Long Ringing Cochlear Microphonics - Not Unique to Auditory Neuropathy Spectrum Disorder in Children

Abstract

Background: The detection of cochlear microphonics in surface recordings has been considered a distinctive sign of outer hair cell integrity in participants with auditory neuropathy spectrum disorder. The study highlights that this notion could result in misdiagnosis of patients.Methods: The study reports three children with sloping hearing loss who were misdiagnosed as auditory neuropathy spectrum disorder based on absence of auditory brainstem response and presence of long ringing cochlear microphonics. The study also reports of three children with abnormal cochlear microphonics who have neurological abnormalities.Results: The abnormal cochlear microphonics (long ringing) with absent auditory brainstem response was noted in sloping hearing loss participants. In addition, abnormal cochlear microphonics was also observed along with auditory brainstem response in children with neurological abnormalities.Conclusion: Our results show that abnormal CM detection in surface recording is not a distinctive feature of auditory neuropathy spectrum disorder and participants who are diagnosed with auditory neuropathy spectrum disorder based on traditional diagnostic criteria should be reviewed to rule out possible misdiagnosis.