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Auditory function in adrenomyeloneuropathy.

Joseph P Pillion1, Hugo W Moser, Gerald V Raymond

  • 1Kennedy Krieger Institute, United States. pillion@kennedykrieger.org

Journal of the Neurological Sciences
|January 18, 2008
PubMed
Summary
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Auditory brainstem responses (ABR) reveal significant hearing pathway abnormalities in adrenomyeloneuropathy (AMN) patients, even when individuals report no hearing difficulties. Early ABR wave components are most frequently affected.

Area of Science:

  • Neuroscience
  • Audiology
  • Genetics

Background:

  • Adrenomyeloneuropathy (AMN) is a rare genetic disorder affecting the nervous system and adrenal glands.
  • Hearing impairment can be a symptom of AMN, but its audiological profile is not fully understood.
  • Auditory pathway integrity is crucial for communication and quality of life.

Purpose of the Study:

  • To investigate auditory pathway function in patients with adrenomyeloneuropathy (AMN).
  • To characterize auditory brainstem responses (ABR), acoustic reflexes, and masking level differences (MLD) in AMN.
  • To correlate audiological findings with the clinical presentation of AMN.

Main Methods:

  • Auditory brainstem responses (ABR) were recorded in 29 AMN patients.
  • Ipsilateral and contralateral acoustic reflexes were assessed.

Related Experiment Videos

  • Masking level difference for speech (MLD) was measured.
  • ABR wave latencies and reflex parameters were analyzed.

    • Main Results:

    • All patients showed abnormalities in ABR components, with Waves V and III most affected.
    • Interpeak latency intervals (I-III, III-V, I-V) were abnormal in a majority of male and female AMN patients.
    • Contralateral acoustic reflexes were abnormal in ~50% of ears, and ipsilateral reflexes in 25%.
    • MLDs were significantly reduced in 72% of patients.
    • Early ABR components (Waves III and I) were the initial sites of impairment.
    • Word recognition in quiet was largely unaffected.
    • Patients often denied significant hearing difficulties despite objective ABR abnormalities.

    Conclusions:

    • The auditory pathway, particularly early ABR components, is frequently affected in AMN.
    • Objective audiological testing reveals significant hearing pathway dysfunction in AMN patients, often unperceived by the patients.
    • These findings highlight the importance of comprehensive audiological assessment in AMN management.