title: Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder.
abstract: OBJECTIVE
  To evaluate the auditory and speech abilities in children with auditory neuropathy spectrum disorder (ANSD) after cochlear implantation (CI) and determine the role of age at implantation.
  
  
  METHODS
  Ten children participated in this retrospective case series study. All children had evidence of ANSD. All subjects had no cochlear nerve deficiency on magnetic resonance imaging and had used the cochlear implants for a period of 12-84 months. We divided our children into two groups: children who underwent implantation before 24 months of age and children who underwent implantation after 24 months of age. Their auditory and speech abilities were evaluated using the following: behavioral audiometry, the Categories of Auditory Performance (CAP), the Meaningful Auditory Integration Scale (MAIS), the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Standard-Chinese version of the Monosyllabic Lexical Neighborhood Test (LNT), the Multisyllabic Lexical Neighborhood Test (MLNT), the Speech Intelligibility Rating (SIR) and the Meaningful Use of Speech Scale (MUSS).
  
  
  RESULTS
  All children showed progress in their auditory and language abilities. The 4-frequency average hearing level (HL) (500Hz, 1000Hz, 2000Hz and 4000Hz) of aided hearing thresholds ranged from 17.5 to 57.5dB HL. All children developed time-related auditory perception and speech skills. Scores of children with ANSD who received cochlear implants before 24 months tended to be better than those of children who received cochlear implants after 24 months. Seven children completed the Mandarin Lexical Neighborhood Test. Approximately half of the children showed improved open-set speech recognition.
  
  
  CONCLUSIONS
  Cochlear implantation is helpful for children with ANSD and may be a good optional treatment for many ANSD children. In addition, children with ANSD fitted with cochlear implants before 24 months tended to acquire auditory and speech skills better than children fitted with cochlear implants after 24 months.
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.
authors.forename: Yuying
authors.initials: Y
authors.lastname: Liu
authors.affiliation: Key Laboratory of Otolaryngology, Head and Neck Surgery (Capital Medical University), Ministry of Education, Beijing Institute of Otolaryngology, Beijing 100005, China.
authors.forename: Ruijuan
authors.initials: R
authors.lastname: Dong
authors.affiliation: Key Laboratory of Otolaryngology, Head and Neck Surgery (Capital Medical University), Ministry of Education, Beijing Institute of Otolaryngology, Beijing 100005, China.
authors.forename: Yuling
authors.initials: Y
authors.lastname: Li
authors.affiliation: Key Laboratory of Otolaryngology, Head and Neck Surgery (Capital Medical University), Ministry of Education, Beijing Institute of Otolaryngology, Beijing 100005, China.
authors.forename: Tianqiu
authors.initials: T
authors.lastname: Xu
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.
authors.forename: Yongxin
authors.initials: Y
authors.lastname: Li
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China; Key Laboratory of Otolaryngology, Head and Neck Surgery (Capital Medical University), Ministry of Education, Beijing Institute of Otolaryngology, Beijing 100005, China. Electronic address: xueqingchen2006@aliyun.com.
authors.forename: Xueqing
authors.initials: X
authors.lastname: Chen
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China. Electronic address: gongss1962@163.com.
authors.forename: Shusheng
authors.initials: S
authors.lastname: Gong
mesh_terms: D000367:Age Factors; D001299:Audiometry; D001307:Auditory Perception; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation / Q000379:methods*; D003054:Cochlear Implants; D015331:Cohort Studies; D005260:Female; D006313:Hearing Loss, Central / Q000601:surgery*; D006801:Humans; D007223:Infant; D007804:Language Development; D008297:Male; D012189:Retrospective Studies; D013060:Speech; D016896:Treatment Outcome
keywords: Auditory dys-synchrony; Auditory neuropathy; Auditory neuropathy spectrum disorder; Children; Cochlear implantation