A Pathophysiological Approach to Spontaneous Orbital Meningoceles: Case Report and Systematic Review.

BACKGROUND: Spontaneous orbital cephaloceles are a rare condition. The purpose of this study is to provide a description of a clinical case and to carry out a systematic literature review. METHODS: A systematic review of the English literature published on the Pubmed, Scopus, and Web of Science databases was conducted, according to the PRISMA recommendations. RESULTS: A 6-year-old patient was admitted for right otomastoiditis and thrombosis of the sigmoid and transverse sinuses, as well as the proximal portion of the internal jugular vein. Radiological examinations revealed a left orbital mass (22 × 14 mm) compatible with asymptomatic orbital meningocele (MC) herniated from the superior orbital fissure (SOF). The child underwent a right mastoidectomy. After the development of symptoms and signs of intracranial hypertension (ICH), endovascular thrombectomy and transverse sinus stenting were performed, with improvement of the clinical conditions and reduction of the orbital MC. The systematic literature review encompassed 29 publications on 43 patients with spontaneous orbital MC. In the majority of cases, surgery was the preferred treatment. CONCLUSIONS: The present case report and systematic review highlight the importance of ICH investigation and a pathophysiological-oriented treatment approach. The experiences described in the literature are limited, making the collection of additional data paramount.

Intraoperative Measurement of Insertion Speed in Cochlear Implant Surgery: A Preliminary Experience with Cochlear SmartNav.

OBJECTIVES: The objectives were to present the real-time estimated values of cochlear implant (CI) electrode insertion speed (IS) during intraoperative sessions using the Cochlear Nucleus SmartNav System to assess whether this measure affected CI outcomes and to determine whether real-time feedback assists expert surgeons in achieving slow insertion. METHODS: The IS was measured in 52 consecutive patients (65 implanted ears) using the CI632 electrode. The IS values were analyzed in relation to procedure repetition over time, NRT ratio, and CI audiological outcomes. RESULTS: The average IS was 0.64 mm/s (SD = 0.24); minimum and maximum values were 0.23 and 1.24 mm/s, respectively. The IS significantly decreased with each array insertion by the operator (p = 0.006), and the mean decreased by 24% between the first and last third of procedures; however, this reduction fell within the error range of SmartNav for IS (+/-0.48 mm/s). No correlation was found between IS and the NRT ratio (p = 0.51), pure-tone audiometry (PTA) at CI activation (p = 0.506), and PTA (p = 0.94) or word recognition score (p = 0.231) at last evaluation. CONCLUSIONS: The estimated IS reported by SmartNav did not result in a clinically significant reduction in insertion speed or an improvement in CI hearing outcomes. Real-time feedback of IS could potentially be used for training, but its effectiveness requires confirmation through additional studies and more accurate tools. Implementation of IS assessment in clinical practice will enable comparisons between measurement techniques and between manual and robot-assisted insertions. This will help define the optimal IS range to achieve better cochlear implant (CI) outcomes.

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.

Cochlear implantation after head and neck radiotherapy: A multicentric study and systematic review.

OBJECTIVE: The objective of this study is to assess whether cochlear implantation is feasible in patients treated with radiotherapy of the temporal bone (for diseased other than vestibular schwannoma), in terms of surgical management and auditory outcome. METHODS: A systematic review of the literature was performed, screening PubMed, Embase, and Scopus databases, according to PRISMA criteria, retrieving 12 articles. Moreover, 9 cases coming from a multicentric study involving Padova and Pisa University Hospitals, were added, for a total of 62 cases of irradiated patients receiving cochlear implantation. RESULTS: In our multicentric study we included a total of 9 patients, 6 males, and 3 females (mean age at first cochlear implantation was 53.89 years ± 21.07), while from the literature we considered 53 cases (mean age at first cochlear implantation 48.78 years ± 12.41). We considered for both groups a set of preoperative imaging abnormalities, intraoperative complications and possible postoperative complications. In our cohort, the preoperative pure tone average (PTA) in the implanted ear was 105.6 dB, and the postoperative one was 34.6 dB. In the literature the preoperative PTA was 111.5 dB (when reported), and the postoperative one 52.8 dB. CONCLUSION: Both our experience and the literature suggest that cochlear implantation in an irradiated ear is feasible with a good auditory outcome. Surgical difficulties and fitting challenges can be easily managed by professionals aware of these findings, making these patients good candidates to this rehabilitative option.

Current trends on subtotal petrosectomy with cochlear implantation in recalcitrant chronic middle ear disorders.

Objective: To establish the safety and effectiveness of subtotal petrosectomy with cochlear implantation in patients affected by chronic middle ear disorders to refractory to previous surgical treatments. Methods: A multicentre, retrospective study was conducted on patients affected by recalcitrant chronic middle ear disorders who underwent cochlear implantation in combination with subtotal petrosectomy. Patients' details were collected from databases of 11 Italian tertiary referral centres. Additionally, a review of the most updated literature was carried out. Results: 55 patients were included with a mean follow-up time of 44 months. Cholesteatoma was the most common middle ear recurrent pathology and 50.9% of patients had an open cavity. 80% of patients underwent a single stage surgery. One case of explantation for device failure was reported among the 7 patients with post-operative complications. Conclusions: Subtotal petrosectomy with cochlear implantation is a benchmark for management of patients with recalcitrant chronic middle ear disorders. A single stage procedure is the most recommended strategy. Optimal follow-up is still debated. Further studies are required to investigate the role of this surgery in paediatric patients.

Cochlear Implantation in Intralabyrinthine Schwannoma: Case Series and Systematic Review of the Literature.

Intralabyrinthine schwannomas (ILS) are rare benign tumours arising from the peripheral branches of the cochlear or vestibular nerves in the membranous labyrinth, intracochlear schwannomas being the most frequent ones. When hearing is no longer feasible on the affected side, surgical removal along with simultaneous cochlear implantation can be proposed to the patient. We hereby present a systematic review of the literature on the topic, as well as two original cases from our centre (Ospedale Università degli Studi di Padova). Cochlear implantation in intracochlear schwannomas is feasible, with overall satisfactory hearing outcomes in accordance with the evidence found in the literature.

Intra-Operative Cochlear Nerve Function Monitoring in Hearing Preservation Surgery: A Systematic Review of the Literature.

With the recent scientific and technical developments, hearing preservation surgery is becoming a growing objective in inner ear pathologies, especially for vestibular schwannomas. In this review, we aim to describe the pros and cons of the following cochlear nerve monitoring techniques: ABRs (auditory brainstem responses), DENM (direct eighth cranial nerve monitoring), EcochG (electrocochleography), CNAP (cochlear compound nerve action potentials), DPOAE (distortion product otoacoustic emissions), PAMRs (postauricular muscle responses). The Cochrane library, Scopus, DynaMed, and PubMed databases were screened to obtain any relevant papers from October 2009 to the present day. Due to the heterogeneity of the existing studies in the literature, there is no way to tell whether a technique is better than another. All authors reported satisfactory outcomes with the cochlear nerve monitoring techniques tested, either alone or in combination.

Primary temporal bone squamous cell carcinoma: Comparing the prognostic value of the American Joint Committee on Cancer TNM classification (8th edition) with the revised Pittsburgh staging system.

BACKGROUND: Retrospectively considering a temporal bone squamous cell carcinomas (TBSCCs) series, our aim was to compare the predictive power of the American Joint Committee on Cancer (AJCC) staging system (8th edition) with the revised Pittsburgh staging system (rPSS) in terms of disease-free survival (DFS), disease-specific survival (DSS), and overall survival (OS). METHODS: Forty-three TBSCCs consecutively treated surgically were reviewed. The prognostic performance of AJCC and rPSS was compared. RESULTS: The areas under the curves for the prediction of DFS, DSS, and OS did not differ significantly between both staging systems (p = 0.518, p = 0.940, and p = 0.910, respectively). Harrel's C-indexes for respectively the AJCC and rPSS were 0.76 and 0.70 for DFS, 0.73 and 0.76 for DSS, 0.66 and 0.63 for OS. CONCLUSION: Comparable prognostic accuracy was observed between AJCC and rPSS. Levels of prognostic performance were only acceptable for both systems, according to Hosmer-Lemeshow scale. Further efforts are needed to define new TBSCC staging modalities with higher prognostic reliability.

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.

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.

Temporal bone carcinoma: Novel prognostic score based on clinical and histological features.

BACKGROUND: This study aimed to develop a novel temporal bone squamous cell carcinoma (TBSCC) prognosis scoring system and compare it with the revised Pittsburgh staging system. METHODS: Forty-four consecutive TBSCC patients were assessed to identify predictors of recurrence. Each predictor's hazard ratio for recurrence was used to develop our novel scoring system. RESULTS: Based on variables with P < .10 in Cox's regression model, our score included: revised Pittsburgh stage; non-anterior spread of T4 carcinoma; dural involvement; and histological grade. A higher recurrence rate (P = .000) and shorter disease-free survival (P = .000) were associated with scores of ≥5. The area under the curve of our score was larger than that of the revised Pittsburgh stage for both recurrence and disease-specific mortality (P = .0178 and P = .0193, respectively). CONCLUSION: Our TBSCC scoring system is based on variables that are obtainable preoperatively from clinical and radiological data and biopsies. Its prognostic value should be validated for published TBSCC series and then in prospective settings.

Factors affecting prognosis in locoregional recurrence of oral squamous cell carcinoma.

BACKGROUND: Recurrence of oral squamous cell carcinoma (rOSCC) after primary treatment is associated with poor survival outcomes. Salvage treatment with further surgery, radiotherapy and chemotherapy has high morbidity, making patient selection crucial. However, in the recurrence setting, reliable stratification data are scarce. Decision-making in this context is consequently complex. We investigated factors influencing overall survival after rOSCC. METHODS: Retrospective cohort study of patients with rOSCC (n = 83) at the Queen Elizabeth Hospital Birmingham, UK between 2006 and 2016. Associations with overall survival were analysed using univariate and multivariate analyses to identify important clinical prognostic indicators. RESULTS: Overall survival at 1 year was 32.5% and at 5 years was 18.1% after a median follow-up of 7.4 months. Multivariate analysis identified four independent predictors of overall survival following rOSCC: size of primary tumour (HR 2.077; 95% CI 1.034-4.172), extent of recurrent disease (HR 3.286; 95% CI 1.545-6.991), history of moderate alcohol consumption (HR 0.351; 95% CI 0.162-0.763), and close or positive margins at primary resection (HR 1.955; 95% CI 1.063-3.595). CONCLUSIONS: We identified four key factors that help prognostication and risk stratification of rOSCC. Given the high morbidity associated with salvage treatment, we recommend that the multidisciplinary team (MDT) and the patient weigh these factors carefully when considering further treatment. Further investigation of the biology underlying these oncophenotypes may contribute to better patient stratification.

Surgical timing for bilateral simultaneous cochlear implants: When is best?

INTRODUCTION: Hearing loss is considered the most common congenital disease and the prevalence of neonatal deafness can be estimated between 1 and 2 cases per 1000 live births. Infant deafness must be diagnosed as early as possible and an effective therapeutic intervention needs to be carried out in order to avoid the serious consequences of hearing deprivation during the evolutionary period: alterations in the development of central auditory pathways and lack of language acquisition. The cochlear implant (CI) has proved to be the best instrument to solve the problem of auditory deprivation. In particular, the bilateral CI gives the patient access to binaural hearing which results in benefits in terms of sound localisation and discrimination. The optimal age of application of the CI is a widely discussed topic in the scientific community and the current guidelines indicate a period between 12 and 24 months of age, even though the supporters of the application before 12 months of age are nowadays increasing. MATERIALS AND METHODS: The study is observational, retrospective, monocentric. 49 paediatric patients (<18 years) with simultaneous bilateral CIs were included. The audiometric threshold and speech tests were carried out during the follow-up 3, 6 and 12 months after the CIs activation and when the patient reached 2 years of age. RESULTS: The statistical analysis showed that undergoing bilateral implantation surgery before 2 years of age allows a satisfactory audiometric performance, while there are no particular benefits in performing the surgery before 1 year of age. As far as the speech outcome is concerned, the statistical analysis didn't show significant correlation between the earlier age of implantation and better speech performance if the operation is carried out before 2.5 years of age. CONCLUSIONS: The results of the study indicate that the optimal age to perform the simultaneous bilateral CIs surgery is between 12 and 24 months, without demonstrating any particular benefit in carrying out the procedure before 1 year of age. This may be clinically relevant in terms of avoiding the risks of diagnostic mistakes and reducing the related surgical risk in children under 1 year of age.

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.

A prospective investigation of predictive parameters for post-surgical recurrences in sinonasal polyposis.

Even after appropriate surgical therapy, a significant number of patients with chronic rhinosinusitis with nasal polyps (CRSwNP) experience recurrences. The end-point of this prospective study was to apply univariate and multivariate statistical models to identify clinical, pathological, and laboratory variables that could predict CRSwNP recurrence after endoscopic sinus surgery. The study enrolled 143 patients between 2010 and 2013, who were all treated by the same surgeon for CRSwNP. Twenty-one patients developed recurrent polyposis. The recurrence rate was significantly higher for the eosinophilic than for the non-eosinophilic type (p = 0.020). Among the patients who developed a recurrence, the disease-free interval was significantly shorter for those with eosinophilic-type polyposis (p = 0.003). Univariate statistical analysis disclosed significant associations between CRSwNP recurrence and age (p = 0.035), allergy (p = 0.014), and eosinophilic granulomatosis with polyangiitis (p = 0.01). The multivariate model showed that only histological evidence of the eosinophilic type of CRSwNP retained an independent prognostic significance in relation to recurrent polyposis (p = 0.033). Judging from our results, it is reasonable to consider both stricter follow-up protocols and postoperative adjuvant medical treatments for patients with a histological diagnosis of eosinophilic-type CRSwNP.

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.

Post-operative steroid treatment for eosinophilic-type sinonasal polyposis.

CONCLUSIONS: The preliminary finding of significantly lower eosinophilic-type CRSwNP recurrence rates in patients with associated asthma or ASA intolerance treated post-operatively with local nasal and oral steroids warrants further investigation in larger, prospective series. OBJECTIVE: A high recurrence rate after functional endoscopic sinus surgery (FESS) has been widely reported in patients with eosinophilic-type chronic rhinosinusitis with nasal polyps (CRSwNP). This study compared the influence of two post-operative medical treatments - local nasal steroids alone vs local nasal and oral steroids - on the recurrence of eosinophilic-type CRSwNP. METHOD: Between January 2009-December 2010, 32 patients with eosinophilic-type CRSwNP received local steroid nasal therapy after FESS (Group A); from January 2011 onwards, another 28 patients were treated post-operatively with the same nasal treatment plus two cycles a year of oral steroids (Group B). All patients in both groups had a follow-up of at least 36 months. RESULTS: Groups A and B did not differ significantly in recurrence rate (31.25% vs 32.14%, p = 1) or disease-free interval (p = 0.91), but Group B patients who were asthmatic or ASA-intolerant had a significantly lower recurrence rate than Group A patients with asthma or ASA intolerance (p = 0.04 and p = 0.004, respectively).