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Auditory neuropathy: an update.

William Peter Rea Gibson1, Halit Sanli

  • 1Sydney Cochlear Implant Centre, University of Sydney, Sydney, Australia. gibsonwp@unwired.com.au

Ear and Hearing
|May 15, 2007
PubMed
Summary
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Auditory neuropathy (AN) patients with normal electric auditory brainstem responses (EABR) after cochlear implantation show better outcomes. Round window electrocochleography (RWECochG) findings like abnormal positive potential (APP) combined with normal EABR predict success in AN ears.

Area of Science:

  • Otoacoustic Emissions and Auditory Evoked Potentials
  • Neuroscience and Audiology
  • Clinical Electrophysiology

Background:

  • Auditory neuropathy (AN) is characterized by abnormal auditory nerve function with present otoacoustic emissions (OAEs) and absent or abnormal auditory brainstem responses (ABR).
  • Electrophysiological assessment is crucial for understanding auditory pathway integrity in AN.
  • Predicting cochlear implant outcomes in AN patients remains a challenge.

Purpose of the Study:

  • To characterize round window electrocochleography (RWECochG) and electric auditory brainstem responses (EABR) in pediatric patients with auditory neuropathy (AN).
  • To evaluate the predictive value of RWECochG and EABR for cochlear implant surgery outcomes in AN.
  • To differentiate AN subtypes based on electrophysiological findings and correlate with post-implantation speech perception.

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Main Methods:

  • Longitudinal study of pediatric cochlear implant patients (1994-2005), including 39 children with AN (78 ears).
  • Electrophysiological testing included RWECochG, ABR, and implant-evoked EABR.
  • Speech perception outcomes were collected prospectively; OAE data were collected retrospectively.

Main Results:

  • All AN ears exhibited large cochlear microphonics (CM) and abnormal positive potential (APP) on RWECochG.
  • EABR results were normal in 45 ears and abnormal in 15 AN ears.
  • Patients with APP and normal EABR showed significantly better two-year post-implant speech perception scores compared to those with abnormal EABR and controls.

Conclusions:

  • RWECochG in AN ears reveals prominent CM and APP, suggesting outer hair cell survival despite inner hair cell loss.
  • Normal EABR in AN patients receiving cochlear implants is a strong predictor of significantly better auditory outcomes.
  • The presence of APP and/or OAE in ears with absent/abnormal ABR may not signify auditory nerve pathology but rather cochlear dysfunction.