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.

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.

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.

Background: Confirmation of permanent hearing loss in a newborn should be followed by a search for an underlying etiology because this may impact hearing loss management and counselling. Methods: Retrospective chart review of all newborns seen at a tertiary referral center after referral from newborn hearing screening over a 20-year period. The changes in the diagnostic protocol over the years are outlined and the most recent protocol includes targeted next-generation sequencing using a panel for known hearing loss causing genes, in all cases of bilateral sensorineural hearing loss (SNHL). Results: Permanent hearing loss was confirmed in 235 of 1,002 neonates. A complete etiological work-up was performed in 138 cases of SNHL (77 bilateral and 61 unilateral), with the underlying cause found in 77.9% and in 67.2% of patients respectively. Genetic causes explained 55 (58.4%) of bilateral cases and in 17 a genetic cause was identified by the gene panel. Pathogenic variants in GJB2 and MYO15A explained most cases of nonsyndromic SNHL. Waardenburg syndrome was the most frequent syndromic cause. Cochlear nerve deficiency and congenital cytomegalovirus infection accounted for the majority of unilateral SNHL. Other causes of congenital hearing loss were conductive hearing loss (n = 12) and auditory neuropathy/dyssynchrony (n = 9). Conclusion: Implementation of targeted next-generation sequencing in the etiological work-up improves the diagnostic yield in congenital SNHL, leaving only about 20% of bilateral and 30% of unilateral cases unsolved.

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.