Outcomes of Cochlear Implantation in Patients With Pendred Syndrome: A Systematic Review and Narrative Synthesis
Journal of International Advanced Otology
Biggs, K., Lovett, A., et al. (2020).
Journal of International Advanced Otology, 16(3), 432-442.
This systematic review investigates the audiometric outcomes of cochlear implantation in children and adults with Pendred syndrome.
No funding received
Inception to June 9, 2020
Experimental studies; observational studies
22
For children and adults with Pendred syndrome (or SLC26A4 mutations), cochlear implantation (CI) provides hearing benefits such as improved speech perception, speech intelligibility, and quality of life in the majority of patients. Pendred patients with nonspecified enlarged vestibular aqueduct (EVA) and no genetic analysis demonstrate superior CI outcomes as compared to patients without EVA. As compared to patients with other genetic hearing loss, patients with Pendred syndrome perform comparably on audiological measures post-CI. A significant number of CI patients with Pendred experienced minor intraoperative complications (i.e., brief cerebrospinal fluid release); however, major complications were not reported. Patients with fluctuating hearing loss benefit from CI and delaying implantation did not improve outcomes; however, hybrid implants were also found to provide benefit and may be appropriate for this presentation of hearing loss. The authors of this review conclude that candidacy for and timing of CI needs to be carefully considered for each individual patient. Additional research on long term outcomes is warranted.
