No abstract

In this study, the overall prevalence of ophthalmologic findings in children with SNHL was 21.7%. Ophthalmologic anomalies were present in 3.7% of children with GJB2 mutations. Routine ophthalmologic examination can be beneficial in the evaluation of children with SNHL.

Psychometric evaluations were performed on a self-perceived localization disabilities and handicaps questionnaire. Twenty individuals with normal hearing bilaterally, twenty with profound unilateral hearing impairment (UHI), and ten with any degree of bilateral hearing impairment participated. Each subject completed the questionnaire. Comparisons of the responses of the subjects with normal hearing and those with UHI revealed significant differences among the groups for both disabilities and handicaps, establishing construct validity. Cronbach's Alpha correlational analyses of the responses of all subjects with hearing impairment revealed correlations of .900 (disabilities) and .800 (handicaps), establishing internal consistency. Each participant with hearing impairment was asked to complete the questionnaire again after three weeks. Pearson's correlational analyses of the responses at time one versus time two revealed correlations of .900 (disabilities) and .700 (handicaps), establishing test/retest reliability. This questionnaire is an appropriate tool for investigating the self-perceived localization disabilities and handicaps of individuals with hearing impairment.

Chiari I malformation may be associated with SNHI. Central nervous system imaging for the evaluation of children with SNHI may be indicated, particularly in individuals with negative results on temporal bone computed tomography and genetic testing.

The chief complaint of individuals with hearing impairment is difficulty hearing in noise, with directional microphones emerging as the most capable remediation. Our purpose was to determine the impact of directional microphones on localization disability and concurrent handicap. Fifty-seven individuals participated unaided and then in groups of 19, using omni-directional microphones, directional-microphones, or toggle-switch equipped amplification. The outcome measure was a localization disabilities and handicaps questionnaire. Comparisons between the unaided group versus the aided groups, and the directional-microphone groups versus the other two aided groups revealed no significant differences. None of the microphone schemes either increased or decreased self-perceived localization disability or handicap. Objective measures of localization ability are warranted and if significance is noted, clinicians should caution patients when moving in their environment. If no significant objective differences exist, in light of the subjective findings in this investigation concern over decreases in quality of life and safety with directional microphones need not be considered.