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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.A retrospective case series.Quaternary referral center for skull base pathologies.Patients with sporadic ICVS managed by wait and scan.Serial resonance imaging (MRI) with size measurement and serial audiological evaluation.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).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.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.

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.Retrospective review.Quaternary referral center for otology and skull base surgery.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).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.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.

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 (

Purpose To compare the relationship between the variable “complication” and the other variables of middle ear cholesteatoma classifications (STAMCO, ChOLE, and SAMEO-ATO). Methods Retrospective study of 110 patients that underwent 132 middle ear surgeries between the 1 January 2012 and the 31 December 2019 for chronic otitis with cholesteatoma classified according to STAMCO, ChOLE, and SAMEO-ATO classifications in a tertiary health care centre. Results Older age, male gender, STAMCO-T, and SAMEO-ATO [O1, T, O2, (s -)] and mastoid involvement (STAMCO-M and ChOLE-Ch) were associated with an increased risk of complication report. Conclusions In our series, statistical analysis pointed out a relationship between surgical complications and age, gender, site, mastoidectomy type, and ossicular chain status at surgery. The choice of variables to be recorded for cholesteatoma staging should be carefully balanced, considering that “complication” variable could be a repetitive item.

Temporal bone fractures are a common lesion of the base of the skull. The diagnosis and management of temporal bone fractures require a multidisciplinary approach. Variable clinical presentations may arise from such fractures, ranging from an asymptomatic course to very serious consequences. The aim of this study was to report our experience with a series of patients with temporal bone fractures and to propose a diagnostic/therapeutic algorithm. This study enrolled 141 patients, 96 (68.1%) males and 45 (31.9%) females, ranging in age from 20 to 60 (average age: 39 &plusmn, 4.1 years), with temporal bone fractures who were referred to Cardarelli Hospital between 2006 and 2018. The present paper presents a classification of temporal bone fractures and typical clinical sequelae and provides an illustration of their prognosis and treatment.

Objective To study the early and late facial nerve (FN) outcomes in different tumor classes in addition to determining the predictive factors for the same. Study design A retrospective clinical study. Setting A quaternary referral otology and skull base center. Patients and methods A retrospective study of 1983 cases of vestibular schwannomas (VSs) with preoperative normal FN function, undergoing total excision with anatomical preservation of the nerve by enlarged translabyrinthine approach (ETLA) were included. FN status was recorded postoperatively at day 1, at discharge, and at 1-year follow-up and were analyzed in different tumor sizes. Results At 1 year, 988 patients with House-Brackmann (H-B) grade I and II FN at day 1 after surgery, 958 (96.9%) maintained their status up-to 1 year. Of the 216 patients with H-B grade III at day 1 after surgery, 113 (52.3%) improved to H-B grade I and II. Similarly, of the 779 patients with H-B grade IV and VI FN function at day 1 after surgery, improvement to H-B III and H-B I and II were noted in 442 (56.7%) and 80 (10.3%) of patients, respectively. Intrameatal and extrameatal tumors upto 2 cm showed better recovery from H-B grade III to H-B I and II and from H-B grade IV and VI to H-B I and III when compared with extrameatal tumors >2 cm (p = 0.001). Conclusion Tumors of smaller sizes have good immediate postoperative FN results and recover well at the end of 1 year while more than 3 cm have poor outcomes and recover poorly at the end of 1 year. When the VSs reaches more than 1 cm, the HB I and II outcomes drop significantly.

Objective To study the clinical presentation, intraoperative findings and surgical management in meningo-encephalic-herniation (MEH) based on the etiology. Study design A retrospective clinical study and is a follow-up on the previously published report in 2009. Setting A quaternary referral otology and skull base center PATIENTS AND METHODS:: The inclusion criteria were intraoperatively verified MEH in patients with a minimum follow-up of 12 months, which yielded 262 operated ears. The data were extracted regarding demographics, laterality, clinical presentation, past surgeries, contralateral-ear condition, intraoperative findings, complications, recurrences, revision-surgeries, audiometric-data, and follow-up. Results The mean age at surgery was 49.7 years with the involvement of right-ear in 53.8% of patients. Lesions were categorized based on the etiology as chronic-otitis-media with/without cholesteatoma-MEH (COM/CHOL-MEH)-47.7%, iatrogenic-MEHs -20.9%; traumatic-MEHs -8% and spontaneous-MEHs -23.3%. At presentation, hearing loss (100 and 98.2%) and otorrhea (65.6 and 49.1%) were predominant in COM/CHOL-MEHs and iatrogenic-MEHs, respectively. On the other hand, meningitis (23.9 and 14.3%) and cerebrospinal fluid-leak (52.4 and 42.8%) were more pronounced in spontaneous and traumatic MEHs, respectively. Surgical approaches included 1) transmastoid, 2) middle-cranial-fossa-approach, 3) combined, and 4) middle-ear-obliteration (MEO) techniques. A total of 52.8% of COM/CHOL-MEHs and 49.1% of iatrogenic-MEHs underwent MEO. Middle-cranial-fossa approach was predominantly used in spontaneous-MEHs (52.5%) and traumatic-MEHs (38.1%). The defect was mostly single (75.2%). Smaller, multiple, bilateral lesions were more common in spontaneous-MEHs with tegmen-tympani involvement (57.4%). Conclusion Incorporating etiology into MEHs is a key-step that can be used as a guidance in choosing the right surgery. MEO is a part of armamentarium, and should be used whenever needed, if the objective is performing a definitive surgery.

Background The wait-and-scan modality has emerged as an important strategy in the management of vestibular schwannoma (VS) as it has been demonstrated that many tumors grow slowly or do not show any growth over long periods. Objective To analyze long-term outcomes of wait-and-scan in the treatment of patients with VS, discuss the factors contributing to the decision making, determine the inherent risks of the policy, and compare our results with literature. Methods In total, 576 patients with sporadic unilateral VS who were managed with wait-and-scan were reviewed retrospectively. Of these, a subset of 154 patients with 5-yr follow-up was separately analyzed. The tumor characteristics including patterns of growth, rate of growth, hearing outcomes, and likely factors affecting the above parameters were analyzed. Results The mean period of follow-up was 36.9 ± 30.2 mo. The mean age was 59.2 ± 11.6 yr. Thirteen different patterns of tumor growth were observed. Eighty-four (54.5%) of 154 tumors with 5-yr follow-up showed no growth throughout 5 yr. Fifty-six (36.4%) tumors showed mixed growth rates. Only 57 (37%) patients had serviceable hearing at the start of follow-up, but 32 (56.1%) maintained it at the end of follow-up. One hundred fifty (26%) of the 576 patients who failed wait-and-scan had to be taken up for surgery. Conclusion While there may be no price to pay in wait-and-scan as far as hearing is concerned, this may not be the case for facial nerve outcomes, wherein the results may be better if the patients are taken earlier for surgery.

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.

Indications for cochlear implantation have extended progressively over the years both from an audiological and clinical point of view, including the introduction of cochlear implants (CI) for hearing rehabilitation following skull base surgeries and neurotological procedures. In the past, cochlear implantation has been used for hearing rehabilitation in radiation-induced sensorineural hearing loss in nasopharyngeal and tonsillar carcinoma with successful outcomes. Here we describe a similar outcome following total deafness after tympanojugular paraganglioma irradiation, which is also the first such report in literature.The irradiated ear of this patient was implanted with a CI through standard posterior tympanotomy.A 26 year old male with multiple paragangliomas with bilateral deafness, secondary to surgery on one ear and radiotherapy for the opposite ear.After a follow-up of 48 months, no local complications have occurred and the hearing results have remained stable with 100% sentence recognition.Bilateral and multiple paragangliomas are rare tumors. Despite the modality of treatment, the hearing is almost always compromised. In these patients, cochlear implantation offers a new perspective for hearing restoration. This report demonstrates that cochlear implantation can be effectively performed after tympanojugular paraganglioma irradiation with long-lasting, satisfactory results, even in the presence of residual tumor.

Background Intrinsic tumors of the facial nerve are a rare entity. Dealing with this subset of tumors is challenging both in terms of decision making and surgical intervention. Objective To review the outcomes of surgical management of facial nerve tumors and cable nerve graft interpositioning. Methods A retrospective analysis was performed at a referral center for skull base pathology. One hundred fifteen patients who were surgically treated for facial nerve tumors were included. In case of nerve interruption during surgery, the cable nerve interpositioning technique was employed wherein the facial nerve palsy lasted for less than 1-yr duration. In cases of facial nerve palsy lasting for greater than 1 yr, the nerve was restituted by a hypoglossal facial coaptation. Results Various degrees of progressive paralysis were seen in 84 (73%) cases. Sixty nine (60%) of the tumors involved multiple segments of the facial nerve. Sixty-two (53.9%) tumors involved the geniculate ganglion. Seventy four (64.3%) of the cases were schwannomas. Hearing preservation surgeries were performed in 60 (52.1%). Ninety one (79.1%) of the nerves that were sectioned in association with tumor removal were restituted primarily by interposition cable grafting. The mean preoperative House-Brackmann grading of the facial nerve was 3.6. The mean immediate postoperative grading was 5.4, which recovered to a mean of 3.4 at the end of 1 yr. Conclusion In patients with good facial nerve function (House-Brackmann grade I-II), a wait-and-scan approach is recommended. In cases where the facial nerve has been interrupted during surgery, the cable nerve interpositioning technique is a convenient and well-accepted procedure for immediate restitution of the nerve.

OBJECTIVEThe aim in this study was to review the technique and outcomes of cable graft interpositioning of the facial nerve (FN) in lateral skull base surgeries.METHODSThe authors retrospectively evaluated data from patients who had undergone cable graft interpositioning after nerve sacrifice during skull base tumor removal between June 1987 and May 2015. All patients had undergone lateral skull base approaches to remove tumors at a quaternary referral center in Italy. Facial nerve function was evaluated before and after surgery using the House-Brackmann (HB) grading system.RESULTSTwo hundred thirteen patients were eligible for study. The mean follow-up was 44.3 months. The most common pathology was vestibular schwannoma (83 cases [39%]), followed by FN tumor (67 cases [31%]). Facial nerve tumors had the highest incidence of nerve interruption (67 [66%] of 102 cases). Preoperative FN function was normal (HB Grade I) in 105 patients (49.3%) and mild (HB Grade II) in 19 (8.9%). At the last postoperative follow-up, 108 (50.7%) of the 213 patients had recovered to Grade III nerve function. Preoperative HB grading of the FN was found to have a significant effect on outcome (p = 0.002).CONCLUSIONSCable graft interpositioning is a convenient and well-accepted procedure for immediate restoration of the FN. The study results, over a large number of patients, showed that the stitch-less fibrin glue–aided coaptation technique yields good results. The best possible postoperative result achieved was an HB Grade III. The chances of a good postoperative result increase when FN function is normal preoperatively. Slow-growing tumors of the cerebellopontine angle had a favorable outcome after grafting.

Object The authors evaluated the behavior of residual tumors and facial nerve outcomes after incomplete excision of vestibular schwannomas (VSs). Methods The case records of all patients who underwent surgical treatment of VSs were analyzed. All patients in whom an incomplete excision had been performed were analyzed. Incomplete excision was defined as near-total resection (NTR), subtotal resection (STR), and partial resection (PR). Tumors in the NTR and STR categories were followed up with a wait-and-rescan approach, whereas the tumors in the PR category were subjected to a second-stage surgery and were excluded from this series. All patients included in the study underwent baseline MRI at the 3rd and 12th postoperative months, and repeat imaging was subsequently performed every year for 7–10 years postoperatively or as indicated clinically. Preoperative and postoperative facial function was noted. Results Of the 2368 patients who underwent surgery for VS, 111 patients who had incomplete excisions of VSs were included in the study. Of these patients, 73 (65.77%) had undergone NTR and 38 (34.23%) had undergone STR. Of the VSs, 62 (55.86%) were cystic and 44 (70.97%) of these cystic VSs underwent NTR. The residual tumor was left behind on the facial nerve alone in 62 patients (55.86%), on the facial nerve and vessels in 2 patients (1.80%), on the facial nerve and brainstem in 15 patients (13.51%), and on the brainstem alone in 25 patients (22.52%). In the 105 patients with normal preoperative facial nerve function, postoperative facial nerve function was House-Brackmann (HB) Grades I and II in 51 patients (48.57%), HB Grade III in 34 patients (32.38%), and HB Grades IV–VI in 20 patients (19.05%). Seven patients (6.3%) showed evidence of tumor regrowth on follow-up MRI. All 7 patients (100%) who showed evidence of tumor regrowth had undergone STR. No patient in the NTR group exhibited regrowth. The Kaplan-Meier plot demonstrated a 5-year tumor regrowth-free survival of 92%, with a mean disease-free interval of 140 months (95% CI 127–151 months). The follow-up period ranged from 12 to 156 months (mean 45.4 months). Conclusions The authors' report and review of the literature show that there is undoubtedly merit for NTR and STR for preservation of the facial nerve. On the basis of this they propose an algorithm for the management of incomplete VS excisions. Patients who undergo incomplete excisions must be subjected to follow-up MRI for a period of at least 7–10 years. When compared with STR, NTR via an enlarged translabyrinthine approach has shown to have a lower rate of regrowth of residual tumor, while having almost the same result in terms of facial nerve function.

To describe our experience in the management of patients with squamous cell carcinoma of the temporal bone (TBSCC) and to identify factors predictive of outcome.Retrospective study.Quaternary referral otology and skull base center.A total of 45 consecutive patients with histologically confirmed TBSCC were treated surgically at our institution between 1993 and 2011. Patients were divided into 5 stage I (11.1%), 6 stage II (13.3%), 15 stage III (31.1%), and 19 stage IV tumors (42.2%) according to the University of Pittsburgh modified TNM staging system.Twenty-one patients underwent lateral temporal bone resection, and 24 underwent subtotal temporal bone resection. Postoperative radiotherapy was performed in 27 cases.The 5-year disease-specific survival (DSS) and recurrence-free survival (RFS) for patients with early-stage disease (Stages I and II) was 100%. The 5-year DSS and RFS rates for patients with advanced disease (Stages III and IV) were 65.1% and 59.6%, respectively. On univariate analysis, factors that had a significant effect on both DSS and RFS were advanced Pittsburgh stage, presence of facial nerve palsy, positive tumor margins, and invasion of the fallopian canal, medial wall, middle ear, mastoid, temporomandibular joint, jugular bulb, and dura. Multivariable analysis identified only dural involvement as an independent predictor for both DSS and RFS.In this study, the Pittsburg staging system allowed an estimation of the prognosis. In fact, the prognosis of TBSCC was strictly correlated to tumor stage. The poor prognosis of advanced stage tumors underlines the importance of early diagnosis. Surgery with or without adjuvant radiotherapy remains the standard of care in the treatment of TBSCC.

Objectives The use of radiation therapy has largely widespread and becomes in many centers the preference modality of treatment for symptomatic patients who are old, medically unfit for surgical therapy, those who refuse surgery and in some recurrent or residual growing tumors. The risk of radiotherapy failure in the treatment of vestibular schwannoma might be underestimated in the literature. The purpose of this study is to show the Gruppo Otologico experience with salvage surgery to better understand the surgical outcomes and difficulties in treating vestibular schwannoma after failed radiotherapy. Study design Retrospective chart review of patients who required salvage surgery of vestibular schwannoma after failed radiotherapy. Settings Quaternary referral otology and skull base center. Results Between 1987 and 2010, 2500 cases of VS underwent surgical treatment at the Gruppo Otologico. Nineteen patients had received stereotactic radiation therapy before the surgical treatment. The interval time between radiotherapy and surgical salvage ranged from 1 to 10 years. In all the cases decision of surgery was taken following an increase in tumor size with or without new onset of symptoms. Complete tumor removal was achieved in 86.6% of the cases through a transotic, transcochlear or enlarged translabyrinthine approach with trans-apical extension. Difficult dissection of the tumor was encountered in 93.3% the cases. The facial nerve was anatomically preserved in 93.3% but its function was worsened in 73.3% of patients after at least 6 months of follow up. Malignant transformation of the vestibular schwannoma was encountered in one patient. Conclusion Complete surgical resection of VS is more difficult after radiotherapy with relatively poor facial nerve outcomes and nearly impossible hearing preservation. Patients who receive radiation therapy for the treatment of vestibular schwannoma should be made aware of its potential complications and risk of failure, especially in young patients and NF2 cases.

Objective: To review the classification and management of petrous bone cholesteatomas (PBCs) at our center and the outcomes of facial nerve (FN) management in these lesions. Methods: This was a retrospective study. The setting was a quaternary referral center for skull base pathology in Italy. A total of 200 patients with 201 PBCs were included in the study. All patients diagnosed radiologically with PBCs were classified according to the Sanna classification. All patients were surgically treated and followed up with radiology. The main outcome measures - classification of PBCs, the surgical approach used, disease control, and FN outcomes - were analyzed. Results: Supralabyrinthine PBCs were the most common type with 92 cases (45.8%) followed by the massive PBCs with 72 cases (35.8%). Preservation of preoperative FN function was highest in the infralabyrinthine (72.2%) and infralabyrinthine-apical (73.3%) types. The transotic approach was used in 66 cases (32.8%) in this series. The modified transcochlear approach type A was applied in 55 cases (27.3%). Active management of the nerve (rerouting, anastomosis, or grafting) was required in 53 cases (26.4%). Postoperatively, of the 116 cases with FN House-Brackmann grade I and II, 107 cases (92.2%) retained the same grade or improved. Recurrence was seen in 7 cases (3.5%). The mean duration of follow-up was 6.3 years. Conclusions: Radical disease clearance must take precedence over hearing and FN preservation in PBCs. Active FN management, including rerouting, end-to-end anastomosis, and cable nerve grafting, routinely come to play in the surgical management of PBCs, and the postoperative FN results after such interventions can be satisfactory in most cases.

Objective: To compare hearing preservation and facial nerve function outcomes in patients undergoing vestibular schwannoma surgery performed using either the middle cranial fossa approach (MCFA) or the retrosigmoid approach (RSA). Materials and Methods: A review of the medical records of patients diagnosed with vestibular schwannoma who underwent surgical tumor removal in a single reference center via the MCFA or the RSA between January 1988 and December 2008 was conducted. Results: During this period, 90 patients underwent surgery via the MCFA while 86 patients received surgical treatment via the RSA. Of the patients subjected to the MCFA, 80.7% were characterized by a House-Brackmann (HB) grade I or II outcome, whereas 96.5% of patients undergoing the RSA were characterized by a HB grade I or II outcome (p = 0.001). This difference appeared only for extrameatal tumors when we compared size-matched tumors (58.3% MCFA vs. 98% RSA; p = 0.0006). There was no statistically significant difference in the hearing outcomes upon consideration of hearing preservation as characterized by the modified Sanna classification system involving classes A and B (18.9% MCFA vs. 10.6% RSA; p = 0.122). Conclusion: No statistically significant difference in hearing preservation was identified when comparing tumors operated upon via the MCFA versus the RSA. However, our results indicate that a higher risk of facial nerve function impairment exists if the surgery is performed via the MCFA under circumstances where the tumor extends to the cerebellopontine angle.

Objectives/Hypothesis: A relation between conventional radiotherapy and the development of intracranial neoplasma is well known, but radiation-associated tumor following stereotactic radiotherapy of vestibular schwannoma is underestimated. In this article we will study this relation by doing a complete literature review on all the malignant intracranial tumors that appeared following radiosurgery and adding a case of malignant vestibular schwannoma following stereotactic radiotherapy in a Neurofibromatosis type 2 patient. Methods: Literature review and discussion. Results: We found 26 cases of malignant brain tumor following stereotactic radiotherapy including our case. In 13 cases the tumor occurred in context of Neurofibromatosis type 2. None of the patients had a tumor size less than 2.5 cm. and the mean latency period between the radiotherapy and malignant tumor development was 5.8 years. Conclusion: Patients with vestibular schwannoma should be made aware of the low incidence of the radiation-induced malignant changes and long-term follow-up is mandatory.

Objectives: In a retrospective analysis of a quaternary referral neuro-otologic private practice, we identify complex cases of petrous bone cholesteatoma (ie, cases with encasement of vital structures such as the internal carotid artery, jugular bulb, and sigmoid sinus, with further extension to the clivus, sphenoid sinus, or rhinopharynx), review surgical approaches and techniques of management of vital structures, and propose the ideal surgical management. Methods: We performed a retrospective case study of 130 cases of petrous bone cholesteatoma submitted to surgery between 1979 and 2009 to identify the complex cases and their classification, approach used, outcomes, and recurrences. Results: Of 130 cases, 13 were complex. Facial palsy was the presenting feature in 11 cases, 7 of which presented with grade VI palsy. A long duration of facial palsy (more than 3 years) was seen in 5 cases. Clival involvement was seen in 6 cases; 1 case extended to the sphenoid sinus, and 1 to the rhinopharynx. The internal carotid artery was encased in 11 cases in the vertical and the horizontal parts. The jugular bulb was involved in 7 cases. Modified transcochlear approaches or infratemporal fossa approaches were used in all cases. There were no recurrences. Conclusions: Classification is fundamental to choosing the right surgical approach. Transotic and modified transcochlear approaches hold the key to treating complex cases. Infratemporal fossa approach type B has to be used for extension into the clivus, sphenoid sinus, or rhinopharynx. Internal carotid artery, jugular bulb, and sigmoid sinus involvement should be identified before operation.

Objective: The objective of this study was to examine the usefulness of intraoperative cochlear nerve monitoring (ICNM) in the preservation of normal and social hearing in vestibular schwannoma (VS) removal. Methods: A retrospective chart review was conducted. Of 1315 patients operated for VS between June 1988 and December 2005, 150 patients were subjected to hearing preservation surgery. Among these, 99 patients with preoperative normal and social hearing (class A and B in the modified Sanna classification) and with a small tumor Results: The hearing preservation rate was 26.7% in cases operated with ICNM and 20.8% in cases without ICNM. The difference did not reach statistical significance (p = 0.79). In subgroup analyses, the ICNM did not prove to contribute to the significantly higher hearing preservation rate. Conclusions: ICNM did not increase the ratio of patients with postoperative normal and social hearing in VS surgery.

Objective: Cholesteatoma of the petrous bone is a rare pathology that grows slowly and is often asymptomatic. High clinical suspect is recommended for its diagnosis. With the advancement of imaging, clinical diagnosis is confirmed and follow up is assured. The present study is presented to state our experience in management of petrous bone cholesteatoma (PBC) and an overview of the outcome results. Methods: This study was a retrospective case review conducted at a private quaternary referral center. From January 1984 to October 2004, 93 of 2,739 patients with chronic otitis media with cholesteatoma were treated surgically for PBC with varying location types. Results: The mean age in this study was 46 years. Ninety-four percent of patients presented with hearing loss. Facial paralysis constituted 55% of the presented manifestations and grade VI palsy was the most common. A massive type of PBC was found to affect 45% of patients and it was mainly the acquired pathology. The surgical approaches varied according to the class type of PBC. Postoperative complications were minimal, and follow up was carried out after 1 year from the surgery date. Conclusion: Petrous bone cholesteatoma presents difficulties in its diagnosis and treatment. Adequate history-taking, high clinical suspicious, with the advancement of imaging techniques made its diagnosis more feasible. Improvement of the lateral skull base approaches rendered possible safe, adequate, and complete removal of the pathology.

Objective To evaluate the results of facial nerve reconstruction by means of grafting and to determine the influence of different variables on final functional recovery. Study design Retrospective case review. Setting Tertiary otologic and skull base referral center. Patients Sixty nine patients underwent facial nerve grafting. Facial nerve tumors (24) or vestibular schwannomas (18) affected most of them. Preoperatively, 47 patients had a clinical facial nerve deficit lasting from 1 to 120 months, with a mean of 20.2 months. Main outcome measures Final facial nerve motor function. Results Among the 56 patients with a follow-up time equal to or longer than 1 year, 26 recovered to a Grade III (46.4%), 14 to a Grade IV (25%), while 16 remained at Grade V or Grade VI according to the House-Brackmann scale. Patients with a preoperative facial nerve deficit for more than 1 year showed the lowest rate of recovery, with only 3 cases (19%) reaching Grade III. Conclusions Duration of the preoperative facial nerve deficit emerged as the most important factor influencing final results. According to the authors' data, the 1-year period after the occurrence of the preoperative clinical deficit seemed to be the cutoff point in achieving a high rate of good postoperative recovery. Among the other factors that might have influenced the final outcome, only the underlying cause of the lesion played a significant role.

Objective To analyze the long term outcomes after surgery in tympanomastoid paragangliomas. Study design Retrospective study. Methods The charts of 145 patients with tympanomastoid paragangliomas managed between 1988 and 2013 were reviewed. The clinical features, audiological data, pre- and postoperative notes were noted. The tumors were staged according to the modified Fish and Mattox classification. The surgical approaches for all patients were formulated according to the surgical algorithm developed at our center. Results 34 (23.5%), 46 (31.7%), 22 (15.2%), 18 (12.4%) and 25 (17.2%) patients were diagnosed to have TMP class A1, A2, B1, B2 and B3 tumors respectively. Gross tumor resection was achieved in 141 (97.2%) patients. The facial nerve was uncovered in four patients and infiltrated in three. The cochlea was found eroded in seven cases. The mean follow-up was 48.4 months. Recurrence was seen in one patient (0.7%). In the cases where the facial nerve was preserved (n = 143), the nerve function was graded as HB grade 1 in 138 patients (97%). Postoperatively, the mean AC showed an improvement in all categories except in class B2 and B3, which corresponds to the classes that include patients who underwent subtotal petrosectomy. Conclusion We report the long term surgical outcomes in tympanomastoid paragangliomas in the largest series published till date. It is possible to completely eradicate all types of tympanomastoid paragangliomas with minimum sequelae by choosing the correct surgical approach to achieve adequate exposure for individual tumor classes as described in our classification and algorithm. Level of evidence IIb.

The authors evaluated the behavior of residual tumors and facial nerve outcomes after incomplete excision of vestibular schwannomas (VSs).The case records of all patients who underwent surgical treatment of VSs were analyzed. All patients in whom an incomplete excision had been performed were analyzed. Incomplete excision was defined as near-total resection (NTR), subtotal resection (STR), and partial resection (PR). Tumors in the NTR and STR categories were followed up with a wait-and-rescan approach, whereas the tumors in the PR category were subjected to a second-stage surgery and were excluded from this series. All patients included in the study underwent baseline MRI at the 3rd and 12th postoperative months, and repeat imaging was subsequently performed every year for 7-10 years postoperatively or as indicated clinically. Preoperative and postoperative facial function was noted.Of the 2368 patients who underwent surgery for VS, 111 patients who had incomplete excisions of VSs were included in the study. Of these patients, 73 (65.77%) had undergone NTR and 38 (34.23%) had undergone STR. Of the VSs, 62 (55.86%) were cystic and 44 (70.97%) of these cystic VSs underwent NTR. The residual tumor was left behind on the facial nerve alone in 62 patients (55.86%), on the facial nerve and vessels in 2 patients (1.80%), on the facial nerve and brainstem in 15 patients (13.51%), and on the brainstem alone in 25 patients (22.52%). In the 105 patients with normal preoperative facial nerve function, postoperative facial nerve function was House-Brackmann (HB) Grades I and II in 51 patients (48.57%), HB Grade III in 34 patients (32.38%), and HB Grades IV-VI in 20 patients (19.05%). Seven patients (6.3%) showed evidence of tumor regrowth on follow-up MRI. All 7 patients (100%) who showed evidence of tumor regrowth had undergone STR. No patient in the NTR group exhibited regrowth. The Kaplan-Meier plot demonstrated a 5-year tumor regrowth-free survival of 92%, with a mean disease-free interval of 140 months (95% CI 127-151 months). The follow-up period ranged from 12 to 156 months (mean 45.4 months).The authors' report and review of the literature show that there is undoubtedly merit for NTR and STR for preservation of the facial nerve. On the basis of this they propose an algorithm for the management of incomplete VS excisions. Patients who undergo incomplete excisions must be subjected to follow-up MRI for a period of at least 7-10 years. When compared with STR, NTR via an enlarged translabyrinthine approach has shown to have a lower rate of regrowth of residual tumor, while having almost the same result in terms of facial nerve function.

Objective To evaluate the role of lateral skull base approaches in the management of benign parapharyngeal space tumors and to propose an algorithm for their surgical approach. Methods Retrospective study of patients with benign parapharyngeal space tumors. The clinical features, radiology and preoperative management of skull base neurovasculature, the surgical approaches and overall results were recorded. Results 46 patients presented with 48 tumors. 12 were prestyloid and 36 poststyloid. 19 (39.6%) tumors were paragangliomas, 15 (31.25%) were schwannomas and 11 (23%) were pleomorphic adenomas. Preoperative embolization was performed in 19, stenting of the internal carotid artery in 4 and permanent balloon occlusion in 2 patients. 19 tumors were approached by the transcervical, 13 by transcervical–transparotid, 5 by transcervical–transmastoid, 6, 1 and 2 tumors by the infratemporal fossa approach types A, B and D, respectively. Total radical tumor removal was achieved in 46 (96%) of the cases. Conclusion Lateral skull base approaches have an advantage over other approaches in the management of benign tumors of the parapharyngeal space due to the fact that they provide excellent exposure with less morbidity. The use of microscope combined with bipolar cautery reduces morbidity. Stenting of internal carotid artery gives a chance for complete tumor removal with arterial preservation.

Objective This study aimed to determine whether postoperative facial nerve paralysis or surgical manipulation causing paralysis could be predicted on train responses during intraoperative facial nerve monitoring in acoustic neuroma surgery. Study Design and Setting This was a prospective study performed at a tertiary referral center. Patients and Methods Train responses were recorded on a floppy disk and compared with postoperative facial nerve function in 51 patients who underwent enlarged translabyrinthine acoustic neuroma surgery. Main Outcome Measures The number, duration, frequency, and peak-to-peak amplitude of train responses were analyzed and compared with postoperative facial nerve function. Results Trains were observed in 42 of 51 patients. Six of seven patients with high-amplitude trains more than 250 μV, and three of five patients with bomber-type high-frequency trains elicited during tumor dissection from the facial nerve or stretching the nerve, showed severe facial nerve dysfunction. On the other hand, seven of the nine patients with no trains also showed severe facial nerve dysfunction. Conclusions The presence of high-amplitude or high-frequency trains elicited by surgical manipulation to the facial nerve seems to indicate a critical situation for the facial nerve. However, certain types of mechanical trauma resulting in severe facial nerve paralysis cannot be identified by train responses.

Geniculate ganglion meningiomas are extremely rare lesions—only 14 cases have been reported in the literature. Two new cases of these tumors are described. On computed tomography and magnetic resonance imaging, both lesions appeared centered on the area of the geniculate ganglion, extending to the tympanic cleft and eroding the middle cranial fossa floor. The first case was treated through a middle cranial fossa approach. Because the tumor was so large in the second case, a subtotal petrosectomy was used. The authors review the literature to clarify the clinical and radiological characteristics of these tumors and their surgical treatment.

A case of a Jehovah's witness affected by an intracanalicular vestibular schwannoma with an extremely fast growth rate is presented. Nine months after presentation, the tumor reached 23 mm in the cerebellopontine angle. A partial removal through a retrosigmoid approach was planned. Because of the presence of a dominant high jugular bulb masquering the internal auditory canal, the intracanalicular portion of the tumor was left in place. The residual tumor grew 12 mm in 2 months. Even after a gross total removal through a middle cranial fossa approach, the tumor recurred, reaching the size of 30 mm in 17 months. A modified transcochlear approach was then performed, and the patient was free of disease at the last radiologic follow-up, 8 months after the surgery. We illustrate our strategy in treating this aggressive benign lesion with unusual behavior.

Summary Heterotopic brain tissue is a rare is congenital anomaly, it may present at any age but it is frequently in infancy. This anomaly can occur most frequently in nasal region, although rests elsewhere in the digestive tract, in facial tissue or in lungs have been reported. Heterotopic brain tissue has been defined as a mass composed of mature brain tissue, outside the cranial cavity or spinal canal. We present a 9 years old girl with history of left chronic otitis and nasal obstruction caused by heterotopic brain tissue in pterygopalatine fossa.

Objective: To study the clinical features, tumor characteristics and outcomes of giant cell tumors (GCTs) in the skull base based on long-term follow-up. We also report the largest series of GCTs in the temporal bone and the lateral skull base. Materials and Methods: A retrospective study was conducted of all GCTs managed at the Gruppo Otologico, a quaternary referral skull base institute, in Italy from 1993 to 2013. The clinical features, investigations, surgical management and follow-up were recorded. The surgical approaches used were infratemporal fossa approach (ITFA) type B and D and middle cranial fossa (MCF) approaches. Results and Observations: A total of 7 patients with GCTs of the skull base were treated at our institution. The principal complaints were hearing loss reported in 6 (85.71%) patients, tinnitus in 5 (71.43%) and swelling in 3 (42.9%). Pure-tone audiometry showed conductive hearing loss in 5 (71.43%) patients. High-resolution CT scan and MRI with gadolinium enhancement were done in all patients. Radiology showed involvement of the ITF and middle ear in 6 (85.71%) patients each, temporomandibular joint in 4 (57.14%) patients, invasions of the squamous part of the temporal bone, mastoid, MCF and greater wing of sphenoid in 3 (42.9%) patients each and the petrous bone in 2 (28.6%) patients. ITFA type B was applied as an approach for tumor removal in 5 (71.43%) patients, including a case where an additional MCF approach was employed, and ITFA type D and the transmastoid approach were applied in 1 (14.3%) patient each. Total tumor removal and successful cure was achieved in 6 (85.71%) patients. Subtotal removal leading to recurrence and eventual mortality was the result in 1 (14.3%) patient. Conclusions: A thorough knowledge of the anatomy of the skull base and the various skull base approaches is necessary to tackle GCTs. ITFA type B and D combined with MCF approaches provide good exposure of the tumor with minimal postoperative sequelae and good locoregional control. Recurrence due to either subtotal removal or suboptimal treatment may have disastrous consequences for the patient.

It was with great interest that we read the article titled “Malignant Transformation of Acoustic Neuroma/Vestibular Schwannoma 10 Years after Gamma Knife Stereotactic Radiosurgery” by Demetriades et al in the September 2010 issue of Skull Base.1

BACKGROUND: Vestibular schwannomas (VSs) are the most common cerebellopontine angle tumors, accounting for 75% of all lesions in this location. OBJECTIVE: To evaluate the results after removal of VS through the enlarged translabyrinthine approach, which is a widening of the classic translabyrinthine approach that gives larger access and provides more room to facilitate tumor removal and to minimize surgery-related morbidities. METHODS: This was a retrospective study of 1865 patients who underwent VS excision through the enlarged translabyrinthine approach between 1987 and 2009. Mean age was 50.39 years. Mean tumor size was 1.8 cm. Median follow-up was 5.7 years. RESULTS: Total removal was achieved in 92.33% of cases; 143 patients had incomplete resection with evidence of regrowth in 8. In the 1742 previously untreated patients, anatomic preservation of facial nerve was achieved in 1661 cases (95.35%), and House-Brackmann grade I or II was reached in 1047 patients (59.87%). Facial nerve outcome was significantly better in tumors ≤ 20 mm. Surgical complications included cerebrospinal fluid leakage in 0.85%, meningitis in 0.10%, intracranial bleeding in 0.80%, non--VII/VIII cranial nerve palsy in 0.96%, cerebellar ataxia in 0.69%, and death in 0.10%. The technical modifications that evolved with increasing experience are described. CONCLUSION: The enlarged translabyrinthine approach is a safe and effective approach for the removal of VS. In our experience, the complication rate is very low and tumor size is still the main factor influencing postoperative facial nerve function with a cutoff point at around 20 mm.

Objective Endolymphatic sac tumor (ELST) is a rare low grade adenocarcinoma of the skull base. During the past decade the number of the reported cases has increased. This study exposes our experience in the management of ELST with a review of the literature. Study design Retrospective study of patients with ELST at a quaternary referral otology and skull base center. Methods A review of the records from the Gruppo Otologico revealed 7 patients treated for ELST. All papers containing series of three or more cases of ELST published in the English literature were selected for analysis. Results Hearing loss and tinnitus were present in almost all our cases. All of them were evaluated with audiometric tests, computed tomography and magnetic resonance imaging. All the patients were treated surgically with preservation of the facial nerve and preoperative embolization was performed in 5 patients. Genetic study was performed on all our cases and revealed the presence of von Hippel–Lindau syndrome in one patient who had the tumor as the initial manifestation of his syndrome. None of the patients received postoperative radiotherapy and one of them had recurrence of the tumor 13 years following surgery. Conclusions Complete surgical resection with preoperative embolization of large tumors is the mainstay treatment for ELST. The facial nerve should not be sacrificed unless it is totally invaded by the tumor. A long term follow up is recommended and the role of postoperative adjunctive radiotherapy is still controversial.

To discuss our management strategy of vestibular schwannoma in patients with neurofibromatosis type 2 (NF2).Retrospective study.Quaternary referral skull base center.Thirty-eight NF2 patients who had undergone 48 operations at the Gruppo Otologico between January 1988 and December 2008. The mean age at time of surgery was 36.3 years (range, 17-65 yr), and the average tumor size was 3.1 cm (range, 0.6-6 cm). There were 27 female and 21 male ears, and 25 cases were right side ears, whereas 23 were left sided. The average follow-up time was 3.7 years. Surgical approaches, hearing, and facial nerve functions, as well as hearing rehabilitation and facial nerve reconstruction outcomes, are discussed.Total tumor resection was achieved in 44 cases (92%). Facial nerve function was postoperatively House-Brackmann grades I-III in 36 cases (77%); it was grade I in 17 cases (35%) and grade II in 8 cases (17%). In 7 cases, hearing preservation was attempted, and a measurable hearing has been recorded in 5 cases (71%). Auditory brainstem implant was inserted in 25 cases, and concomitant cochlear implants were inserted in 5 cases.Early diagnosis and treatment of bilateral vestibular schwannoma in patients with NF2 will achieve the best outcomes regarding facial nerve, hearing preservation, and postoperative complications. The watchful waiting policy will decrease the chance of reaching these goals. Cochlear implants and auditory brainstem implant have made hearing rehabilitation possible in NF2 patients who had bilateral sensorineural hearing loss.

A relation between conventional radiotherapy and the development of intracranial neoplasma is well known, but radiation-associated tumor following stereotactic radiotherapy of vestibular schwannoma is underestimated. In this article we will study this relation by doing a complete literature review on all the malignant intracranial tumors that appeared following radiosurgery and adding a case of malignant vestibular schwannoma following stereotactic radiotherapy in a Neurofibromatosis type 2 patient.Literature review and discussion.We found 26 cases of malignant brain tumor following stereotactic radiotherapy including our case. In 13 cases the tumor occurred in context of Neurofibromatosis type 2. None of the patients had a tumor size less than 2.5 cm. and the mean latency period between the radiotherapy and malignant tumor development was 5.8 years.Patients with vestibular schwannoma should be made aware of the low incidence of the radiation-induced malignant changes and long-term follow-up is mandatory.

Objective To describe and analyze the main outcomes achieved in a series of patients with sporadic vestibular schwannoma (VS) larger than 40 mm in extrameatal diameter, defined as giant VS, submitted to microsurgery by the enlarged translabyrinthine approach with transapical extension. Study design Retrospective chart review. Setting Tertiary referral center. Patients A retrospective chart review was conducted on 2,133 patients who underwent surgery for VS from April 1987 to July 2009. One hundred ten cases of giant VS were elected for analysis. Main outcome measures Extent of removal, residual or recurrent disease, facial nerve integrity during surgery, long-term facial nerve function, and postoperative complications. Results Total removal was accomplished in 91.8% of cases. In 5 patients (4.5%), total removal was accomplished in 2 stages. Near-total removal was performed in 7 patients (6.3%). The facial nerve was anatomically preserved in 76.4% of cases. Intraoperative facial nerve reconstruction was performed in 8 cases. Facial nerve function after 1 year of follow-up was House-Brackmann grades I to III in 75% of cases. There were no deaths in this series. Neurovascular life-threatening complications occurred in 2 patients. Cerebrospinal fluid leak was present in 1.8% of cases. Conclusion Results indicate the enlarged translabyrinthine approach with transapical extension as an elective approach for removal of giant VS. The method permits achievement of a high rate of total removal with low incidence of complications.

Objective: To discuss the classification of petrous bone cholesteatoma (PBC) and add a subclassification; to review the existing literature and to propose the ideal surgical management of PBC based upon the experience of the largest series published in the literature until now. Study Design: Retrospective analysis. Setting: Quaternary referral neuro-otologic private practice. Materials and Methods: The data of 129 patients who underwent surgery for PBC between 1979 and 2008 were analyzed with respect to the classification, type of the approach used, facial nerve lesion and its management, recurrences and outcome. Results: Out of the 129 PBC cases 64 were supralabyrinthine, 9 infralabyrinthine, 7 infralabyrinthine-apical, 48 massive and 1 apical. The facial nerve was involved in 95% of the cases. Hearing could not be preserved in 82% of the cases due to the extent of the lesions and the surgical approaches used. The internal carotid artery, jugular bulb and the lower cranial nerves were infrequently involved, but demanded careful identification and meticulous care to avoid complications. Obliteration of the cavities provided a safe solution for protection of the exposed dura and the vital neurovascular structures. Recurrences were observed in 5 cases. Conclusion: The classification of PBC is fundamental to choose the appropriate surgical approach; the facial nerve is involved in almost all the cases, radical removal takes priority over hearing preservation and cavity obliteration is important to protect the vital neurovascular structures which may be exposed.