Outcomes of Cochlear implantation in early‐deafened patients with Waardenburg syndrome: A systematic review and narrative synthesis

Lovett, Amy; Eastwood, Michael; Metcalfe, Chris; Muzaffar, Jameel; Monksfield, Peter; Bance, Manohar

Abstract

Objective: This systematic review aims to establish the expected hearing and speech outcomes following cochlear implantation (CI) in patients with profound congenital deafness secondary to Waardenburg syndrome (WS). Methods: A systematic review of the literature and narrative synthesis was performed in accordance with the PRISMA statement. Databases searched: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. No limits were placed on language or year of publication. Results: Searches identified 186 abstracts and full texts. Of these, 16 studies met inclusion criteria reporting outcomes in 179 patients and at least 194 implants. Hearing outcomes of those receiving cochlear implantation were generally good. Five studies included genetic analysis of one or more of the participants. A total of 11 peri/post‐operative complications were reported. The methodological quality of included studies was modest, mainly comprising noncontrolled case series with small cohort size. All studies were OCEBM grade III–IV. Conclusion: Cochlear implantation in congenitally deafened children with Waardenburg Syndrome is a well‐established intervention as a method of auditory rehabilitation. Due to the uncommon nature of the condition, there is a lack of large‐scale high‐quality studies examining the use of cochlear implantation in this patient group. However, overall outcomes following implantation are positive with the majority of patients demonstrating improved audiometry, speech perception and speech intelligibility supporting its use in appropriately selected cases.

Citation

Lovett, A., Eastwood, M., Metcalfe, C., Muzaffar, J., Monksfield, P., & Bance, M. (in press). Outcomes of Cochlear implantation in early‐deafened patients with Waardenburg syndrome: A systematic review and narrative synthesis. Laryngoscope Investigative Otolaryngology, https://doi.org/10.1002/lio2.1110

• Journal Article Type: Article
• Acceptance Date: Jun 17, 2023
• Online Publication Date: Jul 11, 2023
• Deposit Date: Jul 25, 2023
• Journal: Laryngoscope Investigative Otolaryngology
• Print ISSN: 2378-8038
• Electronic ISSN: 2378-8038
• Publisher: Wiley Open Access
• Peer Reviewed: Peer Reviewed
• DOI: https://doi.org/10.1002/lio2.1110
• Keywords: Waardenburg syndrome, Cochlear implantation
• Publisher URL: https://onlinelibrary.wiley.com/doi/10.1002/lio2.1110