A 15-year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: II. Management and Outcomes

Morlet, Thierry; O’Reilly, Robert; Pritchett, Cedric; Venskytis, Emily; Parkes, William

doi:10.1097/aud.0000000000001366

Cited by 3 publications

(1 citation statement)

References 54 publications

(82 reference statements)

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“…The management of AN-related hearing deficits is challenging as neural distortion, rather than the ability to detect low-level sounds, is the primary limiting factor. As a consequence, conventional hearing aids, which make sounds louder but not necessarily clearer, are usually ineffective [8,11]. One intervention strategy that has had (limited) success in individuals with AN is the use of remote-microphone listening systems.…”

Section: Introductionmentioning

confidence: 99%

Remediation of Perceptual Deficits in Progressive Auditory Neuropathy: A Case Study

Rance,

Tomlin,

Yiu

et al. 2024

JCM

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Background: Auditory neuropathy (AN) is a hearing disorder that affects neural activity in the VIIIth cranial nerve and central auditory pathways. Progressive forms have been reported in a number of neurodegenerative diseases and may occur as a result of both the deafferentiation and desynchronisation of neuronal processes. The purpose of this study was to describe changes in auditory function over time in a patient with axonal neuropathy and to explore the effect of auditory intervention. Methods: We tracked auditory function in a child with progressive AN associated with Charcot–Marie–Tooth (Type 2C) disease, evaluating hearing levels, auditory-evoked potentials, and perceptual abilities over a 3-year period. Furthermore, we explored the effect of auditory intervention on everyday listening and neuroplastic development. Results: While sound detection thresholds remained constant throughout, both electrophysiologic and behavioural evidence suggested auditory neural degeneration over the course of the study. Auditory brainstem response amplitudes were reduced, and perception of auditory timing cues worsened over time. Functional hearing ability (speech perception in noise) also deteriorated through the first 1.5 years of study until the child was fitted with a “remote-microphone” listening device, which subsequently improved binaural processing and restored speech perception ability to normal levels. Conclusions: Despite the deterioration of auditory neural function consistent with peripheral axonopathy, sustained experience with the remote-microphone listening system appeared to produce neuroplastic changes, which improved the patient’s everyday listening ability—even when not wearing the device.

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Section: Introductionmentioning

confidence: 99%

Remediation of Perceptual Deficits in Progressive Auditory Neuropathy: A Case Study

Rance,

Tomlin,

Yiu

et al. 2024

JCM

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NADH improves AIF dimerization and inhibits apoptosis in iPSCs-derived neurons from patients with auditory neuropathy spectrum disorder

Qiu,

Wang,

Pan

et al. 2024

Hearing Research

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Cortical Auditory Evoked Potential Testing in Children With Auditory Neuropathy Spectrum Disorder

Morlet,

Valania,

Walter

et al. 2024

Am J Audiol

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Purpose: In the present report, we reviewed the role of cortical auditory evoked potentials (CAEPs) as an objective measure during the evaluation and management process in children with auditory neuropathy spectrum disorder (ANSD). Method: We reviewed the results of CAEP recordings in 66 patients with ANSD aged between 2 months and 12 years and assessed the relationship between their characteristics (prevalence, morphology, latencies, and amplitudes) and various clinical features, including the mode of medical management. Results: Overall, the CAEPs were present in 85.2% of the ears tested. Factors such as prematurity, medical complexity, neuronal issues, or presence of syndromes did not have an effect on the presence or absence of CAEPs. CAEP latencies were significantly shorter in ears with cochlear nerve deficiency than in ears with a normal caliber nerve. Three different patterns of CAEP responses were observed in patients with bilateral ANSD and present cochlear nerves: (a) responses with normal morphology and presence of both P1-P2 complex and N2 components, (b) responses with abnormal morphology and presence of the N2 component but undefined P1-P2 complex peak, and (c) entirely absent responses. None of the patients with normal, mild, or moderate degree of hearing loss had a complete absence of CAEP responses. No significant differences were uncovered when comparing the latencies across unaided and aided children and children who later received cochlear implants. Conclusions: The CAEP protocol used in our ANSD program did inform about the presence or absence of central auditory stimulation. Absent responses typically fit into an overall picture of complete auditory deprivation and all of these children were ultimately offered cochlear implants after failing to develop oral language. Present responses, on the other hand, were acknowledged as a sign of some degree of auditory stimulation but always interpreted with caution given that prognostic implications remain unclear.

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A 15-year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: II. Management and Outcomes

Cited by 3 publications

References 54 publications

Remediation of Perceptual Deficits in Progressive Auditory Neuropathy: A Case Study

Remediation of Perceptual Deficits in Progressive Auditory Neuropathy: A Case Study

NADH improves AIF dimerization and inhibits apoptosis in iPSCs-derived neurons from patients with auditory neuropathy spectrum disorder

Cortical Auditory Evoked Potential Testing in Children With Auditory Neuropathy Spectrum Disorder

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