Detection Rates of Cortical Auditory Evoked Potentials at Different Sensation Levels in Infants with Sensory/Neural Hearing Loss and Auditory Neuropathy Spectrum Disorder.

With the introduction of newborn hearing screening, infants are being diagnosed with hearing loss during the first few months of life. For infants with a sensory/neural hearing loss (SNHL), the audiogram can be estimated objectively using auditory brainstem response (ABR) testing and hearing aids prescribed accordingly. However, for infants with auditory neuropathy spectrum disorder (ANSD) due to the abnormal/absent ABR waveforms, alternative measures of auditory function are needed to assess the need for amplification and evaluate whether aided benefit has been achieved.

Auditory neuropathy in Brown-Vialetto-Van Laere syndrome due to riboflavin transporter RFVT2 deficiency.

Aim: Mutations in the genes encoding the riboflavin transporters RFVT2 and RFVT3 have been identified in Brown-Vialetto-Van Laere syndrome, a neurodegenerative disorder characterized by hearing loss and pontobulbar palsy. Treatment with riboflavin has been shown to benefit individuals with the phenotype of RFVT2 deficiency. Understanding the characteristics of hearing loss in riboflavin transporter deficiency would enable early diagnosis and therapy.

The audiological journey and early outcomes of twelve infants with auditory neuropathy spectrum disorder from birth to two years of age.

Objective: To describe the audiological journey of a group of infants with auditory neuropathy spectrum disorder (ANSD) following the fitting of hearing aids, and to investigate the potential benefits of including cortical auditory-evoked potentials (CAEPs) and a measure of functional auditory behaviour during early audiological management.

Design: Results from chart revision of estimated hearing threshold, early behavioural testing, parental observation, and functional auditory behaviour assessments were described, and compared to visual reinforcement audiometry (VRA) thresholds obtained at a mean corrected age of 10 months (SD 3). The relationship with CAEPs and functional performance was examined.

Impact of the presence of auditory neuropathy spectrum disorder (ANSD) on outcomes of children at three years of age.

Objective: To determine the influence of the presence of auditory neuropathy spectrum disorder (ANSD) on speech, language, and psycho-social development of children at three years of age.

Design: A population-based, longitudinal study was performed on outcomes of children with hearing impairment (LOCHI) in Australia. The demographic characteristics of the children were described, and their developmental outcomes were evaluated at three years of age.

Cochlear implantations in children with Waardenburg syndrome: an electrophysiological and psychophysical review.

Waardenburg syndrome presents with dystopia canthorum, pigmentary abnormalities of hair, iris and skin (often a white forelock and heterochromia iridis) and sensorineural deafness. The authors review the electrophysiological and psychophysical findings of implanted children with Waardenburg syndrome at the Sydney Cochlear Implant Centre. Twenty children with Waardenburg syndrome received cochlear implants between 1985 and 2001.

Evaluation of NRT and behavioral measures for MAPping elderly cochlear implant users.

We investigated the acceptability of electrophysiologically derived MAPs and the effect of these MAPs on speech perception in elderly adults using Nucleus 24 cochlear implants. Eight implant recipients aged 75 years or older trialed an electrophysiologically derived MAP and a behavioral MAP. The electrophysiologically derived MAP was based on the threshold and maximum comfort level for electrode 10 and evoked compound action potential thresholds measured on six electrodes using neural response telemetry (NRT).