{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,1]],"date-time":"2026-11-01T01:20:05Z","timestamp":1793496005980,"version":"4.5.0"},"reference-count":30,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2022,10,17]],"date-time":"2022-10-17T00:00:00Z","timestamp":1665964800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background and Objectives: Many otologists face a dilemma in the decision-making process of surgical management of patients with cochlear nerve (CN) aplasia. The goal of this study is to provide fresh evidence on cochlear implantation (CI) results in patients with CN aplasia. Materials and Methods: We scrutinized functional outcomes in 37 ears of 21 children with bilateral CN aplasia who underwent unilateral or bilateral CI based on cross-sectional and longitudinal assessments. Results: The Categories of Auditory Performance (CAP) scores gradually improved throughout the 3-year follow-up; however, variable outcomes existed between individuals. Specifically, 90% of recipients with a 1-year postoperative CAP score \u22641 could not achieve a CAP score over 1 even at 3-year postoperative evaluation, while the recipients with a 1-year postoperative CAP score &gt;1 had improved auditory performance, and 72.7% of them were able to achieve a CAP score of 4 or higher. Meanwhile, intraoperative electrically evoked compound action potential was not correlated with postoperative CAP score. Conclusions: Our results further refine previous studies on the clinical feasibility of CI as the first treatment modality to elicit favorable auditory performance in children with CN aplasia. However, special attention should be paid to pediatric patients with an early postoperative CAP score \u22641 for identification of unsuccessful cochlear implants and switching to auditory brainstem implants.<\/jats:p>","DOI":"10.3390\/medicina58101474","type":"journal-article","created":{"date-parts":[[2022,10,17]],"date-time":"2022-10-17T21:11:51Z","timestamp":1666041111000},"page":"1474","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Functional Outcomes of Cochlear Implantation in Children with Bilateral Cochlear Nerve Aplasia"],"prefix":"10.3390","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3839-2324","authenticated-orcid":false,"given":"Goun","family":"Choe","sequence":"first","affiliation":[{"name":"Department of Otolaryngology-Head and Neck Surgery, Chungnam National University Sejong Hospital, Chungnam National University College of Medicine, Sejong 30099, Korea"},{"name":"Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul 03080, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5042-6048","authenticated-orcid":false,"given":"Young Seok","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul 03080, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seung-Ha","family":"Oh","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul 03080, Korea"},{"name":"Sensory Organ Research Institute, Seoul National University Medical Research Center, Seoul 03087, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sang-Yeon","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul 03080, Korea"},{"name":"Sensory Organ Research Institute, Seoul National University Medical Research Center, Seoul 03087, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun Ho","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul 03080, Korea"},{"name":"Sensory Organ Research Institute, Seoul National University Medical Research Center, Seoul 03087, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,10,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1148\/radiology.202.3.9051033","article-title":"Aplasia and hypoplasia of the vestibulocochlear nerve: Diagnosis with MR imaging","volume":"202","author":"Casselman","year":"1997","journal-title":"Radiology"},{"key":"ref_2","first-page":"1","article-title":"Cochlear implantation versus auditory brainstem implantation in children with auditory nerve deficiencies","volume":"279","author":"Yousef","year":"2021","journal-title":"Eur. Arch. Oto-Rhino-Laryngol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"793","DOI":"10.1097\/01.mao.0000227895.34915.94","article-title":"Internal auditory canal morphology in children with cochlear nerve deficiency","volume":"27","author":"Adunka","year":"2006","journal-title":"Otol. Neurotol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1097\/MAO.0b013e3182804b31","article-title":"Cochlear nerve deficiency and associated clinical features in patients with bilateral and unilateral hearing loss","volume":"34","author":"Nakano","year":"2013","journal-title":"Otol. Neurotol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1097\/MAO.0000000000000997","article-title":"Cochlear implant outcomes in cochlea nerve aplasia and hypoplasia","volume":"37","author":"Birman","year":"2016","journal-title":"Otol. Neurotol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1097\/MAO.0000000000001727","article-title":"Hearing restoration in cochlear nerve deficiency: The choice between cochlear implant or auditory brainstem implant, a meta-analysis","volume":"39","author":"Vesseur","year":"2018","journal-title":"Otol. Neurotol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"220","DOI":"10.21053\/ceo.2015.01487","article-title":"Prediction of Cochlear Implant Outcomes in Patients With Prelingual Deafness","volume":"9","author":"Kang","year":"2016","journal-title":"Clin. Exp. Otorhinolaryngol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"287","DOI":"10.21053\/ceo.2021.00752","article-title":"Potential Implications of Slim Modiolar Electrodes for Severely Malformed Cochleae: A Comparison With the Straight Array With Circumferential Electrodes","volume":"14","author":"Lee","year":"2021","journal-title":"Clin. Exp. Otorhinolaryngol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1773","DOI":"10.1002\/lary.21791","article-title":"The cochleovestibular nerve identified during auditory brainstem implantation in patients with narrow internal auditory canals: Can preoperative evaluation predict cochleovestibular nerve deficiency?","volume":"121","author":"Song","year":"2011","journal-title":"Laryngoscope"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1097\/MAO.0000000000002182","article-title":"Comments on \u201chearing restoration in cochlear nerve deficiency: The choice between cochlear implant or auditory brainstem implant, a meta-analysis\u201d","volume":"40","author":"Brotto","year":"2019","journal-title":"Otol. Neurotol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"147","DOI":"10.5152\/iao.2020.8466","article-title":"A Study of Outcome of Pediatric Cochlear Implantation in Patients with Cochleovestibular Nerve Deficiency","volume":"16","author":"Arumugam","year":"2020","journal-title":"J. Int. Adv. Otol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1097\/MAO.0000000000002849","article-title":"Audiologic Outcome of Cochlear Implantation in Children With Cochlear Nerve Deficiency","volume":"42","author":"Yousef","year":"2021","journal-title":"Otol. Neurotol."},{"key":"ref_13","first-page":"1","article-title":"A predictive model for cochlear implant outcome in children with cochlear nerve deficiency","volume":"9","author":"Han","year":"2019","journal-title":"Sci. Rep."},{"key":"ref_14","first-page":"312","article-title":"Categories of auditory performance","volume":"166","author":"Archbold","year":"1995","journal-title":"Ann. Otol. Rhinol. Laryngology. Suppl."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.artmed.2006.06.003","article-title":"AutoNRT: An automated system that measures ECAP thresholds with the Nucleus Freedom cochlear implant via machine intelligence","volume":"40","author":"Botros","year":"2007","journal-title":"Artif. Intell. Med."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1586\/erd.10.28","article-title":"Cochlear implantation update: Contemporary preoperative imaging and future prospects\u2014The dual modality approach as a standard of care","volume":"7","author":"Vlastarakos","year":"2010","journal-title":"Expert Rev. Med. Devices"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1097\/MAO.0000000000000568","article-title":"Impact of cochlear nerve deficiency determined using 3-dimensional magnetic resonance imaging on hearing outcome in children with cochlear implants","volume":"36","author":"Wu","year":"2015","journal-title":"Otol. Neurotol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1111\/j.0021-8782.2004.00313.x","article-title":"Connections between the facial, vestibular and cochlear nerve bundles within the internal auditory canal","volume":"205","author":"Sezen","year":"2004","journal-title":"J. Anat."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1007\/s00276-008-0311-z","article-title":"The topographical relationships and anastomosis of the nerves in the human internal auditory canal","volume":"30","author":"Tian","year":"2008","journal-title":"Surg. Radiol. Anat."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1177\/0194599815596929","article-title":"Systematic Review of Nontumor Pediatric Auditory Brainstem Implant Outcomes","volume":"153","author":"Noij","year":"2015","journal-title":"Otolaryngol. Head Neck Surg."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1097\/MAO.0000000000002455","article-title":"Effects of Age at Auditory Brainstem Implantation: Impact on Auditory Perception, Language Development, Speech Intelligibility","volume":"41","author":"Aslan","year":"2020","journal-title":"Otol. Neurotol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"16","DOI":"10.3766\/jaaa.21.1.3","article-title":"Comparison of electrically evoked compound action potential thresholds and loudness estimates for the stimuli used to program the Advanced Bionics cochlear implant","volume":"21","author":"Jeon","year":"2010","journal-title":"J. Am. Acad. Audiol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1097\/AUD.0b013e3181906c0f","article-title":"Relation of electrically evoked compound action potential thresholds to behavioral T- and C-levels in children with cochlear implants","volume":"30","author":"Holstad","year":"2009","journal-title":"Ear Hear."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1002\/lary.26154","article-title":"Systematic review of compound action potentials as predictors for cochlear implant performance","volume":"127","author":"Buitenhuis","year":"2017","journal-title":"Laryngoscope"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1979","DOI":"10.1002\/lary.22032","article-title":"Cochlear implantation in children with labyrinthine anomalies and cochlear nerve deficiency: Implications for auditory brainstem implantation","volume":"121","author":"Buchman","year":"2011","journal-title":"Laryngoscope"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"714","DOI":"10.3109\/00016489.2013.776176","article-title":"Auditory neuropathy spectrum disorder: Predictive value of radiologic studies and electrophysiologic tests on cochlear implant outcomes and its radiologic classification","volume":"133","author":"Jeong","year":"2013","journal-title":"Acta Otolaryngol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1097\/AUD.0b013e3181ce693b","article-title":"Cochlear implantation in children with auditory neuropathy spectrum disorder","volume":"31","author":"Teagle","year":"2010","journal-title":"Ear Hear."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1097\/AUD.0000000000000782","article-title":"Recommendations for Measuring the Electrically Evoked Compound Action Potential in Children with Cochlear Nerve Deficiency","volume":"41","author":"He","year":"2020","journal-title":"Ear Hear."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1177\/0194599817718798","article-title":"Cochlear nerve aplasia and hypoplasia: Predictors of cochlear implant success","volume":"157","author":"Peng","year":"2017","journal-title":"Otolaryngol. Head Neck Surg."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"7","DOI":"10.3109\/03005364000000045","article-title":"Categories of auditory performance: Inter-user reliability","volume":"32","author":"Archbold","year":"1998","journal-title":"Br. J. 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