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Cochlear Implant Challenges in Children with Ichthyosis: A Systematic Review
Valeria Caragli1, Laura Luppi1, Nicole Carrie Tegmeyer1
1Otorhinolaryngology-Head and Neck Surgery, Audiology Program, University of Modena and Reggio Emilia, 41125 Modena, Italy.
Genes
|February 26, 2025
Summary
Cochlear implants (CI) offer significant auditory and communicative benefits for patients with ichthyosis, a genetic skin disorder. While complications like wound infections can occur, CI improves quality of life, necessitating multidisciplinary care.
Area of Science:
- Genetics and Dermatology
- Otolaryngology and Audiology
Background:
- Ichthyosis encompasses genetic skin disorders causing scaling.
- Syndromic ichthyosis, like KID syndrome, involves connexin 26 mutations, leading to keratosis, ichthyosis, and deafness.
- Cochlear implantation (CI) is a treatment for severe hearing loss, but skin complications pose challenges.
Purpose of the Study:
- To systematically review literature on cochlear implant (CI) effectiveness in ichthyosis patients.
- To evaluate auditory and communicative outcomes in this population.
- To identify challenges and best practices for CI in ichthyosis.
Main Methods:
- Comprehensive literature search across PubMed, Scopus, and Web of Science.
- Inclusion criteria: genetically confirmed ichthyosis patients who underwent CI.
- PRISMA statement adherence for systematic review methodology.
Main Results:
- Eleven studies including 47 patients met criteria; 40 had GJB2 mutations.
- All patients had severe to profound sensorineural hearing loss.
- CI improved speech discrimination and auditory thresholds in 89.4% of patients, with 78.6% experiencing complications (e.g., wound infections).
Conclusions:
- Cochlear implantation significantly enhances quality of life for ichthyosis patients despite potential complications.
- Multidisciplinary approaches and careful surgical planning are vital for effective management.
- Further research with larger cohorts and longer follow-up is recommended.
