from via a multimode optical fiber. Fiber distance to tissue was varied from , and irradiation angles from . Porcine ear cartilage with remaining perichondrium on top served to represent the human ossicle structure with cholesteatoma. Further ablation experiments were performed on human cholesteatoma and ossicle samples. Ablation craters were measured by optical coherence tomography (OCT), and tissue damage assessed with haematoxylin and eosin (H&E)-stained sections. The ablation depth reached up to when operating at power output without causing damage to the underlying cartilage. Laser pulses with successfully ablated cholesteatoma, but did not damage the ossicles. The results show that a diode laser pulses can be used to effectively remove cholesteatoma, potentially preserving ossicles and hearing ability.

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. Develop an imaging system to enhance the visibility of cholesteatoma tissue and margins during surgery. 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. 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. We prototyped an imaging system that is capable of measuring cholesteatoma tissue autofluorescence.

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.

Auriculocondylar syndrome (ARCND) is an autosomal monogenic disorder characterised by external ear abnormalities and micrognathia due to hypoplasia of the mandibular rami, condyle and coronoid process. Genetically, three subtypes of ARCND (ARCND1, ARCND2 and ARCND3) have been reported. To date, five pathogenic variants of GNAI3 have been reported in ARCND1 patients. Here, we report a novel variant of GNAI3 (NM_006496:c.807C>A:p.(Asn269Lys)) in a Japanese girl with micrognathia using trio-based whole exome sequencing analysis. The GNAI3 gene encodes a heterotrimeric guanine nucleotide-binding protein. The novel variant locates the guanine nucleotide-binding site, and the substitution was predicted to interfere with guanine nucleotide-binding by in silico structural analysis. Three-dimensional computer tomography scan, or cephalogram, displayed severely hypoplastic mandibular rami and fusion to the medial and lateral pterygoid plates, which have been recognised in other ARCND1 patients, but have not been described in ARCND2 and ARCND3, suggesting that these may be distinguishable features in ARCND1.

This study intends to identify factors that could better predict the diagnosis of perilymphatic fistula (PLF) since exploration surgery is currently the only confirmatory method. This retrospective chart review in a tertiary care center is based on all 71 available patient files operated for a suspicion of PLF between 1983 and 2012. History of predisposing factors, clinical findings and investigations were documented pre- and postoperatively. Patients were divided according to intraoperative findings into two groups: group I (fistula negative) and group II (fistula positive). In addition, group II was divided into two subgroups: patients with or without a history of stapedectomy. Both groups were demographically similar. With the exception of history of previous partial stapedectomy ( p  = 0.04), no statistical difference could be identified in predisposing factors and in clinical findings between the two groups. The evolution of symptoms showed an overall improvement of vestibular symptoms (91 %) and cochlear symptoms (53 %) postoperatively. Audiograms showed a significant improvement postoperatively in the pure tone audiometry and bone conduction threshold of group II while the air-bone gap and speech discrimination score did not improve. Group I did not show any significant improvement postoperatively in any audiogram parameter. This study failed to identify factors that could better predict the diagnosis of PLF. However, it shows that middle ear exploration with oval and round window obliteration is effective in PLF especially to decrease vestibular symptoms even when fistula is unidentified intraoperatively.

Carcinoid tumors in the ear canal are very rare. In this report, we experienced a case of carcinoid tumor of the ear canal that underwent total tumor resection. This study included a 39-year-old man presented with a chief complaint of right-sided hearing loss. Computed tomography scan showed a shadow from the ear canal to the right tympanic chamber. There were no suspicious findings of metastasis in the cervical lymph nodes or other organs. At the time of surgery, the tumor was simply removed because it was small and there was no adhesive invasion. Postoperatively, the patient has been under observation for 11.5 years without any recurrence. Carcinoid tumors in the ear canal can rarely metastasize or recur after more than 10 years. It is important to follow up with the patient for a long time after surgery, using the Ki-67 index of the removed tissue as a prognostic reference.

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.

This study aimed to evaluate the clinical features and outcomes of patients with middle-ear granulation pathologies associated with attic retractions. The clinical records of adult patients with middle-ear granulation pathologies and attic retractions confirmed via computed tomography and surgical exploration between January 2012 and January 2019 were retrospectively reviewed. A total of 59 patients were included. Endoscopic examination showed a normal pars tensa but retraction of the pars flaccida in all patients. No granulation tissue or debris were observed. Low-pitched tinnitus was the principal complaint of 55 patients (100 per cent), followed by ear fullness (14 patients, 23.7 per cent). Of the 59 patients, 52 patients (88.1 per cent) underwent canal wall up mastoidectomy and 7 patients (11.9 per cent) underwent endoscopic endaural atticoantrotomy. No ossicular chain destruction was evident. All patients were followed up for 12 months. Tinnitus disappeared completely in 48 patients (81.4 per cent), improved significantly in 9 patients (15.3 per cent) and improved mildly in 2 patients (3.3 per cent). A granulation tissue pathology should be considered when a patient complains of low-pitched tinnitus and exhibits retraction of the pars flaccida. Computed tomography and surgical exploration should be scheduled.