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.

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.

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.

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.

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.

Cochlear implantation and auditory brainstem implantation in neurofibromatosis type 2.

OBJECTIVES/HYPOTHESIS: To report a series of patients with neurofibromatosis type 2 (NF2), where each patient underwent both cochlear implantation and auditory brainstem implantation for hearing rehabilitation, and to discuss factors influencing respective implant success. STUDY DESIGN: Retrospective case series. METHODS: Ten NF2 patients with both cochlear implantations and auditory brainstem implantations were retrospectively reviewed. Speech testing for auditory brainstem implants (ABIs) and cochlear implants (CIs) was performed separately. Scores at last follow-up were obtained for Iowa vowels and consonants, Northwestern University Children's Perception of Speech (NU-CHIPS), and City University of New York (CUNY) sentences. RESULTS: Mean age at time of implant was 37 years for cochlear implantation and 40 years for auditory brainstem implantation (P = .790, t test). Nine of 10 patients had a CI and ABI on contralateral sides, and one had both devices on the same side. Mean duration of deafness in the implanted ear was 4.3 years for both cochlear implantation and auditory brainstem implantation (P = .491, t test). Follow-up range was 1 to 28 years. CI performance on NU-CHIPS was 32% to 100%, and sound + lip-reading CUNY was 56% to 100%. Four patients experienced an eventual decline in CI function to unusable levels. ABI performance on NU-CHIPS was 40% to 80%, and sound + lip-reading CUNY was 38% to 94%. There was no notable decline in ABI function over time. CONCLUSIONS: If the cochlear nerve is intact, cochlear implantation can be an effective strategy for hearing rehabilitation in NF2. However, a significant proportion experience a decline in CI performance related to growing vestibular schwannoma or tumor treatment. Auditory brainstem implantation remains the standard option for surgical hearing rehabilitation in NF2, but peak performance is generally lower than that achievable with cochlear implantation. LEVEL OF EVIDENCE: 4. Laryngoscope, 128:2163-2169, 2018.

Translabyrinthine microsurgical resection of small vestibular schwannomas.

OBJECTIVE Translabyrinthine resection is one of a number of treatment options available to patients with vestibular schwannomas. Though this procedure is hearing destructive, the authors have noted excellent clinical outcomes for patients with small tumors. The authors review their experience at a tertiary acoustic neuroma referral center in using the translabyrinthine approach to resect small vestibular schwannomas. All operations were performed by a surgical team consisting of a single neurosurgeon and 1 of 7 neurotologists. METHODS Data from a prospectively maintained clinical database were extracted and reviewed. Consecutive patients with a preoperative diagnosis of vestibular schwannoma that had less than 1 cm of extension into the cerebellopontine angle, operated on between 2008 and 2013, were included. Patents with neurofibromatosis Type 2, previous treatment, or preexisting facial weakness were excluded. In total, 107 patients were identified, 74.7% of whom had poor hearing preoperatively. RESULTS Pathologically, 6.5% of patients were found to have a tumor other than vestibular schwannoma. Excluding two malignancies, the tumor control rates were 98.7%, as defined by absence of radiographic disease, and 99.0%, as defined by no need for additional treatment. Facial nerve outcome was normal (House-Brackmann Grade I) in 97.2% of patients and good (House-Brackmann Grade I-II) in 99.1%. Complications were cerebrospinal fluid leak (4.7%) and sigmoid sinus thrombosis (0.9%), none of which led to long-term sequelae. CONCLUSIONS Translabyrinthine resection of small vestibular schwannomas provides excellent results in terms of complication avoidance, tumor control, and facial nerve outcomes. This is a hearing-destructive operation that is advocated for selected patients.

Cochlear Nerve Aplasia and Hypoplasia: Predictors of Cochlear Implant Success.

Objective To identify factors predicting performance outcomes following cochlear implantation in patients with cochlear nerve aplasia or hypoplasia. Data Sources Individual patient data extracted from published case series and reports. Review Methods The MEDLINE database, Cochrane Library, Embase, Web of Science, and Google Scholar were queried for "cochlear implant" in conjunction with "aplasia" or "hypoplasia" between 1985 and 2015. Eighteen studies were included describing 97 subjects with individual postimplant auditory data. Postimplant performance was categorized as follows: level 1, nonstimulation/minimal detection; level 2, improved detection; level 3, closed-set speech perception; or level 4, open-set speech perception. The subjects achieving speech perception (levels 3 and 4) were descriptively compared with those who did not. Results Subjects with a hypoplastic cochlear nerve on magnetic resonance imaging had higher reported rates of achieving speech perception than those with an aplastic nerve. Subjects with syndromic medical comorbidities had higher reported rates of nonstimulation than nonsyndromic subjects. The data showed that some children with an aplastic cochlear nerve or those with partial electrode insertion could obtain levels of speech discrimination. Reporting of patient characteristics and auditory outcomes was extremely variable across studies. Conclusion As previously shown, cochlear implant in patients with cochlear nerve aplasia or hypoplasia can provide meaningful hearing for select patients. The current study suggests that presence of a cochlear nerve on magnetic resonance imaging and lack of comorbid medical syndrome are associated with better auditory outcomes in such patients. Future efforts to report individual data in a consistent manner may allow better determination of predictive factors.

Implementation of Image-Guided Cochlear Implant Programming at a Distant Site.

Our objective was to prospectively evaluate implementation of a new cochlear implant (CI) mapping technique, image-guided cochlear implant programming (IGCIP), at a site distant to the site of development. IGCIP consists of identifying the geometric relationship between CI electrodes and the modiolus and deactivating electrodes that interfere with neighboring electrodes. IGCIP maps for 17 ears of 15 adult CI patients were developed at a central image-processing center, Vanderbilt, and implemented at a distant tertiary care center, House Ear Institute. Before IGCIP and again 4 weeks after, qualitative and quantitative measures were made. While there were no statistically significant groupwise differences detected between baseline and IGCIP qualitative or quantitative measures, 11 of the 17 (64.7%) elected to keep the IGCIP map. Computed tomography (CT) image quality appears to be crucial for successful IGCIP, with 100% of those with high-resolution CT scans keeping their maps compared to 53.8% without.

Initial Results of a Safety and Feasibility Study of Auditory Brainstem Implantation in Congenitally Deaf Children.

OBJECTIVE: To determine the safety and feasibility of the auditory brainstem implant (ABI) in congenitally deaf children with cochlear aplasia and/or cochlear nerve deficiency. STUDY DESIGN: Phase I feasibility clinical trial of surgery in 10 children, ages 2 to 5 years, over a 3-year period. SETTING: Tertiary children's hospital and university-based pediatric speech/language/hearing center. INTERVENTION(S): ABI implantation and postsurgical programming. MAIN OUTCOME MEASURE(S): The primary outcome measure is the number and type of adverse events during ABI surgery and postsurgical follow-up, including behavioral mapping of the device. The secondary outcome measure is access to and early integration of sound. RESULTS: To date, nine children are enrolled. Five children have successfully undergone ABI surgery and postoperative behavioral programming. Three children were screen failures, and one child is currently undergoing candidacy evaluation. Expected adverse events have been documented in three of the five children who received the ABI. One child experienced a cerebral spinal fluid leak, which resolved with lumbar drainage. One child demonstrated vestibular side effects during device programming, which resolved by deactivating one electrode. One child experienced postoperative vomiting resulting in an abdominal radiograph. Four children have completed their 1-year follow-up and have speech detection thresholds of 30 to 35 dB HL. Scores on the IT-MAIS/MAIS range from 8 to 31 (out of a total of 40), and the children are demonstrating some ability to discriminate between closed-sets words that differ by number of syllables (pattern perception). CONCLUSION: ABI surgery and device activation seem to be safe and feasible in this preliminary cohort.

Auditory brainstem implant program development.

OBJECTIVE: Auditory brainstem implants (ABIs), which have previously been used to restore auditory perception to deaf patients with neurofibromatosis type 2 (NF2), are now being utilized in other situations, including treatment of congenitally deaf children with cochlear malformations or cochlear nerve deficiencies. Concurrent with this expansion of indications, the number of centers placing and expressing interest in placing ABIs has proliferated. Because ABI placement involves posterior fossa craniotomy in order to access the site of implantation on the cochlear nucleus complex of the brainstem and is not without significant risk, we aim to highlight issues important in developing and maintaining successful ABI programs that would be in the best interests of patients. DATA SOURCES: Especially with pediatric patients, the ultimate benefits of implantation will be known only after years of growth and development. These benefits have yet to be fully elucidated and continue to be an area of controversy. The limited number of publications in this area were reviewed. REVIEW METHODS: Review of the current literature was performed. RESULTS: Disease processes, risk/benefit analyses, degrees of evidence, and U.S. Food and Drug Administration approvals differ among various categories of patients in whom auditory brainstem implantation could be considered for use. CONCLUSION: We suggest sets of criteria necessary for the development of successful and sustaining ABI programs, including programs for NF2 patients, postlingually deafened adult nonneurofibromatosis type 2 patients, and congenitally deaf pediatric patients. Laryngoscope, 127:1909-1915, 2017.

Hearing Outcomes after Middle Fossa or Retrosigmoid Craniotomy for Vestibular Schwannoma Tumors.

OBJECTIVE: The objective of this study was to evaluate hearing outcomes following middle fossa (MF) or retrosigmoid (RS) craniotomy for vestibular schwannoma (VS) removal with the goal of hearing preservation. DESIGN: This is a retrospective series. SETTING: This study was set at a skull base referral center. PARTICIPANTS: In this study, 377 sporadic VS patients underwent primary microsurgery for VS from 2002 to 2012 using the MF (n = 305) or RS (n = 72) approaches. MAIN OUTCOME MEASURES: The main outcome measures were change in pure-tone average (PTA) and word recognition score from pre- to postoperative and surgical complications. RESULTS: Preoperative hearing did not differ between approaches. Tumors were larger in the RS group (mean = 1.78 cm) than the MF group (mean = 0.97 cm) (p ≤ 0.001). Mean times to last audiometric follow-up were MF 1.0 year and RS 0.7 years. Mean decline in hearing from preoperative to last follow-up was greater in the RS group (55.5 dB in PTA and 45.6% in discrimination) than the MF group (38.9 dB and 31.7%) (p ≤ 0.011 and 0.033, respectively). The effect of surgical approach on hearing outcome remained after controlling for tumor size. Facial nerve outcomes and cerebrospinal fluid leak rates were not significantly different. CONCLUSION: Loss of hearing was greater with the RS approach than the MF approach, even when accounting for differences in tumor size. Postoperative facial nerve function and other complications did not differ between approaches.