{"title":"附加残疾儿童人工耳蜗植入后的听觉结果:内耳畸形的分级评估","authors":"Shujiro Minami , Chieko Enomoto , Hidetoshi Kato , Natsumi Tachibana , Motoko Ihara , Masae Shiroma , Kimitaka Kaga","doi":"10.1016/j.ijporl.2025.112490","DOIUrl":null,"url":null,"abstract":"<div><h3>Objective</h3><div>This study aimed to evaluate auditory and speech outcomes after cochlear implantation (CI) in children with additional disabilities (ADs), including autism spectrum disorder (ASD), with a particular focus on the impact of inner ear malformation (IEM) grading based on modiolus and cochlear nerve integrity.</div></div><div><h3>Methods</h3><div>We retrospectively reviewed 58 children with bilateral severe-to-profound sensorineural hearing loss and at least one AD who underwent CI between 2007 and 2022 at a national cochlear implant center. ADs included ASD, intellectual disability (ID), and attention-deficit/hyperactivity disorder (ADHD). IEMs were classified into Grades 0–III based on radiological evaluation. Auditory outcomes were assessed using the Revised Categories of Auditory Performance (R-CAP) and Speech Intelligibility Rating (SIR) scales. Statistical analyses included non-parametric tests and multivariable logistic regression.</div></div><div><h3>Results</h3><div>Among the 58 children, 60 % had ASD and 83 % had ID. IEMs of Grade II or higher were observed in 26 %. Median age at implantation was 29.5 months, and median age at evaluation was 86.5 months. R-CAP scores were significantly higher in children with Grade 0– I IEM than in those with Grade ≥ II (median 8 vs. 2, <em>p</em> < 0.01). R-CAP ≥8, indicating conversational speech understanding, was achieved by 60 % of Grade 0– I cases but only 13 % of those with more severe IEMs. Multivariable analysis showed that mild ID and severe IEMs were independent negative predictors of achieving R-CAP ≥8, while age at assessment was positively associated with better outcomes.</div></div><div><h3>Conclusion</h3><div>CI can provide meaningful auditory development for children with ADs, but outcomes vary by cognitive and anatomical factors. Early implantation remains essential despite challenges in diagnosing conditions like ASD before surgery, supporting the need for ongoing developmental surveillance and individualized habilitation planning.</div></div>","PeriodicalId":14388,"journal":{"name":"International journal of pediatric otorhinolaryngology","volume":"196 ","pages":"Article 112490"},"PeriodicalIF":1.3000,"publicationDate":"2025-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Auditory outcomes after cochlear implantation in children with additional disabilities: A grading-based evaluation of inner ear malformations\",\"authors\":\"Shujiro Minami , Chieko Enomoto , Hidetoshi Kato , Natsumi Tachibana , Motoko Ihara , Masae Shiroma , Kimitaka Kaga\",\"doi\":\"10.1016/j.ijporl.2025.112490\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Objective</h3><div>This study aimed to evaluate auditory and speech outcomes after cochlear implantation (CI) in children with additional disabilities (ADs), including autism spectrum disorder (ASD), with a particular focus on the impact of inner ear malformation (IEM) grading based on modiolus and cochlear nerve integrity.</div></div><div><h3>Methods</h3><div>We retrospectively reviewed 58 children with bilateral severe-to-profound sensorineural hearing loss and at least one AD who underwent CI between 2007 and 2022 at a national cochlear implant center. ADs included ASD, intellectual disability (ID), and attention-deficit/hyperactivity disorder (ADHD). IEMs were classified into Grades 0–III based on radiological evaluation. Auditory outcomes were assessed using the Revised Categories of Auditory Performance (R-CAP) and Speech Intelligibility Rating (SIR) scales. Statistical analyses included non-parametric tests and multivariable logistic regression.</div></div><div><h3>Results</h3><div>Among the 58 children, 60 % had ASD and 83 % had ID. IEMs of Grade II or higher were observed in 26 %. Median age at implantation was 29.5 months, and median age at evaluation was 86.5 months. R-CAP scores were significantly higher in children with Grade 0– I IEM than in those with Grade ≥ II (median 8 vs. 2, <em>p</em> < 0.01). R-CAP ≥8, indicating conversational speech understanding, was achieved by 60 % of Grade 0– I cases but only 13 % of those with more severe IEMs. Multivariable analysis showed that mild ID and severe IEMs were independent negative predictors of achieving R-CAP ≥8, while age at assessment was positively associated with better outcomes.</div></div><div><h3>Conclusion</h3><div>CI can provide meaningful auditory development for children with ADs, but outcomes vary by cognitive and anatomical factors. Early implantation remains essential despite challenges in diagnosing conditions like ASD before surgery, supporting the need for ongoing developmental surveillance and individualized habilitation planning.</div></div>\",\"PeriodicalId\":14388,\"journal\":{\"name\":\"International journal of pediatric otorhinolaryngology\",\"volume\":\"196 \",\"pages\":\"Article 112490\"},\"PeriodicalIF\":1.3000,\"publicationDate\":\"2025-07-14\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"International journal of pediatric otorhinolaryngology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S0165587625002770\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"OTORHINOLARYNGOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"International journal of pediatric otorhinolaryngology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0165587625002770","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"OTORHINOLARYNGOLOGY","Score":null,"Total":0}
Auditory outcomes after cochlear implantation in children with additional disabilities: A grading-based evaluation of inner ear malformations
Objective
This study aimed to evaluate auditory and speech outcomes after cochlear implantation (CI) in children with additional disabilities (ADs), including autism spectrum disorder (ASD), with a particular focus on the impact of inner ear malformation (IEM) grading based on modiolus and cochlear nerve integrity.
Methods
We retrospectively reviewed 58 children with bilateral severe-to-profound sensorineural hearing loss and at least one AD who underwent CI between 2007 and 2022 at a national cochlear implant center. ADs included ASD, intellectual disability (ID), and attention-deficit/hyperactivity disorder (ADHD). IEMs were classified into Grades 0–III based on radiological evaluation. Auditory outcomes were assessed using the Revised Categories of Auditory Performance (R-CAP) and Speech Intelligibility Rating (SIR) scales. Statistical analyses included non-parametric tests and multivariable logistic regression.
Results
Among the 58 children, 60 % had ASD and 83 % had ID. IEMs of Grade II or higher were observed in 26 %. Median age at implantation was 29.5 months, and median age at evaluation was 86.5 months. R-CAP scores were significantly higher in children with Grade 0– I IEM than in those with Grade ≥ II (median 8 vs. 2, p < 0.01). R-CAP ≥8, indicating conversational speech understanding, was achieved by 60 % of Grade 0– I cases but only 13 % of those with more severe IEMs. Multivariable analysis showed that mild ID and severe IEMs were independent negative predictors of achieving R-CAP ≥8, while age at assessment was positively associated with better outcomes.
Conclusion
CI can provide meaningful auditory development for children with ADs, but outcomes vary by cognitive and anatomical factors. Early implantation remains essential despite challenges in diagnosing conditions like ASD before surgery, supporting the need for ongoing developmental surveillance and individualized habilitation planning.
期刊介绍:
The purpose of the International Journal of Pediatric Otorhinolaryngology is to concentrate and disseminate information concerning prevention, cure and care of otorhinolaryngological disorders in infants and children due to developmental, degenerative, infectious, neoplastic, traumatic, social, psychiatric and economic causes. The Journal provides a medium for clinical and basic contributions in all of the areas of pediatric otorhinolaryngology. This includes medical and surgical otology, bronchoesophagology, laryngology, rhinology, diseases of the head and neck, and disorders of communication, including voice, speech and language disorders.