Otology & Neurotology

Comprehensive Analysis of Factors Leading to Poor Performance in Prelingual Cochlear Implant Recipients Objective: To comprehensively analyze the prognostic factors responsible for affecting outcomes following cochlear implantation in prelinguals. Study Design: Retrospective cohort study from June 2004 to November 2015. Setting: Tertiary care center. Patients: Patients who had undergone cochlear implantation during June 2004 to November 2015 for prelingual sensorineural hearing loss with a minimum follow-up of 2 years. Intervention: Patients were evaluated for the presence of 20 risk factors possibly influencing postimplantation outcomes using a questionnaire. Main Outcome Measures: Assessment for speech and auditory function was done at the last follow-up with speech intelligibility ratings (SIR) and categories of auditory performance (CAP) scores, respectively. Results: One hundred fifty-one patients were included in this study. On univariate analysis for CAP, age at implantation, noncompliance to pre and postimplantation auditory and speech habilitation, poor parental motivation, socioeconomic status and literacy were found to be associated with lower scores (p < 0.05). Whereas, for SIR, additionally, attention deficit hyperactivity disorder and inner ear malformation were statistically significant negative predictors on univariate analysis. Finally, factors responsible for low CAP scores on multivariate analysis were poor parental literacy, poor socioeconomic status, irregular pre/postimplantation rehabilitation, and attention deficit hyperactivity disorder. While for SIR, additionally age at implantation was also a significant negative predictor. Increasing IQ and duration of implant use were associated with higher CAP (univariate) and SIR scores (univariate and multivariate) (p < 0.05). Conclusion: We were able to demonstrate negative impact of higher age at implantation, minimal cognitive disorder, adverse parental/socioeconomic profile, and poor compliance to pre/postimplantation auditory verbal habilitation on auditory and speech outcomes. Address correspondence and reprint requests to Kapil Sikka, M.S., Associate Professor, Department of Otorhinolaryngology and Head and Neck Surgery, Teaching Block, 4th Floor, All India Institute of Medical Sciences, Ansari Nagar, East, New Delhi 110029, India; E-mail: Kapil_sikka@yahoo.com The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Evaluating the Long-Term Hearing Outcomes of Cochlear Implant Users With Single-Sided Deafness Objectives: To investigate the long-term outcomes of cochlear implantation in individuals with single-sided deafness (SSD) in terms of speech perception, subjective hearing performance, and sound localization. Methods: Thirty-four subjects with SSD were recruited across two large cochlear implant (CI) centers (Antwerp, Belgium and Perth, Australia). The long-term hearing outcomes (between 4 and 10 years of CI use) were evaluated using speech in noise tests, a subjective hearing performance questionnaire (Speech, Spatial and Qualities Questionnaire [SSQ12]), and sound localization tests. Results: Statistically significant improvements were observed in speech perception in noise and sound localization results postoperatively with the use of a CI in comparison to preoperative measurements. Subjective hearing abilities also significantly improved after long-term CI use. Conclusion: Access to binaural hearing is important for subjects with SSD. CI users with SSD experience long-term benefits in speech understanding, sound localization, and quality of life. Address correspondence and reprint requests to Dayse Távora-Vieira, Otolaryngology, Head & Neck Surgery, Medical School, University of Western Australia, Perth, 102 - 118 Murdoch Drive, Murdoch WA 6150, Australia; Fiona Stanley Hospital, Perth, Australia; E-mail: dayse.tavora@gmail.com The authors report no conflicts of interest Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Application of Kaizen Principles to a Large Cochlear Implant Practice: A Continuous Quality Improvement Initiative at Mayo Clinic Objective: To remove barriers and improve access for patients seeking cochlear implantation. Study Design: Prospective quality improvement study at a large tertiary academic care center. Methods: A Kaizen quality improvement model was applied over the course of a year. Four weeklong meetings were used to identify areas for improvement and remediation. Data were collected at baseline, 90-days, and 1-year postcompletion of the project. Outcome measures included lead times, defined as the wait time between first contact with the clinic and the first appointment, and the wait time between surgery and activation, and cycle times defined as the total test time needed to determine cochlear implant candidacy, and total time needed to complete initial activation. The total inventory kept as clinic stock was also calculated Results: Kaizen team members collected data for each outcome measure. After the Kaizen principles were applied, the following outcomes were observed: median lead times between first contact with the clinic to candidacy testing, candidacy testing to surgery, and surgery to activation of the implant remained stable from baseline to 1-year follow-up. Median cycle time for candidacy testing decreased from 7.3 hours at baseline to 3.0 hours at 1-year follow-up. Cycle times for initial activation of the device did not change over time. The total inventory of clinic stock was reduced by 31%. Conclusions: Though outcomes for lead and cycle times varied, implementation of Kaizen principles was found to be an effective method for completing this quality improvement project at a large cochlear implant program overall. Level of Evidence: 3a Address correspondence and reprint requests to Douglas P. Sladen, Ph.D., Department of Communication Sciences and Disorders, Western Washington University, 516 High Street, Bellingham, WA 98225; E-mail: douglas.sladen@wwu.edu This project was funded through a grant provided by MED EL GMBH. M.L.C. is a consultant for Advanced Bionics Corp., Cochlear Corp., and MED-EL GmbH. M.D.D. is a consultant for MED-EL GmbH. 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

Quality of Life in Jugular Paraganglioma Following Radiosurgery Objective: The treatment paradigm for jugular paraganglioma (JP) has changed considerably over time with the wider adoption of stereotactic radiosurgery (SRS). To the best of the authors' knowledge, there are no published studies that use validated patient-reported outcome measures to ascertain quality of life (QoL) outcomes following SRS for JP when used in single or combined modality treatment regimens. Study Design: Cross-sectional survey. Setting: Tertiary referral center. Patients: Adult patients with JP treated with primary SRS or SRS following primary surgery between 1990 and 2017. Interventions(S): Surgery and/or Gamma Knife SRS. Main Outcome Measures: Global and treatment-related QoL and differences in QoL based on treatment approach. Results: Sixty-nine surveys were distributed and a total of 26 completed surveys were received (38% response rate). Among respondents, the median age at SRS was 53 years and 16 of the 26 patients (62%) were female. Median follow-up was 97 months. Nineteen patients (73%) were treated with primary SRS or staged SRS following intentional subtotal resection (STR; hereafter referred to as "staged SRS"), while the remainder (n = 7, 27%) were treated with SRS for recurrent JP. Median physical and mental health QoL PROMIS-10 T-scores regardless of treatment strategy were 39.8 and 38.8, respectively, while median SF36 physical and mental component subscores were similar to national averages and non-tumor controls. When comparing general physical and mental health QoL scores, there was no significant difference between patients treated with primary or staged SRS and those treated with SRS for recurrent JP. However, age-adjusted swallowing function among patients treated with primary or staged SRS was better than in those patients treated with SRS for recurrent JP (p = 0.05). Conclusions: Patients treated with primary or staged SRS for JP tend to exhibit better swallowing outcomes than those treated with SRS for recurrent JP. However, the majority of overall and disease-specific quality of life measures were not different between groups. Based on the low incidence of new cranial neuropathy following SRS, it is likely that initial surgical morbidity is the primary contributor to this outcome. Disease-specific overall quality of life measures, akin to those already used for other benign skull base tumors, are necessary to better gauge physical and mental health outcomes following treatment for JP. Though limited by small sample size, this represents the first study to gauge QoL outcomes following treatment for JP. Address correspondence and reprint requests to Matthew L. Carlson, M.D., Department of Otolaryngology – Head and Neck Surgery, Mayo Clinic, 200 1st St SW, Rochester, MN 55905; E-mail: carlson.matthew@mayo.edu Internal departmental funding was used without commercial sponsorship or support. Mayo Clinic IRB Approval 16-002291. Presented at the 2018 American Academy of Otolaryngology – Head and Neck Surgery Meeting, October 7–10, Atlanta, GA. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Bilateral Posterior Canal Benign Paroxysmal Positional Vertigo Tends to Reoccur Objective: We analyzed the clinical characteristics of quite a large cohort of patients with bilateral posterior canal benign paroxysmal positional vertigo (bil-BPPV) with respect to the rate and types of recurrence. Study Design: Retrospective case review. Setting: Outpatient dizziness clinic. Patients: About 2,050 patients diagnosed with BPPV during the years 2003 to 2018 were reviewed. Results: Sixty (2.9%) were diagnosed with bil-BPPV. Fifty three (88%) were idiopathic and seven (12%) posttraumatic. Multiple BPPV episodes were encountered in 28 (47%) patients. Patients with bil-BPPV and multiple BPPV episodes were comparable with patients with a single attack of bil-BPPV except for older age and longer follow up. The most frequent type of recurrence following a bil-BPPV episode was posterior canal canalolithiasis (37.5%) and bil-BPPV (31.3%). Combined (posterior and horizontal) BPPV was encountered in 12.5% while horizontal canal involvement was found in only 7% of recurrences. Conclusions: The prevalence of bil-BPPV amongst all BPPV patients was lower than previously reported possibly due to stricter selection criteria. Older age and longer follow up of patients with multiple BPPV episodes than those with a single episode of bil-BPPV is in accordance with the theory of degenerative otoconia loosening. In contrast to the general consensus regarding random recurrences of BPPV side and subtypes, bil-BPPV recurred more frequently than expected. This might be the consequence of individual vulnerability to head movements, increased bone metabolism or an underlying vestibular pathology. Address correspondence and reprint requests to Lea Pollak, M.D., Kibutz Galuyot 4, Nes Ziona 74012, Israel; E-mail: lea.pollak@gmail.com The authors declare no funding sources or acknowledgments. The authors have no conflicts of interest to disclose. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Serum Bilirubin Level as a Potential Marker for the Hearing Outcome in Severe-Profound Bilateral Sudden Deafness Objective: To investigate the association of serum bilirubin level with hearing outcomes in bilateral sudden sensorineural hearing loss (BSSHL) patients. Participants: One hundred thirteen in-patient BSSHL patients were consecutively enrolled between July 2008 and December 2015 in a tertiary center. Main Outcome Measures: Multivariable linear regression, generalized estimating equations (GEE), and stratified analyses were applied to examine the association between serum bilirubin level and hearing outcome measures such as final hearing threshold and absolute and relative hearing gains in BSSHL. Results: After full adjustment for potential confounders, total bilirubin levels (TBIL) were observed to be positively and independently associated with hearing outcomes as measured by final hearing (β [95% confidence interval {CI}]: −1.5 [−2.7, −0.2] dB HL per 1 μmol/L increase in TBIL) and absolute and relative hearing gains (β [95% CI]: 1.4 [0.2, 2.7] dB and 1.6 [0.2, 3.1] dB, respectively) in the severe to profound hearing loss subpopulation. Conclusions: Higher TBIL levels, within the normal or mildly elevated ranges, were independently and significantly associated with better hearing outcome in BSSHL patients with severe to profound hearing loss. Given bilirubin elevation treatments exist, our finding suggests a novel pharmacological strategy for this specific subpopulation. 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 Qiu-Ju Wang, M.D., Ph.D., Department of Otolaryngology–Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital, 100853, Beijing, China; E-mail: wqcr301@vip.sina.com Source of Funding: The work was supported by the National Natural Science Foundation of China (No.81530032 and No.81500794), the National Key Basic Research Program of China (No.2014CB943001), the China Postdoctoral Science Foundation (No. 2017M613326) and the New Researcher Foundation of the PLA General Hospital (No. 14KMZ04). 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 (http://journals.lww.com/otology-neurotology). Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Incus Necrosis and Blood Supply: A Micro-CT and Synchrotron Imaging Study Background: Incus necrosis is a common complication following stapes surgery and is associated with impaired microcirculation. The objective of this study was to investigate the vascular anatomy of the human incus by using light microscopy, micro-computed tomography (micro-CT), and synchrotron phase-contrast imaging (SR-PCI) for a novel three-dimensional (3D) analysis of the middle ear, mucosal folds, major vascular pathways, and intraosseous vascular bone channels. Methods: One-hundred-and-fifty temporal bones from the Uppsala collection were analyzed under light microscopy. Twenty temporal bones underwent high-resolution micro-CT scanning, and an additional seven specimens underwent SR-PCI at the Canadian Lightsource in Saskatoon, Canada. One of these specimens was from an individual who had undergone stapes surgery. Data were processed with volume-rendering software to create 3D reconstructions using scalar opacity mapping for bone transparency, cropping, and soft tissue analyses. Results: Micro-CT and SR-PCI with 3D rendering revealed the extensive vascular plexus within the un-decalcified incus bone communicating with the exterior surface. The relationship between the vessels, lenticular process, and incudostapedial joint were clearly observed. SR-PCI allowed for histologic-level detail while preserving the specimen and its 3D relationships. Conclusion: SR-PCI with 3D reconstructions confirmed the main vascular supply to the lenticular process along the intraosseous lenticular vessels. This is the first synchrotron analysis of a patient having undergone stapes surgery, and it suggests that incus necrosis associated with stapes surgery may be caused by a disruption of the lenticular blood flow induced by the prosthesis loop, and not by strangulation of mucosal vessels as has been previously described. Address correspondence and reprint requests to Helge Rask-Andersen, M.D., Ph.D., Department of Surgical Sciences, Head and Neck Surgery, Section of Otolaryngology, and Departments of Otolaryngology, Uppsala University Hospital, SE-751 85, Uppsala, Sweden; E-mail: helge.rask-andersen@surgsci.uu.se H.M.L., H.R.-A., and S.A. are co-senior authors. Funding: This study was supported by ALF grants from Uppsala University Hospital, Uppsala University and by the Foundation of "Tysta Skolan," the Swedish Deafness Foundation (Hörselskadades Riksförbund – HRF), the Ingrid Löwenström Foundation. Part of the research described in this article was performed at the BioMedical Imaging and Therapy (BMIT) facility at the Canadian Light Source, which is funded by the Canada Foundation for Innovation, the Natural Sciences and Engineering Research Council of Canada, the National Research Council Canada, the Canadian Institutes of Health Research, the Government of Saskatchewan, the Western Economic Diversification Canada, and the University of Saskatchewan. 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

From Mid 16th Century French Court to End 18th Century Danish Court: The Fatal Power of the Mammillary Process Objectives/Hypothesis: Two Royal events marked the history of the mastoid process between the 16th and 18th centuries. The first was the death of François II, King of France, following a complication of a chronic running left ear at the age of 16 years, and the second the death of Justus von Berger, Court physician of Christian VII, King of Denmark, after a trepanation of the mastoid process to heal his right-sided deafness at the age of 68 years. The aim of this study is to analyze these two events again with only original references and to replace them in their historical context. Method: Historical review of original literature dealing with the subject. Results/Conclusion: These two Royal events marked the history of the mastoid process, but they were unlikely to have been avoided during their specific era. Medical knowledge was insufficient to avoid these fatal outcomes. Because of their societal impact, they were known of and largely discussed, notably in the medical world, leading to the eventual modification of medical practice during that time. François II's death had no medical impact but von Berger's death practically stopped, for more than a half century, the development of mastoid surgery. Address correspondence and reprint requests to Albert Mudry, M.D., Ph.D., School of Medicine, Stanford University, Stanford, CA 94305-5739; E-mail: amudry@stanford.edu The author discloses no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Datalogging Statistics and Speech Recognition During the First Year of Use in Adult Cochlear Implant Recipients Objective: To examine device datalogging characteristics and postoperative speech recognition performance in adult cochlear implant (CI) recipients. Study Design: Retrospective study examining datalogging characteristics throughout the first year of device use and postoperative speech recognition outcomes measured at 1 year. Patients: One hundred seventy-seven adults who received a Cochlear CI. Main Outcome Measures: Average values for environmental scene classification (hours of device use per day, and percent of hours per day in quiet, noise, and speech) as reported by Cochlear datalogging over the first year of device use. Speech recognition was assessed at 1 year postactivation. Results: During the first year of devices use, CI listeners >80 years of age used their device significantly less (average = 10.97 h/d) than the youngest adult listeners (18–30 yrs), who used their device an average of 13.29 hours/d. There was no consistent effect of age on the number of hours a CI user listened to speech in noise each day. Correlational and regression analyses suggest that the number of average hours of device use is the primary factor that accounts for variance observed in postoperative consonant-nucleus-consonant word scores. Conclusion: Results suggest that the average number of hours listening to speech in noise is not related to postoperative performance, but the average number of hours of device use per day is correlated with postoperative performance. Further research is needed to determine if these findings are merely correlational or causal in nature. Address correspondence and reprint requests to Kara C. Schvartz-Leyzac, Au.D., Ph.D., Hearing Rehabilitation Center, Department of Otolaryngology, University of Michigan Health Systems, 475 West Market Place, Building 1, Suite A, Ann Arbor, MI 48108; E-mail: kleyzac@med.umich.edu The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Episodic Vestibular Symptoms in Children With a Congenital Cytomegalovirus Infection: A Case Series Objective: Congenital cytomegalovirus (cCMV) infection is the most common non-genetic cause of sensorineural hearing loss in children. Although cCMV-induced vestibular loss is demonstrated in several studies, the occurrence of vertigo has been described in only two cases to date. The aim of this paper is to discuss the underlying pathophysiology of recurrent vestibular symptoms in children with cCMV, based on five cases investigated in our center and an extensive research of the literature. Study Design: Retrospective case series. Setting: Tertiary referral center. Patients: This case series describes five pediatric cCMV-patients (three boys, two girls). Four of them were symptomatic at birth, one was asymptomatic. Three patients underwent cochlear implantation. The age of onset of the vestibular symptoms varied from 2;0 to 7;3 years of age. Intervention: None. Main Outcome Measures: Details regarding the patient history and results of cranial imaging, audiological, vestibular, and neurological assessments were collected retrospectively. Results: The selected cases suffered from recurrent vestibular symptoms. All patients had delayed onset, fluctuating, and/or progressive hearing loss. In all cases, the attacks were accompanied with nausea and vomiting and occurred without clear-cut trigger. Migraine and epilepsy often were proposed as first diagnosis, although they could not be confirmed eventually. Four out of five patients were diagnosed with a peripheral vestibular deficit. Conclusions: Diagnosis of vestibular symptoms in children with cCMV is complex, given the multiple morbidities than can occur. Peripheral vestibular causes should be considered in the diagnosis, as important vestibular deficits are demonstrated in this population. Address correspondence and reprint requests to Cleo Dhondt, M.Sc., Department of Head and Skin, Ghent University Hospital, Ghent University, Corneel Heymanslaan 10 (1P1), B – 9000 Ghent, Belgium; E-mail: Cleo.Dhondt@UGent.be This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. The authors disclose no conflicts of interest. Copyright © 2019 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182

6948891480

alsfakia@gmail.com