Neuroanatomical anomalies due to a defect in the FGF3 gene, associated with the Labyrinthine Aplasia, Microtia and Microdontia syndrome: insights from the placement of auditory brainstem implants in two siblings.

Here, we describe two congenitally deaf male siblings with the same compound heterozygotic, likely pathogenic mutations in the FGF3 gene, associated with the labyrinthine aplasia, microtia and microdontia (LAMM) syndrome. Both children had bilateral cochleovestibular aplasia, precluding cochlear implantation. The elder brother received an auditory brainstem implant (ABI) with very limited auditory responses.

Full-array channel discrimination in cochlear implants: validation and clinical application.

Objective: We sought to validate our proposed tool for estimating channel discrimination of cochlear implant (CI) users along the full electrode array and to assess associations between place-pitch discrimination and speech perception.

Design: In two tests, participants identified one stimulus (probe) as the odd-one-out compared with two reference stimuli. Probe stimuli were evoked using dual electrode stimulation characterised by the current steering coefficient α.

[Hearing without ears].

An Auditory Brainstem Implant (ABI) is a technique developed for patients with severe hearing loss. The ABI consists of a microphone and speech processor located on the scalp, which is connected to a transmitting and receiving coil and electrode on the brain stem placed in the skull. Eligible for an ABI are adults with cochlea and acoustic nerve damage due to neurofibromatosis type 2, and children with congenital malformation or aplasia, cochlear trauma or cochlear ossification after meningitis.

Evidence-Based Inclusion Criteria for Cochlear Implantation in Patients With Postlingual Deafness.

Objectives: This study determined the relationship between preoperative phoneme and word scores and the gain in speech perception after cochlear implantation in a large cohort of patients. The authors aimed to define evidence-based selection criteria for cochlear implantations in adults with postlingual deafness.

Design: This retrospective study included 364 adults with postlingual deafness who received a cochlear implant between 2000 and 2013 at the Leiden University Medical Center.

Intelligibility of the Patient's Speech Predicts the Likelihood of Cochlear Implant Success in Prelingually Deaf Adults.

Objectives: The objective of this study was to determine the validity and clinical applicability of intelligibility of the patient's own speech, measured via a Vowel Identification Test (VOW), as a predictor of speech perception for prelingually deafened adults after 1 year of cochlear implant use. Specifically, the objective was to investigate the probability that a prelingually deaf patient, given a VOW score above (or below) a chosen cutoff point, reaches a postimplant speech perception score above (or below) a critical value. High predictive values for VOW could support preimplant counseling and implant candidacy decisions in individual patients.

Effects of pulse width, pulse rate and paired electrode stimulation on psychophysical measures of dynamic range and speech recognition in cochlear implants.

Objectives: In this article results are described of a study on the effects of stimulation rate (PR), pulse duration (PW), and paired pulsatile stimulation (PPS) versus continuous interleaved sampling (CIS) on speech perception and psychophysical loudness measures.

Methods: During 3 nonconsecutive days, 27 postlingually deafened patients, implanted with either a CII or a HiRes90K with a HiFocus electrode array, were fitted with nine 12-channel strategies after a Latin-square design, systematically investigating the effect of stimulation rate (774-3868 pps/channel), PW (11-43 µsec/phase), and PPS versus CIS. Speech perception was measured in phonemes using open-set monosyllabic words.

Benefits of the HiRes 120 coding strategy combined with the Harmony processor in an adult European multicentre study.

Conclusion: The Harmony processor was found to be reliable, comfortable and offered a substantially increased battery life compared with the previous generation processor. No significant improvement in speech understanding with HiRes was demonstrated from objective measures, but the majority of subjects showed a clear subjective preference for the combination HiRes 120/Harmony processor.

Objectives: To evaluate experience with the Harmony™ sound processor, together with the HiRes 120 strategy.

Assessing the placement of a cochlear electrode array by multidimensional scaling.

Correct placement of the electrode is crucial for cochlear implantation (CI) surgery. It determines the access to the auditory nerve and subsequent hearing performance. Here, we propose an objective measures tool that can partially verify the electrode position.

Cochlear reimplantation with same device: surgical and audiologic results.

Objectives/hypothesis: To study to what extent it is possible to achieve identical insertion depths and to maintain the same performance after cochlear reimplantation.

Study Design: Outcome research on a retrospective case series in a tertiary university referral center.

Methods: Data were collected for 12 adults and three children who underwent reimplantation during the last 3 years with a new HiRes90K device with HiFocus 1J electrode owing to failure of the feed-through seal.

Speech intelligibility as a predictor of cochlear implant outcome in prelingually deafened adults.

Objectives: Among adult patients with prelingual deafness, interindividual variability in speech perception outcome after cochlear implantation is generally large. It was hypothesized that the intelligibility of the patient's own speech may be predictive of speech perception with a cochlear implant. The objectives were (1) to provide a validation of a new test battery as a measurement tool for intelligibility using a group of prelingually deafened patients; (2) to investigate the validity of the test battery as a predictor of postimplant speech perception, based on preliminary data with a cochlear implant from a subgroup of patients; and (3) to investigate the validity of the test battery as a predictor of postimplant health-related quality of life (HRQoL) for the same subgroup of patients.