Citations

OBJECTIVEThe aim of this study was to evaluate the incidence, presentation, and treatment outcomes of trigeminal nerve–mediated symptoms secondary to large vestibular schwannomas (VSs) with trigeminal nerve contact. Specifically, the symptomatic results of pain, paresthesias, and numbness after microsurgical resection or stereotactic radiosurgery (SRS) were examined.METHODSThe authors conducted a retrospective review of a database for concomitant diagnosis of trigeminal neuralgia (TN) or trigeminal neuropathy and VS between 1994 and 2014 at a tertiary academic center. All patients with VS with TN or neuropathy were included, with the exception of those patients with neurofibromatosis Type 2 and patients who elected observation. Patient demographic data, symptom evolution, and treatment outcomes were collected. Population data were summarized, and outcome comparisons between microsurgery and SRS were analyzed at last follow-up.RESULTSSixty (2.2%) of 2771 total patients who had large VSs and either TN or neuropathy symptoms met inclusion criteria. The average age of trigeminal symptom onset was 53.6 years (range 24–79 years), the average age at VS diagnosis was 54.4 years (range 25–79 years), and the average follow-up for the microsurgery and SRS groups was 30 and 59 months, respectively (range 3–132 months). Of these patients, 50 (83%) had facial numbness, 16 (27%) had TN pain, and 13 (22%) had paresthesias (i.e., burning or tingling). Subsequently, 50 (83%) patients underwent resection and 10 (17%) patients received SRS.Treatment of VS with SRS did not improve trigeminal symptoms in any patient. This included 2 subjects with unimproved facial numbness and 4 patients with worsened numbness. Similarly, SRS worsened TN pain and paresthesias in 5 patients and failed to improve pain in 2 additional patients. The Barrow Neurological Institute neuralgia and hypesthesia scale scores were significantly worse for patients undergoing SRS compared with microsurgery.Resection alleviated facial numbness in 22 (50%) patients, paresthesias in 5 (42%) patients, and TN in 7 (70%) patients. In several patients, surgery was not successful in relieving facial numbness, which failed to improve in 17 (39%) cases and became worse in 5 (11%) cases. Also, surgery did not change the intensity of facial paresthesias or neuralgia in 6 (50%) and 3 (25%) patients, respectively. Microsurgery exacerbated facial paresthesias in 1 (8%) patient but, notably, did not aggravate TN in any patient.CONCLUSIONSOverall, resection of large VSs provided improved outcomes for patients with concomitant TN, facial paresthesia, and numbness compared with SRS. However, caution should be used when counseling surgical candidates because a number of patients did not experience improvement. This was especially true in patients with preoperative facial numbness and paresthesias, who frequently reported that these symptoms were unchanged following surgery.

Objectives: To describe the clinical presentation, radiological features, and outcome of patients with plasmacytoma of the temporal bone. Methods: Multicenter retrospective case series of all patients diagnosed with plasmacytoma of the temporal bone between 1990 and 2015. Comprehensive literature review of all previously published cases. Results: A total of seven patients (average age, 57.3 yr; 57% women) met inclusion criteria. Three (43%) had a known history of multiple myeloma (MM). The most common symptoms at presentation included otalgia, headache, and dizziness (43%, each). Five (71%) presented with a vascular appearing middle ear mass visible on otoscopy. Four of these patients did not have an established history of MM and were referred with presumptive diagnoses of paraganglioma or endolymphatic sac tumor (ELST). The average maximum tumor diameter was 3.7 cm (median, 3.5 cm; range, 0.9–6.6 cm) and the most commonly involved temporal bone subsites were the middle ear and mastoid (71% each). MM was ultimately diagnosed in six cases (86%). In addition to the new cases presented herein, 18 previously published reports were analyzed. In the 25 aggregate cases, 44% of cases were associated with MM, and the mastoid (72%) and middle ear (53%) were the most commonly involved temporal bone subsites. Conclusion: Plasmacytoma of the temporal bone is rare, and the clinical presentation can closely mimic that of other primary temporal bone tumors. In patients with an established history of MM, it is imperative to have a high index of suspicion for temporal bone plasmacytoma, even when radiological features suggest an alternate diagnosis.

Objectives: This study characterizes primary petroclival meningioma growth rates, before intervention, using volumetric analysis. In addition, predictors of growth are analyzed. Methods: Patients with previously untreated petroclival meningiomas were retrospectively reviewed (1999–2015). Image analysis software was used to perform volumetric analyses of tumor size and growth. Three-dimensional segmentation volumetric analyses were compared with volumes estimated utilizing three orthogonal dimensions. Tumor growth was defined as a 15% increase in volume. Results: Thirty-four patients who underwent at least two magnetic resonance imaging (MRI) studies before intervention were included. The mean age was 55.2 years, and 65.7% were women. The mean tumor volume at presentation was 5.6 cm 3 (range, 0.1–25.8 cm 3 ) as determined from segmentation volumetric analysis. At a mean follow-up of 44.5 months (range, 3.7–125.1 mo), 88.2% of tumors grew. The mean annual volumetric growth rate was 2.38 cm 3 /yr (−0.63 to 25.9 cm 3 /yr). Tumor volume, T2 hyperintensity within the tumor, peritumoral edema, and ataxia and/or cerebellar symptoms at presentation were all significantly associated with greater rates of tumor growth. Ultimately, 10 (29.4%) patients underwent treatment during the follow-up period. Conclusion: Our experience demonstrates that the vast majority (88%) of untreated petroclival meningiomas grow; the mean volumetric growth rate was noted to be 2.38 cm 3 /yr. We found a significant association between increased growth rate and larger tumor size at diagnosis, T2 hyperintensity within the tumor, peritumoral edema, and the presence of ataxia and/or cerebellar symptoms.

OBJECTIVEPatient-reported outcomes are increasingly used in studies of vestibular schwannoma (VS); however, few studies have examined self-evaluated facial nerve function and its relation to physician-reported outcomes. The primary objective of this study was to compare patient self-evaluations of facial disability with physician-evaluated facial nerve status and with self-evaluations of a healthy control group. The second objective was to provide insight into the controversial subject of the optimal initial management of small- and medium-sized VSs; consequently, the authors compared patient-reported facial nerve disability following treatment via observation (OBS), Gamma Knife surgery (GKS), or microsurgery (MS). Lastly, the authors sought to identify risk factors for facial nerve dysfunction following treatment for small- and medium-sized VSs.METHODSAll patients with a VS 3 cm or smaller that was singly treated with OBS, GKS, or MS at either of 2 independent treatment centers between 1998 and 2008 were retrospectively identified. Longitudinal facial nerve measures and clinical data, including facial nerve evaluation according to the House-Brackmann (HB) grading system, were extracted from existing VS databases. Supplementing the objective data were Facial Disability Index (FDI) scores, which were obtained via survey of patients a mean of 7.7 years after initial treatment.RESULTSThe response rate among the 682 eligible patients was 79%; thus, data from a total of 539 patients were analyzed. One hundred forty-eight patients had been managed by OBS, 247 with GKS, and 144 with MS. Patients who underwent microsurgery had larger tumors and were younger than those who underwent OBS or GKS. Overall, facial nerve outcomes were satisfactory following treatment, with more than 90% of patients having HB Grade I function at the last clinical follow-up. Treatment was the major risk factor for facial nerve dysfunction. Almost one-fifth of the patients treated with MS had an objective decline in facial nerve function, whereas only 2% in the GKS group and 0% in the OBS cohort had a decline. The physical subscale of the FDI in the VS patients was highly associated with HB grade; however, the social/well-being subscale of the FDI was not. Thus, any social disability caused by facial palsy was not detectable by use of this questionnaire.CONCLUSIONSThe majority of patients with small- and medium-sized VSs attain excellent long-term facial nerve function and low facial nerve disability regardless of treatment modality. Tumor size and microsurgical treatment are risk factors for facial nerve dysfunction and self-reported disability. The FDI questionnaire is sensitive to the physical but not the social impairment associated with facial dysfunction.

ObjectivesTo elucidate the clinical behavior, treatment, and outcomes of tenosynovial giant cell tumors (TGCT) involving the temporomandibular joint (TMJ) and adjacent temporal bone.Study DesignRetrospective case series with histopathologic review.MethodsA retrospective chart review was performed identifying and collecting data from all cases of TGCT involving the TMJ and adjacent temporal bone that were treated at the authors' center between January 1960 and December 2015.ResultsEleven histopathologically confirmed cases met inclusion criteria. The median age at diagnosis was 49 years, eight patients were men, and the median follow‐up was 116 months. Computed tomographic (CT) imaging revealed a lytic expansile mass centered on the TMJ. Magnetic resonance imaging (MRI) most commonly exhibited hypointense signal on precontrast T1‐ and T2‐weighted sequences and variable postcontrast enhancement. The median delay in diagnosis was 24 months, and the most common presenting symptoms were hearing loss and pain. All patients underwent surgical resection, eight receiving gross total removal, one receiving near total removal, and two patients from early in the series receiving subtotal resection with neoadjuvant or adjuvant radiation. Histopathological review of surgical specimens revealed chondroid metaplasia in seven tumors. Eight of nine cases receiving gross total or near total resection have no evidence of recurrence to date.ConclusionsTGCT of the TMJ and temporal bone are rare and locally aggressive tumors that commonly present with nonspecific symptoms. A careful review of CT and MRI followed by early biopsy is critical in establishing an accurate diagnosis and facilitating appropriate treatment. TGCT of the TMJ more commonly contain chondroid metaplasia when compared to TGCT at other anatomic locations. Gross total resection is achievable in most cases and offers long‐term cure. Radiation may be considered for recurrent disease or adjuvant therapy following subtotal resection.Level of Evidence4. Laryngoscope, 127:2340–2346, 2017

OBJECTIVEOver the last 30 years, stereotactic radiosurgery (SRS) has become an established noninvasive treatment alternative for small- to medium-sized vestibular schwannoma (VS). This study aims to further define long-term SRS tumor control in patients with documented pretreatment tumor growth for whom conservative observation failed.METHODSA prospective clinical database was queried, and patients with sporadic VS who elected initial observation and subsequently underwent SRS after documented tumor growth between 2004 and 2014 were identified. Posttreatment tumor growth or shrinkage was determined by a ≥ 2-mm increase or decrease in maximum linear dimension, respectively.RESULTSSixty-eight patients met study inclusion criteria. The median pre- and posttreatment observation periods were 16 and 43.5 months, respectively. The median dose to the tumor margin was 13 Gy (range 12–14 Gy), and the median maximum dose was 26 Gy (range 24–28 Gy). At the time of treatment, 59 tumors exhibited extracanalicular (EC) extension, and 9 were intracanalicular (IC). Of the 59 EC VSs, 50 (85%) remained stable or decreased in size following treatment, and 9 (15%) enlarged by > 2 mm. Among EC tumors, the median pretreatment tumor growth rate was 2.08 mm/year for tumors that decreased or were stable, compared with 3.26 mm/year for tumors that grew following SRS (p = 0.009). Patients who demonstrated a pretreatment growth rate of < 2.5 mm/year exhibited a 97% tumor control rate, compared with 69% for those demonstrating ≥ 2.5 mm/year of growth prior to SRS (p = 0.007). No other analyzed variables were found to predict tumor growth following SRS.CONCLUSIONSOverall, SRS administered using a marginal dose between 12–14 Gy is highly effective in treating VSs in which initial observation fails. Tumor control is achieved in 97% of VSs that exhibit slow (< 2.5 mm/year) pretreatment growth; however, SRS is less successful in treating tumors exhibiting rapid growth (≥ 2.5 mm/year).

Background Historically, lumbar drains (LD) have played a prominent role in endonasal skull base surgery. Over the past few decades, advancements in techniques have augmented our ability to successfully reconstruct complex skull base defects and often obviates the need for cerebrospinal fluid (CSF) diversion. Clarity on the appropriate use of LDs is needed. Objective To examine the literature for the need for LDs in contemporary skull base reconstruction after resection of skull base tumors. Methods A systematic literature review of English language articles by using PubMed and Ovid. Search terms included “lumbar drain,” “CSF leak,” and “endoscopic endonasal reconstruction.” Articles were included when they pertained to adults, used current methods for reconstruction (i.e., multilayered repair or vascularized tissue), and addressed CSF leak rates secondary to endoscopic resection of skull base masses. All the studies discussed CSF leaks that resulted from traumatic-, idiopathic-, or sinus surgery-related iatrogenic causes were excluded. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed. Results A total of five articles met inclusion criteria. These comprised 376 endoscopic skull base tumor resection and reconstruction cases; 5.59% developed postoperative CSF leaks. A meta-analysis was conducted by incorporating three studies that met criteria based on testing for heterogeneity. The average odds ratio for postoperative CSF leak for patients who did not have an LD relative to patients who had an LD was 0.590 (95% confidence interval, 0.214–1.630). Given a p value of 0.30, the results demonstrated a lack of statistically significant improvement between patients who had an LD and patients who did not have an LD. Various details, such as the defect size and type of CSF leak, were not consistently reported among studies. Conclusions Available evidence for the use of LDs in skull base surgery is of poor quality. Analysis of the literature revealed heterogenous and varied reporting in the primary literature. Further studies that include randomized controlled trials are needed.

ObjectivePatients with incidental or minimally symptomatic superior semicircular canal dehiscence (SSCD) are usually observed, without surgical repair. However, it remains unknown whether a labyrinthine fistula of the superior semicircular canal is associated with progressive conductive or sensorineural hearing loss over time.Study DesignRetrospective review at two tertiary care academic referral centers.MethodsAdults analyzed were diagnosed with SSCD by high‐resolution temporal bone computed tomography and vestibular evoked myogenic potential testing and observed with a minimum of two sequential audiograms. Patients with other potential causes of hearing impairment were excluded.ResultsA total of 40 ears in 30 adult patients (median age: 59 years; 63% female) were analyzed. Median audiometric follow‐up was 23 months (range 1–136 months). None experienced a sudden hearing loss over the follow‐up period. In patients with audiometric follow‐up of at least 20 months (median 34 months), the median change in air‐conduction pure tone average and air–bone gap was 0.9 decibels (dB) per year (interquartile range [IQR] 0–2.1) and 0.7 dB per year (IQR 0–2.0), respectively. Speech discrimination scores did not differ when comparing median initial (100%) and median final (98%) scores (P = 0.77). There was no statistically significant change in bone‐conduction thresholds at 0.5, 1, 2, and 4 kHz over the period of observation.ConclusionThe risk of progressive hearing loss with observed SSCD is low during short‐ and intermediate‐term follow‐up. Further studies are necessary to determine whether late hearing loss occurs. Such information may be critical toward patient counseling regarding the need for and timing of surgery.Level of Evidence4. Laryngoscope, 127:1181–1186, 2017

Objectives/HypothesisThis study was designed to examine speech recognition and self‐perceived health‐related quality of life (HRQoL) received from cochlear implantation among a cohort of adults and children with a short duration of unilateral hearing loss greater than 6 months, but less than 2 years.Study DesignSingle‐subject repeated measures prospective study.MethodsThis study assessed changes in speech recognition and self‐perceived quality of life by prospectively analyzing data at the preoperative evaluation and at the 3‐month and 6‐month postactivation intervals. Measurement tools included Medical Outcomes Study Questionnaire Short Form 36, Nijmegen Cochlear Implant Questionnaire, Speech Spatial and Qualities of Hearing–Comparative, and speech recognition measures in quiet and in noise.ResultsResults indicated significant improvement in speech recognition, both in quiet and noise. Quality‐of‐life measures showed a significant increase in self‐perceived benefit with disease‐specific instruments, but remained constant with a generic HRQoL instrument.ConclusionsCochlear implantation was a successful intervention for improved hearing in quiet and noise, and a self‐perceived benefit for this group of adults and children with a short duration of unilateral hearing loss.Level of Evidence4 Laryngoscope, 127:1683–1688, 2017

Objective: To report hemifacial spasm (HFS) as a rare presenting symptom of a benign parotid neoplasm involving the stylomastoid foramen. Methods: Case report with literature review. Results: An 18-year-old woman presented with a 1-year history of a progressively enlarging right infra-auricular mass with new onset ipsilateral severe HFS. Clinical vetting unveiled a palpable 2 cm, firm, minimally mobile mass medial to the angle of the mandible. Imaging revealed a heterogeneous intraparotid mass with tumor extension into the stylomastoid foramen. Diagnostic consideration was given to neurogenic tumors and neoplasms of parotid origin. The patient ultimately underwent superficial parotidectomy and the tumor was found superficial and extrinsic to the proximal facial nerve trunk but extended into the stylomastoid foramen. The tumor was removed after performing mastoidectomy with mastoid segment facial nerve decompression. Frozen section pathology was consistent with pleomorphic adenoma. Postoperatively the patient had normal facial nerve function with immediate and complete resolution of HFS. To date there are only three other published reports of benign parotid tumors presenting with HFS and this is the first illustrating intratemporal tumor extension. Conclusions: HFS is a rare presenting symptom associated with benign parotid neoplasms with scarce documentation in the literature. In this index case, tumor extirpation with mastoid segment facial nerve decompression provided immediate and durable resolution of symptoms.