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.

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.

Relevance of Modified Bondy Mastoidectomy in Pediatric Cholesteatoma.

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

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

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

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

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

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

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

Meningiomas of the Internal Auditory Canal.

OBJECTIVE: Identify and define specific preoperative and postoperative characteristics of intracanalicular meningiomas (ICMs) in order to improve their diagnosis and management, and to differentiate them from intrameatal vestibular schwannomas (IMVSs). METHODS: Preoperative symptomatology, magnetic resonance imaging (MRI), and postoperative outcomes of 28 ICMs were analyzed. The results were compared to the literature and IMVSs treated by our group. RESULTS: Anacusis and progressive hearing loss were more frequent in the present population than the cases reviewed (P = .0064 and P = .0001, respectively). Hearing loss affected more than 90% of the patients, with anacusis in 32.1% of the cases. Facial palsy affected 17.9% of the patients. In comparison to IMVSs, preoperative anacusis was more associated to meningiomas (P = .0037), and the facial nerve was more compromised in ICMs than IMVSs, both preoperatively (P = .0011) and at follow-up (P < .0001). According to a re-evaluation of preoperative MRIs and comparison with IMVSs, linear tumor borders, and linear morphology along the internal auditory canal wall, but not the presence of a dural tail, were significantly more present in ICMs (P = .0035, P = .0004, P = .1963, respectively). These characteristics could have led to a correct preoperative diagnosis in 61% of our cases. CONCLUSION: Contrariwise to IMVSs, the frequent preoperative anacusis and facial palsy demonstrate the more aggressive nature of ICMs, which also carry a higher risk of postoperative facial palsy and difficulty to preserve hearing. An attentive evaluation of imaging should ease diagnosis, and asymptomatic or stable ICMs should be enrolled in a wait-and-scan protocol. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E413-E419, 2021.

Decision Making in the Wait-and-Scan Approach for Vestibular Schwannomas: Is There a Price to Pay in Terms of Hearing, Facial Nerve, and Overall Outcomes?

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.

Surgical technique and results of cable graft interpositioning of the facial nerve in lateral skull base surgeries: experience with 213 consecutive cases.

OBJECTIVE The 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. METHODS The 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. RESULTS Two 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). CONCLUSIONS Cable 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.

Rapidly growing cystic vestibular schwannoma with sudden onset facial palsy, ten years after subtotal excision.

An elderly male patient diagnosed with a right-sided cystic vestibular schwannoma (CVS) at our center underwent a translabyrinthine approach with a subtotal excision to preserve the facial nerve (FN). The tumor grew slowly for the first 9 years but in the subsequent 2 years grew rapidly, with the patient developing a FN paralysis. Using the previous approach, a second surgery was done and the tumor was excised, leaving behind a sheath of tumor on the facial and lower cranial nerves. This case demonstrates that CVSs show unpredictable growth patterns and need to be followed up for a longer period of time. Laryngoscope, 128:1649-1652, 2018.

Surgical Management of Intrinsic Tumors of the Facial Nerve.

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.

Surgical Strategy and Facial Nerve Outcomes in Petrous Bone Cholesteatoma.

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.

Outcomes of Drill Canalplasty in Exostoses and Osteoma: Analysis of 256 Cases and Literature Review.

OBJECTIVE: 1) To describe the surgical technique of drill canaplasty for exostosis and osteoma and to evaluate our results. 2) To propose a new grading system for external auditory canal stenosis (EACS). 3) To review the recent literature. STUDY DESIGN: A retrospective review. SETTING: Quarternary referral center for Otology & Skull Base surgery. SUBJECTS AND METHODS: Two hundred seventeen patients (256 ears) with exostosis or osteoma were included in the study. Surgical and audiological parameters were evaluated. RESULTS: Mean age was 51.5 (±13.41) years. One hundred sixty nine cases were men and 48 women. Two hundred forty three (95%) cases were exostosis and 13 (5%) were osteomas. According to the proposed grading system, 81% ears had severe or complete stenosis. Seventy eight (30.5%) ears had a concurrent diagnosis of otosclerosis. Retroauricular approach was used in 245 (95.7%). Intraoperative complications included tympanic membrane (TM) perforation seen in four (2%) and mastoid cell exposure in two (1%). Postoperative stenosis/prolonged healing was seen in 11 (4%) patients and required revision in seven cases. Audiologic analysis available for 153 ears-showed the mean change in air-bone gaps (ABG) of 2.18 dB, pure tone averages (PTA) bone conduction (BC) (0.5-4 kHz) of 0.3 dB. Mean healing rate was available for 246 (96.1%) patients and was found to be 6.35 (4-16) weeks. CONCLUSIONS: A systematically performed drill canalplasty via retroauricular approach, as described in this article, yields excellent postoperative outcomes as seen in our series. Notably, one-third of exostoses patients in this series, also suffered from otosclerosis. The proposed grading system for EACS enables the surgeon to objectively stage the disease.

Staged resection for vestibular schwannoma.

CONCLUSION: Surgery remains the preferred option for large vestibular schwannoma (VS). The presence of unpredictable intraoperative difficulties may convince the operator to suspend the surgery to avoid risks to patient life. Additional surgeries may be mandatory and are better performed using a transcochlear approach. High rates of complications, poor facial nerve results, and a percentage of incomplete removals should be expected in such unfavorable cases. OBJECTIVES: To review the results for nine cases of huge VS treated by staged resection. METHOD: A retrospective case review was performed for all nine patients who underwent staged resection of VS at the Gruppo Otologico between 1984-2012. The decision to perform staged surgery was always made intra-operatively after encountering unpredicted difficulties. RESULTS: The nine patients represented 0.3% of all patients who underwent VS surgery during the same period. Mean tumor size was 4.7 cm (range = 3.0-6.6 cm). Two cases required three surgeries, resulting in a total of 20 operations. In addition, two cases required pre-operative ventriculoperitoneal shunt and one required temporary tracheotomy. After the final stage of surgery, complete removal had been achieved in six of the nine patients. The facial nerve was never preserved anatomically.

The behavior of residual tumors and facial nerve outcomes after incomplete excision of vestibular schwannomas.

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.

Vestibular schwannoma in the only hearing ear: role of cochlear implants.

OBJECTIVES: We sought to delineate the role of cochlear implantation in the management of vestibular schwannoma or other cerebellopontine angle tumors in the only hearing ear. METHODS: We performed a retrospective analysis in a quaternary referral skull base center of all patients who were affected by vestibular schwannoma (or other lesions of the cerebellopontine angle) in the only hearing ear and received a cochlear implant before or after tumor treatment (surgery or radiotherapy) or during the wait-and-scan follow-up. We also performed a systematic review of the English-language literature. RESULTS: The clinical and audiological results of 10 patients are reported. All patients were managed with contralateral cochlear implantation. In 7 patients, cochlear implantation was performed before tumor removal, while hearing in the ear with the tumor was still present. In 3 patients, the implant was placed after curative surgery. Nine of the 10 patients routinely use their implant with subjective benefit and fairly good auditory performance (median disyllabic word recognition, 90%; median sentence comprehension, 75%). The literature search retrieved no major series with assessment of the long-term efficacy of cochlear implantation in this rare clinical scenario. CONCLUSIONS: Patients affected by vestibular schwannoma in their only hearing ear may significantly benefit from a cochlear implant on the contralateral side prior to tumor removal. Recent and significant hearing deterioration and tumor growth represent the main indications for cochlear implantation.

The Gruppo Otologico experience of endolymphatic sac tumor.

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.

Salvage surgery of vestibular schwannoma after failed radiotherapy: the Gruppo Otologico experience and review of the literature.

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.

Rate of recurrent vestibular schwannoma after total removal via different surgical approaches.

OBJECTIVES: The objective of this study was to assess the differences in the recurrence rates of vestibular schwannoma (VS) after total tumor removal through enlarged translabyrinthine (ETL), retrosigmoid (RS), and middle cranial fossa (MCF) approaches. Our results were compared with previously published data, and literature reviews were done to identify the possible causes for the recurrence of VS. METHODS: We performed a retrospective analysis of 2,400 cases of VS that underwent removal at the Gruppo Otologico, Piacenza, Italy, from 1983 until 2010. The minimum postoperative follow-up was 12 months. We also reviewed the previously published data on recurrence rates of VS after ETL, RS, and MCF approaches. RESULTS: Total tumor removal was achieved in 2,252 cases (93.8%). The recurrence rate was 0.05% for the ETL approach, 0.7% for the RS approach, and 1.8% for the MCF approach. Literature reviews of 3 previously published case series utilizing the translabyrinthine approach showed that none of the primary tumors were less than 2.0 cm in size. Recurrences were seen between 1 and 13 years after the initial surgery. CONCLUSIONS: The rate of VS recurrence after total removal is exceptionally low in experienced hands. Undetected microscopic deposits left on crucial points such as the facial nerve, the preserved cochlea nerve, or the fundus of the internal auditory canal could be possible causes for the recurrence. A definite advantage of an ETL approach is the excellent internal auditory canal exposure, resulting in an extremely low rate of VS recurrence. The patients should be followed up to 15 years with gadolinium-enhanced magnetic resonance imaging (with fat suppression sequence in ETL approach cases). Recurrent VS may exhibit a faster growth rate than primary VS.

Surgical results and technical refinements in translabyrinthine excision of vestibular schwannomas: the Gruppo Otologico experience.

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.