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.

Teledelivery of Aural Rehabilitation to Improve Cochlear Implant Outcomes.

OBJECTIVE(S): This randomized controlled study evaluated the effectiveness of a Telehealth Aural Rehabilitation (TeleAR) training protocol to improve outcomes for adult cochlear implant (CI) users. METHODS: This was a multisite clinical study with participants randomized to either an AR treatment or active control group. The AR protocol consisted of auditory training (words, sentences, and speech tracking), informational counseling, and communication strategies. The control group participants engaged in cognitive stimulation activities (crosswords, sudoku, etc.). Each group completed 6 weekly 90-min individual treatment sessions delivered remotely. Twenty postlingually deafened adult CI users participated. Assessments were completed pretreatment and 1 week and 2 months posttreatment. RESULTS: Repeated-measures ANOVA and planned contrasts were used to compare group performance on AzBio Sentences, Hearing Handicap Inventory (HHI), Client Oriented Scale of Improvement (COSI), and Glasgow Benefit Inventory (GBI). The two groups were statistically equivalent on all outcome measures at pre-assessment. There was a statistically significant main effect of time for all measures. Improvement over time was observed for participants in both groups, with greater improvement seen for the AR than the CT group on all outcome measures. The AR group showed medium to large effect sizes on all measures over time, suggesting clinically significant outcomes. CONCLUSION: This randomized controlled study provides evidence of improved speech recognition and psychosocial outcomes following 6 weeks of TeleAR intervention. For adult post-lingually deafened CI users, including those >3 months post-activation, AR treatment can leverage neuroplasticity to maximize outcomes. LEVEL OF EVIDENCE: 2 Laryngoscope, 134:1861-1867, 2024.

Outcomes of audiometric testing in children with auditory neuropathy spectrum disorder.

INTRODUCTION: Auditory Neuropathy Spectrum Disorder (ANSD) is characterized by hearing loss ranging from normal to profound. Additionally, results are confounded by commonly fluctuating hearing thresholds in ANSD. As such, we sought to evaluate results of audiometric testing on children with ANSD and the impact of age and time on testing results. METHODS: Retrospective chart review on children <18 years of age diagnosed with ANSD at two tertiary care academic institutions. Data analyzed included initial audiogram with speech detection thresholds (SDT) and pure tone averages (PTA) as well as most recent unaided audiogram SDT and PTA. RESULTS: 75 ANSD patients were analyzed, of which 32 (42.7%) were female. Bilateral and unilateral ANSD was seen in 55 (73.3%) and 20 (26.7%), respectively. A total of 130 ears with ANSD were assessed with 80 (61.5%) meeting inclusion criteria. Of these patients, the median age in years at first audiogram and most recent audiogram were 1.94 (0.45-13.68) and 4.22 (0.97-14.61), respectively. The median ages at which an SDT and PTA could first be acquired on the audiogram in ANSD patients were 1.94 (0.50-13.68) and 2.86 (0.45-13.68), respectively. The average SDT/PTA at the initial and most recent audiogram were 47.5/45.7 and 49.4/53.0 dB, respectively. SDT to PTA within the same audiogram exhibited strong correlation (r = 0.82, p < 0.001). Similarly, comparison of initial SDT to SDT at a later time interval showed strong correlation (r = 0.73, p < 0.001). SDT and PTA at initial audiogram and PTA at later time demonstrated lower correlation but was still statistically significant (r = 0.49, p < 0.009 and r = 0.51, p < 0.044, respectively). Individual PTA was associated with age (r = -0.56, p < 0.001). CONCLUSIONS: SDT and PTA within the same audiogram and initial SDT to SDT acquired at a later time correlate strongly in audiometric testing in children with ANSD. Although not as strong, initial SDT and PTA still correlate with PTA at a later time interval. These findings suggest that audiometric results yielding a reliable SDT and frequency specific information necessary to calculate the PTA is not typically obtained until 2-3 years old. However, once this information is obtained, the child's hearing is fairly stable but may fluctuate over time.

Effects of AUT00063, a Kv3.1 channel modulator, on noise-induced hyperactivity in the dorsal cochlear nucleus.

The purpose of this study was to test whether a Kv3 potassium channel modulator, AUT00063, has therapeutic potential for reversing noise-induced increases in spontaneous neural activity, a state that is widely believed to underlie noise-induced tinnitus. Recordings were conducted in noise exposed and control hamsters from dorsal cochlear nucleus (DCN) fusiform cells before and following intraperitoneal administration of AUT00063 (30 mg/kg). Fusiform cell spontaneous activity was increased in sound-exposed animals, approximating levels that were nearly 50% above those of controls. Administration of AUT00063 resulted in a powerful suppression of this hyperactivity. The first signs of this suppression began 13 min after AUT00063 administration, but activity continued to decline gradually until reaching a floor level which was approximately 60% of pre-drug baseline by 25 min after drug treatment. A similar suppressive effect of AUT00063 was observed in control animals, with onset of suppression first apparent at 13 min post-treatment, but continuing to decline toward a floor level that was 54% of pre-drug baseline and was reached 28 min after drug treatment. In contrast, no suppression of spontaneous activity was observed in animals given similar injections of vehicle (control) solution. The suppressive effect of AUT00063 was achieved without significantly altering heart rate and with minimal effects on response thresholds, supporting the interpretation that the reductions of hyperactivity were not a secondary consequence of a more general physiological suppression of the brain or auditory system. These findings suggest that Kv3 channel modulation may be an effective approach to suppressing spontaneous activity in the auditory system and may provide a future avenue for treatment of tinnitus resulting from exposure to intense sound.