Citations

Objective: To describe audiometric outcomes following stereotactic radiosurgery (SRS) for jugular paraganglioma (JP). Study Design: Retrospective review. Setting: Tertiary referral center. Patients: Patients with pretreatment serviceable hearing (American Academy of Otolaryngology—Head and Neck Surgery [AAO-HNS] Class A or B) and serial post-SRS audiometric follow-up who underwent Gamma Knife SRS for JP between 1990 and 2017. Intervention(s): Gamma Knife SRS. Main Outcome Measures: Progression to nonserviceable hearing; correlation between baseline hearing and treatment parameters with audiometric outcomes. Results: Of 85 patients with JP who underwent SRS during the study period, 35 (66% female, median age 53) had pretreatment serviceable hearing and serial post-treatment audiometry available for review. Median tumor volume at the time of treatment was 7,080 mm3, median cochlear point dose was 5.8 Gy (interquartile range [IQR] 4.1 to 7.3 Gy), and median marginal and maximum tumor doses were 16 and 32 Gy, respectively. After a median follow-up of 37 months (IQR 16 to 77 mo), the median change in pure-tone average and speech discrimination score in the treated ear was −1.2 dB HL/yr (IQR −4.5 to 0.3) and 0%/yr (IQR 0–3.5%), compared with 0.07 dB HL/yr (IQR −0.03 to 0.12) and 0 %/yr (IQR 0 to 0%) in the contralateral untreated ear. Seven patients developed nonserviceable hearing (AAO-HNS Class C or D) at a median of 13.2 months following SRS (IQR 4.8 to 24 mo). Among those who maintained serviceable hearing, median audiometric follow-up was 42 months (IQR 18 to 77 mo). The Kaplan–Meier estimated rates of serviceable hearing at 1, 3, and 5 years following SRS were 91%, 80%, and 80%, respectively. Sixty percent of patients with pulsatile tinnitus who underwent SRS experienced varying levels of symptomatic improvement following treatment. Conclusion: The short- and intermediate-term risk of progression to nonserviceable hearing following SRS for JP is low. Data regarding the impact of cochlear dose from the vestibular schwannoma literature should not be freely applied to JP, since the impact of SRS parameters on hearing preservation seems to be less significant.

Objective: To examine practice variance of cochlear implant candidacy assessment and off-label indications across centers in the United States. Methods: Cross-sectional survey of the American Neurotology Society (ANS). Results: A total of 81 surveys were returned from ANS members who report regular involvement in cochlear implant care. Overall there was a broad distribution in age and clinical experience, with most respondents reporting ACGME accreditation in neurotology and employment at an academic center. The annual volume of cochlear implant surgeries varied considerably across centers. Seventy-eight percent of respondents performed cochlear implantation for at least one of the following indications within the last 2 years: profound hearing loss in children less than 12 months of age (35, 43%), children with asymmetrical hearing loss where at least one ear was better than performance cutoff for age (25, 31%), adults with asymmetrical hearing where at least one ear was better than the performance cutoff for adult criteria (49, 61%), single-sided deafness (37, 46%), and ipsilateral vestibular schwannoma (28, 35%). Centers with a higher annual implant volume more frequently performed off-label implantation in all queried populations (all, p≤0.001), and performed surgery on infants with congenital deafness at a younger age (p = 0.013), compared with centers with lower surgical volume. When surveyed regarding speech perception testing practices for adult candidacy assessment, 75 (100%) respondents who answered this question reported routine use of AzBio sentences, 42 (56%) CNC word scores, and 26 (35%) HINT testing; only 7 (9%) reported using BKB-SIN testing and 6 (8%) reported using CUNY scores. Fifty-one (68%) reported routine use of speech-in-noise testing to determine adult cochlear implant candidacy, 21 (28%) reported selective use only when patient scores were borderline in quiet, and 3 (4%) reported that their center does not currently use testing in noise for candidacy determination. Nineteen (26%) solely used +10 dB signal-to-noise ratio (SNR), 12 (16%) solely used +5 dB SNR, and 41 (55%) used both +10 and +5 dB SNR. Overall, 19% (N = 14) only perform unilateral implantation in the Medicare population, while 81% (N = 58) consider bilateral implantation. Conclusion: Significant variation in cochlear implant candidacy assessment and off-label implantation exists across centers and providers in the United States resulting in healthcare inequities. The high percentage of surgeons performing implantations for off-label or nontraditional indications reflects the overly restrictive and dated status of current implant guidelines. With greater adoption of more difficult speech perception testing in noise, careful clinical judgment is needed to maintain a favorable risk–benefit balance for prospective implant candidates.

Objective: To examine surgical and device-related cochlear implant practice patterns across centers in the United States. Methods: Cross-sectional survey of the American Neurotology Society (ANS). Results: A total of 81 surveys were returned from ANS members who report regular involvement in cochlear implant care. Overall there was a broad distribution in age and clinical experience, with most respondents reporting Accreditation Council for Graduate Medical Education (ACGME) accreditation in neurotology and employment at an academic center. The annual volume of cochlear implant surgeries varied considerably across centers. Eighty percent of respondents report to offer all three Food and Drug Administration (FDA) approved device brands at their center. In cases where hearing preservation is not a goal, 56% of respondents prefer a perimodiolar electrode design, while 44% prefer a lateral wall electrode. With regard to insertion technique for scala tympani access, 64% prefer inserting through the round window (RW) membrane, 26% prefer an extended round window (ERW) approach, while only 10% prefer a cochleostomy. In cases where hearing preservation is a goal of surgery, 86% of respondents prefer a lateral wall electrode design, while only 14% prefer a perimodiolar electrode design. With regard to insertion technique, 86% prefer RW insertion, while only 9% prefer an ERW approach, and only 5% prefer a cochleostomy. Respondents who prefer RW electrode insertion more commonly use a lateral wall electrode, whereas those who prefer an ERW or cochleostomy approach more commonly use a periomodiolar electrode (p < 0.001). There was a statistically significant greater number of surgeons that prefer lateral wall electrodes (p < 0.001) and RW insertion (p < 0.001) for hearing preservation cases compared with non-hearing preservation cases. For implantation of a hypothetical patient with significant residual hearing, within the range of hybrid device candidacy, the great majority of surgeons prefer a conventional length electrode and only 29% prefer to implant a hybrid device. In cases where hearing preservation is a goal, the overwhelming majority of respondents (96%, n = 74) reported using at least one form of steroid application. Ninety-two percent (n = 71) used intraoperative intravenous steroids, 55% (n = 42) prescribed postoperative oral steroids, 44% (n = 34) bathed the middle ear with steroids during surgery, and 30% (n = 23) prescribed preoperative oral steroids. Together, 65% of respondents use a variation of the tight subperiosteal pocket technique for internal device fixation, 19% prefer a bony well and trough with tie-downs, 10% prefer a bony well and trough without tie-downs, and 5% prefer a screw fixation system. Of the 50 who prefer utilizing a subperiosteal pocket, 31 (62%) incorporate a bony trough and 19 (38%) perform a subperiosteal pocket alone without a bony trough. Conclusion: The results of this survey reflect the evolution in surgical and device-related preferences for cochlear implantation in the United States. An increasing number of surgeons prefer the RW approach for electrode insertion. When hearing preservation is not a goal, there is a near even preference for lateral wall and perimodiolar electrode designs; however, the great majority of surgeons prefer a lateral wall design when attempting to preserve residual acoustic hearing. Even in cases where substantial residual hearing exists, many surgeons prefer conventional length electrodes to hybrid designs. These changes reflect the prioritization of atraumatic surgery and parallel the development of thinner and more atraumatic electrodes.

Study Design: Systematic review. Objectives: The objective of this systematic review was to answer 2 key questions: (1) What is the clinical presentation and probability of symptomatic improvement following treatment for patients with renal cell carcinoma (RCC) of the spine? (2) What is the overall survival of patients diagnosed with spinal metastases from RCC? Methods: A literature review was performed to identify articles that reported on survival, clinical outcomes, and/or prognostic factors in the RCC population with spinal metastases from 1986 to 2016. Results: Forty-eight articles (807 patients) were included. The Fuhrman Nuclear Grade has been significantly associated with survival in previous studies but was underpowered in the current study. The Memorial Sloan-Kettering Cancer Center Score (MSKCC/Motzer) was also underpowered in the current study. From the time of spinal metastasis, the mean and median survival for patients with previously diagnosed primary RCC was 8.75 and 11.7 months, respectively, whereas synchronously diagnosed patients (primary RCC and spinal metastasis) had a mean and median survival of 6.75 and 11 months, respectively. Patients with a “low” (0-8), “intermediate” (9-11), or “high” (12-15) revised Tokuhashi score at initial presentation had a median survival of 5.4, 11.7, and 32.9 months, respectively. Conclusion: Patients with either a synchronous or latent diagnosis of RCC survived greater than 6 months from the time of presentation. Initial Furhman grade, Tokuhashi score, and MSKCC/Motzer can be useful tools in informing patient-specific prognosis for those with metastatic RCC of the spine.

ObjectiveTo determine whether machine learning can accurately classify human papillomavirus (HPV) status of oropharyngeal squamous cell carcinoma (OPSCC) using computed tomography (CT)-based texture analysis.MethodsTexture analyses were retrospectively applied to regions of interest from OPSCC primary tumors on contrast-enhanced neck CT, and machine learning was used to create a model that classified HPV status with the highest accuracy. Results were compared against the blinded review of 2 neuroradiologists.ResultsThe HPV-positive (n = 92) and -negative (n = 15) cohorts were well matched clinically. Neuroradiologist classification accuracies for HPV status (44.9%, 55.1%) were not significantly different (P= 0.13), and there was a lack of agreement between the 2 neuroradiologists (κ = −0.145). The best machine learning model had an accuracy of 75.7%, which was greater than either neuroradiologist (P< 0.001,P= 0.002).ConclusionsUseful diagnostic information regarding HPV infection can be extracted from the CT appearance of OPSCC beyond what is apparent to the trained human eye.

Key Points Rd continuous significantly extended OS compared with MPT and resulted in comparable OS to that with Rd18 in patients with multiple myeloma. Patients achieving complete or very good partial response with Rd benefited greatly from continuous vs fixed treatment in terms of PFS.

Objective Determinants of the increased risk of diffuse large B-cell lymphoma (DLBCL) in SLE are unclear. Using data from a recent lymphoma genome-wide association study (GWAS), we assessed whether certain lupus-related single nucleotide polymorphisms (SNPs) were also associated with DLBCL. Methods GWAS data on European Caucasians from the International Lymphoma Epidemiology Consortium (InterLymph) provided a total of 3857 DLBCL cases and 7666 general-population controls. Data were pooled in a random-effects meta-analysis. Results Among the 28 SLE-related SNPs investigated, the two most convincingly associated with risk of DLBCL included the CD40 SLE risk allele rs4810485 on chromosome 20q13 (OR per risk allele=1.09, 95% CI 1.02 to 1.16, p=0.0134), and the HLA SLE risk allele rs1270942 on chromosome 6p21.33 (OR per risk allele=1.17, 95% CI 1.01 to 1.36, p=0.0362). Of additional possible interest were rs2205960 and rs12537284. The rs2205960 SNP, related to a cytokine of the tumour necrosis factor superfamily TNFSF4, was associated with an OR per risk allele of 1.07, 95% CI 1.00 to 1.16, p=0.0549. The OR for the rs12537284 (chromosome 7q32, IRF5 gene) risk allele was 1.08, 95% CI 0.99 to 1.18, p=0.0765. Conclusions These data suggest several plausible genetic links between DLBCL and SLE.