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.

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.

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.

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.

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.