Citations

Objective: To compare language and audiological outcomes among infants (<9 and <12 mo) and older children receiving cochlear implantation (CI). Study design: Retrospective chart review. Setting: Tertiary academic referral center. Patients: Pediatric patients receiving CI between October 1995 and October 2019. Intervention: Cochlear implantation Main Outcome Measures: Most recent language and audiological assessment scores were evaluated by age group. Results: A total of 118 children were studied, including 19 who were implanted <9 months of age, 19 implanted 9 to <12 months of age, and 80 implanted 12 to <36 months of age. The mean duration of follow-up was 7.4 ± 5.0 years. Most recent REEL-3 receptive (88 ± 12 vs. 73 ± 15; p = 0.020) and expressive (95 ± 13 vs. 79 ± 12; p = 0.013) communication scores were significantly higher in the <9 months group compared to the 9 to <12 months group. PLS and OWLS auditory comprehension and oral expression scores were significantly higher in the <12 months group compared to the 12 to <36 months group. The difference in NU-CHIPS scores between <12 and 12 to <36 months was statistically significant (89% ± 6 vs. 83% ± 12; p = 0.009). LNT scores differed significantly between <9 and 9 to <12 months (94% ± 4 vs. 86% ± 10; p = 0.028). Conclusions: The recent FDA expansion of pediatric CI eligibility criteria to include infants as young as 9 months of age should not serve as a strict clinical cutoff. Rather, CI can be pursued in appropriately selected younger infants to optimize language and audiological outcomes.

Objective: To compare the prevalence of surgical, anesthetic, and device-related complications among infants and older children receiving cochlear implantation (CI). Study Design: Retrospective chart review. Setting: Tertiary academic referral center. Patients: Pediatric patients who underwent CI from November 1990 to January 2020. Intervention: CI. Main Outcome Measures: Surgical, anesthetic, and device-related complication rates were compared by age group (<12 versus 12–23 versus 24+ months with subset analysis of <9 versus 9–11 months). Results: A total of 406 primary pediatric CI surgeries encompassing 482 ears were analyzed, including 45 ears in 23 patients implanted less than 9 months and 89 ears in 49 patients less than 12 months. No anesthetic complications occurred. Postoperative surgical and device-related complication rates were not significantly different among the less than 12, 12 to 23, and 24+ month groups (16% versus 16% versus 12%; p = 0.23) or between the less than 9 and 9 to 11 month groups (22% versus 9%; p = 0.09). Thirty-day readmission was significantly higher for patients less than 12 months compared with patients 24+ months (6% versus <1%; p = 0.011), but was not significantly higher compared with patients 12 to 23 months (6% versus 3%; p = 0.65). Reoperation rates did not differ significantly among the less than 12, 12 to 23, and 24+ month groups (10% versus 7% versus 6%; p = 0.31). Conclusions: The prevalence of surgical, anesthetic, and device related complications was not significantly different among infants implanted less than 9 or less than 12 months of age when compared with older children. These data provide evidence for the continued expansion of pediatric cochlear implant candidacy criteria to include appropriately selected infants less than 9 months of age.

Objective: Reports using large brain tumor and cancer registries suggest that the incidence of vestibular schwannoma is considerably lower in the United States compared with other countries. The current study compares the incidence and disease characteristics of vestibular schwannoma within a large tertiary referral center's brain tumor and cancer registry using nationally mandated reporting protocols to disease incidence in the same population using an externally validated population-based consortium. Study Design: Population-based study spanning 1,945,007 person-years. Setting: Large tertiary referral center. Patients: Adults with sporadic vestibular schwannoma. Main Outcome Measure: Disease incidence rates from 2004 to 2016. Results: From 2004 to 2016, the incidence of vestibular schwannoma in the tumor registry was 1.3 per 100,000 person-years whereas the population-based cohort had an incidence of 4.4 per 100,000 person-years. From 2012 to 2016, the incidence in the tumor registry was 1.4 per 100,000 person-years compared with 5.2 in the population-based cohort. Patients within the population-based cohort were significantly more likely to have smaller tumors at diagnosis (78% intracanalicular versus 45%; p = 0.004) and consequently more likely to undergo management consisting of observation with serial imaging as opposed to treatment with either microsurgery or radiosurgery (71% versus 28%; p = 0.001). Conclusions: The reliance on pathology specimens and cancer-related treatment data for the national registration of new cancer and brain tumor diagnoses may introduce selection bias and underreporting of benign brain tumors that frequently involve observation as a primary treatment modality. This selection bias likely accounts for the discrepant incidence rates of vestibular schwannoma reported between the United States and other countries.

ObjectiveThe degree to which various treatment modalities modify vestibular schwannoma (VS)–associated symptoms has received limited attention. The purpose of this study was to determine how different treatment modalities affect subjective symptoms in those presenting with VS.Study DesignProspective survey.SettingTertiary neurotology referral center.MethodsPatients with sporadic VS who received treatment at our institution were prospectively surveyed with a VS symptom questionnaire. Those who completed a baseline survey prior to treatment and at least 1 posttreatment survey were included. The prospective survey evaluated the severity of self‐reported symptoms (Likert scale, 1‐10), including tinnitus, dizziness or imbalance, headaches, and hearing loss.ResultsA total of 244 patients were included (mean age, 57 years). The mean duration of follow‐up was 2.1 years, and the median number of surveys completed was 2 (interquartile range, 1‐3). Seventy‐eight (32%) cases were managed with observation, 118 (48%) with microsurgery, and 48 (20%) with radiosurgery. Multivariable analyses revealed no statistically significant difference in the change in tinnitus (P =. 15), dizziness or imbalance (P = 0.66), or headaches (P =. 24) among treatment groups. Evaluation of clinically important differences demonstrated that microsurgery leads to significant bidirectional changes in headaches.ConclusionsLimited prospective data exist regarding the progression or resolution of subjective symptoms in those presenting with VS. This study suggests that tinnitus, dizziness or imbalance, and headaches are unlikely to be significantly modified by treatment modality and generally should not be used to direct treatment choice.

Objectives/Hypothesis To describe the pre‐implant audiometric profile of adult cochlear implant (CI) recipients to investigate whether current binaural candidacy requirements prevent access to patients who could benefit from CI. Study Design Retrospective case series. Methods Retrospective review from 2016 to 2018 evaluating preoperative pure‐tone thresholds and speech perception scores in the ipsilateral and contralateral ear. Results A total of 252 adult CI recipients undergoing 270 implants were identified. Median age at time of implantation was 70.5 years (IQR 61.3–78.3) for those undergoing unilateral implantation and 59.0 (IQR 48.0–72.3) for those undergoing bilateral implantation ( P < .01). For unilateral implantation, median pre‐implantation speech perception performance in the ear to be implanted was 8.0% (IQR 0%–26.0%) for CNC word scores, and 9.0% (IQR 0%–34.0%) for AzBio sentence scores in quiet. Median speech perception performance in the contralateral ear was 36.0% (IQR 14.0%–60.0%) on CNC word scores, and 48.5% (IQR 17.5%–76.0%) on AzBio sentence tests. Speech perception scores were significantly different between ears for word and sentence tests ( P < .01). Patients older than age 65 were significantly less likely to undergo bilateral implantation ( P = .03). Conclusions Adult CI recipients exhibit substantially poorer pre‐implant speech perception scores than the commonly utilized ipsilateral qualifying threshold of 50% on sentence testing. Yet, existing insurance paradigms limit patients by excessively stringent binaural best‐aided requirements. This limitation likely leads to worse device performance as patients often wait years for their binaural hearing to qualify while their ear to be implanted potentially experiences an unnecessarily extended duration of deafness. Older patients also experience a unique delay in referral for cochlear implantation. Level of Evidence 3‐ Retrospective review Laryngoscope , 131:E2007–E2012, 2021

Objectives/HypothesisTo examine the hearing status and aural rehabilitative profile in a national cohort of patients with sporadic vestibular schwannoma (VS).Study DesignCross‐sectional surveyMethodsA cross‐sectional survey of Acoustic Neuroma Association members diagnosed with sporadic VS was performed from February 2017 through January 2019. Self‐reported results were used to determine the aural rehabilitative profile of respondents.ResultsAmong survey respondents, 62.2% (546/878) were not using any hearing‐assistive device at time of survey. For the 37.8% (332/878) that were utilizing hearing‐assistive devices, 32.8% (109/332) reported using a behind‐the‐ear hearing aid, 23.8% (79/332) used a contralateral routing of signal (CROS) hearing aid, and 21.7% (72/332) used a bone conduction device. Notably, 41.9% (278/663) of patients who previously underwent tumor treatment reported utilizing a hearing rehabilitation device at some point during VS management compared to 27.0% (58/215) of those undergoing observation with serial imaging (P < .001). Of 275 patients with functional hearing in the ipsilateral ear, 26.5% (73/275) reported having used at least one type of hearing device; 24.0% (66/275) reported use of a conventional hearing aid, 0.7% (2/275) a CROS aid, and 0.4% (1/275) a bone conduction device. Among respondents reporting ipsilateral nonfunctional hearing, 44.9% (258/575) reported having used at least one type of hearing device; 13.0% (75/575) a CROS aid, and 12.3% (71/575) a bone conduction device.ConclusionsEven among a cohort with presumably elevated literacy surrounding hearing rehabilitation options, few patients with a history of unilateral vestibular schwannoma ultimately use hearing assistive devices long‐term, suggesting that most patients sufficiently adjust to unilateral hearing loss or are unsatisfied with the benefits achieved with current device options.Level of Evidence4 Laryngoscope, 131:1378–1381, 2021

Objective: Limited data currently characterize management trends of sporadic vestibular schwannoma (VS) in the United States over recent decades. Serving as the primary patient support organization in the United States, the Acoustic Neuroma Association (ANA) facilitates amalgamation of data from patients treated from 1970 through January 2019. The primary objective of the current study was to characterize the evolution in the management of sporadic VS among ANA survey respondents treated over the last half-century. Study Design/Intervention: Cross-sectional survey. Setting/Patients: ANA survey respondents diagnosed with sporadic VS. Main Outcome Measures: Trends in rates of microsurgery, radiosurgery, and observation from 1970 to 2019. Results: Among 953 patients with VS responding during the study period, 876 (92%) were included. Subjects reported the following management strategies: 220 observation (25%), 454 microsurgery (52%), and 193 radiosurgery (22%). The rate of microsurgery decreased from 86–88% from 1970 to 2000 to 44% in the last decade. The proportion of VS observed increased from 7% of all tumors in the 1990s, to 14% in the 2000s, to 33% in the 2010s. The rate of radiosurgery has increased to 28% in the last decades compared with 7% in the 1990s. The rate of subtotal resection in tumors > 2 cm increased from 34% in the 2000s to 60% in the 2010s (p = 0.0011). Conclusions: Overall, these data support a progression toward fewer patients treated with microsurgery in recent decades. Radiosurgery and observation became increasingly more common after the turn of the century, possibly due to better detection of small and asymptomatic tumors and a greater understanding of the natural history of disease.

Background: Previous cross-sectional studies analyzing quality of life (QOL) outcomes in patients with sporadic vestibular schwannoma (VS) have shown surprisingly little difference among treatment modalities. To date, there is limited prospective QOL outcome data available comparing baseline to posttreatment scores. Study Design: Prospective longitudinal study using the disease-specific Penn Acoustic Neuroma Quality of Life (PANQOL) scale. Setting: Large academic skull base center. Patients: Patients diagnosed with unilateral VS who completed a baseline survey before treatment and at least one posttreatment survey. Main Outcome Measures: Change in PANQOL scores from baseline to most recent survey. Results: A total of 244 patients were studied, including 78 (32%) who elected observation, 118 (48%) microsurgery, and 48 (20%) stereotactic radiosurgery. Patients who underwent microsurgery were younger (p < 0.001) and had larger tumors (p < 0.001) than those who underwent observation or radiosurgery; there was no significant difference in duration of follow-up among management groups (mean 2.1 yrs; p = 0.28). When comparing the total PANQOL score at baseline to the most recent survey, the net change was only –1.1, –0.1, and 0.3 points on a 100-point scale for observation, microsurgery, and radiosurgery, respectively (p = 0.89). After multivariable adjustment for baseline features, there were no statistically significant changes when comparing baseline to most recent scores within each management group for facial function, general health, balance, hearing loss, energy, and pain domains or total score. However, the microsurgical group experienced a 10.8-point improvement (p = 0.002) in anxiety following treatment, compared with 1.5 (p = 0.73) and 5.3 (p = 0.31) for observation and radiosurgery, respectively. Conclusions: In this prospective longitudinal study investigating differences in QOL outcomes among VS treatment groups using the disease-specific PANQOL instrument, treatment did not modify QOL in most domains. Microsurgery may confer an advantage with regard to patient anxiety, presumably relating to the psychological benefit of “cure” from having the tumor removed.

The present state of vestibular schwannoma (VS) management is the product of over a century of technical progress by revolutionary surgeons who transformed a once perilous operation. At the beginning of the 1900s, patients who did not succumb to their disease were treated exclusively with surgery, which itself was almost assuredly devastating. Through the pioneering work of surgeons such as Harvey Cushing, Walter Dandy, William House, and others, safer surgical approaches were established with concurrent advances in neuromonitoring, neuroanesthesia, radiology, and adoption of the operating microscope. Owing to refinements in radiosurgical treatment and a greater understanding of the natural history of disease, there has been a dramatic shift toward more conservative management in recent years. For more than 100 years, the Mayo Clinic in Rochester, MN, has maintained an active and uninterrupted VS practice with activities that are well documented and preserved through the Mayo Clinic historical archives. We herein report representative early cases to illustrate the fascinating evolution in VS surgery over the past century at a single-tertiary referral center. Original clinical, imaging, pathology, and operative reports are presented from each era of management. To accurately portray the medical context of each era, antiquated terminology is intentionally preserved as originally transcribed.