Factors Related to Cholesteatoma Formation in External Auditory Canal Osteomas and Treatment Algorithm.

Objectives: Osteomas in the external auditory canal (EAC) can lead to stenosis, and impair epithelium migration and self-cleaning capability, thereby trapping keratinized epithelium and triggering the development of cholesteatoma. Our study aims to identify the risk of cholesteatoma development in patients with osteoma and proposes a stepwise approach to managing patients with EAC osteoma. Methods: The maximum diameter of the osteoma was measured in axial and coronal views on high-resolution computed tomography (HRCT). We calculated the relative obstruction ratio caused by the osteoma in the axial and coronal views. Prior to surgery, otoscopy was employed to identify pedicle formation. The patients were categorized into 2 groups based on the presence of cholesteatoma. Results: We identified 43 patients diagnosed with EAC osteoma. A total of 9 (20.9%) patients with EAC osteomas developed cholesteatoma and the other 34 (79.1%) did not. The maximum diameter of osteomas with and without cholesteatoma was 12.67 ± 4.09 and 7.67 ± 3.27 mm, respectively (P < .001). In the group without cholesteatoma, 21 osteomas had pedicles while the other 13 did not. In the cholesteatoma group, 2 osteomas had pedicles and 7 did not (P = .037). No difference was observed in the relative obstruction ratio between these 2 groups. Conclusions: Our findings indicate that larger osteomas are more likely to develop cholesteatoma, while the formation of a pedicle may reduce the occurrence of cholesteatoma. In symptomatic patients, preoperative evaluation, including HRCT and otoscopy, is vital for assessing the extent of the osteoma and the potential coexistence of cholesteatoma. These factors are critical for preoperative consultations and surgical planning.

Sequential Impact of Diabetes Mellitus on Deep Neck Infections: Comparison of the Clinical Characteristics of Patients with and without Diabetes Mellitus.

BACKGROUND: Deep neck infections (DNIs) can compromise the airway and are associated with high morbidity and mortality rates. Diabetes mellitus (DM) is a metabolic disorder characterized by chronic hyperglycemia that is associated with several comorbidities. We compared the clinical characteristics of DNI patients with and without DM. METHODS: This study recorded the relevant clinical variables of 383 patients with DNIs between November 2016 and September 2022; of those patients, 147 (38.38%) had DM. The clinical factors between DNI patients with and without DM were assessed. RESULTS: Patients with DM were older (p < 0.001), had higher white blood cell counts (p = 0.029) and C-reactive protein levels (CRP, p < 0.001), had a greater number of deep neck spaces (p = 0.002) compared to patients without DM, and had longer hospital stays (p < 0.001). Klebsiella pneumoniae was cultured more frequently from patients with DM than those without DM (p = 0.002). A higher CRP level (OR = 1.0094, 95% CI: 1.0047-1.0142, p < 0.001) was a significant independent risk factor for DM patients with prolonged hospitalization. The lengths of hospital stays in patients with poorly controlled DM were longer than those with well-controlled DM (p = 0.027). CONCLUSIONS: DNI disease severity and outcomes were worse in patients with DM than those without DM. Antibiotics effective against Klebsiella pneumoniae should be used for DNI patients with DM. DNI patients with DM and high CRP levels had more prolonged hospitalizations. Appropriate blood glucose control is essential for DNI patients with DM.

Importance of age at 2nd implantation and interimplant interval to the outcome of bilateral prelingually deafened pediatric cochlear implantation.

BACKGROUND: In Taiwan, the number of cases of sequential bilateral pediatric cochlear implantation (CI) is increasing but data regarding its effectiveness and impact of the reimbursement policy are lacking. We examined the speech perception and quality of life (QOL) of bilateral prelingually deaf children who underwent sequential CI, considering the effects of age at the time of second implantation and interimplant interval. METHODS: We enrolled 124 Mandarin-speaking participants who underwent initial cochlear implant (CI1) in 2001-2019 and a second CI (CI2) in 2015-2020. Patients were followed up for ≥2 years and were categorized into groups based on age at the time of CI2 implantation (<3.5, 3.6-7, 7.1-10, 10.1-13, and 13.1-18 years) and interimplant interval (0.5-3, 3.1-5, 5.1-7, 7.1-10, and >10 years). We evaluated speech perception, device usage rates, and QOL using subjective questionnaires (Speech, Spatial, and Qualities of Hearing and Comprehension Cochlear Implant Questionnaire). RESULTS: Speech perception scores of CI2 were negatively correlated with ages at the time of CI1 and CI2 implantation and interimplant interval. Older age and a longer interimplant interval were associated with higher nonuse rates for CI2 and worse auditory performance and QOL. Among individuals aged >13 years with interimplant intervals >10 years, up to 44% did not use their second ear. Patients aged 7.1 to 10 years had better speech perception and higher questionnaire scores than those aged 10.1 to 13 and 13.1 to 18 years. Furthermore, patients aged 10.1 to 13 years had a lower rate of continuous CI2 usage compared to those aged 7.1 to 10 years. CONCLUSION: Timely implantation of CI2 is essential to achieve optimal outcomes, particularly among sequentially implanted patients with long-term deafness in the second ear and no improvement with hearing aids following CI1 implantation. For CI2 implantation, an upper limit of age of 10 years and interimplant interval of 7 years are essential to prevent suboptimal outcomes. These data can provide useful information to implant recipients, their families, and medical and audiological professionals, enabling a comprehensive understanding of the benefits and potential impacts of the timing of CI2 implantation.

A Machine Learning Approach to Assess Patients with Deep Neck Infection Progression to Descending Mediastinitis: Preliminary Results.

BACKGROUND: Deep neck infection (DNI) is a serious infectious disease, and descending mediastinitis is a fatal infection of the mediastinum. However, no study has applied artificial intelligence to assess progression to descending mediastinitis in DNI patients. Thus, we developed a model to assess the possible progression of DNI to descending mediastinitis. METHODS: Between August 2017 and December 2022, 380 patients with DNI were enrolled; 75% of patients (n = 285) were assigned to the training group for validation, whereas the remaining 25% (n = 95) were assigned to the test group to determine the accuracy. The patients' clinical and computed tomography (CT) parameters were analyzed via the k-nearest neighbor method. The predicted and actual progression of DNI patients to descending mediastinitis were compared. RESULTS: In the training and test groups, there was no statistical significance (all p > 0.05) noted at clinical variables (age, gender, chief complaint period, white blood cells, C-reactive protein, diabetes mellitus, and blood sugar), deep neck space (parapharyngeal, submandibular, retropharyngeal, and multiple spaces involved, ≥3), tracheostomy performance, imaging parameters (maximum diameter of abscess and nearest distance from abscess to level of sternum notch), or progression to mediastinitis. The model had a predictive accuracy of 82.11% (78/95 patients), with sensitivity and specificity of 41.67% and 87.95%, respectively. CONCLUSIONS: Our model can assess the progression of DNI to descending mediastinitis depending on clinical and imaging parameters. It can be used to identify DNI patients who will benefit from prompt treatment.

Assessment of the Elderly Adult Patients with Deep Neck Infection: A Retrospective Study.

Background: Deep neck infection (DNI) is a potentially lethal infectious disease affecting middle-aged adults and can compromise the airway. There are limited data on the prognosis and outcomes of elderly (aged > 65 years) DNI patients, who tend to be immunocompromised. This study analyzed the clinical characteristics of elderly and adult (aged 18-65 years) DNI patients. Methods: Between November 2016 and November 2022, 398 patients with DNIs, including 113 elderly patients, were admitted to our hospital and enrolled in this study. The relevant clinical variables were investigated and compared. Results: The elderly DNI patients had longer hospital stays (P < .001), higher C-reactive protein levels (P = .021), higher blood sugar levels (P = .012), and a higher likelihood of diabetes mellitus (P = .025) than the adult patients. The higher blood sugar level is an independent risk factor for elderly (odds ratio = 1.005, 95% confidence intervals 1.002-1.008, P < .001). Moreover, the rates of intubation to protect the airway (P = .005) and surgical incision and drainage (I&D; P = .010) were higher in the elderly group. However, there were no group differences in pathogen distributions. Conclusion: The elderly DNI patients in this study had a more severe disease course, and poorer prognosis than the adult patients, as well as higher rates of intubation and I&D. However, the pathogen distributions did not differ significantly between the groups. Prompt intervention and treatment are important for elderly DNI patients.

Assessment of Factors Associated With Long-Term Hospitalization in Patients With a Deep Neck Infection.

BACKGROUND: Deep neck infection (DNI) is a severe infectious disorder of deep neck spaces that can cause serious complications. Long-term hospitalization is when a patient spends more time in the hospital than originally expected for a disease. There are few studies assessing the risk factors associated with long-term hospitalization for a DNI. This study investigated the factors causing DNI patients to experience long-term hospitalization. METHODS: Long-term hospitalization is defined as a length of hospital stay exceeding 28 days (> 4 weeks) in this research. A total of 362 subjects with a DNI between October 2017 and November 2022 were recruited. Among these patients, 20 required long-term hospitalization. The relevant clinical variables were assessed. RESULTS: In a univariate analysis, C-reactive protein (odds ratio [OR] = 1.003, 95% CI: 1.000-1.007, P = .044), involvement of ≥3 deep neck spaces (OR = 2.836, 95% CI: 1.140-7.050, P = .024), and mediastinitis (OR = 8.102, 95% CI: 3.041-21.58, P < .001) were significant risk factors for long-term hospitalization in DNI patients. In a multivariate analysis, mediastinitis (OR = 6.018, 95% CI: 2.058-17.59, P = .001) was a significant independent risk factor for long-term hospitalization for a DNI. There were no significant differences in pathogens between the patients with and without long-term hospitalization (all P > .05). However, the rates of no growth of specific pathogens were significantly different between patients with and without long-term hospitalization, and those with long-term hospitalization had greater rates of growth of specific pathogens (P = .032). The rate of tracheostomy in patients with long-term hospitalization was higher than for those without (P < .001). Nevertheless, the rates of surgical incision and drainage between patients with and without long-term hospitalization did not achieve statistical significance (P = .069). CONCLUSIONS: Deep neck infection (DNI) is a critical, life-threatening disease that could lead to long-term hospitalization. The higher CRP and involvement of ≥3 deep neck spaces were significant risk factors in univariate analysis, while concurrent mediastinitis was an independent risk factor associated with long-term hospitalization. We suggest intensive care and prompt airway protection for DNI patients with concurrent mediastinitis.

An early report of exoscope-assisted otologic surgery.

BACKGROUND: Exoscope has been used in otoneurologic surgery in several reports. However, most ear surgeries are performed using either microscope or endoscopy today. The purpose of this study is to present our subjective and objective experience of using this instrument in our institution. METHODS: Sixteen ears with different severity and condition in 15 consecutive patients were enrolled. A questionnaire with 12 questions was sent to different participants, including surgeons, residents, medical students, and scrub nurses in the operation room. The total score and average score of each item were calculated and analyzed. RESULTS: Exclusive exoscopic operation was performed on 13 patients with 14 procedures. A combination of endoscopy-exoscope and microscope-exoscope was used in one patient, respectively. There were no complications. Hearing recovery or disease extirpation was achieved in most patients. Twenty participants in the operation room filled out the questionnaire. There was no significant difference between the different groups. High ratings were reported in terms of image quality, stereoscopic effects, magnification rate, and the same field of view as the surgeon. Worse ratings were reported in items related to limited working space, increase in eye strain by video observation, and focusing difficulty. CONCLUSION: Exoscopic ear surgery is feasible, safe, and effective in managing various otologic conditions involving external ear, middle ear, mastoid, and lateral skull base. Its high-definition image quality, stereoscopic effects, sufficient magnification, reliability and teaching value made it a potential instrument in general ear surgeries. Further improvements to current drawbacks can be anticipated.

Diplopia in a Child: Gradenigo Syndrome Is an Unforgettable Disease.

Gradenigo syndrome is a clinical triad of abducens nerve palsy, retro-orbital pain (trigeminal ganglionitis), and chronic otorrhea (otitis media). The etiology of Gradenigo syndrome results from apical petrositis secondary to suppurative otitis media. Although apical petrositis has gradually become uncommon in modern society due to the widespread use of antibiotics, Gradenigo syndrome should be considered in the differential diagnosis of a child's diplopia.

Risk Factors of Descending Necrotizing Mediastinitis in Deep Neck Abscesses.

Background and Objectives: Cervical space infection could also extend to the mediastinum due to the anatomical vicinity. The mortality rate of descending necrotizing mediastinitis is 85% if untreated. The aim of this study was to identify risk factors for the progression of deep neck abscesses to descending necrotizing mediastinitis. Materials and Methods: We retrospectively reviewed the medical records of patients undergoing surgical treatment of deep neck abscesses from August 2017 to July 2022. Computed tomography (CT) was performed in all patients. Before surgery, lab data including hemoglobulin (Hb), white blood cell count, neutrophil percentage, C-reactive protein (CRP) level, and blood glucose were recorded. Patients' characteristics including gender, age, etiology, and presenting symptoms were collected. Hospitalization duration and bacterial cultures from the wound were also analyzed. Results: The C-reactive protein (CRP) level was higher in patients with a mediastinal abscess than in patients without a mediastinal abscess (340.9 ± 33.0 mg/L vs. 190.1 ± 72.7 mg/L) (p = 0.000). The submandibular space was more commonly affected in patients without a mediastinal abscess (p = 0.048). The retropharyngeal (p = 0.003) and anterior visceral (p = 0.006) spaces were more commonly affected in patients with a mediastinal abscess. Conclusions: Descending necrtotizing mediastinitis results in mortality and longer hospitalization times. Early detection of a mediastinal abscess on CT is crucial for treatment. Excluding abscesses of the anterior superior mediastinum for which transcervical drainage is sufficient, other mediastinal abscesses require multimodal treatment including ENT and thoracic surgery to achieve a good outcome.

Prognostic factors in elderly patients after an intra-tympanic steroid injection for idiopathic sudden sensorineural hearing loss.

BACKGROUND: Idiopathic sudden sensorineural hearing loss (ISSNHL) presents with emergent hearing impairment and is mainly treated with steroids. However, limited data exist regarding the prognostic factors among elderly patients (>65 years old) who receive an intra-tympanic steroid injection (ITSI). Therefore, we investigated the prognostic factors in these patients. METHODS: Between July 2016 and March 2022, we retrospectively enrolled 105 elderly patients (>65 years old) with unilateral ISSNHL who were treated with an ITSI, and recorded their clinical and audiological variables. RESULTS: The patients had a mean age of 72.03 ± 6.33 years and mean hearing level gain of 22.86 ± 21.84 dB, speech reception threshold (SRT) gain of 15.77 ± 35.27 dB, and speech discrimination score (SDS) gain of 19.54 ± 27.81 %. According to Siegel's criteria, 5 (4.76 %), 44 (41.91 %), 46 (43.81 %), and 10 (9.52 %) patients had complete recovery, partial recovery, slight improvement, and no improvement, respectively. In the univariate analysis, vertigo (odds ratio [OR] = 0.290, 95 % confidence interval [CI]: 0.130-0.651, p = 0.002) and profound hearing loss on pure tone audiometry (PTA; OR = 0.233, 95 % CI: 0.101-0.536, p = 0.004) were negative prognostic factors among elderly ISSNHL patients. In the multivariate analysis, vertigo (OR = 0.300, 95 % CI: 0.128-0.705, p = 0.005) and profound pure tone audiometry (OR = 0.240, 95 % CI: 0.101-0.570, p = 0.001) were independent adverse prognostic factors among elderly ISSNHL patients. CONCLUSIONS: We demonstrated the treatment outcomes of 105 elderly ISSNHL patients after an ITSI. Vertigo and profound PTA are independent adverse risk factors among elderly ISSNHL patients, and patients with these risk factors require active treatment.

Recurrent sudden sensorineural hearing loss-A literature review.

Objective: Sudden sensorineural hearing loss (SSNHL) is one of the few emergent otologic conditions. Although more than half of all patients would benefit from timely treatment, recurrence occasionally develops unexpectedly. The aim of our study is to evaluate the recurrence rate after SSNHL, and present the prognosis after first-episode and recurrent SSNHL. Methods: A literature search was conducted of the PubMed and Embase electronic databases. Results: Seven studies with a total of 3781 patients were included, and 96 patients experienced recurrence. The recurrence rate ranged between 1.4% and 17%. The average time to recurrence was about 2 years in most studies. The majority of the studies reported using systemic steroids for treating first-episode SSNHL, and one study used a plasma expander in patients with recurrent SSNHL. The recovery rate for first-episode patients was 58%-79%, while that for recurrent SSNHL ranged widely, from 21% to 86%. There were no common risk factors for SSNHL recurrence among the studies, although low-frequency hearing loss, the presence of tinnitus during follow-up, and an increased neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio have been proposed. Conclusion: Understanding the recurrence rate, risk factors, and prognosis after recurrence of SSHNL is crucial for comprehensive medical care; in this respect, further prospective studies with long-term follow-up may be instructive. Level of Evidence: 4.

Experience of adult cochlear implantation at a tertiary hospital.

BACKGROUND: The prevalence of adult cochlear implant (CI) surgery is increasing. However, the relevant adult CI data in Taiwan are insufficient due to the relatively small number of adult implant patients. The two main factors hindering adult implantation are the high cost of the surgery itself and inadequate knowledge regarding the effectiveness of CI for hearing and suppression of tinnitus. Here, we present data regarding adult CI outcomes from a single tertiary hospital. METHODS: A total of 116 consecutive adult CI recipients (≥18 years old) who completed at least 12 months of speech perception tests (words and sentences) between January 1999 and December 2020 were enrolled in this retrospective population-based cohort study. Thirty patients completed speech perception (words and sentences) testing as well as three questionnaires relating to quality of life, and 71 completed full tinnitus suppression studies. Subjects' pre- and post-CI questionnaires were evaluated to assess overall CI outcome. RESULTS: For auditory evaluation, the scores of easy sentences (ES), difficult sentences (DS), and phonetically balanced (PB) word recognition tests reached a plateau at 3 months post-CI (p = 0.005, 0.001, and 0.004, respectively) in most subjects. The post-CI scores of bodily pain, mental health, and social role functioning were significantly higher than corresponding pre-CI scores on the SF-36 Health Survey-Taiwan version (p = 0.036, 0.019, and 0.002, respectively). Furthermore, the post-CI scores of basic sound perception, speech production, and advanced sound perception were significantly higher than the corresponding pre-CI scores on the Nijmegen Cochlear Implant Questionnaire (p < 0.001, 0.013, and <0.001, respectively). Self-esteem was significantly correlated with the Categories of Auditory Performance scale and Speech Intelligibility Rating scale at 3, 6, and 9 months post-CI. CI improved tinnitus in approximately 65.1% of 71 adults. Based on the Tinnitus Handicap Inventory, 66.7% of patients were in grade 3-5 before surgery. However, after CI, only 34.4% of patients remained in THI grade 3-5. CONCLUSION: This study confirmed that CI can improve speech perception (words and sentences), physical health, mental health, social interaction, and self-esteem in adult patients with profound hearing loss. CI also significantly alleviated tinnitus. The outcomes of ES, DS, and PB tests at 3 months post-CI were non-inferior to other longer post-CI periods and could be utilized as references for recovery and evaluation of prognosis.

Nodular mass-A case of auricular schwannoma.

Schwannomas are benign tumors derived from the sheath of Schwann cells. Though it is common to see schwannomas in the head and neck region, auricular schwannomas are rare and only few cases have been reported. There are no distinguishing clinical findings or images; therefore, the histopathological diagnosis is mandatory. We describe a case of auricular schwannoma with clinical pictures and discuss the differential diagnoses according to histopathologic findings.

Complications and prognosis associated with intra-tympanic steroid injection to treat sudden sensorineural hearing impairment.

PURPOSE: The purpose of this study was to measure the incidence of complications in sudden sensorineural hearing loss (SSNHL) patients treated with intra-tympanic steroid injection (ITSI) and compare hearing recovery rates. MATERIALS AND METHODS: 123 patients with unilateral SSNHL receiving ITSIs were included in this study. Post-ITSI complications were documented including otalgia, dysgeusia, vertigo (duration>1 h), and persistent eardrum perforation. The pain intensity was evaluated with visual analog scale (VAS). Hearing was measured before ITSI and at 1 month after the final ITSI. We compared our patients' hearing threshold between presence and absence of different complications. RESULTS: 47.2% patients experienced post-injection otalgia with the average VAS score 3.2 (range 2-6). Five (4.1%) and six (4.9%) patients exhibited vertigo and persistent eardrum perforations, respectively. The patients were divided into three groups based on the absence of complications and the presence of vertigo and eardrum perforation. The hearing threshold improvements did not differ significantly among the three groups (p = 0.366). Although the difference was not significant (p = 0.664), the proportion of patients experiencing post-ITSI vertigo who were on contemporaneous oral steroids was lower than the proportion of non-vertigo patients on such steroids. CONCLUSION: The incidences of otalgia, vertigo, and persistent eardrum perforation in SSNHL patients treated with ITSI were 47.2%, 4.1% and 4.9%, respectively. We found no association between concurrent oral steroid use and the incidence of post-ITSI eardrum perforation or vertigo. Although statistical significance was lacking, patients who did not take contemporaneous oral steroids may have a higher rate of prolonged post-ITSI vertigo.

Merocel compression dressing: a novel modified surgical technique for treating auricular pseudocyst.

PURPOSE: Auricular pseudocyst (AP) is a benign, noninflammatory swelling to the ear, located on either the front or side surface. Deroofing surgery with variable compression methods is considered the most effective method. However, post-operative wound pain is the main drawback following compression. We are introducing a novel painless surgical procedure which involves deroofing technique followed by Merocel® compression dressing. METHODS: From 2015 to 2020, thirty-one patients with AP received this new surgical compression method in our university-affiliated tertiary hospital. Retrospective chart review and the analysis of the results were conducted. RESULTS: All patients had unilateral lesions, with left side lesions (58.1%) predominant. The concha cymba (38.7%) and concha cavum (35.8%) were the most common sites. Previous aspiration or drainage had been performed for the cysts in eight patients (25.8%). Only one patient had the recurrence after post-operative 3 months and received the revised surgery without following recurrence and discomfort. Among all patients, 29 (93.5%) patients claimed minimal pain around 0 or 1 in numeric rating scale of pain score. Three patients had mild ecchymosis but recovered after conservative treatment. One patient had mild auricular deformity after surgery due to pre-treatment partial cartilage necrosis. All patients had follow-up for at least 6 months. CONCLUSION: This novel Merocel® compression dressing technique to treat AP after deroofing is an effective procedure. Minimal pain, simple to do and easily acquired materials were the advantages of this novel procedure.

Salivary Duct Carcinoma in the External Auditory Canal Causing Ipsilateral Hearing Loss.

Background: Salivary duct carcinoma (SDC) is a rare aggressive tumor. Most tumors are not confined to the salivary ducts; rather, they invade the major and minor salivary glands. Only a few case reports on such tumors in other primary sites have appeared. Case presentation: A 40-year-old male complained of right hearing loss (a common condition), but we made an extremely rare diagnosis of an SDC in the external auditory canal (EAC). EAC cancers are frequently misdiagnosed. In our patient, the otoscope revealed a smooth, bulging subcutaneous lesion with a non-epithelial defect suggestive of a benign lesion. However, an SDC of the EAC was confirmed through pathological and immunohistochemical analysis. Conclusions: We suggest detailed evaluation of even smooth EAC subcutaneous lesions to avoid erroneous diagnoses. To the best of our knowledge, this is the first report of SDC in the EAC.

Anaplastic Lymphoma Kinase- and CD30-Positive Anaplastic Large-Cell Lymphoma of the External Auditory Canal.

Anaplastic large-cell lymphoma (ALCL), a form of non-Hodgkin's lymphoma, is rare in the external auditory canal (EAC). ALCL in the EAC region is challenging for clinicians and pathologists. We report a 34-year-old male presented with the chief complaint of a painless mass in the left EAC for approximately 2 weeks. Anaplastic lymphoma kinase (ALK)- and CD30-positive ALCL were confirmed using computed tomography, positron emission tomography, histopathological examination and IHC staining. We compared the clinicopathological characteristics of our patient with those of previous cases. The biopsy and IHC findings confirmed the rare diagnosis of ALK- and CD30-positive ALCL of the EAC. Radiotherapy and concurrent chemoradiotherapy are indicated for lymphoma depending on the extent of the disease. Brentuximab vedotin as initial salvage therapy should be considered for recurrent or refractory ALK-positive ALCL.

Clinical characteristics and treatment outcome of adenoid cystic carcinoma in the external auditory canal.

BACKGROUND: This study reviewed the clinical manifestations, pathological findings, and treatment outcomes of adenoid cystic carcinoma (ACC) in the external auditory canal (EAC). METHODS: This was a retrospective review of 12 patients with a diagnosis of ACC in the EAC seen in a single institution over a 30-year period. Data on the demographics, clinical presentation, treatment strategy, and outcome, as well as the pathological features of ACC, were reviewed and analyzed. RESULTS: The male-to-female ratio was 1:3 and the mean patient age was 55.9 years. The most common clinical presentation was otalgia (75%). Ten patients underwent surgical interventions, including radical mastoidectomy in five patients, wide excision in three, and lateral temporal bone resection in two. Adjuvant radiotherapy or concurrent chemoradiotherapy (CCRT) was performed in case of incomplete resection. Two patients underwent non-surgical treatments: radiotherapy in one and CCRT in the other. Microscopic perineural invasion was not associated with otalgia or histological subtype. The mean follow-up period was 84.6 months. Local recurrence occurred in 33% of patients. One-quarter of patients had distant metastasis, and all had lung metastasis. The 5-year overall survival rate for these patients was 82.5%. CONCLUSION: EAC ACC should be included in the differential diagnosis when a patient presents with otalgia and a mass in EAC for more than 6 months, particularly if the patient is a middle-aged female. Otalgia might not be associated with perineural invasion or histological subtype. The lung is the most common site of distant metastasis in patients with EAC ACC. Further studies should determine the optimal treatment protocol for this rare malignancy.