Otology & Neurotology

Outcome of Cochlear Implantation in Children With Narrow Bony Cochlear Nerve Canal Objective: To investigate the diameter of the bony cochlear nerve canal (BCNC) as a prognostic indicator of cochlear implantation (CI) in children. Study Design: Retrospective study. Setting: Tertiary referral center. Patients: Two hundred ninety two prelingual deaf children (323 ears) who received CI before the age of 5 years. Interventions: CI in pediatric patients. Main Outcome and Measures: Postoperative auditory performance was analyzed according to the diameter of the BCNC using the Mann–Whitney U test. Using the images of temporal bone computed tomography, patients were grouped according to the diameter of the BCNC: narrow (<1.4 mm, Group 1), normal (Group 2), and other anomalies (Group 3). Results: Group 1 (10.5%) showed a less significant degree of improvement in category of audiological performance (CAP) score than Groups 2 (57.3%) and 3 (32.2%). Scores obtained from both the open-set monosyllabic words and sentence tests were lower in Group 1 than Groups 2 and 3. Of the patients with narrow BCNC, the CAP score of patient with complete stenosis of BCNC (<0.2 mm) was lower than that of patients with BCNC between 0.2 and 0.8 mm, also 0.8 and 1.4 mm. Conclusions: The improvement in auditory performance was limited in children with narrow BCNC and the narrower the BCNC, the more severe the limitation. However, gradual improvement can be expected and even children with narrow BCNC may be candidates for early CI and rehabilitation. Address correspondence and reprint requests to Jong Woo Chung, M.D., Ph.D., Department of Otorhinolaryngology–Head and Neck Surgery, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, Republic of Korea; E-mail: jwchung@amc.seoul.kr Funding sources and financial disclosure: None. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Bilateral Cochlear Implantation in Vogt-Koyanagi-Harada Syndrome: A Case Report Objective: To evaluate cochlear implantation in Vogt-Koyanagi-Harada syndrome with regard to surgical difficulties and hearing outcomes. Patients: Single case report. Intervention(s): Therapeutic complete electrode insertion during surgery despite evident intra-cochlear fibrosis. Main Outcome Measure(s): Postoperative speech audiometry, categories of auditory performance, and speech intelligibility rating. Results: Vogt-Koyanagi-Harada syndrome is a multisystem autoimmune disease that affects tissues containing melanin. It is characterized by bilateral uveitis with auditory, vestibular, and dermatologic manifestations. Standard treatment comprises aggressive administration of systemic corticosteroids. This report describes the use of bilateral cochlear implants in a 30-year-old Saudi woman who presented with uveitis and was diagnosed with Vogt-Koyanagi-Harada syndrome. She had progressive hearing loss in both ears and experienced minimal improvement with hearing aids. The patient underwent sequential cochlear implantation, which was challenging because it was difficult to insert the electrode in the right ear due to intra-cochlear fibrosis. After more than 5 years of follow-up, she has good hearing in both ears, despite the advancement of ocular disease and recurring visual complaints. Conclusion: This study suggests that patients with Vogt-Koyanagi-Harada syndrome could develop intra-cochlear fibrosis during cochlear implantation due to the autoimmune nature of disease. Moreover, cochlear implantation becomes more difficult with disease advancement. Therefore, it is essential that healthcare professionals consider early detection and prompt treatment of hearing loss in patients with this syndrome. Address correspondence and reprint requests to Nora AlHelali, College of Medicine, King Saud University, Riyadh, Saudi Arabia; E-mail: n.k.alhelali@gmail.com Source of Funding: none were declared. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Preservation of Vestibular Function and Residual Hearing After Round Window Cochlear Implantation Objective: To examine the possible correlation between the loss of short-term residual hearing and vestibular function after cochlear implantation (CI). Study Design: Retrospective patient review. Setting: Academic tertiary referral center. Patients: One hundred twenty patients with normal vestibular function (VF) in the caloric testing and residual hearing (RH) at the frequencies 250, 500, and 1000 Hz on the surgery side between 2008 and 2016 were included in the study. Intervention: Primary CI on the first side via round window with a conventional full-length electrode. Main Outcome Measures: Changes of RH and VF 7 weeks after surgery were analyzed. Preservation of RH was defined as measurable postoperative thresholds at the frequencies 250, 500, and 1000 Hz in the pure-tone audiogram. Preservation of the VF after CI was assessed both by an absolute and relative threshold in the caloric testing. Results: Seven weeks after implantation, the preservation of RH was achieved in 52 (43.3 %) patients and the preservation of VF in 95 (79.2 %) patients on the operated side. There was no significant statistical correlation between these two parameters. Conclusion: The loss of RH was more than twice as frequent as the loss of VF. However, no statistical correlation between the failure of the two inner ear functions was found. The exact causes are still unknown, but our data could give an indication that there may be different underlying pathomechanisms. Address correspondence and reprint requests to Konrad Johannes Stuermer, M.D., Department of Otorhinolaryngology, Head and Neck Surgery, Medical Faculty, University of Cologne, Kerpener Street 62, 50937 Cologne, Germany; E-mail: konrad.stuermer@uk-koeln.de All authors contributed substantially to the article in conception, design, and writing. This material has never been published and is not currently under evaluation in any other peer-reviewed publication. The study meets the requirements of the local ethics committee (institutional review board). The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

A Validated Questionnaire to Assess the Severity of Persistent Postural-Perceptual Dizziness (PPPD): The Niigata PPPD Questionnaire (NPQ) Objective: To establish a questionnaire to diagnose and assess the severity of persistent postural-perceptual dizziness (PPPD). Study Design: Retrospective chart review. Setting: Tertiary referral center. Patients: Fifty PPPD patients and 50 consecutive control patients with other vestibular disorders. Interventions: Patients answered questions on three exacerbating factors of PPPD (upright posture/walking, movement, and visual stimulation), and each factor was evaluated using four questions scoring the severity from 0 (none) to 6 (unbearable). Somatic and psychological distress was evaluated by the Visual Analog Scale (VAS) and the Hospital Anxiety and Depression Scale (HADS), respectively. Main Outcome Measures: The questionnaire's reliability was tested by Cronbach's alpha, and it was validated by examining the differences in the questionnaire's scores between PPPD patients and controls. The area under the curve (AUC) of the receiver operating characteristic curve for each factor was calculated. Results: Cronbach's alpha coefficient was >0.8 for all factors, except the movement factor. There were no significant differences in the VAS and HADS scores between the two groups. However, the combined and individual questionnaire scores for each factor were higher in PPPD patients than in controls, indicating the questionnaire's high validity. The AUC was widest for the visual stimulation factor (0.830), and a score of 9 (full score 24) had the best sensitivity (82%) and specificity (74%) for discriminating PPPD patients from controls. Conclusions: We developed a questionnaire that exhibited high reliability and validity in evaluating PPPD severity. The visual stimulation factor may be the most characteristic among the three exacerbating factors. This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0 Address correspondence and reprint requests to Chihiro Yagi, M.D., Department of Otolaryngology, Head and Neck Surgery, Niigata University Graduate School of Medical and Dental Sciences, Asahimachi 1, Niigata city, Niigata 951-8510, Japan; E-mail: sorasora.yoky@gmail.com This work was supported by Grants-in-Aid from the Ministry of Education, Culture, Sports, Science and Technology of Japan to C.Y. and A.H. This study was presented at the 54th Annual Spring Meeting for American Neurotology Society held at Austin, Texas (May 3–4, 2019). The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Endre Hőgyes (1847–1906), Forgotten Father of the Vestibulo-Ocular Reflex Objective: Throughout the history of vestibular research, the discovery of the vestibulo-ocular reflex in 1881 by Endre Hőgyes (1847–1906) is rarely mentioned. The aim of this study is to review Hőgyes' vestibular research articles, all originally written in Hungarian and emphasize their epoch-making content. Main Data Sources: Hőgyes' vestibular publications, originally written in Hungarian, which describe various eye movements of the rabbit in response to vestibular stimulation by rotation about three axes. Results: Hőgyes was the first to use a three-axis turntable on an experimental animal, in this case a rabbit. He found that depending on the plane of rotation, different types of binocular eye movements were produced. He then demonstrated by destructive and excitatory experiments, the anatomical pathways and the physiological function producing this phenomenon. Ultimately, he explained the exact connections between the inner ear labyrinth and certain muscle contractions during eye movements. He identified this pathway as the "associating center of the ocular movements," later defined as the vestibulo-ocular reflex. Hőgyes' discovery was only superficially noted during his lifetime and ignored after his death. Conclusion: Hőgyes was the first to demonstrate the vestibulo-ocular reflex. He was forgotten during the ensuing 140 years probably because his articles were appeared only in Hungarian and because a short time later, Róbert Bárány's award of the Nobel Prize overshadowed many of Bárány's predecessors and contemporaries, including Hőgyes and relegated them to the background. Address correspondence and reprint requests to László T. Tamás, M.D., 9028, Győr, Vak Bottyán u.14/A, Hungary; E-mail: ltamas@gyor.net Dr. L.T.T. and Dr. A.M. helped in data collection, analysis, and interpretation, critically reviewing and editing the manuscript. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Negative Predictive Value of Non-Echo-Planar Diffusion Weighted MR Imaging for the Detection of Residual Cholesteatoma Done at 9 Months After Primary Surgery Is not High Enough to Omit Second Look Surgery Objectives: To evaluate non echo-planar diffusion weighted magnetic resonance imaging (non-EP DW MRI) at 9 months after primary surgery to rule out residual cholesteatoma in patients scheduled before second-look-surgical exploration. Study Design: Prospective observational study. Setting: Secondary teaching hospital. Patients/Interventions: Patients who were scheduled for second-look-surgery after primary canal wall up repair of cholesteatoma underwent 1.5 T MRI including non-EP DWI and high-resolution coronal T1 and T2-FS SE sequences. Main Outcome Measures: Imaging studies were evaluated for the presence of cholesteatoma by three independent observers. Intraoperative observations were regarded the standard of reference. Ear, nose, throat (ENT) surgeons were blinded for imaging findings. The primary outcome was the negative predictive value (NPV) of MR imaging, secondary outcomes were sensitivity, specificity, and positive predictive value. Results: Thirty-three patients underwent both MRI and surgery, among whom 22 had a cholesteatoma. Mean time between primary surgery and MRI was 259 days (standard deviation [SD] 108). NPV of non-EP DW MRI in detecting recurrent cholesteatoma was 53% (95% CI: 32–73%). Sensitivity and specificity were 59% (39–77%) and 91% (62–98%), respectively. The positive predictive value was 93% (69–99%). In five out of nine false-negative cases, recurrent cholesteatoma measured 3 mm or less. Using a 3 mm detection threshold, NPV increased to 79%. Conclusion: Non-EP DW MRI cannot replace second look surgery in ruling-out residual cholesteatoma at 9 months after primary surgery. It could be used in a follow-up strategy in low risk patients. Further research is needed which types of residual cholesteatoma are not revealed by MRI. Address correspondence and reprint requests to Roelof J. Horn, M.D., Department of Radiology, St Anna Ziekenhuis, PO BOX 90, 5660 AB Geldrop, The Netherlands; E-mail: r.horn@st-anna.nl Authors' contributions: All authors agree to be accountable for all aspects related to accuracy or integrity of this work. Study concepts/design: R.J.H., J.W.C.G., P.P.G.B. Data acquisition and analysis: R.J.H., J.W.C.G., K.E.D. Literature research: R.J.H. Statistical analysis: H.J.Z. Manuscript drafting, editing and final approval: all authors. §Present address of author Roelof J. Horn: Department of Radiology, St Anna Ziekenhuis Geldrop, The Netherlands. Present address of author Peter-Paul G. van Benthem: Department of ORL-HNS, Leiden University Medical Centre, Leiden, The Netherlands. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

First MRI With New Cochlear Implant With Rotatable Internal Magnet System and Proposal for Standardization of Reporting Magnet-Related Artifact Size Objective: To report on the first known magnetic resonance imaging (MRI) with a new cochlear implant (CI) with rotatable internal magnet system, to review the literature on MRI in cochlear implantees, and to advocate for standardization of reporting magnet-related artifact size. Study Design: Case report and review of literature. Setting: Tertiary care hospital. Results: A patient with congenital rubella and bilateral profound hearing loss was incidentally found to have a petroclival meningioma. After resection and radiosurgery, she underwent cochlear implantation with the Advanced Bionics HiRes Ultra 3D device (Advanced Bionics LLC, Valencia, CA) with rotatable internal magnet system, due to need for imaging surveillance of residual meningioma. During 1.5 T MRI brain scan without a head wrap, she experienced no adverse events. The images obtained were adequate for visualization of residual tumor. Implant recipients with non-rotatable magnets who undergo MRI, with or without recommended head wrap, may suffer various complications. All images in patients with retained internal magnets are subject to magnet-related artifact, but reports regarding its size are variable and lack detail on how measurements are made. Conclusions: MRI in patients with a new CI device with rotatable magnet system may be performed without discomfort or device dislodgement at 1.5 T, even without a head wrap, though external magnet replacement may require multiple attempts due to internal magnet realignment. Despite significant artifact, the structure of interest may still be visualized for accurate diagnosis. Measuring magnet-related artifact size should be standardized by reporting artifact in radii at the image level of maximal signal loss. Address correspondence and reprint requests to Samuel P. Gubbels, M.D., University of Colorado, Aurora, CO; E-mail: samuel.gubbels@ucdenver.edu S.P.G. has grant support from the National Institutes of Health/National Institutes on Deafness and Other Communicative Disorders (R01 DC013912–01). S.P.G. has received an honorarium from Decibel Therapeutics for ad hoc consulting and receives consulting fees for Scientific Advisory Board Membership with Applied Genetic Technologies Corporation and Roche Pharmaceuticals. S.P.G. also is an ad hoc consultant for Cochlear Corporation and receives research funding support for Med-El Corporation. No funding or other support was required for this study. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Abnormal Tectorial Membranes in Sensorineural Hearing Loss: A Human Temporal Bone Study Hypothesis: This study evaluates the morphological changes of the tectorial membrane (TM) in conjunction with degeneration of hair cells, interdental cells, and presence of endolymphatic hydrops (EH) in sensorineural hearing loss (HL) in the human using histopathology techniques. Background: The TM plays an important role in mechanical transduction of acoustic energy, and pathology of the TM may result in HL. Methods: All temporal bone (TB) specimens from the Massachusetts Eye and Ear Otopathology Laboratory from patients with various causes of sensorineural HL and morphological abnormalities of the TM were evaluated. Cases with a history of cochlear trauma (other than acoustic trauma) and/or severe postmortem artifacts were excluded. The TBs were processed histologically, and the status of hair cells, supporting cells, interdental cells, presence of EH, and postmortem time were tabulated. Results: Two thousand two hundred ninety TBs from 1340 individuals were evaluated, and 164 of 748 TBs from the otological disorders in which the TM were abnormal, met the inclusion criteria. The most common disorders were idiopathic sudden deafness (57.1%), genetic etiology (53.7%), and ototoxicity (40.0%), as compared with cases with presbycusis (2.9%). EH was found in 33.3% of all cases with an identified abnormality of the TM. Abnormalities of the TM were 1) deformed, 2) shrunken, 3) detached from the limbus, 4) encapsulated, or 5) missing. Encapsulated, shrunken and missing patterns (36, 35, 31%, respectively) were the most common. Conclusion: A relative high prevalence of EH among disorders with TM abnormalities suggests a possible common pathophysiology in both. In addition, anatomic abnormalities of the TM may play a role in the pathophysiology of HL in these disorders. Address correspondence and reprint requests to Joseph B. Nadol, Jr., M.D., Otopathology Laboratory, Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114; E-mail: joseph_nadol@meei.harvard.edu This work was supported by grant U-24DC013983 from the National Institute on Deafness and Other Communication Disorders (NIDCD). The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Clinical Applicability of a Preoperative Angular Insertion Depth Prediction Method for Cochlear Implantation Objective: Evaluation of the accuracy and clinical applicability of a single measure cochlear implant angular insertion depth prediction method. Background: Cochlear implantation outcomes still vary extensively between patients. One of the possible reasons could be variability in intracochlear electrode array placement. For this reason, single measure methods were suggested to preoperatively predict angular insertion depths. Based on a previously performed accuracy study in human temporal bones, we were interested in determining the extent to which the method could be applied in a clinical setting. Methods: A retrospective analysis was performed on pre- and postoperative radiographic images of 50 cochlear implant recipients. Preoperatively predicted angular insertion depths were compared with angular insertion depths measured on postoperative ground truth. The theoretical prediction error was computed under the assumption that all achieved insertions were matching the preoperatively assumed linear insertion depth. More importantly, the clinical prediction error was assessed using two different software tools performed by three experienced surgeons. Results: Using the proposed method we found a theoretical prediction error of 5 degrees (SD = 41 degrees). The clinical prediction error including the cases with extracochlear electrodes was 70 degrees (SD = 96 degrees). Conclusions: The presented angular insertion depth prediction method is a first practical approach to support the preoperative selection of cochlear implant electrode arrays. However, the presented procedure is limited in that it is unable to predict the occurrence of insertion results with extracochlear electrodes and requires user training. Address correspondence and reprint requests to Wilhelm Wimmer, Ph.D., ARTORG Center for Biomedical Engineering Research, University of Bern, Bern, Switzerland; E-mail: wilhelm.wimmer@artorg.unibe.ch This research project was supported by the Eurostars E! 11597 RCI research grant. The authors have no conflicts of interest to disclose. 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 (www.jcraniofacialsurgery.com). Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Age Dependent Cost-Effectiveness of Cochlear Implantation in Adults. Is There an Age Related Cut-off? Objective: To analyze the impact of age at implantation on the cost-effectiveness of cochlear implantation (CI). Study Design: Cost-utility analysis in an adapted Markov model. Setting: Adults with profound postlingual hearing loss in a "high income" country. Intervention: Unilateral and sequential CI were compared with hearing aids (HA). Main Outcome Measure: Incremental cost-effectiveness ratio (ICER), calculated as costs per quality adjusted life year (QALY) gained (in CHF/QALY), for individual age and sex combinations in relation to two different willingness to pay thresholds. 1 CHF (Swiss franc) is equivalent to 1.01 USD. Results: When a threshold of 50,000 CHF per QALY is applied, unilateral CI in comparison to HA is cost-effective up to an age of 91 for women and 89 for men. Sequential CI in comparison to HA is cost-effective up to an age of 87 for women and 85 for men. If a more contemporary threshold of 100,000 CHF per QALY is applied, sequential CI in comparison to unilateral CI is cost-effective up to an age of 80 for women and 78 for men. Conclusions: Performing both sequential and unilateral CI is cost-effective up to very advanced ages when compared with hearing aids. Address correspondence and reprint requests to Roman D. Laske, M.D., Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital Zurich, Frauenklinikstr. 24, 8091 Zürich, Switzerland; E-mail: laske@gmx.ch R.D.L. and M.D. have contributed equally for this manuscript. Sources of support: None. Disclosure of funding: None. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Alexandros Sfakianakis
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