Endoscopic Cartilage Modified Inlay Technique With Preserving External Auditory Canal Skin for Repairing Posterior Marginal Perforation.

Objective: To evaluate the graft success rate and hearing gain of endoscopic cartilage myringoplasty with preserving external auditory canal (EAC) skin for posterior marginal perforation. Materials and Methods: We performed a prospective study in 36 patients with posterior marginal perforation who underwent endoscopic cartilage modified inlay technique with preserving EAC skin. Patients were followed up for 12 months. Results: All the 36 patients with unilateral posterior marginal perforation were included in this study. Clinical inosculation of the perichondrium graft and remnant tympanic membrane (TM) occurred at 2 to 3 weeks postoperatively. Simultaneously, neovascularization was initiated at the perichondrium graft. The perichondrium graft had been clinically incorporated to the EAC skin at 2 to 3 weeks postoperatively, which could not be distinguished. At 12 months, residual perforation was seen in inferior margin in one but reperforation was not. The graft success rate was 97.2% (35/36). The mean preoperative air bone gap (ABG) was 29.7 ± 3.8 dB, and the mean postoperative ABG at 12 months was 15.2 ± 3.1 dB; the difference between these values was significant (P < .05; paired-samples t test). The successful surgery rate (postoperative ABG ≤ 20 dB) was 91.7% (33/36). No any intratympanic or EAC cholesteatoma was noted during the follow-up period. Conclusions: Endoscopic cartilage modified inlay technique with preserving EAC skin for repairing posterior marginal perforation is simple and minimally invasive technique, which had shorter operative time, similar graft success rate compared with raising a tympanomeatal flap techniques.

CyberKnife stereotactic radiosurgery for extramedullary plasmacytoma in the external auditory canal: illustrative case.

BACKGROUND: Plasmacytoma, a rare plasma cell disorder, often presents as a solitary or multiple tumors within the bone marrow or soft tissues, typically associated with multiple myeloma. Extramedullary plasmacytomas (EMPs), particularly those located in the external auditory canal (EAC), are exceedingly rare and pose significant treatment challenges given their location, anatomical complexity, and high risk of recurrence. OBSERVATIONS: The authors report the case of a 72-year-old male with a history of multiple myeloma, presenting with recurrent left EAC plasmacytoma. After initial conventional radiotherapy for the lesion, a recurrence was documented in 7 years. The patient subsequently underwent stereotactic radiosurgery, which proved successful, leading to complete resolution of the lesion without any long-term adverse effects or radiation-related complications over a 45-month period. LESSONS: This case is a unique instance of utilizing stereotactic radiosurgery for recurrent EMP in the EAC, highlighting its potential as an effective approach in managing complex plasmacytomas.

[Facial nerve protection in surgery for rare tumors of the internal auditory canal].

Objective:To explore strategies for preserving facial nerve function during surgeries for rare tumors of the internal auditory canal. Methods:A total of 235 cases of internal auditory canal tumors treated between 2010 and 2023 were included, encompassing vestibular schwannomas, cavernous hemangiomas, meningiomas, and other rare tumors. Various data, including clinical presentations, imaging classifications, and treatment processes, were meticulously analyzed to delineate the characteristics of rare tumors and assess pre-and postoperative facial nerve function. Results:Among all internal auditory canal tumors, vestibular schwannomas accounted for 91.9%. In rare tumors, facial nerve schwannomas constituted 5.3%, cavernous hemangiomas 26.3%, meningiomas 15.8%, and arterial aneurysms 10.5%. Significantly, patients with cavernous hemangiomas displayed pronounced invasion of the facial nerve by the tumor, in contrast to other tumor types where clear boundaries with the facial nerve were maintained. During surgery, individualized approaches and strategies for facial nerve protection were implemented for different tumor types, involving intraoperative dissection, tumor excision, and facial nerve reconstruction. Conclusion:Preservation of the facial nerve is crucial in the surgical management of rare tumors of the internal auditory canal. Accurate preoperative diagnosis, appropriate timing of surgery, selective surgical approaches, and meticulous intraoperative techniques can maximize the protection of facial nerve function. Personalized treatment plans and strategies for facial nerve functional reconstruction are anticipated to enhance surgical success rates, reduce the risk of postoperative facial nerve dysfunction, and ultimately improve the quality of life for patients.

[Primary central nervous system lymphoma presenting as a unilateral internal auditory canal lesion: a case report].

A 61-year-old man with right hearing loss and staggering for seven months was diagnosed with sudden deafness although previous evaluation with MRI indicated minor abnormal findings. During follow-up, he developed hypogeusia, right facial nerve palsy, pain in right mandible, right-sided temporal pain, and cerebellar ataxia. Cerebrospinal fluid examination at admission revealed reduced glucose concentration and elevated soluble interleukin-2 receptor (sIL-2R) level, whereas serum sIL-2R level was within the normal range. Brain MRI showed a swollen contrast-enhanced lesion extending from the right internal auditory canal to the middle cerebellar peduncle. Gallium-67 (67Ga) single-photon emission-computed tomography-computed tomography (SPECT-CT) revealed abnormal accumulation at the lesion site. Pathologic analysis of the tumor after resection led to the diagnosis of primary central nervous system lymphoma. In the present case, the MRI and 67Ga SPECT-CT characteristics were distinct from those of vestibular schwannoma. In addition, elevation of sIL-2R in the cerebrospinal fluid but not in serum was useful for differential diagnosis.

A Peculiar Case of Isolated Bilateral Tympanic Plate Fractures.

Tympanic plate fractures are uncommon injuries and carry the risk of external auditory canal stenosis. These injuries are often associated with fractures of adjacent bones like the mandible, maxilla, and temporal bone. Isolated bilateral tympanic bone fractures have rarely been reported. The most frequently advocated treatment for these injuries is surgical to prevent canal stenosis in the future. The effectiveness of non-operative management has been seldom reported. In the current case report, we present an uncommon injury with isolated bilateral tympanic plate fractures secondary to trauma to the mandible with no associated mandible or condylar fractures that were treated non-operatively. The functional outcomes were favorable at one year of follow-up.

Removal of Cyanoacrylate Glue From the External Auditory Canal With Hydrogen Peroxide and Acetic Acid: A Case Report.

Ear canal foreign bodies are commonly encountered in the field of otolaryngology. This is especially common in the pediatric otolaryngology discipline. As a foreign body, cyanoacrylate glue (also called "super glue") can be difficult to remove and cause significant patient distress. Multiple solvents can be described as aiding in removing such foreign bodies. Here, a case is described in which hydrogen peroxide and acetic acid were used sequentially to remove cyanoacrylate glue from the external auditory canal. We describe a technique allowing en bloc removal of the cyanoacrylate glue. Thankfully, the patient's hearing returned to baseline post-operatively with minimal complications. Overall, cyanoacrylate glue can be a very difficult foreign body to remove from the ear canal. In this case, the sequential use of hydrogen peroxide and acetic acid to soak the glue was a safe and successful method to facilitate glue removal from the ear canal.

Surgical Option for External Auditory Canal Cholesteatoma: A Case Report.

External Auditory Canal Cholesteatomas (EACC), is an exceptionally rare condition with a prevalence of only 0.1-0.5% among new patients1. EACC are known to possess bone eroding properties, causing a variety of complications, similar to the better-known attic cholesteatomas. We describe here the novel surgical management of a case of EACC. She is 38-year-old female who presented with otorrhea for 6 months. Clinical examination and radiological investigations suggested the diagnosis of an external auditory canal cholesteatoma. The patient underwent modified radical mastoidectomy with type 1 tympanoplasty with meatoplasty. Post-operatively, the patient showed marked clinical improvement.

Hypertrophic pachymeningitis of the internal auditory canal and cerebellopontine angle from a large recurrent attic cholesteatoma.

OBJECTIVES: To describe a rare complication of cholesteatoma. METHODS: Case report with literature review. RESULTS: We report a case of a 37-year-old male who presented for evaluation of otorrhea, headache, and progressive left sensorineural hearing loss. Clinical and radiologic evaluation demonstrated a large recurrent attic cholesteatoma with erosion into the lateral and superior semicircular canals, and diffuse enhancement of the internal auditory canal and cerebellopontine angle suggestive of hypertrophic pachymeningitis secondary to cholesteatoma. After treatment with a course of antibiotics and canal wall down mastoidectomy surgery for cholesteatoma exteriorization, he experienced improvement of his symptoms and resolution of hypertrophic pachymeningitis. CONCLUSION: Hypertrophic pachymeningitis is a rarely described complication of cholesteatoma. In the context of cholesteatoma, treatment with antibiotics and surgical removal or exteriorization of cholesteatoma are effective treatments for HP.

The effective surgical management of traumatic external auditory canal atresia resulting from a dog bite using a stent silicone prosthesis.

INTRODUCTION: Acquired external auditory canal (EAC) atresia is a very rare condition, especially due to direct trauma. The challenge lies in the difficulty of treatment and the tendency to recur even after a long period. Here we present a case report of treatment of recurrent EAC atresia resulting from a dog bite using a silicone stent prosthesis. CASE PRESENTATION: A 4-year-old, bitten by a dog, suffered severe head injuries, including scalp avulsion and left auricle avulsion. Initial treatment included wound closure, antibiotics, and vaccines. However, complications arose, leading to left EAC atresia and hearing loss. Surgical interventions, including canaloplasty and skin grafting, were conducted, with recurrence prompting second surgery with using a silicon prosthetic post-surgery. Follow-up for up to 14 months showed no evidence of recurrence of atresia, with complete improvement in hearing. DISCUSSION: Acquired stenosis of the External Auditory Canal (EAC) can result from various factors, predominantly otitis externa and traumatic incidents, including previous surgeries. Though direct trauma is rare, it can lead to stenosis. Surgical intervention is often necessary for post-traumatic cases, involving removal of obstructions, canal widening, and reconstruction. Management depends on maturity of stenosis, with non-surgical methods for soft stenoses and surgical procedures for mature ones, often supplemented by postoperative stents to preventing restenosis. CONCLUSION: Acquired external auditory canal atresia remains a rare, difficult-to-treat entity with a high rate of recurrence. The use of a silicone prosthesis stent after surgery can be considered an effective method in preventing recurrence.

Inverted Follicular Keratosis: A Rare Presentation in External Ear Canal.

Inverted follicular keratosis is a benign skin lesion that typically presents as an asymptomatic, solitary nodule that may mimic malignant lesions, especially squamous cell carcinoma, both clinically and pathologically. It is developed from the hair follicle infundibulum and can be considered a variant of seborrheic dermatitis. We present a case of a 26-year-old gentleman with a soft tissue mass at the external auditory canal which was later confirmed as inverted follicular keratosis and was treated by endoscopic transcanal excision of the mass with temporalis fascia graft reconstruction. He is currently under surveillance follow-up with no symptoms and signs of recurrence.

Relapsing Irritated Seborrheic Keratosis of External Auditory Canal and its Management.

Seborrheic keratosis lesions are tumors that rarely occur in the external auditory canal. There are several histological variants, and one of them is the irritated seborrheic keratosis. This kind has been scarcely reported in the literature. We report a 62 years old man with this lesion that relapses after monopolar electrocautery, and finally is removed by skin flap dissection.

Xanthogranuloma in the External Auditory Canal in Childhood, an Unexpected Lesion.

Pedunculated lesions in the external auditory canal present a broad differential diagnosis. It is crucial to rule out malignant neoplasms and consider the possibility of xanthogranulomas, which are very rare in this location. Management is determined by associated complications, and otolaryngologists must consider this for appropriate treatment.

Palliative Treatment of Leptomeningeal Carcinomatosis From Renal Cell Carcinoma With Local CyberKnife Radiotherapy and Systemic Pazopanib Therapy: A Case Report.

Leptomeningeal carcinomatosis (LMC) from renal cell carcinoma (RCC) is rare. There is no established treatment strategy for LMC, and the prognosis is extremely poor. We describe a case of LMC from RCC treated with local CyberKnife radiotherapy (CKR) and systemic therapy with pazopanib. The patient was a 63-year-old man with brain metastases from right RCC. Surgery and CKR were performed for the brain metastases, and the lesions were subsequently controlled. The patient developed isolated lesions in the pituitary stalk, right internal auditory canal, left ventricular choroid plexus (CP), left facial nerve, and medulla oblongata after the surgery and CKR for brain metastases. We diagnosed LMC and treated the patient with systemic therapy with pazopanib. We performed local therapy with CKR for lesions of the pituitary stalk, right internal auditory canal, left facial nerve, and medulla oblongata. The CP lesion was not treated with CKR because the lesion tended to shrink after systemic therapy with pazopanib. There were no symptoms due to LMC until the end of life and no adverse events due to CKR. Ten years and five months after the nephrectomy for RCC, one year and four months after the initial CKR for brain metastases, and nine months after the diagnosis of LMC, the patient died due to pleural effusion from lung metastases. Our case suggests that CKR combined with pazopanib may be effective as a palliative treatment for LMC from RCC.

Finding the Silver Bullet for Persistent Foramen Hushke.

We report a case of persistent foramen Hushke and embark on a literature search from 1990 to 2021. The search was done using electronic databases of PubMed and Google Scholar using the MESH words 'TMJ herniation', 'persistent foramen Hushke', 'TMJ prolapse', 'EAC dehiscence', and 'ear canal mass'. A total of 37 other case reports were included after excluding duplicates and non-English publications. The most common presentations, treatment modalities, complications, and outcomes were discussed. Common presentations include otalgia, tinnitus, otorrhea, and aural fullness. Surgical intervention shows a good outcome, whereas conservative treatment shows a mixed response. Despite the overwhelming success of surgical interventions in treating symptomatic persistent foramen of Hushke, it is still early to establish a guideline to manage these patients, as this condition is rare and presents with variable symptoms. More high-quality studies and a long-term follow-up of the patients may be essential to observe and compare the outcome and recurrence rate of temporomandibular joint (TMJ) herniation.

Carcinomas of the external auditory canal: Management and results: A multicenter REFCOR propensity score matching study.

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.

Treatment outcome and prognostic factors of external auditory canal squamous cell carcinoma: A retrospective study in a tertiary center.

Objective: Squamous cell carcinoma (SCC) of the external auditory canal (EAC) is a rare malignancy with various treatment strategies and outcomes. The purpose of this study was to evaluate the clinical characteristics and survival outcomes and identify prognostic factors in patients with SCC of EAC. Methods: Twenty-one patients with SCC of EAC treated in a single tertiary center between 2009 and 2021 were retrospectively reviewed and analyzed. The modified Pittsburgh classification system was applied for staging. Factors associated with survival were identified by univariate survival analysis. Results: The mean age at diagnosis was 61 years (range: 41-79 years). Early-stage (T1 + T2) accounts for 38.1% of the series and advanced-stage (T3 + T4) accounts for 61.9%. Eighteen (85.7%) patients underwent primary surgery with curative intent. The 5-year overall survival rate of the 21 patients was 67.4%. Tumor invasion to the otic capsule, eustachian tube, sigmoid sinus, and dura were associated with poor prognosis in univariate analysis (p = .046; .008; .027; and .08, respectively). Conclusions: Factors predictive of less favorable survival include the history of COM, tumor invasion to the otic capsule, eustachian tube, sigmoid sinus, and dura. It is important to make a precise and systemic preoperative evaluation of disease extent. Level of Evidence: 4.

Fungal Otitis Externa (Otomycosis) Associated with Aspergillus Flavus: A Case Image.

A 48-year-old man presented with a chief complaint of intermittent right ear otorrhea of several-month duration, occasional otalgia and progressive unilateral hearing impairment. He also reported frequent episodes of headache and pressure in the sinuses and maxilla. Previous systemic treatment with antibiotics failed to alleviate the symptoms. A head/neck CT showed completely normal mastoid, middle ear and external auditory canal regions without any evidence of opacification or bone erosion. Otoscopic examination of the right ear disclosed aggregates of dried, brown, fibrillar material and debris occluding the external auditory canal and obstructing the otherwise intact tympanic membrane. Dilation of the external auditory canal or thickening of the tympanic membrane were not appreciated. The canal was debrided and the fibrillar material was placed in formalin. Histopathologic examination revealed numerous branching, septated fungal hyphae organized in densely-packed clusters. In other areas, the fungal hyphae abutted or were attached to lamellated collections of orthokeratin. As highlighted by GMS staining, the fungi were morphologically compatible with Aspergillus species. The clinicopathologic findings supported a diagnosis of fungal otitis externa, while the numerous anucleate squamous cells were compatible with colonization of an underlying, probably developing, cholesteatoma. Culture of material isolated from the external auditory canal confirmed the presence of Aspergillus flavus. In this illustrative case, we present the main clinical and microscopic characteristics of Aspergillus-related otomycosis developing in the setting of a tautochronous cholesteatoma.

Detailed analysis of inner ear malformations in CHARGE syndrome patients - correlation with audiological results and proposal for computed tomography scans evaluation methodology.

OBJECTIVES: The aim was to describe the spectrum of inner ear malformations in CHARGE syndrome and propose a Computed Tomography (CT) detailed scan evaluation methodology. The secondary aim was to correlate the CT findings with hearing thresholds. METHODS: Twenty ears of ten patients diagnosed with CHARGE syndrome were subjected to CT analysis focusing on the inner ear and internal acoustic canal. The protocol used is presented in detail. ASSR results were analyzed and correlated with inner ear malformations. RESULTS: Cochlear hypoplasia type III was the most common malformation found in 12 ears (60%). Cochlear hypoplasia type II, aplasia with a dilated vestibule, and rudimentary otocyst were also identified. In 20%, no cochlear anomaly was found. The lateral Semicircular Canal (SCC) absence affected 100% of ears, the absence of the posterior SCC 95%, and the superior SCC 65%. Better development of cochlea structures and IAC correlated significantly with the lower hearing thresholds. CONCLUSION: This study demonstrated that rudimentary SCC or a complete absence of these SCCs was universally observed in all patients diagnosed with CHARGE syndrome. This finding supports the idea that inner ear anomalies are a hallmark feature of the CHARGE, contributing to its distinct clinical profile. The presence of inner ear malformations has substantial clinical implications. Audiological assessments are crucial for CHARGE syndrome, as hearing loss is common. Early detection of these malformations can guide appropriate interventions, such as hearing aids or cochlear implants, which may significantly improve developmental outcomes and communication for affected individuals. Recognizing inner ear malformations as a diagnostic criterion presents implications beyond clinical diagnosis. A better understanding of these malformations can advance the knowledge of CHARGE pathophysiology. It may also help guide future research into targeted therapies to mitigate the impact of inner ear anomalies on hearing and balance function.

Facial Nerve Schwannoma Treatment with Stereotactic Radiosurgery (SRS) versus Resection followed by SRS: Outcomes and a Management Protocol.

Background Stereotactic radiosurgery (SRS) and resection are treatment options for patients with facial nerve schwannomas without mass effect. Objective This article evaluates outcomes of patients treated with SRS versus resection + SRS. Method We retrospectively compared 43 patients treated with SRS to 12 patients treated with resection + SRS. The primary study outcome was unfavorable combined endpoint, defined as worsening or new clinical symptoms, and/or tumor radiological progression. SRS (38.81 ± 5.3) and resection + SRS (67.14 ± 11.8) groups had similar clinical follow-ups. Results At the time of SRS, the tumor volumes of SRS (mean ± standard error; 1.83 ± 0.35 mL) and resection + SRS (2.51 ± 0.75 mL) groups were similar. SRS (12.15 ± 0.08 Gy) and resection + SRS (12.16 ± 0.14 Gy) groups received similar radiation doses. SRS group (42/43, 98%) had better local tumor control than the resection + SRS group (10/12, 83%, p = 0.04). Most of SRS (32/43, 74%) and resection + SRS (10/12, 83%) group patients reached a favorable combined endpoint following SRS ( p = 0.52). Considering surgical associated side effects, only 2/10 patients of the resection + SRS group reached a favorable endpoint ( p < 0.001). Patients of SRS group, who are > 34 years old ( p = 0.02), have larger tumors (> 4 mL, 0.04), internal auditory canal (IAC) segment tumor involvement ( p = 0.01) were more likely to reach an unfavorable endpoint. Resection + SRS group patients did not show such a difference. Conclusion While resection is still needed for larger tumors, SRS offers better clinical and radiological outcomes compared to resection followed by SRS for facial schwannomas. Younger age, smaller tumors, and non-IAC situated tumors are factors that portend a favorable outcome.

Risk factors for occurrence and progression of external auditory canal cholesteatoma.

OBJECTIVE: External auditory canal cholesteatoma (EACC) is characterized by retained squamous debris within the external canal and variable amounts of localized bone destruction. The etiology of primary EACC remains incompletely understood. This study was conducted to analyze the clinical features and backgrounds of patients with primary EACC and to clarify the risk factors for the occurrence and progression of EACC. METHODS: Sixty-nine ears of 62 patients diagnosed with primary EACC were included in this study (EACC group). Additionally, 74 ears of 60 patients with chronic otitis media (COM) with perforation who underwent tympanoplasty or myringoplasty were included as controls (COM group). We retrospectively investigated the clinical features, life history, and medical history of the patients in both groups. In addition, to investigate the risk factors for progression of EACC, we compared the clinical features and medical history of patients with stage IV (advanced) disease versus stage I + II (mild) disease. RESULTS: The inferior wall of the bony canal was the main structure affected in patients with primary EACC of all stages. The following factors were significantly more common in the EACC than COM group: older age, female sex, left-sided disease, osteoporosis, renal dysfunction, anemia, and treatment with bisphosphonates. Among these, the most significant factor associated with EACC was renal dysfunction (odds ratio, 11.4; 95 % confidence interval, 2.32-55.9). The significant factors observed in patients with stage IV disease were younger age, male sex, posterior wall involvement, and otorrhea. Surgical treatment was required for more than half of the patients with stage III and IV EACC. CONCLUSION: Patients with renal dysfunction are at risk of primary EACC. In particular, younger patients and relatively younger elderly patients with posterior wall involvement have a risk of progression to advanced-stage EACC. Canalplasty should be considered in patients with EACC who have these risk factors to prevent progression to advanced-stage disease.