Millions of people across the world suffer from disabling hearing loss. Appropriate interventions lead to improved speech and language skills, educational advancement, and improved social integration. A major limitation to improving care is identifying those with disabling hearing loss in low-resource countries.

This review article summarises information on currently available hearing screening platforms and technology available from published reports and the authors’ personal experiences of hearing loss identification in low-resource areas of the world. The paper reviews the scope and capabilities of portable hearing screening platforms, including the pros and cons of each technology and how they have been utilised in low-resource environments.

Portable hearing screening tools are readily available to assess hearing loss in low-resource areas. Each technology has advantages and limitations that should be considered when identifying the optimal methods to assess needs in each country.

The purpose of this study was to evaluate the effect of lengthening of voice onset time and burst duration of selected speech stimuli on perception by individuals with auditory dys-synchrony. This is the second of a series of articles reporting the effect of signal enhancing strategies on speech perception by such individuals.

Two experiments were conducted: (1) assessment of the ‘just-noticeable difference' for voice onset time and burst duration of speech sounds; and (2) assessment of speech identification scores when speech sounds were modified by lengthening the voice onset time and the burst duration in units of one just-noticeable difference, both in isolation and in combination with each other plus transition duration modification.

Lengthening of voice onset time as well as burst duration improved perception of voicing. However, the effect of voice onset time modification was greater than that of burst duration modification. Although combined lengthening of voice onset time, burst duration and transition duration resulted in improved speech perception, the improvement was less than that due to lengthening of transition duration alone.

These results suggest that innovative speech processing strategies that enhance temporal cues may benefit individuals with auditory dys-synchrony.

Description of a female patient with diagnosed Kjer's disease and sensorineural hearing loss, who specifically complained of a progressive inability to understand speech in noisy situations.

Case report.

A 30-year-old, Caucasian woman with Kjer's disease.

Audiological assessment showed low-frequency sensorineural hearing loss and a disproportionate deterioration in speech discrimination. This inconsistency gave rise to suspicion of possible aggravation. Follow-up testing showed that brainstem responses were absent, while clear otoacoustic emissions and cochlear microphonics were present. Hearing aids were fitted but no improvement was shown.

This case shows a combination of auditory neuropathy and Kjer's optic neuropathy. It also illustrates that the combination of unexplained hearing loss and apparently inconsistent audiometric outcomes may be associated with auditory neuropathy. Such unexpected hearing evaluation outcomes may be due to other neurological conditions, such as Kjer's disease.

Auditory neuropathy is a disorder characterised by preservation of outer hair cell function, with normal otoacoustic emissions and/or cochlear microphonics, but an absent or distorted auditory brainstem response.

This study aimed to objectively assess hearing thresholds in patients with auditory neuropathy, using the auditory steady state response.

Thirteen patients with auditory neuropathy and 15 normal hearing subjects were examined. Audiological evaluation included basic audiological tests, otoacoustic emissions, auditory brainstem response and auditory steady state response.

In the auditory neuropathy patients, the auditory brainstem response was absent in 11 patients, while the auditory steady state response was absent in only three.

The auditory steady state response may serve as a valuable objective measure for assessing the hearing threshold across different frequencies in patients with auditory neuropathy. We recommend that auditory steady state response be used to complete the evaluation of patients with auditory neuropathy.