The impact of cochlear implantation on speech and language outcomes in children with asymmetric sensorineural hearing loss.

INTRODUCTION: Cochlear implantation (CI) is considered in children with asymmetric sensorineural hearing loss (ASHL), or severe to profound sensorineural hearing loss in one ear, and better hearing contralaterally. The benefits of a CI in these children are not well established. This study investigated the impact of unilateral CI on speech and language outcomes in pediatric patients with ASHL. METHODS: The retrospective chart review identified pediatric patients who underwent cochlear implantation for ASHL at a tertiary single center institution between 2014 and 2017. Children who underwent cochlear implantation in the poorer ear and maintained a hearing aid in the better ear were included, while children with cognitive impairment or prior cochlear nerve deficiency and auditory neuropathy were excluded. Speech and language measures were evaluated prior to implantation and at regular six-month intervals postoperatively. Ear specific Audiometry was evaluated at every visit and varied developmentally appropriate speech recognition tasks, including questionnaires, closed-set, and open-set measures, accounted for advancing childhood milestones. Specific measures of speech articulation, expressive and receptive language, and vocabulary were assessed including: Goldman Fristoe Test of Articulation (GFTA-3), Clinical Evaluation of Language Fundamentals (CELF-P2 or CELF-5), Receptive One-Word Picture Vocabulary Test (ROWPVT) and Expressive One Word Picture Vocabulary Test (EOWPVT). The mean difference and standard deviation of speech and language outcomes at preoperative and postoperative evaluations were calculated. The change in outcomes were further assessed over each post-operative time point. RESULTS: Twenty-six children with asymmetric sensorineural hearing loss were included in the analysis, with 11 (42%) having bilateral enlarged vestibular aqueducts (EVA). At time of CI, unaided mean Pure Tone Average (PTA-4) for the better ear was 62Db HL, and worse ear was 92Db HL. There was an improvement in speech recognition in the implanted ear as expected. All preoperative speech and language evaluations were conducted within 7 months of cochlear implantation. Twenty-three patients had at least 1 postoperative re-evaluation. Fourteen children had preoperative and postoperative assessments with the same tool, with 13 patients (93%) showing improvement in at least 1 domain. For patients with repeated assessment within 12 months of surgery, we observed an average improvement in standard scores of: GFTA-3 (+11.2), CELF-P2 Core Language (+10.3), CELF-5 Core Language (+7.0), ROWPVT (+2.8) and EOWPVT (+4.5). Individual results are described. CONCLUSION: Improvement in speech and language measures can be demonstrated in children undergoing unilateral cochlear implantation for asymmetric sensorineural hearing losses. These children, who are not typical CI candidates, can benefit from a CI in the poorer ear.

Usability of a Mobile App for Improving Literacy in Children With Hearing Impairment: Focus Group Study.

BACKGROUND: Children with hearing loss, even those identified early and who use hearing aids or cochlear implants, may face challenges in developing spoken language and literacy. This can lead to academic, behavioral, and social difficulties. There are apps for healthy children to improve their spoken language and literacy and apps that focus on sign language proficiency for children with hearing loss, but these apps are limited for children with hearing loss. Therefore, we have developed an app called Hear Me Read, which uses enhanced digital stories as therapy tools for speech, language, and literacy for children with hearing loss. The platform has therapist and parent/child modes that allow (1) the selection of high-quality, illustrated digital stories by a speech-language pathologist, parent, or child; (2) the modification of digital stories for a multitude of speech and language targets; and (3) the assignment of stories by a therapist to facilitate individualized speech and language goals. In addition, Hear Me Read makes the caregiver a core partner in engagement through functionality, whereby the caregiver can record video and audio of themselves to be played back by the child. OBJECTIVE: This study aimed to evaluate the user experience of the Hear Me Read app through a focus group study with caregivers and their children. METHODS: We recruited 16 participants (8 children with and without hearing loss and 8 caregivers) to participate in 1-hour focus groups. Caregivers and children interacted with the app and discussed their experiences through a semistructured group interview. We employed thematic analysis methods and analyzed the data. We used feedback from the focus group to improve the elements of the app for a larger clinical trial assessing the impact of the app on outcomes. RESULTS: We identified three themes: default needs, specific needs, and family needs. Participants found the app to be esthetically pleasing and easy to use. The findings of this study helped us to identify usability attributes and to amend app functionalities to best fit user needs. Caregivers and children appreciated the enhancements, such as highlighting of parts of speech and caregiver reading of video playback, which were made possible by the digital format. Participants expressed that the app could be used to enhance family reading sessions and family interaction. CONCLUSIONS: The findings from this focus group study are promising for the use of educational apps designed specifically for those with hearing loss who are pursuing listening and spoken language as a communication outcome. Further investigation is needed with larger sample sizes to understand the clinical impact on relevant language and literacy outcomes in this population.

Positive Outcomes and Surgical Strategies for Bilateral Cochlear Implantation in a Child With X-Linked Deafness.

OBJECTIVES: To recognize that bilateral cochlear implantation (CI) in X-linked deafness is safe, describe techniques to maximize successful electrode placement and minimize surgical risks, and recognize that normalization of hearing as well as language acquisition is achievable. METHODS: A 6-month-old male patient presented with bilateral profound sensorineural hearing loss and was confirmed to have X-linked deafness secondary to POU3F4 gene mutation. Due to lack of benefit from amplification, he underwent bilateral CI in a staged fashion at 12 (right) and 15 months (left) of age. A transmastoid-facial recess approach was used bilaterally utilizing perimodiolar electrodes. High-flow gushers were controlled with muscle plugs. C-arm fluoroscopy was used during insertion of electrodes. RESULTS: Follow-up data are available for 12 months. No complications were encountered, including no postoperative cerebrospinal fluid (CSF) leakage or facial stimulation. Postoperative audiograms in aided conditions showed hearing thresholds <40 dB. At 11 months following activation of his second CI, he scored in the normal range for his chronological age on standardized language measures. CONCLUSION: With careful preparation and the assistance of intraoperative fluoroscopy, CI in patients with congenital X-linked deafness can be done safely. Performing bilateral CI followed by dedicated auditory-verbal rehabilitation may allow patients to achieve normal language development.