PURPOSE:To study the effects that the abnormal eye movements of patients with Duane retraction syndrome have on the development of binocular function.METHODS:Pattern reversal visual evoked responses (VEPs) to 15-minutes-of-arc and 60-minutes-of-arc checks and binocular beat VEPs to diffuse sinusoidally modulated 18- and 20-Hz stimuli were recorded in 10 patients with Duane retraction syndrome who maintain binocular function by using an abnormal head posture. Visual acuity, stereoacuity, and eye movements were measured. The results have been compared to those from 10 normal subjects.RESULTS:The patients with Duane retraction syndrome had reduced stereoacuity compared to the normal control group (TNO mean, 82.5 seconds of arc compared to 37.5 seconds of arc; Titmus mean, 143 seconds of arc compared to 44 seconds of arc). The binocular beat VEPs showed a significantly reduced difference beat response at 2 Hz in the patients with Duane syndrome compared to normal subjects (mean signal-to-noise ratio 2.40 +/- 1.05 compared to 4.30 +/- 2.66; t = 2.21, df = 18, P < 0.05). Binocular enhancement of the P100 pattern reversal amplitude to 15-minute checks was increased in these patients, because of a reduction of the monocular P100 amplitudes compared to the normal group.CONCLUSIONS:Patients with Duane syndrome who maintain binocular function using an abnormal head posture have reduced stereoacuity and show electrophysiological evidence of reduced cortical binocular interaction.

OBJECTIVE:Evaluation of a short latency component (SLC) observed in profoundly deaf young children during recordings of the auditory brain stem response (ABR) before cochlear implantation. DESIGN:Seventy young children (ages 2 to 11 yr) were investigated as part of their routine audiologic and diagnostic assessment, before cochlear implantation. The ABR was evoked using click stimuli and tone pips (500 Hz and 1 kHz) at intensity levels up to 105 dB nHL. The incidence of the SLC arising at a latency of 3 msec with high level click stimuli, in addition to residual ABR waves, was assessed. RESULTS:An SLC was observed in a total of 18 of the 70 children investigated. It was present in 15 out of 31 congenitally deaf children (48%) compared with only 2 out of 33 children deafened after meningitis (6%). A chi-squared test employing a 2 x 2 contingency table shows that this dependency on etiology of deafness is highly statistically significant (p < 0.001). The presence of the SLC in the congenitally deaf children is related to age at the time of the Electric Response Audiometry test and is more likely to be observed in the younger child (p < 0.01). CONCLUSIONS:Interpretation of the ABR in profoundly deaf children should take into consideration the possible presence of the SLC. An evoked potential arising from stimulation of the vestibular system, particularly the vestibular nuclei, is proposed as a likely origin for this component. Damage to sensory cells in the vestibular portion of the labyrinth might explain why an SLC is seen rarely in cases of deafness after meningitis.