Single-stage reconstruction of a large defect of the ear.

To reconstruct a large anterior skin and cartilage defect of the upper half of the external ear in an elderly patient after cancer surgery, different techniques are possible, but single-stage procedures should be advised. Combining flaps with reliable vascular supply, like the revolving door post-auricular flap and a mastoid advancement flap, is an attractive single-stage reconstructive option to rebuild a sturdy auricle.

Questioning the value of stalk drilling after external auditory canal osteoma excision: case series, literature review, and meta-analysis.

OBJECTIVES: To question the value of drilling the site of the stalk ("insertion site" or "stalk" drilling) of a pedunculated external auditory canal osteoma (EACO) in reducing recurrence. DATA SOURCES: A retrospective medical chart review of all patients treated for EACO in one tertiary medical center, a systematic literature review using Medline via "PubMed", "Embase", and "Google scholar" search, and a meta-analysis of the proportion for recurrence of EACO with and without drilling. RESULTS: The local cohort included 19 patients and the EACO origin was the anterior EAC wall in 42% and the superior EAC wall in 26%. The most common presenting symptoms were aural fullness and impacted cerumen (53% each), followed by conductive hearing loss (42%). All patients underwent post-excision canaloplasty, and one sustained EACO recurrence. Six studies suitable for analysis were identified (63 EACOs). Hearing loss, aural fullness, otalgia, and cerumen impaction were the most common clinical presentations. The most common EACO insertion site was the anterior EAC wall (37.5%), followed by the superior EAC and posterior walls (25% each). The inferior EAC wall was least affected (12.5%). There was no significant difference in recurrence between EACOs whose stalk insertions were drilled (proportion 0.09, 95% confidence interval [CI] 0.01-0.22) to the ones whose insertion was not drilled (proportion 0.05, 95% CI 0.00-0.17). The overall recurrence proportion was 0.07 (95% confidence interval 0.02-0.15). CONCLUSION: EACO insertion site drilling does not reduce recurrence and should be avoided in the absence of a definite pedicle projecting to the EAC lumen.

Blue laser for the exclusive endoscopic transcanal approach to middle ear paraganglioma.

BACKGROUND: The management of glomus tympanicum tumours can be challenging. Blue laser coagulation may improve bleeding control thus facilitating an endoscopic transcanal excision. The objective of this presentation is to illustrate the authors' experience using this novel tool. METHODS: Case report of a patient that underwent exclusive endoscopic transcanal blue laser surgery of a class A2 glomus tympanicum tumour in a tertiary referral center. CONCLUSION: The present study provides evidence of the safety and efficacy of endoscopic blue laser surgery, for the minimally invasive treatment of early-stage glomus tympanicum tumours.

Melanoma of the external auditory canal: case report and systematic literature review.

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.

Reconstruction of a full-thickness upper third defect of the helix ear using a single-stage chondrocutaneous composite transposition flap.

The external ear is a crucial part of the ear's anatomy for both functional and aesthetic purposes. We present a defect after the extirpation of an invasive squamous cell carcinoma, where the final defect involved the superior third of the outer ear, missing both cartilage and skin. The regional chondrocutaneous composite transposition flap of the ipsilateral auricular concha is a one-stage technique that successfully preserves the helical rim's shape and size.

Clinical outcome and prognostic factors in adenoid cystic carcinoma of the external auditory canal: proposal for a refined T-stage classification system.

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.

Surgical management of endolymphatic sac tumor: classification, outcomes and strategy. A single institution's experience.

PURPOSE: To review the resections of endolymphatic sac tumor (ELST) and describe our experience in the surgical management of ELST. METHODS: Retrospective investigation of consecutive patients who underwent resection of ELSTs at our hospital between 1999 and 2019. The symptoms, diagnosis, surgical findings, and outcomes were analyzed to develop a tumor staging system and corresponding surgical strategy. RESULTS: Retrospective review revealed the surgical treatment of 22 ELSTs. Based on intraoperative findings of tumor extent and size, ELSTs were classified into two types. Type-I (n = 6) referred to the small tumors that were locally confined with limited invasion of semicircular canals and dura; type-II (n = 16) referred to the large tumors that presented extensive erosion of at least one anatomic structure apart from the semicircular canals and the dura around endolymphatic sac. In this case series, Type-I ELST is amenable to resection through a transmastoidal approach, and subtotal petrosectomy is appropriate for the resection of type-II ELST. Sensorineural hearing loss (SNHL) is the most commonly preoperative symptom in both two types of cases. Five type-II ELSTs experienced recurrence and underwent reoperation, whereas all type-I ELSTs did not. CONCLUSION: ELST usually results in SNHL (95%) at the time of diagnosis. The surgical strategy and prognosis of ELST resections are different between type-I and type-II: type-I ELST is amenable to transmastoidal approach with the preservation of facial nerve, whereas type-II ELST increase the surgical difficulty and the risk of recurrence, and subtotal petrosectomy is the basic requirement for the resection of type-II ELST.

The Diagnosis and Management of Advanced Endolymphatic Sac Tumor.

Endolymphatic sac tumor (ELST) is a group of low-grade malignant tumors originating from the endolymphatic sac of the inner ear. It is rare in the clinic and has the biological characteristics of slow growth and local aggression. Due to the lack of specificity in the clinical manifestations of patients with ELST, many cases have entered the advanced stage at the time of diagnosis. However, there are still great challenges in the treatment of advanced ELSTs. Here, the authors describe a case of advanced ELST, which relapsed after 2 operations. This time, the authors chose the transotic approach for tumor resection, which achieved the goal of complete resection of the tumor, and the patient recovered smoothly after surgery. There were no surgical complications and no tumor recurrence after the follow-up. Through literature review and our own experience, the authors suggest that complete surgical resection is the first choice for both primary and recurrent advanced ELSTs. The choice of a reasonable surgical approach is the key to ensuring complete resection of the tumor, while preoperative angiography and embolization, fine treatment of important structures during surgery, and postoperative long-term follow-up are equally important for patients with advanced ELST to obtain a good prognosis.

Primary external auditory canal meningioma: Case report and review of the literature.

Meningiomas are tumors that arise from arachnoid cells attached to both the pia mater and the inner portion of the arachnoid. They are common intracranial tumors, representing 12-25% of intracranial neoplasms. Intracranial meningiomas can spread extracranially to involve surrounding structures, including the ear and temporal bone. Ectopic meningiomas, described as primary meningiomas with no intracranial involvement, are rare. We describe a case of a primary external auditory canal meningioma with no evidence of intracranial involvement. We present pre-operative imaging findings proving no intracranial involvement prior to surgical intervention. A literature review of this uncommon clinical entity is presented and a discussion regarding its prognosis and treatment is reviewed.

Endolymphatic sac tumor: single-institution series of seven cases with updated review of literature.

BACKGROUND: Endolymphatic sac tumour (ELST) is a rare low-grade locally aggressive neoplasm arising from the endolymphatic duct or sac. It presents mostly with vestibulo-cochlear symptoms either sporadically or as part of von Hippel-Lindau (VHL) syndrome. Micro-neurosurgical excision remains the cornerstone of therapy with the role of radiotherapy (RT) being controversial. This is a clinico-pathological analysis of consecutive ELST patients presenting to a single-institution in India. METHODS: Neuropathology database of a tertiary-care comprehensive cancer centre was searched electronically to identify consecutive patients with histopathological diagnosis of ELST registered at the institute over last one decade. Data regarding demographic profile, clinical presentation, histopathological features, treatment details and outcomes were retrieved from electronic medical records for this retrospective analysis. RESULTS: Electronic search identified seven unique patients with biopsy-proven ELST registered at the institute between 2009 and 2020. Median age of the study cohort was 39 years (range 24-65 years) with strong male predilection (5:2 ratio) and left-sided preponderance (71%). Most common presenting symptoms were hearing loss (86%) and earache (71%) on affected side followed by headache (43%). All patients underwent maximal safe resection at initial diagnosis and were followed-up closely with periodic surveillance imaging. Two patients underwent salvage RT using high-precision conformal techniques at recurrence/progression. CONCLUSION: ELST is a rare low-grade locally aggressive neoplasm that arises generally as part of VHL syndrome or sometimes sporadically. Gross total resection provides the best chance of cure with RT being reserved for unresectable disease, large residue, medical inoperability, or as salvage therapy for recurrent/progressive tumor.

[Neuroendocrine adenoma middle ear]. / Neiroendokrinnye adenomy srednego ukha.

Neuroendocrine adenoma (NEA) is an extremely rare pathology and accounts for less than 2% of all ear tumors. The article provides an overview of the diagnosis, classification, treatment methods and algorithm for monitoring patients with NEA of the middle ear. 6 cases of NEA of the tympanic cavity are described in detail, which were diagnosed and treated in GBUZ NIKIO named after N.N. L.I. Sverzhevsky DZM for the last 5 years. Diagnosis of NEA of the middle ear is possible when performing high-resolution multislice computed tomography of the temporal bones and magnetic resonance imaging with contrast enhancement, however, the neoplasm can be verified only after a histological examination with the determination of the immunophenotype. Differential diagnosis of NEA of the tympanic cavity with other processes of the middle ear must be carried out at each stage of the diagnostic path. Surgical treatment, depending on the volume of education, allows you to remove it completely and improve the auditory function.

Reconstruction of the external auditory canal: The tragal flap revisited and review of contemporary reconstructive techniques.

BACKGROUND: Primary malignancies arising in the external auditory canal (EAC) are rare and usually are treated surgically. We review techniques to reconstruct the EAC following ablative surgery, and introduce a rarely utilized tragal skin flap which has particular advantages for reconstruction of limited anterior EAC defects. METHODS: The terms "tragal flap", "external auditory canal", "preauricular tragal flap", "reconstructive techniques" were searched on PubMed and Google Scholar. RESULTS: Our review identified one description of a tragal flap to reconstruct the EAC following resection of a malignancy. We add an additional case of a preauricular tragal flap to reconstruct the anterior EAC following resection of a recurrent basal cell carcinoma located in the EAC that led to a circumferential defect. CONCLUSION: There are several surgical techniques that can be utilized to reconstruct the EAC. We describe a novel tragal flap used to reconstruct the anterior EAC following resection of a recurrent tumor.

Piezoelectric canalplasty for exostoses and osteoma.

OBJECTIVE: To evaluate the safety profile and surgical technique for removal of symptomatic exostoses and osteoma of the external auditory canal with a micro-oscillating piezoelectric device. METHOD: A chart review was conducted on patients undergoing piezoelectric canalplasty between 2019 and 2021 at tertiary referral hospital. Surgery was performed by two surgeons with varying experience. Bone removal was achieved using both osteotomy and osteoplasty. Postoperative complications, operative time and hearing outcome were evaluated. RESULTS: The study comprised 16 patients (16 ears). No major complications occurred. The skin of the auditory canal was completely preserved in all patients without injury to the tympanic membrane. Except for one patient with known noise-induced hearing loss, there was no postoperative deterioration of the bone-conduction threshold more than 10 dB HL at any frequency. The difference of the bone-conduction threshold in pure-tone audiometry (average for 0.5, 1, 2 and 4 kHz) three weeks postoperatively had a median of 0.6 dB ± 5.7. One patient complained of temporary new tinnitus. One patient had prolonged wound healing. Mean operative time was comparable with literature data. CONCLUSION: The atraumatic characteristics of the piezoelectric instrument enable low-risk removal of external auditory canal exostoses and osteoma. Through the combination of precise osteotomy and osteoplasty, this novel instrument has the potential to become established in routine canalplasty.

Degree of preoperative hearing loss predicts time to early mobilization following vestibular schwannoma microsurgery.

OBJECTIVE: To test the hypothesis that severe to profound preoperative hearing loss predicts less acute postoperative vestibulopathy following microsurgical removal of vestibular schwannoma (VS) allowing for earlier postoperative mobilization and hospital discharge. METHODS: Patients with VS who underwent microsurgery and were found to have preoperative severe to profound hearing loss (pure tone average [PTA] > 70 dB HL) were matched 1:1 by age and tumor size to a group of randomly selected controls with preoperative serviceable hearing. RESULTS: A total of 57 patients met inclusion criteria and were matched to controls. Median age at the time of microsurgery was 56 years. The median PTA and WRS for cases were 91 dB HL (interquartile range [IQR] 78-120) and 0% (IQR 0-0), respectively. Median tumor size was 14.2 mm (IQR 10.9-20.9). A total of 35 (61%) patients exhibited nystagmus after surgery associated with acute vestibular deafferentation. Median time to ambulation in the hallway was 2 days. Controls exhibited similar tumor size (12.7 mm, p = 0.11) and age (57 years, p = 0.52). Preoperative hearing loss did not predict severity or duration of postoperative nystagmus or days to discharge; however, those with Class D hearing exhibited a shorter time to ambulation (p = 0.04). CONCLUSION: Following microsurgical removal of VS, preoperative profound hearing loss was associated with a shorter time to postoperative mobilization; however, there were no observed associations with duration or severity of nystagmus and time to hospital discharge. Although not a predictor of nystagmus, preoperative profound hearing loss may portend quicker recovery from clinically significant postoperative vestibulopathy.

Grading system and surgical approaches for endolymphatic sac tumors.

PURPOSE: Endolymphatic sac tumors (ELSTs) are rare, low-grade adenocarcinomas arising from the endolymphatic sac. This study aims to present a novel grading system for ELSTs to determine the optimal management strategy. METHODS: We performed a retrospective analysis of 16 patients with 17 ELSTs. The tumor location and involved adjacent neurovascular structures on CT and MRI were selected to establish the grading system. RESULTS: Based on the novel grading system, grade III a tumors were most common (7/17), followed by grade I (4/17), grade II (3/17), and grade III b (3/17) tumors. Eight advanced ELSTs (grade III a and III b) received an infra-temporal fossa approach, while the other 6 early stage ELSTs (grade I and II) underwent either a retrolabyrinthine approach with posterior petrosectomy or a translabyrinthine approach combined with subtotal temporal bone resection. Hearing preservation was achieved in 2 grade I patients. Postoperative facial nerve function was HB II in 1 grade III a patient who underwent anterior facial nerve transposition and was HB III in 4 advanced patients who received facial nerve grafts with the great auricular nerve or facial-hypoglossal nerve anastomosis. The mean follow-up time was 35.1 months. Two grade III patients and 1 grade II patient had tumor recurrence during follow-up, among whom 1 grade III b patient had two cases of recurrence. CONCLUSION: A correct initial diagnosis was established in all patients after meticulous imaging studies. Surgical resection is still the first choice to manage patients with ELSTs. The novel grading system enables surgeons to select tailored surgical approaches. Long-term follow-up is necessary following surgical intervention.

The endolymphatic sac tumor: challenges in the eradication of a localized disease.

OBJECTIVE: Identify the critical points that lead to recurrences and lack of radicality in endolymphatic sac tumors (ELSTs). STUDY DESIGN: Retrospective case study and review of the literature. SETTING: Tertiary referral center. PATIENTS: Thirteen cases of ELST were included in the study and their preoperative, intraoperative and postoperative data were analyzed and compared to a review of the literature. INTERVENTION(S): Therapeutical. MAIN OUTCOME MEASURE(S): Prevalence of recurrent and residual tumors, comparison to the literature and analysis of ELST characteristics. RESULTS: Diagnosis was made 26 ± 17 months after the onset of symptomatology, and an ELST was preoperatively suspected in only six cases. At the time of surgery, 10 patients suffered from hearing loss. Preoperative symptoms or audiometry could not predict labyrinth infiltration, although speech discrimination scores were significantly associated with labyrinth infiltration (p = 0.0413). The labyrinth was infiltrated in 8 cases (57.1%), and in 7 cases (46.7%) the tumor eroded the carotid canal, whereas 6 cases (40%) presented an intradural extension. A gross total resection was achieved in 11 cases. There were two residual tumors, one of which because of profuse bleeding, and one recurrence (23.1%). A mean of 22.8% of recurrent or residual tumors are described in the literature based on 242 published cases, in more than half of the cases as a consequence of subtotal tumor resection (STR). CONCLUSIONS: Recurrence derives mostly from the difficulty to identify the extension of the tumor due to the extensive bone infiltration. Accurate diagnosis and correct preoperative planning, with embolization when possible, will facilitate surgery and avoid STR due to intraoperative bleeding. Long follow-ups are important in order to avoid insidious recurrences.

Parotidectomy in the management of squamous cell carcinoma of the external auditory canal.

PURPOSE: To investigate optimal approaches for appropriate removal of the parotid gland in the management of squamous cell carcinoma (SCC) of the external auditory canal (EAC) at different tumor stages. METHODS: In total, 39 patients with SCC of EAC treated at the Second Affiliated Hospital of Nanchang University between September 2003 and April 2019 were enrolled in this study. All patients underwent lateral temporal bone resection or subtotal temporal bone resection. Total parotidectomy was performed in patients with direct parotid invasion. Superficial parotidectomy was performed in patients with parotid node metastasis and patients with advanced stages without evidence of parotid involvement. RESULTS: The mean follow-up period was 68.7 months. Local recurrences or distant metastases occurred in five patients (12.8%). The 5-year overall survival rate was 78.4%. The 5-year survival rate was 100% in early stage (T1 and T2) patients, and 58.9 and 50.0% in patients staged III and IV, respectively. Direct parotid invasion was observed in only advanced-stage patients, while parotid node metastasis was noted in both early and advanced-stage patients preoperatively. There were no significant differences (χ2 = 0.1026; p = 0.749) between different tumor primary locations. However, soft tissue or preauricular organs became vulnerable once the anterior wall was infiltrated or eroded. CONCLUSION: Parotid management is important for achieving safer and wider tumor-free margins. Total parotidectomy should be mandatory for all advanced-staged (T3 and T4) patients. An optimal decision for parotid management in early stages depends on the infiltration or erosion of the anterior wall of the EAC.

Schwannoma in the External Auditory Canal.

ABSTRACT: Schwannoma is a slow-growing, benign tumor originating from the Schwann cells of peripheral nerve sheaths. Schwannoma in the external auditory canal is rare but should be included in the possible diagnosis of a cystic mass. If schwannoma is suspected, computed tomography or magnetic resonance imaging should be performed to explore tumor size, location, possible extension, and origin before excision. The authors here present a large schwannoma that almost completely occluded the external auditory canal and was completely excised using a transcanal approach.

Adenomatous Neuroendocrine Tumors of the Middle Ear in a Young Man With Conductive Hearing Loss.

ABSTRACT: Neuroendocrine neoplasms of the ear pose a diagnostic challenge because clinical symptoms are no specific. Definite diagnosis is made by histopathological analysis with immunohystochemical evalutation. Therapy consists in a complete surgical resection. Controversial terminology of the neoplasm arises from the differentiation of these tumors composed of both endocrine and exocrine glands. Middle ear localization is an extremely rare presentation but less aggressive than gastrointestinal tract or lung localizations which are the most frequent. Radio-diagnostic analyses are necessary follow-up for preventing or detecting recurrence or metastasis. The authors present a case of neuroendocrine tumor of the middle ear in a young male of 37 years old who presented unilateral right hearing loss and tinnitus. A transcanal tympanoplasty was performed. There were no intraoperative complications and the postoperative period was uneventful.

Adenoid cystic carcinoma of external auditory canal: A rare disease.

External auditory canal, like other epithelialized surfaces is predisposed to malignancies such as Squamous cell carcinomas (SCC), melanomas and adenocarcinomas. In this background, malignancies like adenoid cystic carcinoma (ACC) are a rare occurrence and need to be thoroughly evaluated both locally and for distant extension. The malignancy needs to be addressed with an aggressive approach surgically with adequate marginal clearance. The role of radiation is debatable considering the outcomes in the limited data. Here we present the case of a 35-year-old female who came in with otalgia and otorrhea associated with a mass in the external auditory canal. After biopsy and imaging, a diagnosis of adenoid cystic carcinoma was made and the patient was managed accordingly.