Cochlear health in a cohort of cochlear implant users carrying the p.Pro51Ser variant in the COCH gene (DFNA9): A cross-sectional study evaluating the changes in the electrically evoked compound action potential (eCAP).

The present study focuses on DFNA9, an autosomal dominant disorder caused by pathogenic variants in the COCH gene. These mutations induce the formation of aggregates that are toxic to the fibrocytes in the extracellular matrix, ultimately leading to degeneration of spiral ganglion neurons (SGNs), which are crucial for transmitting auditory signals from the cochlea to the brain. An important tool for evaluating the function of the SGNs, which are the target cells of a cochlear implant (CI), is the electrically evoked compound action potential (eCAP).

Cognitive Changes Up to 4 Years After Cochlear Implantation in Older Adults: A Prospective Longitudinal Study Using the RBANS-H.

Objectives: Hearing loss is a worldwide health problem that currently affects around 20% of the world's population. Untreated hearing loss can have a significant impact on daily life, it can cause social isolation, loneliness, frustration, and higher anxiety and depression rates. Furthermore, older adults with hearing impairment have a higher risk for accelerated cognitive decline compared with normal-hearing individuals.

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.

Impact of hearing impairment and cochlear implantation on productivity and social well-being in a professionally active but severely hearing-impaired group: protocol of the 'Hear again, work again' longitudinal prospective cohort study.

Introduction: Severe hearing loss is a sensory deficit with considerable impact on the patient's daily life and on society. Previous research has established occupational obstacles in professionally active patients with hearing loss. However, studies investigating the impact of severe hearing loss and cochlear implantation (CI) on work performance using a quantitative and longitudinal study design and validated questionnaires are lacking.

Delayed Auditory Brainstem Responses in Prelingually Deaf and Late-Implanted Cochlear Implant Users.

Neurophysiological studies in animals and humans suggest that severe hearing loss during early development impairs the maturation of the auditory brainstem. To date, studies in humans have mainly focused on the neural activation of the auditory brainstem in children treated with a cochlear implant (CI), but little is known about the pattern of activation in adult CI users with early onset of deafness (prelingual, before the age of 2 years). In this study, we compare auditory brainstem activation in prelingually deaf and late-implanted adult CI users to that in postlingually deaf CI users.

The influence of newborn hearing screening on the age at cochlear implantation in children.

Objectives/hypothesis: To evaluate the influence of the introduction of newborn hearing screening programs on the age at cochlear implantation in children.

Study Design: Retrospective, multicenter cohort study.

Methods: All 1,299 pediatric cochlear implant users who received their implants before the age of 5 years between 1995 and 2011 in the Medical University Hannover, Germany and University Medical Center Utrecht, the Netherlands were enrolled in this study.

Altered cortical activity in prelingually deafened cochlear implant users following long periods of auditory deprivation.

Auditory stimulation during childhood is critical for the development of the auditory cortex in humans and with that for hearing in adulthood. Age-related changes in morphology and peak latencies of the cortical auditory evoked potential (CAEP) have led to the use of this cortical response as a biomarker of auditory cortical maturation including studies of cortical development after deafness and subsequent cochlear implantation. To date, it is unknown whether prelingually deaf adults, with early onset deafness (before the age of 2 years) and who received a cochlear implant (CI) only during adulthood, would display absent or aberrant CAEP waveforms as predicted from CAEP studies in late implanted prelingually deaf children.

Sound localization abilities of unilateral hybrid cochlear implant users with bilateral low-frequency hearing.

Objective: To assess the sound localization abilities of subjects unilaterally implanted with a hybrid cochlear implant in different sound localization conditions.

Study Design: A prospective, single-subject repeated measures design was performed to assess the sound localization abilities in 5 different listening conditions: combined (hybrid cochlear implant and contralateral acoustic hearing), bimodal, bilateral acoustic, ipsilateral acoustic, and contralateral acoustic.

Setting: Tertiary referral center.

Bilateral cochlear implantation in children: a systematic review and best-evidence synthesis.

Objectives/hypothesis: To evaluate the effectiveness of bilateral cochlear implantation over unilateral implantation in children with sensorineural hearing loss.

Data Sources: Pubmed, Embase, and Web of Science.

Review Methods: All studies comparing a bilateral cochlear implant group with a unilateral implant group were included.

Bilateral cochlear implantation in children and the impact of the inter-implant interval.

Objectives/hypothesis: To determine the effectiveness of simultaneous versus sequential bilateral cochlear implantation on postoperative outcomes in children with bilateral deafness and to evaluate the impact of the inter-implant interval and age at second implantation on postoperative outcomes in children who already received their first cochlear implant.

Data Sources: PubMed, Embase, and Web of Science.

Review Methods: All studies comparing the effects of simultaneous with sequential bilateral cochlear implantation on postoperative outcomes and those evaluating the impact of the inter-implant interval and age at second implantation were retrieved.

Hearing preservation surgery: cochleostomy or round window approach? A systematic review.

Objectives/hypothesis: An increasing number of patients with low-frequency residual hearing are fitted with a cochlear implant. The challenge is to optimize cochlear implant device properties and develop atraumatic surgical techniques to preserve residual hearing. In view of the ongoing debate about the optimal procedure for opening the cochlea during cochlear implantation, we reviewed the evidence on the round window and the cochleostomy insertion techniques and compared their effects on postoperative residual hearing.

The cost-utility of bilateral cochlear implantation: a systematic review.

Objectives/hypothesis: The (cost-)effectiveness and the subsequent reimbursement of bilateral cochlear implantation has been vigorously debated. Throughout the world healthcare commissioners are still struggling with the decision to reimburse bilateral implantation. Given this debate, this study's objective was to review the literature on the cost-utility of bilateral cochlear implantation in both children and adults, and study the impact of the used cost and quality-of-life estimates.