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Transient deafness due to temperature-sensitive auditory neuropathy

A Starr1, Y Sininger, M Winter

  • 1House Ear Institute, Los Angeles, California, USA.

Ear and Hearing
|July 10, 1998
PubMed
Summary
This summary is machine-generated.

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Children with auditory neuropathy experience temporary deafness during fevers. This is likely due to a demyelinating disorder affecting the auditory nerve, causing a conduction block when body temperature rises.

Area of Science:

  • Pediatric Neurology
  • Neurophysiology
  • Auditory System Disorders

Background:

  • Auditory neuropathy is a hearing disorder where the cochlea functions normally but the auditory nerve does not transmit sound signals effectively.
  • Transient deafness during febrile states in children suggests a temperature-sensitive neurological component.

Observation:

  • Three children (two siblings and one unrelated) presented with temporary hearing loss during fevers.
  • Electrophysiological tests revealed abnormal auditory brainstem responses (ABRs) and normal otoacoustic emissions (OAEs), indicating cochlear function was intact.
  • Hearing impairment worsened significantly with elevated body temperature, affecting pure-tone thresholds and speech comprehension.

Findings:

  • The children were diagnosed with auditory neuropathy, characterized by impaired auditory nerve function.

Related Experiment Videos

  • A conduction block in the auditory nerves, likely due to a demyelinating disorder, was identified as the cause of temporary deafness during fever.
  • The condition in the siblings appears to be inherited as a recessive disorder.

    • Implications:

    • This study elucidates the mechanism of transient deafness in children with auditory neuropathy, linking it to febrile states.
    • Understanding this temperature-dependent demyelination is crucial for diagnosis and management of auditory nerve disorders.
    • Genetic counseling may be warranted for families with a history of auditory neuropathy.