Results of Cochlear Implantation in Patients with Congenital Rubell-Retrospective Study.

Background/Objectives: Congenital rubella syndrome (CRS) is an infection caused by rubella virus transmitted to the fetus during pregnancy, which can cause congenital hearing loss. Cochlear implant can be an effective therapy in patients with severe to profound bilateral hearing loss. The aim of this study was to evaluate the benefits of cochlear implantation in patients with profound hearing loss caused by congenital rubella syndrome. Methods: In total, 38 patients with profound hearing loss caused by intrauterine rubella virus infection were considered for cochlear implantation. Patients ranged in age from 8 to 72 years on the day of surgery, with a mean age of 27 years and median of 25 years (SD = 13.2). Preoperatively, all patients underwent pure-tone audiometry, and free-field speech audiometry was conducted in a quiet environment with the patient wearing a fitted hearing aid. Postoperatively, patients underwent pure-tone audiometry to assess residual hearing, and free-field speech audiometry was conducted when the patients had an active implant. Results: The average preoperative hearing threshold (averaged across the seven frequencies from 0.125 to 8 kHz) was 99.2 dB HL (SD = 6.79), while the average postoperative hearing threshold was 103.4 dB HL (SD = 5.74). Twelve months after the operation, patients achieved a WRS in quiet scores ranging from 10% to 90%, with an average of 59.1% and median of 70% (SD = 25.8). Conclusions: Rubella during pregnancy can lead to severe congenital defects, with sensorineural hearing loss being the most common. Cochlear implants appear to be an effective treatment for profound hearing loss caused by congenital rubella syndrome.