Objectives Cerebellopontine angle (CPA) tumors, including vestibular schwannomas (VS), often require microsurgical resection. These are typically surgical procedures performed by co-surgeons, and some controversy and confusion exist regarding use of common procedural terminology (CPT) codes for billing, given the American Medical Association (AMA) describes two distinct coding sets for such cases. This investigation endeavored to: (1) Comprehensively review all surgical coding options; (2) instruct on application; and (3) explore contemporary coding trends among surgeons. Design A multi-disciplinary task force (otologist/neurotologists and neurosurgeons) performed direct correspondence with various surgical societies, private consultancies, and the AMA. Survey instruments were distributed to active surgeons. Setting Investigational period: 2022 to 2025. Survey instruments distributed at the 2023 American Neurotology Society (ANS) Fall Meeting. Participants ANS, American Academy of Otolaryngology (AAO-HNSF), and the North American Skull Base Society (NASBS) representatives. Outcomes Measures Usage rate for CPT code sets (percentage of responders). Results Two CPT coding systems are currently accepted for surgical management of CPA tumors. The traditional/legacy codes (Indicator-2) allow use of co-surgery modifiers (-62). The newer approach/resection codes (skull base codes; Indicator-1) restrict modifier use. The history, application, and controversies surrounding the codes are detailed herein. About 40% of surgeons reported use of the traditional codes, while 45 to 49% use the skull base codes. Response rate was 31% of the active ANS membership in 2023. Conclusion The AMA recognizes two CPT code sets for surgical management of CPA tumors. Both are appropriate, if properly applied. Contemporary surgeons likely utilize the sets in equal proportions.

OBJECTIVE:Characterize pathogenic variants, rates of mosaicism, genetic testing yield, and disease severity among patients with NF2-related schwannomatosis dichotomized by diagnosis before or after the age of 30. STUDY DESIGN:Retrospective analysis. SETTING:Tertiary referral center multidisciplinary NF2 clinic from 2021 to 2024. PATIENTS:Patients with NF2-related schwannomatosis. INTERVENTION:Next-generation sequencing. MAIN OUTCOME MEASURE:Rates of mosaicism, genetic testing rates and yield, and overall disease severity by tumor burden. RESULTS:From 2021 to 2024, there were 32 patients ≥30 years of age and 39 patients diagnosed at younger ages. Patients diagnosed ≥30 years exhibited decreased likelihood of receiving genetic testing (53% vs. 85%; P=0.009) and decreased genetic yield (defined as identification of a pathogenic genetic variant in those undergoing testing; 65% vs. 85%; P=0.20). Both age groups demonstrated similar rates of pathogenic variant type with loss-of-function being the predominant variant detected in 73% vs. 67%, for ≥30 vs. <30 years of age; P=0.90. Those ≥30 years harbored fewer number of average anatomic regions involved by tumor (2.3 vs. 3.4; P<0.001) and decreased total number of tumors (7 vs. 12; P<0.001). CONCLUSIONS:Patients diagnosed with NF2-related schwannomatosis at age ≥30 years exhibited reduced disease severity compared with those diagnosed at a younger age despite harboring similar distributions of genetic pathogenic variants inclusive of loss-of-function pathogenic variants. These observations emphasize the importance of considering patient age in addition to genetic testing for diagnostic framing tailored to patients' biology and clinical context to optimize care in the setting of NF2-related schwannomatosis.

OBJECTIVE:Auditory nerve monitoring through electrically evoked compound action potentials (eCAP) can be used to assess cochlear neural integrity during vestibular schwannoma (VS) resection. Herein, we report on 5 cochlear implant (CI) patients who underwent VS resection with present eCAP responses both intraoperatively and postoperatively. Patients either did not perceive any sound through their CI or rapidly adapted to electrical stimulation, suggesting that the presence of eCAP does not guarantee auditory nerve function in patients with VS resection. PATIENTS:Five patients who underwent simultaneous VS translabyrinthine resection and cochlear implantation. INTERVENTIONS:Simultaneous cochlear implantation and translabyrinthine microsurgical VS resection. MAIN OUTCOME MEASURE:Electrically evoked compound action potential. RESULTS:All patients had eCAP responses present during their VS resection and for at least 50 days post-surgery. Three of the 5 patients did not perceive any sound with their cochlear implant. This shows that the presence of an eCAP alone does not guarantee a functional auditory nerve or auditory perception. The other 2 patients could hear non-speech sounds, but these sounds quickly became inaudible, indicating that their auditory nerve lacked the ability to integrate the electrical stimulus over time. CONCLUSIONS:The presence of an eCAP response does not guarantee auditory sensation through a CI. Although eCAP measurements provide insight into the function of the distal end of the nerve, it does not ascertain integrity or function of the larger auditory nerve pathway. Therefore, eCAPs should not be used in isolation to monitor auditory nerve function during VS resection.

OBJECTIVE:Wait-and-scan surveillance is now commonly employed for initial management of small- and medium-sized vestibular schwannomas. Although small differences in tumor size are unlikely to impact outcomes significantly, treatment with either radiosurgery or microsurgery is usually recommended following radiological detection of tumor growth. The objective of the current study was to identify potential inflection points in vestibular schwannoma tumor size, where the risks of treatment with single-fraction stereotactic radiosurgery (SRS) accelerate to inform timing of intervention. METHODS:Adult (≥ 18 years old) patients with sporadic vestibular schwannoma who underwent SRS from 2000 through 2022 were included. RESULTS:A total of 749 patients with a median age at SRS of 62 years were studied, the majority (n = 566 [76%]) of whom had tumors extending into the cerebellopontine angle (CPA) at SRS. The optimal tumor size cut point to predict SRS failure and need for salvage treatment was 4 mm or more of CPA extension (c-index 0.59, HR 3.60, p = 0.01). The optimal tumor size cut point to predict the outcome of facial nerve paresis was 13 mm or more of CPA extension, resulting in a c-index of 0.63 (HR 2.88, p = 0.01). Among patients with at least 3 months of surveillance before SRS, those with a tumor growth rate ≥ 2.5 mm/year were more likely to undergo salvage treatment than those with a growth rate < 2.5 mm/year, although this difference did not achieve statistical significance (HR 1.82, p = 0.18). CONCLUSIONS:The risk of SRS failure requiring salvage treatment and the risk of post-SRS facial nerve paralysis increase at sizes of approximately 4 and 13 mm extension into the CPA, respectively, providing a size threshold anchor to help guide treatment decision-making regarding timing of SRS. Furthermore, rapid tumor growth during the initial wait-and-scan period may be associated with an increased risk of radiosurgical failure, which may influence choice of treatment.

OBJECTIVE:The objective of this study was to describe the long-term efficacy of single-fraction stereotactic radiosurgery (SRS) for the primary treatment of sporadic vestibular schwannoma. METHODS:Adult (≥ 18 years of age) patients with sporadic vestibular schwannoma who underwent SRS from 2000 through 2022 were included. RESULTS:A total of 749 patients met inclusion criteria, the majority (n = 566, 76%) of whom had tumors extending into the cerebellopontine angle at SRS. The median patient age at SRS was 62 years, half (50%) of the patients were women, and 744 (99%) exhibited House-Brackmann grade I facial nerve function at SRS. A total of 42 patients experienced radiosurgical failure and underwent salvage treatment; the median duration of follow-up for the patients who did not undergo salvage treatment was 7.0 years. Overall tumor control rates (95% CI, number still at risk) at 1, 3, 5, 10, and 15 years after SRS were 100% (100%-100%, 718), 98% (97%-99%, 589), 96% (94%-97%, 464), 92% (90%-95%, 258), and 91% (89%-94%, 125), respectively. Patient age (hazard ratio [HR] for a 10-year increase of 0.92, 95% CI 0.71-1.19; p = 0.5), presence of a macrocystic tumor (HR 0.59, 95% CI 0.14-2.46; p = 0.5), and treated tumor volume (HR for a 1-cm3 increase of 1.02, 95% CI 0.88-1.18; p = 0.8) were not significantly associated with the risk of salvage. Three distinct post-SRS tumor behavior patterns were observed, with 13% of patients demonstrating tumor pseudoprogression, all but 4 of whom demonstrated pseudoprogression by year 5 post-SRS. CONCLUSIONS:SRS demonstrates durable tumor control through 15 years of follow-up in most patients (91%); however, a minority are found to still experience SRS failure even beyond 10 years of surveillance. Although occurring in only a minority of patients, tumor pseudoprogression can be evident out to 5 years or longer post-SRS.

OBJECTIVE:The Complete Cochlear Implant Care (CCIC) model is a highly coordinated care delivery model reducing and condensing in-person visits for cochlear implant (CI) patients, leveraging telehealth and remote programming. Clinical, quality-of-life, implementation, patient experience, and financial outcomes of the clinical trial are reported. STUDY DESIGN:Prospective, nonrandomized, 2-arm clinical trial. SETTING:Tertiary referral CI center. PATIENTS:Adult CI candidates. INTERVENTIONS:Cochlear implantation. MAIN OUTCOME MEASURES:Speech perception, quality of life, implementation, patient experience, and financial outcomes for both CCIC and traditional care delivery models. RESULTS:Patients participating in the traditional and CCIC delivery models demonstrated 12-month postoperative speech perception scores comparable to nationally reported outcomes (CNC 61% and 72% and AzBio in quiet 75% and 87% for traditional and CCIC cohorts, respectively). Quality-of-life evaluation using the CIQOL-10 demonstrated similar 12-month postoperative scores between groups (52 and 54 for traditional and CCIC cohorts, respectively). The CI Patient Experience Survey demonstrated high satisfaction outcomes in ease of communication, preparedness for surgery, and overall care in both cohorts. Implementation analysis of the CCIC model revealed perceived benefits from staff and clinicians; however, support for expansion of the program was contingent upon the availability of app-based or web-based remote programming technology. Finally, financial analysis revealed decreased out-of-pocket costs for CCIC patients. CONCLUSION:The CCIC model has the potential to dramatically streamline hearing health care delivery and reduce out-of-pocket costs for CI recipients. Clinical outcomes between cohorts were comparable in this feasibility study; however, a sufficiently powered trial is required to definitively comment on clinical outcomes with the new care delivery model.

The leading cause of trigeminal neuralgia (TGN) relies on the microvascular conflict between the superior cerebellar artery (SCA) loop and the dorsal root entry zone of the trigeminal nerve (TN). However, lesions along the TN have been described as a possible cause of TGN for direct mass effect or indirect vascular transposition. Thus, the surgical approach to TGN in patients harboring cerebellopontine angle or Meckel's cave tumor should be methodically chosen. The retrosigmoid (RS) approach with suprameatal extension offers direct access to the TN in both its cisternal and Meckel's cave segment, allowing optimal TN decompression from vascular and tumoral components. Although the RS approach with suprameatal extension has been described in numerous studies,1-4 videos detailing its key steps in addressing a multicomponent TGN are lacking. In this video, we highlight the case of a 46 year-old woman with 6 months of medically refractory typical TGN with a right en plaque meningioma involving the petrous bone, petroclival junction, Meckel's cave, and tentorium. In addition, magnetic resonance imaging was suspicious for a compressive SCA loop over the dorsal root entry zone. The patient underwent a RS approach with suprameatal extension for subtotal resection of the tumor and microvascular decompression of the TGN. The patient recovered with no complications and TGN resolved.

Objective: To report the novel use of intraoperative electrically evoked stapedial reflex (eSR) for cochlear nerve monitoring during simultaneous translabyrinthine resection of vestibular schwannoma (VS) and cochlear implantation. Study Design: Clinical capsule report with video demonstration. Setting: Tertiary academic referral center. Patient: A 58-year-old female presented with a small right intracanalicular VS with associated asymmetrical right moderate to severe sensorineural hearing loss, poor word recognition, tinnitus, and disequilibrium. Based on patient symptomatology and goals, simultaneous CI with translabyrinthine resection of the VS was performed. Intervention: Cochlear implantation before the tumor was resected facilitated intraoperative eSRs by delivering repeated single-electrode stimulations through the cochlear implant (CI) electrode during tumor resection. A pulse duration of 50-us and a current amplitude of 200-CL or 648-us was used to elicit eSRs visible through the facial recess. Intraoperative eSR was monitored in conjunction with electrically evoked compound action potentials via neural response telemetry and electrical auditory brainstem response. Results: Despite the transient evoked compound action potentials amplitude and electrical auditory brainstem response latency changes, the visually observed eSR was preserved and remained robust throughout tumor dissection, indicating an intact cochlear nerve. Four weeks postoperatively, the patient exhibited open-set speech capacity (14% CNC and 36% AzBio in quiet). Conclusion: The current study demonstrates the feasibility of using intraoperative eSR via a CI electrode to monitor cochlear nerve integrity during VS resection, which may indicate successful CI outcomes. These preliminary findings require further substantiation in a larger study.

Objective To analyze the use of electrical field imaging (EFI) in the detection of extracochlear electrodes in cochlear implants (CI) Study Design Retrospective cohort study Setting Tertiary academic medical center Methods EFI patterns from 6 recipients with extracochlear electrodes were compared with 10 recipients with all intracochlear electrodes to identify differences in EFI patterns between these two groups. For the extracochlear group, EFI patterns were analyzed using two methods: visual inspection and calculation of the average impedance difference (AID) between specific EFI curves. These results were then compared with information from CT and plain film imaging, surgical notes, and audiology tests to confirm the placement of the electrodes in each recipient. Results An average AID of 0.06 kΩ was calculated from the EFI patterns for recipients with complete electrode insertion, whereas an average AID of 0.15 kΩ was calculated from the EFI curves measured for the extracochlear electrodes. Visual analysis of the EFI impedance curves for extracochlear electrodes showed a distinct separation from those measured for intracochlear electrodes. Based on AID analysis and visual inspection the EFI, patterns were in close agreement with the number of extracochlear electrodes detected based on either imaging, intraoperative surgical note, or audiological findings. Conclusion EFI is a noninvasive, inexpensive, and time-efficient measurement that can be used to detect extracochlear electrodes and monitor electrode migration from the cochlea.

OBJECTIVES:To compare electrical stimulation and speech perception in vestibular schwannoma (VS) patients across treatment modalities and standard cochlear implant (CI) patients. STUDY DESIGN:Retrospective review. SETTING:Tertiary academic center. PATIENTS:Sixty-seven CI ears consisting of 23 standard CI controls and 44 VS patients with ipsilateral CI. VS patients were separated by treatment modality: 24 microsurgical resection, 15 radiation therapy, and 5 observation. Eleven VS patients with CI did not receive auditory sensation from electrical stimulation. INTERVENTIONS:VS microsurgical resection, radiation therapy, and observation. MAIN OUTCOME MEASURES:Threshold (T-levels), comfort (C-levels), consonant-nucleus-consonant (CNC) word score, and AzBio sentence recognition scores. RESULTS:Patients who underwent microsurgical resection required significantly higher ( p < 0.001) T-levels and C-levels compared with the standard CI group. Stimulation levels in the VS radiation therapy and observation groups were higher but not significantly different compared with standard CI controls. Overall rate of CI nonstimulation across all VS patients was 25%: 38% for microsurgery and 13% for radiation. Average CNC score for microsurgery group was 18% compared with 44% for radiation, 55% for observation, and 68.5% for standard CI controls. CNC word and AzBio scores were significantly lower ( p < 0.001) for the microsurgery group compared with standard CI group. CONCLUSIONS:Resection may negatively affect auditory nerve function, leading to higher stimulation levels, increased risk of nonstimulation, and poorer postoperative CI speech outcomes compared with observation or radiosurgery. When medically appropriate, nonsurgical VS management may be used to allow for optimization of CI programming and postoperative hearing outcomes.

Introduction: Vestibular schwannomas (VS) are the most common tumor of the cerebellopontine angle. As VSs arise from the vestibular nerve, most displace CN VII anteriorly. An anteriorly displaced CN VII does not obscure access to the presenting pole of the VS via a suboccipital or translabyrinthine approach. Rarely, CN VII is found on the posterior, presenting, surface of the VS—adding difficulty to the surgical resection. We present surgical and clinical results in a series of patients undergoing microsurgery for VS in whom CN VII was displaced posteriorly.

Objectives:We investigated the extent of access to Meckel's cave (MC) and the middle cranial fossa (MCF) protecting the internal carotid artery (ICA) using the retrosigmoid approach with endoscopic-assisted reverse anterior petrosectomy (EA-RAP). Methods:Five specimens were dissected using the limited and extended EA-RAP. Based on the bone removal of the internal acoustic meatus (IAM) and subarcuate fossa, exposure of the MC and ICA were statistically compared. Results:The limited and extended EA-RAP allowed access to the medial and anterior MC (4 mm posterior to the first genu of the cavernous ICA, and 20 mm posterior to foramen rotundum [FR]). The access to the lateral MC varied with distance of 12 and 8 mm medial to the foramen ovale for the limited and extended EA-RAP, respectively.In the extended EA-RAP, the exposure of the ICA was gained by drilling with the 0-degree endoscope (3 mm) versus 45-degree endoscope (9 mm). The working distances from the midpoint of the IAM to the most medial point of the exposed ICA was 24 mm. The most lateral point of the exposed ICA varied between 0- and 45-degree endoscopes with a distance of 21 and 13 mm, respectively. Conclusion:A coronal plane from the posterior genu of the cavernous ICA and a sagittal plane to the common crus of the semicircular canals can define the area of MCF accessed by the EA-RAP. Drilling of the temporal bone should be carefully customized according to the patient and can be aided by endoscopic assistance for direct visualization to minimize the risk of injuries to ICA.

Objective:To report the first steps of a project to automate and optimize scheduling of multidisciplinary consultations for patients with longstanding dizziness utilizing artificial intelligence. Study Design:Retrospective case review. Setting:Quaternary referral center. Methods:A previsit self-report questionnaire was developed to query patients about their complaints of longstanding dizziness. We convened an expert panel of clinicians to review diagnostic outcomes for 98 patients and used a consensus approach to retrospectively determine what would have been the ideal appointments based on the patient's final diagnoses. These results were then compared retrospectively to the actual patient schedules. From these data, a machine learning algorithm was trained and validated to automate the triage process. Results:Compared with the ideal itineraries determined retrospectively with our expert panel, visits scheduled by the triage clinicians showed a mean concordance of 70%, and our machine learning algorithm triage showed a mean concordance of 79%. Conclusion:Manual triage by clinicians for dizzy patients is a time-consuming and costly process. The formulated first-generation automated triage algorithm achieved similar results to clinicians when triaging dizzy patients using data obtained directly from an online previsit questionnaire.

Introduction  Skull base neuroanatomy is traditionally learned through two-dimensional anatomical atlases, which while are of unquestionable value, lack the nuanced association of three-dimensional relationships between fundamental anatomical structures relevant to surgical approaches. Surgically focused step-by-step anatomical dissections can augment trainee learning of complex skull base techniques, particularly multistep and nuanced techniques such as translabyrinthine and transcochlear approaches. Methods  Translabyrinthine and transcochlear approaches were performed on six sides of three formalin-fixed latex-injected specimens. The study objective was the completion and photo documentation of the steps involved in the approach in order to provide a comprehensive, intelligible, and anatomically oriented resource for multilevel trainees. Illustrative case examples were prepared to supplement approach dissections. Results  The translabyrinthine and transcochlear approaches offer unique lateral windows through the temporal bone into the posterior fossa, providing excellent access to pathology at the petrous apex, internal auditory canal, Meckel's cave, and anterolateral brainstem. The transcochlear approach, which is an anterior extension of the translabyrinthine, particularly provides excellent exposure of the prepontine region and clivus. Important surgical considerations include patient position, temporal bone drilling and identification of critical landmarks, dural opening and identification of neurovascular structures, and reconstruction/closure techniques. Conclusion  The translabyrinthine and transcochlear approaches are fundamental techniques for lateral skull base and posterior fossa pathologies. Both approaches are hearing-sacrificing but often require minimal to no cerebellar retraction and deliver unique visualization of prepontine neurovascular structures. This step-by-step approach guide provides a unique practical and high-yield surgically oriented learning resource for neurosurgery and otolaryngology trainees.

OBJECTIVES:Radiation treatment, particularly at a young age, creates theoretical risk for long-term adverse radiation effects, including the development of malignancy. The literature is sparse on radiation-induced vestibular schwannomas (VSs). METHODS:A retrospective review was performed for cases of suspected radiation-induced VS at 2 high-volume centers. Only cases where radiation included coverage of the posterior fossa were included with those diagnosed within 3 years of radiation treatment being excluded. Patient and tumor characteristics were collected. A systematic literature review was also performed for any previously published series on radiation-induced VS. RESULTS:Eight cases of radiation-induced VS were identified with a median follow-up 125 months (range 7-131). The median age at incident radiation was 15 years (range 2-46). The median age at VS diagnosis was 57 years (range 26-83) with median interval from radiation to diagnosis of 51-years (range 15-66). The median tumor size was 6 mm (range 3-21). Two patients underwent surgical resection. Lesions were described as soft and highly vascular, with medium to high adherence to the facial nerve. Five articles with a total of 52 patients were identified, median age at VS diagnosis was 42-years (range 23-73) with a median interval from radiation to diagnosis of 19 years (range 15-23). CONCLUSIONS:The development of VS following radiation exposure appears rare and our understanding of the condition remains incomplete. Further studies are required to determine the best management of these patients and determine whether there is a causative relationship between radiation exposure and the development of VS.

OBJECTIVES:Describe a single institution's cochlear implant outcomes for patients with inner ear schwannomas (IES) in the setting of various tumor management strategies (observation, surgical resection, or stereotactic radiosurgery [SRS]). STUDY DESIGN:Single-institution retrospective review. PATIENTS:Patients diagnosed with isolated, sporadic IES who underwent cochlear implantation (CI). INTERVENTIONS:CI with or without IES treatment. MAIN OUTCOME MEASURES:Speech perception outcomes, tumor status. RESULTS:Twelve patients with IES underwent CI with a median audiologic and radiologic follow-up of 12 months. Six patients underwent complete resection of the tumor at the time of CI, four underwent tumor observation, and two underwent SRS before CI. At 1 year after CI for all patients, the median consonant-nucleus-consonant (CNC) word score was 55% (interquartile range, 44-73%), and the median AzBio sentence in quiet score was 77% (interquartile range, 68-93%). Overall, those with surgical resection performed similarly to those with tumor observation (CNC 58 versus 61%; AzBio in quiet 74 versus 91%, respectively). Patients who underwent tumor resection before implantation had a wider range of speech performance outcomes compared with patients who underwent tumor observation. Two patients had SRS treatment before CI (10 months previous and same-day as CI) with CNC word scores of 6 and 40%, respectively. CONCLUSIONS:Patients with IES who underwent CI demonstrated similar speech performance outcomes (CNC 56% and AzBio 82%), when compared with the general cochlear implant population. Patients who underwent either tumor observation or surgical resection performed well after CI.