Cochlear Implantation in Patients with Pendred Syndrome

Should we provide cochlear implants earlier for children with large vestibular aqueduct syndrome?

Patterson and colleagues (2021) examined outcomes of nine pediatric patients with Pendred syndrome who received cochlear implants between 2003–2017.

Pendred syndrome is an autosomal recessive disorder defined by the combination of sensorineural hearing loss, goiter and an enlarged vestibular aqueduct (EVA). All patients had bilateral mild to profound sensorineural hearing loss, used hearing aids prior to cochlear implantation, and had enlarged vestibular aqueducts. Eight of the nine participants had cochlear dysplasia. The age at implantation was between nine months and seven years.

All electrodes were successfully implanted into the scala tympani, and all patients had good postoperative audiological outcomes. Postoperatively, the average AzBio sentence score was 97 percent. The patients were enrolled in a mainstream educational setting or early intervention school setting and were using remote microphone technology at school.

The authors concluded that cochlear implantation is an effective and successful treatment for children with Pendred syndrome for whom traditional amplification provide limited benefit. Outcomes have been proven highly successful overall with a low rate of complications despite the abnormal inner ear anatomy (Hall et al 2019).

References

Hall AC, Kenway B, Sanlit S, et al. (2019) Cochlear implant outcomes in large vestibular aqueduct syndrome—should we provide cochlear implants earlier? Otol Neurotol 40(e769–e773).

Patterson TE, Gonzalez VB, Carron JD. (2021) Cochlear implantation in patients with Pendred Syndrome. Am J Otolaryngol. 42(6).