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Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
The outcome of cochlear implantation among children with genetic syndromes
Farid Alzhrani1, Rayan Alhussini2, Rawan Hudeib2
1King Abdullah Ear Specialist Center (KAESC), College of Medicine, King Saud University, PO Box 245, Riyadh, 11411, Saudi Arabia. faalzhrani@ksu.edu.sa.
Insights
Children with genetic syndromes who received cochlear implants showed similar auditory and speech outcomes compared to non-syndromic children. These findings support cochlear implantation for genetic syndromes, improving sensory perception and quality of life.
Area of Science:
- Otolaryngology
- Genetics
- Pediatrics
Background:
- Genetic syndromes can impact hearing and speech development.
- Cochlear implantation is a common treatment for pediatric hearing loss.
Purpose of the Study:
- To evaluate the efficacy of cochlear implantation in children with specific genetic syndromes.
- To compare auditory and speech outcomes between syndromic and non-syndromic pediatric cochlear implant recipients.
Main Methods:
- A case-control study was conducted at a tertiary referral center.
- Pediatric patients with Waardenburg syndrome, Usher syndrome, Dandy-Walker syndrome, or albinism were compared to a matched control group.
- Auditory abilities, speech intelligibility, and pure tone thresholds were assessed.
Main Results:
- The study included 13 syndromic and 12 non-syndromic children.
- No significant differences in auditory ability or speech intelligibility were found between the groups.
- Final pure tone averages (PTA) indicated normal-to-mild hearing loss in both groups (26 dB HL syndromic, 23 dB HL control).
Conclusions:
- Cochlear implantation yields comparable auditory perception and speech intelligibility in children with genetic syndromes versus those without.
- Genetic syndromes should not preclude cochlear implant candidacy.
- Cochlear implantation positively impacts sensory perception and lifestyle for children with these genetic syndromes.
Objective:
To assess the outcome and efficacy of cochlear implantation in children with genetic syndromes.
Method:
Study design: case-control study.
Setting:
A cochlear implantation tertiary referral center.
Patients:
All pediatric cochlear implantation recipients with Waardenburg syndrome, Usher syndrome, Dandy-Walker syndrome, or albinism. A control group was appropriately matched to the syndromic group with regard to age at implantation and duration of device use.
Intervention:
Cochlear implantation.
Main Outcome Measures:
Subjects' auditory abilities, speech intelligibility, and pure tone thresholds were compared between the syndromic and non-syndromic group.
Results:
A total of 25 subjects (13 syndromic and 12 non-syndromic) participated in the study. Neither auditory ability nor speech intelligibility scores differed significantly by group. The final PTA of both the groups showed normal-to-mild hearing loss: 26 dB HL in the syndromic group and 23 dB HL for the control group.
Conclusions:
Cochlear implant recipients with genetic syndromes achieved similar levels auditory perception and speech intelligibility as their peers with a genetic syndrome. The presence of any of the genetic syndromes described herein should not be a contraindication to cochlear implant provision, as it would have a positive impact on the patients' sensory perception and lifestyle.
