{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T02:05:47Z","timestamp":1785377147977,"version":"3.55.0"},"reference-count":50,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","license":[{"start":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T00:00:00Z","timestamp":1638316800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"<jats:sec>\n            <jats:title>Objective:<\/jats:title>\n            <jats:p>To assess outcomes in autoimmune inner ear disease (AIED) after cochlear implantation (CI) through systematic review and meta-analysis.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Databases Reviewed:<\/jats:title>\n            <jats:p>PubMed, MedLine, Embase, and CINAHL<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods:<\/jats:title>\n            <jats:p>Databases were queried for inclusion of AIED patients who underwent CI with outcomes recorded \u22653 months postoperatively. We examined demographics, pure-tone average (PTA), speech perception, preoperative imaging, intraoperative management, and postoperative complications.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results:<\/jats:title>\n            <jats:p>Twenty-six articles encompassing 124 patients met inclusion criteria. Mean implantation age was 26.2 years (range 4\u201365 years) with average length of follow-up at 28.2 months (range 3\u2013120 months). Meta-analysis demonstrated significant improvement in speech perception following CI. There was a statistically significant improvement in speech recognition score (SRS) (standard mean difference [SMD] = 6.5, 95% confidence interval [CI], 4.8-8.0, <jats:italic toggle=\"yes\">P<\/jats:italic> &lt; 0.0001) as well as word recognition score (WRS) (SMD = 5.5, 95% CI, 4.2-6.8, <jats:italic toggle=\"yes\">P<\/jats:italic> &lt; 0.0001) after CI. Anomalous preoperative radiologic manifestations were reported by 57.7% (15\/26) studies. Disease activity\u2013related intraoperative adjustment was noted in 57.7% (15\/26) studies; common consequences were cochlear drill-out (53.3%), difficult round window insertion (26.7%), and scala vestibuli insertion (26.7%). Frequent postoperative complications noted in 26.9% (7\/26) studies included systemic AIED flares (71.4%) and wound healing delay (42.9%).<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusion:<\/jats:title>\n            <jats:p>Findings of this systematic review of AIED cochlear implant literature demonstrate a lack of consistent reporting standards for PTA and speech perception as well as a lack of long-term follow-up. Despite these findings, meta-analysis suggests that CI is a viable treatment for improving speech perception in AIED patients.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1097\/ono.0000000000000006","type":"journal-article","created":{"date-parts":[[2022,8,5]],"date-time":"2022-08-05T20:02:31Z","timestamp":1659729751000},"page":"e006","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":4,"title":["Cochlear Implantation and Perioperative Management in Autoimmune Inner Ear Disease"],"prefix":"10.1097","volume":"1","author":[{"given":"Nikita","family":"Deshpande","sequence":"first","affiliation":[{"name":"Georgetown University School of Medicine, Washington, DC"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathan","family":"Aminpour","sequence":"additional","affiliation":[{"name":"Georgetown University School of Medicine, Washington, DC"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hui","family":"Cheng","sequence":"additional","affiliation":[{"name":"Bioinformatics and Biostatistics Collaboration Core, National Institute on Deafness and Other Communication Disorders, NIH, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Dixon","family":"Johns","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2014Head and Neck Surgery, MedStar Georgetown University Hospital, Washington, DC."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Hoa","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2014Head and Neck Surgery, MedStar Georgetown University Hospital, Washington, DC."},{"name":"Auditory Development and Restoration Program, Otolaryngology Surgeon-Scientist Program, National Institute on Deafness and Other Communication Disorders, NIH, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2021,12]]},"reference":[{"key":"R1-20241014","doi-asserted-by":"crossref","first-page":"426","DOI":"10.1097\/01.moo.0000136101.95662.aa","article-title":"Autoimmune inner ear disease.","volume":"12","author":"Ruckenstein","year":"2004","journal-title":"Curr Opin Otolaryngol Head Neck Surg"},{"issue":"5 Pt 1","key":"R2-20241014","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1177\/000348947908800501","article-title":"Autoimmune sensorineural hearing loss.","volume":"88","author":"McCabe","year":"1979","journal-title":"Ann Otol Rhinol Laryngol"},{"key":"R3-20241014","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1111\/coa.12432","article-title":"Current understanding of the pathogenesis of autoimmune inner ear disease: a review.","volume":"40","author":"Goodall","year":"2015","journal-title":"Clin Otolaryngol"},{"key":"R4-20241014","first-page":"157","article-title":"Cochlear implantation in a patient with Cogan\u2019s syndrome, chronic ear disease and on steroid therapy.","volume":"57","author":"Low","year":"2000","journal-title":"Adv Otorhinolaryngol"},{"key":"R5-20241014","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1080\/14670100.2018.1548077","article-title":"An unusual case of post-cochlear implant performance degradation in a patient with suspected Cogan\u2019s syndrome.","volume":"20","author":"Low","year":"2019","journal-title":"Cochlear Implants Int"},{"key":"R6-20241014","doi-asserted-by":"crossref","first-page":"2557","DOI":"10.1002\/lary.23604","article-title":"Spectrum of immune-mediated inner ear disease and cochlear implant results.","volume":"122","author":"Malik","year":"2012","journal-title":"Laryngoscope"},{"key":"R7-20241014","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1097\/00129492-200307000-00012","article-title":"Cochlear implantation and Cogan syndrome.","volume":"24","author":"Pasanisi","year":"2003","journal-title":"Otol Neurotol"},{"key":"R8-20241014","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1080\/00016480260094974","article-title":"Cochlear implants in systemic autoimmune vasculitis syndromes.","author":"Quaranta","year":"2002;548","journal-title":"Acta Otolaryngol Suppl"},{"key":"R9-20241014","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1007\/s00296-009-1259-y","article-title":"Cochlear implantation in a bilateral sensorineural hearing loss patient with relapsing polychondritis.","volume":"32","author":"Seo","year":"2012","journal-title":"Rheumatol Int"},{"key":"R10-20241014","doi-asserted-by":"crossref","first-page":"770","DOI":"10.3766\/jaaa.16.10.2","article-title":"Considerations for cochlear implantation of children with sudden, fluctuating hearing loss.","volume":"16","author":"Sweetow","year":"2005","journal-title":"J Am Acad Audiol"},{"key":"R11-20241014","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1097\/MAO.0000000000001613","article-title":"Cochlear implantation in cochlear ossification: retrospective review of etiologies, surgical considerations, and auditory outcomes.","volume":"39","author":"Vashishth","year":"2018","journal-title":"Otol Neurotol"},{"key":"R12-20241014","doi-asserted-by":"crossref","first-page":"2478","DOI":"10.1002\/lary.21060","article-title":"Cochlear implantation in patients with autoimmune inner ear disease including cogan syndrome: a comparison with age- and sex-matched controls.","volume":"120","author":"Wang","year":"2010","journal-title":"Laryngoscope"},{"key":"R13-20241014","doi-asserted-by":"crossref","first-page":"1875","DOI":"10.1001\/jama.290.14.1875","article-title":"Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial.","volume":"290","author":"Harris","year":"2003","journal-title":"JAMA"},{"key":"R14-20241014","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1016\/j.semarthrit.2003.10.002","article-title":"Autoimmune sensorineural hearing loss: clinical course and treatment outcome.","volume":"34","author":"Loveman","year":"2004","journal-title":"Semin Arthritis Rheum"},{"key":"R15-20241014","first-page":"591","article-title":"Autoimmune inner ear disease: clinical and laboratory findings and treatment outcome.","volume":"37","author":"Dayal","year":"2008","journal-title":"J Otolaryngol Head Neck Surg"},{"key":"R16-20241014","doi-asserted-by":"crossref","first-page":"1337","DOI":"10.1097\/MAO.0b013e3181f0c699","article-title":"Cochlear implantation outcomes in patients with autoimmune and immune-mediated inner ear disease.","volume":"31","author":"Aftab","year":"2010","journal-title":"Otol Neurotol"},{"key":"R17-20241014","doi-asserted-by":"crossref","first-page":"e694","DOI":"10.1097\/MAO.0000000000002262","article-title":"Bilateral cochlear implantation in Vogt-Koyanagi-Harada syndrome: a case report.","volume":"40","author":"AlHelali","year":"2019","journal-title":"Otol Neurotol"},{"key":"R18-20241014","doi-asserted-by":"crossref","first-page":"3201","DOI":"10.1007\/s00405-014-3376-9","article-title":"Cochlear implantation in patients with Cogan syndrome: long-term results.","volume":"272","author":"Bacciu","year":"2015","journal-title":"Eur Arch Otorhinolaryngol"},{"key":"R19-20241014","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1179\/1754762811Y.0000000020","article-title":"Surgical aspects of cochlear implantation in syndromic children.","volume":"13","author":"Bajaj","year":"2012","journal-title":"Cochlear Implants Int"},{"key":"R20-20241014","doi-asserted-by":"crossref","first-page":"494","DOI":"10.3109\/00016489.2010.535214","article-title":"Cochlear implant in Cogan syndrome.","volume":"131","author":"Bovo","year":"2011","journal-title":"Acta Otolaryngol"},{"key":"R21-20241014","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1177\/0003489420938827","article-title":"Cochlear implantation as a treatment for sudden autoimmune sensorineural hearing loss in a patient affected by eosinophilic granulomatosis with polyangiitis: a case report and a review of literature.","volume":"130","author":"Cacco","year":"2021","journal-title":"Ann Otol Rhinol Laryngol"},{"key":"R22-20241014","first-page":"7395460","article-title":"Cochlear ossification in a patient with Cogan\u2019s syndrome undergoing bilateral cochlear implantation.","volume":"2018","author":"Cassis","year":"2018","journal-title":"Case Rep Otolaryngol"},{"key":"R23-20241014","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1017\/S002221510999137X","article-title":"Sweet\u2019s disease and profound, bilateral, sensorineural hearing loss.","volume":"124","author":"Cheng","year":"2010","journal-title":"J Laryngol Otol"},{"key":"R24-20241014","doi-asserted-by":"crossref","first-page":"928","DOI":"10.1017\/S002221510013899X","article-title":"Cochlear implantation in patients suffering from Cogan\u2019s syndrome.","volume":"111","author":"Cinamon","year":"1997","journal-title":"J Laryngol Otol"},{"key":"R25-20241014","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1177\/0194599820902387","article-title":"Metachronous sudden sensorineural hearing loss: patient characteristics and treatment outcomes.","volume":"162","author":"Cooper","year":"2020","journal-title":"Otolaryngol Head Neck Surg"},{"key":"R26-20241014","doi-asserted-by":"crossref","first-page":"241","DOI":"10.3109\/16513860903076407","article-title":"A rare association between neurofibromatosis type I and Cogan\u2019s syndrome: case report.","volume":"7","author":"Forli","year":"2009","journal-title":"Audiol Med"},{"key":"R27-20241014","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1017\/S0022215107008742","article-title":"Side selection for cochlear implantation in a case of Cogan\u2019s syndrome.","volume":"122","author":"Im","year":"2008","journal-title":"J Laryngol Otol"},{"key":"R28-20241014","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1159\/000477534","article-title":"Histopathology of the human inner ear in the Cogan syndrome with cochlear implantation.","volume":"22","author":"Kamakura","year":"2017","journal-title":"Audiol Neurootol"},{"key":"R29-20241014","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1016\/j.anl.2010.04.005","article-title":"Cochlear implantation in a patient with atypical Cogan\u2019s syndrome complicated with hypertrophic cranial pachymeningitis.","volume":"37","author":"Kawamura","year":"2010","journal-title":"Auris Nasus Larynx"},{"key":"R30-20241014","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1159\/000318870","article-title":"Long-term evaluation of Cochlear implantation in Cogan syndrome.","volume":"72","author":"Kontorinis","year":"2010","journal-title":"ORL J Otorhinolaryngol Relat Spec"},{"key":"R31-20241014","doi-asserted-by":"crossref","first-page":"n71","DOI":"10.1136\/bmj.n71","article-title":"The PRISMA 2020 statement: an updated guideline for reporting systematic reviews.","volume":"372","author":"Page","year":"2021","journal-title":"BMJ"},{"key":"R37-20241014","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1136\/ebmental-2019-300117","article-title":"How to perform a meta-analysis with R: a practical tutorial.","volume":"22","author":"Balduzzi","year":"2019","journal-title":"Evid Based Ment Health"},{"key":"R38-20241014","doi-asserted-by":"crossref","first-page":"e168","DOI":"10.1097\/MAO.0000000000002933","article-title":"Outcome of cochlear implantation in NLRP3-related autoinflammatory inner ear disorders.","volume":"42","author":"Kim","year":"2021","journal-title":"Otol Neurotol"},{"key":"R39-20241014","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1179\/1754762811Y.0000000005","article-title":"Cochlear implantation in a child with CINCA syndrome who also has wide vestibular aqueducts.","volume":"13","author":"Bates","year":"2012","journal-title":"Cochlear Implants Int"},{"key":"R40-20241014","doi-asserted-by":"crossref","first-page":"916","DOI":"10.1001\/jamaoto.2020.1584","article-title":"Assessment of speech understanding after cochlear implantation in adult hearing aid users: a nonrandomized controlled trial.","volume":"146","author":"Buchman","year":"2020","journal-title":"JAMA Otolaryngol Head Neck Surg"},{"key":"R41-20241014","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1044\/jshr.2103.507","article-title":"Speech-discrimination scores modeled as a binomial variable.","volume":"21","author":"Thornton","year":"1978","journal-title":"J Speech Hear Res"},{"key":"R42-20241014","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1007\/BF02439981","article-title":"Cochlear implantation in patients with Cogan\u2019s syndrome: a review of four cases.","volume":"254","author":"Minet","year":"1997","journal-title":"Eur Arch Otorhinolaryngol"},{"key":"R43-20241014","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1159\/000343189","article-title":"Factors affecting auditory performance of postlinguistically deaf adults using cochlear implants: an update with 2251 patients.","volume":"18","author":"Blamey","year":"2013","journal-title":"Audiol Neurootol"},{"key":"R44-20241014","doi-asserted-by":"crossref","first-page":"1062","DOI":"10.1093\/brain\/123.5.1062","article-title":"The neurocognition of language.","volume":"123","author":"Gerhand","year":"2000","journal-title":"Brain"},{"issue":"3 Pt 1","key":"R45-20241014","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1097\/00005537-200003000-00024","article-title":"Characterization of an experimentally induced inner ear immune response.","volume":"110","author":"Ma","year":"2000","journal-title":"Laryngoscope"},{"key":"R46-20241014","doi-asserted-by":"crossref","first-page":"836","DOI":"10.1055\/s-2004-826004","article-title":"[Obliterated cochlea in Cogan\u2019s syndrome \u2013 implications for cochlear implant surgery].","volume":"83","author":"Aschendorff","year":"2004","journal-title":"Laryngorhinootologie"},{"key":"R47-20241014","first-page":"360","article-title":"Scala vestibuli cochlear implantation for labyrinthine ossification.","volume":"11","author":"Steenerson","year":"1990","journal-title":"Am J Otol"},{"key":"R48-20241014","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1111\/j.1749-6632.1997.tb51890.x","article-title":"Systemic autoimmune diseases associated with hearing loss.","volume":"830","author":"Arnold","year":"1997","journal-title":"Ann N Y Acad Sci"},{"key":"R49-20241014","first-page":"490","article-title":"Labyrinthine ossification: differences between two types of ectopic bone.","volume":"6","author":"Kotzias","year":"1985","journal-title":"Am J Otol"},{"issue":"5 Pt 1","key":"R50-20241014","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1002\/lary.5541040510","article-title":"Temporal bone imaging for cochlear implantation.","volume":"104","author":"Seidman","year":"1994","journal-title":"Laryngoscope"},{"issue":"6_suppl","key":"R51-20241014","doi-asserted-by":"crossref","first-page":"38S","DOI":"10.1177\/0003489419834311","article-title":"Cochlear implant surgery through round window approach is always possible.","volume":"128","author":"Bae","year":"2019","journal-title":"Ann Otol Rhinol Laryngol"},{"key":"R52-20241014","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1097\/00129492-200205000-00019","article-title":"Nucleus multichannel cochlear implantation in partially ossified cochleas using the Steenerson procedure.","volume":"23","author":"Bacciu","year":"2002","journal-title":"Otol Neurotol"},{"key":"R53-20241014","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1177\/0194599818764329","article-title":"Minimum reporting standards for adult cochlear implantation.","volume":"159","author":"Adunka","year":"2018","journal-title":"Otolaryngol Head Neck Surg"},{"key":"R54-20241014","doi-asserted-by":"crossref","first-page":"1845","DOI":"10.1002\/lary.29393","article-title":"Cochlear implantation in meniere\u2019s disease: a systematic review and meta-analysis.","volume":"131","author":"Villavisanis","year":"2021","journal-title":"Laryngoscope"},{"key":"R55-20241014","doi-asserted-by":"crossref","first-page":"3554","DOI":"10.1044\/2019_JSLHR-H-19-0142","article-title":"Cochlear implant quality of life (CIQOL): development of a profile instrument (CIQOL-35 profile) and a global measure (CIQOL-10 Global).","volume":"62","author":"McRackan","year":"2019","journal-title":"J Speech Lang Hear Res"}],"container-title":["Otology &amp; Neurotology Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.lww.com\/10.1097\/ONO.0000000000000006","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,15]],"date-time":"2024-10-15T00:10:32Z","timestamp":1728951032000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/10.1097\/ONO.0000000000000006"}},"subtitle":["A Systematic Review and Meta-Analysis"],"short-title":[],"issued":{"date-parts":[[2021,12]]},"references-count":50,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2021]]}},"URL":"https:\/\/doi.org\/10.1097\/ono.0000000000000006","relation":{},"ISSN":["2766-3604"],"issn-type":[{"value":"2766-3604","type":"print"}],"subject":[],"published":{"date-parts":[[2021,12]]}}}