The Benefits of Early Cochlear Implantation for Speech Development in Children With Usher Syndrome: Literature Review

Introduction Usher syndrome (USH) is a rare autosomal recessive genetic disorder characterized by sensorineural hearing loss (SNHL), vision loss (retinitis pigmentosa), and occasional balance impairment. Depending on the severity and onset of hearing loss and coexisting vestibular dysfunction, USH is divided into three clinical types – USH1, USH2, USH3 – as well as atypical USH which combines features of all these three. The purpose of this review is to present the impact of cochlear implantation on speech development in children diagnosed with all types of Usher syndrome. Material and methods All relevant publications published in from 2013 to 2023 were retrieved from PubMed based on the keywords Usher syndrome, Usher syndrome diagnostics, Usher syndrome hearing loss, Usher syndrome cochlear, and Usher syndrome speech. Exactly 67 papers were selected. Results Bilateral cochlear implantation in children with Usher syndrome is beneficial for audiological and verbal development provided that hearing loss is detected early and implantation done promptly. In USH1 the preferred age is before 3 in the case of severe to profound congenital SNHL; in USH2 and USH3 the optimal time for implantation is hampered by the difficulty of estimating when hearing loss occurred and its rate of progression. After bilateral cochlear implantation, studies showed improvements in the categories of auditory performance (CAP), speech intelligibility rate (SIR), meaningful auditory integration scale (MAIS), meaningful use of speech scale (MUSS), and speech reception score (SRS), together with good development of speech perception and verbal communication. Conclusions Early diagnosis (before the onset of vision loss) and early bilateral cochlear implantation in children who have suffered severe to profound SNHL due to Usher syndrome reduces disability and maximizes auditory–oral communication skills, significantly increasing their quality of life.