Lohse, Christine M.

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.

Abstract: Calciphylaxis is a rare, painful, and life-threatening condition with a high mortality rate. Although the etiology of calciphylaxis is not well understood, it has been proposed that calcium deposition within and around subcutaneous vessels restricts blood flow chronically, thereby predisposing the patient to acute pannicular and dermal thrombosis. Given increasing recognition of the role of hypercoagulability in calciphylaxis, this retrospective cohort study sought to evaluate the presence of thromboses and dermal angioplasia in calciphylaxis. Moreover, we aimed to validate previous observations about the histopathology of calciphylaxis compared with skin biopsies from patients with end-stage renal disease but without calciphylaxis. After a meticulous clinical chart review, we assessed the corresponding skin biopsies for the presence of vessel calcification, thromboses, and dermal angioplasia in skin biopsies from patients with calciphylaxis (n = 57) and compared with those from patients with end-stage renal disease but without calciphylaxis (n = 26). Histopathologic findings were correlated with clinical features such as chronic kidney disease, diabetes, or associated malignancy. Our results validated a prior observation that calciphylaxis was significantly more likely to show calcification of dermal vessels and diffuse dermal thrombi. This study reports the frequent finding of dermal angioplasia, a potential marker of chronic low-grade ischemia, as another frequent microscopic finding in calciphylaxis. Among cases of calciphylaxis, histopathologic changes in patients with chronic kidney disease were indistinguishable from those in patients without chronic kidney disease, thereby implying a final common pathogenic pathway in both uremic and nonuremic calciphylaxis. In future, larger, prospective studies may be useful in validating these findings.

Tumor cell proliferation rate determined by either Ki-67 index or mitotic count (MC) has shown to be a prognostic factor for gastrointestinal neuroendocrine tumors in general, and after its incorporation in the 2010 World Health Organization tumor grading system, it has become essentially mandatory in pathology reports for all gastrointestinal neuroendocrine tumors, regardless of tumor location. Nevertheless, clinical significance for the Ki-67 index or MC has not been well demonstrated in small intestinal neuroendocrine tumor (SINET), especially those without distant metastasis, the majority of which have very low proliferation rates. We assessed the clinical behavior of 130 SINETs in relation to stage, Ki-67 index, MC, and other pathologic features. Most SINETs (86%) were grade 1 and 14% were grade 2. There were no grade 3 tumors or poorly differentiated neuroendocrine carcinomas. On multivariate analysis, age, Ki-67 index >5%, MC >10/50 high-power field, stage IV, and liver metastases were associated with increased risk of death in all patients. When both stage and grade were considered, Ki-67 index >5% was associated with a nearly 4-fold increased risk of death in stage IV cases (n=60). In contrast, Ki-67 index did not show prognostic value for patients with stages I to III disease (n=70), although MC >1/50 high-power field was significantly associated with death on multivariable analysis. Our study confirms that liver metastasis and increased tumor cell proliferation rate are independent prognostic factors for SINETs, but shows that most SINETs have a very low proliferation rate, which limits its value for predicting tumor behavior. By combining staging and grading information, we demonstrate different roles and cutoff values of Ki-67 index and MC in SINET with different stages.

Objectives: To compare the diagnostic yield of high-resolution volumetric T2-weighted MRI (HRT2-MRI) with high-resolution computed tomography (HRCT) for diagnosis of large vestibular aqueduct (LVA). Study Design: Three board-certified neuroradiologists performed an independent, blinded radiological review for diagnosing LVA with 2:1 age-matched controls on patients with both HRCT and HRT2-MRI imaging. Setting: Tertiary referral center. Patients: All patients between 2002 and 2016 with hearing loss who underwent both HRCT and HRT2-MRI and were diagnosed with LVA on either modality. Main Outcome Measures: Concordance rate for LVA between HRCT and HRT2-MRI. Results: Concordance rate for HRCT and HRT2-MRI for diagnosing LVA was 88% (124/141) when assessing both the midpoint and external aperture diameters. Fifteen ears had LVA on computed tomography (CT), but not on magnetic resonance imaging (MRI); in comparison, two ears had LVA on MRI, but not on CT (p = 0.002). Excellent inter-rater reliability among the three radiologists was demonstrated. Conclusion: Historically, HRCT has been the imaging modality of choice for diagnosing LVA. Although a higher concordance rate of HRT2-MRI was found compared with previous studies utilizing earlier MRI technology, HRCT still detected a larger number of patients with clinically significant hearing loss compared with MRI. Given the high concordance rate and efficacy of both modalities in diagnosing LVA, the ultimate decision of which modality to choose may depend on other patient-specific and clinical factors.

Obesity in children is associated with several co-morbidities including dyslipidemia. Obstructive sleep apnea (OSA) is commonly seen in obese children. In adults, diagnosis of OSA independent of obesity is associated with cardiometabolic risk factors including dyslipidemia. There is limited data on the impact of treatment of OSA on lipids in children. The objective of the study was to examine the impact of treatment of OSA on lipids in 24 obese children. Methods: Seventeen children were treated with continuous positive airway pressure (CPAP) and five underwent adenotonsillectomy. Mean apnea hypopnea index prior to treatment was 13.0 + 12.1 and mean body mass index (BMI) was 38.0 + 10.6 kg/m2. Results: Treatment of OSA was associated with improvement in total cholesterol (mean change = −11 mg/dL, p < 0.001), and low-density lipoprotein cholesterol (mean change = –8.8 mg/dL, p = 0.021). Conclusion: Obese children should be routinely screened for OSA, as treatment of OSA favorably influences lipids and therefore decreases their cardiovascular risk.

Objectives To compare renal functional changes after percutaneous cryoablation ( PCA ) or partial nephrectomy ( PN ). Patients and Methods Patients who underwent PCA or PN for a solitary renal mass at a single institution were identified (2003–2013). Estimated glomerular filtration rates ( eGFR s) were calculated at baseline, discharge, and at the 3‐month follow‐up using the Chronic Kidney Disease Epidemiology Collaboration equation. Changes in renal function were compared between groups using 1:1 propensity score ( PS ) matching, adjustment for PS quintile, and inverse probability weighting ( IPW ). Results There were 2 040 procedures available for the PS analyses, including 448 PCA and 1 592 PN . After PS adjustments, there were no significant differences in baseline clinical features between PCA and PN patients. In the PS ‐matched analysis, the change in eGFR from baseline to discharge for PCA and PN patients was −3.1 and −1.1 mL /min/1.73 m 2 , respectively ( P = 0.038), with percentage changes of −4.5% and 0% respectively ( P = 0.006). From baseline to the 3‐month follow‐up, the absolute change in eGFR for PCA and PN patients was −4.3 and −2.1 mL /min/1.73 m 2 , respectively ( P = 0.008), and the percentage change was −6.1% and −2.4% respectively ( P = 0.005). Similar results were obtained after adjusting for PS quintiles and in the IPW analysis. Importantly, the rate of chronic kidney disease stage progression at the 3‐month follow‐up was similar between the groups (21% vs 18%). Conclusions Our results confirm that both PCA and PN have a minor impact on renal function. While we observed a statistically greater decline in eGFR after PCA compared with PN , both approaches result in excellent preservation of renal function.

OBJECTIVEThe management of vestibular schwannoma (VS) remains controversial. One commonly cited advantage of microsurgery over other treatment modalities is that tumor removal provides the greatest chance of long-term cure. However, there are very few publications with long-term follow-up to support this assertion. The purpose of the current study is to report the very long-term risk of recurrence among a large historical cohort of patients who underwent microsurgical resection.METHODSThe authors retrospectively reviewed the medical records of patients who had undergone primary microsurgical resection of unilateral VS via a retrosigmoid approach performed by a single neurosurgeon-neurotologist team between January 1980 and December 1999. Complete tumor removal was designated gross-total resection (GTR), and anything less than complete removal was designated subtotal resection (STR). The primary end point was radiological recurrence-free survival. Time-to-event analyses were performed to identify factors associated with recurrence.RESULTSFour hundred fourteen patients met the study inclusion criteria and were analyzed. Overall, 67 patients experienced recurrence at a median of 6.9 years following resection (IQR 3.9–12.1, range 1.2–22.5 years). Estimated recurrence-free survival rates at 5, 10, 15, and 20 years following resection were 93% (95% CI 91–96, 248 patients still at risk), 78% (72–85, 88), 68% (60–77, 47), and 51% (41–64, 22), respectively. The strongest predictor of recurrence was extent of resection, with patients who underwent STR having a nearly 11-fold greater risk of recurrence than the patients treated with GTR (HR 10.55, p < 0.001). Among the 18 patients treated with STR, 15 experienced recurrence at a median of 2.7 years following resection (IQR 1.9–8.9, range 1.2–18.7). Estimated recurrence-free survival rates at 5, 10, 15, and 20 years following GTR were 96% (95% CI 93–98, 241 patients still at risk), 82% (77–89, 86), 73% (65–81, 46), and 56% (45–70, 22), respectively. Estimated recurrence-free survival rates at 5, 10, and 15 years following STR were 47% (95% CI 28–78, 7 patients still at risk), 17% (5–55, 2), and 8% (1–52, 1), respectively.CONCLUSIONSLong-term surveillance is required following microsurgical resection of VS even after GTR. Subtotal resection alone should not be considered a definitive long-term cure. These data emphasize the importance of long-term follow-up when reporting tumor control outcomes for VS.