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Conclusions:EABR is a reliable and effective way of objectively confirming device function and implant-responsiveness of the peripheral auditory neurons up to the level of the brainstem in cases of inner ear malformation.Objective:To investigate the usefulness of measuring the intra-operative electrically evoked compound action potential (ECAP) and electrically evoked auditory brainstem response (EABR) in patients with and without congenital inner ear anomalies during cochlear implantation.Method:Thirty-eight consecutive children (40 ears) aged 5 or younger with congenital profound hearing loss. Twenty-four (25 ears) lacked congenital inner ear anomalies. The 14 patients (15 ears) with a malformation had common cavities (four ears), incomplete partition type I (three ears), cochlea hypoplasia type III (three ears), enlarged vestibular aqueduct (four ears), and cochlear nerve canal stenosis (one ear). Main outcome measures are ECAP and EABR responses.Results:Of the 25 ears lacking any malformation, 21, three, and one showed ‘Good’, ‘Variable’, and ‘No’ ECAP responses, respectively, and 24 and one showed ‘Good’ and ‘Variable’ intra-cochlear responses, respectively. Of the 15 ears with a malformation, two showed ‘Good’ ECAP responses, nine had ‘Variable’ ECAP responses, and four showed ‘No’ ECAP responses. Moreover, five showed ‘Good’ EABR responses and 10 showed ‘Variable’ EABR responses. [ABSTRACT FROM PUBLISHER]

Conclusions: Our study revealed that VEMPs in responses to clicks and 500 Hz STB were abnormal in 25% of athletes with congenital profound sensorineural hearing loss for the Deaf Olympic Games. These results confirm that a part of deaf athletes with congenital vestibular failure is well compensated for higher level sport activities. Objectives: The aim of this study was to investigate the appearance rate of VEMP, vestibular failure and its compensation in Japanese deaf athletes by the VEMP test, caloric test and balance test. Methods: Sixteen subjects among the Deaf Olympic Games athletes of Japan volunteered to participate in this study. All the subjects have congenital profound sensorineural hearing loss. Results: In the VEMP test, 24 ears (75%) showed normal click-evoked responses, 5 ears (16%) showed decreased click-evoked responses, 3 ears (9%) showed no click-evoked responses, 28 ears (87.5%) showed normal 500 Hz short tone burst (STB)-evoked responses and 4 ears (12.5%) showed decreased responses. In the ice-water caloric test, 11 subjects (69%) showed normal responses bilaterally, 3 subjects showed bilateral responses with asymmetrical responses and 2 subjects (12%) showed no response bilaterally. All the subjects showed normal balance test results. [ABSTRACT FROM AUTHOR]

Congenitally deaf infants and children commonly suffer vestibular failure in both ears, and impairment of postural control, locomotion, and gait. The development of gross motor functions, such as head control, sitting, and walking is likely to be delayed, but fine motor function is usually preserved unless disorders of the central nervous system are present. These children can eventually catch up with their normal peers in terms of development and growth as a result of central vestibular compensation. The visual and somatosensory systems, pyramidal and extrapyramidal motor system (cerebellum, basal ganglia, cerebrum) and intellectual development, compensate for vestibular failure in infants and children with congenitally hypoactive or absent function of the semicircular canals and otolith organs. [ABSTRACT FROM AUTHOR]

Conclusions. The horizontal portion of the petrous carotid artery (HPCA) of mandibulofacial dysostosis (MFD) patients had anterior-inferior displacement or was aplasia at birth. Our findings indicate that most MFD patients have aplasia of the internal carotid artery. This information may be very important for safe reconstruction surgery in atresia. Objective. MFD rarely has malformations of the cardiovascular system such as those seen in Goldenhar syndrome. This study aimed to compare MFD patients and normal subjects with respect to the length and diameter of the HPCA. Subjects and methods. We radiographically studied 22 MFD patients (44 sides) using high-resolution computed tomography (HRCT), measuring the length and diameter of the HPCA and the angle between the right and left HPCA using computer software. Findings were compared with those in 86 ears with normal auricles (control group) using the nonparametric test. Results. The HPCA in MFD patients averaged 23.2 mm in length and 4.8 mm in diameter. The angle between the right and left HPCA averaged 100.2°. In MFD patients, on average, HPCA were 2.0 mm shorter (p=0.001) and 0.4 mm narrower (p=0.016) than in control subjects. Mean angle between right and left HPCA was 5.4° more acute in MFD patients than in control subjects (p=0.026). Moreover, the genu of the vertical and horizontal petrous ICA in the MFD group was on average 3.2 mm more inferior than in controls (p<0.001). [ABSTRACT FROM AUTHOR]

To estimate vestibulo-collic reflexes in patients with spinocerebellar degeneration (SCD), vestibular evoked myogenic potentials (VEMPs) were recorded in 16 patients with SCD and in 9 normal subjects. The patients with SCD were classified into three groups: those with olivo-ponto-cerebellar ataxia (OPCA; n=10); those with cortical cerebellar atrophy (CCA; n=3); and those with Machado-Joseph disease (MJD; n=3). While all of the patients with OPCA and CCA showed biphasic responses (p13-n23), 2 of the 3 patients with MJD showed abnormal VEMPs bilaterally. Three sides in these 2 patients showed an absence of VEMPs, and one side showed a remarkably delayed p13. These two patients showed little response in caloric tests. These results suggest that the vestibulo-collic reflex as well as the vestibulo-ocular reflex could be damaged in MJD patients but preserved in OPCA and CCA patients. [ABSTRACT FROM AUTHOR]

We report results of vestibular-evoked myogenic potentials (VEMPs) in patients with cerebellopontine angle tumors and compare results obtained using clicks with those obtained using 500 Hz short tone bursts (STB). We reviewed the records of 87 patients with cerebellopontine angle tumors. Clicks (0.1 ms, 95 dB nHL) were presented to all patients and STB (500 Hz, rise/fall time 1 ms, plateau time 2 ms, 95 dB nHL) were presented to 27 patients. Click-evoked VEMPs were abnormal in 69/87 patients (79%; no response in 55 patients, decreased response in 14 patients, normal response in 18 patients). STB-evoked VEMPs were abnormal in 22/27 patients (82%; no response in 18 patients, decreased response in 4 patients, normal response in 5 patients). Click- and STB-evoked VEMPs were identical in 23/27 patients (85%). Two patients showed normal STB-evoked VEMPs and decreased click-evoked VEMPs, and 2 patients showed decreased STB-evoked VEMPs and absent click-evoked VEMPs. These results confirm our previous study in a small number of patients. Vestibular afferents seem to respond better to 500 Hz STBs than to clicks. [ABSTRACT FROM AUTHOR]