BACKGROUND AND OBJECTIVES:Jugular foramen schwannoma is a rare benign tumor arising from the lower cranial nerves. After reviewing their experience with microsurgical management of jugular foramen schwannomas through lateral skull base and transcervical approaches, the authors recommend a management algorithm to direct simplified surgical management. METHODS:Cases of jugular foramen schwannomas treated by the senior author between 1983 and 2024 were analyzed retrospectively. Sociodemographic data, clinical presentation, radiological and intraoperative tumor characteristics, histopathological results, surgical approaches, and postoperative course until the last visit were thoroughly documented and analyzed. RESULTS:A total of 53 cases of jugular foramen tumors were treated over a 40-year period. Age at presentation ranged from 13 to 76 years, with a mean age of 42.84 years. Women comprised 53% (n = 28) of the total patients operated on, whereas the rest, 47% (n = 25), were men. The most common presenting symptoms were hearing loss and dysphagia. Tumors were classified according to the Kaye and Pellet classification system into classes A (11.32%), B (39.62%), C (15.09%), and D (33.96%). The most commonly used surgical approach was the petro-occipital trans-sigmoid approach, either alone or combined with other approaches, and gross total removal was achieved in 98% of patients. New postoperative paralysis in previously normal lower cranial nerve function was evident in 44% (n = 8) of patients who had normal lower cranial nerve function before surgery. The duration of follow-up ranged from 12 to 144 months, with a mean follow-up period of 63.46 months. Three patients (5.6%) had tumor recurrence. CONCLUSION:Based on their experience, the authors have proposed a treatment algorithm that guides the best surgical strategy. Even if gross total tumor removal of jugular foramen area lesions can safely be achieved nowadays, an extensive understanding of microsurgical anatomy of the area and surgical training is mandatory.

OBJECTIVES:To analyze the facial nerve outcomes after removing the infratemporal fossa approach type A (IFTA) with anterior rerouting for class C-D tympanojugular paragangliomas (TJPs). METHODS:We evaluated the medical records and follow-up visits of patients with class C-D TJPs treated with IFTA between 1989 and 2023 at 2 quaternary referral institutions. RESULTS:Of the 258 patients surgically treated for modified Fisch Class C-D TJPs, 154 cases were selected based on the selection criteria: 29 (18.8%) patients were classified as having a Class C1 TJP, while 86 (55.8%) had a Class C2 and 39 (25.3%) had a Class C3-4 TJP. Among the included subjects, 146/154 (94.81%) had normal-to-moderately impaired (grades I-II and III) facial nerve function at the last follow-up, including 27/29 (93.1%) patients with C1 TJPs, 81/86 (94.19%) patients with C2 TJPs, and 38/39 (97.44%) patients with C3-4 TJPs. Total tumor removal was achieved in 25 out of 29 (86.2%) patients with C1 TJPs, 67 out of 86 (77.9%) patients with C2 TJPs, and 31 out of 39 (79.5%) patients with C3-4 TJPs. CONCLUSIONS:The main goal of TJP treatment is complete tumor removal, especially in young patients with a higher expected lifespan. IFTA is an appropriate approach for the resection of class C-D TJPs. With anterior rerouting of the facial nerve, adequate exposure to the jugular foramen, infralabyrinthine areas, and intratemporal internal carotid artery minimizes morbidity and avoids mortality. Postoperative facial asymmetry, mostly of recovery to grade I-II and III, is the price paid to achieve a higher total tumor resection rate and a lower rate of recurrence and complications.

(1) Study the preoperative predictive factors (such as duration of symptoms, tumor size, and cystic component) and their effects on the type of resection and facial nerve (FN) outcomes. (2) Analyze the intraoperative predictive factors, such as the extent of resection and intraoperative FN stimulation. (3) Compare our approaches (enlarged translabyrinthine approach, transotic and transcochlear approaches) to other approaches (retro sigmoid) and review the literature comparing different approaches. (4) To inquire if the surgical decision (total vs. non-total removal) and outcomes (complications and facial nerve function) of large and giant VS surgery have changed during 35 years at Gruppo Otologico. A total of 567 cases out of 3707 were enrolled. The inclusion criteria were patients with tumors > 30 mm extrameatal diameter undergoing a translabyrinthine approach with a minimum follow-up of 12 months. The mean duration of symptoms was 35.9 months, pronounced more in the elderly (53.4 months) than in younger individuals (33.9 months). The mean tumor diameter was 36.6 mm, and 50.4

OBJECTIVES:To highlight our over 40 years of experience with subtotal petrosectomy (STP), we aim to present updated information on its technique, indications, and long-term outcomes. STUDY DESIGN:A retrospective case series study was conducted in a private quaternary skull base center. MATERIALS AND METHODS:The medical records of 900 cases of STP performed between 1983 and 2023 were thoroughly evaluated in this study. The assessment encompassed symptoms and signs, preoperative and postoperative audiological parameters, radiologic and surgical details, diagnoses, and complications. RESULTS:Six hundred eighty-seven (65.2%) patients had undergone multiple surgeries before a STP was performed. The most common indication for STP was middle ear cholesteatoma, with 344 (38.2%) patients. Challenging cases of cochlear implantation, meningoencephalic herniation, class B3 paragangliomas, and temporal bone fractures, with 281 (31.2%), 68 (7.6%), 52 (5.8%), and 37 (4.1%) cases, respectively. The mean follow-up time for our patients was 40.7 months, with a median follow-up of 24.6 months. The most common complications were recidivism and postauricular wound fistula, occurring in 12 and 9 patients, respectively. To the best of our knowledge, this series is the largest documented cohort in the literature to date. CONCLUSIONS:STP should be regarded as the preferred approach for a range of challenging situations in otology, as it offers the potential for a definitive cure through radical clearance. In addition, this procedure can be safely combined with various other otologic and lateral skull base surgical procedures, including hearing implantation.

Despite the spread of novel-generation cochlear-implant (CI) magnetic systems, access to magnetic resonance imaging (MRI) for CI recipients is still limited due to safety concerns. The aim of this study is to assess and record the experiences of Hires Ultra 3D (Advanced Bionics) recipients who underwent an MRI examination. A multicentric European survey about this topic was conducted focusing on safety issues, and the results were compared with the current literature. We collected a total of 65 MRI scans performed in 9 otologic referral centers for a total of 47 Hires Ultra 3D recipients, including, for the first time, 2 children and 3 teenagers. Preventive measures were represented by scanning time and sedation for children. Head wrapping was used in eight cases, and six of the eight cases received local anesthesia, even if both measures were not needed. Only three patients complained of pain (3/65 examinations, 4.6%) due to the tight head bandage, and one of the three cases required MRI scan interruption. No other adverse events were reported. We believe that these results should encourage MRI execution in accordance with manufacturer recommendations for Ultra 3D recipients.

Lateral skull base (LSB) and middle ear pathologies often involve the facial nerve (FN), and their treatment may require FN sacrifice. Cases with unidentifiable proximal stump or intact FN with complete FN palsy, necessitate FN anastomosis with another motor nerve in order to restore innervation to the mimicking musculature. The results of hypoglossal-to-facial nerve anastomosis (HFA) and masseteric-facial nerve anastomosis in patients with facial paralysis after middle ear and LSB surgeries, are presented and compared. Adult patients with total definitive facial paralysis after middle ear or LSB surgery undergoing facial nerve reanimation through hypoglossal or masseteric transfer anastomosis were enrolled. The facial nerve function was graded according to the House Brackmann grading system (HB). The facial function results at 3 months, 6 months, 12 months, 18 months and at the last follow up (more than 18 months) are compared. 153 cases of LSB and middle ear surgery presented postoperative facial palsy and underwent facial nerve reanimation surgery with HF in 85 patients (55.5

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.

To evaluate (1) Audiological and surgical outcomes in patients with otosclerosis following cochlear implantation. (2) surgical difficulties and outcomes between both groups. (3) Audiological outcomes between both groups. Retrospective study conducted at Otology and Skull Base Surgery Center. Data were analyzed from 111 patients with otosclerosis (114 ears) who underwent cochlear implant surgery using the cochlear implant database. Demographic characteristics (age, sex, and operated ear), auditory outcomes, and operative details (extent of cochlear ossification, surgical approach [posterior tympanotomy or subtotal petrosectomy], electrode insertion [partial/complete, scala tympani or vestibuli], and complications) were analyzed Auditory outcomes were assessed over at least one year follow-up period using pure tone audiometry and speech discrimination scores. Patients were divided into two groups (with and without cochlear ossification) to compare auditory outcomes and surgical outcomes. The mean age of patients with ossified and non-ossified cochlea was 60.04 and 62.22 years respectively. Sixty-five of 114 ears had cochlear ossification, with complete round window involvement in 75.4

The term petrous bone cholesteatoma (PBC) represents a slow-growing epidermal lesion arising from the petrous part of the temporal bone. It is a rare incidence accounting for only 4–9

Petrous bone cholesteatomas (PBCs) are slow-growing expansile epidermoid lesions arising in the petrous portion of the temporal bone with an incidence of 4 to 9% of all petrous pyramid lesions. Surgery remains the mainstay of treatment of PBCs, aiming for macroscopic disease clearance along with complete control and safety of the surrounding important neurovascular structures. Transpetrous surgical approaches (transotic, infratemporal fossa type b, transcochlear or combined approaches) still represent the mainstay of surgery for PBCs. Postoperative follow-up (FU) has shifted from high resolution CT scan of the temporal bone and brain MRI with/without gadolinium enhancement to MRI non-EPI diffusion-weighted images (DWI). We report the surgical outcomes of nearly 280 cases of PBCs treated from 1983 to 2022 in a quaternary referral center for otology and lateral skull base surgery, focusing of facial nerve, hearing and other cranial nerves outcomes. Also, a comparison between recurrence rates before and after the MRI-DWI era will be provided.

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.

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.

ObjectivesTo 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.MethodsThis 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.ResultsFNSs 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.ConclusionFNSs 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.

Cochlear implant (CI) surgery in specific situations necessitates a different surgical technique. The objective of subtotal petrosectomy (STP) with closure of the external auditory canal (EAC) and eustachian tube (ET), combined to cavity obliteration with abdominal fat, is to isolate the cavity from the external environment. Moreover, this technique can be used to obtain an easier access to and better visibility of the middle ear and cochlea. The decision to perform STP is not taken lightly when normal hearing is still present, but in a CI candidate, this consideration is of importance only if residual hearing is present and electroacoustic stimulation is intended. Finally, an STP should be performed and preferred to a standard cochlear implantation technique in the presence of otitis media, cholesteatoma, cochlear ossification, inner ear malformation or fracture of the temporal bone.

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.

Objective: The aim of this study was to analyze the results of labyrinthectomy and cochlear implantation (CI) on hearing, vertigo, and tinnitus and evaluate the adequacy of labyrinthectomy and CI for the treatment of end stage Menière's Disease (MD). Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Charts of 22 patients undergoing labyrinthectomy and CI in the same ear for intractable vertigo and hearing loss with both preoperatory and postoperatory documentation available, were reviewed. Intervention(s): Therapeutic. Main Outcome Measure(s): Auditory outcomes were assessed with pure tone and speech audiometry, and compared with the preoperatory audiometric evaluation. Dizziness was graded according to the Dizziness Handicap Inventory Questionnaire (DHI). Tinnitus outcomes were assessed by the tinnitus handicap inventory (THI). Results: Post-CI pure tone average had a statistically significant improvement ( p = 0.035, paired t test). Speech audiometry resulted in a non-statistically significant speech discrimination score variation ( p = 0.056, paired t test). Postoperatory THI had a statistically significant variation ( p = 0.0001, paired t test). Sixty seven per cent of the patients had complete resolution of the vestibular symptoms in their operated ear, however, patients over 70 years old had significantly more failures as evinced by the postoperative DHI ( p = 0.0109, Fisher's exact test). Conclusions: Patients affected by end stage MD or secondary MD, with vertigo and severe hearing loss can successfully undergo labyrinthectomy and CI. Caution should be reserved in elderly patients for a risk of persistent instability. The CI confers significant benefit in hearing rehabilitation and tinnitus suppression.

SUMMARY Disregarding the widely used division of skull base into anterior and lateral, since the skull base should be conceived as a single anatomic structure, it was to our convenience to group all those approaches that run from the antero-lateral, pure lateral and postero-lateral side of the skull base as “Surgery of the lateral skull base”. “50 years of endeavour” points to the great effort which has been made over the last decades, when more and more difficult surgeries were performed by reducing morbidity. The principle of lateral skull base surgery, “remove skull base bone to approach the base itself and the adjacent sites of the endo-esocranium”, was then combined with function preservation and with tailoring surgery to the pathology. The concept that histology dictates the extent of resection, balancing the intrinsic morbidity of each approach was the object of the first section of the present report. The main surgical approaches were described in the second section and were conceived not as a step-by-step description of technique, but as the highlighthening of the surgical principles. The third section was centered on open issues related to the tumor and its treatment. The topic of vestibular schwannoma was investigated with the current debate on observation, hearing preservation surgery, hearing rehabilitation, radiotherapy and the recent efforts to detect biological markers able to predict tumor growth. Jugular foramen paragangliomas were treated in the frame of radical or partial surgery, radiotherapy, partial “tailored” surgery and observation. Surgery on meningioma was debated from the point of view of the neurosurgeon and of the otologist. Endolymphatic sac tumors and malignant tumors of the external auditory canal were also treated, as well as chordomas, chondrosarcomas and petrous bone cholesteatomas. Finally, the fourth section focused on free-choice topics which were assigned to aknowledged experts. The aim of this work was attempting to report the state of the art of the lateral skull base surgery after 50 years of hard work and, above all, to raise questions on those issues which still need an answer, as to allow progress in knowledge through sharing of various experiences. At the end of the reading, if more doubts remain rather than certainties, the aim of this work will probably be achieved.

Abstract Objective We present an extremely rare report of congenital cholesteatoma (CC) in identical twins with atypical location. Methods Two consecutive cases of CC in identical twins from diagnosis to treatment and a literature review are presented. Results The twin children presented to our otology and lateral skull base clinic with different clinical picture. Both underwent thorough audiological workup and imaging and early surgical intervention. The 6-year follow-up of these two children has been uneventful with no recurrence of disease and hearing restoration. Conclusion Multiple theories exist to explain this rare pathology. Modified Levenson’s criteria define it as white mass behind intact tympanic membrane. Atypical location of CC with recurrent otitis media is not uncommon; hence, further study is required. To our best knowledge, this is the second reported occurrence in identical twins but first report with differential presentation and atypical location in both cases.

Objectives/Hypothesis To describe the technique of subtotal petrosectomy (STP), to analyze the outcomes, and to review the literature Study Design A retrospective review. Methods Four hundred sixty cases of STP performed for various indications were included in the study, which was conducted at a quaternary referral center for otology and skull base surgery. Surgical and audiological parameters, and complications were evaluated. Our results were compared with the existing literature on the subject. Results Two hundred ninety‐seven (64.6%) patients had been subjected to multiple surgeries before an STP was performed. The most common indication for STP was recurrent chronic otitis with or without cholesteatoma, with 165 (35.9%) patients. Difficult cases of cochlear implantation, temporal bone fractures, and class B3 tympanomastoid paragangliomas were the next most common indications, with 91 (19.8%), 43 (9.4%), and 38 (8.3%) cases, respectively. The median follow‐up of the patient pool was 36 ± 19 months. Recidivism and postauricular wound fistula were the most common complications, seen in five (1.1%) patients each. This series of STP is the largest reported in the literature Conclusions STP is a very useful and safe surgical tool in the management of a variety of problematic situations in otology, as it offers the possibility of a definitive cure by offering radical clearance. This procedure can be combined safely with hearing implantation procedures. Level of Evidence 4. Laryngoscope , 127:2833–2842, 2017