Optimal Volume of the Residual Tumor to Predict Long-term Tumor Control Using Stereotactic Radiosurgery after Facial Nerve-preserving Surgery for Vestibular Schwannomas.

BACKGROUND: Intended subtotal resection (STR) followed by adjuvant gamma knife radiosurgery (GKRS) has emerged as an effective treatment option for facial nerve (FN) preservation in vestibular schwannomas (VSs). This study aimed to identify the optimal cut-off volume of residual VS to predict favorable outcomes in terms of both tumor control and FN preservation. METHODS: This retrospective study assessed the patients who underwent adjuvant GKRS for residual VS after microsurgery. A total of 68 patients who had been followed up for ≥ 24 months after GKRS were included. Tumor progression was defined as an increase in tumor volume (TV) of ≥ 20%. House-Brackmann grades I and II were considered to indicate good FN function. RESULTS: The median residual TV was 2.5 cm³ (range: 0.3-27.4). The median follow-up period after the first adjuvant GKRS was 64 months (range: 25.7-152.4). Eight (12%) patients showed tumor progression. In multivariate analyses, residual TV was associated with tumor progression (P = 0.003; hazard ratio [HR], 1.229; 95% confidence interval [CI], 1.075-1.405). A residual TV of 6.4 cm³ was identified as the cut-off volume for showing the greatest difference in progression-free survival (PFS). The 5-year PFS rates in the group with residual TVs of < 6.4 cm³ (54 patients) and that with residual TVs of ≥ 6.4 cm³ (14 patients) were 93.3% and 69.3%, respectively (P = 0.014). A good FN outcome was achieved in 57 (84%) patients. Residual TV was not associated with good FN function during the immediate postoperative period (P = 0.695; odds ratio [OR], 1.024; 95% CI, 0.908-1.156) or at the last follow-up (P = 0.755; OR, 0.980; 95% CI, 0.866-1.110). CONCLUSION: In this study, residual TV was associated with tumor progression in VS after adjuvant GKRS following STR. As preservation of FN function is not correlated with the extent of resection, optimal volume reduction is imperative to achieve long-term tumor control. Our findings will help surgeons predict the prognosis of residual VS after FN-preserving surgery.

Will the real Moebius syndrome please stand up? A systematic review of the literature and statistical cluster analysis of clinical features.

Moebius syndrome is a highly variable syndrome with abducens and facial nerve palsy as core features. Strict diagnostic criteria do not exist and the inconsistency of the associated features makes determination difficult. To determine what features are associated with Moebius syndrome we performed a systematic literature review resulting in a composite case series of 449 individuals labeled with Moebius syndrome. We applied minimum criteria (facial and abducens palsy) to determine the prevalence of associated clinical features in this series. Additionally, we performed statistical cluster analysis to determine which features tended to occur together. Our study comprises the largest series of patients with Moebius syndrome and the first to apply statistical methodology to elucidate clinical relationships. We present evidence for two groups within the Moebius diagnosis. Type 1: exhibiting micrognathia, limb anomalies and feeding/swallowing difficulty that tend to occur together. Type 2: phenotypically diverse but more associated with radiologically detectable neurologic abnormalities and developmental delay.

Facial nerve dysfunction after superficial parotidectomy with or without continuous intraoperative electromyographic neuromonitoring: a prospective randomized pilot study.

PURPOSE: There are no randomized trials comparing the incidence or severity of facial nerve dysfunction after superficial parotidectomy with or without continuous intraoperative electromyographic neuromonitoring. This pilot study aimed to assess the variability in outcomes to help determine the needs and possible ethical issues in a full-scale study. METHODS: Prospective randomized pilot study comparing the incidence and grade of facial nerve dysfunction among 106 patients subjected to superficial parotidectomy with or without continuous four channels electromyographic neuromonitoring (52 monitored patients and 54 controls). RESULTS: The incidences of immediate (38.3% vs. 51.8%, p = 0.1) and late facial dysfunction, up to 180 days following surgery, (3.8% vs. 5.5%, p = 0.4) were similar between monitored patients and controls. Immediate facial nerve dysfunction with a House-Brackmann ≥ grade III was more frequent among the non-monitored patients (57.8% vs. 30%, p = 0.2), and outcomes were significantly poorer in this group (mean sum score of 68.7 vs. 81.5, p = 0.002), when assessed with the regional Sunnybrook scale. A full-scale prospective randomized study to detect a significant reduction in the incidence of immediate facial nerve dysfunction with the use of continuous intraoperative electromyographic neuromonitoring, with 80% power and a 5% significant level, would require 560 patients allocated to the monitored and control groups. Considering a mean rate of 30 patients/year/center, such a study would require the participation of five centers for 4 years. CONCLUSIONS: In the present pilot study, the incidences of immediate and late facial nerve dysfunction were similar between patients with benign parotid tumors subjected to superficial parotidectomy with or without continuous intraoperative electromyographic neuromonitoring. However, immediate facial dysfunction was more severe among the non-monitored patients.

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.

Universal Bypass for Treatment of Symptomatic Moyamoya Disease or Moyamoya Syndrome. Analysis of a Personal Case Series on Behalf of the Italian Moyamoya Association.

BACKGROUND: Moyamoya (MM) is a very rare cerebrovascular disease, particularly in Caucasians. We describe the results of an Italian case series where the mainstay of treatment was a bypass or a combined approach. METHODS: An analysis of a prospectively collected database was carried out. The main objective was to investigate (1) the risk of perioperative stroke and surgical complications, (2) the risk of new ischemic events, and (3) the risk of new hemorrhages at follow-up (mean follow-up: 2.2 years). RESULTS: Between January 2011 and January 2015 we carried out 34 bypasses in 23 patients with MM (15 MM disease, 5 unilateral MM, 3 MM syndrome); mean age was 34 (range:1-57). The mortality and definitive morbidity rates were 0 %. Two patients suffered from transient aphasia and one developed partial palsy of the facial nerve. Five of the 12 patients with preoperative fixed deficits improved. No patient with preoperative ischemia experienced new ischemic symptoms. Rebleeding occurred in 1 of the 11 patients with a hemorrhagic presentation (9 %). CONCLUSIONS: The bypass/combined approach to MM appears to have a favorable risk profile and preventive effectiveness, particularly on TIAs and ischemic stroke.

Somatic tinnitus prevalence and treatment with tinnitus retraining therapy.

OBJECTIVES: Somatic tinnitus originates from increased activity of the dorsal cochlear nucleus, a cross-point between the somatic and auditory systems. Its activity can be modified by auditory stimulation or somatic system manipulation. Thus, sound enrichment and white noise stimulation might decrease tinnitus and associated somatic symptoms. The present uncontrolled study sought to determine somatic tinnitus prevalence among tinnitus sufferers, and to investigate whether sound therapy with counselling (tinnitus retraining therapy; TRT) may decrease tinnitus-associated somatic symptoms. METHODS: To determine somatic tinnitus prevalence, 70 patients following the TRT protocol completed the Jastreboff Structured Interview (JSI) with additional questions regarding the presence and type of somatic symptoms. Among 21 somatic tinnitus patients, we further investigated the effects of TRT on tinnitus-associated facial dysesthesia. Before and after three months of TRT, tinnitus severity was evaluated using the Tinnitus Handicap Inventory (THI), and facial dysesthesia was assessed with an extended JSI-based questionnaire. RESULTS: Among the evaluated tinnitus patients, 56% presented somatic tinnitus-including 51% with facial dysesthesia, 36% who could modulate tinnitus by head and neck movements, and 13% with both conditions. Self-evaluation indicated that TRT significantly improved tinnitus and facial dysesthesia in 76% of patients. Three months of TRT led to a 50% decrease in mean THI and JSI scores regarding facial dysesthesia. CONCLUSIONS: Somatic tinnitus is a frequent and underestimated condition. We suggest an extension of the JSI, including specific questions regarding somatic tinnitus. TRT significantly improved tinnitus and accompanying facial dysesthesia, and could be a useful somatic tinnitus treatment.

Surgical management of posterior petrous meningiomas.

BACKGROUND AND PURPOSE: The aim of the study is to present our experience in the surgical treatment of posterior petrous meningiomas in regard to clinical presentation, surgical anatomy, complications and long-term functional postoperative results. MATERIAL AND METHODS: A series of 48 consecutive patients operated on for posterior petrous meningiomas at the authors' institution between 2002 and 2011 is reported. The main symptom on first admission was hypoacusis, impairment of the fifth cranial nerve and cerebellar ataxia. The tumour was found to be attached to the premeatal dura in 46%, the inframeatal dura in 29% and the postmeatal dura in 25% of cases. Tumour resection was categorized as grade I in 16 patients, grade II in 29 patients, grade III in 1 patient and grade IV in 2 patients, according to the Simpson classification system. The petrosal approach and retrosigmoid approach were suitable for posterior petrous meningiomas. RESULTS: Postoperative facial nerve dysfunction appeared in 8 and further deteriorated in 2 patients. Hearing function deteriorated after surgery in 8 and improved in 2 cases. Perioperative death occurred in two patients. Tumour recurrence was observed in two patients, and both underwent a second operation and postoperative stereotactic radiotherapy. CONCLUSIONS: Surgical treatment of posterior petrous meningiomas has become increasingly safe but these tumours still remain a surgical challenge because of the relatively high incidence of permanent complications associated with their removal. The site of displacement of the cranial nerves depending on the dural origin of the tumour has the most influence on the related difficulties in its removal.

Characteristics and outcome of facial nerve palsy from Lyme neuroborreliosis in the United States.

OBJECTIVES: Facial palsy is the most common manifestation of Lyme neuroborreliosis (LNB) in the United States. This study aimed to describe features of patients with early LNB presenting with facial palsy and to determine if corticosteroids in addition to antibiotic therapy was associated with unfavorable outcome. METHODS: Retrospective analysis of participants enrolled in clinical studies investigating Lyme disease (N = 486) identified 44 patients who had facial palsy from LNB. The House-Brackmann scale was used to quantify the facial nerve dysfunction. RESULTS: Most patients presented in the summer months. Erythema migrans, frequently associated with systemic symptoms, occurred in 29 patients. Thirteen patients presented with bilateral facial palsy, usually with sequential involvement. Fourteen patients had painful radiculopathy. Of the 38 patients treated with antibiotics before the resolution of the palsy who had complete follow-up, 24 received both antibiotics and corticosteroids. Of these 38 patients, 34 recovered completely, 3 had nearly complete recovery, and 1 had moderate dysfunction. There were no differences between the treatment groups in achieving complete resolution of the palsy at 12 months or in time to complete recovery. INTERPRETATION: A history of rash compatible with erythema migrans or febrile illness in the weeks preceding the palsy are helpful clues pointing toward LNB and should be actively sought when evaluating patients with acute-onset peripheral facial palsy, particularly bilateral facial palsy. Treatment with antibiotic therapy is highly effective and most patients will fully recover facial nerve function. Adjunctive corticosteroid therapy appears to not affect the speed of recovery or overall outcome in this retrospective observational study.

Cranial nerve vascular compression syndromes of the trigeminal, facial and vago-glossopharyngeal nerves: comparative anatomical study of the central myelin portion and transitional zone; correlations with incidences of corresponding hyperactive dysfunctional syndromes.

OBJECTIVE: The aim of this study was to evaluate the anatomy of the central myelin portion and the central myelin-peripheral myelin transitional zone of the trigeminal, facial, glossopharyngeal and vagus nerves from fresh cadavers. The aim was also to investigate the relationship between the length and volume of the central myelin portion of these nerves with the incidences of the corresponding cranial dysfunctional syndromes caused by their compression to provide some more insights for a better understanding of mechanisms. METHODS: The trigeminal, facial, glossopharyngeal and vagus nerves from six fresh cadavers were examined. The length of these nerves from the brainstem to the foramen that they exit were measured. Longitudinal sections were stained and photographed to make measurements. The diameters of the nerves where they exit/enter from/to brainstem, the diameters where the transitional zone begins, the distances to the most distal part of transitional zone from brainstem and depths of the transitional zones were measured. Most importantly, the volume of the central myelin portion of the nerves was calculated. Correlation between length and volume of the central myelin portion of these nerves and the incidences of the corresponding hyperactive dysfunctional syndromes as reported in the literature were studied. RESULTS: The distance of the most distal part of the transitional zone from the brainstem was 4.19 ± 0.81 mm for the trigeminal nerve, 2.86 ± 1.19 mm for the facial nerve, 1.51 ± 0.39 mm for the glossopharyngeal nerve, and 1.63 ± 1.15 mm for the vagus nerve. The volume of central myelin portion was 24.54 ± 9.82 mm(3) in trigeminal nerve; 4.43 ± 2.55 mm(3) in facial nerve; 1.55 ± 1.08 mm(3) in glossopharyngeal nerve; 2.56 ± 1.32 mm(3) in vagus nerve. Correlations (p < 0.001) have been found between the length or volume of central myelin portions of the trigeminal, facial, glossopharyngeal and vagus nerves and incidences of the corresponding diseases. CONCLUSION: At present it is rather well-established that primary trigeminal neuralgia, hemifacial spasm and vago-glossopharyngeal neuralgia have as one of the main causes a vascular compression. The strong correlations found between the lengths and volumes of the central myelin portions of the nerves and the incidences of the corresponding diseases is a plea for the role played by this anatomical region in the mechanism of these diseases.

Efficacy of modified face lift incision for the resection of benign parotid gland tumor located anteriorly or superiorly.

OBJECTIVES: The goals of resection of benign parotid gland tumor are complete resection of lesion and preservation of the facial nerve function. Traditionally, the bayonet-shaped incision (Blair incision: BI) and the modified face lift incision (mFLI) are commonly used for parotidectomy. However, concerns exist about the adequacy of exposure and identification of the facial nerve in anterior or superior parotid lesions. The aim of this study was to compare the surgical outcomes between BI and mFLI and to evaluate the adequacy, possible indications, and limitations of mFLI for the resection of benign parotid gland tumors located anteriorly or superiorly. METHODS: This retrospective study analyzed the medical records of 175 patients with various types of benign parotid tumor who underwent partial parotidectomy via BI (97 patients) or mFLI (78 patients). Tumors were divided into five categories depending on their location: anterior, superior, inferior, middle, and deep lobe tumors. The outcomes of operation were analyzed according to tumor location between the incision types. RESULTS: Tumor locations were not significantly different between the two groups. Transient facial palsy occurred in 23 out of 152 patients (15.1%); permanent palsy was not observed in either group. The incidence rates of facial palsy were higher among patients with superior and deep lobe tumors; in the mFLI and BI groups, proportions of superior tumors were 22.2% and 27.2%, respectively, and those of deep lobe tumors were 35.7% and 23.5%, respectively. With regard to superior and anterior tumors, the incidence rate of postoperative facial palsy was insignificantly lower in the mFLI group (10.5%) than in the BI group (18.2%). CONCLUSIONS: There were no differences in the incidence rates of postoperative facial palsy between mFLI and BI for any tumor location. Use of the mFLI is feasible for the resection of most benign parotid tumors located anteriorly or superiorly.

Combined sialendoscopic/mini-preauricular microscopic approach for large proximal parotid sialolithiasis.

OBJECTIVE: To describe and evaluate a combined approach for sialendoscopic stone localization with microscopic mini-preauricular incision external stone extraction as a gland-sparing minimally invasive surgical management in cases of large proximal duct or intraparenchymal parotid gland sialolithiasis. METHODS: A retrospective chart review of a single primary surgeon's patient series of 21 cases operated in a 5-year period in a tertiary care university and private practice hospitals. RESULTS: Study included 16 males and five females, with age range 12-68 years (mean 40.9 ± 14.5). Nineteen out of the 21 patients had their stones completely removed (90.5%), with two not completing the procedure due to inability of intraoperative endoscopic stone visualization. In total 25 stones were extracted with six patients having two stones. Longest diameter of single (or first) stone was 5-16 mm (mean 9.1 ± 2.9) and second was 3-5 mm (mean, 3.9 ± 0.6). Endoscopic findings showed 14/25 stones in the proximal main parotid duct and 11/25 in one of its secondary parenchymal branches. Stents were used in 4/19 cases (21.1%). No major complications occurred. Minor complications included two postoperative conservatively managed seromas. All 19 cases had completely intact facial nerve function, good parotid salivary flow and acceptable esthetic result after median follow-up period of 26 months (range 6-62). CONCLUSION: The combined sialendoscopic/microscopic mini-preauricular approach is a highly effective and safe gland-preserving method for large proximal parotid sialolithiasis management with a main limitation being inability to visualize the stone endoscopically.

Facial nerve palsy: etiology and approach to diagnosis and treatment.

Facial nerve palsy has a broad differential diagnosis and possible psychological and anatomical consequences. A thorough investigation must be performed to determine the cause of the palsy and to direct treatment. If no cause can be found, therapy with prednisone with or without an antiviral medication can be considered and begun as early as possible after onset of symptoms. Resolution and time to recovery vary with etiology, but overall prognosis is good.

Facial nerve dehiscence and cholesteatoma: Pediatrics vs adults.

OBJECTIVES: the aim of this paper is to study the incidence of facial canal dehiscence (FCD) in pediatric and non-pediatric patients, analyzing eventual differences in frequency, localization, primary or revision surgery and duration of the disease. METHODS: 527 patients suffering from chronic otitis media with acquired cholesteatoma, divided into two groups, those aged 18 years or over (470 patients), and those aged below 18 years (57 patients). RESULTS: Total incidence of FCD in adult population was 25,7% (121/470) and 7% (4/57) in pediatric one. Globally higher values were found in revision surgery, 43,5% (40/92) in adults and 16,7% (1/7) in children. Diseases longer than 5 years have been correlated to higher incidence of FCD in adults, 29,9% (109/364), than in pediatrics, 7,3% (3/41). No statistical significant difference has been found in those with a disease shorter than 5 years: 11,3% in adults (12/106) and 6,2% in children (1/16). The majority of patients, both adults and pediatrics, had a dehiscence in the tympanic tract of facial nerve. No statistical correlation regarding concomitant semicircular canal fistula and FCD has been found due to the absence of data in children. CONCLUSIONS: Incidence of FCD is higher in adult population than in pediatric. Adults have a higher incidence in primary surgery than children. No statistical significant difference has been found in adults and pediatrics with a less than 5 years disease, while diseases longer than 5 years expose adults to higher risk of FCD. Middle tract of tympanic segment is the most involved site of dehiscence in both populations.

Sarcoidosis of the central nervous system: Safety and efficacy of treatment, and experience of biological therapies.

OBJECTIVES: Neurological complications of sarcoidosis are uncommon and the natural history and optimal treatments under-researched. With the advent of biological therapies, it is important to define the clinical characteristics and immunopathology of the disease. PATIENTS AND METHODS: Patients referred to and treated within the Centre for Neurosarcoidosis over a 15 year period who had biopsy proven "highly probable" disease of the central nervous system were studied prospectively. RESULTS: Corticosteroids were used effectively in all patients, immunosuppression in 79 % and TNFα antagonists in 23 %. Treatment with steroids alone inevitably led to relapse, and low dose immunosuppression was ineffective in those with severe forms of the disease. Use of biological therapies substantially improved outcome. Patients with cranial neuropathy had an excellent outcome. Those with pachymeningitis had marked radiological abnormalities but less disablement. Those with leptomeningitis had an invasive, destructive disease which responded well to treatment but with residual neurological impairments. Treatment was required for many years, but the risk of relapse following treatment withdrawal was low. Infective complications arose in six. There were two deaths, neither directly related to the neurological disease, nor its treatment. CONCLUSIONS: This prospective study of the natural history and treatment response in neurosarcoidosis provides evidence that the use of high dose immunosuppression and early and prolonged use of biological therapies is associated with greatly improved outcomes and lower mortality. The data may be used to plan further studies and treatment trials, and provide class IV evidence for the effectiveness of biological agents in the treatment of Neurosarcoidosis.

Postoperative Functional Preservation of Facial Nerve in Cystic Vestibular Schwannoma.

OBJECTIVE: To retrospectively analyze clinical and surgical data of a consecutive series of 26 patients with unilateral cystic vestibular schwannomas. METHODS: Tumors were classified as type A (central cyst) and type B (peripheral cyst) and as small (tumor diameter <3 cm) and large (tumor diameter >3 cm). All patients underwent microsurgical removal via retrosigmoid approach. The course of the facial nerve (FN) was classified as anterior, anterior-inferior, anterior-superior, and dorsal to the tumor's surface. RESULTS: Mean patient age was 53.5 years. Mean tumor size was 3.2 cm. There were 22 cases classified as type A and only 4 as type B. Total or near-total resection (>95%) was achieved in 16 cases (61.5%), subtotal removal (90%-95%) was achieved in 9 cases (34.6%), and partial removal (<90%) was achieved in 1 case (3.9%). Position pattern of FN was anterior-inferior in 10 cases (38.4%), anterior-superior in 10 cases (38.4%), anterior in 23.2% of 6 cases. At hospital discharge, FN function was House-Brackmann grade I-V in 9 (36%), 10 (38%), 3 (12%), 3 (12%), and 1 (4%) patients; at final follow-up, House-Brackmann grades I, II, III, and IV accounted for 18 (72%), 6 (24%), 1, and 1 cases. During follow-up ranging from 6 months to 10 years, reoperation for growing of residue was never necessary. CONCLUSIONS: According to the literature and the results of our series, microneurosurgery of cystic vestibular schwannomas is associated with good outcomes in terms of extent of resection and FN function. In particular, long-term FN function is much more satisfactory than short-term function. In most cases, microsurgery represents the treatment of choice of cystic vestibular schwannomas.

[Defects of facial nerve canal according to a character and localisation of lesions in middle ear]. / Ubytki kostne kanalu nerwu VII a charakter i lokalizacja zmian w uchu srodkowym.

AIM: The aim of this study is the assessment the occurence of facial nerve canal (Fallopian canal) dehiscence in patients operated due to chronic medial otitis (depending on the location and type of inflammatory lesions) and in patients operated due to otosclerosis. Facial nerve paresis in patients with dehiscence found during surgery was also assessed. MATERIAL AND METHODS: The study group consisted of 456 patients operated at the Department of Otolaryngology at the University Hospital in Krakow, 359 due to chronic otitis media and 97 due to otosclerosis. Facial nerve canal dehiscence was found during surgery in 26 patients (6% of operated patients) more frequently in men. RESULTS: In most cases (54%) dehiscence was observed in patients with chronic medial otitis with cholesteatoma (27% with choleseatoma and 27% with cholestatoma and granulation), and equally (23% each) dehiscences were observed in patients with granulation and even in patients with otosclerosis and chronic simple otitis media. In over than half (54%) of patients with dehiscence inflammatory lesions involved all the middle ear spaces, in 15% the tympanic cavity, attic and antrum while in 4% of patients inflammatory lesions were limited to the tympanic cavity, attic or antrum. CONCLUSIONS: The facial canal nerve dehiscence is observed in majority in patients with cholestatoma and granulation, especially in tympanic region. In some cases its coexistence with circuit canals dehiscence.

Prevalence of Spontaneous Asymptomatic Facial Nerve Canal Meningoceles: A Retrospective Review.

BACKGROUND AND PURPOSE: The prevalence of patent facial nerve canals and meningoceles along the facial nerve course is unknown. This study aimed to assess the frequency of such findings in asymptomatic patients. MATERIALS AND METHODS: A retrospective review was completed of patients with high-resolution MR imaging of the temporal bone whose clinical presentations were unrelated to facial nerve pathology. Facial nerve canals were assessed for the presence of fluid along each segment and meningoceles within either the labyrinthine segment (fluid-filled distention, ≥1.0-mm diameter) or geniculate ganglion fossa (fluid-filled distention, ≥2.0-mm diameter). If a meningocele was noted, images were assessed for signs of CSF leak. RESULTS: Of 204 patients, 36 (17.6%) had fluid in the labyrinthine segment of the facial nerve canal and 40 (19.6%) had fluid in the geniculate ganglion fossa. Five (2.5%) had meningoceles of the geniculate ganglion fossa; no meningoceles of the labyrinthine segment of the canal were observed. No significant difference was observed in the ages of patients with fluid in the labyrinthine segment of the canal or geniculate ganglion compared with those without fluid (P = .177 and P = .896, respectively). Of the patients with a meningocele, one had a partially empty sella and none had imaging evidence of CSF leak or intracranial hypotension. CONCLUSIONS: Fluid within the labyrinthine and geniculate segments of the facial nerve canal is relatively common. Geniculate ganglion meningoceles are also observed, though less frequently. Such findings should be considered of little clinical importance without radiologic evidence of CSF otorrhea, meningitis, or facial nerve palsy.

Retractorless Surgery for Giant Vestibular Schwannomas via the Retrosigmoid Approach.

BACKGROUND: A fixed retractor is routinely used during surgery for vestibular schwannoma to maintain the surgical corridor; however, brain injuries can be caused by use of retractors. The aim of this study was to present strategies for retractorless surgery for giant vestibular schwannomas and compare retractorless surgery with traditional retractor-assisted surgery to illustrate feasibility and potentially superiority of retractorless surgery. METHODS: Clinical data of 61 patients with giant (≥4 cm diameter) vestibular schwannomas undergoing craniotomy were retrospectively analyzed. Patients were divided into 2 cohorts: 1) 35 patients with traditional retractor surgery performed between June 2016 and July 2017; 2) 26 patients with retractorless surgery performed between June 2016 and July 2018. Duration of operation, intraoperative blood loss, extent of resection, rate of retention of facial nerve function, postoperative brain injury rate, intracranial infection rate, hospitalization time, and grade of facial nerve function were compared between the 2 groups. RESULTS: The incidence of postoperative brain injury was 3.84% in the retractorless surgery group, which was significantly lower compared with the traditional retractor surgery group (22.86%) (P < 0.05). No significant differences were found regarding the other characteristics compared. CONCLUSIONS: Through appropriate comprehensive measures, retractorless surgery for giant vestibular schwannomas is an achievable goal. This procedure can reduce the incidence of postoperative brain injury with satisfactory tumor resection.

Amphiphysin-IgG autoimmune neuropathy: A recognizable clinicopathologic syndrome.

OBJECTIVE: To define the clinicopathologic features of amphiphysin-immunoglobulin G (IgG)-mediated neuropathy. METHODS: Patients examined at our institution from January 1, 1995, to September 30, 2018, with amphiphysin-IgG by indirect immunofluorescence and Western blot, were reviewed. Their phenotypes were compared to cases of coexisting collapsin response-mediator protein-5 (CRMP5)-IgG or anti-neuronal nuclear antibody type 1 (ANNA1-IgG) and CRMP5-IgG autoimmunity. Improvement in modified Rankin Scale (mRS) (≥1) on follow-up was considered a favorable outcome. Amphiphysin RNA expression was assessed in healthy nerves. RESULTS: Fifty-three amphiphysin-IgG-positive cases were identified. Of 33 (60%) patients with neuropathy, 21 had amphiphysin-IgG alone, and 12 had coexisting autoantibodies (ANNA1-IgG, n = 8; CRMP5-IgG, n = 2; ANNA1-IgG and CRMP5-IgG, n = 2). The neuropathies in isolated amphiphysin-IgG autoimmunity included polyradiculoneuropathy (62%), diffuse sensory neuronopathy (35%), and facial neuropathy with gastroparesis (3%). Among these, pain (80%), breast cancer (63%), and CNS (57%) involvements commonly coexisted, and neuropathy frequently prompted breast cancer diagnosis (76%). Stiff-person spectrum disorder was the most common CNS accompaniment (45%). Nerve biopsies showed axonal loss (n = 6/6), subperineurial edema (n = 4/6), and CD4 predominant inflammation (n = 2/6). Median mRS score at last follow-up was 3.5; 58% of patients were immunotherapy-responsive. Patients with amphiphysin-IgG alone had more favorable immunotherapy response than patients with CRMP5-IgG polyneuropathy (n = 45) (44% vs 16%, p = 0.028, odds ratio 4.2, 95% confidence interval 1.1 to 15.5). Only 1/9 (11%) patients with amphiphysin-IgG with coexisting CRMP5-IgG or ANNA1-IgG had immunotherapy response. RNA amphiphysin expression occurred at low levels in nerve. CONCLUSION: Amphiphysin-IgG autoimmune neuropathy has a recognizable phenotype, is frequently immune responsive, and can prompt early diagnosis of breast cancer.

Management of complications in cochlear implantation.

CONCLUSION: Cochlear implantation (CI) remains a safe procedure with a low complication rate. Nevertheless, advances in surgical techniques and the optimization of treatment modalities would further reduce complications. OBJECTIVE: To assess the complications of CI and describe the management of each complication encountered at our hospital. PATIENTS AND METHODS: This study involved 720 patients that underwent implantation from November 1988 through April 2007. Mean age at implantation was 13.6 years (range 12 months to 83 years). Patients were followed up regularly with a mean follow-up of 42 months (range 4-81 months). RESULTS: No death was attributed to device implantation. Major complications included: device failure in 12 patients, misplaced electrodes in 4, hematoma in 3, flap necrosis in 3, magnet displacement in 2, facial nerve twitching in 2, electrode exposure in 2, external auditory canal keratoma in 1, immediate facial nerve paralysis in 1, and skin flap irritation due to the suture material in 1. The overall major complication rate was 4.2% (30/720), and there were 37 minor complications (5.1%), which were resolved by conservative treatment or minor intervention. Minor complications included temporary vertigo in 17 patients, facial twitching in 11, hematoma in 4, subcutaneous emphysema in 3, and temporary facial nerve paralysis in 2.