An atlas of gross and histologic lesions and immunohistochemical immunoreactivity during the temporal progression of aerosolized Lassa virus induced hemorrhagic fever in cynomolgus macaques.

Lassa virus (LASV) causes an acute multisystemic hemorrhagic fever in humans known as Lassa fever, which is endemic in several African countries. This manuscript focuses on the progression of disease in cynomolgus macaques challenged with aerosolized LASV and serially sampled for the development and progression of gross and histopathologic lesions. Gross lesions were first noted in tissues on day 6 and persisted throughout day 12. Viremia and histologic lesions were first noted on day 6 commencing with the pulmonary system and hemolymphatic system and progressing at later time points to include all systems. Immunoreactivity to LASV antigen was first observed in the lungs of one macaque on day 3 and appeared localized to macrophages with an increase at later time points to include immunoreactivity in all organ systems. Additionally, this manuscript will serve as a detailed atlas of histopathologic lesions and disease progression for comparison to other animal models of aerosolized Arenaviral disease.

Impact of the collaborative care model on medical spending.

OBJECTIVES: The collaborative care model integrates mental health care into primary care. In 2017, CMS created new billing codes to reimburse collaborative care. We measured the impact of a program supported by these codes on medical spending. STUDY DESIGN: Quasi-experimental. METHODS: We identified a commercially insured and managed Medicare sample of 825 patients who received collaborative care services in 8 primary care practices. We used propensity score matching to match treated patients to potential controls, resulting in 569 patients per group. We performed a difference-in-differences regression analysis to evaluate the impact of collaborative care on total medical spending, including medical, psychiatric, and pharmaceutical claims. RESULTS: Collaborative care patients' mean total medical cost began to fall after a patient's third month in the program and fell below the mean cost of control patients at month 7. Difference-in-differences regressions indicate a nonsignificant savings in total medical cost of $29.35 per member per month for patients in collaborative care compared with matched controls (95% CI, -$226.52 to $167.82). Treated members incurred $34.11 (95% CI, $31.95-$36.27) higher primary care costs that were directly attributed to collaborative care, $19.91 (95% CI, $4.84-$34.98) higher costs for other mental or behavioral health care, and a nonsignificant reduction of $91.34 (95% CI, -$319.32 to $136.63) in inpatient costs. CONCLUSIONS: Modest spending on collaborative care services to address the behavioral health needs of patients did not increase overall health care costs. This is the first economic study of a collaborative care program supported by the new billing codes.

Lassa antiviral LHF-535 protects guinea pigs from lethal challenge.

LHF-535 is a small molecule antiviral currently in development for the treatment of Lassa fever, a zoonotic disease endemic in West Africa that generates significant morbidity and mortality. Current treatment options are inadequate, and there are no approved therapeutics or vaccines for Lassa fever. LHF-535 was evaluated in a lethal guinea pig model of Lassa pathogenesis, using once-daily administration of a fixed dose (50 mg/kg/day) initiating either 1 or 3 days after inoculation with a lethal dose of Lassa virus. LHF-535 reduced viremia and clinical signs and protected all animals from lethality. A subset of surviving animals was rechallenged four months later with a second lethal challenge of Lassa virus and were found to be protected from disease. LHF-535 pharmacokinetics at the protective dose in guinea pigs showed plasma concentrations well within the range observed in clinical trials in healthy volunteers, supporting the continued development of LHF-535 as a Lassa therapeutic.

Predictors of hearing functional outcome following surgery for cerebellopontine angle meningioma.

OBJECTIVE: Cerebellopontine angle (CPA) meningiomas can affect hearing function and require expeditious treatment to prevent permanent hearing loss. The authors sought to determine the factors associated with functional hearing outcome in CPA meningioma patients treated with surgery and/or radiation therapy in the form of either stereotactic radiosurgery or stereotactic radiation therapy. METHODS: Consecutive patients with CPA meningiomas who had presented at our hospital from 2008 to 2018 were identified through retrospective chart review. Hearing function (as defined by pure tone average (PTA) and speech discrimination score (SDS) on Audiogram) was assessed before and after surgery for CPA meningioma. Audiograms with PTA > 50 dB and SDS < 69% were defined as poor hearing functional outcome. Multivariable Cox Proportional Hazards Regression Model was used to assess the associations between pre-operative hearing functional assessment and post-operative hearing functional outcomes. RESULTS: The study cohort included 31 patients (80.6% females, with a mean age of 61.3 ± 15.2 years) with a median clinical follow-up of 5 months (range: 1 week-98 months). The mean pre-operative PTA and SDS were 23.8 ± 11.2 dB and 64.4 ± 22.2% respectively. At the last visit, there was significant hearing recovery, with an improvement of 29.7 ± 18.0 dB (p < 0.001) and 87.6 ± 17.8% (p < 0.001) in PTA and SDS respectively. After adjusting for age, gender, tumor volume, location, and tumor classification, Multivariable Cox Proportional Hazards Regression Model was conducted which revealed that patients undergoing surgery through retro sigmoid approach [Hazards Ratio (HR): 32.1, 95% Confidence Interval (CI): 2.11-491.0, p = 0.01] and gross total resection (GTR) (HR: 2.99, 95% CI: 1.09-9.32, p = 0.05) had significantly higher risk of poor hearing functional outcome compared to petrosal approach and near/subtotal resection. Moreover, patients with poor preoperative hearing had 85% higher chance of poor hearing functional outcome postoperatively (HR: 0.15, 95%CI: 0.03-0.59, p = 0.007). CONCLUSION: Postoperative improvement in hearing is a reasonable expectation following surgery for CPA meningioma. Preoperative hearing, surgical approach and extent of surgical resection are predictive factors of postoperative hearing function outcome and can therefore aid in identification of patients at higher risk of hearing loss.

Role of cerebral digital subtraction angiography in the evaluation of pulse synchronous tinnitus.

OBJECTIVES: The aim of this study was to evaluate the value of digital subtraction angiography (DSA) in the diagnostic evaluation of a highly selected patient population presenting with pulse-synchronous tinnitus (PST). METHODS: We retrospectively reviewed the charts of all patients referred for evaluation of possible vascular etiology of pulsatile tinnitus. Patients were evaluated with regards to presenting signs, comorbidities, non-invasive imaging results, angiographic findings and outcomes. RESULTS: Fifteen patients underwent cerebral DSA. Dural arteriovenous fistula (dAVF) was identified in six patients, and five patients had other significant vascular pathology identified on DSA. Seven patients with 'negative' non-invasive imaging were found to have significant pathology on DSA. CONCLUSIONS: Catheter angiography may have a significant yield in appropriately selected patients presenting with pulse synchronous tinnitus.

MRI evaluation of axillary neurovascular bundle: Implications for minimally invasive proximal humerus fracture fixation.

BACKGROUND: Percutaneous fixation of proximal humeral fractures places the axillary nerve and posterior humeral circumflex artery at risk for injury. Safe operative zones for the axillary nerve are described based on external measurements from anatomic landmarks, but no study to date has incorporated advanced imaging to help guide surgical procedures in the region of the axillary neurovascular bundle (ANVB). We sought to define the location and trajectory of the ANVB in relation to osseous landmarks using magnetic resonance imaging (MRI) measurements. METHODS: Retrospective review of 750 consecutive MRI studies was performed with 55 imaging studies meeting inclusion criteria for patient positioning, image alignment, and quality. Five measurements were performed including the distance from mid-lateral acromion to lateral ANVB, mid-lateral acromion to medial ANVB, greater tuberosity to lateral ANVB, vertical distance between inferior anatomic neck and lateral ANVB, and angle the ANVB crosses the humerus. Height, gender, and age were recorded. Analysis was performed using ANOVA and Pearson correlation tests. RESULTS: The lateral ANVB was below the inferior articular margin of the humeral head by an average of 12.9 ± 3.9 mm and within a 22 mm window. It was an average of 57.4 ± 5.1 mm from the lateral mid-acromion, and 34.7 ± 4.3 mm below the greater tuberosity. The angle formed by the ANVB crossing the humerus averaged 19.5 ± 3.9 degrees upward from medial to lateral. Height and gender directly impacted measurements. CONCLUSIONS: The use of the inferior humeral head articular margin provides a radiographic landmark to aid intraoperative lateral ANVB assessment which may be helpful during percutaneous fracture fixation.

Bilateral Auditory Brainstem Implants in Patients With Neurofibromatosis 2.

OBJECTIVE: To discuss indications for bilateral auditory brainstem implants (ABIs), compare audiometric outcomes of unilateral vs bilateral ABIs, and determine if patients have improved outcomes with addition of a second-side implant. STUDY DESIGN: Retrospective review of 24 patients with neurofibromatosis 2 (NF2) who underwent sequential placement of ABIs from 1989 to 2019. SETTING: Tertiary referral center. METHODS: Charts were reviewed for indication for second-side surgery, use of implants, and audiometric outcomes. Implants placed in the past 30 years were included in the study. Northwestern University Children's Perception of Speech (NU-CHIPS) and/or City University of New York (CUNY) sentence scores were compared in unilateral and bilateral conditions. RESULTS: Indications for a second-side implant included first-side implants with severe nonauditory symptoms (11), marginal audiometric results (9), outdated technology (2), or deterioration of first side (2). Seven patients are bilateral users and 1 patient discontinued bilateral use after a year due to no significant improvement over unilateral use. One patient with initial bilateral use was lost to follow-up. Thirteen patients are unilateral users due to nonaudiometric side effects or poor audiometric outcomes with the first side. Two patients are complete nonusers. Seventy-five percent had improved audiometric outcomes after the second-side implant, and 20% had stable findings. CONCLUSIONS: Second-side ABIs should be consider in patients with poor performance from a first-side implant. Most patients demonstrate subjective improvement with the second ABI. More research is needed for better objective assessments of improvements.

Meniere's disease: Medical management, rationale for vestibular preservation and suggested protocol in medical failure.

Meniere's disease is a peripheral audiovestibular disorder characterized by vertigo, hearing loss, tinnitus, and aural fullness. Management of these symptoms includes medical and surgical treatment. Many patients with Meniere's disease can be managed using nonablative therapy, such as intratympanic steroids and endolymphatic shunt surgery, prior to ablative techniques such as intratympanic gentamicin. Recognition of concurrent migraine symptoms may aid in medical therapy and also underscore the importance of preserving vestibular function where possible. The goal of this review is to explain the importance of nonablative therapy options and discuss treatment protocols after medical failure.

A wider influence: The impact of formal humanitarian otology training on otology-neurotology fellows.

OBJECTIVES: Long-term commitment to humanitarian surgical outreach requires the opportunity, resources, and time to participate, but perhaps more importantly, it requires a preceding successful outreach experience. The Accreditation Council for Graduate Medical Education (ACGME) expects physician trainees to achieve six Core Competencies: patient care, medical knowledge, interpersonal and communication skills, professionalism, practice-based learning and improvement, and systems-based practice. This study investigates the challenges and benefits of a supervised humanitarian experience with a focus on ACGME Core Competencies, future global outreach, and commitment to education. STUDY DESIGN: Physician survey and program curriculum review. METHODS: Participants in a supervised humanitarian educational experience at a single Otology-Neurotology Fellowship Program between the years of 2006 and 2019 were surveyed. Barriers to participation, challenges, benefits, prior and ongoing humanitarian efforts, and education endeavors were discussed. RESULTS: Challenges including time away from fellowship, logistical difficulties with Resident Review Committee recognition of operative case volume, and civil unrest were encountered. Benefits within all six ACGME Core Competencies were achieved. International collaboration with local providers, patients, and families with diverse socioeconomic and cultural backgrounds allowed bidirectional education while striving for safe, innovative solutions in resource-poor environments. CONCLUSIONS: A supervised education experience with a sustainable humanitarian otology program offers Neurotology fellows an early positive experience near the end of their formal training. ACGME Professionalism goals were achieved through building collegial relationships with local otolaryngologists and staff, while fostering a sense of responsibility to assist those in developing nations. Participation correlated with an ongoing commitment to humanitarian efforts and education post-fellowship. LEVEL OF EVIDENCE: 4.

The effect of increased cost-sharing on low-value service use.

We examine the effect of a value-based insurance design (VBID) program implemented at a large public employer in the state of Oregon. The program substantially increased cost-sharing for several healthcare services likely to be of low value for most patients: diagnostic services (e.g., imaging services) and surgeries (e.g., spinal surgeries for pain). Using a difference-in-differences design coupled with granular, administrative health insurance claims data over the period 2008-2012, we estimate the change in low-value service use among beneficiaries before and after program implementation relative to a comparison group not exposed to the VBID. Our findings suggest that the VBID significantly reduced the use of targeted services, with an implied elasticity of demand of -0.22. We find no evidence that the VBID led to substitution to non-targeted services or increased overall healthcare costs. However, we also observe no evidence that the program led to cost-savings.

Simultaneous Cochlear and Auditory Brainstem Implantation in Children With Severe Inner Ear Malformations: Initial Surgical and Audiological Results.

OBJECTIVE: To report the initial surgical and audiological outcomes of three pediatric patients with severe inner ear malformations who were simultaneously implanted with cochlear and brainstem implants in the same surgical session. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Three pediatric patients with severe inner ear malformations between ages of 1.9 to 2.5 years, who were simultaneously implanted with cochlear implant in one ear and auditory brainstem implant in the other ear. INTERVENTION(S): Simultaneous application of cochlar implant in one ear, auditory brainstem implant in the other ear. MAIN OUTCOME MEASURES: Free field thresholds with cochlear and brainstem implants. Surgical issues are also discussed. RESULTS: The study is descriptive in nature. Free field thresholds with each device alone and together showed good progress. One of the patients had slower progress possibly due to comorbid CHARGE syndrome. CONCLUSIONS: The results showed good progress in terms of audition with both devices. Simultaneous cochlear and brainstem application serves as a remedy for pediatric patients who are candidates for cochlear implant on one side and brainstem implant on the other side. With this simultaneous application precious time for auditory development is not lost.

Early Clinical and Economic Outcomes of Prophylactic and Acute Pathologic Fracture Treatment.

INTRODUCTION: Pathologic fractures often contribute to adverse events in metastatic bone disease, and prophylactic fixation offers to mitigate their effects. This study aims to analyze patient selection, complications, and in-hospital costs that are associated with prophylactic fixation compared with traditional acute fixation after completed fracture. MATERIALS AND METHODS: The Nationwide Inpatient Sample database was queried from 2002 to 2014 for patients with major extremity pathologic fractures. Patients were divided by fixation technique (prophylactic or acute) and fracture location (upper or lower extremity). Patient demographics, comorbidities, complications, hospitalization length, and hospital charges were compared between cohorts. Preoperative variables were analyzed for potential confounding, and χ2 tests and Student's t tests were used to compare fixation techniques. RESULTS: Cumulatively, 43,920 patients were identified, with 14,318 and 28,602 undergoing prophylactic and acute fixation, respectively. Lower extremity fractures occurred in 33,582 patients, and 10,333 patients had upper extremity fractures. A higher proportion of prophylactic fixation patients were white ( P = .043), male ( P = .046), age 74 years or younger ( P < .001), and privately insured ( P < .001), with decreased prevalence of obesity ( P = .003) and/or preoperative renal disease ( P = .008). Prophylactic fixation was also associated with decreased peri- and postoperative blood transfusions ( P < .001), anemia ( P < .001), acute renal failure ( P = .010), and in-hospital mortality ( P = .031). Finally, prophylactic fixation had decreased total charges (-$3,405; P = .001), hospitalization length ( P = .004), and extended length of stay (greater than 75th percentile; P = .012). CONCLUSION: Prophylactic fixation of impending pathologic fractures is associated with decreased complications, hospitalization length, and total charges, and should be considered in appropriate patients.

Benefits of Cochlear Implantation for Single-Sided Deafness: Data From the House Clinic-University of Southern California-University of California, Los Angeles Clinical Trial.

OBJECTIVES: Cochlear implants (CIs) have been shown to benefit patients with single-sided deafness (SSD) in terms of tinnitus reduction, localization, speech understanding, and quality of life (QoL). While previous studies have shown cochlear implantation may benefit SSD patients, it is unclear which point of comparison is most relevant: baseline performance before implantation versus performance with normal-hearing (NH) ear after implantation. In this study, CI outcomes were assessed in SSD patients before and up to 6 mo postactivation. Benefits of cochlear implantation were assessed relative to binaural performance before implantation or relative to performance with the NH ear alone after implantation. DESIGN: Here, we report data for 10 patients who completed a longitudinal, prospective, Food and Drug Administration-approved study of cochlear implantation for SSD patients. All subjects had severe to profound unilateral hearing loss in one ear and normal hearing in the other ear. All patients were implanted with the MED-EL CONCERTO Flex 28 device. Speech understanding in quiet and in noise, localization, and tinnitus severity (with the CI on or off) were measured before implantation (baseline) and at 1, 3, 6 mo postactivation of the CI processor. Performance was measured with both ears (binaural), the CI ear alone, and the NH ear alone (the CI ear was plugged and muffed). Tinnitus severity, dizziness severity, and QoL were measured using questionnaires administered before implantation and 6 mo postactivation. RESULTS: Significant CI benefits were observed for tinnitus severity, localization, speech understanding, and QoL. The degree and time course of CI benefit depended on the outcome measure and the reference point. Relative to binaural baseline performance, significant and immediate (1 mo postactivation) CI benefits were observed for tinnitus severity and speech performance in noise, but localization did not significantly improve until 6 mo postactivation; questionnaire data showed significant improvement in QoL 6 mo postactivation. Relative to NH-only performance after implantation, significant and immediate benefits were observed for tinnitus severity and localization; binaural speech understanding in noise did not significantly improve during the 6-mo study period, due to variability in NH-only performance. There were no correlations between behavioral and questionnaire data, except between tinnitus visual analog scale scores at 6 mo postactivation and Tinnitus Functional Index scores at 6 mo postactivation. CONCLUSIONS: The present behavioral and subjective data suggest that SSD patients greatly benefit from cochlear implantation. However, to fully understand the degree and time course of CI benefit, the outcome measure and point of comparison should be considered. From a clinical perspective, binaural baseline performance is a relevant point of comparison. The lack of correlation between behavioral and questionnaire data suggest that represent independent measures of CI benefit for SSD patients.

Immune-Mediated Systemic Vasculitis as the Proposed Cause of Sudden-Onset Sensorineural Hearing Loss following Lassa Virus Exposure in Cynomolgus Macaques.

Lassa virus (LASV) causes a severe, often fatal hemorrhagic disease in regions in Africa where the disease is endemic, and approximately 30% of patients develop sudden-onset sensorineural hearing loss after recovering from acute disease. The causal mechanism of hearing loss in LASV-infected patients remains elusive. Here, we report findings after closely examining the chronic disease experienced by surviving macaques assigned to LASV exposure control groups in two different studies. All nonhuman primates (NHPs) developed typical signs and symptoms of Lassa fever, and seven succumbed during the acute phase of disease. Three NHPs survived beyond the acute phase and became chronically ill but survived to the study endpoint, 45 days postexposure. All three of these survivors displayed continuous disease symptoms, and apparent hearing loss was observed using daily subjective measurements, including response to auditory stimulation and tuning fork tests. Objective measurements of profound unilateral or bilateral sensorineural hearing loss were confirmed for two of the survivors by brainstem auditory evoked response (BAER) analysis. Histologic examination of inner ear structures and other tissues revealed the presence of severe vascular lesions consistent with systemic vasculitides. These systemic immune-mediated vascular disorders have been associated with sudden hearing loss. Other vascular-specific damage was also observed to be present in many of the sampled tissues, and we were able to identify persistent virus in the perivascular tissues in the brain tissue of survivors. Serological analyses of two of the three survivors revealed the presence of autoimmune disease markers. Our findings point toward an immune-mediated etiology for Lassa fever-associated sudden-onset hearing loss and lay the foundation for developing potential therapies to prevent and/or cure Lassa fever-associated sudden-onset hearing loss.IMPORTANCE Lassa virus is one of the most common causes of viral hemorrhagic fever. A frequent, but as yet unexplained, consequence of infection with Lassa virus is acute, sudden-onset sensorineural hearing loss in one or both ears. Deafness is observed in approximately 30% of surviving Lassa fever patients, an attack rate that is approximately 300% higher than mumps virus infection, which was previously thought to be the most common cause of virus-induced deafness. Here, we provide evidence from Lassa virus-infected cynomolgus macaques implicating an immune-mediated vasculitis syndrome underlying the pathology of Lassa fever-associated deafness. These findings could change the way human Lassa fever patients are medically managed in order to prevent deafness by including diagnostic monitoring of human survivors for onset of vasculitides via available imaging methods and/or other diagnostic markers of immune-mediated vascular disease.

Revision Surgery for Vestibular Schwannomas.

Objectives To describe clinical outcomes of patients undergoing revision surgery for vestibular schwannomas. Design Retrospective case series. Setting Tertiary private neurotologic practice. Participants Patients who underwent revision surgeries for recurrent/residual vestibular schwannomas between 1985 and 2015. Main Outcome Measures Degree of resection, facial nerve function, cerebrospinal fluid (CSF) leak. Results A total of 234 patients underwent 250 revision surgeries for recurrent/residual vestibular schwannomas. Of these, 86 carried a diagnosis of neurofibromatosis type 2 (NF2). The mean number of prior surgeries was 1.26, and 197 (85%) prior surgeries had been performed elsewhere. The average age at surgery was 43. The most common approach employed at the time of revision surgery was translabyrinthine (87%), followed by transcochlear (6%), middle fossa (5%), and retrosigmoid (2%). Gross total resection was achieved in 212 revision surgeries (85%). Preoperative House-Brackmann facial nerve function was similar in non-NF2 and NF2 groups (mean: 2.7). Mean postoperative facial nerve function at last follow-up was 3.8 in the non-NF2 group and 3.9 in the NF2 group. History of radiation and the extent of resection were not associated with differences in facial nerve function preoperatively or postoperatively. CSF leaks occurred after 21 surgeries (8%), and six (2%) patients required reoperation. Conclusions This is the largest series of revision surgery for vestibular schwannomas to date. Our preferred approach is the translabyrinthine craniotomy, which can be readily modified to include the transcochlear approach for improved access. CSF leak rate slightly exceeds that of primary surgery, and gross total resection is achievable in the vast majority of patients.

Growth hormone-secreting pituitary macroadenoma presenting concurrently with non-Hodgkin's lymphoma and responding to doxorubicin treatment: case report and review of the literature.

We present a case report of a patient who presented with nausea and vomiting, as well as acromegalic features. Following testing, a pituitary adenoma and mediastinal non-Hodgkin's lymphoma were diagnosed. Following two cycles of R-CHOP chemotherapy, imaging showed significant decrease in size of the sellar tumor. Following resection of tumor, both frozen and permanent section revealed only necrotic material. Further research into the potential utility of doxorubicin for the treatment of recurrent or refractory pituitary adenomas may be warranted.

Pearls for the middle fossa approach in acoustic neuroma surgery.

PURPOSE OF REVIEW: To discuss the use of the middle fossa craniotomy for resection of vestibular schwannomas; to present pearls of and modifications to the approach. RECENT FINDINGS: The middle fossa craniotomy allows for hearing preservation in the resection of intracanalicular vestibular schwannomas. Over recent years, the approach has been modified to address larger tumors with a limited cerebellopontine angle component. Positive identification of the superior semicircular canal allows for rapid exposure of the internal auditory canal (IAC). Removal of cerebrospinal fluid from the posterior fossa during exposure of the IAC allows for removal of the middle fossa retractor; reinforced silicone sheeting is used to protect the middle fossa dura during further drilling. The use of the endoscope has allowed for more complete dissection at the fundus of the IAC, including tumors lateral to the transverse crest. SUMMARY: Technical modifications to the middle fossa craniotomy have allowed for a shorter duration of temporal lobe retraction intraoperatively and more complete resection of tumors with fundal involvement.

Early Communication Development of Children with Auditory Brainstem Implants.

The auditory brainstem implant (ABI) is an auditory sensory device that is surgically placed on the cochlear nucleus of the brainstem for individuals who are deaf but unable to benefit from a cochlear implant (CI) due to anatomical abnormalities of the cochlea and/or eighth nerve, specific disease processes, or temporal bone fractures. In the United States, the Food and Drug Administration has authorized a Phase I clinical trial to determine safety and feasibility of the ABI in up to 10 eligible young children who are deaf and either derived no benefit from the CI or were anatomically unable to receive a CI. In this paper, we describe the study protocol and the children who have enrolled in the study thus far. In addition, we report the scores on speech perception, speech production, and language (spoken and signed) for five children with 1-3 years of assessment post-ABI activation. To date, the results indicate that spoken communication skills are slow to develop and that visual communication remains essential for post-ABI intervention.

Assessing the Benefit-Risk Profile for Pediatric Implantable Auditory Prostheses.

BACKGROUND/AIMS: Children with congenital cochleovestibular abnormalities associated with profound hearing loss have few treatment options if cochlear implantation does not yield benefit. An alternative is the auditory brainstem implant (ABI). Regulatory authority device approvals currently include a structured benefit-risk assessment. Such an assessment, for regulatory purposes or to guide clinical decision making, has not been published, to our knowledge, for the ABI and may lead to the design of a research program that incorporates regulatory authority, family, and professional input. METHODS: Much structured benefit-risk research has been conducted in the context of drug trials; here we apply this approach to device studies. A qualitative framework organized benefit (speech recognition, parent self-report measures) and risk (surgery- and device-related) information to guide the selection of candidates thought to have potential benefit from ABI. RESULTS: Children with cochleovestibular anatomical abnormalities are challenging for appropriate assessment of candidacy for a cochlear implant or an ABI. While the research is still preliminary, children with an ABI appear to slowly obtain benefit over time. A team of professionals, including audiological, occupational, and educational therapy, affords maximum opportunity for benefit. CONCLUSIONS: Pediatric patients who have abnormal anatomy and are candidates for an implantable auditory prosthetic require an individualized, multisystems review. The qualitative benefit-risk assessment used here to characterize the condition, the medical need, potential benefits, risks, and risk management strategies has revealed the complex factors involved. After implantation, continued team support for the family during extensive postimplant therapy is needed to develop maximum auditory skill benefit.

Endoscope-assisted middle fossa craniotomy for resection of inferior vestibular nerve schwannoma extending lateral to transverse crest.

OBJECTIVE The authors describe their results using an endoscope as an adjunct to microsurgical resection of inferior vestibular schwannomas (VSs) with extension into the fundus of the internal auditory canal below the transverse crest. METHODS All patients who had undergone middle fossa craniotomy for VSs performed by the senior author between September 2014 and August 2016 were prospectively enrolled in accordance with IRB policies, and the charts of patients undergoing surgery for inferior vestibular nerve tumors, as determined either on preoperative imaging or as intraoperative findings, were retrospectively reviewed. Age prior to surgery, side of surgery, tumor size, preoperative and postoperative pure-tone average, and speech discrimination scores were recorded. The presence of early and late facial paralysis, nerve of tumor origin, and extent of resection were also recorded. RESULTS Six patients (all women; age range 40-65 years, mean age 57 years) met these criteria during the study period. Five of the 6 patients underwent gross-total resection; 1 patient underwent a near-total resection because of a small amount of tumor that adhered to the facial nerve. Gross-total resection was facilitated using the operative endoscope in 2 patients (33%) who were found to have additional tumor visible only through the endoscope. All patients had a House-Brackmann facial nerve grade of II or better in the immediate postoperative period. Serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery class A or B) was preserved in 3 of the 6 patients. CONCLUSIONS Endoscope-assisted middle fossa craniotomy for resection of inferior vestibular nerve schwannomas with extension beyond the transverse crest is safe, and hearing preservation is feasible.