Int J Pediatr Otorhinolaryngol 2018 Mar 30;106:64-67. Epub 2017 Dec 30.
Dell Medical School at the University of Texas at Austin, Department of Otolaryngology/ENT, 6811 Austin Center Blvd Suite 300, Austin, TX 78731, USA. Electronic address:
Cockayne Syndrome (CS) is a rare, autosomal recessive disorder characterized by a spectrum of phenotypic abnormalities, including progressive sensorineural hearing loss (SNHL) that involves both peripheral and central components. To date, a single series of CS patients undergoing cochlear implant (CI) placement has been reported; this study reports on additional previously unreported pediatric CI recipients. Subjective benefits were noted early after activation in both patients, and speech perception scores improved over time as well, varying from 42 to 70% (versus 0-12% previously). Read More