Hearing Preservation in Pediatric Cochlear Implantation.

OBJECTIVE: Currently, there is a paucity of literature evaluating hearing preservation outcomes in children following cochlear implantation. The objective of the current study is to report pediatric hearing preservation results following cochlear implantation with conventional full-length electrodes. STUDY DESIGN: Retrospective review (2000-2016). SETTING: Tertiary referral center. PATIENTS: All pediatric patients with a ≤ 75 dB preoperative low-frequency pure tone average (LFPTA; 250-500 Hz average), who underwent cochlear implantation with a conventional length electrode. INTERVENTION(S): Cochlear implantation. MAIN OUTCOME MEASURE(S): Complete, partial, minimal, or no hearing preservation following cochlear implantation (Skarzynski et al., 2013); maintenance of functional low frequency hearing (≤85 dB LFPTA). RESULTS: A total of 43 ears, in 35 pediatric patients, met inclusion criteria. The mean age at time of implantation was 8.6 years (range, 1.4-17.8 yr), 20 (57.1%) patients were female, and 25 (58.1%) cases were left-sided.The mean preoperative ipsilateral low frequency PTA and conventional four-frequency PTA (500, 1000, 2000, 3000 Hz average) were 54.2 dB (range, 15-75 dB) and 82.2 dB (range, 25-102.5 dB), respectively. The mean low frequency PTA and conventional four-frequency PTA shifts comparing the pre- and first postoperative audiogram were Δ25.2 dB (range, -5 to 92.5 dB) and Δ18.3 dB (range, -8.8 to 100 dB), respectively. Overall, 17 (39.5%) ears demonstrated complete hearing preservation, 19 (44.2%) ears partial hearing preservation, 0 minimal hearing preservation, and 7 (16.3%) exhibited no measurable acoustic hearing after surgery. In total, 28 (65.1%) ears maintained functional low-frequency hearing (i.e., ≤85 dB LFPTA) based on the initial postoperative audiogram. There was no statistically significant difference in the initial low frequency PTA shift comparing lateral wall and perimodiolar electrodes (Δ22.2 versus Δ28.1 respectively; p = 0.44), cochleostomy and round window insertions (Δ25.2 vs. Δ24.7 respectively; p = 0.95), or statistically significant association between age at implantation and low frequency PTA shift (r = 0.174; p = 0.26).In total, 22 ears in 19 patients had serial audiometric data available for review. Over a mean duration of 43.8 months (range, 2.6-108.3 mo) following surgery, the mean low frequency PTA and conventional four-frequency PTA shift comparing the initial postoperative and most recent postoperative audiogram was Δ9.7 dB (range, -27.5 to 57.5 dB) and Δ8.1 dB (range, -18.8 to 31.9 dB), respectively. CONCLUSIONS: Varying levels of hearing preservation with conventional length electrodes can be achieved in most pediatric subjects. In the current study, 82% of patients maintained detectable hearing thresholds and 65% maintained functional low-frequency acoustic hearing. These data may be used to guide preoperative counseling in pediatric patients with residual acoustic hearing. Additionally, the favorable rates of hearing preservation achieved in children provide further evidence for the expansion of pediatric cochlear implant candidacy to include patients with greater degrees of residual hearing.

Cochlear implantation in children with auditory neuropathy spectrum disorder: long-term outcomes.

BACKGROUND: Best practices concerning the audiological management of the child diagnosed with auditory neuropathy spectrum disorder (ANSD) have not been definitively defined nor fully understood. One reason is that previous studies have demonstrated conflicting findings regarding the outcomes of cochlear implantation for children with ANSD. Thus, the question remains whether children with ANSD are able to achieve similar outcomes following cochlear implantation as those children with sensorineural hearing loss (SNHL). PURPOSE: To assess speech perception outcomes for children with cochlear implants who have a diagnosis of ANSD as well as their age-matched peers who have sensorineural hearing loss. RESEARCH DESIGN: Retrospective study STUDY SAMPLE: Thirty-five subject pairs (n = 70) ranging in age at implant activation from to 10 to 121 mo (mean 39.2 mo) were included in this retrospective study. Subjects were matched on variables including age at initial implant activation and months of implant use at postoperative test point. DATA COLLECTION AND ANALYSIS: Speech recognition scores for monosyllabic and multisyllabic stimuli were compared across the subject groups. For those not developmentally and/or linguistically ready for completion of open-set speech recognition testing with recorded stimuli, GASP (Glendonald Auditory Screening Procedure) word recognition and/or questionnaire data using either the LittlEARS or Meaningful Auditory Integration Scale were compared across the groups. Statistical analysis using a repeated-measures analysis of variance (ANOVA) evaluated the effects of etiology (ANSD or SNHL) on postoperative outcomes. RESULTS: The results of this study demonstrate that children with ANSD can clearly benefit from cochlear implantation and that their long-term outcomes are similar to matched peers with SNHL on measures of speech recognition. There were no significant differences across the ANSD and SNHL groups on any of the tested measures. CONCLUSION: Cochlear implantation is a viable treatment option for children with a diagnosis of ANSD who are not making auditory progress with hearing aids that have been fit using the Desired Sensation Level method (DSL v5.0). Expected outcomes of cochlear implantation for children with ANSD, excluding children with cochlear nerve deficiency, are no different than for children with non-ANSD SNHL. These results are important for counseling families on the expected outcomes and realistic expectations following cochlear implantation for children with ANSD who demonstrate no evidence of cochlear nerve deficiency.