Hearing Preservation With Standard Length Electrodes in Pediatric Cochlear Implantation.

OBJECTIVE: Preserving low frequencies following cochlear implantation improves outcomes and allows patients to use a combination of electrical and acoustic stimulation. This importance has been reflected in advances in electrode design and refined surgical techniques. Full insertion of standard length electrodes may be advantageous over shortened electrodes because more electrodes can be activated over time if low frequency hearing loss progresses. Surgeons must counsel patients over this choice but data is lacking regarding the degree and likelihood of hearing preservation achievable with standard length electrodes in children. We report our experience using standard length cochlear implant arrays for hearing preservation in children. METHODS: Retrospective case series. INCLUSION CRITERIA: preoperative hearing ≤85 dB HL at 250 Hz and aged ≤18 years. Hearing preservation percentages are calculated using the HEARRING group formula. (Equation is included in full-text article.)Preservation of > 75% was considered complete, 25 to 75% partial, and 1 to 25% minimal. Patients were implanted with either MED-EL FLEX28 or Cochlear Nucleus CI522. Standardized operative technique with facial recess approach, posterior tympanotomy and minimally traumatic round window insertion. RESULTS: Fifty-two implantations in 27 pediatric patients met inclusion criteria. Mean age at implantation: 9.8 years. Average latest audiogram: 8 months. Mean total pre- and postoperative pure-tone averages were 82.8 and 92.6 dB. Seventeen (33%) ears demonstrated complete hearing preservation, 22 (42%) ears partial hearing preservation, 7 (13%) minimal hearing preservation, and 6 (12%) exhibited no acoustic hearing postoperatively. Mean hearing preservation was 55.5%. CONCLUSION: Hearing preservation is achievable to varying degrees in pediatric cochlear implantation using standard length electrodes though it is difficult to predict preoperatively which children may benefit. This study is among the largest additions to the knowledge base for this patient group.

Cochlear implantation in children with sickle cell disease.

Sickle cell disease (SCD) is associated with sensorineural hearing loss (SNHL). Although the hearing loss is usually mild, some develop severe-to-profound hearing loss, in whom cochlear implants (CI) may be an option. We present the cases of two children with SCD who developed bilateral severe-to-profound SNHL and underwent cochlear implantation. One patient became profoundly deaf after an acute episode of dizziness. Imaging indicated bilateral cochlear ossification, making subsequent cochlear implant surgery challenging. The second patient developed bilateral severe-to-profound SNHL following acute vaso-occlusive crises. She went on to have uncomplicated cochlear implant surgery. These cases illustrate the variable manner in which children with SCD may develop SNHL, and the difficulties associated with managing such cases. We recommend that children with SCD should undergo regular audiological assessment. Furthermore, clinicians should be aware of the risk of cochlear fibrosis and ossification and ensure prompt assessment following an acute vaso-occlusive crisis or unexplained vestibulocochlear event.