Transient deafness due to temperature-sensitive auditory neuropathy

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.
• 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.