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Objective: To report a common site of external ear canal erosion in multiple pathologies, located inferiorly at 6 o'clock., Patients: Otology patients who came in 2023 for treatment of external auditory canal erosions., Intervention: This clinical capsule is an observational report of the external canal's propensity to erosion at the 6 o'clock location. Patient treatments were canalplasty, mastoidectomy, and medical management., Main Outcome Measure: Documentation of the propensity to erosion at the 6 o'clock location in the external auditory canal. Locations of the niduses of prior series of external auditory canal pathologies are documented., Results: Eight patients are presented with external auditory canal erosion in 10 ears originating at the 6 o'clock position medial to the bony-cartilaginous junction. No other patient with spontaneous canal erosion presented with their nidus of pathology in another canal location. (A review of 42 case series of 291 patients found that keratosis obturans and bisphosphonate-induced osteonecrosis tended to arise from the same 6 o'clock lateral bony canal location, while 26% of necrotizing otitis externa cases arose there.)., Conclusions: The "6 o'clock spot" in the external canal is a common location of canal erosion for spontaneous wax and keratin collections and may be the precursor to keratosis obturans, bisphosphonate-induced osteonecrosis of the ear canal, and necrotizing otitis externa., Competing Interests: The authors have no conflicts of interest to report, and this study had no funding., (Copyright © 2024, Otology & Neurotology, Inc.)

Objective: To systematically review the literature and understand the behavior, diagnosis, management, and mortality of metastasis to the external auditory canal (EAC)., Databases Reviewed: PubMed/Medline, EMBASE, Web of Science., Methods: Studies from 1948 to June 2023 describing metastasis to the EAC were included. Non-English literature was excluded. Data extraction: Study design, age, sex, pathology, primary site, staging, additional sites of metastasis, time to EAC metastasis from diagnosis, time from diagnosis of EAC metastasis to death, symptoms, exam and imaging findings, and management., Results: Data were synthesized qualitatively with means calculated. Thirty-two studies met the criteria, totaling 37 patients with EAC metastasis. Mean age was 58 years; 73% were male. The most common pathologies were adenocarcinoma (37.8%), acute myelogenous leukemia (8.1%), and renal cell carcinoma (8.1%). Sites of primary malignancy were hematologic (10.8%), breast (8.1%), esophagus (8.1%), renal (8.1%), and prostate (8.1%). Within the temporal bone, 73% had isolated EAC metastasis. Median time to EAC metastasis from the diagnosis of primary malignancy was 18 months. Metastasis to the EAC was the first presentation of malignancy in 21.6% of patients. Median time to death was 4.5 months. Symptoms included hearing loss (59.5%), otalgia (27.0%), otorrhagia (24.3%), facial paralysis (21.6%), otorrhea (16.2%), and aural fullness (13.5%). On imaging, bony erosion was present in 50% of cases. Treatment was primarily palliative with excision and radiation., Conclusions: EAC metastasis has a distinct presentation from other temporal bone subsites. Early biopsy to establish a diagnosis and intervene is critical., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2024, Otology & Neurotology, Inc.)

This study aimed to compare total endoscopic ear surgery (TEES) and microscopic postauricular canal-wall-down tympanomastoidectomy (CWD) in cholesteatoma surgery in our clinic. This study included 59 patients, of whom 30 and 29 were operated on with CWD in 2016-2018 and TEES in 2019-2021, respectively and compared regarding intraoperative findings, hearing outcomes, long-term outcomes, and recidivism rates between groups. This study excluded patients in stage IV according to the European Academy of Otology and Neurotology/Japan Otological Society Staging System on Middle Ear Cholesteatoma, aged < 18, with congenital cholesteatoma, who underwent revision surgery. Two patients in the TEES group had recidivism (6.9%), with recurrent disease observed in both patients and residual disease in none, whereas 3 patients in the CWD group had recidivism (10%), including recurrent disease in 2 and residual disease in 1 patient. Tympanic membrane perforation occurred in 2 (6.9%) and 1 (3.3%) patients in the TEES and CWD groups, respectively. The 2 groups revealed no significant difference in terms of recidivism and perforation rates (P=1.000, P = .612). The CWD group had a longer mean operation time (225.54 ± 47.86 minutes) than the TEES group (160.55 ± 24.98 minutes) (P < .001). The 2 groups demonstrated no significant difference regarding pre- and postoperative air-bone gap (ABG) and ABG gain (P = .105, P=.329, P=.82, respectively). Total endoscopic ear surgery provides similar results in terms of hearing, recidivism, and long-term outcomes with the microscopic CWD approach. However, the CWD approach is still important, especially in patients in advanced stages.

This article represents the first reported case in the external auditory canal of epithelioid fibrous histiocytoma (EFH), a rare benign cutaneous epithelioid neoplasm. Immunostaining revealed uncommon negative staining for anaplastic lymphoma kinase (ALK) expression. This case and literature review outline the diagnostic strategy for this highly unusual neoplasm. Laryngoscope, 134:3371-3373, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Transcanal endoscopic ear surgery is a minimally invasive procedure that allows a clear visualization of the middle ear. Recently, indications for endoscopic surgery have been expanding. We performed combined underwater endoscopic and microscopic surgery for external auditory canal cholesteatoma, the computer tomography of which indicates the possibility of cholesteatoma not only in the canal wall but also in the mastoid. The 30° endoscope and underwater technique makes the surgical view clear, and we could remove the cholesteatoma without canalplasty. To the best of our knowledge, no case of external auditory canal cholesteatoma treated with underwater endoscopic and microscopic surgery has been previously reported. This case indicates that the procedure could be a good indication for external auditory canal cholesteatoma., Competing Interests: Declaration of Conflicting InterestsThe author(s) declare no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Melanoma of the external auditory canal (EAC) is particularly rare and poorly understood, with limited available data on management and survival. This systematic review aims to analyze existing data and provide insights into the management and prognosis the beginning of EAC melanoma. It is conducted using Pubmed and Scopus databases from the beginning to July 2023 and it follows the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) 2020 guidelines. Searches are performed using the search string "(melanoma) AND (external auditory canal)"., The review includes a total of 30 patients diagnosed with EAC melanoma, supplemented by an additional case from the authors' clinical experience. The role of Breslow thickness as a determining factor for the choice of surgery remains inconclusive due to limited available data. Sentinel lymph node biopsy and adjuvant therapy are sparingly employed, indicating the need for standardized guidelines. Patients in the study demonstrate a 50% overall survival rate at 5 years., EAC Melanoma is a rare and aggressive malignancy with limited therapeutic guidelines. Surgical interventions, including wide local excision and lateral temporal bone resection, are the primary treatment options for patients without distant metastases., (Copyright © 2024 Società Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of Pathology.)

Thrombosis is a characteristic symptom of coronavirus disease 2019 (COVID-19). Here, we present a case of external ear stenosis caused by arterial thrombosis after COVID-19 infection. To the best of our knowledge, this is the first report of external ear stenosis related to COVID-19. A 62-year-old man presented with left hearing loss. The patient had a history of hospitalization for COVID-19 treatment 11 months prior to visiting our hospital. He had been experiencing ear fullness and tinnitus after COVID-19 treatment. Physical examination revealed severe left external ear canal stenosis with a subcutaneous mass. Surgical removal of the subcutaneous mass was performed. Histopathological analysis revealed that a subcutaneous thrombosis caused the external ear canal stenosis. This case describes an unusual case of external ear canal stenosis after COVID-19. Clinical and pathological findings indicate that COVID-19 affected the external ear canal. In addition, histopathological results confirmed the formation of arterial thrombosis in the temporal bone region after COVID-19 treatment. This case shows the broad range of body sites that can be involved with thrombotic events with COVID including the subcutaneous tissue around the outer ear. This observation would be helpful in investigating or explaining the various otological symptoms of COVID-19., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Trichoblastoma（TB） is a rare germ cell skin adnexal tumor of the hair, and it is a rare follicular tumor of the skin that differentiates from the hair germ epithelium and is often regarded as a benign skin tumorHowever, it is poorly confined and has a local infiltrative growth pattern. tb occurs in the head and neck region, especially in the face, and presents clinically as a slow growing, well-defined and elevated nodule. TB is routinely treated surgically. Due to the lack of universally accepted treatment guidelines or protocols, the recurrence rate after surgery is high, which makes clinical cure more difficult. In this study, a 65-year-old female patient was found to have a swelling with recurrent rupture and pus flow from the right external auditory canal opening and the auricular cavity. After initial misdiagnosis as otitis externa, she was treated with conventional anti-infective therapy, but her symptoms did not resolve and gradually worsened before coming to our hospital. The condition presented in this case is relativelyrare,therepre,timely and accurate diagnosis and treatment are crucial for prognosis improvement of such diseases., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Background: Antiresorptive drugs are widely used to reduce bone mineral loss in patients with osteoporosis and to prevent skeletal-related events in patients with metastatic cancers and multiple myeloma (MM). Both the bisphosphonates (BP) and denosumab typically used in this indication were shown to be effective and relatively safe. Obviously, this medication could have some adverse effects; one of them is osteonecrosis of the external auditory canal. Only sporadic cases of external auditory canal osteonecrosis have been published yet. Here, we provide a case of denosumab-related osteonecrosis of the external auditory canal successfully treated surgically in the early stage of the disease. Case report: A 68-year-old patient with breast cancer underwent comprehensive oncological treatment, including denosumab administration. She was diagnosed with osteonecroses in the jaw and ear canal. The necrotic bones in both regions were resected with primary wound closure. Both affected sites healed well with no signs of necrosis recurrence. Conclusions: Osteonecrosis of the external auditory canal is a rare but probably underdiagnosed complication of antiresorptive medication. It has a negative impact on patient quality of life if left untreated. Early surgical treatment appears to be effective. The authors highlight several similarities with medication-related osteonecrosis of the jaw. Therefore, an analogous disease staging and treatment rationale is suggested., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Squamous cell carcinoma is the most common pathological type of external and middle ear malignancy. Squamous cell carcinoma of the external auditory canal and middle ear is closely associated with long-term chronic inflammatory irritation. The patient was admitted with a major complaint of a "stuffy feeling in the left ear for more than three months." The results of the otoendoscopic examination were as follows: The left external auditory canal was filled with a granuloma-shaped mass with an unsmooth surface that showed obvious oozing of blood. The tympanic membrane was not visible. After the completion of the relevant examinations, radiofrequency resection of the external auditory canal mass was performed under otoendoscopy and the wound healed well with postoperative dressing changes. Pathological results revealed a well-differentiated keratinizing cutaneous squamous cell carcinoma and there was no recurrence throughout the next 5 years after the operation. Proper preoperative evaluation and the correct selection of surgical approaches can be of great importance. The primary complication of ear squamous cell carcinoma was local recurrence with a low incidence of metastasis. Early and complete resection was determined to be the optimal course of treatment., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: To analyse prognostic factors and survival outcomes of malignant tumors of the external auditory canal, to investigate the role of regional surgery, and adjuvant radiotherapy in early stages and to investigate the role of surgery in operable T4 stage., Setting: A retrospective analysis was conducted on all patients prospectively included in the national database of the French Expertize Network for Rare ENT Cancers (REFCOR) from January 2000 to December 2016., Participants: 103 patients from 19 reference centers were included. A propensity score matching analysis was applied to enable comparisons between treatments., Main Outcomes and Measures: Event-free survival, overall survival and factors of poor prognosis of the cohort were described. The interest of local and regional surgery and postoperative radiotherapy were evaluated., Results: The factors of poor prognosis on event-free survival were immunosuppression (p = 0.002), Karnofsky status less than 90% (p = 0.02), body mass index less than 19 Kg / m2 (p = 0.0009), peripheric facial palsy (p = 0.0016), and positive margin (p = 0.0006). In early stages, locoregional surgery was associated with an increase in event-free survival (p = 0.003, HR = 0.21) versus local surgery alone, while postoperative radiotherapy was not associated with an increase in event-free survival (p = 0.86, HR = 0.91) or overall (p = 0.86, HR = 0.91). In locally advanced stages, locoregional surgery followed by radiotherapy was associated with an increase in event-free survival (p = 0.03, HR = 0.39) and overall (p = 0.02, HR = 0.34) versus chemoradiotherapy alone., Conclusion and Relevance: Regional surgery is recommended for early stages of cancers of the external auditory canal. In operable cases, locoregional surgery followed by radiotherapy is recommended., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Cartilaginous choristoma is a rare benign lesion of the external auditory canal, generally found incidentally on physical exam in an asymptomatic patient. Our patient had the largest cartilaginous choristomas described in the literature to date. Additionally, this reviews the nomenclature and differential diagnoses of masses within the external auditory canal., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: Hemangioma is a common soft tissue tumor that can be categorized mainly into cavernous, capillary, and venous subtypes. It usually develops in the head and neck and rarely in the external auditory canal (EAC)., Methods: A systematic review of patients with capillary hemangioma of the EAC was performed. A computerized systematic search was conducted in PubMed, Scopus, and Web of Science to extract relevant studies for a qualitative review., Results: A total of 38 patients were included in the systematic review, and half of them were men. Hearing loss was the most common presenting feature (n = 19; 50%), followed by aural fullness (n = 15; 39.4%). Cavernous hemangioma was the predominant type (n = 22, 57.8%). Endaural and transcanal surgical approaches were the most frequent, with ten (26.3%) and nine (23.6%) cases, respectively. Most of the patients (n = 31; 81.5%) had no recurrence., Conclusions: Hemangioma of the EAC may not be uncommon as healthcare professionals may expect. Hearing loss, aural fullness, and tinnitus are possible symptoms, but many patients remain asymptomatic. Surgical excision is the main effective management option, and complete resolution is the most prevalent outcome. Computed tomography assists in the diagnosis, but histopathological examination after resection is mandatory for the definitive diagnosis., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

A 12-year-old male neutered European Shorthair cat was presented for pruritus in the right ear region, bleeding from ear canal and a suspected polyp-like mass in its lumen.After the diagnostic imaging a biopsy of the mass was taken and submitted for histopathological evaluation. Histopathologic examination led to the diagnosis of low grade mast cell tumor. The subsequent staging examinations included ultrasonography of the liver and spleen as well as a complete blood count. Total ear canal ablation was performed on the same day, and the removed ear canal was again submitted for histopathologic evaluation of the surgical margins. The excision incision margins were free from infiltrating tumor cells. The cat was euthanised 14 months after the surgery. It is unknown whether the reasons for this were associated to metastatic spread of the initial mast cell tumor.A mast cell tumor in the ear canal is an unusual and rare finding, however it should be included in the list of differential diagnoses for ear canal tumors., Competing Interests: Die Autoren bestätigen, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

The expression of EGFR and p16 in the external auditory canal squamous cell carcinoma (EACSCC) and their impacts on oncological outcomes were not well studied. Seventeen-one consecutive patients who were treated for EACSCC at Kobe University Hospital from 1995 to 2018 were enrolled in this study. The expression of EGFR, and p16 were evaluated and their impacts on oncological outcomes were statistically analyzed. Positive expression of EGFR was observed in 62 patients (87%). Strong positive expression of p16 were observed in 18 patients (32.4%), and weakly positive expression in 30 patients (42.3%), respectively. While the number of the patients with negative EGFR expression were limited, all the surgically treated patients with negative EGFR expression have been alive without disease. In the patients with T3 & T4a EACSCC, prognosis of the patients with positive p16 expression EACSCC tended to be better than those with negative p16 expression. These results suggest the clinical significance of EGFR and p16 expressions in the patients with advanced EACSCC to predict oncological outcomes.

Melanocytic nevus occurring in the external auditory canal (EAC) is uncommon. Therefore, in the absence of sufficient clinical experience, this disease may be challenging to be suspected even with a physical examination. Herein, clinical and demographic features of intradermal nevus in the EAC were investigated. Patients with an intradermal nevus on histopathological examination (n = 15; 2 men and 13 women; P = .005) were included from a pool of patients who underwent surgical resection for EAC tumors between November 2011 and March 2022. Data were retrospectively collected on their sex, appearance of the lesions, surgical method, and outcomes. Nine patients had a dome shape and 6 patients had a papillomatous shape. Ten patients had dark colors and 5 patients had pale colors. All pale colors appeared only in the dome-shaped nevus, and only dark colored papillomatous nevi were observed. A significant difference was noted in terms of the frequency of occurrence by colors (dark or pale) according to shape (dome or papillomatous) (P = .044). No patient showed recurrence or EAC stenosis after circumferential excision. In women, a dome-shaped or papillomatous mass with dark color in the EAC may likely be predicted as the nevus. However, it can also occur in men, and even if it is a pale color, in the case of a dome shape, taking the nevus into account is important. Moreover, successful treatment outcomes were achieved through circumferential excision., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Aims: Adenoid cystic carcinoma (ACC) grows slowly and is characterised by potential recurrence and metastasis to distant organs. This study aimed to evaluate the risk factors for locoregional recurrence (LRR) and distant metastasis in patients with ACC of the external auditory canal (EAC)., Materials and Methods: Demographic, pathological, therapeutic and survival data of 143 patients with EAC ACC were reviewed in this study. Univariate and multivariate Cox proportional hazard regression analyses were carried out to determine the risk factors for LRR and distant metastasis. Factors associated with overall survival after LRR and distant metastasis were also analysed., Results: During a median follow-up of 49 months, 31 of 143 patients were observed with LRR and 34 developed distant metastasis. Bone invasion and histological subtype were independent risk factors for locoregional recurrence-free survival. T stage and LRR were independent risk factors for distant metastasis-free survival. Salvage surgery and adjuvant radiotherapy or chemoradiotherapy for LRR resulted in better survival, whereas extrapulmonary metastasis and LRR were associated with a higher risk of poor survival after distant metastasis., Conclusion: Patients with distant metastases, especially those with LRR, are at significant risk of poor prognosis. Our findings emphasise the importance of long-term regular follow-up and recommend surgical intervention with radiotherapy for recurrent EAC ACC., (Copyright © 2023. Published by Elsevier Ltd.)

Objective: This study aimed to investigate the significance of parotid gland invasion in predicting distant metastasis of adenoid cystic carcinoma in the external auditory canal., Study Design: Single-institution retrospective cohort study., Methods: A retrospective review of patients with adenoid cystic carcinoma of the external auditory canal who underwent surgery was performed. Information on patient demographics, parotid gland invasion, tumor stage, perineural invasion, lymphovascular invasion, and follow-up data were collected and analyzed., Results: One hundred twenty-nine patients were identified for review. Parotid gland invasion was noted in 45 patients (34.9%). Parotid gland invasion was significantly associated with tumor stage, perineural invasion, distant metastasis, and postoperative adjuvant therapy. Distant metastasis was noted in 30 patients (23.3%). Multivariate Cox proportional hazards analysis identified parotid gland invasion as an independent risk factor for predicting distant metastasis. The 5-year distant metastasis-free survival rate was 83.6% for patients without parotid gland invasion and 61.8% for patients with parotid gland invasion (p = 0.010)., Conclusions: The parotid gland invasion rate is relatively high in adenoid cystic carcinoma of the external auditory canal and is significantly related to tumor stage. Parotid gland invasion is associated with worse distant metastasis-free survival., Level of Evidence: 4 Laryngoscope, 134:419-425, 2024., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

Hyperpneumatization of the temporal bone (HPTB) is a rare finding, and its clinical impact is poorly described in literature. We report the case of a 34-year-old woman with no previous otologic condition, complaining of paroxysmal vertigo and right pulsatile tinnitus shortly after a first scuba diving session. Clinical neurotologic assessment found a right vestibular impairment. Cranial tomodensitometry showed a bilateral hyperpneumatization of the petrosal air cell system. Brain magnetic resonance imaging to rule out any retrocochlear pathology was normal, although the cochleo-vestibular nerve (CVN) appeared to be over lengthened in a narrowed internal auditory canal (IAC), especially on the right-hand side. Pain was alleviated by administration of a low-dose anti-epileptic drug. The clinical, neurological, and radiological findings evoked a right vestibulopathy generated by a limited compression of the CVN; HPTB appeared to contribute to the narrowing of the IAC, especially on the right-hand side. [ABSTRACT FROM AUTHOR]

Squamous cell carcinoma of the external auditory canal (EACSCC) is an extraordinarily rare and aggressive malignant disease. Establishment of EACSCC cell line with robust molecular characteristics is essential for the basic and translational research of EACSCC. Here, we show the newly established EACSCC cell line SCEACono2, derived from a patient with well-to-moderately differentiated EACSCC. We analyzed histologic and genetic features of SCEACono2 hiring multiple experiments, including next-generation sequencing (NGS). Immunocytochemical staining of SCEACono2 showed positivity of p53 and SCC1/2. Furthermore, SCEACono2 exhibited a unique characteristic that cytokeratin, vimentin as well as cancer stem cell markers (CD44, CD133, ALP and Oct3/4) were positive. SCEACono2 had an ability to form tumors at the temporal lesion xenograft nude mice model. NGS revealed that SCEACono2 harbored the somatic mutations of TP53 (p.G245S) and NOTCH1 (p.A465T). RNA-seq and downstream bioinformatics analysis revealed significant enrichment of genes involved in inflammation and cell adhesion in SCEACono2 compared to SCC-9 and HSC-4. STR profiling indicated no evidence of cross-contamination. In conclusion, SCEACono2 could serves as a promising and robust research resource of EACSCC in vitro and in vivo., (© 2023. The Author(s).)

Malignant tumors of the external auditory canal are rare tumors and very often diagnosed at an advanced stage due to non-specific symptoms. The best treatment is the radical surgery, eventually followed by radiotherapy. A multidisciplinary team is essential to realize an optimal management. Prognosis remains unfavorable for advanced tumors., Competing Interests: Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.

This article provides a historical overview of disorders of the external ear, focusing on advances in technology, pharmacology, and education that have been beneficial. While the diagnosis and treatment of various conditions involving the external ear canal and auricle continue to evolve, it remains to be seen how the latest challenges will be met., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Squamous cell carcinoma of the external auditory canal (EACSCC) is a rare condition. However, a standard treatment has not yet been established. We retrospectively evaluated the efficacy, adverse events, and feasibility of TPF-CCRT (concomitant chemoradiotherapy with docetaxel, cisplatin, and 5-fluorouracil) in patients with advanced EACSCC., Methods: Thirty-five consecutive patients with advanced EACSCC (T3, T4) initially treated with TPF-CCRT at Kobe University Hospital were included. T4 diseases with invasion of the brain, internal carotid artery, or internal jugular vein were classified as T4b, and those without these features were classified as T4a., Results: Five-year overall survival rates for T3 and T4 were 100% and 64.2%, respectively. A significant difference was observed between T4a and T4b (82.4% vs. 30%, p = 0.007). Five-year progression-free survival rates of T3, T4a, and T4b were 100%, 68%, and 20% (p = 0.022), respectively., Conclusions: TPF-CCRT should be considered as a plausible treatment option for advanced EACSCC., (© 2023 Wiley Periodicals LLC.)

Necrotizing otitis externa is a progressive infectious disease involving the external auditory canal and even the skull base, which can lead to serious complications and even death if not treated in time. In this paper, the latest advances in etiology, pathogenesis, clinical manifestations, diagnosis and treatment were reviewed based on previous literature, providing reference for clinical diagnosis, treatment and future research., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

The incidence of sebaceous carcinoma (SC) in the outer one-third of the external auditory canal is considered extremely rare, and only eight case reports have been published. We present a case of a male patient in his late 70s known case of hypertension on indapamide. His medical history included a postspinal tumour that had been treated with surgery and radiation more than 40 years ago and current complaints of right ear pain and purulent discharge. A right ear soft granular tissue mass was found. Complete debulking of the right ear mass was done in conjunction with middle ear exploration, moderately differentiated SC diagnosis was made based on the histopathological analysis. The patient was free of recurrence but then died of an unrelated pulmonary infection. SC should be suspected in elderly patients who present with long-term complaints of a mass with or without otalgia, and these patients should be examined for the presence of such tumours especially if they report a history of radiation. Additionally, more research is warranted to investigate the association of diuretics with SC., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Facial nerve schwannomas are rare, benign, slow-growing tumors that can occur in any segment of the facial nerve, although 71% of cases are intratemporal. Surgical resection can lead to facial nerve injury. Facial function recovery after reanimation is usually not better than House-Brackmann (HB) grade III. Thus, for cases of intratemporal facial nerve schwannomas (IFNSs) with favorable facial function (HB grade I or II), observation by periodic magnetic resonance imaging is the mainstay of management. Here, we present a case of a large IFNS with normal facial function in which the mass fully occluded the external auditory canal. The occlusion caused squamous debris to accumulate, potentially leading to cholesteatoma. Faced with this therapeutic dilemma, we chose surgical resection with the patient's informed consent. Stripping surgery was achieved with normal postoperative facial function. There was no postoperative facial paralysis or recurrence at 2-year follow-up. We describe the experience of diagnosis and treatment process for this case, and discuss the possibility of total resection of the tumor with preserving the integrity of facial nerve.

Angiolymphoid hyperplasia (AH) was first described by Wells and Whimster in 1969 as a benign vasoproliferative pathology with a varied infiltrate of eosinophils, lymphocytes, and plasmatic cells. Clinical presentation has been described in the literature as small red-bluish nodules, less than 3 cm in diameter that can bleed in 25% of the cases and be pruritic and painful in 37% and 20% of the cases, respectively. Particularly, AH can appear in the ear; nevertheless, other regions have been affected, including the scalp, lips, tongue, orbit, muscle, and bone. Most of these cases have occurred in adults with an unknown etiology; however, an inflammatory process has been associated in approximately 20% with eosinophilia. No malignancy has been reported.

Purpose: Although adenoid cystic carcinoma (ACC) of the external auditory canal (EAC) has a different pathophysiology from squamous cell carcinoma, the same staging system is used. The aim of this study was to propose a refined staging system, which is more suitable for ACC of the EAC., Methods: A total of 25 patients who were diagnosed with ACC of the EAC were reviewed. The modified Pittsburgh staging system (mPSS) that is universally used for temporal bone malignancy was refined for ACC (rPSS). The limited (< 0.5 cm) lateral soft tissue involvement was classified as T1 and extensive (≥ 0.5 cm) lateral soft tissue involvement as T2. The disease-free survival rate (DFSR) was assessed in the patients who underwent surgical treatment according to two staging systems; mPSS and rPSS., Results: When staging using mPSS, most patients (96.0%, n = 24) were classified as T4. However, when rPSS was used, T1, T2, T3, and T4 stage occupied 36.0% (n = 9), 40.0% (n = 10), 12.0% (n = 3), 12.0% (n = 3), respectively. There was no difference in DFSR according to the T stage using mPSS (p = 0.466). However, when rPSS was used, the DFSR showed significant correlation with the T stage (p = 0.032)., Conclusions: Clinical T stage of mPSS was not sufficient to predict survival rate in ACC of the EAC, and we propose that the information on the lateral soft tissue involvement needs to be added to the existing staging system., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

In this case report, a 68-year-old woman, with known insulin-dependent diabetes and myelomatosis, presented with ear pain in her right ear. Otomicroscopy showed exposed bone in the external auditory canal. The patient was examined with wound swab, biopsies, MRI and PET-CT scans to rule out necrotizing external otitis, cholesteatoma and malignancy. Later, the patient's bisphosphonate treatment for myelomatosis was suspected, because osteonecrosis of the external auditory canal is a rare side effect to this treatment. The bone lesion improved after local debridement and cessation of the bisphosphonate treatment.

Abstract: External ear melanomas are relatively rare and usually occur in the regions of helix and ear lobes. Rarer still are primary melanomas of the external auditory canal. We report findings of melanoma of the external auditory canal on 68 Ga-FAPI PET/CT in a 56-year-old man who presented with sharp pain in the external auditory canal for 7 months., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Malignant neoplasms of the external auditory canal (EAC) are rare. No consensus on management has emerged., Objective: To determine possible risk factors influencing tumorgenesis and prognosis of EAC carcinoma., Materials and Methods: 108 patients (87 men/21 women) with an average age of 74 ± 13.8 years were recruited from 2005 to 2019 at Department of Otorhinolaryngology, Head and Neck Surgery Heidelberg. The follow-up interval was 43.62 ± 55.39 months. Partial and (sub)total ablative otis, supplementary surgery (petrosectomy, parotidectomy, neck dissection, mastoidectomy) and adjuvant radio(chemo)therapy belonged to treatment options. TNM status was determined at time of diagnosis using the AJCC staging system., Results: 63.9% of patients underwent a total ablative otis. Tumor recurrence was seen in 24.1%. The 1-year survival rate was 87%, the 5-year survival rate was 52%, the mean overall survival (OS) was 3.82 ± 4.6 years. Male EAC carcinoma patients had a better OS (p < 0.001), PFS (p < 0.001) and DSS (p = 0.02) than females. T1 patients had a better OS (p = 0.01), PFS (p = 0.01) and DSS (p < 0.001) than T4 patients. Lymph node but not distant metastasis, tumor grading, perineural, venous and lymphatic invasion, histology, age and tumor localization influenced the OS in EAC carcinoma patients (p = 0.04). The more radical the ablative otis, the worse the OS (p = 0.002), PFS (p = 0.02) and DSS (p < 0.001). Radio(chemo)therapy did not improve the OS., Conclusions: EAC carcinoma are difficult to treat and benefit from early diagnosis so that a radical combined treatment approach does not need to be used., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: The aims of our study were to investigate the clinical and audiometric outcome of the surgical treatment of postinflammatory medial meatal fibrosis (PIMMF) and to review the histopathologic changes in the specimens of the fibrotic plug, in order to try to shed light on the pathogenesis of the disease., Materials and Methods: The clinical records and the histopathologic specimens of all patients who underwent tympanomeatoplasty for PIMMF at the ENT Clinic of the University of Erlangen between 2006 and 2020 were evaluated retrospectively., Results: Thirty-four patients (41 primary surgical procedures) made up our study cohort. Of this, 28 cases were managed by means of meatoplasty and 13 cases with tympanomeatoplasty. The mean preoperative air-bone gap (ABG) was 27.8 dB (10-44 dB). Postoperative ABG was significantly improved compared to preoperative values at both short- and long-term follow-ups ( P < .001 for both). No significant difference was noted between short-term and long-term ABG ( P = .240). An ABG ≤20 dB was achieved in 65.8% of patients (short term) and 50% (long term). The overall rate of revision surgery for restenosis was 29.3% (12/41). Histopathologic reevaluation of the fibrotic plugs revealed a mosaic of patterns with frequent occurrence of secondary cholesteatoma-like lesions and keloid-like tissue changes. Lichenoid submucosal inflammation and increased ectopic ceruminous gland lobules were seen less frequently., Discussion: The moderate long-term outcome of surgical management and the identification of histologic changes with therapeutic implications might pave the way for alternative nonsurgical treatment options.

Objectives/hypothesis: Mycosis Fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma. Disease involvement of specific locations may be more significant than simply the symptoms associated with that site; it is possible that involvement of certain sites could be associated with poor prognosis. We aimed to evaluate the outcomes of patients with MF with documented involvement of the EAC and external ear., Study Design: Retrospective analysis., Methods: We retrospectively reviewed 40 patients with MF that were treated by otologists between 2012 and 2021., Results: We report the largest series of patients with MF involving the external ear and EAC. Of the 40 patients included in this study, 17 presented with Mycosis Fungoides in the otologic region (MFO). Of these 17 MFO patients, 2/17 had involvement of the external ear only, 3/17 of the EAC only, 11/17 of both the external ear and EAC, and 1/17 of the periauricular skin. Of note, 11/14 (79%) patients presenting with EAC disease died compared to11/26 (42%) of patients without involvement. In addition, eight of the 13 (62%) patients with external ear involvement died compared to 14/27 (52%) of patients without involvement. Ear canal involvement was associated with a statistically significant shorter overall survival duration in patients with MF (p = 0.03). Furthermore, disease in the EAC was found to have a hazard ratio value of 2.565 (CI 1.102-5.970)., Conclusions: Involvement of the EAC by MF portends a poor prognosis. This finding highlights the need for a more in-depth otologic evaluation of patients with MF., Level of Evidence: 4 Laryngoscope, 133:1486-1491, 2023., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

A 74-year-old Japanese woman with a 1-year history of right preauricular pain and a 2-month history of bleeding from the right ear was admitted to our department. Tumor was observed in the anterior wall in the right external auditory canal. Bony swelling of the right preauricular area was palpated. Computed tomography revealed an ill-defined, osteogenic tumor around the mandibular condyle with a destructive bony lesion involving the temporal bone. Magnetic resonance imaging revealed a 2.0 × 1.5 × 1.3-cm solid tumor around the mandibular condyle, exhibiting a low-intensity signal on T1-weighted imaging and an isointense central area surrounded by low-signal intensity on T2-weighted imaging. Histological examination of biopsy specimens revealed diffuse-type tenosynovial giant cell tumor (D-TGCT). After the feeding arteries for the tumor were embolized, the patient underwent surgery with combined temporal craniotomy and mandibular condylectomy. The soft and cystic tumor with calcification located in the extradural space was totally resected along with the mandibular condyle. No facial paralysis or recurrence was evident as of 6 months postoperatively. To date, only 23 cases of D-TGCT arising in the temporomandibular joint (TMJ) with ear involvement have been reported since 2011. We report successful resection of a rare case of D-TGCT arising in the TMJ.

Objective: To characterize cell line CAE606 derived from a squamous cell carcinoma (SCC) of the external auditory canal (EAC) and to show its usefulness as a model for testing candidate therapeutic agents., Study Design: Preclinical translational research., Setting: Biomedical research institute., Methods: The cell line was initiated from a moderately differentiated T2N0M0 EAC SCC. We studied its histologic and genetic features as well as growth and invasion parameters. Sensitivity to cell CDK4/6 cell cycle inhibitor palbociclib was analyzed., Results: CAE606 cells expressed heavy molecular weight cytokeratin, p63, and vimentin. The population doubling time was 25.8 hours, and the cells showed fast collective cell migration in a wound-healing assay. Short tandem repeat analysis confirmed it to be derived from the primary tumor of the patient. Next-generation sequencing revealed alterations in cell cycle regulation genes, including inactivating mutations in CDKN2A and TP53 and high-level amplification of CCND1 and EGFR. CAE606 showed a strong decrease of phospo-Rb expression upon exposure to the CDK4/6 inhibitor palbociclib, causing significant growth inhibition with an IC 50 of 0.46 µM., Conclusion: This is the first report of a stable EAC SCC cell line. Its genetic features make it a useful tool for preclinical testing of new therapeutic agents for EAC SCC, particularly those targeting cell cycle regulation in combination with radio- and chemotherapy or other specific signaling pathway inhibitors., (© 2022 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Solitary fibrous tumors (SFTs) originating from the external auditory canal are uncommon; only few cases have been reported in the literature. In this article, we report a case of a 35-year-old man who presented with a 6-month history of a gradual swelling in the entrance of the left external auditory meatus associated with hearing loss. The tumor was surgically removed, and histological examination showed spindle-cell proliferation with a collagenous stroma. Immunohistochemically, the tumor cells were positive for CD34 confirming the diagnosis of an SFT. Although SFTs are benign, complications such as relapses and metastasis after excision were reported. Thus, a careful and long follow-up is recommended.

Schwannomas in the middle ear and external auditory canal are exceedingly rare. The facial nerve, chorda tympani nerve, and Jacobson's nerve have rarely been reported as the origins of primary schwannomas in the middle ear cavity. We experienced a case of carotid sympathetic plexus (CSP) schwannoma that arose from the carotid canal and extended into the middle ear and external auditory canal. The tumor presented bone erosion of the carotid canal, and it adhered tightly to the internal carotid artery. This report represents the first documented case of a CSP schwannoma, which involved the middle ear and external auditory canal.

Background: Adenoid cystic carcinoma of the external auditory canal is a rare primary malignancy, and surgery is the primary management strategy. This study aims to optimize management strategies and improve prognosis of adenoid cystic carcinoma of the external auditory canal., Methods: Seventeen patients with adenoid cystic carcinoma of external auditory canal who had been admitted to a single institution from January 2008 to March 2019 were recruited and retrospectively reviewed. Among patients with T1 tumors, 2 underwent local external auditory canal resection, 1 received lateral temporal bone resection+superficial parotidectomy. Among patients with T2 tumors, all 5 patients underwent lateral temporal bone resection+superficial parotidectomy. Among patients with T3 tumors, 3 underwent subtotal temporal bone resection+superficial parotidectomy, 2 underwent subtotal temporal bone resection+superficial parotidectomy+radiotherapy, and 1 underwent extended temporal bone resection+superficial parotidectomy+radiotherapy. Among patients with T4 tumors, 2 underwent subtotal temporal bone resection+superficial parotidectomy and 1 underwent extended temporal bone resection+total parotidectomy+radiotherapy., Results: The common manifestations included otalgia (82.4%), hearing loss (23.5%), external auditory canal mass (23.5%), otorrhea (17.6%), and aural fullness (5.9%). In the study, 5/17 (29.4%) patients had been misdiagnosed preoperatively, 5/17 (29.4%) patients revealed local recurrence, and 3/17 patients (17.6%) were identified with distant metastasis postoperatively. The 3- and 5-year overall survival rates were 88.2% and 82.3%, respectively. There was no significant difference in overall survival (P=.746) and disease-free survival (P=.933) between patients receiving different surgical approaches. Three out of 17 patients (17.6%) died of T2, T3, and T4 diseases, respectively., Conclusion: Otalgia is the most common manifestation of adenoid cystic carcinoma of the external auditory canal, and misdiagnosis is frequently encountered. Surgery is the preferred therapy, and local resection is associated with relapse, lateral temporal bone resection is strongly recommended in patients with early-stage tumor. Regular follow-up should be routinely conducted postoperatively to early identify local recurrence.

Introduction: External auditory canal cholesteatoma (EACC) is a rare disease, with an estimated incidence of approximately 1:1,000 adult and 1.6:1,000 pediatric otologic patients. Systematic studies of chronic ear disease and taste alteration prior to surgery are rare; in fact, there are no such studies for EACCs. Therefore, we describe chorda tympani nerve (CTN) dysfunction and the related clinical consequences in EACC patients., Methods/study Design: Between 1992 and 2021, we retrospectively analyzed the symptoms, signs, and radiological and intraoperative descriptions of CTN involvement in 73 patients. Liquid taste tests and, since 2009, Taste StripsTM as well as an olfactory screening test (Smell DiskettesTM) have been performed for all symptomatic patients and, when feasible, all other EACC patients., Results: Ten of 73 patients complained subjectively of dysfunction, and 8 showed abnormal taste test results. Four patients complained of olfactory dysfunction (3 cases with pathological taste tests). Gustatory dysfunction was most frequent in radiogenic EACC cases (n = 4), followed by postoperative EACC (n = 3). Two postoperative patients were asymptomatic despite abnormal test results. Rarely, patients with idiopathic (n = 2) and posttraumatic (n = 1) EACC showed acute taste dysfunction that was confirmed in each with abnormal test results., Discussion/conclusion: CTN dysfunction often developed asymptomatically in chronic ears, except for idiopathic and posttraumatic EACCs under previous healthy middle ear conditions. Taste disturbance is not a cardinal symptom of EACC, but objective testing suggests that up to one out of 10 EACC patients with advanced disease may experience regional gustatory dysfunction prior to surgery. Especially in context of a new and acute presentation, regional taste dysfunction may alert the clinician of potential progressive EACC invasion and danger to the facial nerve., (© 2023 S. Karger AG, Basel.)

Objective: To compare the incidence and the histopathological aspect of spontaneous and two induced Mongolian gerbils' models of cholesteatoma: External Auditory Canal (EAC) obliteration model and the Auditory Tube (AT) cauterization model., Methods: Fifty-four ears of 27 animals were divided into EAC obliteration, AT cauterization, and control groups and histologically assessed for cholesteatoma incidence and classification at intervals of 2, 4, 8, and 16 weeks., Results: Cholesteatoma was diagnosed in 30 of the 53 ears evaluated with a significantly higher incidence in groups that received some type of intervention (p<0.0001). It was not possible to histologically distinguish cholesteatomas of the same stage between the study groups., Conclusion: Although we observed a significant increase in cholesteatoma incidence with the two methods used when compared to the control group, all developed cholesteatomas were apparently identical from a histological point of view., (Copyright © 2021 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.)

Syringocystadenoma papilliferum is a rare hamartomatous adnexal tumor and appears mainly in the head and neck region. Rarely, such tumors may appear in the external auditory canal. There are only 14 described cases of the syringocystadenoma papilliferum in the external auditory canal in the Medline database. In this study, we present the first reported case of syringocystadenoma papilliferum arising in the modified radical mastoidectomy and occurring 12 years after ear surgery. A 26-year-old man was admitted to our clinic with complaints of discharge from the left ear, ear fullness, and hearing loss. These symptoms had been worsening for 3 years. Twelve years previously, he had undergone modified radical mastoidectomy by Bondy due to a cholesteatoma. Otoscopic examinations revealed otorrhea and a reddish mass measuring 1 × 0.8 cm arising from the tegmen tympanum and fundus of the mastoid cavity. A surgical biopsy was conducted with local anesthesia in the medical outpatient clinic, and it revealed syringocystadenoma papilliferum. A tumor resection was performed with a retroauricular approach. The bone attachment of the tumor was drilled out, and the ear cavity was covered with a mashed split skin graft. The ear cavity healed, and no tumor recurrence was observed during a 9-month follow-up period. Our case and literature analysis of previously described cases led to the conclusion that cases of syringocystadenoma papilliferum of the external auditory canal may be associated with different medical histories, age distributions, and origins than other syringocystadenoma papilliferums of the head and neck region. Tubular adenoma may occur together with syringocystadenoma papilliferum in 20% of cases. Malignant transformation of syringocystadenoma papilliferum may occur but is extremely rare. Computed tomography imaging may predict malignancy of the tumor. Tumors specific to the external auditory canal may occur in the modified radical mastoidectomy after middle ear surgery.

Abstract: Microsecretory adenocarcinoma (MSA) of the salivary gland is a new entity recently added to the World Health Organization Classification of Head and Neck Tumors. This tumor is characterized by a recurrent MEF2C-SS18 translocation. We present a nodular tumor confined to the dermis of the ear canal of a 44-year-old patient, which demonstrated classic histopathologic features and molecular alteration of MSA. Specifically, the tumor was composed of numerous tubules and microcysts filled with abundant basophilic mucinous secretion and associated with a fibromyxoid stroma. The tumor cells were diffusely positive for CK7 and SOX10 and variably positive for S100 and p63. Breakapart fluorescence in situ hybridization for SS18 confirmed rearrangement of this gene. Together, these findings support a primary cutaneous MSA, presumably arising from ceruminous glands of the ear canal. Based on current knowledge of its salivary gland counterpart, cutaneous MSA is expected to be locally invasive but unlikely to recur or metastasize on complete excision., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Epithelial migration has been associated with the self-cleansing mechanism of the ear. The rate and pattern of epithelial migration in healthy and pathological ears are reviewed., Methods: Two authors independently screened articles over one month using the following search terms: epithelial migration, epithelial, tympanic membrane, external auditory canal and mastoidectomy cavity., Results: Ten studies were included. The fastest rate of epithelial migration was observed in the external auditory canal, with a mean of 144.75 μm per day, whereas the slowest epithelial migration was seen in post-mastoidectomy cavities, with a rate of 20 μm per day. Epithelial migration was present in both studies involving post-mastoidectomy cavities., Conclusion: Epithelial migration is faster in healthy tympanic membrane than in pathological tympanic membrane. The rate of epithelial migration in the external auditory canal was higher in the pathological group than in the healthy group. Epithelial migration is present in post-mastoidectomy cavities.

Background: In humans, the acidic pH of the ear canal plays a protective role against infection and a change towards alkalinity of the external auditory canal (EAC) is a local factor in the progression of acute to chronic otitis externa (OE). The use of acidic preparations alone for treatment of OE without concurrent antibiotic use is well-documented in humans. In dogs, only one study has investigated the EAC pH in healthy dogs and in dogs with OE, and investigations to understand the role of EAC pH in the pathogenesis of canine OE are lacking., Hypothesis/objectives: To obtain physiological EAC pH values in beagle dogs. To develop a model of re-acidification of the EAC in dogs and to investigate how an acidic solution may accelerate the return to a physiological pH., Animals: Ten healthy beagle dogs in a laboratory setting., Materials and Methods: A model of re-acidification of the EAC was developed by instillation of a pH 10.1 phosphate-buffered saline (PBS) solution and the subsequent acidic effect of an ear cleaner containing lipacids was evaluated in this model., Results: Mean physiological EAC pH was 6.12 (± 0.36). EAC re-acidification took up to 9 h in this model. Mean pH values dropped immediately to 6.38 (± 0.27) on ears treated with an acidic ear cleaner. No abrupt drop was observed of the mean pH values for the control ears., Conclusion and Clinical Importance: This study confirms that physiological EAC pH in dogs is acidic. This model of re-acidification of the EAC pH allows investigations on acidic properties of topical ear products in healthy ears., (© 2022 The Authors. Veterinary Dermatology published by John Wiley & Sons Ltd on behalf of ESVD and ACVD.)