Balance and sound conditions in adults with bilateral cochlear implants.

Purpose: Determine if 1) balance is impaired in patients with bilateral cochlear implants compared to healthy controls; 2) the presence of sound, non-speech or speech, affects standing balance.

Materials And Methods: Four patients with bilateral cochlear implants were tested on three balance conditions on Romberg tests on medium density compliant foam with eyes closed, with head stationary or moving in yaw or pitch, under 5 sound conditions: no sound, ambient background noise, pink noise, foreign language, English language.

Results: Dependent measure were duration of standing and kinematics.

Early-onset severe neuromuscular phenotype associated with compound heterozygosity for OPA1 mutations.

Introduction: Pathogenic mutations in the OPA1 gene are the most common identifiable cause of autosomal dominant optic atrophy (DOA), which is characterized by selective retinal ganglion cell loss, a distinctive pattern of temporal pallor of the optic nerve and a typical color vision deficit, with variable effects on visual acuity. Haploinsufficiency has been suggested as the major pathogenic mechanism for DOA. Here we present two siblings with severe ataxia, hypotonia, gastrointestinal dysmotility, dysphagia, and severe, early-onset optic atrophy who were found to be compound heterozygotes for two pathogenic OPA1 mutations.

Comprehensive diagnostic battery for evaluating sensorineural hearing loss in children.

Objective: Selection of diagnostic tests for children with sensorineural hearing loss (SNHL) is influenced by clinical suspicion. Testing results reported in the literature are similarly biased. We evaluate the usefulness of a comprehensive diagnostic battery for each child.

Neuroimaging with near-infrared spectroscopy demonstrates speech-evoked activity in the auditory cortex of deaf children following cochlear implantation.

Cochlear implants (CI) are commonly used to treat deafness in young children. While many factors influence the ability of a deaf child who is hearing through a CI to develop speech and language skills, an important factor is that the CI has to stimulate the auditory cortex. Obtaining behavioral measurements from young children with CIs can often be unreliable.

Characteristics of malfunctioning channels in pediatric cochlear implants.

Objectives/hypothesis: To examine the characteristics of pediatric cochlear implant channel malfunction preceding device failure.

Study Design: : Retrospective review.

Methods: All pediatric patients who underwent cochlear implantation at a tertiary academic medical center were reviewed regarding device type, reason for replacement, time to replacement, and timing and pattern of channel faults in failed versus nonfailed devices.

Neurocognitive functioning of a child with partial trisomy 6 and monosomy 21.

This case study describes the neurocognitive presentation of a child with identified genetic abnormalities of trisomy 6 and monosomy 21 who was evaluated as part of a standard medical protocol for cochlear implantation following diagnosis of profound sensorineural hearing loss. This child received neurocognitive testing prior to cochlear implantation and approximately 12 months post-activation of his cochlear implant. While he has not fully developed oral language, his presentation suggested improvement in overall skills since the activation of the cochlear implant; however, less than would be expected for a typically developing child.

Intra-operative monitoring of cochlear function during cochlear implantation.

The objective of this study was to determine if intra-operative auditory monitoring is feasible during cochlear implantation and whether this can be used as feedback to the surgeon to improve the preservation of residual hearing. This prospective non-randomised study was set in a paediatric tertiary referral hospital. Thirty eight consecutive paediatric patients undergoing cochlear implantation who had measurable auditory thresholds pre-operatively were divided into two cohorts.

Cognition in children with sensorineural hearing loss: etiologic considerations.

Objectives: A considerable amount of literature has documented the impact of hearing impairment on spoken language skills in deaf children referred for cochlear implantation. Critical areas of neurocognitive development in the acquisition of visual (manual) language also appear to be impacted, although the evidence is less robust. The present study focused on the development of visual and fine motor skills in a sample of preschool-age children diagnosed with sensorineural hearing loss with no known neurologic conditions (n=36).

Dyslipidemia and auditory function.

The relationship between dyslipidemia and hearing is unclear. This study was conducted to investigate whether elevated serum lipid levels impact auditory function in humans and in guinea pigs. In the human study, a cross-sectional study of 40 volunteers with dyslipidemia was conducted.

Endoscopically guided placement of prefabricated cochlear implant electrodes in a common cavity malformation.

Objective: To devise a safe and effective method of optimal customized electrode placement in the common cavity of children with cochleovestibular malformations.

Methods: Specialized electrodes were manufactured on the basis of three-dimensional data obtained from the high resolution computed tomography (HRCT) scans of the temporal bones of these two children. Electrode positioning was achieved with direct endoscopic view of the cavity utilizing a three-hole common cavity technique.

Steady-state response audiometry in a group of patients with steeply sloping sensorineural hearing loss.

Objectives: The purpose of this study was to examine the predictive value of auditory steady-state response (ASSR) evoked potential thresholds and predicted behavioral thresholds in a group of children with steeply sloping sensorineural hearing loss (HL).

Study Design: Case series.

Methods: Twenty-nine children with sloping sensorineural HL underwent behavioral audiometric evaluation, impedance testing, distortion product otoacoustic emissions, and steady-state response testing.

Auditory steady-state response audiometry in profound SNHL: the impact of abnormal middle ear function.

Auditory steady-state response (ASSR) audiometry is a commercially available tool that is used to predict behavioral auditory threshold levels. Its particular value stems from the technology's ability to measure frequency-specific responses in the background electroencephalogram to auditory stimuli presented across a broad range of frequencies and sound pressure levels. It is clearly of benefit when used to assess threshold levels in infants and children with severe-to-profound hearing impairment (i.

Neonatal hearing loss in the indigent.

Objective: To compare the risk factor profile for neonatal hearing loss (HL), and the follow-up rate of those identified with HL in an indigent population with those in an insured population.

Study Design: Retrospective review.

Methods: We studied 4526 neonates from the high-risk nursery or neonatal intensive care unit from two adjacent hospitals in Houston, Texas.