Cochlear Implantation in Patients With Usher Syndrome Type IIa Increases Performance and Quality of Life.

Objectives: Usher syndrome type IIa (USH2a) is characterized by congenital moderate to severe hearing impairment and retinitis pigmentosa. Hearing rehabilitation starts in early childhood with the application of hearing aids. In some patients with USH2a, severe progression of hearing impairment leads to insufficient speech intelligibility with hearing aids and issues with adequate communication and safety.

Cochlear implantation and clinical features in patients with Noonan syndrome and Noonan syndrome with multiple lentigines caused by a mutation in PTPN11.

Existing literature only reports a few patients with Noonan syndrome (NS) and Noonan syndrome with multiple lentigines (NSML) who underwent cochlear implantation (CI). The present study describes four NS patients and one NSML patient with a PTPN11 mutation. They all had severe to profound hearing loss, and they received a CI.

Speech Characteristics and Intelligibility in Adults with Mild and Moderate Intellectual Disabilities.

Purpose: Adults with intellectual disabilities (ID) often show reduced speech intelligibility, which affects their social interaction skills. This study aims to establish the main predictors of this reduced intelligibility in order to ultimately optimise management.

Method: Spontaneous speech and picture naming tasks were recorded in 36 adults with mild or moderate ID.

The effect of device use after sequential bilateral cochlear implantation in children: An electrophysiological approach.

Objectives: In many studies evaluating the effect of sequential bilateral cochlear implantation in congenitally deaf children, device use is not taken into account. In this study, however, device use was analyzed in relation to auditory brainstem maturation and speech recognition, which were measured in children with early-onset deafness, 5-6 years after bilateral cochlear implantation. We hypothesized that auditory brainstem maturation is mostly functionally driven by auditory stimulation and is therefore influenced by device use and not mainly by inter-implant delay.

Wireless and acoustic hearing with bone-anchored hearing devices.

Objective: The efficacy of wireless connectivity in bone-anchored hearing was studied by comparing the wireless and acoustic performance of the Ponto Plus sound processor from Oticon Medical relative to the acoustic performance of its predecessor, the Ponto Pro.

Study Sample: Nineteen subjects with more than two years' experience with a bone-anchored hearing device were included. Thirteen subjects were fitted unilaterally and six bilaterally.

Active Middle Ear Implantation: Long-term Medical and Technical Follow-up, Implant Survival, and Complications.

Objective: To evaluate the long-term medical and technical results, implant survival, and complications of the semi-implantable vibrant soundbridge (VSB), otologics middle ear transducer (MET), and the otologics fully implantable ossicular stimulator (FIMOS).

Study Design: Retrospective cohort study.

Patients: Patients with chronic external otitis and either moderate to severe sensorineural or conductive/mixed hearing loss.

Frequency-dependent loudness balancing in bimodal cochlear implant users.

Conclusion In users of a cochlear implant (CI) and a hearing aid (HA) in contralateral ears, frequency-dependent loudness balancing between devices did, on average, not lead to improved speech understanding as compared to broadband balancing. However, nine out of 15 bimodal subjects showed significantly better speech understanding with either one of the fittings. Objectives Sub-optimal fittings and mismatches in loudness are possible explanations for the large individual differences seen in listeners using bimodal stimulation.

Three-year experience with the Sophono in children with congenital conductive unilateral hearing loss: tolerability, audiometry, and sound localization compared to a bone-anchored hearing aid.

Bone conduction devices (BCDs) are advocated as an amplification option for patients with congenital conductive unilateral hearing loss (UHL), while other treatment options could also be considered. The current study compared a transcutaneous BCD (Sophono) with a percutaneous BCD (bone-anchored hearing aid, BAHA) in 12 children with congenital conductive UHL. Tolerability, audiometry, and sound localization abilities with both types of BCD were studied retrospectively.

Temporal Cortex Activation to Audiovisual Speech in Normal-Hearing and Cochlear Implant Users Measured with Functional Near-Infrared Spectroscopy.

Background: Speech understanding may rely not only on auditory, but also on visual information. Non-invasive functional neuroimaging techniques can expose the neural processes underlying the integration of multisensory processes required for speech understanding in humans. Nevertheless, noise (from functional MRI, fMRI) limits the usefulness in auditory experiments, and electromagnetic artifacts caused by electronic implants worn by subjects can severely distort the scans (EEG, fMRI).

Matching Automatic Gain Control Across Devices in Bimodal Cochlear Implant Users.

Objectives: The purpose of this study was to improve bimodal benefit in listeners using a cochlear implant (CI) and a hearing aid (HA) in contralateral ears, by matching the time constants and the number of compression channels of the automatic gain control (AGC) of the HA to the CI. Equivalent AGC was hypothesized to support a balanced loudness for dynamically changing signals like speech and improve bimodal benefit for speech understanding in quiet and with noise presented from the side(s) at 90 degree.

Design: Fifteen subjects participated in the study, all using the same Advanced Bionics Harmony CI processor and HA (Phonak Naida S IX UP).

Audiometric Characteristics of a Dutch DFNA10 Family With Mid-Frequency Hearing Impairment.

Objectives: Mutations in EYA4 can cause nonsyndromic autosomal dominant sensorineural hearing impairment (DFNA10) or a syndromic variant with hearing impairment and dilated cardiomyopathy. A mutation in EYA4 was found in a Dutch family, causing DFNA10. This study is focused on characterizing the hearing impairment in this family.

Bone-anchored hearing implants in single-sided deafness patients: Long-term use and satisfaction by gender.

Objectives/hypothesis: To examine the long-term satisfaction and possible effects of gender in patients with single-sided deafness (SSD) who underwent bone-anchored hearing implant (BAHI) surgery.

Study Design: Retrospective case-control study.

Methods: All (n = 145) consecutive SSD patients fitted with a BAHI between January 2001 and October 2011 were asked to complete a questionnaire consisting of the Abbreviated Profile of Hearing Aid Benefit (APHAB), the Communication Profile for the Hearing Impaired (CPHI), and the SSD questionnaire.

Long-term Compliance and Satisfaction With Percutaneous Bone Conduction Devices in Patients With Congenital Unilateral Conductive Hearing Loss.

Objective: Patients with congenital unilateral conductive hearing loss (UCHL) can either be watchful monitored or treated surgically through the fitting of a percutaneous bone conduction device (BCD) or, in some cases, atresia repair. The current study evaluated the long-term compliance and satisfaction with a percutaneous BCD in this specific population.

Study Design: Fifty-three consecutive patients with congenital UCHL treated with a percutaneous BCD in our tertiary referral center between 1998 and 2011 were identified.

Nanogrooved surface-patterns induce cellular organization and axonal outgrowth in neuron-like PC12-cells.

Modulation of a materials surface topography can be used to steer various aspects of adherent cell behaviour, such as cell directional organization. Especially nanometric sized topographies, featuring sizes similar to for instance the axons of the spiral ganglion cells, are interesting for such purpose. Here, we utilized nanosized grooves in the range of 75-500 nm, depth of 30-150 nm, and pitches between 150 nm and 1000 nm for cell culture of neuron-like PC12 cells.

Long-term outcomes on spatial hearing, speech recognition and receptive vocabulary after sequential bilateral cochlear implantation in children.

Sequential bilateral cochlear implantation in profoundly deaf children often leads to primary advantages in spatial hearing and speech recognition. It is not yet known how these children develop in the long-term and if these primary advantages will also lead to secondary advantages, e.g.

Single-sided deafness and directional hearing: contribution of spectral cues and high-frequency hearing loss in the hearing ear.

Direction-specific interactions of sound waves with the head, torso, and pinna provide unique spectral-shape cues that are used for the localization of sounds in the vertical plane, whereas horizontal sound localization is based primarily on the processing of binaural acoustic differences in arrival time (interaural time differences, or ITDs) and sound level (interaural level differences, or ILDs). Because the binaural sound-localization cues are absent in listeners with total single-sided deafness (SSD), their ability to localize sound is heavily impaired. However, some studies have reported that SSD listeners are able, to some extent, to localize sound sources in azimuth, although the underlying mechanisms used for localization are unclear.

An experimental objective method to determine maximum output and dynamic range of an active bone conduction implant: the Bonebridge.

Introduction: Recently, a new active bone conduction implant, the Bonebridge, was introduced. This transcutaneous device is proposed as an alternative to previous percutaneous systems. The current study aims to determine the maximum output (MO) of the Bonebridge by making use of Bonebridge-generated sound pressure levels in the occluded ear canal of the unaided ear.

Cochlear implantation in late-implanted prelingually deafened adults: changes in quality of life.

Background: With expanding inclusion criteria for cochlear implantation, the number of prelingually deafened persons who are implanted as adults increases. Compared with postlingually deafened adults, this group shows limited improvement in speech recognition. In this study, the changes in health-related quality of life in late-implanted prelingually deafened adults are evaluated and related to speech recognition.

Amplification options for patients with mixed hearing loss.

Objectives: To compare amplification options for patients with mixed hearing loss. Devices tested include percutaneous and transcutaneous bone conductors (BCDs) and middle ear implants with their actuator directly coupled to the cochlea.

Setting: Tertiary academic medical center.

Similar phenotypes caused by mutations in OTOG and OTOGL.

Objectives: Recently, OTOG and OTOGL were identified as human deafness genes. Currently, only four families are known to have autosomal recessive hearing loss based on mutations in these genes. Because the two genes code for proteins (otogelin and otogelin-like) that are strikingly similar in structure and localization in the inner ear, this study is focused on characterizing and comparing the hearing loss caused by mutations in these genes.

Auditory cortical maturation in children with sequential bilateral cochlear implants.

Objective: To assess the effect of sequential bilateral cochlear implantation on auditory, cortical maturation after various periods of unilateral cochlear implant use.

Study Design: Prospective cohort study.

Setting: Tertiary academic referral center.

Dysfluencies in the speech of adults with intellectual disabilities and reported speech difficulties.

Background: In individuals with an intellectual disability, speech dysfluencies are more common than in the general population. In clinical practice, these fluency disorders are generally diagnosed and treated as stuttering rather than cluttering.

Purpose: To characterise the type of dysfluencies in adults with intellectual disabilities and reported speech difficulties with an emphasis on manifestations of stuttering and cluttering, which distinction is to help optimise treatment aimed at improving fluency and intelligibility.

Amplification options in unilateral aural atresia: an active middle ear implant or a bone conduction device?

Background: There is no consensus on treatment of patients with congenital unilateral aural atresia. Currently, 3 intervention options are available, namely, surgical reconstruction, application of a bone-conduction device (BCD), or application of a middle ear implant.

Objective: The present study aims to compare the BCD with the application of a middle ear implant.

Multicenter study with a direct acoustic cochlear implant.

Objective: To confirm the clinical efficacy and safety of a direct acoustic cochlear implant.

Study Design: Prospective multicenter study.

Setting: The study was performed at 3 university hospitals in Europe (Germany, The Netherlands, and Switzerland).

Evaluation of a new powerful bone-anchored hearing system: a comparison study.

Background: The recent introduction of digital hearing aid technology for bone-conduction devices employing percutaneous stimulation may be beneficial for patients with conductive and mixed hearing loss and single sided deafness.

Purpose: Performance of a recently released sound processor for bone-anchored implants, the Ponto Pro Power from Oticon Medical (bone-conduction device 2 [BCD2]), was compared with that of the Baha Intenso from Cochlear (bone-conduction device 1 [BCD1]).

Research Design: Direct comparison of the subject's own device (BCD1) with the new device (BCD2) was examined in a nonrandomized design.