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).
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).
Has anything good come from the COVID-19 pandemic? While it may be disheartening to try to find positive outcomes, researchers continue to investigate the long-term effects of the virus and its vaccinations and report both negative and positive, findings. Parrino et al. (2021) published a report on three individuals with rapid onset unilateral tinnitus after…
What is listening fatigue? Fatigue in general refers to a “weariness” resulting from exertion. In many children who are Deaf and/or hard of hearing (D/HH), listening fatigue occurs due to their need for increased concentration and attention. In many situations, children who are D/HH often have to work harder and pay closer attention than their…
The impact of sound exposure in humans has been the subject of thousands of research studies. Of course, animal models have been used in these studies primarily to infer how exposure might affect the human auditory system. Far fewer studies have looked at how noise affects the other members of the animal kingdom. Increased interest in…