ISSN 1308-7649 | E-ISSN 2148-3817
Original Article
Performance of Cochlear Implants in Pediatric Patients with Auditory Neuropathy Spectrum Disorder
1 Department of Ear Nose Throat Head and Neck Surgery, UNICAMP (Campinas University, São Paulo, Brazil), Campinas, Brazil  
2 Centro de Biologia Molecular e Engenharia Genética, UNICAMP (Campinas University, São Paulo, Brazil), Campinas, Brazil  
J Int Adv Otol 2016; 12: 8-15
DOI: 10.5152/iao.2016.2232
Key Words: Auditory neuropathy spectrum disorder, evoked auditory brainstem response, otoacoustic emissions, GJB2, hearing loss, cochlear implants

OBJECTIVE: To describe the performance and results of CIs (cochlear implant) in patients with AN (auditory neuropathy) and to present a medical literature review.


MATERIALS and METHODS: Retrospective chart review of patients with AN who were treated with CI. The mesh terms used for the review in the Pubmed and Scopus databases were as follows: “hearing loss, cochlear implants, rehabilitation of persons with hearing impairment, auditory neuropathy”. Statistical Analyses: The Mann-Whitney test was performed.


RESULTS: The sample consisted of 10 patients. The mean age at surgery was 4.3 years, range 2-16 years. The average length of CI use was 5.2 years. The comparison of hearing levels before and after CI use showed a significant improvement in all patients, with p<0.05. All of them also reported an increase in overall satisfaction 1 year after the procedure. A CI is the standard treatment for the hearing rehabilitation of patients with severe profound hearing loss who do not benefit from conventional hearing aids. There are diseases such as an that also invoke a discussion in the literature regarding CI benefits.


CONCLUSION: Individuals with AN demonstrated a significant gain in hearing levels and language use with CI. 

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