Management of Petrous Bone Cholesteatoma: The Gruppo Otologico Experience.

BACKGROUND: The term petrous bone cholesteatoma (PBC) refers to lesions extending deep to the bony labyrinth via superior, inferior, and posterior cell tracts. PBC is a rare incidence accounting for only 4-9% of petrous bone lesions. Lesions of petrous bone represent a real surgical challenge due to its complex relationship with critical neurovascular structures. OBJECTIVE: To demonstrate our 40-plus years' experience in the management of PBC, depict the clinical features of PBC according to Sanna's Classification, evaluate the postoperative follow-up of surgically treated PBC patients, and determine the recurrence rate. STUDY DESIGN: Retrospective medical record review. MATERIAL AND METHODS: Medical records of 298 PBC cases operated from the year 1983 to 2024 were thoroughly evaluated. RESULTS: A total of 298 PBC cases were surgically treated at our center. The average age at presentation in this series was 47 years. Males are more affected than females with a male-to-female ratio of 2.2:1. The most common presenting symptoms were hearing loss (84%), tinnitus (48%), and facial nerve paralysis (45%). Mixed hearing loss (41%) was the commonest audiometric pattern of hearing loss followed by conductive hearing loss (26%) and profound sensorineural hearing loss (4%) and a total of 86 (29%) had anacusis at presentation. On preoperative facial nerve function examination, 133(45%) of patients had various degrees of paresis and complete paralysis whereas 55% had normal HB-I function. The commonest degree of paresis noted was HB-III (18%) followed by HB-VI (5%). A total of 150 (50%) patients had previous otologic surgery and two-thirds of these cases had two or more prior otologic surgeries. According to Sanna's PBC Classification system, we identified that the supralabyrinthine class (44%) is the commonest of all classes followed by massive (33%), infralabyrinthine-apical (9%), infralabyrinthine(8%), and apical (5%) classes in that order. However, only ten patients had congenital type of PBC. Extension to clivus, sphenoid, nasopharynx, intradural space, and occipital condyle was found in 8, 2, 1, and 2 cases respectively. The most commonly used surgical approaches at our center were TO, MTCA with rerouting of the facial nerve, and TLAB with external auditory canal (EAC) closure. Postoperative complications were minimal and the duration of follow-up ranged from one to 458 months with a mean duration of 65 months. Residual lesions were evident in 11 cases (3.7%), with the surgical cavity, middle and posterior fossa dura, and jugular bulb being the commonest sites. CONCLUSION: Petrous bone cholesteatoma represents diagnostically and surgically challenging lesions of temporal bone which are usually frustrating to the treating surgeon. A high index of clinical suspicion, thorough clinical evaluation examination, and preoperative radiologic evaluation make the diagnosis easier. Preoperative anatomic classification of the lesion enables the physician to choose the appropriate surgical approach. Sanna's classification is widely used to classify PBC in relation to the labyrinthine block. Radical disease removal should always come before hearing preservation. Cavity obliteration is the solution to the problems related to a large cavity. Finally, advancements in lateral skull base approaches create adequate surgical access for the complete removal of the lesion with excellent control of critical neurovascular structures.

Long-Term Surgical Outcome of Class A and B Tympanomastoid Paragangliomas.

OBJECTIVE: To analyze the long-term facial function as well as overall postoperative condition in surgically treated tympanomastoid PGL patients. STUDY DESIGN: Retrospective study. METHOD: The medical records of patients with surgically managed class A and B tympanomastoid PGLs between 1983 and 2023 were thoroughly evaluated. RESULT: Our center has treated a total of 213 cases of tympanomastoid PGL surgically. The mean age of patients was 54, and the male-to-female ratio was 1:6. The most common symptoms at presentation were hearing loss (80%), pulsatile tinnitus (77%), and vertigo (15%). According to the modified Fisch classification, 45% of the cases were classified as class A (A1 and A2), while 55% were classified as class B (B1, B2, and B3). All class A and most class B1 and B2 tumors were removed either with transcanal or retroauricular-transcanal approaches. However, more advanced class B3 lesions were removed with subtotal petrosectomy (SP) along with middle ear obliteration. Facial nerve outcome was excellent in all class A and B cases, while chances of postoperative paresis slightly increased with the size and extent of the tumor (p < 0.05). The hearing outcome is excellent for class A1, A2, B1, and B2 tumors, whereas more advanced class B3 cases have a loss of air conduction (AC) and increased bone conduction (BC) threshold (p < 0.05). Complete surgical removal was achieved in 97% of our cases. The most common late complication was permanent TM perforation (7%), and the recurrence rate was 3%. CONCLUSIONS: Tympanomastoid PGL represents the most common neoplasm of the middle ear space. The most frequent presenting symptoms include pulsatile tinnitus and hearing loss, whereas the presence of retrotympanic mass was evident in all cases at the time of initial otoscopic evaluation. Proper documentation of facial function and audiometric evaluation are crucial elements of preoperative workup. The most preferred preoperative radiologic examination is high-resolution computer tomography (HRCT), whereas magnetic resonance imaging (MRI) with or without gadolinium enhancement is reserved for cases with a dilemma of carotid artery or jugular bulb involvement. The main goal of tympanomastoid PGL treatment is complete disease removal with preservation of hearing and facial functions. Surgical treatment remains the preferred treatment modality with the benefits of complete disease removal, lower rate of recurrence and complication, and acceptable postoperative hearing level. Here, we present our 40 years of experience, which, to the very best of our knowledge, is the largest series of tympanomastoid PGL in the English literature.

Tumor progression in tympanojugular paragangliomas: the role of radiotherapy and wait and scan.

INTRODUCTION: Tympanojugular paragangliomas (TJ PGLs) are rare tumors characterized by bone infiltration and erosion and a close relationship with critical structures, such as cranial nerves and internal carotid artery. For these reasons, their management represents a tough challenge. Since the fifties, radio-therapy (RT) has been proposed as an alternative treatment aimed at avoiding tumor progression. However, the indolent nature of the tumor, characterized by slow growth, is a crucial factor that needs to be considered before offering radiation. METHODS: This study aims to examine tumor progression in RT patients through a systematic review of the literature and in TJ PGL patients who underwent solely wait and scan at our department. RESULTS: The rate of tumor progression in the RT group was 8.9%, while in the wait and scan cohort was 12.9%. This data suggests the innate slow growth of PGLs. However, it is not possible to draw certain conclusions because of the wide heterogeneity of the studies. CONCLUSION: When complete surgical excision of TJ PGLs is not feasible, appropriate counseling and patient selection, including comprehensive tumor classification, should be performed before proposing RT to control tumor progression, since wait and scan may represent a reasonable option in selected cases.

Superior petrosal vein sacrifice in translabyrinthine approach for resection of vestibule schwannoma.

PURPOSE: The aim of this study was to evaluate the safety and surgical outcome of superior petrosal vein (SPV, Dandy's vein) sacrifice in translabyrinthine approach (TLA) for resection of vestibule schwannoma (VS) as compared with SPV preservation, with further investigation of preoperational factors associated with the implement of SPV sacrifice. METHODS: The authors prospectively collected data from patients surgically treated for VS through TLA between June 2021 and April 2022 at the Gruppo Otologico. RESULTS: There were 30 and 49 patients in SPV sacrifice and preservation groups, respectively. SPV sacrifice group had significantly larger tumor size (2.46 vs. 1.40 cm), less percentage of solid tumor (26.7% vs. 83.7%), higher incidence of brainstem compression (80% vs. 26.5%), and higher percentage of facial numbness (20.0% vs. 4.1%) than SPV preservation group. Gross total resection (GTR) rates were 73.3% after SPV sacrifice and 87.8% after SPV preservation. Facial nerve preservation rates were similar. No complication related with SPV sacrifice was observed. Logistic regression analysis showed tumor size and complete solid consistency as significant risk factors associated with SPV sacrifice. ROC curve further demonstrated tumor size as a fair predictor (AUC = 0.833), with optimum cutoff value of 1.68 cm. CONCLUSION: SPV sacrifice via TLA as needed is a safe and effective maneuver for removal of relatively large VS. Tumor size and consistency can be used as a guidance in preoperational decision-making, with cutoff value of 1.68 cm and cystic formation as predictive indicators.

Characteristics and Management of Facial Nerve Schwannomas and Hemangiomas.

OBJECTIVES: To characterize facial nerve (FN) schwannomas (FNSs) and FN hemangiomas (FNHs) and their clinical features and management strategies, and to describe the results of cable nerve grafting after FN sectioning during tumor removal. METHODS: This retrospective study included 84 FNS cases and 42 FNH cases managed between July 1989 and July 2020 at a quaternary referral center for skull base pathology. Clinical details, locations, management, and results of cable nerve grafting at 1 year and during an average period of 3.12 years were evaluated. Sural nerve interpositioning was performed for patients who experienced FN paralysis for less than 1 year and underwent nerve sectioning during tumor removal. RESULTS: FNSs more often involved multiple segments compared with FNHs. The cerebellopontine angle and the mastoid segments were involved in 16 (19.1%) and 34 (40.5%) FNS cases, respectively; however, the cerebellopontine angle and the mastoid segments were involved in 0 and 7 (16.7%) FNH cases, respectively. Sectioned nerves of 99 patients (78.6%) were restored using interposition cable grafting. At the last follow-up evaluation, 56.3% of FNSs and 60.7% of FNHs attained House-Brackmann (HB) grade III. Lower preoperative HB grades were associated with poorer postoperative outcomes. For FNSs, the mean HB grades were 4.13 at 1 year postoperatively and 3.75 at the last follow-up evaluation ( p = 0.001); however, for FNHs, the mean HB grades were 4.04 postoperatively and 3.75 at the last follow-up evaluation. Therefore, extradural coaptation yielded better outcomes. CONCLUSION: FNSs can occur along any part of the FN along its course, and FNHs are concentrated around the area of geniculate ganglion. The results of cable inter positioning grafts are better in patients with preoperative FN-HB-III or less when compared with higher grades. The outcome of the interpositioning continues to improve even after 1 year in extradural coaptation.

Late complications of cochlear implant: a case report of necrotizing meningoencephalitis similar to a CPA tumor.

OBJECTIVE: Report a case of localized necrotizing meningoencephalitis as the cause of functional hearing loss after cochlear implant (CI) surgery. CASE REPORT: A 12-year-old with bilateral CI presented to our quaternary center due to severe functional hearing loss after 11 years since left ear CI surgery. CT with contrast was conducted showing a CPA tumor-like mass. Pre-operative computed tomography (CT) scans and magnetic resonance imaging (MRI) performed at the age of 1 year showed no inner ear abnormalities and in particular no evidence of a tumor in the cerebellopontine angle (CPA). CONCLUSION: Following removal of the CI and the mass, histopathological, immunohistochemical and cultural examinations revealed a necrotizing meningoencephalitis, with the CI electrode as the focus.

Targeted metabolomics detects a putatively diagnostic signature in plasma and dried blood spots from head and neck paraganglioma patients.

Head and neck paragangliomas (HNPGLs), rare chemoresistant tumors curable only with surgery, are strongly influenced by genetic predisposition, hence patients and relatives require lifetime follow-up with MRI and/or PET-CT because of de novo disease risk. This entails exposure to electromagnetic/ionizing radiation, costs, and organizational challenges, because patients and relatives are scattered far from reference centers. Simplified first-line screening strategies are needed. We employed flow injection analysis tandem mass spectrometry, as used in newborn metabolic screening, to compare the plasma metabolic profile of HNPGL patients (59 samples, 56 cases) and healthy controls (24 samples, 24 cases). Principal Component Analysis (PCA) and Partial Least Discriminant Analysis (PLS-DA) highlighted a distinctive HNPGL signature, likely reflecting the anaplerotic conversion of the TCA cycle to glutaminolysis and catabolism of branched amino acids, DNA damage and deoxyadenosine (dAdo) accumulation, impairment of fatty acid oxidation, switch towards the Warburg effect and proinflammatory lysophosphatidylcholines (LPCs) signaling. Statistical analysis of the metabolites that most impacted on PLS-DA was extended to 10 acoustic neuroma and 2 cholesteatoma patients, confirming significant differences relative to the HNPGL plasma metabolomic profile. The best confusion matrix from the ROC curve built on 2 metabolites, dAdo and C26:0-LPC, provided specificity of 94.29% and sensitivity of 89.29%, with positive and negative predictive values of 96.2% and 84.6%, respectively. Analysis of dAdo and C26:0-LPC levels in dried venous and capillary blood confirmed that dAdo, likely deriving from 2'-deoxy-ATP accumulated in HNPGL cells following endogenous genotoxic damage, efficiently discriminated HNPGL patients from healthy controls and acoustic neuroma/cholesteatoma patients on easily manageable dried blood spots.

Management of Vestibular Schwannoma with Normal Hearing.

INTRODUCTION: This work aimed to study the management of vestibular schwannoma (VS) patients with normal hearing (NH). METHODS: A retrospective study was undertaken in a Quaternary referral center for skull base pathologies. Among 4,000 VS patients 162 met our strict audiological criteria for NH. These patients were divided into 2 management groups, wait and scan (W&S) (45/162, 25%) and operated patients (123/162, 75%), and 6 patients were included in both groups. RESULTS: Our management strategy achieved the goals for treatment of VS. First goal, all tumors were completely removed except for 2 intentional residuals. Second goal, facial nerve (FN) function preservation (House Brackmann I, II, and III) was 95.9%. Third goal, possible hearing preservation (HP) attempts occurred in (50/122) (40.9%) with an HP rate in 44% of the patients. Additionally, there were only 2 cases of postoperative complications with no CSF leakage. The prospect of HP in NH patients did not differ with respect to tumor size. However, patients with normal preoperative ABR seemed to have better chances of HP and good FN function and vice versa. HP rate was superior for the MCFA as opposed to the RS + RLA. W&S group demonstrated hearing stability in 88.9% of the patients and FN function stability of HB I in 100% of the patients. CONCLUSIONS: Surgical resection is a reasonable and definitive management option for VS with NH. Nevertheless, choosing to manage cases with observation remains an appropriate management option for NH patients. ABR might be considered as an adjuvant tool indicating better prognosis for HP.

Wait and Scan Management of Intra-canalicular Vestibular Schwannomas: Analysis of Growth and Hearing Outcome.

OBJECTIVE: To report on the results of intracanalicular vestibular schwannomas (ICVS) that were managed by wait and scan and to analyze the possible predictors of tumor growth and hearing deterioration throughout the observation period. STUDY DESIGN: A retrospective case series. SETTING: Quaternary referral center for skull base pathologies. PATIENTS: Patients with sporadic ICVS managed by wait and scan. INTERVENTION: Serial resonance imaging (MRI) with size measurement and serial audiological evaluation. MAIN OUTCOME MEASURE: Tumor growth defined as 2 mm increase of maximal tumor diameter, further treatment, and hearing preservation either maintain initial modified Sanna hearing class, or maintain initial serviceable hearing (class A/B). RESULTS: 339 patients were enrolled. The mean follow-up was 36.5±31.7 months with a median of 24 months. Tumor growth occurred in 141 patients (40.6%) either as slow growth (SG) in 26.3% of cases or fast growth (FG) in 15.3% of cases. Intervention was performed in only 64 cases (18.8%). Out of 271 patients who underwent hearing analysis, 86 patients (33.5%) showed hearing deterioration to a lower hearing class of the modified Sanna classification. Tumor growth and older age were predictors of hearing deterioration. Of the 125 cases with initial serviceable hearing (Class A/B), 91 cases (72.8%) maintained serviceable hearing at last follow-up. Tumor growth and a worse initial pure tone average (PTA) were predictors of hearing deterioration. CONCLUSIONS: Wait and scan management of ICVS is a viable option and only 18.8% of patients needed further treatment. Hearing tends to deteriorate over time.

The otologic approach in the management of posterior petrous surface meningiomas.

PURPOSE: Report our experience in the management of posterior petrous surface meningiomas (PPSMs), and identify features that affect hearing, facial nerve (FN) function, and control of the disease. METHODS: Retrospective case series of 131 patients surgically managed for PPSMs. FN status, hearing and tumour radicality were assessed and compared between patients with tumours of different locations (Desgeorges classification) and internal auditory canal involvement (IAC). RESULTS: At the time of surgery 74.8% of patients had a hearing loss. Hearing was mostly unserviceable in tumors attached to the meatus. Pure tone audiometry did not correlate to IAC extension, while speech discrimination scores were statistically worse when the tumor occupied the IAC (unpaired t test, p = 0.0152). Similarly, extrameatal tumors undergoing removal by otic preserving techniques maintained postoperative hearing, whereas hearing worsened significantly in tumors involving the IAC (paired t test, p = 0.048). The FN was affected preoperatively in 11.4% of cases. Postoperative FN palsy was significantly correlated to the IAC involvement (Fisher's exact test, p = 0.0013), while it was not correlated to tumor size. According to the Desgeorges classification, a postoperative FN palsy complicated the majority of anteriorly extending tumors and, two-fifths of meatus centred tumors. 75% of posterior located tumors had a postoperative FN grade I HB. CONCLUSIONS: Since the involvement of the IAC by the tumor affects both hearing and FN function, the IAC is of primary importance in PPSMs and should be studied and addressed as much as the tumor location in the CPA.

Comparison of Lower Cranial Nerve Function Between Tympanojugular Paraganglioma Class C1/C2 With and Without Intracranial Extension: A Four-Decade Experience.

OBJECTIVE: To compare preoperative and postoperative lower cranial nerve (LCN) function between Class C1 and C2 tympanojugular paraganglioma (TJP) with/without intracranial intradural (Di)/extradural (De) extensions, according to the experience of a single surgeon over four decades. STUDY DESIGN: Retrospective review. SETTING: Quaternary referral center for otology and skull base surgery. MATERIAL AND METHODS: A chart review was conducted of all the patients operated for C1/C2 TJPs from September 1983 to December 2018. The tumors were classified as: Limited-Group (C1/C2 without Di/De extensions) and Extended-Group (C1/C2 with Di/De extensions). RESULTS: Of 159 patients, 107 (67.3%) were women; the mean age at surgery was 46.5 years. The Limited-Group (56.6%) comprised C1 (41.1%) and C2 (58.9%) tumors; the Extended-Group (43.4%) comprised C1+Di/De (14.5%) and C2+Di/De (85.5%) tumors. The prevalence of preoperative LCN palsy was 11.9 times higher in Extended than Limited tumors: 61.9% versus 4.9% (p < 0.05). The risk for postoperative LCN palsy was 4.7 times greater in Extended than Limited tumors: 29.2% versus 12.9%, p = 0.01. CONCLUSION: Especially in younger patients, complete removal of Limited C1/C2 tumors, before they extend intracranially, reduces the risk of dysfunctionality of LCNs and the burden of residual tumor. The incidence of new tumors increased over four decades. However, new-postoperative LCN palsy did not occur in any Limited C1/C2 tumors operated after the year 2000, and declined to less than 10% of Extended C1/C2 tumors.

Relevance of Modified Bondy Mastoidectomy in Pediatric Cholesteatoma.

Modified Bondy mastoidectomy is a type of canal wall down mastoidectomy well described in literature for adult patients. We present our experience with the use of modified Bondy mastoidectomy in pediatric population. Using retrospective chart review, pediatric patients, who underwent modified Bondy procedure for attic cholesteatoma between 1983 and 2015 at our quaternary referral center for otology and lateral skull base surgery, were analyzed after obtaining permission from institutional review board. The demographic data, air-bone gap before and after surgery (at a follow up of 1 month, 6 months, 2 year and 5 years), intraoperative findings and postoperative outcomes were recorded. A total of 36 (5.8%) pediatric cholesteatoma patients underwent Modified Bondy procedure. Out of these, 5-year follow up was available for 31 patients and they were included in audiological analysis. Air-bone gap was maintained at preoperative levels or improve in all the patients during follow up and there were no incidences of sensorineural hearing loss. Two patients (5.5%) were found to harbor residual cholesteatoma and two patients (6.4%) of the 31 patients who had follow up of 5 years, developed recurrent disease during follow up. Modified Bondy procedure, when done in appropriately selected patients, is a reliable, effective and reasonably safe technique for pediatric cholesteatoma.

Long term outcomes of canal wall up and canal wall down tympanomastoidectomies in pediatric cholesteatoma.

Cholesteatomas in children have a more aggressive growth pattern compared to adults, which leads to a higher incidence of both residual and recurrent disease. A staged canal wall-up or a canal wall-down tympanomastoidectomy (CWUT and CWDT, respectively) is selected depending on the extent of the disease and condition of the middle ear (ME) cleft and mastoid. Endoscopic ear surgery (EES) has been recently introduced as an adjuvant tool for the treatment of this pathology even in the pediatric population. OBJECTIVES: To analyze long term outcomes of CWUT and CWDT in the pediatric population, focusing on residual and recurrence rates of cholesteatoma and hearing results. A literature review including cases treated with EES were discussed. MATERIAL AND METHODS: Pediatric patients treated for cholesteatoma involving both the ME and mastoid with a follow-up (FU) of at least 4 years were retrospectively analyzed in a quaternary referral center for otology and lateral skull base surgery. Patients were grouped according to the surgical technique (CWUT versus CWDT). Rates of residual and recurrent cholesteatoma after each surgical technique were reported and compared. Mean Air-Bone Gap (ABG) of 0.5-1-2-4 KHz was measured and reported before the first surgery and at the last post-operative FU. RESULTS: Two-hundred and thirty-six cases fulfilled our inclusion criteria. The mean FU was 100.4 ± 44.2 months (median 89 months). One-hundred and five (44.5%) cases underwent a CWUT, whereas 131 (55.5%) a CWDT. A second stage surgery was performed in 73.5% of CWUT and 58.7% of CWDT. Among the CWUT group, residual cholesteatoma occurred in 22 (21%) ears and recurrence in 24 (22.9%). Patients undergoing CWDT showed lower rates of both residual and recurrent cholesteatoma (7.6% and 2.3%, respectively). ABG improvement was noted for both groups, even though CWUT showed better post-operative hearing results. CONCLUSIONS: The CWDT technique offers a definite surgical therapy, with minimal residual and recurrence rates and audiological results comparable to the CWUT technique. EES must still prove its added benefit or equivalence to pure microscopic approaches.

Simultaneous Cochlear Implantation After Translabyrinthine Vestibular Schwannoma Resection: A Report of 41 Cases.

OBJECTIVE: To study the auditory outcome of simultaneous translabyrinthine vestibular schwannoma (VS) resection and cochlear implantation (CI) after successful cochlear nerve preservation. STUDY DESIGN: A retrospective case series and patient questionnaire. SETTING: Quaternary referral center for skull base pathologies. PATIENTS: Patients with small (<2 cm) sporadic or neurofibromatosis 2 related tumors were included in this study. INTERVENTION: Vestibular schwannoma resection + simultaneous cochlear implantation. MAIN OUTCOME MEASURE: Audiological performance postimplantation and perceived patients' benefits. RESULTS: Forty-one patients were included. Thirty-three were sporadic VS and eight were neurofibromatosis 2. Auditory perception postimplantation was achieved in 33 patients (80.5%). At the last follow-up, 20 patients (48.8%) were users and 21 (51.2%) were nonusers. In the users' group, and after 1 year of implant activation, vowel identification was 75.3%, disyllabic word recognition 54%, sentence recognition 60.7%, and common phrase comprehension 61%, whereas in the nonusers' group and after 1 year of implant activation, vowel identification was 22.9%, disyllabic word recognition 14.8%, sentence recognition 15.3%, and common phrase comprehension 14%. Sixteen users were classified into 10 high performers, three intermediate performers, and three poor performers. In the user' group, the mean postimplantation pure tone average was 63.4 dB and the mean speech discrimination score was 63.7%. CONCLUSIONS: Simultaneous CI and VS resection is a viable option with many patients achieving auditory perception and nearly half the patients are CI users at long follow-up.

Effect of Sigmoid Sinus Position on the Difficulty and Approaches to Cochlear Implantation Surgery.

OBJECTIVES: To assess the effect of the position of the SS on CI regarding the ability to perform posterior tympanotomy, round window visibility, and mastoid pneumatization. MATERIALS AND METHODS: This is a prospective study, including 65 adult patients with CI performed at our center during 2017. We used 3 methods to assess SS position using a computed tomography (CT) scan. Lee's line passing through the tympanic segment of the facial nerve. Park's line passing through the facial nerve and round window membrane. Our proposed method using a parallel line from the external auditory canal and passing through the facial nerve. Relation to mastoid pneumatization on CT and to intraoperative round window visibility were assessed in relation to intraoperative position of the SS. RESULTS: The method by Park et al. was statistically significant (p<0.001); however, a cutoff point could not be set. Lee's method was statistically insignificant (p=0.091). Our proposed method was statistically significant with a cutoff point at ≤2.46 mm (p=0.001). SS position did not affect pneumatization nor round window visibility. CONCLUSION: The position of SS preoperatively using a CT might suggest the inability to perform posterior tympanotomy and the need to change side or approach. However, it does not affect neither mastoid pneumatization nor visibility of the round window niche through the facial recess.

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

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

"Large and giant vestibular schwannomas: overall outcomes and the factors influencing facial nerve function".

(1) To study the overall outcomes of patients surgically treated for large/giant vestibular schwannomas (VSs) and (2) to identify and analyze preoperative and intraoperative prognostic factors influencing facial nerve (FN) outcome. A retrospective clinical study was conducted at a quaternary referral otology and skull-base center. A total of 389 cases were enrolled. The inclusion criteria were patients with tumor > 30 mm undergoing surgery with a minimum follow-up of 12 months. Neurofibromatosis-II, previous radiotherapy, revision surgeries, preoperative FN House-Brackmann (HB) grade > I, partial resections, incomplete records, or those lost to follow-up for a minimum period of 1 year were excluded. In addition, partial resections and cases where FN was sacrificed intraoperatively were also excluded and were analyzed separately. The mean duration of symptoms was 35.4 months, pronounced more in elderly (58.3 months) than in younger individuals (28.4 months). Mean tumor diameter was 36 mm and 52.7% was cystic. Total resection (TR), near total resection (NTR), and subtotal resections (STR) were achieved in 77.4%, 9.5%, and 13.2% of cases, respectively. Regrowth was observed only after STR (19.6%). Good (HB I-II), moderate (HB III), and poor (HB IV-VI) FN functions were observed in 36.8%, 51.7%, and 11.6% cases, respectively. Younger individuals underwent TR in 259 (86.9%) cases against 42 (46.2%) in elderly individuals. Non-total resections (NTR/STR) were performed in 49 (53.8%) cases in elderly as against 39(13.1%) in younger individuals. Good FN outcome was observed in 28 (57.1%) cases of non-total resections in elderly as against 13 (33.3%) cases in younger individuals. On multiple logistic regression analysis, size of the tumor, preoperatively prolonged duration of symptoms, profound deafness, and antero-superiorly located FN with respect to the tumor played a detrimental role in the final facial nerve outcome postoperatively. On the contrary, in large tumors (3-3.9 cm), presence of vertigo/disequilibrium had a relatively better impact on final FN outcome. Partial resections accounted for 41(7.8%) cases and FN was interrupted in 71(13.6%) cases in total. Factors detrimental to better FN outcome were giant VSs (> 4 cm), antero-superiorly located FN intratumorally, preoperatively prolonged duration of symptoms, and profound deafness. In large tumors (3-3.9 cm), presence of vertigo/disequilibrium had a better impact on FN outcome. Understandably, cases with TR in comparison with NTR/STR had worse FN outcomes. In comparison with younger patients, elderly patients underwent higher NTR/STR resulting in better FN outcomes. The above factors can be used as prognosticators for patient counseling and surgical decision making.