Cochlear implantation improves auditory performance and speech intelligibility among patients with Usher
syndrome, according to findings published in European Archives of Oto-Rhino-Laryngology. Pedro
Clarós, MD, and colleagues examined auditory performance and speech intelligibility in children up
to 10 years following cochlear implantation, comparing 35 children with Usher syndrome with 46
patients who were non-syndromic. Mean age at implantation among children with Usher syndrome was
6.3. Post-implantation values were compared between patients with Usher syndrome and non-syndromic
children, with the following results: pure tone average, 25.0 dB HL versus 28.4 dB HL; central
auditory processing, 5.3 versus 5.1; speech intelligibility rating, 4.1 versus 3.9; meaningful
auditory integration scale, 82.3% versus 80.5%; and meaningful use of speech scale, 81.8% versus
76.6%. Patients with Usher syndrome reached a higher score ceiling sooner than non- syndromic
patients. Children who received an implant before age 3 attained significantly higher results
than older children in both groups (P<0.005).
