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Objective: To discuss the potential benefit of belzutifan therapy in a patient with von Hippel-Lindau (VHL) disease-associated endolymphatic sac tumor (ELST)., Patients: Case report., Interventions: Clinical details of a patient with residual ELST after hearing preservation surgery who initiated belzutifan therapy postoperatively for concurrent renal cell carcinoma, as well as literature review of belzutifan and ELST., Main Outcome Measures: The patient remained without radiologic evidence of growth of her residual tumor at 17 months post-initiation of belzutifan. It is unknown whether this represents therapeutic drug effect, nonviability of residual tumor, or slow tumor growth not captured radiographically within the duration of follow-up., Conclusions: Belzutifan could have direct therapeutic benefit in patients with VHL-associated ELST., Competing Interests: The authors otherwise have no relevant disclosures with regard to financial support, acknowledgement, or conflicts of interest., (Copyright © 2024, Otology & Neurotology, Inc.)

Objective: To evaluate the literature and summarize cochlear implantation (CI) outcomes after intralabyrinthine schwannoma (ILS) excision and tumor observation with CI., Data Sources: OVID Medline, Embase, Web of Science; conception to 2024., Review Methods: A literature review was performed using subject headings, MeSH terms, and keywords. Abstracts and full texts were reviewed by two independent reviewers and adjudicated by a third. Inclusion criteria included studies with ILS and CI with reported audiologic outcomes. Subjects were analyzed into two groups, ILS resection with CI and in situ ILS with CI. Patients with NF2 were included. The main outcome of interest was CI audiometric performance level, with secondary outcomes of CI user status and open-set speech attainment., Results: There were 29 articles with a total of 93 patients who met inclusion criteria. The resection group had 17% low performers, 44% intermediate performers, and 38% high performers. The in situ group had 40% low performers, 32% intermediate performers, 27% high performers. The resection group had 69 patients with 97% maintaining user status and 92% with open-set speech recognition. The observation group had 24 patients, with 87% user rate and 86% achieving open-set speech recognition. There was a greater percentage of NF2 diagnosis seen in the in situ group., Conclusion: There is a paucity of literature on CI and ILS. Patients are managed with both resection of tumor and implantation in situ. Early data are limited, with improvement in hearing outcomes and high user rates in both populations., Level of Evidence: N/A Laryngoscope, 134:3910-3920, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Purpose: In patients with inner ear schwannomas (IES), reports on hearing rehabilitation with cochlear implants (CI) have increased over the past decade, most of which are case reports or small case series. The aim of this study is to systematically review the reported hearing results with CI in patients with IES considering the different audiologic outcome measures used in different countries., Methods: According to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline, a search of published literature was conducted. We included patients with IES (primary or with secondary extension from the internal auditory canal (IAC) to the inner ear, sporadic or NF2 related) undergoing cochlear implantation with or without tumour removal. The audiological results were divided into the categories "monosyllables", "disyllables", "multisyllabic words or numbers", and "sentences"., Results: Predefined audiological outcome measures were available from 110 patients and 111 ears in 27 reports. The mean recognition scores for monosyllabic words with CI were 55% (SD: 24), for bisyllabic words 61% (SD: 36), for multisyllabic words and numbers 87% (SD: 25), and 71% (SD: 30) for sentences. Results from for multisyllabic words and numbers in general showed a tendency towards a ceiling effect. Possible risk factors for performance below average were higher complexity tumours (inner ear plus IAC/CPA), NF2, CI without tumour removal ("CI through tumour"), and sequential cochlear implantation after tumour removal (staged surgery)., Conclusion: Hearing loss in patients with inner ear schwannomas can be successfully rehabilitated with CI with above average speech performance in most cases. Cochlear implantation thus represents a valuable option for hearing rehabilitation also in patients with IES while at the same time maintaining the possibility of MRI follow-up. Further studies should investigate possible risk factors for poor performance. Audiological tests and outcome parameters should be reported in detail and ideally be harmonized to allow better comparison between languages., Competing Interests: Declarations Conflict of interest The institutions of SKP and TR receive research grants from Cochlear Ltd., Sydney, Australia, and MED-EL, Innsbruck, Austria. This work was supported by intramural funds from Martin Luther University Halle-Wittenberg only., (© 2024. The Author(s).)

Clinical Case: The 59-year-old patient complained of hearing loss on the left, ear murmur for a long time, periodic pain and discomfort in the left ear, dizziness for 6 months. She was found to have concurrent vestibular schwannoma in the internal auditory canal and temporal bone paraganglioma. Both tumors were removed in one operation. The schwannoma was removed by translabirinth access due to preoperative deafness, while the glomus tumor was removed during this access. Postoperative biopsy showed the presence of two unrelated diseases: paraganglioma (ICD-0 code 8690/3) and schwannoma (ICD-0 code 9560/0).

We report the case of a 42-year - old female with familiar form von Hippel-Lindau disease (VHL) and recurrent endolymphatic sac tumour (ELST), which was presented like non-homogenous, solid and cystic expansion of the left petrous temporal bone. Histologically, there was found lamellae of bone with adjacent ligament and with papillary projections with fibrovascular core. The papillae were lined by a single layer of cuboidal epithelium with hyperchromatic and lightly pleomorphic nuclei. Sporadically, small cystic formations with eosinophilic, PAS positive secretion were noted. Imunohistochemically, the cuboidal cells showed diffuse positivity for vimentin, epithelial membrane antigen (EMA), cytokeratin AE1/AE3 and S100 protein (weakly). Other markers examined, including TTF1, PAX8 and CD10, were negative. Endolymphatic sac tumour is rare low-grade malignant epithelial tumour arising from the endolymphatic sac in the temporal bone, which occurs in 1 out of 30 000 births, with just fewer than 300 cases reported in the literature. About one third of cases are associated with von Hippel- Lindau disease, an autosomal dominant familial cancer syndrome.

Background: Paragangliomas in the central nervous system account for 0.6% of all head and neck neoplasms, with glomus tympanicum being the most common middle ear tumor. Carcinoid tumors are neuroendocrine tumors, representing less than 1% of neuroendocrine neoplasms in the middle ear. Misdiagnoses have been reported in the literature regarding glomus and carcinoid tumors, however, none have been in the central nervous system or middle ear., Case Description: A 70-year-old female with a history of left temporal lobe tumor underwent unsuccessful resection due to intraoperative bleeding at an outside institution. However, biopsy prior to aborting the case led to the diagnosis of paraganglioma. Eight years postoperatively, the patient presented at our institution with acute confusion, aphasia, and altered mental status. Imaging revealed a 4cm left temporal intraparenchymal hematoma at the known tumor site with concern for intracranial tumor extension. Surgical resection was performed and previous symptoms resolved. Final pathology revealed a Grade II atypical carcinoid tumor with an unusually high Ki-67 of 50%., Conclusions: Carcinoid tumors of the middle ear constitute a differential diagnosis for patients presenting with temporal lobe hemorrhage. A combination of immunohistochemical staining with electron microscopy can assist in differentiating the tumor types. This atypical presentation for a carcinoid tumor in the middle ear suggests the need to consider carcinoid as the diagnosis in patients with a middle ear tumor invading into the temporal lobe and causing hemorrhage. These tumors may demonstrate an unusually high Ki-67 rate, in which case they should be treated aggressively., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Background: Aural polyps consider as one of the most notable progressive lesions of middle ear. Chronic otitis media can result in development of aural polyps that ascends from the tympanic cavity outwards to the external auditory canal, leading to tympanic membrane perforation and hearing loss. Morbidity and mortality are usually rare, however, failure of diagnosis or prolonged negligence by the patient could increase the likelihood of cholesteatoma or malignant squamous carcinoma., Aim: To investigate the causes of middle ear aural polyps in adult patients selected from Kut and Diwaniyah cities in Iraq., Methodology: 40 patients which of those 14 males and 26 females were included in this study based upon the clinical manifestation of the illness and the suffering from a mass in the tympanic cavity, partial or complete hearing loss, otorrhea and dizziness. Otoscope was used to confirm location, shape, size of the polyps, and the status of the tympanic membrane., Results: The mean age of the involved patients in the study was (44.65 ± 10.9) years. Age range was between (20-60) years. The highest frequency of patients with aural polyps was seen in patients at their third to fifth decade of age. There were 14(35%) men, and 26(65%) women. Findings showed that mean age of men was (44.07 ± 13.7) years, and women was (44.96 ± 9.4) years, (p=0.405). Clinical examination revealed 27(67.5%) patients with cholesteatoma, 10(25%) with inflamed mass in the middle ear, and 3(7.5%) were with cancerous tumor. Correlative analysis showed no significant correlation between age and causes of aural polyps (p=0.664), and no correlation between sex and causes of aural polyps (p=0.524)., Conclusion: Aural polyps are rare disease in Iraqi patients. However, treating and monitoring patients with ongoing chronic otitis media is essential in preventing the development of aural masses in middle ear and other complications if left untreated. Early diagnosis and treatment can prevent reoccurrence and other complications.

Purpose: Most ELST data in the literature are case studies or limited to small cohorts (< 16 patients). We evaluated the main clinical signs observed at endolymphatic sac tumor (ELST) diagnosis in patients with or without Von Hippel-Lindau disease., Methods: We conducted a comprehensive literature search in PubMed, Scopus, and Web of Science. We included studies with at least 1 patient, of any age, affected by sporadic or VHL-related ELSTs reporting levels of hearing loss and facial nerve function and a comprehensive description of presenting symptoms at ELST diagnosis. We combined data for proportional meta-analysis. p values of 0.05 were considered statistically significant. Methodological quality was evaluated. Analyses were performed with MedCalc 14.8.1 software., Results: A total of 26 studies, including 113 patients and 118 cases of ELSTs were included. Pooled proportion rates (95% CI) of overall hearing loss was 88.7%, (82.4-93.4), severe hearing loss was 21.6% (12.8-32.1) profound hearing loss was 39.8% (28.7-51.5), vertigo/imbalance was 42.0% (33.8-50.5), tinnitus was 61.8% (53.4-69.8) and facial nerve palsy was 30.6% (23.2-38.9). Generally, symptoms were homogeneous or moderately heterogeneous among included studies., Conclusion: This is the first systematic review of clinical presentations at ELST diagnosis. The most serious clinical events include profound hearing loss and facial impairment. Fluctuating hearing loss, tinnitus and vertigo are frequently reported and may confound correct and prompt ELST diagnosis., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

A 52-year-old man presented to our emergency department with an acute onset of right-sided facial nerve (FN) palsy of House-Brackmann grade V. Electroneurography (ENoG) was conducted with no response at the right FN, as compared with the left FN (0%). We performed a biopsy of the right middle ear mass and histological studies showed the tumor to be neuroendocrine tumors (NET) of the middle ear. We resected the tumor with canal wall down mastoidectomy and reconstructed the posterior meatal wall with soft tissue. Three months after surgery, the FN paralysis had improved with House-Brackmann grade II. We reviewed cases of NET with FN palsy, and nine patients, including our case, have been reported. Our case is the first report of ENoG for the description of FN palsy due to NET. Although the ENoG value was 0%, it was remarkably improved by surgery. The other cases of NET patients with FN palsy also recovered FN function after surgery. These results suggest that it is recommended to perform the total resection of the tumor to improve the FN function., (Copyright © 2020. Published by Elsevier B.V.)

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., (Published by Elsevier Inc.)

Aims: Although inactivation of the von Hippel-Lindau gene (VHL) on chromosome 3p25 is considered to be the major cause of hereditary endolymphatic sac tumours (ELSTs), the genetic background of sporadic ELST is largely unknown. The aim of this study was to determine the prevalence of VHL mutations in sporadic ELSTs and compare their characteristics to VHL-disease-related tumours., Methods: Genetic and epigenetic alterations were compared between 11 sporadic and 11 VHL-disease-related ELSTs by targeted sequencing and DNA methylation analysis., Results: VHL mutations and small deletions detected by targeted deep sequencing were identified in 9/11 sporadic ELSTs (82%). No other cancer-related genetic pathway was altered except for TERT promoter mutations in two sporadic ELST and one VHL-disease-related ELST (15%). Loss of heterozygosity of chromosome 3 was found in 6/10 (60%) VHL-disease-related and 10/11 (91%) sporadic ELSTs resulting in biallelic VHL inactivation in 8/10 (73%) sporadic ELSTs. DNA methylation profiling did not reveal differences between sporadic and VHL-disease-related ELSTs but reliably distinguished ELST from morphological mimics of the cerebellopontine angle. VHL patients were significantly younger at disease onset compared to sporadic ELSTs (29 vs. 52 years, p < 0.0001, Fisher's exact test). VHL-disease status was not associated with an increased risk of recurrence, but the presence of clear cells was found to be associated with shorter progression-free survival (p = 0.0002, log-rank test)., Conclusion: Biallelic inactivation of VHL is the main mechanism underlying ELSTs, but unknown mechanisms beyond VHL may rarely be involved in the pathogenesis of sporadic ELSTs., (© 2021 The Authors. Neuropathology and Applied Neurobiology published by John Wiley & Sons Ltd on behalf of British Neuropathological Society.)

Rationale: Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm causing oncogenic osteomalacia. Surgery remains the definitive treatment for PMT, and radiotherapy is seldom employed. However, surgery for PMT involving the head and neck is often difficult due to the local invasion and complicated anatomy. We report the first case of PMT, which was successfully treated with the combination of radiotherapy and supplementation of activated vitamin D., Patient Concerns: A 55-year-old woman suffered from pain in the hip and bilateral femur. Serum phosphate and calcium decreased to abnormal levels. Serum alkaline phosphatase and fibroblast growth factor 23 increased to abnormal levels. The hearing loss of the right ear had continued and a middle ear tumor was revealed., Diagnoses: Subsequent biopsy provided the diagnosis of PMT that caused oncogenic osteomalacia. These clinical and pathological characteristics were consistent with and provided the final diagnosis of benign PMT., Interventions: Surgery of the PMT was difficult and the patient underwent radiotherapy. The prescribed dose was 36 Gy in 10 fractions. Simultaneously, the patient started supplementation of 1,25-dihydroxyvitamin D3 (1-2 μg/day) and continued for 2 years., Outcomes: Near-complete resolution of the symptoms was achieved and abnormal laboratory values recovered. At 5 years of follow-up, the irradiated tumor showed no regrowth. Severe hearing loss of the right ear was not observed., Lessons: Radiotherapy was effective for the PMT and could be an important treatment option for inoperable cases., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Mucosal melanoma arising in the middle ear or eustachian tube is uncommon. We present a patient with hearing loss and otalgia found to have mucosal melanoma which occurred in the eustachian tube with extension into the middle ear cavity and external ear canal. Otologic clinics was consulted and biopsy of the mass located at the external canal was performed to ascertain the pathological diagnosis. The patient refused immunotherapy and surgery instead of undergoing radiotherapy and died from hepatic metastasis 8 months later. The mucosal melanoma originated from the eustachian tube with extension into the external ear canal is exceedingly rare, and the differential diagnosis should be considered for tumors in external ear canal.

Purpose: Intralabyrinthine schwannomas (ILS) are rare, benign, slow-growing tumors arising from schwann cells of the cochlear or vestibular nerves within the bony labyrinth. This study provides insight into the management of this rare tumor through a large case series., Materials and Methods: After Institutional Review Board approval, a retrospective chart review was performed of all ILS patients treated at our institution between 2007 and 2019., Results: 20 patients (9 male, 11 female) with ILS were managed at our institution. The right ear was affected in 9 patients (45%) and the left in 11 (55%). Subjective hearing loss was endorsed by all 20 patients. Average pure tone average at presentation was 72 dB nHL. Nine tumors (45%) were intravestibular, 6 (30%) were intracochlear, 4 (20%) were transmodiolar and 1 (5%) was intravestibulocochlear. Hearings aids were used in 3 patients (15%), BiCROS in 2 (10%), CI in 2 (10%), and bone conduction implant in 1 (5%). Vestibular rehabilitation was pursued in 5 patients. Surgical excision was performed for one patient (5%) via translabyrinthine approach due to intractable vertigo. No patients received radiotherapy or intratympanic gentamicin injections., Conclusion: ILS presents a diagnostic and management challenge given the similarity of symptoms with other disorders and limited treatment options. Hearing loss may be managed on a case-by-case basis according to patient symptoms while vestibular loss may be mitigated with vestibular therapy. Surgical excision may be considered in patients with intractable vertigo, severe hearing loss with concurrent CI placement, or in other case-by-case situations., (Copyright © 2021. Published by Elsevier Inc.)

Middle ear (ME) benign tumors are rare, and among them is meningioma. An ME meningioma might be isolated or merely a lateral extension of a CPA meningioma. We report a case with presentation of ME effusion followed by the appearance of an aural polyp after repeated myringotomies. Computed tomography (CT) revealed a benign-looking ME and mastoid mass. After debulking and biopsy, it turned out to be a meningioma. However, when MRI was performed, a large CPA meningioma was detected. ME masses are rare; however, they might be encountered, and CT must be performed followed by biopsy or total removal. In case of detection of a tumor with probable intracranial connection as meningioma, an MRI should be performed to exclude intracranial extension.

Background: Definitive treatment of primary middle ear lymphoepithelial carcinoma (LEC) is not well established owing to the rarity of this disease entity. We report a case of locally advanced primary middle ear LEC treated with concurrent chemoradiation, with good oncologic outcomes., Case Presentation: A 46 year-old female of Cantonese (Southern Chinese) descent presented with a four-month history of left sided hearing loss and non-pulsatile tinnitus, associated with progressive ipsilateral facial weakness. She had a left facial palsy (House-Brackmann 2) which then deteriorated to complete palsy over 2 weeks. Otoscopic examination of the left ear revealed a red-hued mass replacing the tympanic membrane. There was no cervical lymphadenopathy. Fibreoptic nasoendoscopy was unremarkable. Pure tone audiometry revealed profound mixed left hearing loss with type B impedance. Computed tomography of the temporal bone showed an ill-defined left middle ear mass with erosion of the malleus, tegmen tympani and facial canal. Magnetic Resonance Imaging showed an avidly enhancing lesion involving the dura of the left middle cranial fossa, tympanic and labyrinthine portions of the facial nerve. This mass extended into the apex of the left internal acoustic canal and basal turn of the cochlea. Histopathology confirmed EBV-positive primary middle ear LEC. Concurrent chemoradiation comprising 70Gy of intensity-modulated radiation therapy and 3 cycles of concurrent Cisplatin based chemotherapy over a 6 week period was administered. The patient achieved near-complete disease resolution on 3 month post-treatment imaging. Serum EBV DNA titres declined to undetectable levels and the patient is disease-free at 18 months post-diagnosis., Discussion and Conclusion: Concurrent chemoradiation with curative intent may be a viable treatment option for locally advanced middle ear LEC not amenable to surgical resection due to expected surgical morbidity. It confers good oncologic outcomes that mimic the response in other head and neck EBV-related lymphoepithelial carcinomas.

Background: Angiomyolipoma (AML) is a common, chronic disease usually localized in kidney and liver organs; but occurring frequently outside the kidney or liver., Case Presentation: We describe an unusual case of a 62-year-old female patient with AML in the middle ear. She presented with left earache, tinnitus and hearing loss. Preoperative computed tomography and magnetic resonance imaging seemed to reveal a middle ear cholesteatoma. The patient underwent surgical resection of this lesion, and the AML was finally confirmed by histopathological examination. The patient was discharged on the 8th postoperative day and did not seek further treatment., Conclusions: Extraperitoneal AML is rare and accurately identified by histopathology. The recommended management is surgery for AML in the middle ear.

Objectives: The role of traditional nuclear medicine studies in the management of malignant otitis externa (MOE) is unclear and there are ongoing debates regarding their diagnostic value. The authors perform a systematic review and meta-analysis to assess the sensitivity and specificity of traditional nuclear medicine studies in the diagnosis of MOE., Methods: In accordance with PRISMA guidelines, a query of the Medline, Embase, Web of Science, and Cochrane databases was undertaken. The primary outcomes of interest were the sensitivity and specificity of traditional nuclear medicine studies to detect MOE., Results: Of the initial 1317 hits from the four databases, 20 articles with a combined 608 patients were ultimately included in the review. The pooled sensitivities for Technetium-99 and Gallium-67 were 85.1% (95% CI, 72.0-98.1%) and 71.2% (95% CI, 55.1-87.3%) respectively. The available evidence suggested poor specificity of these modalities, but was insufficient for meta-analysis. Neither modality was shown to be effective in the assessment of disease resolution., Conclusion: The sensitivities of Technetium-99 and Gallium-67 to detect MOE are less favorable than was initially thought. Given this finding and their poor specificity, lack of anatomic resolution, unproven ability to detect disease resolution and variable availability, this review does not support the routine use of these studies in the management of MOE., Level of Evidence: N/A Laryngoscope, 130:1812-1816, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Background: Endolymphatic sac tumor (ELST) is an extremely rare disease that does not have established clinical guidelines. Objective: To provide guidance for the diagnosis and management of ELSTs. Methods: We retrospectively reviewed the medical records of patients who were suspected to have a lesion in the location of endolymphatic sac. Seven patients were included. Histopathologic diagnosis confirmed five ELSTs and two other diseases. Results: All of the enrolled patients had hearing impairment. Facial palsy was found in four out of five patients with ELST, whereas none of the patients in other disease group showed facial palsy. CT and MRI findings were consistent with those in previous literatures; however, angiographic findings were not consistent with those previously reported. All patients underwent surgery. Recurrence occurred in two patients with ELST, although there was no visible remnant tumor after the operation. These patients were treated with stereotactic radiosurgery. Conclusion: Hearing impairment and facial palsy were representative symptoms of ELST. All ELSTs showed 'salt and pepper' signal in MRI T1-weighted images, and smaller tumors could have blood supply from AICA in the radiologic study. Complete surgical resection with optional stereotactic radiosurgery should be considered in ELST.HighlightsHearing impairment and facial weakness are remarkable symptoms of ELST compared to other diseases that invade the endolymphatic sac.All ELSTs showed 'salt and pepper' signal in MRI T1-weighted images.ELST less than 3 cm in diameter could have blood supply from AICA.A complete surgical excision with optional stereotactic radiosurgery is required.

Introduction: The case report describes an atrial dissociation in a 9-year-old male boxer diagnosed by ECG and echocardiography. Torticollis, severe neck pain and auscultatory arrhythmia were found during the clinical examination. In addition, a mass was found in the left tympanic bulla and histologically a carcinoma of the middle ear was diagnosed.

Objective: To date, prediction models for estimating risk of acquiring non-serviceable hearing in subjects with observed vestibular schwannoma (VS) have evaluated outcomes primarily based on features at initial diagnosis. Herein, we evaluate the association of rate of hearing decline during the initial period of observation with time to non-serviceable hearing. If significant, rate of hearing decline may inform decision making after an introductory period of observation., Setting: Two tertiary care centers., Patients: VS patients with serviceable hearing who underwent at least three audiograms and two magnetic resonance imaging (MRI) studies before intervention or being lost to follow-up. The rate of change in pure-tone average (PTA) and word recognition score (WRS) was calculated as the score from the second audiogram minus the score from the first audiogram, divided by the duration in months between the two., Main Outcome Measure(s): Serviceable hearing, defined as PTA ≤50 dB HL and WRS ≥50%., Results: Among 266 patients meeting inclusion criteria, 52 developed non-serviceable hearing at last follow-up. Kaplan-Meier estimated rates of maintaining serviceable hearing (95% CI; number still at risk) at 1, 3, 5, 7, and 10 years were 97% (95-100; 206), 78% (72-85; 98), 68% (60-77; 39), 60% (50-73; 17), and 44% (29-67; 2), respectively. In a univariable setting, each 1 dB increase per month in the rate of initial PTA change was associated with a 96% increased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 1.96; 95% CI 1.44-2.68; p < 0.001). Each 1% increase per month in the rate of initial WRS change was associated with a decreased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 0.79; 95% confidence interval [CI] 0.66-0.94; p = 0.009). After multivariable adjustment, both rate of PTA change (HR 2.42; 95% CI 1.72-3.41; p < 0.001) and rate of WRS change (HR 0.81; 95% CI 0.67-0.99; p = 0.043) remained statistically significantly associated with time to non-serviceable hearing., Conclusion: Rate of early PTA and WRS decline during the initial period of observation are significantly associated with time to development of non-serviceable hearing. This information may facilitate accurate patient counseling and inform decision-making regarding prospective disease management.

Objective: Metastatic lesions to the internal auditory meatus (IAM) and/or the cerebellopontine angle (CPA) are rare and may appear like a vestibular schwannoma (VS). We herein raise the issue of the diagnosis and treatment of nine malignant cases of the CPA and IAM among three referral centers in France and Japan. The aim of this study was 1) to report malignant lesions of the CPA, their diagnosis and treatment, 2) to review the literature, 3) to propose criteria of suspicion for malignant tumors of the CPA., Methods: Nine patients who had malignant lesions of the CPA and/or IAM for whom the final diagnosis was made by surgery, lumbar puncture, or PET scan were included. The main outcomes measured were: rapid onset of symptoms, association of cochlea-vestibular symptoms with facial palsy, and MRI analysis., Results: Among the nine patients with malignant tumor of the CPA, 8 of them (89%) had a facial palsy associated with cochlea-vestibular symptoms. Rapid growth of the tumor was observed in 77% (7/9) of the cases in a mean time interval of 4.6 months. The initial diagnosis evoked was VS in 44% of the cases (4/9). Atypical MRI aspect was seen in 67% of the cases (6/9) with bilateral tumors in 55% of cases (5/9)., Conclusion: Although rare, malignant tumors of the CPA and/or IAM should be evoked in case of association of cochleovestibular symptoms and facial palsy, rapid onset and atypical MRI aspect.

Summary The patient developed repeated itching and scabbing at the mouth of the left external auditory canal 5 years ago. In the last two years, the tumor is enlarged. After admission, the left external auditory canal can be seen as a reddish mass, brittle and easy to bleed. CT of temporal bone showed that the soft tissue shadow of left external auricle and external auditory canal was thickened. Postoperative pathological findings: (left external auditory canal) basal cell squamous cell carcinoma. According to the history, physical examination and laboratory examination, the diagnosis is considered as basal squamous cell carcinoma of the external auditory meatus., 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.)

Rhabdomyosarcoma is a rare malignant tumour of striated muscle accounting for 3% of all primary soft tissue tumours of the middle ear cavity. We describe a unique case of a rhabdomyosarcoma of the middle ear cavity in a four-year-old boy, presenting as an otherwise asymptomatic ipsilateral Horner's syndrome. This case involved tumour erosion through the tensor tympani semicanal, accessing the carotid canal and the sympathetic plexus running through it. This child was treated successfully with chemotherapy and proton beam therapy, and remains disease free at six years of follow-up. This case highlights the importance of investigating patients with non-resolving ear infections, polyps or granulation tissue.

Competing Interests: Die Autoren geben an, dass kein Interessenkonflikt besteht.

The aim of the study was to present a case of small cell carcinoma of external auditory canal and associated facial nerve paralysis. Extrapulmonary small cell carcinoma from the head and neck is rarely described and carries a poor prognosis. To our knowledge, this is the first case report describing small cell carcinoma of the EAC in our nationality. This study includes a case report and review of the literature., 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 prognosis of advanced temporal bone cancer is poor, because complete surgical resection is difficult to achieve. Chemoradiotherapy is one of the available curative treatment options; however, its systemic effects on the patient restrict the use of this treatment. A 69-year-old female (who needed peritoneal dialysis) presented at our clinic with T4 left external auditory canal cancer and was treated with cetuximab plus radiotherapy (RT). The primary lesion showed complete response. The patient is currently alive with no evidence of disease two years after completion of the treatment and does not show any late toxicity. This is the first advanced temporal bone cancer patient treated with RT plus cetuximab. Cetuximab plus RT might be a treatment alternative for patients with advanced temporal bone cancer., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Schneiderian papillomas (ISP) of the middle ear are uncommon conditions, with only 45 cases published within literature. They are locally aggressive tumours, with a high rate of recurrence and associated malignancy. We present a rare case of a 53-year-old man presenting with unilateral pulsatile tinnitus, otorrhoea, aural fullness, pruritis and hearing loss. Angiography was employed to exclude a glomus tumour and the patient underwent a modified radical mastoidectomy. Tissue samples confirmed a histological diagnosis of ISP of the middle ear. Follow-up magnetic resonanc imaging one year postoperatively showed no evidence of disease recurrence.

Objective: To apply regenerative treatment for tympanic membrane (TM) perforation to patients with severe calcification of the TM, cholesteatomas, or tumors localized to the tympanic cavity., Study Design: Controlled clinical pilot study., Setting: General hospitals., Patients: Forty-five patients (age: 8-85; M = 19, F = 26) were selected from patients with or without TM perforation for the regenerative treatment. Twenty-five patients had cholesteatomas, 3 had tumors, and 17 had severe TM calcification. Patients were classified into three groups based on TM perforation size: less than 1/3 of the TM as Grade I (n = 5), 1/3 to 2/3 as Grade II (n = 19), and over 2/3 as Grade III (n = 21). Twenty patients who underwent standard tympanoplasty type I were selected as historical controls., Materials and Methods: Materials for the TM repair included gelatin sponge with basic fibroblast growth factor and fibrin glue. After lesions were removed through the TM perforation, gelatin sponge immersed in basic fibroblast growth factor was placed over the perforation. Fibrin glue was then dripped onto the sponge. Treatment efficacy was evaluated 6 months posttreatment., Results: Complete closure of the TM perforation was achieved in 91% (n = 41/45) of the patients in this regenerative treatment. Improvement in average hearing levels and air-bone gap were much better with this treatment than in the historical control group., Conclusion: This new regenerative therapy is useful not only for patients with simple TM perforations but also for those with cholesteatomas, tumors, or severe calcification without requiring conventional surgical procedures. This regenerative therapy is an easy, safe, cost-effective, and minimally-invasive treatment.

Objective: To summarize the clinical characteristics and therapeutic experiences of the middle ear teratoma in infants. Methods: Three cases of middle ear teratoma, from 2012-2015 in Beijing Friendship Hospital were analyzed. Results: The three cases all developed slowly and presented unilateral otorrhea and hearing loss. Otoscopy showed the granulation tissue in the external ear canal. Audiological changes varied according to the degree of severity. Imaging features showed the pocket-like occupancy lesions in the Eustachian tube area. The temporal bone CT showed mass with soft tissue density usually involved in the mastoid and tympanic cavity. MRI showed mixed signal intense on both T1 and T2 weighted imaging. All the three cases received neoplasm resection of the middle ear. Only one case received tympanoplasty surgery at the same time. And all the pathology results displayed mature teratoma. The follow-up time was 17 to 54 months. MRI showed complete removal of the tumor. Conclusions: Teratoma are rare in the head and neck neoplasm. When the infants suffer from the unilateral otorrhea, hearing loss, and granulation tissue formed in the external ear canal, it should be vigilant for teratoma. The differential diagnosis is middle ear cholesteatoma, congenital first branchial cyst or fistula, and middle ear carcinoma. Temporal bone CT combined with MRI could improve the accuracy of diagnosis. It should be totally resection as soon as possible if there is no contraindication. Postoperative follow-up and imaging examination are necessary to eliminate tumor recurrence.

Nodular fasciitis is a benign myofibroblastic tumor. Its uncommon variant, proliferative fasciitis (PF), can present in an even less common intradermal form. We report a case of intradermal PF of the ear in a 45-year-old man who presented with recurrent episodes of pain and swelling of the lesion. Histologic examination showed a dermal, nodular proliferation of ganglion-like basophilic fibroblasts with prominent nuclei and nucleoli, admixed with foamy histiocytes and areas of spindle cells arranged in intersecting fascicles in a fibromyxoid background. Lesional cells stained positive for smooth muscle actin and were negative for AE1/AE3, p63, and Melan-A. CD68 highlighted intervening histiocytes. We postulate that the underlying chondrodermatitis nodularis helicis was a triggering etiology, consistent with the current speculation that intradermal PF results from trauma.

Objective: To highlight a case from 1960 connecting endolymphatic sac tumour and von Hippel-Lindau disease., Case Report: In 1960, a 24-year-old woman presented with unilateral hearing loss, pulsatile tinnitus and a mass visible on otoscopy. The patient underwent surgical biopsy, which was complicated by haemorrhage, and ultimately resulted in death. At autopsy, a destructive temporal bone neoplasm with cystic and papillary architecture was observed that had eroded into the otic capsule. Intra-abdominal lesions consistent with von Hippel-Lindau disease were also observed, and the surgeon postulated a connection between endolymphatic sac tumour and von Hippel-Lindau disease., Method: A review of the literature was carried out using PubMed., Conclusion: Endolymphatic sac tumours are rare neoplasms of the temporal bone that can occur sporadically or as part of von Hippel-Lindau disease. The connection between endolymphatic sac tumour and von Hippel-Lindau disease was first proposed in 1988 and formalised in 1997. We believe that this case represents the first documented connection between endolymphatic sac tumour and von Hippel-Lindau disease.

Hypothesis: Cochlear dysfunction is not common in human meningioma of the internal auditory canal., Background: Meningiomas arising from the cerebellopontine angle and internal auditory canal typically cause hearing loss. Cochlear dysfunction is known to contribute to sensorineural hearing loss induced by vestibular schwannoma, the most common tumor of the internal auditory canal. Detailed cochlear histopathology in meningioma has not been reported., Methods: Retrospective analysis of cochlear histopathology in five unoperated and five operated meningiomas of the internal auditory canal identified after screening human temporal bone collections from three academic medical centers., Results: While some dysfunction of all analyzed cochlear cell types was identified, a predominant or exclusive loss of hair cells was not observed in any meningioma. Only 14.3% of temporal bones showed significantly more hair cell damage on the side of the tumor when compared with the contralateral ear; cochlear neuronal damage was more prevalent in meningiomas. The incidence of hydrops, perilymphatic precipitate, or endolymphatic precipitate was low., Conclusions: Substantial cochlear damage in human meningioma of the internal auditory canal is not common. This may explain the anecdotal hearing improvement observed after surgical resection of meningioma. Our findings underline the importance of developing therapeutic strategies to prevent cochlear neuronal degeneration due to tumors of the internal auditory canal.

Tympanic paragangliomas are common benign tumors of the middle ear, made up of neuroendocrine cells dispersed along the major vascular axes of the head, the neck and the vertebral column. The revealing symptoms are hearing loss and a pulsatile tinnitus. Otoscopy often shows pulsatile retrotympanic reddish mass. MRI and somatostatin analogue scintigraphy (OctreoScan) are the imaging modality of choice. OctreoScan allows physicians to detect other tumor locations. The dosage of urinary methoxyl derivatives is determined, complemented by the screening of other components of multiple endocrine neoplasias (MEN). Biopsy is contraindicated because of the risk of hemorrhage. Surgery is the treatment of choice. We report the case of F. A., a 75-year old patient, admitted with tympanic paraganglioma revealed by hearing loss in his right ear (occurred 4 years before) associated with pulsatile tinnitus concomitant with the heart beating in his ear. He underwnt otoscopy showing retrotympanic reddish pulsatile mass in his right ear (A). (B) MRI of the brain showed total involvement of the middle ear and of the mastoid cells, without identifiable tumoral process, with distinct hyperintensity on diffusion-weighted imaging and strong contrast-enhancement. MEN assessment was without abnormalities, including 24h methoxyl derivatives, parathyroid hormone and calcitonin. The diagnosis of paraganglioma was retained because of the strong contrast-enhancement. OctreoScan was indicated before surgery.

Endolymphatic sac tumour (ELST) is infrequent, as emerges from small series reported in the literature. It is a slow-growing malignancy with local aggressiveness and a low risk of distant metastases. It is often misdiagnosed because of the late onset of symptoms and difficulty in obtaining a biopsy. Its frequency is higher in von Hippel-Lindau (VHL) disease (a genetic systemic syndrome involving multiple tumours), with a prevalence of around 25%. The diagnosis is based on radiology, with specific patterns on contrast-enhanced MRI and typical petrous bone erosion on bone CT scan. Our experience of ELST in the years between 2012-2015 concerns 7 cases, one of which was bilateral, in patients with VHL disease. Four of the 7 patients underwent 5 surgical procedures at our institution. Each case is described in detail, including clinical symptoms, and the intervals between symptom onset, diagnosis and therapy. Postoperative morbidity was low after early surgery on small tumours, whereas extensive surgery for large tumours was associated with loss of cranial nerve function (especially VII, IX, X). The critical sites coinciding with loss of neurological function were the fallopian canal, jugular foramen, petrous apex and intradural extension into the posterior cranial fossa. Early surgery on small ELST is advocated for patients with VHL disease, in whom screening enables a prompt diagnosis and consequently good prognosis., (© Copyright by Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale, Rome, Italy.)

Objectives: (1) Report a rare case of translabyrinthine resection of a sporadic vestibular schwannoma (VS) and concurrent cochlear implantation (CI). (2) Discuss pre-, intra-, and post-operative considerations in this unique patient population. (3) Describe surgical and audiologic outcomes reported in this population., Methods: Case report and review of the literature. PubMed search 'Cochlear Implantation'[Mesh] AND ('Neuroma, Acoustic'[Mesh] OR 'VESTIBULAR SCHWANNOMA'[All Fields] OR 'SCHWANNOMA'[All Fields]) limited to humans and English language. Returned 64 search results, abstracts and references of relevant papers reviewed., Results: A 75-year-old male with longstanding history of slowly progressive severe hearing loss and tinnitus presented for evaluation of worsening imbalance, vertigo, and nausea. Workup revealed a 7 mm right intracanalicular mass on MRI concerning for vestibular schwannoma. Audiogram showed bilateral, symmetric, severe-to-profound sensorineural hearing loss, with poor open-set speech comprehension while bilaterally aided. He underwent successful concurrent right translabyrinthine resection of his VS with complete preservation of the cochlear nerve and uncomplicated cochlear implantation., Discussion: Literature review revealed few previous reports of simultaneous VS and CI. The vast majority of these were in patients with neurofibromatosis Type 2 in whom auditory outcomes were poor. This patient represents one of the few cases of concurrent translabyrinthine tumor removal and CI for a spontaneous VS., Conclusion: Single-stage cochlear implantation and translabyrinthine tumor resection is a feasible and safe option to consider for auditory rehabilitation in rare situations.

Purpose: Skull base osteomyelitis (SBOM) is an inflammatory process which often arises from malignant otitis externa (MOE); the diffuse skull base and adjacent soft tissue involvement may be mistaken at initial imaging for advanced nasopharyngeal carcinoma (NPC), especially if there is no prior knowledge of MOE, direct spread from the sphenoid sinus or in atypical presentations of MOE. This study aims to evaluate imaging features on MR that may differentiate SBOM from NPC., Materials and Methods: The MR examinations of 26 patients diagnosed with SBOM between January 1996 and January 2013 were retrospectively reviewed. Comparison was also made with the MR images of 22 consecutive patients with newly diagnosed advanced T3 and T4 NPC between July 2011 and August 2012. Imaging features in both conditions were compared, including the presence of a nasopharyngeal bulge, nasopharyngeal mucosal irregularity, lateral extension, architectural distortion (or lack thereof), increased T2 signal and enhancement patterns., Results: The most prevalent findings in SBOM were lateral extension, increased T2 signal in adjacent soft tissues, lack of architectural distortion and enhancement greater than or equal to mucosa. The combination of these 4 findings was found to best differentiate SBOM from advanced NPC, and found to be statistically significant (p<0.001)., Conclusion: We suggest that the combination of lateral extension, increased T2 signal, lack of architectural distortion and enhancement greater than or equal to mucosa is helpful in differentiating SBOM from advanced NPC., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Middle ear adenoma (MEA) is a rare benign tumour. Here we report a case of MEA in a 39-year-old man with hearing loss. Some significant histopathological features of MEA and neuroendocrine differentiation identified by immunohistochemistry are described. The tumour showed remarkable and rare degenerative histological characteristics. There were a few tumour cells which were covered by degenerated tissue. Consequently, following frozen biopsy, the tumour was misdiagnosed as chronic ear inflammation. No recurrence was seen in this patient after 45 months of follow-up; regular clinical and radiological follow-up is necessary since recurrence is possible. A literature review for MEA is presented and this type of tumour is discussed clinically, microscopically and immunohistochemically. The differential diagnosis and associated treatment are described., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Objectives: Intraoperative electrocochleography (ECochG) provides valuable information regarding inner ear function during surgical procedures. Here, we report findings from ECochG collected at three time intervals during the management of a patient with symptoms of inner ear hydrops., Patients: A 38-year-old man with an endolymphatic sac tumor who demonstrated classic Ménière's symptoms., Interventions: Endolymphatic shunt, tumor biopsy, and definitive resection of an endolymphatic sac tumor., Main Outcome Measures: Intraoperative ECochG measurements at three time intervals, using frequency-specific tone bursts and clicks., Results: Across time intervals, the amplitude of ECochG summation potential (SP) responses decreased for tone bursts of all frequencies. On the other hand, the SP response to the click stimulus, and the ratio of the SP to action potential (AP), basically remained unchanged across time points., Conclusions: Results suggest that the absolute amplitude of the SP response to frequency-specific tone bursts may be more sensitive to inner ear hydrops than the traditionally used SP/AP ratio as a response to click stimuli.