Hearing and speech outcomes in children with cochlear nerve hypoplasia who underwent cochlear implantation

Abstract

Background: Data on hearing and speech outcomes following cochlear implantation in prelingual sensorineural hearing loss (SNHL) children with cochlear nerve hypoplasia, especially in Indonesia, is still limited. Purpose: To evaluate the hearing and speech function of prelingual SNHL children who had undergone cochlear implantation. Method: A retrospective cross-sectional study was conducted on 28 children who had undergone cochlear implantation procedures at the Department of Otorhinolaryngology and Head and Neck Surgery, Dr. Cipto Mangunkusumo National General Hospital, Jakarta, Indonesia, for more than 12 months. The hearing and speech function of the subjects were assessed using the modified Categories of Auditory Performance (CAP-II) and the speech intelligibility rating (SIR) scores. Result: Children with cochlear nerve hypoplasia showed an increase in CAP-II and SIR scores after using cochlear implants for more than 12 months (versus pre-surgery, high CAP score [≥5]: 89.3% [25/28 children] vs 3.6% [1/28 children] and high SIR score [≥3]: 82.1% [23/28 children] vs 7.1% [2/28 children]; both p<0.001). Post-operative CAP-II and post-operative SIR scores were highly positively correlated (r=0.705, p<0.001). Type of cochlear nerve hypoplasia (unilateral vs bilateral) were not significantly associated with post-operative CAP-II and SIR scores (p=0.382 and p=0.459, respectively). Conclusion: Prelingual SNHL children with cochlear nerve hypoplasia may still get the benefit from cochlear implantation, as shown by a remarkable improvement in hearing and speech function as assessed with the CAP-II and SIR scores.

Keywords: auditory perception, cochlear implants, cochlear nerve hypoplasia, prelingual sensorineural hearing loss, speech intelligibility