Auditory function in adrenomyeloneuropathy

Joseph P. Pillion; Hugo W. Moser; Gerald V. Raymond

doi:10.1016/j.jns.2007.12.011

Auditory function in adrenomyeloneuropathy

Joseph P. Pillion, Hugo W. Moser, Gerald V. Raymond

Research output: Contribution to journal › Article › peer-review

10 Scopus citations

Abstract

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.

Original language	English (US)
Pages (from-to)	24-29
Number of pages	6
Journal	Journal of the Neurological Sciences
Volume	269
Issue number	1-2
DOIs	https://doi.org/10.1016/j.jns.2007.12.011
State	Published - Jun 15 2008
Externally published	Yes

Bibliographical note

Funding Information:
The study was supported by the Johns Hopkins University School of Medicine General Clinical Research Center (M01-RR00052) and the National Institutes of Health (R21 NS 045716, NINDS). We thank the patients and their families for their continued support for these studies. We acknowledge the contributions of K. Hollandsworth, W. Foreman, A. Snitcher, J. Pittman, and the entire ALD research team for providing excellent support.

Keywords

Acoustic reflex
Adrenomyeloneuropathy (AMN)
Auditory brainstem response (ABR)
Masking level difference (MLD)
Very long chain fatty acids (VLCFA)
Word recognition

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10.1016/j.jns.2007.12.011

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abstract = "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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note = "Funding Information: The study was supported by the Johns Hopkins University School of Medicine General Clinical Research Center (M01-RR00052) and the National Institutes of Health (R21 NS 045716, NINDS). We thank the patients and their families for their continued support for these studies. We acknowledge the contributions of K. Hollandsworth, W. Foreman, A. Snitcher, J. Pittman, and the entire ALD research team for providing excellent support.",

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N2 - 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.

AB - 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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Auditory function in adrenomyeloneuropathy

Abstract

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Keywords

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