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Updated: Apr 23, 2026
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
The development of auditory skills in young children with Mondini dysplasia after cochlear implantation
Xueqing Chen1, Fei Yan2, Bo Liu1
1Department of Otolaryngology, Head and Neck Surgery, Beijing Tongren Hospital and Beijing Institute of Otolaryngology, Capital Medical University, Beijing, China.
Insights
Cochlear implantation effectively improves auditory skills in young children with Mondini dysplasia, showing rapid development similar to peers with normal inner ears. This intervention is beneficial for this specific hearing loss condition.
Area of Science:
- Pediatric audiology
- Otolaryngology
- Developmental pediatrics
Background:
- Mondini dysplasia is a congenital inner ear malformation.
- Profound hearing loss in young children requires timely intervention.
- Cochlear implantation is a standard treatment for pediatric hearing loss.
Purpose of the Study:
- To compare auditory skill development in young children with Mondini dysplasia versus those with normal inner ears after cochlear implantation.
- To evaluate the long-term (3-year) efficacy of cochlear implantation in these groups.
- To assess specific auditory skill subcategories using the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS).
Main Methods:
- A cohort of 545 young children (7-36 months) with prelingual severe to profound hearing loss received cochlear implants.
- Subjects were divided into two groups: Mondini dysplasia (n=31) and radiologically normal inner ears (n=514).
- Auditory skills were assessed using IT-MAIS pre-surgery and at multiple intervals up to 36 months post-implantation.
Main Results:
- No significant overall auditory skill differences between groups at pre-surgery, 1, 12, 24, and 36 months post-implantation.
- Significant differences in auditory skills were observed at 3, 6, and 9 months post-implantation.
- Children with Mondini dysplasia demonstrated significant auditory skill improvement over time, with rapid development in the first year.
Conclusions:
- Cochlear implantation is an effective intervention for young children with Mondini dysplasia.
- Auditory skill development in children with Mondini dysplasia post-implantation mirrors that of children with normal inner ears.
- The study supports cochlear implantation as a viable treatment for Mondini dysplasia, leading to comparable auditory outcomes.
Abstract:
The aim of this study is to survey and compare the development of auditory skills in young children with Mondini dysplasia and profoundly-deaf young children with radiologically normal inner ears over a period of 3 years after cochlear implantation. A total of 545 young children (age 7 to 36 months) with prelingual, severe to profound hearing loss participated in this study. All children received cochlear implantation. Based on whether or not there was a Mondini dysplasia as diagnosed with CT scanning, the subjects were divided into 2 groups: (A) 514 young children with radiologically normal inner ears and (B) 31 young children with Mondini dysplasia. The Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to assess the children's auditory skills that include vocalization changes, spontaneous alerting to sounds in everyday living environments, and the ability to derive meaning from sounds. The assessment was performed prior to surgery and at 1, 3, 6, 9, 12, 24, and 36 months after implant device switch-on. The mean scores for overall auditory skills were not significantly different between groups A and B at pre-surgery, 1, 12, 24, and 36 months post-surgery, but were significantly different at 3, 6, and 9 months post-surgery. The mean scores for all auditory skills in children with Mondini dysplasia showed significant improvement over time. The mean scores for the three subcategories of auditory skills in children with Mondini dysplasia also showed significant differences at pre-surgery, 1, 3, 6, and 9 months, however, there were no significant differences at 12, 24, and 36 months. Overall, the auditory skills of young children with Mondini dysplasia developed rapidly after cochlear implantation, in a similar manner to that of young children with radiologically normal inner ears. Cochlear implantation is an effective intervention for young children with Mondini dysplasia.
