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Updated: Oct 26, 2025
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Audiological and Speech Performance After Cochlear Implantation in Cochlear Aplasia Deformity
1College of Medicine, King Abdullah Ear Specialist Center, King Saud University, Riyadh, SAU.
Insights
Cochlear implantation can restore hearing in children with inner ear malformations like cochlear aplasia. A one-year follow-up showed significant speech development after unilateral cochlear implantation.
Area of Science:
- Otolaryngology
- Pediatric audiology
- Medical imaging
Background:
- Inner ear malformations are congenital conditions causing prelingual sensorineural hearing loss in children.
- Cochlear aplasia, a specific malformation, presents diagnostic challenges, particularly differentiating cystic subtypes.
Observation:
- This case report details a child with bilateral, radiologically diagnosed cochlear aplasia.
- The child received a unilateral cochlear implant in the left ear.
Findings:
- One year post-surgery, audiological results indicated positive outcomes.
- At 16 months postsurgery, the child had a vocabulary of 200 words and could form two-word sentences.
Implications:
- Cochlear implantation is a viable option for pediatric hearing loss due to inner ear malformations.
- Difficulties in classifying cystic cavities should not prevent cochlear implant trials if the inner ear anatomy is suitable and the cochleovestibular nerve is present.
Abstract:
Inner ear malformation is a congenital anomaly associated with prelingual sensory neural hearing loss in the pediatric population. This is a case report of bilateral radiologically diagnosed cochlear aplasia in a child who underwent unilateral left cochlear implantation with audiological results at one-year follow-up after surgery. Sixteen months after the cochlear implantation surgery, the child could produce 200 words and say a sentence with two words. In certain cases of inner ear malformation, the subgrouping of cystic cavity can be difficult and should not delay the trial of cochlear implant provided an acceptable anatomical appearance of the inner ear with cochleovestibular nerve existence and a proper electrode used for implantation.
