Long-term facial nerve outcome in surgically treated petrous bone cholesteatoma patients.

INTRODUCTION: 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% of all petrous bone lesions. PBC represents a real surgical challenge due to its complex relationship with critical neurovascular structures. OBJECTIVE: To demonstrate our experience in using various options of facial nerve (FN) management during surgical treatment of PBC and analyze the long-term facial function outcomes. STUDY DESIGN: Retrospective medical record review in a quaternary skull-base center. MATERIALS AND METHODS: Medical records of 298 PBC cases operated between the years 1983 and 2024 were thoroughly evaluated. Sanna's classification scheme was used to classify cases into the appropriate class and the House-Brackmann (HB) grading system of the FN was used to assess the facial function pre- and postoperatively. RESULTS: A total of 298 PBC cases were surgically treated at our center. Males constitute 68% (n = 203) of total operated PBC patients while the rest 32% (95) were females, making the male-to-female ratio 2.2:1. The age in this series ranged from 9 to 85. According to updated Sanna's PBC classification, 44% were supralabyrinthine, 33% were massive, 9% were infralabyrinthine-apical, 8% were infralabyrinthine, and 5% were apical. On preoperative FN function examination, 45% (n = 133) of patients had various degrees of paresis and complete paralysis whereas 55% (n = 165) had normal FN function. The commonest degree of paresis observed was HB-III (18.5%) followed by HB-IV (5.7%). A total of 40(13.4%) patients, however, had complete facial paralysis at the initial presentation. The facial function was compromised more frequently in supralabyrinthine and massive PBCs. Among the total patients who had an abnormal facial function at presentation, 52% had paresis/paralysis for less than a year whereas 48% had FN paresis/paralysis for greater than one year. The most commonly used surgical approaches at our center were transotic (TO), modified transcochlear type A (MTCA) with rerouting of the FN, and translabyrinthine (TLAB) with external auditory canal (EAC) closure. Intraoperatively an intact fallopian canal was found only in 33% (n = 99) cases whereas the rest 67% (n = 199) had erosion of the fallopian canal either with intact (n = 24) or infiltrated FN (n = 175). The FN was maintained in the bony fallopian canal in 35% (n = 104) cases. An active form of FN management, however, was required in 65% (n = 194) of cases either intraoperatively or as a second-stage procedure. Factors affecting postoperative FN function include age, preoperative FN function (HB), duration of paralysis, class of PBC, surgical approach, and method of active FN management. CONCLUSION: PBCs represent diagnostically and surgically challenging lesions of temporal bone which are usually frustrating to the treating surgeon. Preoperative anatomic classification of the lesion enables the physician to choose the appropriate surgical approach. Sanna's classification is the most widely accepted system used to classify PBC. This classification is based on the relation of the lesion to the labyrinthine block. Active FN management, including rerouting, end-to-end anastomosis, cable nerve grafting, and masseteric-to-FN anastomosis routinely come into play in the surgical management of PBCs, and the postoperative FN results after such interventions can be satisfactory in most cases.

Cochlear implantation in otosclerosis: surgical and audiological outcomes between ossified and non-ossified cochlea.

AIM: 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. STUDY DESIGN AND SETTING: Retrospective study conducted at Otology and Skull Base Surgery Center. SUBJECTS AND METHODS: 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. RESULTS: 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% of these patients, while the rest had partial or complete basal turn ossification. Subtotal petrosectomy was performed in 63.1% and 28.6% of ossified and non-ossified cochlea respectively while the rest underwent cochlear implantation through posterior tympanotomy. Only one case had scala vestibuli insertion and four had incomplete electrode insertion. Six patients underwent re-implantation due to infection, device failure, and erosion of the posterior canal wall. Auditory outcomes among patients with ossified otosclerosis were slightly better than those without ossification but this difference was not statistically significant. CONCLUSION: Cochlear implantation for otosclerosis yields excellent auditory outcomes with a low rate of surgical complications, despite the high incidence of cochlear ossification.

Head and neck paragangliomas: Recent advances in translational and clinical research and guidelines for patient care.

Head and neck paragangliomas (HNPGLs), rare neuroendocrine tumors that mainly arise from parasympathetic ganglia along the cranial nerves, are challenging due to anatomic origin, tendency to aggressive neurovascular and skull base infiltration, unpredictable metastatic potential, radio-chemoresistance, and risk of multiplicity. Symptoms range from mild to life threatening depending on location/size, but rarely relate to catecholamine excess. Risk factors include female sex and pathogenic germline variants in genes affecting hypoxia signaling (foremost succinate dehydrogenase genes). Diagnostic work-up relies on imaging, measurements of plasma free metanephrines/methoxytyramine, genetic testing, and pathology/immunohistochemistry. Management is tailored to patient/tumor characteristics and encompasses wait-scan, upfront surgery, debulking surgery, and radiotherapy. Presurgical embolization is recommended, except for small tympanic and tympanomasoid tumors. Presurgical stenting is required for internal carotid artery involvement, and two-stage surgery for intradural extension. Current treatments for metastatic/inoperable HNPGL are non-curative, and long-term follow-up should be recommended for all patients to monitor local recurrence and new tumors.

Masseteric-facial anastomosis and hypoglossal-facial anastomosis after lateral skull base and middle ear surgery.

INTRODUCTION: 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. METHODS: 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. RESULTS: 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%) and MF in 68 patients (44.5%). The duration of the FN palsy before reconstructive surgery was inversely associated to better FN results, in particular with having a grade III HB (p = 0.003). Both techniques had significantly lower HB scores when an interval between palsy onset and reanimation surgery was 6 months or less (MF p = 0.0401; HF p = 0.0022). Patients who underwent a MF presented significant improvement of the FN function at 3 months from surgery (p = 0.0078). At the last follow-up, 63.6% recovered to a grade III HB and 22.7% to a grade IV. On the other hand, the first significant results obtained in the HF group were at 6 months from surgery (p < 0.0001). 67.8% of patients had a grade III HB after a HF at the last follow-up, 28.8% a grade IV. FN grading at 6 months from surgery was significantly lower in the MF group compared to the HF (p = 0.0351). The two techniques had statistically similar results at later follow-up evaluations. DISCUSSION/CONCLUTION: MF was associated to initial superior results, presenting significant facial recovery at 3 months, and significantly better functional outcomes at 6 months from surgery compared to HF. Although later results were not significantly different in this study, earlier results have an important role in order to limit the duration of risk of corneal exposure.

Gruppo Otologico's Experience in Managing the So-Called Inoperable Tympanojugular Paraganglioma.

Objective: to identify advanced or "so-called inoperable" cases of tympanojugular paragangliomas (PGLs) and analyze how each case is surgically managed and followed afterward. Study Design: a retrospective case series study. Methods: Out of 262 type C and D TJPs and more than 10 cases of advanced or so-called inoperable cases, files of 6 patients with a diagnosis of advanced tympanojugular PGLs who were referred to an otology and skull-base center between 1996 and 2021 were reviewed to analyze management and surgical outcomes. The criteria for choosing these cases involve having one or more of the following features: (1) a large-sized tumor; (2) a single ipsilateral internal carotid artery (ICA); (3) involvement of the vertebral artery; (4) a considerable involvement of the ICA; (5) an extension to the clivus, foramen magnum, and cavernous sinus; (6) large intradural involvement (IDE); and (7) bilateral or multiple PGLs. Results: The age range at presentation was 25-43 years old, with a mean of 40.5 years: two females and four males. The presenting symptoms were glossal atrophy, hearing loss, pulsatile tinnitus, dysphonia, shoulder weakness, and diplopia. The modified Infratemporal Fossa Approach (ITFA) with a transcondylar-transtubercular extension is the principal approach in most cases, with additional approaches being used accordingly. Conclusions: The contemporary introduction of carotid artery stenting with the direct and indirect embolization of PGLs has made it possible to operate on many cases, which was otherwise considered impossible to treat surgically. Generally, the key is to stage the removal of the tumor in multiple stages during the management of complex PGLs to decrease surgical morbidities. A crucial aspect is to centralize the treatment of PGLs in referral centers with experienced surgeons who are trained to plan the stages and manage possible surgical complications.

Surgical management of chondrosarcomas of the skull-base and temporal bone.

OBJECTIVES: To analyze the overall long-term outcome of surgically treated skull base and temporal bone chondrosarcomas. METHODS: The medical records of patients with surgically treated skull base and temporal bone chondrosarcomas between 1983 and 2024 were thoroughly evaluated. RESULTS: Out of a total of over 5000 skull base surgeries performed at our center, only 29 patients had histopathologically confirmed chondrosarcomas of the skull base and temporal bone. The mean of patients age was 45.6, and the male-to-female ratio was 1.9:1. The most common symptoms included hearing loss (58.6%), tinnitus (41.4%), diplopia (31%), dysphonia (24.1%), dysphagia (20.7%), vertigo (10.3%), and dizziness (10.3%). The most frequent locations of lesions among the 29 patients are as follows: petroclival region (34.5%), jugular foramen (27.6%), petrous apex (17.2%), middle ear (13.8%), others (3.4%). TO, IFTA, IFTB, IFTC, POTS, and combined surgical approaches were commonly used. The rate of gross total removal and recurrence was 82.6% and 13.8% respectively. The follow-up duration of 6 patients was more than five years and less than ten years whereas ten patients had more than ten years of follow-up. CONCLUSIONS: Chondrosarcoma of the skull base and temporal bone is a very rare pathology. Due to its multiple potential sites of origin and histological specificity, it presents us with significant challenges. Gross total removal is the primary treatment for chondrosarcoma of the skull base and temporal bone. Personalized decision-making should be considered based on the following aspects: tumor, patient, and surgeon's factors. Postoperative radiation therapy is complementary to surgical treatment in grades II and III lesions to achieve long-term survival.

Customized Vestibular Rehabilitation for Vestibular Schwannoma Excision via Translabyrinthine Approach: A Single-Center Experience.

Objectives: To evaluate the effectiveness of intensive customized vestibular rehabilitation after vestibular schwannoma (VS) excision. Methods: 52 patients who underwent VS removal via a translabyrinthine approach from 2020 to 2022 were involved in this study. Bedside examination, video head impulse test (vHIT), functional head impulse test (fHIT), and the dizziness handicap inventory (DHI) were performed before and after the rehabilitation, which consisted of 10 sessions of specifically designed vestibular, visual, and physical integrated training. Results: After rehabilitation, the vHIT showed overall unchanged values on the affected and healthy side. In contrast, the scores of fHIT, which explores the higher connection of the vestibular system with visual and cerebellar pathways, improved on both the pathological and healthy sides after training (p-value 0.004 and 0.000, respectively). The effectiveness of the rehabilitation was reinforced by the DHI scores, which were considerably lower after training. Conclusions: To our knowledge, this is the first study to explore fHIT outcomes after removal of VS, estimating the impact of rehabilitation on the overall compensation process. The outcomes support the role of extensive postsurgical rehabilitation in the compensatory process, even just a few days after surgery.

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.

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.