Multi-frequency Electrocochleography Measurements can be Used to Monitor and Optimize Electrode Placement During Cochlear Implant Surgery Objective: To report the use of multi-frequency intra-cochlear electrocochleography (ECOG) in monitoring and optimizing electrode placement during cochlear implant surgery. An acoustic pure tone complex comprising of 250, 500, 1000, and 2000 Hz was used to elicit ECOG, or more specifically cochlear microphonics (CMs), responses from various locations in the cochlea. The most apical cochlear implant electrode was used as the recording electrode. Study Design: Clinical capsule report. Setting: Tertiary academic referral center. Results: ECOG measurements were performed during cochlear implant surgery in an adult patient with significant residual acoustic hearing. The 500, 1000, and 2000 Hz CM tracings from the most apical electrode showed an amplitude peak at three different instances during the early phase of cochlear implant electrode insertion. These results are consistent with the tonotopic organization of the cochlea. During final electrode placement a slight advancement of the electrode array resulted in a correlated decrease in 250, 500, and/or 1000 Hz CM amplitude. The electrode array was retracted and repositioned which resulted in a recovery of CM amplitude. Intraoperative CM thresholds revealed a correlation of r = 0.87 with preoperative audiometric thresholds. Conclusion: We present a report on simultaneous multi-frequency ECOG monitoring during cochlear implant surgery. Multi-frequency ECOG can be used to differentiate between electrode trauma and the advancement of the apical electrode beyond the CM source in the cochlea. Address correspondence and reprint requests to Aniket A. Saoji, Ph.D., Department of Otolaryngology–Head and Neck Surgery, Mayo Clinic, 200 1st St SW, Rochester, MN 55905; E-mail: saoji.aniket@mayo.edu A. A. S. is a consultant for Advanced Bionics. Originating Institution: Department of Otolaryngology–Head and Neck Surgery, Mayo Clinic School of Medicine, Rochester, MN 55905. The research hardware and software used in this study was provided by Advanced Bionics (a cochlear implant manufacturer). No funding or other support was required for this study. This study was approved by the Mayo Clinic Institutional Review Board (IRB# 18-008396). C.L.W.D. is a consultant for Advanced Bionics, Cochlear Corporation and Envoy Medical. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

The Role of Body Mass Index on Hearing Outcomes After Stapes Surgery Background: Increased body mass index (BMI) has been associated with postoperative complications in multiple surgical specialties. In otologic surgery involving the stapes, where appropriate patient positioning and surgical dexterity are necessary for proper prosthesis placement, patients with higher BMI could make surgery more challenging. The purpose of this study is to evaluate the impact of BMI on outcomes after stapes surgery. Methods: Medical records were examined from January 2015 to December 2017 in patients undergoing stapedectomy or stapedotomy at two tertiary care facilities. A retrospective cohort analysis comparing hearing outcomes on postoperative audiogram following procedural intervention with BMI was performed. The primary outcome was postoperative air-bone gap (dB) on audiogram. Results: Two hundred sixty three stapedotomy and stapedectomy patients were included in the study. Six percent had BMI less than 18.5 (underweight), 30% had BMI between 18.6 and 24.9 (normal weight), 38% had BMI between 25 and 29.9 (overweight), 24% had BMI above 30 (obese). The mean preoperative and postoperative air-bone gap (ABG) was 27.0 dB (standard deviation [SD] 15) and 10.4 dB (SD 10.6), respectively, with a postoperative ABG less than or equal to 20 dB in 87% of cases. There were no significant differences in postoperative ABG results when comparing the BMI categories (p = 0.11). Conclusions: Stapedotomy and stapedectomy are effective surgeries that can be performed by experienced surgeons with successful hearing improvement. While high BMI patients may be a challenge to position appropriately for their surgery, this does not translate to altered otologic outcomes for these patients. Address correspondence and reprint requests to Carleton Eduardo Corrales, M.D., Otology, Neurotology & Skull Base Surgery, Division of Otolaryngology–Head and Neck Surgery, Brigham and Women's Hospital, Harvard Medical School, 45 Francis Street, Boston, MA 02115; E-mail: ccorrales@bwh.harvard.edu Funding: None. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Quality of Life in Vestibular Schwannoma Patients: A Longitudinal Study Objective: To examine differences in quality of life in patients with vestibular schwannoma following a single treatment modality: observation, stereotactic radiosurgery (SRS), or microsurgery. Study design: Retrospective review. Setting: Tertiary academic medical center. Patients: Patients diagnosed with sporadic vestibular schwannoma who had completed at least 2 Penn Acoustic Neuroma Quality of Life (PANQOL) surveys. Interventions: Treatment via SRS, microsurgery, or observation. Main Outcome Measures: PANQOL total survey and subdomain scores. Results: One hundred and thirty-four patients (94 observations, 24 SRS, 16 microsurgeries) were included. The mean number of PANQOL surveys completed was 2.8 (range 2–5). The total PANQOL scores were significantly lower in the SRS group, but not the microsurgery group, compared with observation at the time of diagnosis (observation 550.4 ± 58.4; SRS 471.4 ± 37.4; microsurgery 492.6 ± 40.7; p = 0.03). Over time, there were no significant differences in the change of PANQOL scores across the 3 groups (SRS PANQOL score worsened 6.8/year compared with observation, p = 0.3; microsurgery PANQOL score worsened 7.8/year compared with observation, p = 0.5). Anxiety was the only subdomain that significantly worsened over time in the microsurgery group (microsurgery PANQOL score worsened 3.8/year compared with observation; p = 0.009). Conclusion: Despite differences in PANQOL scores at baseline, changes in total PANQOL score over time were not found to be statistically significant, regardless of the treatment group chosen. Overall, these results hold implications for patient counseling when considering treatment choice and quality of life predictions. Address correspondence and reprint requests to Lauren E. Miller, MD MBA, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles Street, Boston, MA 02114; E-mail: Lauren_miller@meei.harvard.edu Funding disclosure: No funding to disclose. The Institutional Review Board at the Hospital of the University of Pennsylvania approved this study (Protocol 814830). The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Machine Learning and Cochlear Implantation—A Structured Review of Opportunities and Challenges Objective: The use of machine learning technology to automate intellectual processes and boost clinical process efficiency in medicine has exploded in the past 5 years. Machine learning excels in automating pattern recognition and in adapting learned representations to new settings. Moreover, machine learning techniques have the advantage of incorporating complexity and are free from many of the limitations of traditional deterministic approaches. Cochlear implants (CI) are a unique fit for machine learning techniques given the need for optimization of signal processing to fit complex environmental scenarios and individual patients’ CI MAPping. However, there are many other opportunities where machine learning may assist in CI beyond signal processing. The objective of this review was to synthesize past applications of machine learning technologies for pediatric and adult CI and describe novel opportunities for research and development. Data Sources: The PubMed/MEDLINE, EMBASE, Scopus, and ISI Web of Knowledge databases were mined using a directed search strategy to identify the nexus between CI and artificial intelligence/machine learning literature. Study Selection: Non-English language articles, articles without an available abstract or full-text, and nonrelevant articles were manually appraised and excluded. Included articles were evaluated for specific machine learning methodologies, content, and application success. Data Synthesis: The database search identified 298 articles. Two hundred fifty-nine articles (86.9%) were excluded based on the available abstract/full-text, language, and relevance. The remaining 39 articles were included in the review analysis. There was a marked increase in year-over-year publications from 2013 to 2018. Applications of machine learning technologies involved speech/signal processing optimization (17; 43.6% of articles), automated evoked potential measurement (6; 15.4%), postoperative performance/efficacy prediction (5; 12.8%), and surgical anatomy location prediction (3; 7.7%), and 2 (5.1%) in each of robotics, electrode placement performance, and biomaterials performance. Conclusion: The relationship between CI and artificial intelligence is strengthening with a recent increase in publications reporting successful applications. Considerable effort has been directed toward augmenting signal processing and automating postoperative MAPping using machine learning algorithms. Other promising applications include augmenting CI surgery mechanics and personalized medicine approaches for boosting CI patient performance. Future opportunities include addressing scalability and the research and clinical communities’ acceptance of machine learning algorithms as effective techniques. Address correspondence and reprint requests to Matthew G. Crowson, M.D., F.R.C.S.C., Department of Otolaryngology-HNS, Sunnybrook Health Sciences Center, 2075 Bayview Avenue, Toronto, Ontario M4N 3M5, Canada; E-mail: matthew.g.crowson@gmail.com This article was accepted for presentation at CI2019: 16th Symposium on Cochlear Implants In Children, Hollywood, Florida, July 2019. Each of the authors indicated above has contributed to, read, and approved this manuscript. In consideration of the journal reviewing and editing my submission, the authors undersigned transfer, assign, and otherwise convey all copyright ownership in the event that such work is published. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Natural History of Facial Weakness Following Surgery of the Cerebellopontine Angle: A Tertiary Care Cohort Objective: Facial function is a key outcome in surgery of the cerebellopontine angle (CPA). This study describes the natural history of facial weakness after CPA surgery. Study design and setting: Retrospective study of two tertiary centers. Patients and intervention: Patients undergoing surgery for CPA tumors from 2003 to 2017 with preoperatively normal facial function and without subsequent surgical or adjuvant therapy. Main outcome measure: Serial facial nerve examinations using the House–Brackmann (HB) scale. Results: Of 301 patients examined, 149 (49.5%) had facial weakness postoperatively and 74.5% of these occurred within 24 hours. Of patients with HB-1 function within 24 hours after surgery, 95.3% had HB-1 function and 100% had HB-3 or better function at last follow-up. Of patients with HB-4 or worse function within 24 hours after surgery, 22.6% ultimately achieved HB-1 function and 83.9% ultimately achieved HB-3 or better function. By 180 days postoperatively, 83.7% of patients’ facial function had stabilized. At last follow-up, 3.0% of patients had facial function poorer than HB-3, which was associated with subtotal resections (12.9% vs. 4.4% for near total and 0.6% for gross total resections, p = 0.002), aspirin use (10.6% vs. 1.6%, p = 0.001), larger tumors (p < 0.0005), longer surgery (p < 0.002), and immediate versus delayed postoperative facial weakness (p = 0.002). Conclusions: Facial function over time after CPA surgery is presented. While facial function immediately after surgery correlates with future function, delayed weakness or improvement is common. These data inform patient counseling both preceding and following surgery. Address correspondence and reprint requests to Christopher A. Schutt, M.D., Department of Neurotology, Michigan Ear Institute, 30055 Northwestern Highway, Suite #101, Farmington Hills, MI 48334; E-mail: caschutt13@gmail.com The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Understanding the Dizziness Handicap Inventory (DHI): A Cross Sectional Analysis of Symptom Factors That Contribute to DHI Variance Objective: The Dizziness Handicap Inventory (DHI) is the most commonly used quality of life measure for vestibular disorders. However, there is wide variability in scores, and little is known about which variables contribute to dizziness-related quality of life. Our goal was to investigate the key demographic and symptom-related factors to that account for DHI variance. Study Design: Cross-sectional survey. Setting: Tertiary referral center. Patients: Adult patients presenting to a dizziness clinic. Main Outcome Measure: DHI variance explained by multiple linear regression. Results: Seventy subjects were included in our study. We performed univariate analyses on numerous demographic and dizziness-related factors, and constructed a multivariate model based on explaining the highest variance in the data with the least number of independent variables. Several validated quality of life and mental health survey scores (SF-36—quality of life; PHQ-9—depression; and GAD-7—anxiety) were significantly correlated with higher DHI scores. Additional factors, including the number of dizzy days per month, the number of dizziness descriptors (spinning, lightheadedness, disequilibrium, etc.), and the number of dizziness triggers (loud sounds, stress, riding in a car, etc.), were all significantly associated with higher DHI scores; a multiple linear regression model showed that these three aforementioned factors combined accounted for 56% of the variability in the DHI scores (p < 0.0001). Adding an index of depression, as measured by the PHQ-9, increased the adjusted R2 to 64% (p < 0.0001). Conclusions: DHI score variability is explained by mental health and quality-of-life measures in addition to the daily burden of dizziness. Address correspondence and reprint requests to Jeffrey D. Sharon, M.D., Assistant Professor, Department of Otolaryngology – Head and Neck Surgery, University of California – San Francisco School of Medicine, 2380 Sutter Street, San Francisco, CA 94115; E-mail: jeffrey.sharon@ucsf.edu The senior author (Dr. J.D.S.) is a paid consultant for Oticon. The authors disclose no conflicts of interest. Supplemental digital content is available in the text. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (http://journals.lww.com/otology-neurotology). Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Auditory Characteristics in Patients With Mucopolysaccharidosis Objective: The purpose of the study was to evaluate audiologic findings according to mucopolysaccharidosis (MPS) subtypes and to estimate hearing changes as the disease progressed, as well as the therapeutic effect of enzyme replacement therapy on the hearing apparatus. Methods: A total of 124 patients who were diagnosed with MPS between September 1994 and December 2016 were retrospectively analyzed. Play audiometry or pure-tone audiometry was performed for hearing assessment, and auditory brainstem response was conducted in patients with poor compliance. Results: In total 124 patients were identified, ranging in age at diagnosis from 0 to 33 years. Fourteen of the patients had been diagnosed with type I, while 91 had type II, 2 had type III, 14 had type IV, and 3 had type VI. Mean bone conduction and air conduction for the better ear were 26.13±16.95 dB and 34.77 ± 20.00 dB in all patients, and 34.20±7.64 dB and 40.70±9.67 dB in patients with MPS II. The average auditory brainstem response threshold was 68.96 ±21.93 dB nHL. The most common type of hearing loss was pure sensorineural hearing loss in all subtypes, and the degree of hearing loss was variable mostly within the mild to severe range. The increase in the hearing threshold was also significantly correlated with the disease duration. However, the change in hearing level was not correlated with the duration of enzyme replacement therapy. Conclusions: Hearing impairment in MPS patients is common and is aggravated as the disease progresses. Thus, adequate intervention and hearing rehabilitation might play an important role in managing hearing disabilities in MPS patients. Address correspondence and reprint requests to Il Joon Moon, M.D., Ph.D., Department of Otorhinolaryngology—Head and Neck Surgery, Sungkyunkwan University School of Medicine, Samsung Medical Center, 81 Irwon-ro, Gangnam-gu, 06351 Seoul, Korea; E-mail: iljoon.moon@samsung.com; Dong-Kyu Jin, M.D., Ph.D., Department of Pediatrics, Sungkyunkwan University School of Medicine, Samsung Medical Center, 81 Irwon-ro, Gangnam-gu, 06351 Seoul, Korea; E-mail: jindk@skku.edu I.J.M. and D.-K.J contributed equally to this study. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Benign Paroxysmal Positional Vertigo in the Elderly: A Single-center Experience Objectives: Dizziness and balance problems are common in the elderly, and benign paroxysmal positional vertigo (BPPV) is one of the most common causes of dizziness. The aim of this study is to investigate the subtype distribution of geriatric BPPV in a single tertiary referral center, and compare the treatment efficacy according to the subtype of BPPV. Study Design: Retrospective study. Setting: Tertiary referral academic center. Patients: The consecutive 316 elderly patients diagnosed with BPPV between March 2013 and March 2019 were included. Main Outcome Measures: Using a head-roll and Dix-Hallpike tests, subtype of BPPV was determined. Once the diagnosis of BPPV was made, patients were treated by its corresponding canalith repositioning maneuver (CRM). Results: Among 316 elderly patients with BPPV, 143 patients (45%) were diagnosed with posterior semicircular canal BPPV, 46 patients (15%) were diagnosed with lateral semicircular canal (LSCC) canalolithiasis, 126 patients (40%) were diagnosed with LSCC cupulolithiasis, and 1 patient (0%) was diagnosed with anterior semicircular canal BPPV. While 66 and 63% of the patients with posterior semicircular canal BPPV and LSCC canalolithiasis recovered after one session of CRM, only 32% of the patients with LSCC cupulolithiasis recovered after one session of CRM. Conclusion: The proportion of LSCC cupulolithiasis was higher in the elderly, and treatment efficacy by CRM is lower in LSCC cupulolithiasis than other subtypes of BPPV. High prevalence of LSCC cupulolithiasis may be explained by a delay between onset of BPPV and patient's presentation to the tertiary referral hospital or pathophysiology of ageotropic positional nystagmus other than otoconial attachment on the LSCC cupula in the elderly. Address correspondence and reprint requests to Chang-Hee Kim, M.D., Ph.D., Department of Otorhinolaryngology—Head and Neck Surgery, Konkuk University Medical Center, Konkuk University School of Medicine, 120-1 Neungdong-ro (Hwayang-dong), Gwangjin-gu, Seoul 143-729, Republic of Korea; E-mail: ryomachang@gmail.com This article was supported by Konkuk University in 2019. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Long-term Outcomes in Down Syndrome Children After Cochlear Implantation: Particular Issues and Considerations Objective: The aim of the study was to analyze the long-term outcomes after cochlear implantation in deaf children with Down syndrome (DS) regarding age at the first implantation and refer the results to preoperative radiological findings as well as postoperative auditory and speech performance. Additionally, the influence of the age at implantation and duration of CI use on postoperative hearing and language skills were closely analyzed in children with DS. Study Design: Retrospective analysis. Setting: Referral center (Cochlear Implant Center). Materials and Methods: Nine children with Down syndrome were compared with 220 pediatric patients without additional mental disorders or genetic mutations. Patients were divided into four categories depending on the age of the first implantation: CAT1 (0–3 yr), CAT2 (4–5 yr), CAT3 (6–7 yr), and CAT4 (8–17 yr). The auditory performance was assessed with the meaningful auditory integration scales (MAIS) and categories of auditory performance (CAP) scales. The speech and language development were further evaluated with meaningful use of speech scale (MUSS) and speech intelligibility rating (SIR). The postoperative speech skills were analyzed and compared between the study group and the reference group by using nonparametric statistical tests. Anatomic abnormalities of the inner ear were examined using magnetic resonance imaging (MRI) and high-resolution computed tomography of the temporal bones (HRCT). Results: The mean follow-up time was 14.9 years (range, 13.1–18.3 yr). Patients with DS received a multichannel implant at a mean age of 75.3 months (SD 27.9; ranging from 21 to 127 mo) and 220 non-syndromic children from reference group at a mean age of 51.4 months (SD 34.2; ranging from 9 to 167 mo). The intraoperative neural response was present in all cases. The auditory and speech performance improved in each DS child. The postoperative mean CAP and SIR scores were 4.4 (SD 0.8) and 3.2 (SD 0.6), respectively. The average of scores in MUSS and MAIS/IT-MAIS scales was 59.8% (SD 0.1) and 76.9% (SD 0.1), respectively. Gathered data indicates that children with DS implanted with CI at a younger age (<6 years of age) benefited from the CI more than children implanted later in life, similarly in a control group. There were additional anomalies of the temporal bone, external, middle, or inner ear observed in 90% of DS children, basing on MRI or HRCT. Conclusions: The early cochlear implantation in children with DS is a similarly useful method in treating severe to profound sensorineural hearing loss (SNHL) as in non-syndromic patients, although the development of speech skills present differently. Due to a higher prevalence of ear and temporal bone malformations, detailed diagnostic imaging should be taken into account before the CI qualification. Better postoperative outcomes may be achieved through comprehensive care from parents/guardians and speech therapists thanks to intensive and systematic rehabilitation. Address correspondence and reprint requests to Pedro Clarós, M.D., Ph.D., Clarós Clinic, c./Vergós 31, 08017 Barcelona, Spain; E-mail: clinica@clinicaclaros.com The authors report no potential conflict of interest. The authors report no financial and material support for the research and the work in the manuscript. The principal investigator of the research, Pedro Clarós (M.D. Ph.D.), states that he had full access to all data in the study and takes responsibility for the integrity of the data and accuracy of the data analysis. In their research three authors, i.e., Pedro Clarós (M.D. Ph.D.; Clarós Clinic, Cochlear Implant Center, Barcelona, Spain), Agnieszka Remjasz (M.D.; Clarós Clinic, Cochlear Implant Center, Barcelona, Spain; Department of Otorhinolaryngology at Stefan Zeromski Specialist Hospital, Cracow, Poland), and Andrzej Wiatrow (M.Sc.; Institute of Psychology Polish Academy of Sciences, Warsaw, Poland), conducted and were responsible for the data analysis. ORCID iD for P. Clarós: 0000-0002-7567-0370. ORCID iD for A. Clarós: 0000-0001-6084-3470. ORCID iD for A. Clarós-Pujol: 0000-0002-9288-8849. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

NF2-Related Intravestibular Schwannomas: Long-Term Outcomes of Cochlear Implantation Objective: Intravestibular schwannomas (IVS) are uncommon tumors in Neurofibromatosis type 2 (NF2) and are mainly associated with multiple internal auditory meatus (IAM) and cerebellopontine angle (CPA) tumors. They usually induce profound hearing loss which can be rehabilitated by cochlear implantation (CI). The aim of this study was to analyze the long-term outcomes of CI during the unpredictable evolution of NF2 disease. Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Three adults with neurofibromatosis type 2 and intravestibular schwannomas, and who were cochlear implant recipients. Interventions: Periodic radiologic follow-up, tumor resection, and hearing rehabilitation. Main Outcome Measures: Audiological evolution, tumor evolution, surgical outcome, cochlear implant outcome. Results: Three NF2 patients (mean age at diagnosis, 26.3 ± 3.2 yr) were identified with IVS in the period between 2000 and 2017. IVS were first observed by serial MRI and profound hearing loss occurred in this ear after 4 ± 1.5 years of follow-up. IVS were removed via a translabyrinthine approach, and ipsilateral cochlear implantations were simultaneously performed. In two patients, large contralateral CPA tumors had previously been removed without hearing preservation, whereas in the third patient, a small, growing contralateral VS was excised via a retrosigmoid approach 6 months after IVS removal/cochlear implantation with serviceable hearing preservation. In all cases, CI provided good hearing outcomes. In two cases, hearing outcomes were even better for more than 5 years when ipsilateral intracanalicular vestibular schwannomas were removed in either the same or subsequent procedures. Conclusions: Rehabilitation of hearing with CI provides a favorable long-term outcome in patients with NF2-related IVS which could be altered by the occurrence of other intracanalicular and/or CPA NF2-related tumors. Address correspondence and reprint requests to Olivier Sterkers, M.D., Ph.D., GH Pitié-Salpêtrière, Service d’Otologie, Implants Auditifs et Chirurgie de la base du Crâne, Bâtiment Castaigne, 47-83 Boulevard de l’Hôpital, 75651 Paris cedex 13, France; E-mail: olivier.sterkers@aphp.fr; Hao Wu, M.D., Ph.D., Department of Otolaryngology–Head and Neck Surgery, Shanghai Ninth People's Hospital, No 639 Zhizaoju Road, Shanghai 200011, China; E-mail: wuhao622@sina.cn The authors from China were supported by the projects of the National Natural Science Foundation of China (81200742) and the Shanghai Scientific and Technological Innovation Action Plan (17441903600) in this study. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company