Carlson, Matthew L.

OBJECTIVE The object of this study was to ascertain outcomes of cochlear implantation (CI) following stereotactic radiosurgery (SRS) for vestibular schwannoma (VS). METHODS The authors conducted a retrospective chart review of adult patients with VS treated with SRS who underwent CI between 1990 and 2019 at a single tertiary care referral center. Patient demographics, tumor features, treatment parameters, and pre- and postimplantation audiometric and clinical outcomes are presented. RESULTS Seventeen patients (18 ears) underwent SRS and ipsilateral CI during the study period. Thirteen patients (76%) had neurofibromatosis type 2 (NF2). Median age at SRS and CI were 44 and 48 years, respectively. Median time from SRS to CI was 60 days, but notably, 4 patients underwent SRS and CI within 1 day and 5 patients underwent CI more than 7 years after SRS. Median marginal dose was 13 Gy. Median treatment volume at the time of SRS was 1400 mm 3 (range 84–6080 mm 3 , n = 15 patients). Median post-CI PTA was 28 dB HL, improved from 101 dB HL preoperatively (p < 0.001). Overall, 11 patients (12 ears) exhibited open-set speech understanding. Sentence testing was performed at a median of 10 months (range 1–143 months) post-CI. The median AzBio sentence score for patients with open-set speech understanding was 76% (range 19%–95%, n = 10 ears). Two ears exhibited Hearing in Noise Test (HINT) sentence scores of 49% and 95%, respectively. Four patients achieved environmental sound awareness without open-set speech recognition. Two had no detectable auditory percepts. CONCLUSIONS Most patients who underwent CI following SRS for VS enjoyed access to sound at near-normal levels, with the majority achieving good open-set speech understanding. Implantation can be performed immediately following SRS or in a delayed fashion, depending on hearing status as well as other factors. This strategy may be applied to cases of sporadic or NF2-associated VS.

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ObjectiveTo examine the impact of treatment on disease‐specific health‐related quality of life (HRQOL) among patients with sporadic vestibular schwannoma (VS).Study DesignNational cross‐sectional study.SettingTertiary referral center and the Acoustic Neuroma Association.Subjects and MethodsAdult patients with VS who completed the Penn Acoustic Neuroma Quality of Life (PANQOL) survey. Patients were classified by treatment at the most recent survey as microsurgery, radiosurgery, observation, or recently diagnosed (RD). PANQOL scores were compared among VS treatment groups and with nontumor controls using analysis of covariance.ResultsA total of 1362 respondents with VS were analyzed. Total PANQOL scores differed significantly by treatment (P =. 024) with adjusted means of 60, 63, 65, and 62 for the microsurgery, radiosurgery, observation, and RD groups, respectively. Subdomain scores for facial function, balance, hearing loss, and pain differed significantly (P <. 05) by treatment. Comparisons that surpassed thresholds for clinical significance included microsurgery compared to radiosurgery and observation for facial function, microsurgery compared to observation and RD for hearing loss, and microsurgery compared to radiosurgery and observation for pain. Subdomain scores for anxiety, facial function, balance, hearing loss, energy, and pain and total PANQOL scores for nontumor controls were significantly higher (ie, more favorable) compared to the VS groups (P <. 05). General health scores did not differ significantly between the nontumor controls and the VS groups (P =. 19).ConclusionDifferences in HRQOL among treatment modalities are small and variably exceed the minimal clinically important difference. The impact of a VS diagnosis on HRQOL supersedes that of the treatment type.

Stapedectomy remains a joint key‐indicator case with ossiculoplasty for otolaryngology residents in the United States. Yet, residents consistently report feeling inadequately prepared to perform stapes surgery following graduation. Applying recently described age‐ and sex‐standardized incidence rates of surgically confirmed cases of otosclerosis to the US populace, upper and lower estimates of residents’ case exposure to stapedectomy can be approximated. With this, uppermost projections estimate 6484 new cases of stapes surgery are performed annually nationwide. With approximately 1424 otolaryngology residents nationally, the average case exposure is 7.8 stapedectomies throughout their training, with upper and lower estimates of 17.1 and 4.2 cases, respectively. As such, proficiency in stapedectomy is no longer a realistic expectation for US graduating residents. This reality supports the removal of “stapedectomy” from the list of 14 key‐indicator case requirements, leaving ossiculoplasty as its own key‐indicator case, thereby reinforcing true competence in this fundamental procedure for the graduating otolaryngologist.

Objectives: To evaluate blood–labyrinth barrier permeability using delayed gadolinium enhanced three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) in patients with untreated sporadic vestibular schwannoma (VS) to interrogate the etiopathogenesis of progressive sensorineural hearing loss. Design: Prospective case series. Setting: Single institution tertiary care center. Participants: Patients with previously untreated small to medium-sized unilateral VS undergoing 3D-FLAIR imaging 10 minutes (immediate) and 5 to 8 hours (delayed) after administration of intravenous gadolinium. Main Outcome Measures: Comparison of cochlear and vestibular signal intensity ratios (SIRs) across immediate and delayed images and across the tumor and nontumor ear. Results: Six of eight (75%) patients demonstrated asymmetric enhancement of inner ear structures on delayed contrast-enhanced imaging. Delayed mean cochlea and vestibule SIRs were significantly greater than immediate SIRs (cochlea: 1.91 versus 1.21 [p = 0.02]; vestibule 1.74 versus 1.15 [p = 0.02]). A higher vestibule SIR was statistically significantly associated with poorer pure-tone average and word recognition scores (p = 0.001). Conclusion: Ears with VS exhibited increased enhancement on delayed 3D FLAIR imaging. These findings suggest that alterations in blood–labyrinth barrier permeability may explain progressive sensorineural hearing loss in a subset of patients with untreated VS.

Objective: Definitive treatment of sporadic vestibular schwannoma (VS) following documented growth is common practice at most centers in the United States. However, as a natural extension of this paradigm, very little evidence exists surrounding the natural history of growing tumors. The primary objective of the current work was to describe the natural history of sporadic VS following documentation of initial tumor growth. Study Design: Retrospective cohort study. Setting: Tertiary referral center. Patients: Patients diagnosed with sporadic VS between January 1, 2001 and December 31, 2015 who elected continued observation despite having volumetric growth ≥20% of original tumor volume on interval magnetic resonance imaging (MRI). Main Outcome Measure: Survival free of subsequent volumetric growth. Results: Of 361 patients undergoing observation with serial imaging during the study period, 85 patients met inclusion criteria at a median age of 66 years (interquartile ranges [IQR] 55–71). Within this cohort, 40 patients demonstrated subsequent volumetric growth at a median of 1.7 years (IQR 1.0–2.6) from the date of initial MRI that documented growth. The median volumetric growth was 43% (IQR 28–57), and the median growth rate was 0.026 cm3 per year (IQR 0.009–0.107). Survival free of subsequent volumetric growth rates (95% CI; number still at risk) at 1, 2, 3, 4, and 5 years were 93% (87–99; 75), 67% (58–79; 45), 54% (43–67; 29), 44% (33–59; 19), and 41% (30–56; 12), respectively. Conclusions: In a cohort exclusively comprised of sporadic VS with documented growth, over 40% of tumors demonstrated no subsequent volumetric growth after 5 years of continued MRI surveillance. These data challenge the supposition that once growth occurs, all tumors will exhibit sustained growth. Continued observation after documented growth of sporadic VS is a reasonable consideration in appropriately selected cases.

Objective: Describe the outcomes of treatment for patients with cholesteatomas that are medially invasive to the otic capsule, petrous apex, and/or skull base. Study Design: Retrospective case series Setting: Two tertiary care academic centers. Patients: Patients surgically managed for medially-invasive cholesteatoma at two tertiary care institutions from 2001 to 2017. Interventions: Surgical management of medially-invasive cholesteatomas. Main Outcome Measures: The presenting symptoms, imaging, pre- and post-operative clinical course, and complications were reviewed. Results: Seven patients were identified. All patients had pre-operative radiographic evidence of invasive cholesteatoma with erosion into the otic capsule beyond just a lateral semicircular canal fistula. Five patients had a complex otologic history with multiple surgeries for recurrent cholesteatoma including three with prior canal wall down mastoidectomy surgeries. Average age at the time of surgery was 41.3 years (range 20-83). Two patients underwent a hearing preservation approach to the skull base while all others underwent a surgical approach based on the extent of the lesion. Facial nerve function was maintained at the pre-operative level in all but one patient. No patient developed cholesteatoma recurrence. Conclusions: The medially-invasive cholesteatoma demonstrates an aggressive, endophytic growth pattern, invading into the otic capsule or through the perilabyrinthine air cells to the petrous apex. Surgical resection remains the best treatment option for medially-invasive cholesteatoma. When CSF leak is a concern, a subtotal petrosectomy with closure of the ear is often necessary.

Podcasts are online digital audio programs that are disseminated via online subscription that are easily accessible through computers or smartphones. Increasingly, residents and medical students are prioritizing podcasts for asynchronous medical education due to ease of use, convenience (eg, use while exercising or commuting), and repeatability. Some trainees have found podcasts more useful than traditional didactic lectures. Given the increasing requirements of social distancing and the need for distance medical education platforms, podcast production can serve as a useful tool to complement resident and medical student education and is a resource that will remain accessible in perpetuity. An otolaryngology specialty podcast, “Headmirror’s ENT in a Nutshell,” was created to augment asynchronous learning and address the acute need for distance learning opportunities. Over the first 7 weeks of production, 50 episodes were created. Episodes were posted on www.headmirror.com, with subscription services available through Apple Podcast, Spotify, and other platforms.

Electrocochleography (ECochG) is an electrophysiological technique that records electrical potentials generated by different components of the inner ear and peripheral cochlear nerve in response to acoustic stimulation. ECochG responses can be analyzed into (1) cochlear microphonics (CM), (2) auditory nerve neurophonics, (3) summating potential, and (4) compound action potential. Over the past few decades, there have been ongoing refinements in technique and updates in the understanding of recorded potentials. Historically, ECochG found its main application in the diagnostic evaluation of Meniere's disease (MD). However, in the last decade, the focus has shifted towards cochlear implantation (CI). In patients with residual hearing after CI, combined electric and acoustic stimulation has resulted in improved hearing and speech outcomes. Despite efforts to mitigate trauma during electrode insertion, hearing preservation rates vary after surgery. During implantation, real‐time ECochG offers an opportunity to measure frequency specific CMs elicited from a localized region in the cochlea as the surgeon inserts the electrode array. In extracochlear ECochG recordings, the recording electrode can be placed on the promontory, the stapes, or the tympanic membrane. Intracochlear ECochG can be performed by inserting a recording electrode into the cochlea or by using one of the CI electrodes as the recording electrode. The loss of intraoperative ECochG signal may indicate cochlear trauma from electrode insertion, but the association between intraoperative ECochG changes and cochlear trauma remains controversial. The ability to monitor cochlear trauma during CI electrode placement holds promise to improve hearing preservation outcomes, modify surgical techniques, and change electrode design. The goal of this review is to provide a comprehensive overview of the electrophysiology and history of ECochG, discuss its recent applications in CI, and explore the ongoing research in this expanding field.