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

Abstract

ObjectiveThis 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).MethodsA 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.ResultsSearches 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.ConclusionCochlear 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.

Auditory and speech outcomes of cochlear implantation in patients with Waardenburg syndrome: a meta-analysis

“…At present, most studies show that patients with WS recover well after CI, but some studies still report that the postoperative effect on patients is not good ( 7 ). Lovett et al ( 2 ) compared the hearing and speech outcomes of WS patients before and after CI. The results showed that CI can be an effective way for improving the hearing and speech ability of WS patients.…”

“…Waardenburg syndrome (WS), discovered and named by Dutch physician Waardenburg in 1951 ( 1 ), is an autosomal dominant genetic disorder primarily characterized by auditory pigmentary abnormalities. Its key manifestations include inner canthus heterotopia, iris heterochrony, white hair on the forehead, and hereditary sensorineural deafness ( 2 ). WS is closely related to the abnormal migration and differentiation of melanocytes.…”