Vestibular function in children with auditory neuropathy spectrum disorder

Nash, Robert; Veness, John; Wyatt, Michelle; Raglan, Ewa; Rajput, Kaukab

doi:10.1016/j.ijporl.2014.05.008

Cited by 10 publications

(5 citation statements)

References 15 publications

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“…Considering otoacoustic emissions were not performed in these BVH patients, auditory neuropathy could have been missed during the determination of the etiology. Nevertheless, speech audiometry was performed on these patients, and as poor speech recognition is also a criterion of auditory neuropathy disorder, this obstacle could partially be overcome by these tests ( 32 , 63 – 65 ). Notably, in approximately half of the patients, no to moderate hearing loss was found by using pure tone and speech audiometry.…”

Section: Discussionmentioning

confidence: 99%

Bilateral Vestibular Hypofunction: Insights in Etiologies, Clinical Subtypes, and Diagnostics

et al. 2016

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ObjectiveTo evaluate the different etiologies and clinical subtypes of bilateral vestibular hypofunction (BVH) and the value of diagnostic tools in the diagnostic process of BVH.Materials and methodsA retrospective case review was performed on 154 patients diagnosed with BVH in a tertiary referral center, between 2013 and 2015. Inclusion criteria comprised (1) imbalance and/or oscillopsia during locomotion and (2) summated slow phase velocity of nystagmus of less than 20°/s during bithermal caloric tests.ResultsThe definite etiology of BVH was determined in 47% of the cases and the probable etiology in 22%. In 31%, the etiology of BVH remained idiopathic. BVH resulted from more than 20 different etiologies. In the idiopathic group, the percentage of migraine was significantly higher compared to the non-idiopathic group (50 versus 11%, p < 0.001). Among all patients, 23.4% were known with autoimmune disorders in their medical history. All four clinical subtypes (recurrent vertigo with BVH, rapidly progressive BVH, slowly progressive BVH, and slowly progressive BVH with ataxia) were found in this population. Slowly progressive BVH with ataxia comprised only 4.5% of the cases. The head impulse test was abnormal in 94% of the cases. The torsion swing test was abnormal in 66%. Bilateral normal hearing to moderate hearing loss was found in 49%. Blood tests did not often contribute to the determination of the etiology of the disease. Abnormal cerebral imaging was found in 21 patients.ConclusionBVH is a heterogeneous condition with various etiologies and clinical characteristics. Migraine seems to play a significant role in idiopathic BVH and autoimmunity could be a modulating factor in the development of BVH. The distribution of etiologies of BVH probably depends on the clinical setting. In the diagnostic process of BVH, the routine use of some blood tests can be reconsidered and a low-threshold use of audiometry and cerebral imaging is advised. The torsion swing test is not the “gold standard” for diagnosing BVH due to its lack of sensitivity. Future diagnostic criteria of BVH should consist of standardized vestibular tests combined with a history that is congruent with the vestibular findings.

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Section: Discussionmentioning

confidence: 99%

Bilateral Vestibular Hypofunction: Insights in Etiologies, Clinical Subtypes, and Diagnostics

et al. 2016

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“…Vestibular dysfunction has been described in the wider auditory neuropathy spectrum disorder literature using a variety of methods. One study of 35 children with auditory neuropathy spectrum disorder reported the presence of vestibulopathy in 42 per cent of the cohort using rotational chair testing and clinical examination, 23 whereas other studies utilising caloric and clinical examination described prevalence of between 50 and 100 per cent. 24,25 A recent study using vestibular evoked myogenic potential, caloric testing, video head impulse testing and suppression head impulse paradigm testing in a group of 22 children with auditory neuropathy spectrum disorder reported 91 per cent with abnormal cervical vestibular evoked myogenic potential, 86 per cent with abnormal ocular vestibular evoked myogenic potential, 70 per cent with abnormal rate on caloric testing, and most revealing, no overt corrective saccades on video head impulse testing and normal anti-compensatory saccades on suppression head impulse paradigm testing.…”

Section: Discussionmentioning

confidence: 99%

The audiovestibular profile of Brown-Vialetto-Van Laere syndrome

et al. 2021

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Background Brown-Vialetto-Van Laere syndrome, a rare disorder associated with motor, sensory and cranial nerve neuropathy, is caused by mutations in riboflavin transporter genes SLC52A2 and SLC52A3. Hearing loss is a characteristic feature of Brown-Vialetto-Van Laere syndrome and has been shown in recent studies to be characterised by auditory neuropathy spectrum disorder. Method This study reports the detailed audiovestibular profiles of four cases of Brown-Vialetto-Van Laere syndrome with SLC52A2 and SLC52A3 mutations. All of these patients had auditory neuropathy spectrum disorder. Results There was significant heterogeneity in vestibular function and in the benefit gained from cochlear implantation. The audiological response to riboflavin therapy was also variable, in contrast to generalised improvement in motor function. Conclusion We suggest that comprehensive testing of vestibular function should be conducted in Brown-Vialetto-Van Laere syndrome, in addition to serial behavioural audiometry as part of the systematic examination of the effects of riboflavin.

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“…Some patients present as a fluctuating hearing loss, appearing to have “good” and “bad” hearing days, because sounds may fade in and out or appear “out of sync” (National Institute of Deafness and Other Communication Disorders, 2018). Other symptoms may include varying degrees of motor incoordination, which family/caregivers may report as “clumsiness.” About 40% of children with ANSD have vestibulopathy, which typically is not associated with abnormal findings on imaging (Nash et al, 2014).…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Children who do not demonstrate good progress for speech and language development with appropriate amplification should be considered CI candidates regardless of audiometric thresholds, because in ANSD the question is not how severe is the hearing loss but how severe is the dys-synchrony! About 10% of CI patients have ANSD (Nash et al, 2014). CIs are successful if the underlying pathophysiology is a biochemical abnormality of neurotransmitters or synaptic defects between IHC and the auditory nerve.…”

Section: Managementmentioning

confidence: 99%

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Auditory Neuropathy Spectrum Disorder: When the Audiogram Does Not Tell the Full Story

2020

Word of Mouth

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Vestibular function in children with auditory neuropathy spectrum disorder

Cited by 10 publications

References 15 publications

Bilateral Vestibular Hypofunction: Insights in Etiologies, Clinical Subtypes, and Diagnostics

Bilateral Vestibular Hypofunction: Insights in Etiologies, Clinical Subtypes, and Diagnostics

The audiovestibular profile of Brown-Vialetto-Van Laere syndrome

Auditory Neuropathy Spectrum Disorder: When the Audiogram Does Not Tell the Full Story

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