Cochlear Implantation in Adults With Asymmetric Hearing Loss: Speech Recognition in Quiet and in Noise, and Health Related Quality of Life.

OBJECTIVE: To examine the possible speech recognition and health related quality of life (HRQoL) benefits of cochlear implantation among adults with asymmetric sensorineural hearing loss. STUDY DESIGN: Retrospective chart review, single-subject design. METHODS: A total of 45 adult cochlear implant recipients with asymmetric sensorineural hearing loss where performance for the best-aided condition exceeded 60% correct open set sentence recognition in quiet, and the implanted ear met traditional candidacy criteria. End point testing of the implanted ear was evaluated with use of the Consonant-Vowel Nucleus-Consonant (CNC) word test and AzBio sentence test materials in quiet, and bimodally with the AzBio sentence test materials in noise at +5 dB signal-to-noise ratio (SNR). HRQoL was measured using the Nijmegen Cochlear Implant Questionnaire (NCIQ). RESULTS: Measured in quiet, with the non-implanted ear plugged, the average CNC word scores increased from 9.1% preoperatively to 55.7% (p < 0.01) at the 6-month post-activation test interval. Similarly, average AzBio sentence scores in quiet, with the non-implanted ear plugged, increased from 13.9% preoperatively to 73.4% (p < 0.01) at the 6-month post-activation test interval. Finally, in the bilateral/bimodal condition, the AzBio sentence score in +5 dB SNR improved from an average of 26.8% preoperatively to 52.4% (p < 0.01) at the 6-month test interval. Results of the NCIQ showed improved scores on all six subdomains. CONCLUSIONS: These data demonstrate significant benefit of cochlear implantation among a group of postlingually deafened adults whose preoperative hearing and aided speech recognition fell outside of the currently specified Food and Drug Administration candidacy guidelines. Results of this study support the evaluation of a candidate's speech recognition in noise in the best-aided condition to adequately assess candidacy for a cochlear implant.

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.

Health-related quality of life outcomes following adult cochlear implantation: A prospective cohort study.

INTRODUCTION: The benefits of cochlear implantation extend beyond improved speech recognition and into overall health-related quality of life (HRQoL). Several measures of HRQoL, categorized as generic or disease specific, have been used in the cochlear implant literature. The clinical utility of generic HRQoL measures have been reported to be variable by previous investigators. The degree to which HRQoL correlates to speech perception is largely unknown. METHODS: A prospective single-subject design at a large tertiary care center. Self-reported HRQoL was measured at the preoperative and 12-month post-activation test intervals. The measures of HRQoL included a generic form, (Medical Outcome Study Short Form; SF-36), and disease specific form (Nijmegen Cochlear Implant Questionnaire; NCIQ). Speech recognition was measured at the preoperative, 6- and 12-months post-activation test intervals using the Consonant-Nucleus-Consonant (CNC) monosyllabic word test. RESULTS: A total of 61 patients (mean 67 years; range 30-87 years) were included in the final analysis. Average speech recognition on the CNC word test was 10% pre-operatively, and 66.7% at 12-months post-activation. The HRQoL scores improved significantly for seven of the eight subdomains of the NCIQ, and one of the nine domains of the SF-36. CONCLUSION: Cochlear implantation significantly improves HRQoL, regardless of age. Disease specific measures, such as the NCIQ, are better able to demonstrate differences in HRQoL compared to general health surveys.

Early outcomes after cochlear implantation for adults and children with unilateral hearing loss.

OBJECTIVES/HYPOTHESIS: This study was designed to examine speech recognition and self-perceived health-related quality of life (HRQoL) received from cochlear implantation among a cohort of adults and children with a short duration of unilateral hearing loss greater than 6 months, but less than 2 years. STUDY DESIGN: Single-subject repeated measures prospective study. METHODS: This study assessed changes in speech recognition and self-perceived quality of life by prospectively analyzing data at the preoperative evaluation and at the 3-month and 6-month postactivation intervals. Measurement tools included Medical Outcomes Study Questionnaire Short Form 36, Nijmegen Cochlear Implant Questionnaire, Speech Spatial and Qualities of Hearing-Comparative, and speech recognition measures in quiet and in noise. RESULTS: Results indicated significant improvement in speech recognition, both in quiet and noise. Quality-of-life measures showed a significant increase in self-perceived benefit with disease-specific instruments, but remained constant with a generic HRQoL instrument. CONCLUSIONS: Cochlear implantation was a successful intervention for improved hearing in quiet and noise, and a self-perceived benefit for this group of adults and children with a short duration of unilateral hearing loss. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:1683-1688, 2017.

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.

Outcomes of cochlear implantation in children with auditory neuropathy.

Ten children who were diagnosed with auditory neuropathy were matched with ten children who were diagnosed with other etiologies. All twenty children received cochlear implants at the Mayo Clinic in Rochester, Minnesota. Various measures were used to compare the outcomes for the two groups. We compared the children's unaided and aided audiograms, and measures of threshold and comfort levels. Performance on age appropriate speech perception tests was measured. Electrically elicited auditory brainstem response, predicted Neural Response Telemetry thresholds, and visually detected electrical stapedius reflexes were compared. Parental report of cochlear implant benefit was evaluated using either the Meaningful Auditory Integration Scale or the Infant-Toddler Meaningful Auditory Integration Scale depending on the age of the child. We also compared educational placement and communication mode. The results of this study demonstrated that there were no important differences in cochlear implant benefit between the two groups. In light of these findings, we support the use of cochlear implants as a viable option for selected children with auditory neuropathy.