Imaging findings in pediatric single‐sided deafness and asymmetric hearing loss

Lipschitz, Noga; Kohlberg, Gavriel D.; Scott, Marcia S.; Greinwald, John H.

doi:10.1002/lary.28095

Cited by 13 publications

(11 citation statements)

References 18 publications

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“…In both studies, cochlear nerve deficiency was detected in 50% of the cases, and even 100% of the infants [43], showing that cochlear nerve deficiency is a common cause of UHL, especially in severe to profound congenital UHL. In addition to cochlear nerve deficiency, cochlear dysplasia and an enlarged vestibular aqueduct were detected as structural malformations which could explain the UHL [42]. Investigations of structural malformations are clinically relevant for the evaluation of the etiology of the hearing loss.…”

Section: Discussionmentioning

confidence: 99%

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Research Insights on Neural Effects of Auditory Deprivation and Restoration in Unilateral Hearing Loss: A Systematic Review

Vanderauwera

Hellemans

Verhaert

2020

JCM

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Neuroplasticity following bilateral deafness and auditory restoration has been repeatedly investigated. In clinical practice, however, a significant number of patients present a severe-to-profound unilateral hearing loss (UHL). To date, less is known about the neuroplasticity following monaural hearing deprivation and auditory input restoration. This article provides an overview of the current research insights on the impact of UHL on the brain and the effect of auditory input restoration with a cochlear implant (CI). An exhaustive systematic review of the literature was performed selecting 38 studies that apply different neural analyses techniques. The main results show that the hearing ear becomes functionally dominant after monaural deprivation, reshaping the lateralization of the neural network for auditory processing, a process that can be considered to influence auditory restoration. Furthermore, animal models predict that the onset time of UHL impacts auditory restoration. Hence, the results seem to advocate for early restoration of UHL, although further research is required to disambiguate the effects of duration and onset of UHL on auditory restoration and on structural neuroplasticity following UHL deprivation and restoration. Ongoing developments on CI devices compatible with Magnetic Resonance Imaging (MRI) examinations will provide a unique opportunity to investigate structural and functional neuroplasticity following CI restoration more directly.The review by Van Wieringen et al. on the etiology of congenital UHL reported that congenital causes mainly consist of cytomegalovirus infection, congenital nerve deficiency, syndromal causes, bacterial meningitis, inner ear malformation, or unknown etiology. In older children and adults, predominant acquired causes of UHL include vestibular schwannoma (34 per million per year), trauma (due to temporal bone fracture), Ramsay Hunt syndrome (herpes zoster virus), auto-immune hearing loss, Menière's disease and idiopathic sudden sensorineural hearing loss (incidence of 27 per 100,000), chronic otitis media, and failed middle ear surgery, rendering the prevalence much higher [1,2]. UHL has been shown to decrease sound localization, a central binaural hearing process [3]. Moreover, UHL during early childhood negatively impacts the development of oral language (for a review on language impact, see Van Wieringen et al. [1]). Compared to bilateral deafness, dedicated research on UHL is more recent and less is known about the neural changes following UHL. Hence, the primary objective of the present study is to summarize and evaluate current research insights on structural and functional brain plasticity following unilateral hearing deprivation caused by UHL. In the present systematic review, neural plasticity is studied both in auditory and non-auditory cortices. We thereby especially emphasize factors that can potentially influence the observed neural plasticity, such as the age of onset of the UHL, the duration of the UHL, and the deafness side. For the age of onse...

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Section: Discussionmentioning

confidence: 99%

“…Anatomical T1/T2 neuroimaging A study by Lipschitz et al [42] examined structural neuroimaging scans (CT and MRI) of 170 pediatric patients (age 0-18 years) with UHL. They reported that in nearly half of the sample, structural malformations were detected that could be associated with the hearing loss.…”

Section: Structural Plasticitymentioning

confidence: 99%

Research Insights on Neural Effects of Auditory Deprivation and Restoration in Unilateral Hearing Loss: A Systematic Review

Vanderauwera

Hellemans

Verhaert

2020

JCM

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“…Etiological evaluation is a crucial step toward better management with an effective counseling process for patients and parents. Magnetic resonance imaging is an important diagnostic tool that also helps in informed therapy planning [14]. Unfortunately, up until now, there is no supporting evidence for the efficacy of MVD of the vestibulocochlear nerve for SNHL.…”

Section: Discussionmentioning

confidence: 99%

Single-sided deafness related to neural vascular conflict: A case report from Saudi Arabia

Mona¹

2020

Int J Case Rep Images

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The impact of single-sided deafness (SSD) is well known, particularly on children. These include difficulties in speech comprehension with background noise, localization, and spatial appreciation. The social and emotional status of the child may be compromised. In addition, parents are keen to understand the etiology and consequences of SSD. Different etiologies are correlated with SSD, and etiology identification is a crucial step toward better management and parent counseling. SSD related to neural vascular conflict (NVC) of the cerebellopontine angle is uncommon. This is the first case, up to my knowledge so far, to be reported in Saudi Arabia. A 13-year-old Saudi girl presented with a history of difficulty in hearing in the left ear, and audiological evaluation demonstrated left-sided profound sensorineural hearing loss. Upon further investigation, magnetic resonance imaging (MRI) showed a left-sided neural vascular conflict of the anterior inferior cerebellar artery (AICA) loop and the left cochlear nerve. In this case, SSD was related to NVC since clinical history, and other investigations did not justify another cause. This report will embrace the role of audiological and radiological investigation, which in turn will help with management recommendations, and help clinicians in dealing with similar challenges.

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“…While several past studies have evaluated CND in pediatric patients with UHL, the literature has reported widely variable prevalence of CND ranging from 9 to 50% [Clemmens et al, 2013;Nakano et al, 2013;Paul et al, 2017;Usami et al, 2017;van Beeck Calkoen et al, 2017;Lipschitz et al, 2020;Pollaers et al, 2020;Tahir et al, 2020]. Evaluation of CND in pediatric patients with AHL has been limited in the literature, with studies reporting a range of 0-28% prevalence of CND [Usami et al, 2017;Lipschitz et al, 2020]. Of note, studies that have compared CND prevalence in both UHL and bilateral pediatric HL have demonstrated significantly higher rates of CND in UHL (39-50%) as compared to bilateral HL (5%) [Nakano et al, 2013;Pollaers et al, 2020].…”

Section: Introductionmentioning

confidence: 99%

Cochlear Nerve Deficiency in Pediatric Unilateral Hearing Loss and Asymmetric Hearing Loss

et al. 2022

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Introduction: The rates of cochlear nerve abnormalities and cochlear malformations in pediatric unilateral hearing loss (UHL) are conflicting in the literature, with important implications on management. The aim of this study was to investigate the incidence of cochlear nerve deficiency (CND) in pediatric subjects with UHL or asymmetric hearing loss (AHL). Methods: A retrospective chart review of pediatric subjects <18 years of age evaluated for UHL or AHL with fine-cut heavily T2-weighted magnetic resonance imaging (MRI) between January 2014 and October 2019 (n = 291) at a tertiary referral center was conducted. MRI brain and computed tomography temporal bone were reviewed for the presence of inner ear malformations and/or CND. Status of the ipsilateral cochlear nerve and inner ear was evaluated. Pure tone average (PTA) at 500, 1,000 and 2,000 Hz was assessed. Results: 204 subjects with UHL and 87 subjects with AHL were included. CND (aplasia or hypoplasia) was demonstrated in 61 pediatric subjects with UHL (29.9%) and 10 with AHL (11.5%). Ipsilateral cochlear malformations were noted in 25 subjects with UHL (12.3%) and 11 with AHL (12.6%), and ipsilateral vestibular malformations in 23 (11.3%) and 12 (13.8%) ears, respectively. Median PTA was statistically significantly higher in ears with CND (98.33) than ears with normal nerves (90.84). Discussion/Conclusion: Imaging demonstrated a high incidence of inner ear malformations, particularly CND, in pediatric subjects with UHL. Auditory findings indicated CND cannot be ruled out by thresholds alone as some CND ears did demonstrate measurable hearing. Radiologic evaluation by MRI should be performed in all patients within this population to guide counseling and management of hearing loss based on etiology, with implications on candidacy for cochlear implantation.

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Imaging findings in pediatric single‐sided deafness and asymmetric hearing loss

Cited by 13 publications

References 18 publications

Research Insights on Neural Effects of Auditory Deprivation and Restoration in Unilateral Hearing Loss: A Systematic Review

Research Insights on Neural Effects of Auditory Deprivation and Restoration in Unilateral Hearing Loss: A Systematic Review

Single-sided deafness related to neural vascular conflict: A case report from Saudi Arabia

Cochlear Nerve Deficiency in Pediatric Unilateral Hearing Loss and Asymmetric Hearing Loss

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