Facial surface electromyography: A systematic review on the state of the art and current perspectives.

PURPOSE: Peripheral facial nerve palsy is a severely disabling condition. In current clinical practice, the commonest tools to assess facial palsy are grading scales, digital face image analyses or facial muscle electrophysiology. However, these techniques suffer from subjectivity or invasiveness and cannot be applied as part of a routine clinical assessment. Therefore, novel non-invasive office-based tools are needed. Surface electromyography (sEMG) may potentially fulfill the requirements of objectivity, low examiner-dependence, and minimal invasiveness. The aim of this systematic review is to define the state of the art on the use of sEMG for facial nerve functional assessment. MATERIALS AND METHODS: Pubmed, Scopus and Web of Science databases were systematically searched. The study protocol was registered on PROSPERO in January 2023. The review was conducted according to the PRISMA guidelines. RESULTS: After the application of inclusion-exclusion criteria, 15 manuscripts with adequate relevance to this topic were included in the review. CONCLUSIONS: Facial sEMG represents a potentially useful tool to implement objective quantification of facial nerve function in clinical practice. Given the heterogeneity of methods and analysis in the available studies, sEMG results are hardly comparable. The introduction of methodological guidelines, followed by large prospective studies on well-defined subsets of patients with facial nerve impairment, is advocated.

Remote Check as a tele-health instrument for cochlear implant recipients: Analysis of impact and feasibility of application.

PURPOSE: The number of cochlear implant (CI) surgeries is growing over time, with the risk of overloading CI centers in the post-surgical management. Telemedicine is a possible solution to address this phenomenon. Remote Check (RC) is an application that is specific for CI recipients monitoring. The aim of this study is to evaluate the feasibility of application, potential economic impact and patients' acceptance of RC. METHODS: The study is retrospective investigation, data on clinical, audiological features (from remote and on-site evaluation), and satisfaction surveys of 66 consecutive patients were collected; costs required for RC and for on-site evaluations were recorded as well. RESULTS: One hundred and ninety RC sessions were completed by the patients (2.88 sessions per patient). RC and on-site audiometry significantly correlated except for the 500 Hz frequency. Estimated costs for the Italian National Health System for RC review and on-site evaluations were 1.32€ and 3.49€ per minute, respectively. High satisfaction for RC was reached in 91 % of patients. CONCLUSION: RC revealed to be a reliable, cost-effective and well accepted tool in CI monitoring. This study preliminarily supports the hypothesis that RC could be a valid instrument to reduce CI management overload in the outpatient clinic.

Bilateral simultaneous cochlear implants in children: Best timing of surgery and long-term auditory outcomes.

PURPOSE: Evaluate the hearing outcomes of bilateral deaf children implanted simultaneously and define the most appropriate timing for surgery. MATERIALS AND METHODS: Audiological CI results were retrieved in both the short-term and long-term period and compared by stratifying the patients into different subcohorts according to their age at surgery. Additional data collected were age at implant activation, etiology and timing of onset of deafness, presence of psychomotor delay. RESULTS: fifty-six bilaterally implanted children were included. The short-term outcomes differed significantly when comparing groups of different ages at implantation: younger patients achieved better aided pure tone audiometry results. Considering long-term follow-up, a significant correlation was identified between an early age at implantation and the hearing outcome at ages 2 to 5 years. Perceptive levels were better at 4 years of age in the younger group. No significant differences were found between children implanted at before 12 months and between 12 and 16 months of age. CONCLUSIONS: The results of the analyzed follow-up data support the hypothesis that children implanted at before 24 months are expected to have better hearing performances. Nevertheless, these results are referred to a widely heterogeneous group of patients and the duration of auditory deprivation should be considered.

Diagnostic, Prognostic, and Therapeutic Role for Angiogenesis Markers in Head and Neck Squamous Cell Carcinoma: A Narrative Review.

Despite refinements to diagnostic and therapeutic approaches over the last two decades, the outcome of patients with head and neck squamous cell carcinoma (HNSCC) has not shown substantial improvements, especially regarding those with advanced-stage disease. Angiogenesis is believed to be a turning point in the development of solid tumors, being a premise for mass growth and potential distant dissemination. Cancer-induced angiogenesis is a result of increased expression of angiogenic factors, decreased expression of anti-angiogenic factors, or a combination of both. The assessment of angiogenesis has also emerged as a potentially useful biological prognostic and predictive factor in HNSCC. The aim of this review is to assess the level of current knowledge on the neo-angiogenesis markers involved in the biology, behavior, and prognosis of HNSCC. A search (between 1 January 2012 and 10 October 2022) was run in PubMed, Scopus, and Web of Science electronic databases. After full-text screening and application of inclusion/exclusion criteria, 84 articles are included. The current knowledge and debate on angiogenesis in HNSCC presented in the eligible articles are stratified as follows: (i) diagnostic markers; (ii) prognostic markers; (iii) predictive markers; and (iv) markers with a potential therapeutic role. Angiogenesis is a biological and pathological indicator of malignancies progression and has negative implications in prognosis of some solid tumors; several signals capable of tripping the "angiogenic switch" have also been identified in HNSCC. Although several studies suggested that antiangiogenic agents might be a valuable adjunct to conventional chemo-radiation of HNSCC, their long-term therapeutic value remains uncertain. Further investigations are required on combinations of antiangiogenic agents with conventional chemotherapeutic ones, immunotherapeutic and molecularly targeted agents in HNSCC. Additional data are necessary to pinpoint which patients could benefit most from these treatments.

Tumor Microenvironment in Sporadic Vestibular Schwannoma: A Systematic, Narrative Review.

Although diagnosis and treatment of vestibular schwannomas (VSs) improved in recent years, no factors have yet been identified as being capable of predicting tumor growth. Molecular rearrangements occur in neoplasms before any macroscopic morphological changes become visible, and the former are the underlying cause of disease behavior. Tumor microenvironment (TME) encompasses cellular and non-cellular elements interacting together, resulting in a complex and dynamic key of tumorigenesis, drug response, and treatment outcome. The aim of this systematic, narrative review was to assess the level of knowledge on TME implicated in the biology, behavior, and prognosis of sporadic VSs. A search (updated to November 2022) was run in Scopus, PubMed, and Web of Science electronic databases according to the PRISMA guidelines, retrieving 624 titles. After full-text evaluation and application of inclusion/exclusion criteria, 37 articles were included. VS microenvironment is determined by the interplay of a dynamic ecosystem of stromal and immune cells which produce and remodel extracellular matrix, vascular networks, and promote tumor growth. However, evidence is still conflicting. Further studies will enhance our understanding of VS biology by investigating TME-related biomarkers able to predict tumor growth and recognize immunological and molecular factors that could be potential therapeutic targets for medical treatment.

Facial nerve sacrifice in lateral approaches to the skull base: Simultaneous reconstruction by graft interposition.

PURPOSE: To evaluate the outcome of facial nerve (FN) cable graft interposition in lateral skull base surgery. MATERIALS AND METHODS: A group of 16 patients who underwent FN graft interposition procedure was retrospectively considered. Postoperative FN function was evaluated using the House-Brackmann (HB) grading system, the Sunnybrook Facial Grading System (SFGS), the Facial Disability Index (FDI) and the Oral Functioning Scale (OFS) questionnaires. RESULTS: 56.2% of patients had a good postoperative FN outcome (HB grade II-III). Postoperative electromyography (EMG) showed re-innervation potentials in 60% of patients; median age of these patients was significantly lower compared to who did not manifest re-innervation (p = 0.039). CONCLUSION: FN primary reconstruction remains the advisable rehabilitative option when the nerve is interrupted during lateral skull base surgeries, allowing to satisfactory postoperative results in more than half of patients. EMG confirmed the restoring of nerve conduction and it was more frequent in younger patients. The SFGS, the FDI and the OFS are important tools especially in the setting of a rehabilitation program.

Cochlear implant in vestibular schwannomas: long-term outcomes and critical analysis of indications.

PURPOSE: To describe our institutional experience in cochlear implantation after vestibular schwannoma (VS) resection, and compare the audiological outcomes between sporadic and neurofibromatosis type 2 (NF2) VS sub-cohorts of patients, and in relation to preoperative contralateral hearing. METHODS: Seventeen patients (8 sporadic and 9 NF2-associated VSs) who had undergone VS resection and cochlear implant (CI) were analyzed retrospectively. Audiological outcomes at 24 months were correlated with preoperative clinical variables. The results according to VS type (sporadic vs. NF2-associated) and contralateral hearing (impaired vs. normal) were compared. RESULTS: Fourteen CIs were actively used by the patients (77.8%). Twenty-four months after CI activation, the median postoperative PTA (pure tone average) was 45.6 dB nHL and a measurable WRS (Word Recognition Score) was achieved by 44.4% of patients (median WRS = 40%). The median postoperative PTA in the implanted ear resulted better in the group with an impaired contralateral hearing (36.3 dB nHL vs. 78.8 dB nHL, p = 0.019). Good preoperative contralateral hearing status (A-B classes of AAO-HNS) was a negative prognostic factor for CI performance on open-set discrimination (OR = 28.0, 95% CI 2.07-379.25, p = 0.012). CONCLUSIONS: CI is a viable rehabilitative option for patients with sporadic or NF2-associated VS. A good contralateral hearing adversely affects CI outcome and should be taken into consideration for patients' selection and rehabilitation programs.

Temporal Bone Squamous Cell Carcinoma: Molecular Markers Involved in Carcinogenesis, Behavior, and Prognosis: A Systematic Review.

Temporal bone squamous cell carcinoma (TBSCC) is an uncommon malignancy with a poor prognosis in advanced cases. The dismal outcome of advanced TBSSC cases is largely due to the cancer's local aggressiveness and the complex anatomy of this region, as well as to persistent pitfalls in diagnosis and treatment. Molecular changes occur in malignancies before any morphological changes become visible, and are responsible for the disease's clinical behavior. The main purpose of this critical systematic review is to assess the level of knowledge on the molecular markers involved in the biology, behavior, and prognosis of TBSCC. A search (updated to March 2022) was run in PubMed, Scopus, and Web of Science electronic databases without publication date limits for studies investigating molecular markers in cohorts of patients with primary TBSCC. The search terms used were: "temporal bone" OR "external auditory canal" OR "ear", AND "cancer" OR "carcinoma" OR "malignancy". We preliminarily decided not to consider series with less than five cases. Twenty-four case series of TBSCC were found in which different analytical techniques had been used to study the role of several biomarkers. In conclusion, only very limited information on the prognostic role of molecular markers in TBSCC are currently available; prospective, multi-institutional, international prognostic studies should be planned to identify the molecular markers involved in the clinical behavior and prognosis of TBSCC. A further, more ambitious goal would be to find targets for therapeutic agents able to improve disease-specific survival in patients with advanced TBSCC.

Liquid Crystalline Nanoparticles Conjugated with Dexamethasone Prevent Cisplatin Ototoxicity In Vitro.

The conjugation of drugs with nanoparticles represents an innovative approach for controlled and targeted administration of therapeutic agents. Nanoparticle-based systems have been tested for the inner ear therapy, increasing the drug diffusion and being detected in all parts of the cochlea when locally applied near the round window. In this study, glycerol monooleate liquid crystalline NanoParticles were conjugated with Dexamethasone (NPD), a hydrophobic drug already used for inner ear treatments but defective in solubility and bioavailability. NPD has been tested in vitro in the cell line OC-k3, a model of sensory cells of the inner ear, and the therapeutic efficacy has been evaluated against cisplatin, a chemotherapeutic compound known to induce ototoxicity. After comparing the physical chemical characteristics of NPD to the equivalent naïve nanoparticles, an initial investigation was carried out into the nanoparticle's uptake in OC-k3 cells, which takes place within a few hours of treatment without causing toxic damage up to a concentration of 50 µg/mL. The NPD delivered the dexamethasone inside the cells at a significantly increased rate compared to the equivalent free drug administration, increasing the half-life of the therapeutic compound within the cell. Concerning the co-treatment with cisplatin, the NPD significantly lowered the cisplatin cytotoxicity after 48 h of administration, preventing cell apoptosis. To confirm this result, also cell morphology, cell cycle and glucocorticoids receptor expression were investigated. In conclusion, the NPD system has thus preliminarily shown the potential to improve the therapeutic efficacy of treatments delivered in the inner ear and prevent drug-induced ototoxicity.

Health-Related Quality of Life in Patients With ANCA-Associated Vasculitis and Sinonasal Involvement: A Single-Center Cross-Sectional Study.

BACKGROUND/OBJECTIVE: The aim of this study was to assess the impact of sinonasal morbidity on quality of life (QoL) in antineutrophil cytoplasmic antibody-associated vasculitis (AAV). METHODS: This cross-sectional case-control study enrolled 71 patients-44 AAV cases with (ear, nose, and throat [ENT]-AAV) or without ENT involvement (non-ENT-AAV) undergoing multidisciplinary evaluations and 27 chronic rhinosinusitis (CRS) cases. Three validated QoL questionnaires (Sino-Nasal Outcomes Test-22 [SNOT-22], Nasal Obstruction Symptom Evaluation [NOSE], and Short-Form 36) were administered, and the 3 groups were compared. RESULTS: The ENT-AAV patients were significantly younger (p = 0.01), with less antineutrophil cytoplasmic antibody positivity frequency (p = 0.035) and lower renal involvement (p = 0.003) than the non-ENT-AAV patients.The SNOT-22 questionnaire demonstrated significantly greater sinonasal morbidity in ENT-AAV patients compared with CRS patients (p < 0.001). The NOSE score of ENT-AAV patients was comparable to those of CRS patients, but higher than that of non-ENT-AAV patients (p < 0.001). The SNOT-22 and NOSE scores positively correlated with disease activity (p = 0.037; p = 0.004, respectively). Short-Form 36 domain-by-domain analysis revealed a significantly poorer QoL in ENT-AAV patients, especially with physical functioning being progressively impaired in CRS, non-ENT-AAV, and ENT-AAV patients (p < 0.001). No significant differences in QoL came to light when AAV patients were stratified according to current systemic o local treatments. CONCLUSIONS: The QoL in AAV patients is significantly reduced, especially in the presence of ENT involvement. The AAV-related nasal morbidity is consistent and comparable to that reported by CRS patients. It significantly affects patients' QoL and in particular social functioning, leading to limitation in daily/work activities. Organ-focused questionnaires and multidisciplinary management are warranted to pursue a treat-to-target approach in these patients.

Temporal bone carcinoma: testing the prognostic value of a novel clinical and histological scoring system.

PURPOSE: Our group recently proposed the novel Padova prognostic scoring system for temporal bone carcinoma (TBSCC) that considers: the revised Pittsburgh staging system; radiological dura mater involvement; non-anterior spread (medial, inferior or posterior into the temporal bone and skull base) of T4 tumors; and histological grade. The aim of the present study was to validate this prognostic TBSCC scoring system in a case series selected from the literature. METHODS: A search was run to identify studies on TBSCC reporting the variables included in our score for each patient. Then our system was applied to the data extracted. RESULTS: Only two published investigations reported all the clinical and pathological data required for our scoring system. In one series from the Gruppo Otologico in Piacenza (Italy), a significantly higher recurrence rate (p = 0.008), shorter disease-free survival (DFS) (p = 0.001), higher disease-specific mortality (DSM) (p = 0.006), and shorter disease-specific survival (DSS) (p = 0.004) were associated with scores ≥ 5. Receiver operating curve (ROC) analysis showed an AUC of 0.804 for TBSCC recurrence, and 0.832 for DSM. In a series from Kyushu University Hospital (Japan), a significantly higher DSM (p = 0.018) and shorter DSS (p = 0.021) were associated with scores ≥ 5. ROC analysis showed an AUC of 0.812 for tumor relapse and 0.790 for DSM. CONCLUSION: Our TBSCC Padova scoring system confirmed its validity when applied to the only two international TBSCC series providing the required data. These preliminary results need to be confirmed in a multi-center prospective setting.

Hearing preservation in small acoustic neuroma: observation or active therapy? Literature review and institutional experience.

BACKGROUND: Current imaging modalities enable early diagnosis of a large number of small acoustic neuromas in patients with well-preserved hearing, whose best management is still a debatable matter. METHODS: Comparing the hearing outcome of the various therapeutical approaches, including observation, radiosurgery, or resective surgery, is not easy because of the numerous classifications measuring hearing in different ways. In this review, a literature review was performed and papers selected dealing with small tumors, short- and long-term hearing, tumor control or radical resection, and effect of pre-treatment hearing and size on outcome. Two different surgical institutional series of sporadic vestibular schwannomas provided us comparative data on the outcome of observation alone. RESULTS AND CONCLUSIONS: Our experience suggests that active treatment with hearing preservation surgery, unlike observation alone, offers a better chance of hearing preservation, also enabling a more effective treatment of the tumor and an appropriate rehabilitation with hearing aids or cochlear implants.

Cortactin and phosphorylated cortactin tyr466 expression in temporal bone carcinoma.

PURPOSE: Cortactin is a multidomain protein engaged in several cellular mechanisms involving actin assembly and cytoskeletal arrangement. Cortactin overexpression in several malignancies has been associated with increased cell migration, invasion, and metastatic potential. Cortactin needs to be activated by tyrosine or serine/threonine phosphorylation. The role of cortactin and phosphorylated cortactin (residue tyr466) was investigated in temporal bone squamous cell carcinoma (TBSCC). MATERIALS AND METHODS: Immunohistochemical expression of cortactin and phosphorylated cortactin (residue tyr466) was assessed in 27 consecutively-operated TBSCCs. RESULTS: Several clinicopathological variables correlated with recurrence (pT stage, dura mater involvement), and disease-free survival (DFS) (cT stage, pT stage, pN status, dura mater involvement). Twenty-three of 24 immunohistochemically evaluable TBSCCs were cortactin-positive. Median cortactin expression was 75.0%. Cortactin reaction in the cytoplasm was more intense in carcinoma cells than in normal adjacent tissue. Recurrence and DFS rates did not correlate with cortactin and phosphorylated cortactin (residue tyr466) expression in TBSCC specimens. CONCLUSIONS: Cortactin upregulation in TBSCC supports the conviction that inhibiting cortactin functions could have selective effects on this malignancy. Multi-institutional studies should further investigate the role of cortactin and phosphorylated cortactin in TBSCC, and their potential clinical application in integrated treatment modalities.

Observation and partial targeted surgery in the management of tympano-jugular paraganglioma: a contribution to the multioptional treatment.

The objective of this study was to assess the value of a limited tumor excision in tympano-jugular paragangliomas treated with observation with the goal of preventing, or repairing, a neural (VII cranial nerve) or brain damage and continue an otherwise correct observation. This is a retrospective case review. Each patient was submitted to a complete neuro-radiological work-up for diagnosis including CT, MRI, and angiography if needed of tympano-jugular paraganglioma class C. All the cases were submitted to observation and in 16 cases partial targeted surgery was performed. In 10 cases, the procedure involved a targeted removal of the tumor growing in contact with, or invading, the Fallopius. In four cases, the procedure was a petro-occipital trans-sigmoid approach to remove the intradural portion of tumor producing the picture of brain stem compression. In two cases, there was the excision of the bleeding tumor surfaces in the outer ear canal. Follow-up ranged from 4 to 20 years. Success was considered the maintenance of observation without the change of class of tumor extension. In 13 over 16 cases, the observation could be continued, as it still was the more favorable option between natural and surgical morbidity. In the other three cases, a newly installed paralysis of the 9-10 cranial nerves due to tumor growth involved a correction of the strategy to a radical procedure. Partial targeted surgery was directed to cases submitted to observation. It allowed to prevent, or repair, an impending, or actual damage to the facial nerve or the brain stem and to continue the abstentional treatment by keeping the balance between natural and therapy morbidity in favor of observation.

Temporal bone carcinoma: a first glance beyond the conventional clinical and pathological prognostic factors.

Temporal bone squamous cell carcinoma (TBSCC) is an uncommon, aggressive malignancy with a poor prognosis in advanced cases. The dismal outcome is partially related to: the lack of reliable clinical or pathological prognostic factors and the largely unstandardized surgical and integrated treatments adopted. There is an undeniable need for novel diagnostic/therapeutic strategies to improve the prognosis. The purpose of this critical review was to explore the level of available knowledge concerning the molecular markers involved in the biology of TBSCC that have a prognostic potential. The Pub-Med and Scopus electronic databases were searched without publication date limits for studies investigating molecular markers in cohorts of patients with primary TBSCC. The search terms used were: "temporal bone cancer", "temporal bone carcinoma", "temporal bone malignancy", "ear cancer", "ear carcinoma", and "ear malignancy". We decided preliminarily not to consider series with less than five cases. Nine retrospective case series of TBSCC were found in which different analytical techniques had been used to study the role of several biomarkers (HPV, vimentin, transforming growth factor ß, CD105, RECK, matrix metalloproteinase-9, MASPIN, EBV, p16, TP53 mutation, pSTAT3, relaxin-2). CD105 expression (in tumor vessel endothelial cells) and MASPIN cytoplasmic expression (in carcinoma cells) were, respectively, found directly and inversely related with the neoplasm's recurrence rate. CD105 expression was also inversely related with disease-free survival in TBSCC. A future goal of such analyses should be to ascertain the radio- and chemo-sensitivity profiles of individual TBSCCs, enabling truly personalized therapies. A further, more ambitious goal will be to find targets for therapeutic agents that might prove crucial in improving the disease-specific survival for patients with advanced TBSCC.

Surgical treatment of acoustic neuroma: Outcomes and indications.

AIM: Surgery for acoustic neuroma has developed over the years with a gradual improvement in outcome. The goal of surgery should be cure of the disease and a low morbidity, preserving facial nerve function - and hearing, too, whenever feasible (i.e. in small tumors). The most appropriate approach must be decided case by case. BACKGROUND: Results of microsurgery remain heterogeneous, however, probably due to the different amount of experience gained at different centers. MATERIALS AND METHODS: Surgical series reported in the recent literature (2012-2015) were critically reviewed, along with some older papers with particularly representative series. RESULTS AND CONCLUSION: Considering patients' age, surgical morbidity of different approaches, tumor size and hearing, some principles can be identified on which to base surgical indications with a view to achieving the goals of curing the disease with a low morbidity, preserving facial nerve function and hearing, too, whenever feasible (i.e. in patients with small tumors). Different therapeutic approaches are discussed.

Temporal bone carcinoma: Classical prognostic variables revisited and modern clinico-pathological evidence.

AIM: Prognostic factors, rational management, and the ongoing investigations regarding temporal bone squamous cell carcinoma (TBSCC) have been critically reviewed. BACKGROUND: TBSCC is an uncommon, aggressive malignancy. Although some progress has been made in treating this aggressive tumor, the prognosis in advanced cases remains poor. MATERIALS AND METHODS: A systematic search of the literature for articles published between 2009 and October 2014 was performed using the PubMed (http://www.pubmed.gov) electronic database. RESULTS: Given the particular anatomical site of TBSCC, its prognosis is significantly influenced by any direct involvement of nearby structures. The extent of the primary tumor is generally considered one of the most important prognostic factors and it is frequently related to prognosis even more strongly than N stage. For TBSCC, biomarker investigations in surgical specimens are only just beginning to appear in the oncological literature. CONCLUSION: Given the particular features of TBSCC, the sub-specialty of otologic oncology seems to be emerging as a defined area of practice involving multidisciplinary team comprising oto-neurosurgeons, head and neck surgeons, plastic surgeons, oncologists, radiotherapists, dedicated radiologists, and pathologists.

Recurrent squamous cell carcinoma of the temporal bone: critical analysis of cases with a poor prognosis.

PURPOSE: Temporal bone squamous cell carcinoma (TBSCC) is an uncommon, aggressive malignancy with a significant recurrence rate. We reviewed our experience with recurrent TBSCCs. MATERIALS AND METHODS: Clinicopathological and therapeutic variables potentially associated with disease-free survival (DFS) and disease-specific survival (DSS) were assessed in 17 TBSCC patients who died of their disease after treatment. RESULTS: TBSCC recurrences were treated with surgery in 12 cases (palliative in 11, with curative intent in 1) and palliative chemotherapy in 5; the median DFS and DSS were 6 and 16 months, respectively. The mean DFS and DSS were longer in patients who had primary lateral temporal bone resection (LTBR) rather than subtotal temporal bone resection (STBR) (p=0.0173 and p=0.03, respectively). Patients given non-surgical palliative treatment for recurrences had a longer mean DSS than those who underwent surgery (trend toward significance, p=0.09). CONCLUSIONS: Our results reflect the aggressive nature of TBSCC recurrences. Our findings seem to support the use of non-surgical treatments (chemotherapy, radiotherapy, or specialist palliative care) in patients with loco-regionally advanced recurrent TBSCC. Salvage surgery might be considered for early recurrences when radicality is still achievable. Precise guidelines for the rational follow-up of surgically-treated TBSCCs need to be shared between tertiary centers.

Bilateral chondrosarcoma of the jugular foramen: literature review and personal experience.

Chondrosarcomas (CS) are slow-growing malignant cartilaginous tumors with locally invasive behavior. They account for only 0.15% head and neck neoplasia. There have been no reports in the management of bilateral skull base CS in the literature to date. The synchronous presentation of bilateral CS of the jugular foramen (JF) was diagnosed in a 22-year-old woman with right abducens nerve palsy. Once evaluated the collateral intracranial venous discharge, the lesions were removed in two surgical stages through a bilateral petro-occipital trans-sigmoid (POTS) approach performing a bilateral closure of sigmoid sinus. The patient is disease free 15 years after surgery. No complications occurred. Diplopia improved after excision of the tumor on the right side. A review of relevant English literature was performed. The POTS approach to the JF proved to be safe and effective. Staged radical surgery alone, assessing intracranial venous flow at all stages of surgery, was a valid strategy for bilateral CS, achieving long-term disease control, avoiding early adjuvant radiotherapy, and carrying no complications.