Citations

ObjectivesTo analyze factors that influence hearing preservation over time in cochlear implant recipients with conventional‐length electrode arrays located entirely within the scala tympani.Study DesignCase series with planned chart review.SettingSingle tertiary academic referral center.Subjects and MethodsA retrospective review was performed to analyze a subgroup of cochlear implant recipients with residual acoustic hearing. Patients were included in the study only if their electrode arrays remained fully in the scala tympani after insertion and serviceable acoustic hearing (≤80 dB at 250 Hz) was preserved. Electrode array location was verified through a validated radiographic assessment tool. Patients with <6 months of audiologic follow‐up were excluded. The main outcome measure was change in acoustic hearing thresholds from implant activation to the last available follow‐up.ResultsA total of 16 cases met inclusion criteria (median age, 70.6 years; range, 29.4‐82.2; 50% female). The average follow‐up was 18.0 months (median, 16.1; range, 6.2‐36.4). Patients with a lateral wall electrode array were more likely to have stable acoustic thresholds over time (P <. 05). Positive correlations were seen between continued hearing loss following activation and larger initial postoperative acoustic threshold shifts, though statistical significance was not achieved. Age, sex, and noise exposure had no significant influence on continued hearing preservation over time.ConclusionsTo control for hearing loss associated with interscalar excursion during cochlear implantation, the present study evaluated patients only with conventional electrode arrays located entirely within the scala tympani. In this group, the style of electrode array may influence residual hearing preservation over time.

OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) of the eighth cranial nerve (CN) are exceedingly rare. To date the literature has focused on MPNSTs occurring after radiation therapy for presumed benign vestibular schwannomas (VSs), while MPNSTs arising without prior irradiation have received little attention. The objectives of the current study are to characterize the epidemiology, clinical presentation, disease course, and outcome using a large national cancer registry database and a systematic review of the English literature. Additionally, a previously unreported case is presented. METHODS The authors conducted an analysis of the Surveillance, Epidemiology, and End Results (SEER) database, a systematic review of the literature, and present a case report. Data from all patients identified in the SEER database with a diagnosis of MPNST involving the eighth CN, without a history of prior radiation, were analyzed. Additionally, all cases reported in the English literature between January 1980 and March 2015 were reviewed. Finally, 1 previously unreported case is presented. RESULTS The SEER registries identified 30 cases between 1992 and 2012. The average incidence was 0.017 per 1 million persons per year (range 0.000–0.0687 per year). The median age at diagnosis was 55 years, and 16 (53%) were women. Thirteen cases were diagnosed upon autopsy. Of the 17 cases diagnosed while alive, the median follow-up was 118 days, with 3 deaths (18%) observed. When compared with the incidence of benign VS, 1041 VSs present for every 1 MPNST arising from the eighth CN. Including a previously unreported case from the authors' center, a systematic review of the English literature yielded 24 reports. The median age at diagnosis was 44 years, 50% were women, and the median tumor size at diagnosis was 3 cm. Eleven patients (46%) reported isolated audiovestibular complaints typical for VS while 13 (54%) exhibited facial paresis or other signs of a more aggressive process. Treatment included microsurgery alone, microsurgery with adjuvant radiation, or microsurgery with chemoradiation. Sixty-one percent of patients receiving treatment experienced recurrence, 22% of which were diagnosed with drop metastases to the spine. Ultimately, 13 patients (54%) died of progressive disease at a median of 3 months following diagnosis. The ability to achieve gross-total resection was the only feature that was associated with improved disease-specific survival. CONCLUSIONS MPNSTs of the eighth CN are extremely rare and portend a poor prognosis. Nearly half of patients initially present with findings consistent with a benign VS, often making an early diagnosis challenging. In light of these data, early radiological and clinical follow-up should be considered in those who elect nonoperative treatment, particularly in patients with a short duration of symptoms or atypical presentation. These data also provide a baseline rate of malignancy that should be considered when estimating the risk of malignant transformation following stereotactic radiosurgery for VS.

ObjectiveTo describe surgical outcomes and perioperative complications in patients taking antiplatelet and anticoagulation medications while undergoing cochlear implantation.Study DesignCase series with chart review.SettingTwo tertiary otologic referral center.Subjects and MethodsForty‐six adult patients (2005‐2014) who underwent cochlear implantation while on perioperative antiplatelet and/or anticoagulation therapy without interruption. Outcomes included estimated intraoperative blood loss, perception of increased difficulty secondary to bleeding, and postoperative complications attributable to continuing anticoagulation or antiplatelet therapy.ResultsThe cases of 46 patients (mean age, 69.5 years; 30.2% female) were analyzed. Of these, 39 patients were taking aspirin, 10 warfarin, and 7 clopidogrel. Aside from 3 (6.5%) patients with postoperative bruising without hematoma and 1 patient (2.2%) who reported self‐resolving oral cavity blood, no intraoperative or postoperative complications occurred in any patient that could be ascribed to antiplatelet or anticoagulant use.ConclusionThese data demonstrate that the risk of perioperative complications from continued antiplatelet or anticoagulation therapy is low. For subjects who are at risk of major complications (eg, deep venous thrombosis, pulmonary embolism, myocardial infarction, cerebrovascular accident) from temporary medication cessation, continuing antiplatelet or anticoagulation therapy through the perioperative period is an appropriate strategy.

Objectives/HypothesisThe primary goals of microsurgery for vestibular schwannoma (VS) include preservation of neural function and complete tumor removal. In a subset of patients, adherent tumor remnant may be intentionally left behind in order to minimize risk of new neurologic deficits. It is not well established if residual tumor volume predicts likelihood of tumor remnant growth.MethodsPatients with sporadic VS who underwent near‐total (NTR) or subtotal (STR) VS resection between 2000 and 2014 were reviewed. Postoperative tumor remnants were volumetrically contoured using T1‐weighted gadolinium‐enhanced magnetic resonance imaging obtained within 3 months of surgery.ResultsA total of 103 patients met study criteria, and the median duration of radiographic follow‐up was 41 months (mean 56.1 months, range 12–150 months). Fifty patients underwent NTR and 53 received STR. Overall 14 (13.6%) tumors recurred at a median of 41.0 months. Patients who underwent STR were over 13 times more likely to recur compared with those treated with NTR (hazard ratio 13.31; 95% confidence interval 1.71–103.91; P = 0.014). The median time to recurrence following NTR was 124 months compared to 32 months after STR (P < 0.001).ConclusionsLong‐term follow‐up in patients undergoing incomplete resection is essential. Near‐total resection has a significantly lower rate of recurrence compared to STR. Maximal surgical resection should be the goal in VS microsurgery. The decision to pursue less than complete resection should be based on intraoperative impression, when it is felt that continued dissection of adherent disease would compromise neurologic outcome.Level of Evidence4. Laryngoscope, 126:1877–1882, 2016

Objectives/HypothesisEvaluate presentation, management, and clinical outcomes of patients with temporal bone Langerhans cell histiocytosis (LCH).Study DesignRetrospective chart review.MethodsReviewed all patients with temporal bone LCH at a tertiary academic referral center between 1978 and 2014. Presentation, disease course, intervention, and clinical outcomes were analyzed.ResultsBetween 1978 and 2014, 29 temporal bones in 20 patients (12 males; median age 32 years, range 1.3–88 years) were diagnosed with temporal bone LCH, representing 4% of all patients diagnosed with LCH at our institution during that time. Twelve (60%) patients presented purely with head and neck disease, nine (45%) with bilateral temporal bone involvement, seven (35%) with intracranial spread, and eight (40%) with multisystem disease. The most common presenting symptoms were otorrhea (n = 11; 55%) and subjective hearing loss (n = 10; 50%). Treatment included primary chemotherapy or radiation alone(n = 7; 35%), local resection alone (n = 2; 10%), and chemotherapy or radiation with surgery(n = 11; 55%). Ten patients suffered local or systemic relapse (50%; median 12 months). Median follow‐up for patients was 31 months. One patient died secondary to pulmonary complications of multisystem LCH.ConclusionsLCH is an uncommon histiocytic disorder with a range of clinical manifestations and disease severity. Otologic involvement is rare, and frequently manifests with symptoms similar to otomastoiditis. Evaluation and close follow‐up of both ears is important. Bilateral simultaneous or sequential LCH can be seen in up to 45% of cases. Patients with temporal bone LCH should be managed by a multidisciplinary team with treatment tailored to the patient. Relapse is more common in patients with multisystem involvement. Even in subjects with isolated otologic involvement, long‐term follow‐up is critical, with relapses occurring years beyond treatment.Level of Evidence4. Laryngoscope, 126:1899–1904, 2016

Objectives/HypothesisWe sought to analyze key factors that influence the management of lateral skull base defects and implement an algorithm to improve outcomes of reconstruction.Study DesignRetrospective chart review.SettingSingle tertiary academic referral center.MethodsAdults who underwent temporal bone resection were included. Variables included tumor characteristics, ablative procedure, reconstructive method, and wound complications.ResultsSeventy‐one patients were studied. Lateral temporal bone resection was performed in 55 patients (77%), subtotal temporal bone resection in 14 (20%), and total petrosectomy in two (3%). Primary closure was achieved in 46 patients (35%), with rotational muscle flaps in 21 (46%). The pectoralis major myocutaneous flap (PMMF) was used in nine patients (13%) without major complications. Single‐stage free tissue transfer was undertaken in 10 patients (14%) with no flap failures. Primary closure was achieved in 16 of 32 of previously untreated cases (50%) compared to nine of 39 cases of previously treated disease (23%) (P = 0.03). Pedicled or free flaps were used in 18 of 39 cases of previously treated disease (46%) compared to three of 32 previously untreated cases (9%) (P < 0.001). Pedicled or free flaps were used in 13 of 22 cases (59%) in which partial or total auriculectomy was performed, whereas primary closure was achieved in 39 of 49 cases (80%) in which the pinna was preserved (P < 0.001).ConclusionAdvanced tumors that require adjuvant therapy, revision cases for recurrent disease, and cases involving auriculectomy warrant free flap or PMMF reconstruction, with the latter preferred in poor free flap candidates.Level of Evidence4. Laryngoscope, 126:1911–1917, 2016

ObjectiveTo characterize disease presentation and outcomes following surgical treatment of parotid malignancies with occult temporal bone facial nerve (FN) involvement.Study DesignCase series with chart review.SettingTertiary academic referral center.Subjects and MethodsThirty consecutive patients (mean age 58 years; 77% men) who underwent surgery for parotid malignancies with occult perineural involvement of the intratemporal FN were included. Primary outcome measures included margin status and recurrence.ResultsThe mean duration of clinical follow‐up was 49 months, and the most common presenting symptom was FN paresis (n = 23; 77%) followed by pain (n = 15; 50%). To obtain a proximal FN margin, 27 patients (90%) underwent mastoidectomy, and 3 patients (10%) had lateral temporal bone resection. The intratemporal FN margin was cleared in 26 patients (87%), most commonly in the mastoid segment (60%). Adjuvant therapy was given in 25 patients (83%). Ten patients (33%) experienced locoregional (4; 13%) and/or distant (8; 27%) recurrence at a median of 19 months (mean 26, 2–54 months) following surgery. Locoregional failure was significantly more common in cases with a positive intratemporal FN margin (66% vs 8%; P =. 045). Overall 1‐, 3‐, and 5‐year disease‐specific survival rates were 83%, 79%, and 72%, respectively.ConclusionsPerineural invasion of the intratemporal FN by parotid malignancy is uncommon. Normal preoperative FN function does not preclude histopathologic involvement. Temporal bone FN exploration should be considered when a positive margin is encountered at the stylomastoid foramen, as failure to do so is associated with an increased rate of locoregional recurrence.

ObjectiveTo characterize the evolving management of vestibular schwannoma (VS) in the United States.Study DesignRetrospective analysis of the Surveillance, Epidemiology, and End Results (SEER) database.SettingSEER database.Subjects and MethodsAll patients with a diagnosis of VS were analyzed. Data were described and compared using trend analyses and univariate and multivariable logistic regression.ResultsA total of 8330 patients (average age 54.7 years, 51.9% female) were analyzed. The mean incidence was approximately 1.1 per 100,000 per year and did not vary significantly across time; however, from 2004 to 2011, there was a statistically significant decrease in tumor size category at time of diagnosis (P <. 01). Overall, 3982 patients (48%) received primary microsurgery, 1978 (24%) radiation therapy alone, and 2370 (29%) observation. Within the microsurgical cohort, 732 (18%) underwent subtotal resection, and of those, 98 (13.4%) received postoperative radiation therapy. Multivariable regression revealed that surgical treatment was more common in younger patients and larger tumor size categories (P <. 05). Management trend analysis revealed that microsurgery was used less frequently over time (P <. 0001), observation was used more frequently (P <. 0001), and the pattern of radiation therapy remained unchanged. Linear regression was used to create an equation that was applied to predict future management practices. These data predict that by 2026, half of all cases of VS will be managed initially with observation.ConclusionWhile the incidence of VS has remained steady, tumor size at time of diagnosis has decreased over time. Within the United States there has been a clear, recent evolution in management toward observation.

OBJECT The primary goals of this study were: 1) to examine the influence of disease and treatment on headache in patients with sporadic vestibular schwannoma (VS); and 2) to identify clinical predictors of long-term headache disability. METHODS This was a cross-sectional observational study with international multicenter enrollment. Patients included those with primary sporadic < 3-cm VS and a separate group of general population control subjects without tumors. Interventions included a postal survey incorporating the Headache Disability Inventory (HDI), the Hospital Anxiety and Depression Scale, and a VS symptom questionnaire. The main outcome measures were univariate and multivariable associations with HDI total score. RESULTS The overall survey response rate was 79%. Data from 538 patients with VS were analyzed. The mean age at time of survey was 64 years, 56% of patients were female, and the average duration between treatment and survey was 7.7 years. Twenty-seven percent of patients received microsurgery, 46% stereotactic radiosurgery, and 28% observation. Patients with VS who were managed with observation were more than twice as likely to have severe headache disability compared with 103 control subjects without VS. When accounting for baseline differences, there was no statistically significant difference in HDI outcome between treatment modalities at time of survey. Similarly, among the microsurgery cohort, the long-term risk of severe headache disability was not different between surgical approaches. Multivariable regression demonstrated that younger age, greater anxiety and depression, and a preexisting diagnosis of headache were the primary predictors of severe long-term headache disability, while tumor size and treatment modality had little influence. CONCLUSIONS At a mean of almost 8 years following treatment, approximately half of patients with VS experience headaches of varying frequency and severity. Patient-driven factors including age, sex, mental health, and preexisting headache syndrome are the strongest predictors of long-term severe headache disability. Tumor size and treatment modality have less impact. These data may assist with patient counseling regarding long-term expectations following diagnosis and treatment.