Auditory Brain Stem Response Predictors of Hearing Outcomes after Middle Fossa Resection of Vestibular Schwannomas.

Objectives To analyze the relationship between preoperative and intraoperative auditory brain stem response (ABR) characteristics and hearing outcomes in patients with vestibular schwannomas (VS) undergoing hearing preservation (HP) surgery via a middle cranial fossa (MCF) approach. Design Prospective study. Setting Academic tertiary skull base referral center. Methods Pre- and postoperative pure-tone average (PTA) and word recognition score (WRS) were examined. Intraoperative ABR wave III latency, wave V latency, and amplitude were recorded. HP was defined as postoperative WRS ≥50%. Participants Adult patients with VS and WRS ≥50% who underwent MCF tumor resection between November 2017 and September 2019. Main Outcome Measures Postoperative hearing outcomes. Results Sixty patients were included. Mean tumor size was 9.2 mm (range, 3-17). HP rates were 56.7% for the cohort and 69.7% for tumors <10 mm. A complete loss of wave V was associated with an 82.9% increase in postoperative PTA ( p < 0.001) and 97.2% decrease in WRS ( p < 0.001), whereas a diminished wave V was correlated with 62.7% increase in PTA ( p < 0.001) and 55.7% decrease in WRS ( p = 0.006). A diminished or absent wave V, but not increased wave III/V latency or decreased wave V amplitude, was correlated with a decline in postoperative hearing class ( r = 0.735, p < 0.001). Receiver-operating characteristic analysis demonstrated that a stable wave V has the highest accuracy in predicting HP (sensitivity of 82.6%, specificity of 84.8%). Conclusion Of the examined preoperative and intraoperative ABR characteristics, a stable wave V intraoperatively was the strongest predictor of HP after MCF resection of VS. Level of Evidence Level III.

Does Medical School Geography and Ranking Influence Residency Match in Otolaryngology?

OBJECTIVE: To elucidate the associations between geographic locations, rankings, and size/funding of medical schools and residency programs among the current otolaryngology residents. METHODS: This retrospective cross-sectional study queried otolaryngology residency program websites for relevant publicly accessible information. Location was categorized as Midwest, Northeast, South, and West. Ranking was according to Doximity (residency) and US News and World Report (medical school). Medical school and residency programs were labeled large if they had >704 students or >15 residents, respectively. RESULTS: A total of 1413 residents from 98 (89%) otolaryngology residency programs were included. Residents attending their home medical schools (18%) were equally distributed among regions (P = .845). Residents who attended medical schools in the same US regions (54%) were more likely from top-25 (P = .001) or private (P < .001) medical schools. Southern residents were most likely (64%) and Western residents were least likely (39%) from regional medical schools (P < .001), while residents from Midwest and Northeast had similar rates (54%-55%). The percentage of Midwest residents coming from regional medical schools has decreased from the 2013 to 2014 residency cycle (P = .037). Completing undergraduate school, medical school, and residency in the same region (38%) was also highest in the South (45%) and lowest in the West (25%) (P < .001). Residents at top-ranked residency programs were more likely from top-ranked (P < .001), large (P = .025), and private (P = .018) medical schools. CONCLUSION: There exist significant associations between otolaryngology residents' medical school location, ranking, size, and funding source and their residency destination. More than half of the current otolaryngology residents attended medical school in the same geographic region, and about one-fifth have attended medical school and residency at the same institution. Future studies are warranted to evaluate how these results change as the match process evolves in the future. LEVEL OF EVIDENCE: N/A.

Determinants of Survival in Skull Base Chondrosarcoma: A National Cancer Database Study.

OBJECTIVE: Chondrosarcomas of the skull base are rare tumors most commonly treated surgically with or without adjuvant radiation therapy. Using the National Cancer Database (NCDB), we analyzed overall survival (OS), treatment modalities, and prognosticators. METHODS: The NCDB was queried for all cases of histologically confirmed skull base chondrosarcoma treated between 2004 and 2015, excluding patients with more than 1 malignant tumor, on palliative care, receiving unrelated concurrent treatments, or having less than 1 month of follow-up. The χ2 test for categorical variables, Cox proportional hazards models, and Kaplan-Meier log-rank analysis were used to test associations with the use of adjuvant radiation, OS, and survival time. RESULTS: A total of 498 patients with skull base chondrosarcoma were identified in the NCDB. Of them, 224 (45.0%) and 198 (39.8%) were treated with either surgery alone or surgery with adjuvant radiation therapy, respectively. Patients more likely to undergo surgery with adjuvant radiation had higher tumor grade (P = 0.008), later year of diagnosis (P = 0.002), positive surgical margins (P < 0.001), and treatment at an academic institution (P = 0.02). Patient, tumor, and socioeconomic factors associated with worse OS on multivariate analysis included the Charlson/Deyo Comorbidity Score ≥2 (P = 0.017), as well as clear cell (P = 0.02) and dedifferentiated (P = 0.006) histology. Age, tumor grade, tumor size, treatment modality, insurance status, facility type, and urban/rural population did not show a statistically significant impact on OS. CONCLUSION: The mainstay of treatment for skull base chondrosarcoma is surgery, with consideration of adjuvant radiation. This study demonstrated worse overall survival associated with more frail patients and aggressive histology types. It is important to consider these factors when planning the clinical management of these patients.

Determinants of Survival in Skull Base Osteosarcoma: A National Cancer Database Study.

OBJECTIVE: Skull base osteosarcoma is a rare and aggressive tumor that is most commonly treated with primary surgical resection and adjuvant chemoradiation. Using the National Cancer Database, we analyzed demographic and clinical prognosticators for overall survival (OS). METHODS: The National Cancer Database was queried for cases of histologically confirmed skull base osteosarcoma treated between 2004 and 2015, excluding patients receiving palliation or having <1 month of follow-up. A total of 314 patients treated with surgery alone (n = 82), surgery with adjuvant radiotherapy (n = 35), surgery with chemotherapy (n = 114), or trimodality therapy (n = 56) were identified. The χ2 test for categorical variables, Cox proportional hazards models, and Kaplan-Meier log-rank analysis were used to test associations with treatment, OS, and survival time. RESULTS: None of the studied demographic characteristics (age, sex, race, overall health) and socioeconomic factors (income and average regional education) were associated with OS (none P < 0.05). Treatment modalities also did not show a significant association with OS (none P < 0.05). Certain tumor characteristics showed an association with OS, with fibroblastic and Paget histologic subtypes (each P = 0.003), poorly differentiated tumor grade (P = 0.03), and tumor size >5 cm (P = 0.045) associated with poorer OS. CONCLUSIONS: Tumor histologic subtype, advanced tumor grade, and greater tumor size are predictors of worse OS in skull base osteosarcoma. No significant differences in OS were identified based on treatment modality, which warrants further investigation.

Perilymphatic Fistula: A Review of Classification, Etiology, Diagnosis, and Treatment.

A perilymphatic fistula (PLF) is an abnormal communication between the perilymph-filled inner ear and the middle ear cavity, mastoid, or intracranial cavity. A PLF most commonly forms when the integrity of the oval or round window is compromised, and it may be trauma-induced or may occur with no known cause (idiopathic). Controversy regarding the diagnosis of idiopathic PLF has persisted for decades, and the presenting symptoms may be vague. However, potential exists for this condition to be one of the few etiologies of dizziness, tinnitus, and hearing loss that can be treated surgically. The aim of this review is to provide an update on classification, diagnosis, and treatment of PLF. Particular attention will be paid to idiopathic PLF and conditions that may have a similar presentation, with subsequent information on how best to distinguish them. Novel diagnostic criteria for PLF and management strategy for PLF and PLF-like symptoms is presented.

Clinical Characteristics, Complications, and Reasons for Readmission following Lingual Tonsillectomy.

This brief communication regards the indications and complications for and rates of readmission following lingual tonsillectomy. The National Readmissions Database (NRD) 2013-2014 was queried for all cases of lingual tonsillectomy occurring from 2013 to 2014. Among 602 lingual tonsillectomies (mean age 36.5 years, 58.2% male), the common indications for surgery were obstructive sleep apnea (58.7%), lingual tonsil hypertrophy/infection (18.8%), and neoplasia (15.9%). Overall, 49 (8.2%) of the cases were readmitted (95% confidence interval, 4.8%-13.5%), with an average readmission duration of 3.0 days and average readmission cost of $25.4K. The most common diagnoses at readmission were bleeding (1.9% of all lingual tonsillectomy cases); dysphagia (1.7%); fever, nausea, vomiting, or diarrhea (1.6%); acute pain (1.6%); and airway obstruction (1.4%). There were no mortalities during primary or subsequent admissions. Lingual tonsillectomy across age groups is relatively free of adverse events, with overall readmission and complication rates similar to those of palatine tonsillectomy.

Simultaneous cerebrospinal fluid and hematologic metastases in a high-grade ependymoma.

BACKGROUND: Ependymomas are relatively uncommon tumors that constitute about 7% of all primary intracranial neoplasms. Among these, high-grade ependymomas are locally aggressive and recur most commonly at the primary site following resection. Ependymomas are also known to be the one glial neoplasm that tends to frequently metastasize inside and outside the central nervous system (CNS) that complicates workup and management. Metastasis due to surgical manipulation is common and neurosurgeons should be well-versed in the most effective methods to remove these tumors in order to avoid such metastases. CASE DESCRIPTION: Here, we report a case of a 28-year-old female who initially presented with a parenchymal World Health Organization (WHO) grade III anaplastic ependymoma of the occipital lobe without metastasis. After multiple resections, the patient showed no evidence of disease recurrence for 2 years. During follow-up, new metastasis to the frontal lobe as well as to the lung were discovered 2 years after the initial surgery, without recurrence at the tumor's primary site. CONCLUSIONS: While uncommon, this case demonstrates the possibility for ependymomas to metastasize via cerebrospinal fluid to other locations within the CNS and hematologically to extraneural locations without recurring locally.

Intraosseous mucoepidermoid carcinoma: Outcome review.

OBJECTIVE: Identify the effect of patient characteristics, disease traits, and treatment modality on patient outcomes in the rare disease process of intraosseous mucoepidermoid carcinoma. STUDY DESIGN: Retrospective review of institutional case records and literature. METHODS: This study includes one case report, a literature review of the MEDLINE database from 1950 through June 2017 using keywords "intraosseous" and "mucoepidermoid," and a query of the University of California, Los Angeles, Department of Pathology database for all documented cases of intraosseous mucoepidermoid carcinoma of the head and neck. RESULTS: Indicators of poorer prognosis were male gender (P = 0.0071) and higher histological grade (P = 0.0095). Lesion site, size, association with odontogenic cyst, and treatment type did not have a statistically significant correlation with patient outcomes. There also was no statistically significant correlation observed between treatment modality and recurrent or progressive disease when stratified by histological grade of the cancer. CONCLUSION: This study identified male gender and high histological tumor grade as poor prognostic indicators; however, it did not reveal a statistically significant relationship between treatment modality and patient outcomes. Data regarding patient outcomes following treatment was limited due to loss to follow-up, suggesting that further investigation is required. Based on this review, decisions regarding treatment should be clinically guided and individually tailored to the patient's baseline health, disease severity, and the patient's treatment goals. A multi-disciplinary conference, as was utilized in the presented case report, may be the best approach to treatment planning for these patients at this time. LEVEL OF EVIDENCE: 4. Laryngoscope, 128:1083-1092, 2018.

Surgical nuances of partial sacrectomy for chordoma.

BACKGROUND: Sacral chordomas are rare, slow growing, locally aggressive tumors. Unfortunately, aggressive surgical resection is often associated with increased neurological morbidity. METHODS: This technical note focuses on the utilization of partial sacrectomy for the resection of complex spinal chordomas. RESULTS: The case presented documents the potential range of postoperative morbidity seen in patients undergoing partial sacrectomy for chordomas. Despite iatrogenic morbidity and tumor recurrence, with the cooperation of medical and surgical spine specialists, majority of patients can achieve good long-term outcomes. CONCLUSIONS: Sacral chordomas are rare lesions and pose a therapeutic challenge for spinal surgeons and oncologists. En-bloc surgical resection (e.g., partial sacrectomy) is the treatment of choice for these lesions, and the cooperation between subspecialists can lead to good neurologic outcomes, particularly if gross total resection is achieved.

Surgical nuances of circumferential lumbar spondylectomy: A case report and short literature review.

BACKGROUND: Chordomas are uncommon malignant bone tumors that are often minimally symptomatic for several years. By the time they are diagnosed, these lesions are typically large, involve major neural, bony, and vascular structures, and are no longer readily resectable. This leads to a high recurrence rate. CASE DESCRIPTION: In this case report, we present a 67-year-old male with nonmechanical axial back pain, neurogenic claudication, and a large mass centered at the L3 level on magnetic resonance imaging consistent with a locally invasive chordoma. The patient underwent surgical resection that required a complete lumbar spondylectomy utilizing a three-stage approach, leading to incomplete tumor excision. The patient's residual postoperative symptoms included paresthesias/numbness in the right anterior thigh and a partial (4/5) right-sided foot drop. At the time of discharge, there were plans for future proton beam therapy. CONCLUSIONS: Because of their relative resistance to chemotherapeutic agents, the optimal surgical management of chordomas is gross total en-bloc excision. Unfortunately, this is rarely feasible.

Adult sacrococcygeal teratoma with coccygectomy: A case report with a review of the literature.

BACKGROUND: Saccrococcygeal teratomas (SCT) are derived from embryonic germ cell layers. They frequently present at the base of the coccyx within the pelvis. While these tumors are common in children, they are exceedingly rare in adults. In adults, a majority of these tumors are intrapelvic and associated with a low risk of malignant transformation. Therefore, this contributes to a good prognosis following resection of mostly benign lesions. CASE DESCRIPTION: An adult female with chronic pelvic pain presented with a sacral teratoma. She failed conservative treatment and underwent a coccygectomy with an en-bloc excision of the tumor. Microscopic histological analysis showed no evidence of immature or malignant elements, confirming the diagnosis of a mature, benign, cystic SCT. CONCLUSIONS: Mature SCTs in adults are rare malignant lesions. In this case, the patient was cured following primary surgical excision requiring en-bloc coccygectomy.

Facial nerve stimulation following cochlear implantation for X-linked stapes gusher syndrome leading to identification of a novel POU3F4 mutation.

We report a case of a nine-year-old male who presented with facial nerve stimulation four years after cochlear implantation. Computed tomography was performed revealing a dilated internal auditory meatus and the cochlear implant electrode was found to be protruding into the fallopian canal at the level of the geniculate ganglion. Subsequent genetic analysis demonstrated X-linked deafness type 2 (DFNX2) caused by a novel c.769C > T nucleotide change in the POU domain, class 3, transcription factor 4 gene (POU3F4). Inactivation of electrodes 1 and 19-21 successfully abated facial nerve stimulation.

A treatment planning comparison between modulated tri-cobalt-60 teletherapy and linear accelerator-based stereotactic body radiotherapy for central early-stage non-small cell lung cancer.

We evaluated the feasibility of planning stereotactic body radiotherapy (SBRT) for large central early-stage non-small cell lung cancer with a tri-cobalt-60 (tri-(60)Co) system equipped with real-time magnetic resonance imaging (MRI) guidance, as compared to linear accelerator (LINAC)-based SBRT. In all, 20 patients with large central early-stage non-small cell lung cancer who were treated between 2010 and 2015 with LINAC-based SBRT were replanned using a tri-(60)Co system for a prescription dose of 50Gy in 4 fractions. Doses to organs at risk were evaluated based on established MD Anderson constraints for central lung SBRT. R100 values were calculated as the total tissue volume receiving 100% of the dose (V100) divided by the planning target volume and compared to assess dose conformity. Dosimetric comparisons between LINAC-based and tri-(60)Co SBRT plans were performed using Student׳s t-test and Wilcoxon Ranks test. Blinded reviews by radiation oncologists were performed to assess the suitability of both plans for clinical delivery. The mean planning target volume was 48.3cc (range: 12.1 to 139.4cc). Of the tri-(60)Co SBRT plans, a mean 97.4% of dosimetric parameters per patient met MD Anderson dose constraints, whereas a mean 98.8% of dosimetric parameters per patient were met with LINAC-based SBRT planning (p = 0.056). R100 values were similar between both plans (1.20 vs 1.21, p = 0.79). Upon blinded review by 4 radiation oncologists, an average of 90% of the tri-(60)Co SBRT plans were considered acceptable for clinical delivery compared with 100% of the corresponding LINAC-based SBRT plans (p = 0.17). SBRT planning using the tri-(60)Co system with built-in MRI is feasible and achieves clinically acceptable plans for most central lung patients, with similar target dose conformity and organ at risk dosimetry. The added benefit of real-time MRI-guided therapy may further optimize tumor targeting while improving normal tissue sparing, which warrants further investigation in a prospective feasibility clinical trial.