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The suppression head impulse paradigm (SHIMP) involves suppression of the vestibulo-ocular reflex (VOR) and anticompensatory saccades generated thereof. SHIMP is gaining importance to understand vestibular compensation with its different parameters (VOR gain/peak saccadic velocity PSV/latency of saccades). SHIMP studies are emerging in adults, but pediatric studies have hardly been performed. This study is a retrospective case note audit over a period of 2 months in a tertiary pediatric vestibular center in the United Kingdom to investigate whether SHIMP is safe/robust to be used in children conforming to existing standards/norms in normal children and whether it yields any meaningful inferences in pediatric vestibular hypofunction. This is the largest pediatric SHIMP study to date. A total of 44 referred children (6-18 years, female children>male children) with a range of complaints from dizziness, imbalance, motor incoordination, postural instability, and hearing loss were included, and their SHIMP parameters were measured. All children underwent comprehensive functional/objective audiovestibular assessments. Two groups were defined--Group A with normal vestibular function and Group B with abnormal vestibular function. The normal population showed an average SHIMP VOR gain of 0.98+/-0.08 and latency of overt saccades at 215.68+/-46.16 milliseconds agreeing with published evidence. The PSV of overt saccades was 315.39+/-56.3°/s, and there was a gain asymmetry of 7.42+/-4.68 between the sides. Statistically significant differences with moderate/large effect sizes were observed between the groups in terms of VOR gain and PSV but not in saccade latencies. Covert saccades were rare in SHIMP, while overt saccades were observed in 100% of children. VOR gain difference between the head impulse paradigm (HIMP) and the SHIMP was significant as well. We observed statistically significant differences in side asymmetry of VOR gain between the groups. Furthermore, we identified a group of children with cerebellar lesions where overt saccades in SHIMP were rather low in number. Further research is recommended to investigate pediatric PSV, asymmetry, and inability to generate overt saccades that may suggest useful means to assess compensation and central function. We conclude that SHIMP yields valuable information and is a safe, easy to perform, and a reliable test that should be used in children to supplement HIMP. [ABSTRACT FROM AUTHOR]

Vestibular schwannomas are benign nerve sheath tumours that arise on the vestibulocochlear nerves. Vestibular schwannomas are known to occur in the context of tumour predisposition syndromes NF2 -related and LZTR1 -related schwannomatosis. However, the majority of vestibular schwannomas present sporadically without identification of germline pathogenic variants. To identify novel genetic associations with risk of vestibular schwannoma development, we conducted a genome-wide association study in a cohort of 911 sporadic vestibular schwannoma cases collated from the neurofibromatosis type 2 genetic testing service in the north-west of England, UK and 5500 control samples from the UK Biobank resource. One risk locus reached genome-wide significance in our association analysis (9p21.3, rs1556516, P = 1.47 × 10−13, odds ratio = 0.67, allele frequency = 0.52). 9p21.3 is a genome-wide association study association hotspot, and a number of genes are localized to this region, notably CDKN2B-AS1 and CDKN2A/B , also referred to as the INK4 locus. Dysregulation of gene products within the INK4 locus have been associated with multiple pathologies and the genes in this region have been observed to directly impact the expression of one another. Recurrent associations of the INK4 locus with components of well-described oncogenic pathways provides compelling evidence that the 9p21.3 region is truly associated with risk of vestibular schwannoma tumorigenesis. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To explore the usefulness of the responses of video head impulse testing (vHIT) in assessing symptomatic patients with superior semicircular canal dehiscence (SSCD). METHODS: This was a prospective case-control study performed in a tertiary skull base referral Centre in the UK. It included all patients diagnosed with SSCD from January 2015 to January 2019 and compared to a control group of age and gender-matched unaffected individuals. The main outcome of the study was semicircular canal vestibule-ocular reflex (VOR) gains during vHIT assessment and link to patients' symptoms. RESULTS: A total of 28 patients were diagnosed with SSCD during the study period and completed the vHIT assessment. Reduced VOR gains (<0.8) were noted in 57% of patients (n = 16). Half of these (n = 8) were in canals other than the superior semicircular (posterior only: n = 5; lateral and posterior: n = 3). Three patients (10.7%) had abnormal responses in 2 canals. The canals in the contralateral side were affected in 56.5% of the cases. There was no correlation with the patients' symptoms. Results were directly comparable with the control group vHIT results with no identifiable statistically significant differences on comparison of the ipsilateral SSCD side with a randomly selected side from the control group (all comparisons: P > .05). CONCLUSION: SSCD can affect the vestibular responses from all 3 semicircular canals; not necessarily the superior one. Similar responses were found in a control group of normal subjects. Although the use of vHIT in the assessment of SSCD is not diagnosis-specific, it can still help with identifying the impact of surgery on all canals prior to any intervention in order to avoid bilateral vestibular failure. [ABSTRACT FROM AUTHOR]