Contralateral hearing aid use in adult cochlear implant recipients: retrospective analysis of auditory outcomes.

OBJECTIVE: To investigate retrospectively the frequency of usage of bimodal stimulation among cochlear implant (CI) users, as well its clinical benefit relative to unilateral use. DESIGN: All subjects had been monitored with the clinical Minimal Outcome Measurements test battery. STUDY SAMPLES: 103 adults with bilateral postlingual profound sensorineural hearing loss and unilateral CI use were extracted from the local database. These were divided into two groups: those who only used a CI and those who used bimodal stimulation. RESULTS: The preoperative contralateral residual hearing in the bimodal group was significantly better than that of the CI-only group. In both groups, speech perception in quiet and in noise improved after CI, with no significant difference between postoperative unimodal conditions. For the bimodal group, an additional significant improvement was found for the bimodal condition compared to the unimodal. CONCLUSION: Given the observed auditory benefit of bimodal stimulation in comparison to unimodal stimulation and given the finding that degree of residual hearing is not correlated with bimodal benefits, it is recommended to encourage CI recipients to continue contralateral HA use after CI. As a result of expanding CI criteria worldwide, the population of bimodal users is expected to grow in the near future.

The use of melatonin for auditory brainstem response audiometry in children with comorbidities.

PURPOSE: In this study, the efficacy and feasibility of melatonin in young children with and without comorbidities, undergoing auditory brainstem response audiometry (ABR) was evaluated. The aim of this study was primarily to evaluate the use of melatonin for ABR investigations in children with comorbidities. Second, the efficacy of melatonin was evaluated based on several factors like sleep-onset latency, sleep duration, frequency of awakenings as well as adverse events. METHODS: Click-induced ABR tests were performed at the outpatient clinic between January, 2018 and August, 2020. Investigations were considered successful when binaural testing was completed. A dose of melatonin depending on age, 5 mg for children younger than 6 years and 10 mg if older than 6 years, was administered after placement of electrodes. RESULTS: 131 children were included in this study. 87% of all ABR investigations were performed successfully. Comorbidities such as neurodevelopmental disorders or developmental delays were present in 70% of all children. There was no significant difference in age (p = 0.36) or gender (p = 0.97) between the success and failed group. In addition, comorbidities were equally distributed between both groups. Mean sleep duration was 38 (SD 21) min and sleep-onset latency was 28 (SD 20) min No adverse events were documented. CONCLUSION: Melatonin is effective for ABR examinations in infants and children with and without comorbidities. Furthermore, it allows for sequential testing in those at risk for progressive hearing loss. Clear instructions to caregivers and expertise of audiologists are a prerequisite for optimal outcomes.

Targeted Next-Generation Sequencing in Children With Bilateral Sensorineural Hearing Loss: Diagnostic Yield and Predictors of a Genetic Cause.

OBJECTIVE: To investigate the diagnostic yield of targeted next-generation sequencing using hearing loss panels and to identify patient-related factors that are associated with a definite genetic cause. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Children with congenital or late-onset, bilateral sensorineural hearing loss. INTERVENTIONS: Diagnostic. MAIN OUTCOME MEASURES: The number of patients with a definite genetic diagnosis. RESULTS: We report on 238 patients with hearing loss: 130 were male and 108 were female. About 55% had congenital hearing loss. A genetic cause was identified in 94 of the patients (39.5%), with 72.3% of these showing nonsyndromic and 27.6% showing syndromic hearing loss. The diagnostic yield was highest among North African patients (66.7%). A multiple linear regression model shows that profound hearing loss, family history of hearing loss, congenital hearing loss, and North African ethnicity are significantly related to identifying a genetic cause. CONCLUSIONS: Targeted next-generation sequencing using a panel of hearing loss genes identified a genetic diagnosis in almost 40% of children with bilateral sensorineural hearing loss. We describe the predictors of a genetic diagnosis, and this information may be used during genetic counseling.

Neural Substrates of Tinnitus in an Auditory Brainstem Implant Patient: A Preliminary Molecular Imaging Study Using H2 15 O-PET Including a 5-year Follow-up of Auditory Performance and Tinnitus Perception.

INTRODUCTION: It was previously demonstrated that tinnitus due to profound unilateral hearing loss can be treated by the use of electrical stimulation via a cochlear implant (CI) with long-lasting positive effects. In cases where patients are not suitable for cochlear implantation due to aplasia/hypoplasia, cochlear malformations etc., an auditory brainstem implant (ABI) may be a solution. While auditory performance with ABI is well investigated, it is currently unknown whether stimulation through ABI also renders tinnitus reduction in patients with incapacitating tinnitus. The current case study reports on the subjective tinnitus perception during a 5-year follow-up period. In addition, a first H2O PET imaging study in an ABI patient is carried out revealing underlying neural substrates of tinnitus. METHODS: A 56-year-old male single-sided deaf patient with incapacitating tinnitus received an ABI after insufficient auditory performances and only minor tinnitus reduction with CI. Audiological follow-up was carried out during a 5-year follow-up period comprising pure-tone audiometry, speech-in-quiet testing, speech-in-noise testing, tinnitus questionnaires (tinnitus questionnaire and numeric rating scale) and the HISQUI19 questionnaire. To investigate the neural substrates of tinnitus in this subject, H2O PET tomography scans were acquired in three different conditions: 1) ABI switched off which was considered as the resting-state measurement rendering the loudest possible tinnitus for the patient (ABI OFF); 2) ABI switched on causing a small suppression of tinnitus due to electrical stimulation (ABI ON); 3) ABI switched on and 70 dB SPL white noise presented directly to the external audio processor through a direct audio cable providing the maximum tinnitus suppression for the patient (NOISE). RESULTS: Subjectively the patient reported a significant tinnitus reduction after implantation which remained stable over time with a decrease in tinnitus questionnaire from grade 4 to grade 2 and a 50% reduction in the numeric rating scale (from 8 to 4) during the 5-year period. Comparing the ABI OFF and ABI ON conditions, significant increase in regional cerebral blood flow (rCBF) was observed in brain areas involved in the salience network showing already suppression of tinnitus only by electrical stimulation in the absence of auditory stimuli. The NOISE condition showed relatively decreased rCBF in the insula (as well as in the orbitofrontal cortex) as compared with the ABI OFF condition. Abnormally activated areas comprising the salience network may have been significantly suppressed by the NOISE condition both by acoustic and electrical stimulations of the auditory pathway. Moreover, the NOISE condition showed significantly decreased rCBF in the parahippocampus as compared with the ABI OFF condition. This finding supports the idea of distinct tinnitus generators depending on the amount of hearing loss. CONCLUSION: The reduction of tinnitus in the current ABI subject may be attributable to partial peripheral reafferentation-induced deactivation of the parahippocampus-based tinnitus generator as well as the salience network. Further validation is required by the use of a follow-up study with a larger number of subjects.

Auditory Performances in Older and Younger Adult Cochlear Implant Recipients: Use of the HEARRING Registry.

OBJECTIVE: To evaluate the hearing outcomes of cochlear implantation in different age groups by using data collected in the HEARRING registry. METHODS: A multicenter study. Data of 146 patients were collected in a HEARRING registry. Patients were divided into three different age groups; ≤ 55 years old (age group 1, n = 66), 56 to 69 years old (age group 2, n = 45), and ≥ 70 years old (age group 3, n = 35). Speech in quiet (SPIQ), speech in noise (SPIN), and hearing implant sound quality index (HISQUI19) scores were evaluated for the different age groups at different test moments (preoperatively, 3, 6, 12, and 24 mo after first fitting). RESULTS: A statistically significant difference (p < 0.01) was found between preoperative scores and the scores on all the follow-up moments across all age groups. For SPIQ and SPIN, none of the time points showed a statistically significant age effect (p = 0.88 and p = 0.89). For HISQUI19 scores, a statistically significant age effect was found at 12 months after first fitting. The oldest age group scored significantly lower on the HISQUI19 compared with the youngest age group. CONCLUSION: Hearing outcomes of adult cochlear implant users of different age groups were evaluated. The SPIQ and SPIN tests showed no significant differences between the different age groups. Nevertheless, the youngest group scored significantly better on self -perceived benefit (HISQUI19) with a cochlear implant compared with the oldest age group.Further research is needed to receive more insight into cochlear implantation in the elderly and its implications on rehabilitating and supporting this expanding older population.

Impaired Cognitive Functioning in Cochlear Implant Recipients Over the Age of 55 Years: A Cross-Sectional Study Using the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing-Impaired Individuals (RBANS-H).

Primary Objective: To compare cognitive functioning among experienced, unilateral cochlear implant (CI) recipients and normal-hearing (NH) controls by means of the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing-impaired individuals (RBANS-H). Methods: Sixty-one post-lingually and bilaterally severely hearing-impaired CI recipients (median age: 71.0, range: 58.3 to 93.9 years) with at least 1 year of CI experience (median: 12.4, range: 1.1 to 18.6 years) and 81 NH control participants (median age: 69.9, range: 50.1 to 87.1 years) took part in this cross-sectional study. The RBANS-H was performed, as well as an audiometric assessment, including best-aided speech audiometry in quiet (monosyllabic words) and in noise (Leuven Intelligibility Sentences test). Results: The RBANS-H performances of the CI recipients (mean: 88.1 ± 14.9) were significantly poorer than the those of the NH participants (mean: 100.5 ± 13.2), with correction of age, sex, and education differences (general linear model: p = 0.001). The mean difference, corrected for the effects of these three demographic factors, was 8.8 (± 2.5) points. Additionally, in both groups, a significant correlation was established between overall cognition and speech perception, both in quiet and in noise, independently of age. Conclusion: Experienced, unilateral CI recipients present subnormal cognitive functioning, beyond the effect of age, sex and education. This has implications for auditory rehabilitation after CI and may highlight the need for additional cognitive rehabilitation in the long term after implantation. Long-term prospective and longitudinal investigations are imperative to improve our understanding of cognitive aging in severely hearing-impaired individuals receiving CIs and its association with CI outcomes.

The Repeatable Battery for the Assessment of Neuropsychological Status for Hearing Impaired Individuals (RBANS-H) before and after Cochlear Implantation: A Protocol for a Prospective, Longitudinal Cohort Study.

Background: Currently, an independent relationship between hearing loss and cognitive decline in older adults is suggested by large prospective studies. In general, cochlear implants improve hearing and the quality of life in severely to profoundly hearing impaired older persons. However, little is known about the effects of cochlear implantation on the cognitive evolution in this population. Aim of the study: The primary goal of this prospective, longitudinal cohort study is to explore the cognitive profile of severely to profoundly postlingually hearing impaired subjects before and after cochlear implantation. In addition, the current study aims to investigate the relationship between the cognitive function, audiometric performances, quality of life, and self-reliance in these patients. Methods: Twenty-five patients aged 55 or older, scheduled for cochlear implantation, will be enrolled in the study. They will be examined prior to implantation, at 6 and 12 months after implantation and annually thereafter. The test battery consists of (1) a cognitive examination, using the Repeatable Battery for the Assessment of Neuropsychological Status adapted for Hearing impaired persons (RBANS-H), (2) an audiological examination, including unaided and aided pure tone audiometry, speech audiometry in quiet and speech audiometry in noise, (3) the administration of four questionnaires evaluating quality of life and subjective hearing benefit and (4) a semi-structured interview about the self-reliance of the participant. Discussion: Up until now only one study has been conducted on this topic, focusing on the short-term effects of cochlear implantation on cognition in older adults. The present study is the first study to apply a comprehensive neuropsychological assessment adapted for severely to profoundly hearing impaired subjects in order to investigate the cognitive capabilities before and after cochlear implantation. Trial registration: The present protocol is retrospectively registered at Clinical Trials (ClinicalTrials.gov) on June 9th, 2016. The first participant was enrolled on June 22nd, 2015. The protocol identifier is NCT02794350.

Otitis media with effusion: an underestimated cause of hearing loss in infants.

OBJECTIVE: Otitis media with effusion (OME) is the major reason for failure of neonatal hearing screening. However, little is known about the impact on hearing status of OME in infants during the first months of life. PATIENTS: Infants who failed universal newborn hearing screening. METHODS: Prospective evaluation for the presence of OME, the degree of hearing loss and the disease evolution. RESULTS: Between 2007 and 2008, approximately 152 infants were referred because of unilateral or bilateral failure on universal newborn hearing screening with an automated auditory brainstem response device. Eighty-four (55.3%) had OME, 20 of them were lost to follow-up. The remainder 64 infants comprised 37 boys and 27 girls, with a median age of 49 days (range, 40-65 d) at admission. Auditory brainstem response thresholds were 50 dB nHL (range, 40-60) in both ears. A spontaneous resolution of OME was documented in 15 infants. In the remaining, hearing normalized after tympanocentesis or placement of ventilation tubes. Normal hearing could be ascertained in all children at a median age of 4.8 months (range, 3.3-7.8 mo). In the group of infants analyzed, no permanent hearing loss could be detected. CONCLUSION: OME is an important cause of transient, moderately severe hearing loss during the first months of life--a critical period for development of the auditory system. Active treatment should be considered if spontaneous resolution does not occur to prevent any delay in language acquisition and to exclude an underlying sensorineural hearing loss.