Auditory Outcomes Following Cochlear Implantation in Children with Unilateral Hearing Loss.

OBJECTIVE: Unilateral hearing loss (UHL) in children is associated with speech and language delays. Cochlear implantation (CI) is currently the only rehabilitative option that restores binaural hearing. This study aims to describe auditory outcomes in children who underwent CI for UHL and to determine the association between duration of hearing loss and auditory outcomes. STUDY DESIGN: Retrospective case series. SETTING: Three tertiary-level, academic institutions. PATIENTS: Children <18 years with UHL who underwent CI between 2018 and 2021. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Speech perception and Speech, Spatial and Qualities of Hearing Scale (SSQ) were assessed postimplantation. Scores >50% on speech perception and SSQ scores >8 points were considered satisfactory. Associations between duration of UHL and implantation age and outcomes were assessed using Spearman's rank correlation. RESULTS: Of the 38 children included, mean age at CI was 7.9 ± 3.2 years and mean UHL duration was 5.0 ± 2.8 years. Mean datalogging was 8.1 ± 3.1 hours/day. Mean auditory testing scores were SSQ, 7.9 ± 1.2; BABY BIO, 68.1 ± 30.2%; CNC, 38.4 ± 28.4%; WIPI, 52.5 ± 23.1%. Scores >50% on CNC testing were achieved by 40% of patients. SSQ scores >8 points were reported by 78% (7/9) of patients. There were no significant correlations between UHL duration and auditory outcomes. CONCLUSION: Overall, children with UHL who undergo CI can achieve satisfactory speech perception scores and SSQ scores. There were no associations between duration of hearing loss and age at implantation with auditory outcomes. Multiple variables may impact auditory outcomes, including motivation, family support, access to technology, and consistent isolated auditory training postactivation and should be taken into consideration in addition to age at implantation and duration of UHL in determination of CI candidacy.

Health-Related Quality of Life in Children With Unilateral Sensorineural Hearing Loss Following Cochlear Implantation.

OBJECTIVE: Evaluate health-related quality of life (HR-QOL) benefits with cochlear implantation (CI) in children with unilateral sensorineural hearing loss (USNHL) versus bilateral sensorineural hearing loss (BSNHL). STUDY DESIGN: A cross-sectional survey of parents of children who underwent CI for USNHL and BSNHL. SETTING: Tertiary care academic centers. METHODS: The "Children with cochlear implants: parental perspectives" survey was administered. Parents rated responses on a 5-point Likert scale. Scores greater than 3.0 were considered favorable. Responses were recorded within 8 domains and groups were compared with respect to domain scores. Analysis of covariance models was used to compare groups while adjusting for age at implantation and duration of implant use. RESULTS: There were 31 patients with USNHL and 27 patients with BSNHL. The average age of implantation in BSNHL patients was 1.9 and 6.7 years for USNHL. Parents of all children answered favorably in all domains. When adjusted for age at implantation and duration of implant use, parents of BSNHL children responded significantly more favorably only in 2 domains. When comparing patients with older age or prolonged duration of hearing loss in the USNHL cohort, there were favorable responses in all domains with no significant differences between groups. CONCLUSION: There are HR-QOL benefits of CI in USNHL children; less pronounced favorable results were noted only in 2 domains when compared to BSNHL children. Benefits were noted with CI in USNHL children at an older age at implantation or prolonged duration of hearing loss. Therefore, these factors should not be absolute contraindications for CI in USNHL.

Intraoperative Neural Response Telemetry and Auditory Outcomes in Pediatric Cochlear Implantation.

OBJECTIVE: To evaluate the relationship between intraoperative neural response telemetry (NRT) and postoperative auditory testing outcomes in children. STUDY DESIGN: Retrospective study. SETTING: Tertiary-care academic center. METHODS: Children who underwent cochlear implantation using the Cochlear Corporation device between 2010 and 2019 were included. Associations of average NRT and the slope of amplitude with postoperative auditory outcomes including functional auditory measure Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), and speech perception testing (consonant-nucleus-consonant [CNC], Pediatric AzBio [BABY BIO], Hearing In Noise Test [HINT], and Northwestern University Children's Perception of Speech [NU-CHIPS]), measured between 6 and 57 months after implantation, were assessed using Spearman's rank correlation (ρ). RESULTS: Thirty-eight patients (19 female, 19 male) and 54 ears were included. The median age of implantation was 20.6 months (range 9.6 months to 10.6 years). Eight (21%) children had neurologic disorders such as stroke, epilepsy, cerebral palsy, and other causes. Thirteen (34%) children had connexin mutations. Average NRT was not significantly correlated with postoperative auditory outcomes (IT-MAIS [ρ = -0.08, p = .74], CNC [ρ = 0.19, p = .32], BABY BIO [ρ = 0.21, p = .29], HINT [ρ = 0.05, p = .83]) and NU-CHIPS (ρ = 0.21, p = .28). The average slopes of amplitude and comfort level were not strongly correlated with any auditory outcomes (p > .05). CONCLUSIONS: Intraoperative NRT was not correlated with any postoperative functional auditory outcomes. Patient counseling should include discussions that a subpar intraoperative cochlear response does not preclude favorable speech and auditory outcomes.

Speech and Language outcomes in Auditory Neuropathy Spectrum Disorder (ANSD) children managed with amplification.

OBJECTIVE: To evaluate speech and language outcomes in children with Auditory Neuropathy Spectrum Disorder (ANSD) without significant comorbidities who received hearing rehabilitation in the form of hearing aids and/or cochlear implantation. METHODS: Retrospective chart review of pediatric ANSD patients at a large academic tertiary care institution from 2010 to 2019. Patients were included if they received a diagnosis of bilateral ANSD, had minimal to no comorbidities, and had speech and language testing (SLT) on at least two occasions. RESULTS: 51 patients were reviewed and 7 met inclusion criteria. Average age at ANSD diagnosis was 1 year and 11 months, and average age of hearing aid fitting was 3 years and 3 months. Hearing loss ranged from mild to profound, with four of the children wearing behind (BTE) hearing aids and three eventually receiving cochlear implants. Four of five patients who received hearing aids prior to their first speech and language evaluation demonstrated a delay at their initial evaluation, and all five patients continued to demonstrate a delay at their most recent evaluation, despite appropriate audiologic management and speech and language therapy. There were two patients who were unaided at the time of their initial and latest evaluations; one patient showed a delay at both timepoints, and one patient showed no speech delay at either timepoint. CONCLUSIONS: Pediatric ANSD patients, who are otherwise typically developing and received hearing rehabilitation and speech and language therapy, continue to show a speech and language delay (SLD). This outcome underscores the importance of close monitoring of speech and language development, providing early amplification and/or cochlear implantation, and promoting additional education and psychosocial support.

Pediatric Bilateral Sensorineural Hearing Loss: Minimum Test Battery and Referral Criteria for Cochlear Implant Candidacy Evaluation.

Among the various cochlear implant systems approved by the Food and Drug Administration, current labeling for pediatric usage encompasses (1) bilateral profound bilateral sensorineural hearing loss in children aged 9 to 24 months and bilateral severe to profound sensorineural hearing loss in children older than 2 years; (2) use of appropriately fitted hearing aids for 3 months (this can be waived if there is evidence of ossification); and (3) demonstration of limited progress with auditory, speech, and language development. Pediatric guidelines require children to have significantly worse speech understanding before qualifying for cochlear implantation. The early years of life have been shown to be critical for speech and language development, and auditory deprivation is especially detrimental during this crucial time.Level of evidence: 2.

Auditory testing outcomes with hearing aids in patients with auditory neuropathy spectrum disorder.

OBJECTIVE: The objective of this study is to evaluate the audiologic outcomes with hearing aids in pediatric patients with auditory neuropathy spectrum disorder (ANSD) using the Infant Toddler-Meaningful Auditory Integration Scale (IT-MAIS), and the Ling 6 Sound Test (Ling 6). STUDY DESIGN: Case series. SETTING: Single tertiary care academic medical center. SUBJECTS AND METHODS: All pediatric patients with a confirmed diagnosis of ANSD on Auditory Brainstem Response (ABR) testing who presented to a single tertiary medical center between September 2008 and September 2018 were included. Only patients that underwent Infant Toddler-Meaningful Auditory Integration Scale (IT-MAIS) and/or Ling 6 Sound Test (Ling 6) were included in the study. Audiologic testing performed after cochlear implantation was excluded. RESULTS: 60 pediatric patients with ANSD were analyzed. There were 10 patients included in the study with documented hearing aid use who underwent IT-MAIS and/or Ling 6 testing. Average IT-MAIS score improved by 20.4% after initial or extended trial of amplification. Similarly, average Ling 6 score improved from 3.6 to 4.8 after initial or extended trial of amplification. The four patients who did not receive amplification had higher average IT-MAIS and Ling 6 scores. CONCLUSION: In most children with ANSD, IT-MAIS and Ling 6 Sound Test scores improved with initial hearing aid use and over time with extended hearing aid use. Long-term prospective, multi-institutional studies are needed to determine the impact of the natural history of ANSD, comorbidities, and socioeconomic variables on auditory function testing results in children with ANSD using hearing aids.

Speech and language outcomes in mild-moderate unilateral sensorineural hearing loss.

OBJECTIVE: The impact of mild-moderate unilateral sensorineural hearing loss (USNHL) on speech and language delay (SLD) is not well established. Objectives included (1) determining SLD prevalence in patients with mild-moderate USNHL in comparison to prevalence in the general population and severe-profound USNHL patients and (2) examining speech, language, and auditory function testing (SLAT) results in USNHL patients. METHODS: A retrospective chart review of pediatric patients with USNHL, classified using pure tone averages (PTA) into mild-moderate (PTA 21-60) and severe-profound (PTA ≥ 61) USNHL groups was conducted. Abnormal SLAT values defined SLD. Prevalence and association of SLD based on USNHL severity was calculated. Onesample binomial tests compared observed frequencies of SLD to reported values. RESULTS: Forty-nine patients were identified with USNHL; 34 patients underwent SLAT. SLD frequency for mild-moderate USNHL was 25% (95% CI, 9-49%), higher than the general population rate (5.95%). No statistically significant difference was noted between SLD frequency in mild-moderate versus severe-profound USNHL. There were no significant correlations between SLAT measures and PTA thresholds. CONCLUSION: There was a statistically significant increase in SLD in mild-moderate USNHL compared to the general population. There were no correlations between SLAT measures and PTA thresholds. Children with USNHL need close monitoring of speech, language and auditory development and functioning. Studies with larger sample sizes will help delineate if these findings truly reflect results in children with USNHL.

Utility of a multidisciplinary approach to pediatric hearing loss.

INTRODUCTION: Because management of hearing loss (HL) often requires multiple specialists, a multidisciplinary clinic, Pediatric Hearing Management Clinic, (PHMC) was established to coordinate care for children with newly diagnosed HL. METHODS: Retrospective review of patients seen in PHMC from February 2009 to April 2010. RESULTS: Clinic information was available for 40/41 of the patients and was included in the study. 37/41 had confirmed HL. HL was categorized into bilateral/symmetric [15], bilateral/asymmetric [12] and unilateral [10]. Sixteen patients subsequently received hearing amplification after PHMC visit. Follow up was successfully established with otolaryngology in 23/32 (72%) patients, audiology in 29/40 (73%) patients, speech pathology in 11/12 (92%) patients, and ophthalmology in 15/30 (50%) patients. Twenty-nine patients completed evaluations of PHMC. The mean score in six total categories was 3.8/4.0 (4.0 as the highest satisfaction). CONCLUSION: A multidisciplinary clinic approach provides a convenient and effective way to coordinate care for children with HL.