The effect of hearing aid treatment on health-related quality of life in older adults with hearing loss.

OBJECTIVE: To investigate the effect of hearing aid (HA) intervention on long-term health-related quality of life (HRQoL) changes in first-time and experienced HA users using the 15D questionnaire. Secondly, the study explored the relationship between clinical parameters and changes in 15D scores. DESIGN: A prospective observational study. STUDY SAMPLE: The study population included 1562 patients (1113 first-time and 449 experienced HA users) referred for HA rehabilitation. All patients responded to the 15D at baseline, two months after HA fitting, and at long-term follow-up (698 ± 298 d). RESULTS: Among both first-time and experienced HA users, significant improvements in hearing-dimension (15D-3) score were observed at two-month follow-up which sustained at long-term follow-up. 15D total scores significantly decreased at long-term follow-up. Self-reported hearing abilities, word recognition scores, and HA use time were significantly and positively correlated to increased 15D. CONCLUSIONS: Both groups of HA users reported improved hearing-related QoL after HA treatment which sustained at long-term follow-up but the improvement in total 15D total score did not sustain for either group. The results suggest that HA intervention positively affects hearing-related QoL among older adults with hearing loss, and the findings support the use of 15D as a tool for the evaluation of HA treatment effects.

A case report of sudden-onset auditory neuropathy spectrum disorder associated with Brown-Vialetto-Van Laere syndrome (riboflavin transporter deficiency).

OBJECTIVE: The purpose of this paper is to describe a child with auditory neuropathy spectrum disorder (ANSD) associated with Brown-Vialetto-Van Laere (BVVL) syndrome, which is a rare, inherited, neurodegenerative disorder that is caused by defects in riboflavin transporter genes. DESIGN: We report the audiological and clinical profile of a child who presented with a complaint of sudden loss of speech understanding associated with an atypical form of ANSD. He was later diagnosed with BVVL. STUDY SAMPLE: An 11-year-old boy with ANSD associated with BVVL. RESULTS: The patient's severe neurological symptoms improved within a year of supplementation with high doses of riboflavin. His fluctuating hearing loss and 0% WDS remained unchanged. The patient was able to use hearing aids without any discomfort after treatment initiation, but he stopped using them again due to a lack of benefit in speech understanding. Although cochlear implantation was recommended, the patient and his family decided not to consider it for another year since they still had hope for complete recovery. CONCLUSIONS: Sudden-onset ANSD can be the earliest sign of undetected BVVL syndrome. Early detection of BVVL is crucial since all symptoms can be reversible with an early intervention of high doses of riboflavin supplementation.

Learning difficulties and auditory processing deficits in a clinical sample of primary school-aged children.

Objective: The current study examined the relationship between learning and auditory processing (AP) ability in a clinical sample of children with and without learning difficulties (LD).Design: A non-randomised, cross-sectional, single measure research design was used.Study sample: The participants consisted of 50 children (7.7-10.8 years) who had been referred for a clinical AP assessment based on having been referred from a school-based AP screening. These children had previously been identified as having (n = 14) or not having (n = 36) LD.Results: Children with LD performed significantly worse than children without LD on frequency patterns with linguistic reports (FPlinR and FPlinL), dichotic digits (DD) and Auditory Word Memory - Forward (ANMF) tests, with significant correlations being observed between these variables and the learning score. The multiple linear regression showed that FPlinR, DDR and ANMF scores explained 50% of the variance in the learning score.Conclusion: The present study's results are most consistent with risk factor models linking AP to learning abilities in children where reduced AP abilities could put children at greater risk for LD. Further investigations into the potential relationships between AP, cognition, speech and language development, and learning ability in children are warranted.

The frequency-following response as an assessment of spatial processing.

Objective: It is important to detect children with difficulties distinguishing speech-in-noise early. Prompt identification may be assisted by an evoked potential. The aims of the present study were: 1) to evaluate the frequency-following response (FFR) as a measure of binaural processing and spatial listening and, 2) to investigate the relationship between the FFR and a behavioural measure of binaural processing and spatial listening. Design: A single group, repeated measures design. The FFR was recorded in two different spatial conditions and amplitudes compared to spatial listening ability. Study Sample: Thirty-two children (aged 6.0 to 13.1 years) with a range of spatial processing abilities as measured behaviourally using the Listening in Spatialised Noise Sentences test (LiSN-S). Results: FFR waveforms were elicited using speech-like stimuli in co-located and separated conditions. A significant (p≤0.005) spatial advantage effect was observed with larger amplitudes in the separated condition. No correlations were observed between FFR amplitude and LiSN-S results. Conclusions: The FFR shows promise as a measure of binaural processing and spatial listening, but could be measuring different processes to those measured by the LiSN-S.

Efficacy of a deficit specific auditory training program for remediation of temporal patterning deficits.

Objective: To determine the efficacy of a targeted auditory training programme as a remediation approach for children diagnosed with a temporal patterning disorder. Design: Temporal Patterning scores were determined at two-time points pre-intervention and again post-training. Measures were then repeated in half of the participants after a further non-training period to determine the sustainability of effects. Cognitive skills and outcome measures were assessed at all time-points. Study Sample: Ten children aged between 7 and 12 years of age, diagnosed with a temporal patterning processing disorder, were enrolled in the training programme. Results: A group mean of 9.2 hours training was completed. Conclusion: Significant, sustainable improvements on the Frequency Pattern Test were found (2.5 SD increase in score relative to mean of age matched-peers) at the completion of training. Duration of training did not predict the degree of improvement. Cognitive skills did not show significant changes in ability. Significant, sustainable improvements in temporal patterning ability were seen after completion of the training programme. No associated changes in cognitive skills were seen, suggesting independence of the skills. Deficit-specific approaches are available across the traditional test battery, however, determining the appropriate management plan for a child diagnosed with an auditory processing disorder requires a patient-centric approach.

Applying U.S. national guidelines for ototoxicity monitoring in adult patients: perspectives on patient populations, service gaps, barriers and solutions.

OBJECTIVES: To promote establishment of effective ototoxicity monitoring programs (OMPs), this report reviews the U.S. national audiology guidelines in relation to "real world" OMP application. Background is provided on the mechanisms, risks and clinical presentation of hearing loss associated with major classes of ototoxic medications. DESIGN: This is a non-systematic review using PubMed, national and international agency websites, personal communications between ototoxicity experts, and results of unpublished research. Examples are provided of OMPs in various healthcare settings within the U.S. civilian sector, Department of Defense (DoD), and Department of Veterans Affairs (VA). STUDY SAMPLE: The five OMPs compared in this report represent a convenience sample of the programs with which the authors are affiliated. Their opinions were elicited via two semi-structured teleconferences on barriers and facilitators of OMP, followed by a self-administered questionnaire on OMP characteristics and practices, with responses synthesized herein. Preliminary results are provided from an ongoing VA clinical trial at one of these OMP sites. Participants were 40 VA patients who received cisplatin chemotherapy in 2014-2017. The study arms contrast access to care for OMP delivered on the treatment unit versus usual care as provided in the audiology clinic. RESULTS: Protocols of the OMPs examined varied, reflecting their diverse settings. Service delivery concerns included baseline tests missed or completed after the initial treatment, and monitoring tests done infrequently or only after cessation of treatment. Perceived barriers involved logistics related to accessing and testing patients, such as a lack of processes to help patients enter programs, patients' time and scheduling constraints, and inconvenient audiology clinic locations. Use of abbreviated or screening methods facilitated monitoring. CONCLUSIONS: The most effective OMPs integrated audiological management into care pathways of the clinical specialties that prescribe ototoxic medications. More OMP guidance is needed to inform evaluation schedules, outcome reporting, and determination of actionable ototoxic changes. Guidance is also lacking on the use of hearing conservation approaches suitable for the mass testing needed to support large-scale OMP efforts. Guideline adherence might improve with formal endorsement from organizations governing the medical specialty stakeholders in OMP such as oncologists, pulmonologists, infectious disease specialists, ototolaryngologists and pharmacists.

Monitoring neonates for ototoxicity.

OBJECTIVES: Neonates admitted to the neonatal intensive care unit (NICU) are at greater risk of permanent hearing loss compared to infants in well mother and baby units. Several factors have been associated with this increased prevalence of hearing loss, including congenital infections (e.g. cytomegalovirus or syphilis), ototoxic drugs (such as aminoglycoside or glycopeptide antibiotics), low birth weight, hypoxia and length of stay. The aetiology of this increased prevalence of hearing loss remains poorly understood. DESIGN: Here we review current practice and discuss the feasibility of designing improved ototoxicity screening and monitoring protocols to better identify acquired, drug-induced hearing loss in NICU neonates. STUDY SAMPLE: A review of published literature. CONCLUSIONS: We conclude that current audiological screening or monitoring protocols for neonates are not designed to adequately detect early onset of ototoxicity. This paper offers a detailed review of evidence-based research, and offers recommendations for developing and implementing an ototoxicity monitoring protocol for young infants, before and after discharge from the hospital.

Chemoradiation-induced hearing loss remains a major concern for head and neck cancer patients.

OBJECTIVE: Review of the literature regarding hearing loss in patients with head and neck cancer treated with chemoradiation. DESIGN: Studies in the literature are reviewed that pertain to hearing loss sustained in head and neck cancer patients receiving cisplatin-based chemoradiation. Personal observations noted while treating these patients are also detailed. STUDY SAMPLE: PubMed was searched for pertinent articles regarding hearing loss in head and neck cancer patients receiving cisplatin chemotherapy and/or radiation. RESULTS: Studies on the incidence and severity of hearing loss in head and neck cancer patients are limited, but those studies suggest that the risk of hearing loss is greater with higher-dose regimens. CONCLUSIONS: Newer cisplatin chemotherapy regimens using lower, weekly doses may be associated with a lower incidence and severity of hearing loss; however, large prospective studies are needed. Such information will be paramount to effective pre-treatment counselling of head and neck cancer patients.

Evidence-based interventions of dichotic listening training, compensatory strategies and combined therapies in managing pupils with auditory processing disorders.

OBJECTIVE: The need to develop a measure of managing children with a single profile of auditory processing disorders (APDs), and differentiate between true and artefactual improvements necessitated the study. The study also sought to determine the efficacy of interventions - both single and combined on APD, against no-treatment. DESIGN: A randomised controlled trial of interventions (RCT) was adopted. Participants were randomly allocated to each of the intervention groups or the no intervention group. The 10 weeks intervention included 45 minutes three times a week therapeutic intervention on listening with noise and sound localisation ability in the home and school environments. STUDY SAMPLE: 80 pupils (7-11 years) with a single profile of APD participated in the study. RESULTS: Treatments were effective on the cocktail party and sound localisation. The best result was realised with the combined therapy (CT), and there was no significant difference in performance in the remaining treatment groups. CONCLUSION: The intervention groups were beneficial to pupils with APD and should be adopted by clinicians.

Auditory adaptation testing as a tool for investigating tinnitus origin: two patients with vestibular schwannoma.

OBJECTIVE: To enhance the understanding of tinnitus origin by disseminating two case studies of vestibular schwannoma (VS) involving behavioural auditory adaptation testing (AAT). DESIGN: Retrospective case study. STUDY SAMPLE: Two adults who presented with unilateral, non-pulsatile subjective tinnitus and bilateral normal-hearing sensitivity. At the initial evaluation, the otolaryngologic and audiologic findings were unremarkable, bilaterally. Upon retest, years later, VS was identified. RESULTS: At retest, the tinnitus disappeared in one patient and was slightly attenuated in the other patient. In the former, the results of AAT were positive for left retrocochlear pathology; in the latter, the results were negative for the left ear although a moderate degree of auditory adaptation was present despite bilateral normal-hearing sensitivity. Imaging revealed a small VS in both patients, confirmed surgically. CONCLUSION: Behavioural AAT in patients with tinnitus furnishes a useful tool for exploring tinnitus origin. Decrease or disappearance of tinnitus in patients with auditory adaptation suggests that the tinnitus generator is the cochlea or the cochlear nerve adjacent to the cochlea. Patients with unilateral tinnitus and bilateral, symmetric, normal-hearing thresholds, absent other audiovestibular symptoms, should be routinely monitored through otolaryngologic and audiologic re-evaluations. Tinnitus decrease or disappearance may constitute a red flag for retrocochlear pathology.