Otology & Neurotology

Treatment Analysis and Overall Survival Outcomes of Patients With Bilateral Vestibular Schwannoma Objectives: To investigate the clinical presentation, treatment breakdown, and overall survival (OS) outcomes of patients with neurofibromatosis type 2 (NF2)-associated bilateral vestibular schwannoma (NVS). Methods: The 2004 to 2016 National Cancer Database was queried for patients with a diagnosis of VS. The "Laterality" code was used to stratify patients into sporadic unilateral vestibular schwannoma (UVS) and NVS. Results: Of the 33,839 patients with VS, 155 (0.46%) were coded for NVS with an average age and tumor size of 37.4 ± 20.5 years and 23.5 ± 18.2 mm. Patients underwent observation (45.3%), surgery (29.3%), and radiotherapy (20.0%), and had a 5.8% 5-year mortality rate. Compared with UVS, NVS was negatively associated with receiving surgery (40.2% versus 29.3%, p = 0.02) while watchful observation was more prevalent (30.1% versus 45.3%, p = 0.001). In NVS, undergoing surgery was associated with larger tumor size (34.5 ± 21.2 versus 17.8 ± 13.3 mm, p = 0.001) and shorter diagnosis-to-treatment time (49.1 ± 60.6 versus 87.0 ± 78.5 d, p = 0.02), radiotherapy was associated with older age (44.4 ± 18.9 versus 35.2 ± 20.6 yr, p = 0.02) and longer diagnosis-to-treatment time (85.9 ± 77.9 versus 53.9 ± 65.5 d, p = 0.04), and observation was associated with smaller tumor size (17.8 ± 15.9 versus 28.0 ± 19.2 mm, p = 0.01). Kaplan–Meier log-rank analysis demonstrated similar 10-year OS between NVS and UVS patients (p = 0.58) without factoring the earlier age of presentation. Furthermore, there were no temporal changes in presentation/management of NVS, and OS was not dependent on the received treatment (p = 0.30). Conclusions: With younger age, larger tumors, and more conservative management, NVS's OS was not treatment-dependent and was similar to sporadic UVS, though the latter should not be interpreted as similar life expectancies due to the much earlier presentation. Address correspondence and reprint requests to Hamid R. Djalilian, M.D., Division of Neurotology and Skull Base Surgery, Department of Otolaryngology–Head and Neck Surgery, University of California Irvine, 19182 Jamboree Road, Otolaryngology-5386, Irvine, CA 92697; E-mail: hdjalili@hs.uci.edu K.G. and M.A. have contributed equally to this work. Presented as a podium at the 2020 Triological Society: Combined Sections Meeting, Coronado, CA, January 23–25, 2020. Financial Disclosure: M.A. is supported by the National Center for Research Resources and the National Center for Advancing Translational Sciences, National Institutes of Health, through Grant TL1TR001415. The authors disclose no conflicts of interest. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Reliability of Primary Health Care Audiograms by Non-Qualified Examiners—An Analysis of 1,224 Cases Objective: To evaluate the accuracy of primary health care audiograms conducted by non-qualified examiners in a non-standard acoustic environment. Study Design: Retrospective chart review. Setting: Referring primary health care institutions and hospital hearing center. Patients: One thousand two hundred twenty four adult patients evaluated for hearing aid fitting at North Karelia Central hospital in years 2017 and 2018. Main Outcome Measures: Intraclass correlation coefficient (ICC), mean threshold differences, and mean absolute errors were used to assess agreement between primary health care and hospital audiograms. Primary health care audiometry sensitivity, specificity and positive (PPV) and negative (NPV) predictive values were calculated for hearing aid candidacy in general and open ear mold candidacy in particular. Results: ICC for both better ear hearing level (BEHL0.5,1,2,4  kHz) and pure-tone average (PTA0.5,1,2,4  kHz) in frequencies 0.5, 1, 2, and 4 kHz was 0.82, and in individual full octave frequencies from 0.125 to 8 kHz ranged from 0.70 to 0.83. Mean threshold differences in BEHL0.5,1,2,4  kHz and PTA0.5,1,2,4  kHz were 1.8 and 1.6 dB and mean absolute errors 4.9 and 5.3 dB, respectively. Sensitivity for hearing aid candidacy was 0.97, specificity 0.58, PPV 0.92, and NPV 0.79. Conclusions: Primary health care audiometry is reasonably accurate, allowing preselection of patients to adequate hearing care pathways. Address correspondence and reprint requests to Jukka Kokkonen, M.D., Department of Otorhinolaryngology, North Karelia Central Hospital, Tikkamäentie 16, 80210 Joensuu, Finland; E-mail: jukka.kokkonen@siunsote.fi Sources of support or funding received: none. The authors disclose no conflicts of interest. 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 © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Cochlear Implant Outcomes in Neurofibromatosis Type 2: Implications for Management Objective: To describe our institutional experience with cochlear implantation (CI) for rehabilitation of hearing loss in Neurofibromatosis type 2 (NF2) patients. Study Design: Retrospective review between 1989 and 2019. Setting: Tertiary-care center. Patients: Twenty-four patients (67% female, mean age 45.6years) with NF2. Management of their ipsilateral vestibular schwannoma included microsurgery (n=12), stereotactic radiation (n=5), and observation (n=7). Interventions: Cochlear implantation. Main Outcome Measures: Ability to obtain open-set speech, daily device usage and long-term device benefit. Results: All patients achieved some degree of sound awareness with CI. Nineteen patients (79%) achieved open-set speech understanding with a mean word-recognition score of 43% (range 0-88%). Patients with tumors 1.5 cm or less demonstrated the better speech understanding, without significant differences among treatment modalities. For tumors greater than 1.5 cm, patients who underwent microsurgery had a lower rate of open-set speech understanding compared to those treated with radiation or observation. Regular daily device use in 83% of patients was found. Long-term use (>10years) was observed in several patients, though some ultimately required reimplantation with an auditory brainstem implant due to progressive tumor growth. Mean follow-up duration was 4.1 years (range 0.4-15). Conclusions: Cochlear implantation can be an effective treatment for hearing loss in NF2 patients provided the cochlear nerve is intact, regardless of prior management for the ipsilateral tumor. The degree of benefit varies and is influenced by tumor size. Management strategies that preserve the cochlear nerve maximize the interval during which a CI could be of benefit to NF2 patients. Address correspondence and reprint requests to David R. Friedmann, M.D., M.Sc., Department of Otolaryngology, New York University School of Medicine, 530 1st Avenue Suite 7Q, New York, NY 10016; E-mail: david.friedmann@nyulangone.org The following manuscript was presented in oral form at the Annual North American Skull Base Society Meeting, February 9, 2020 in San Antonio, TX. J.T.R. and W.H.S. are consultants for Cochlear Pty Limited and receive research support. Institutional review board approval: New York University IRB Approval #i19-01139. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Cochlear Implantation in Sporadic Vestibular Schwannoma and Other Retrocochlear Pathology: A Case Series Objective: To describe outcomes with cochlear implantation (CI) for rehabilitation of hearing loss in patients with sporadic vestibular schwannomas (VS) and other retrocochlear pathologies. Study Design: Retrospective review. Setting: Tertiary-care center. Patients: Twenty three cases in 19 patients (53% men, mean age 55.8 yr) with non-neurofibromatosis type 2 related retrocochlear pathology. Interventions: Unilateral or bilateral CI. Main Outcome Measures: Word recognition score, device usage. Results: Etiology of deafness included sporadic VS (n = 9, 39%), radiation after head and neck or central nervous system (CNS) malignancy (n = 8, 35%), superficial siderosis (n = 3, 13%), neurosarcoidosis (n = 2, 9%), and pontine stroke (n = 1, 4%). Mean follow-up duration was 2.3 years (standard deviation [SD] 3.0; range, 0.2–9.4). Auditory perception was achieved in 20 out of 22 patients (91%) who have been activated. Mean WRS in patients with sporadic VS was 18% (SD 20; range, 0–44). Mean WRS in patients with non-VS retrocochlear pathology was 55% (SD 30; range, 0–94). Data logs showed 7.0 h/d of average use (SD 4.3; range, 0–13). Conclusions: Appropriately selected patients with retrocochlear pathology may benefit from CI so long as the patient has a cochlear fluid signal and an intact cochlear nerve. Patients with sporadic VS patients and normal contralateral hearing exhibited guarded outcomes with CI, whereas most patients with non-VS retrocochlear pathologies demonstrated open-set speech understanding scores comparable to or slightly worse than conventional CI candidates. Since variable performance benefit is observed with CI in patients with retrocochlear pathology, counseling is imperative to align patient expectations with realistic outcomes. Address correspondence and reprint requests to John Thomas Roland, M.D., Department of Otolaryngology–Head & Neck Surgery, New York University School of Medicine, 550 1st Avenue, New York, NY 10016; E-mail: J.Thomas.RolandJr@nyulangone.edu Institutional Review Board Approval: New York University IRB Approval #i19-01139. Conflict(s) of Interest to Declare: J.T.R. and S.O.M. are consultants for Cochlear Pty Limited and receive research support. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Increased Radiosurgery Toxicity Associated With Treatment of Vestibular Schwannoma in Multiple Sclerosis Objective: Explore the risk of radiation-induced neurotoxicity in patients with multiple sclerosis (MS) treated with stereotactic radiosurgery (SRS) and better understand the pathophysiology of radiation-induced injury in the central nervous system (CNS). Patients/Intervention: We present the clinical course and magnetic resonance imaging (MRI) findings of a 52-year-old woman with a history of relapsing remitting MS, who developed radiation-induced neurotoxicity following CyberKnife SRS (25 Gy in five fractions) for a left-sided vestibular schwannoma (VS). Main Outcome Measure: Risk of radiation-induced damage following SRS to the CNS, including radiation type and dose, toxicity, and time to symptom onset, in patients with MS. Results: Our patient developed increased imbalance (grade 2 toxicity) 3 months following CyberKnife SRS. Brain MRI showed new fluid-attenuated inversion recovery (FLAIR) hyperintensity in the pons and cerebellum. Neurotoxicity from SRS is rare. However, our literature review showed that 19 patients with MS who underwent intracranial radiation therapy sustained radiation-induced toxicity. The potential mechanisms for increased toxicity in MS could be due to a combination of demyelination, inflammatory, and/or vascular changes. Efficacy of treatments including steroids, bevacizumab, and hyperbaric oxygen therapy is currently unknown. Conclusion: Treatment options of SRS and surgery for VS should be carefully considered as patients with known MS may be at increased risk for radiation-induced damage following SRS to the CNS. Thoughtful radiosurgical planning and dosing accounting for this inherent risk is essential for managing patients with MS and VS. Address correspondence and reprint requests to Katherine Wallerius, M.D., Department of Otolaryngology—Head and Neck Surgery, Medstar Georgetown University Hospital, 3800 Reservoir Road NW, Building 1, Washington, DC 20007; E-mail: kpw30@georgetown.edu Conflicts of Interest and Source Funding: No funding organization contributed to the design or conduct of this study; collection, management, analysis, or interpretation of the data; or preparation, review, or decision to submit this article for publication. The authors have no funding, financial relationships, or conflicts of interest to disclose. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Effects of Presentation Level on Spatial Hearing With and Without Bone-Conduction Amplification in Congenital Unilateral Aural Atresia Objective: This study assessed the effect of ipsilateral bone-conduction amplification on spatial hearing abilities in subjects with congenital unilateral aural atresia (CUAA). Patients: Twelve patients with unilateral conductive hearing loss secondary to CUAA and normal hearing in the contralateral ear were tested. Most (75%) had limited experience with a bone-conduction hearing aid (BCHA). Intervention: Performance was evaluated with and without a BCHA fitted acutely on a softband. Main Outcome Measures: Spatial hearing abilities were evaluated in two paradigms. Spatial release from masking was evaluated by comparing masked sentence recognition with a target and two speech maskers either colocated at 0 degree or with the maskers separated at +90 degrees and −90 degrees. Sound source localization was evaluated in a 180 degrees arc of loudspeakers on the horizontal plane. Performance was evaluated at 50 and 75 dB SPL, and results were compared for patients tested with and without a BCHA. Results: Group level results indicate similar spatial release from masking in the aided and unaided conditions at both presentation levels. Localization at 50 dB SPL was similar aided and unaided, but at 75 dB SPL the root mean square error was lower unaided than aided (17.2 degrees vs 41.3 degrees; p = 0.010). Conclusions: Use of a BCHA in patients with CUAA may interfere with auditory cues required for sound source localization when the signal level is intense enough to overcome the patient's conductive hearing loss. These findings have potential clinical implications in fitting of BCHAs to support optimal spatial hearing in patients with CUAA. Address correspondence and reprint requests to Michael W. Canfarotta, M.D., Department of Otolaryngology/Head and Neck Surgery, Houpt Building, Room G-190, 170 Manning Drive, CB 7070, Chapel Hill, NC 27599-7070; E-mail: michael.canfarotta@unchealth.unc.edu M.W.C., S.L.G.K., and E.B. designed experiments, wrote the paper, and contributed significantly to analysis and revisions leading to its final form. This project was funded in part by the NIH through NIDCD (T32 DC005360 and R01 000397). Portions of these data were presented at the 7th International Congress on Bone Conduction Hearing and Related Technologies in December 2019 in Miami, FL. The authors have no conflicts of interest to disclose. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Transcanal Endoscopic Management of Glomus Tympanicum: Multicentric Case Series Objective: The aim of this study was to report a multicentric surgical experience in the exclusive endoscopic management of glomus tympanicum (GT). Study Design: Retrospective case series review at two institutions. Setting: Tertiary referral centers. Patients: The study included 30 patients who underwent exclusive transcanal excision of GT between 2010 and 2017 at the two referral centers. Interventions: Exclusive endoscopic transcanal excision of GT type A1, A2, and B1 (modified Fisch-Mattox classification). All surgical procedures were performed by two senior surgeons (L.P.; M.B.). Main Outcome Measures: For each procedure, intraoperative features of the disease, postoperative complications, and functional outcomes were evaluated. Recurrent or residual diseases were clinically and radiologically assessed during the follow-up period. Results: None of the patients treated with transcanal endoscopic approach (TEA) experienced intraoperative complications, nor required conversion to microscopic approach. Gross total resection (GTR) was obtained in 90% of the cases, while a near total resection was advocated when the residual pathology had a close relationship with the internal carotid artery. Mean hospitalization time was 1.6 (±0.8 SD) days and no postoperative complications were reported. No recurrences were reported in the GTR group after a mean follow-up period of 38.1 (±28.7 SD) months. Conclusions: Middle ear paragangliomas with no mastoid involvement (Class A1, A2, and B1) can be safely managed by means of a transcanal endoscopic approach. Low rate of postoperative complications, short hospitalization, and high rate of gross total resection demonstrate that TEA is a safe and effective procedure. Address correspondence and reprint requests to Matteo Fermi, M.D., Department of Otorhinolaryngology, Head & Neck Surgery, University Hospital of Modena, Modena, Italy; E-mail: matteo.fermi.med@gmail.com Funding Disclosures: No funding is reported for the present study. The authors disclose no conflicts of interest. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Price Differences Between Otic and Ophthalmic Drops Objective: Otic drops are one of the most frequently prescribed medications in otolaryngology. However, some forms of these drops can be very expensive, and ophthalmic formulations are sometimes used by practitioners to decrease the cost for their patients. The goal of this study is to determine the cost differential between otic and ophthalmic drops. Methods: Pharmacies in New York City, Chicago, and Portland were surveyed in the span of 6 weeks by telephone to evaluate the current prices of various otic and ophthalmic drops commonly prescribed by otolaryngologists. Results: A total of 83 pharmacies were surveyed. Surveyed drugs included ciprofloxacin/dexamethasone (cip/dex) otic, neomycin/polymyxin B sulfates/hydrocortisone (neo/poly/HC) otic, acetic acid (AA) otic, acetic acid/hydrocortisone (AA/HC) otic, ofloxacin otic, ofloxacin ophthalmic, and tobramycin/dexamethasone (tob/dex) ophthalmic. Per milliliter, cip/dex otic was consistently the most expensive, with a median price of $38.00 per milliliter. Among the antibiotic drops, neo/poly/HC otic was the least expensive formulation with a median price of $11.60 per milliliter. Ofloxacin ophthalmic was significantly less expensive than the otic formulation, at $11.00 and $31.00 per milliliter, respectively (adjusted p < 0.001). In general, otic and ophthalmic formulations were not significantly different according to manufacturing information, although ophthalmic drops were more often packaged in a sterile fashion and with less abrasive ingredients for use in the more sensitive tissue of the eye. Conclusion: Significant and meaningful price differences exist between otic and ophthalmic drops. Given the safety of ophthalmic drops used in the ears, they may be a more cost-effective alternative to traditionally prescribed otic drops when clinically appropriate. Address correspondence and reprint requests to Lawrence Lustig, M.D., 180 Fort Washington Avenue, New York, NY 10032; E-mail: lrl2125@cumc.columbia.edu Funding: None. The authors disclose no conflicts of interest. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Cool OtOprotective Ear Lumen (COOL) Therapy for Cisplatin-induced Hearing Loss Hypothesis: Localized cooling of the external ear has a protective effect on the susceptibility to cisplatin-induced hearing loss. Background: We previously demonstrated significant protection from cisplatin-induced hearing loss using cool water ear canal irrigation. However, the study was limited to a single bolus injection of cisplatin and an acute time period. Here, we examined the application of localized cooling of the ear canal with repeated doses of cisplatin, over an expanded period of time, and using two methods of cooling. Methods: Twenty-four guinea pigs (12 male and 12 female) underwent auditory physiological testing (auditory brainstem response and distortion product otoacoustic emissions at 8–32 kHz) and pre/postadministration of cisplatin. Cisplatin (4 mg/kg i.p.) was administered in 3 weekly single injections for a total of 12 mg/kg. While anesthetized, the left ears of the guinea pigs were exposed to either cool water (22°C; ICS Water Caloric Irrigator), a cool ear bar (15°C, cooled by a Peltier device; TNM, Scion NeuroStim), or left uncooled as a sham control. The animals were tested 3 days post each dosage and 1 month post the final dose. At the end of the experiment the animals were euthanized for histological evaluation. Results: We found that hearing loss was significantly reduced, and hair cell survival greatly improved, in animals that received cooling treatments compared to cisplatin-only control animals. No significant difference was observed between the two methods of cooling. Conclusion: Localized cooling of the ear canal during administration of cisplatin mitigated loss of auditory function and loss of hair cells. Address correspondence and reprint requests to Christopher Spankovich, University of Mississippi Medical Center, Jackson, MS, 39216; E-mail: cspankovich@umc.edu This work was funded by the University of Mississippi Medical Center, American Otological Society, an ONR N00014-18-1-2716. The authors disclose no conflicts of interest. 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 © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

Failure and Revision Surgery After Cochlear Implantation in the Adult Population: A 10-year Single-institution Retrospective and Systematic Review of the Literature Objective: To characterize failure rate and etiology after cochlear implantation; to identify predictors and describe outcomes after implant failure. Study Design: Retrospective chart review and systematic review of the literature using PubMed and Embase. Setting: Academic Cochlear Implant Center. Subject Population: Four hundred ninety-eight devices in 439 distinct adult patients. Interventions: Unilateral or bilateral cochlear implantation. Main Outcome Measures: Implant failure rate and etiology. Results: A total of 32 devices (5.9%) failed in 31 patients encompassing the following failure types in accordance with the European Consensus Statement of Cochlear Implants: 17 device failures (53.1%), 11 failures due to performance decrement/adverse reactions (34.4%), and 4 medical reasons (12.9%). There was no significant difference in age, sex, or manufacturer between patients with and without failures. Twenty-five percent of patients with failure leading to explantation had childhood onset of deafness compared to 12.1% of patients with adult-onset hearing loss (OR = 2.42; p = 0.04). Performance decrement/adverse reaction patients had an older average age at implantation compared to device failure patients (mean 68.5 yr 95% CI: 59.9–77.1 vs mean 47.6 yr, CI: 39.9–55.3, p < 0.01). There was no significant difference in time to failure, sex, or device manufacturer between the different types of failures. Twenty-nine patients who experienced CI failure underwent a revision surgery, while the remaining two opted for explantation without reimplantation. One patient who underwent revision surgery subsequently presented with a second failure and underwent a second revision, which was successful. In our systematic review, 815 citations were reviewed, and 9 studies were selected for inclusion. Overall failure rate across all studies was 5.5%. Device failure was the leading cause of failure in the majority (6/9) of studies, accounting for 40.8% of all failures. Medical reasons were the second leading cause at 33.6%, followed by performance decrement/adverse reaction (20.9%) and other (4.8%). Conclusions: Cochlear implant failure is a rare phenomenon. Childhood-onset of hearing loss appears to be associated with an increased risk of overall failure. Older patients are at increased risk for performance decrement/adverse reaction. Revision surgery success rates remain very high and patients with failure of any cause should be offered explantation with concurrent reimplantation. Address correspondence and reprint requests to Michael J. Ruckenstein, M.D., Vice Chairman, Department of Otolaryngology-Head and Neck Surgery, Hospital of the University of Pennsylvania, 3400 Spruce Street, Silverstein 5, Philadelphia, PA; E-mail: michael.ruckenstein@pennmedicine.upenn.edu The authors disclose no conflicts of interest. Copyright © 2020 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

#

Medicine by Alexandros G. Sfakianakis,Anapafseos 5 Agios Nikolaos 72100 Crete Greece,00302841026182,00306932607174,alsfakia@gmail.com,

Telephone consultation 11855 int 1193,