X-Linked Deafness Type 2 and Poor Response to Cochlear Implantation: A Case Report

Purpose: Hearing loss (HL) affects about one of every five children by the age of 18 years, and up to 60% can be related to genetic factors. The aim of this study is to present an unusual case of poor response to cochlear implantation in a child with X-linked deafness type 2. Method: This case report follows the “CARE” checklist. Results: The following report presents a 2-year-old child with bilateral nonsyndromic sensorineural HL with incomplete cochlear partition type III. The child has had a cochlear implant since 14 months of age. Genetic studies were performed after surgery. Next-generation sequencing analysis revealed two variants in GJB2 and POU3F4 genes. Conclusion: This case study enables us to suggest auditory-verbal therapy and hearing aid trials before CI surgery in the absence of auditory dyssynchrony.