Evaluation of the Vestibular System and Etiology in Children with Unilateral Sensorineural Hearing Loss

Abstract

OBJECTIVE:The aim of this study was to evaluate the vestibular system of children with unilateral sensorineural hearing loss (USNHL), investigate the etiological factors of USNHL and analyze whether a genetic predisposition exists. MATERIALS and METHODS:Thirty-three children aged less than 18 years with USNHL, who visited the ear, nose, and throat (ENT) department between January 2004 and December 2012, were included in this study. Cases with conductive hearing loss were excluded from the study. The patients were subjected to etiologic, genetic, and ophthalmologic evaluation; radiologic imaging; electronystagmography (ENG); and vestibular evoked myogenic potential (VEMP) tests. The control group, which included 25 healthy children (13 males and 12 females), had undergone audiological assessment and were subjected to ENG and VEMP tests. RESULTS:All of the patients had severe-to-profound hearing loss. Mumps immunoglobulin G was positive in 22 (66.7%) of 33 patients. The 35delG mutation was not found in any of the patients. All of the patients underwent temporal computed tomography (CT) and magnetic resonance imaging (MRI). Inner ear anomaly was present in 51.5% of the patients. Overall, 21 of 31 ENG patients had canal paresis in the affected ear. The VEMP response was absent on the affected side in three patients. The n23 latency average of the patient group was longer than that of the control group. CONCLUSION:Because USNHL causes irreversible problems in children, early diagnosis and auditory rehabilitation are very important. As USNHL is accompanied by inner ear anomaly, children with USNHL should undergo temporal bone CT and MRI. To evaluate the vestibular system, ENG and VEMP are non-invasive and diagnostic tests.

Vestibular Infant Screening (VIS)–Flanders: results after 1.5 years of vestibular screening in hearing-impaired children

“…Regarding the relation with the laterality of hearing loss, the current study could not reveal a significantly different cVEMP refer rate in the group with a unilateral and bilateral sensorineural hearing loss, thereby highlighting the necessity of vestibular assessment even in infants with only unilateral hearing loss. These research findings are consistent with previous studies that also detected vestibular dysfunction in children with unilateral sensorineural hearing loss 58,59 . Concerning the relation with the degree of hearing loss, a significantly higher cVEMP refer rate occurred in infants with severe-profound compared to mild-moderate sensorineural hearing loss in the current study.…”

A Systematic Review on the Association Between Vestibular Dysfunction and Balance Performance in Children With Hearing Loss

“…However, they did not find that balance performance was significantly different between children with unilateral and bilateral vestibular dysfunction. In contrast, Birdane et al (2016) found that children with unilateral vestibular dysfunction had normal results on balance tests (n = 33). This suggests that children are able to effectively use vestibular compensation such that the peripheral vestibular input from one ear may be sufficient for normal balance ability.…”

“…While the majority of studies report some association between vestibular testing results and balance function, two studies (Potter & Silverman 1984;Birdane et al 2016) came to the conclusion that there was no such association. In both instances, either the measure used to quantify vestibular function (in the case of Potter & Silverman 1984) or the balance outcome measure (in Birdane et al 2016) are generally not well accepted measures due to their test performance. Potter and Silverman (1984), which was among the first published in this area, used the Southern California Postrotary Nystagmus Test as a measure of vestibular function.…”

“…Two studies reported no association between vestibular function testing results and balance results (Potter & Silverman 1984; Birdane et al 2016). For example, Birdane et al (2016) only considered children with unilateral hearing loss. Among those children with unilateral hearing loss, all their participants had either normal vestibular function or unilateral vestibular dysfunction.…”

Inner-ear malformations as a cause of single-sided deafness

“…investigated the aetiological factors of unilateral SNHL, together with vestibular system evaluation findings, in 33 patients. 24 They found that 51.5 per cent of their cases had inner-ear malformations, predominantly lateral semicircular canal aplasia ( n = 11, 33.3 per cent). They focused on vestibular structures, and did not investigate the status of the cochlear nerve and other inner-ear malformations.…”