Auditory brainstem response findings and peripheral auditory sensitivity in adrenoleukodystrophy

Pillion, Joseph P.; Kharkar, S.; Mahmood, Asif; Moser, Hugo W.; Shimizu, Hiroshi

doi:10.1016/j.jns.2006.04.001

Cited by 6 publications

(6 citation statements)

References 40 publications

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“…Although hearing loss was present among some of the remaining subjects in this study in the higher frequency region, peripheral auditory sensitivity was within normal limits in the majority of patients in spite of the presence of marked ABR abnormalities. Previous studies have shown that the prevalence of hearing loss in AMN does not exceed that found in age and sex matched control groups [5]. These findings are in agreement with other studies that have reported that peripheral hearing loss is not commonly found in disorders impacting on the auditory pathways at brainstem levels [8].…”

Section: Discussionsupporting

confidence: 91%

“…Several studies measuring ABR latencies in AMN have shown significantly prolonged absolute peak latencies and interpeak intervals [4][5][6]17]. In our study, all waves were delayed or absent with wave V being the most delayed, then wave III and wave I.…”

Section: Discussionsupporting

confidence: 55%

“…2 as well as an earlier paper [5], suggest Wave V and Wave I-V interpeak latency intervals are typically affected to the greatest degree in AMN, the alternative approach shown in Fig. 4 to analysis of the ABR results is revealing [16] and showed that, particularly for the male subjects with AMN, waves III and I tend to be the first abnormal ABR component.…”

Section: Discussionmentioning

confidence: 51%

“…However, it has recently been reported that the prevalence of hearing loss in patients with AMN does not exceed that found in age and sex matched normative control groups [5]. ABR findings typically are markedly abnormal in the majority of patients with AMN [4][5][6]. It has been reported for a limited number of patients with AMN that acoustic reflexes are preserved [6] although acoustic reflex abnormalities have been reported by other investigators in several patients [4].…”

Section: Introductionmentioning

confidence: 95%

Section: Introductionmentioning

confidence: 96%

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Auditory function in adrenomyeloneuropathy

Pillion

Moser

Raymond

2008

Journal of the Neurological Sciences

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Auditory brainstem responses (ABR), ipsilateral and contralateral acoustic reflexes and the masking level difference for speech (MLD) were studied in 29 patients with adrenomyeloneuropathy (AMN). Abnormalities were seen for all ABR components with Waves V and III affected to the greatest degree. For male patients with AMN, the I-III, III-V and I-V interpeak latency intervals were abnormal for a majority of patients. For female patients with AMN, the I-V and III-V interpeak latency intervals were abnormal for a majority of patients with the I-III interval less affected. Contralateral acoustic reflexes were elevated or absent for approximately 50% of ears. Ipsilateral acoustic reflexes were abnormal for 25% of ears. MLDs were significantly reduced in 72% of patients. When considered in terms of the earliest ABR wave abnormality, the earlier components of the ABR (i.e., Waves III and I) were the initial components impaired for the majority of ears. Word recognition in quiet was relatively unimpaired for all subjects. Despite the presence of marked ABR abnormalities, patients with AMN denied the presence of significant difficulty hearing.

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Section: Discussionsupporting

confidence: 91%