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Auditory neuropathy/dys-synchrony: diagnosis and management
Charles I Berlin1, Linda Hood, Thierry Morlet
1Department of Otolaryngology, Kresge Hearing Research Laboratory, Louisiana State University Health Sciences Center, New Orleans, LA 70112, USA.
Mental Retardation and Developmental Disabilities Research Reviews
|December 4, 2003
Summary
Auditory neuropathy/dys-synchrony (AN/AD) affects up to 10% of deaf individuals. Diagnosis requires distinguishing cochlear microphonics from neural responses using auditory brainstem responses (ABRs).
Area of Science:
- Audiology
- Neuroscience
- Otoacoustic Emissions
Background:
- Auditory brainstem responses (ABRs) and otoacoustic emissions (OAEs) are objective measures of auditory function.
- Normal OAEs indicate healthy cochlear outer hair cell function; ABRs indicate synchronous neural response.
Purpose of the Study:
- To describe auditory neuropathy/dys-synchrony (AN/AD), a condition where standard auditory tests may be inconsistent.
- To highlight diagnostic challenges and management strategies for AN/AD.
Main Methods:
- Utilizing ABRs with condensation and rarefaction clicks to differentiate cochlear microphonics (CM) from neural responses.
- Comparing ABRs, OAEs, and behavioral audiograms to identify discrepancies indicative of AN/AD.
Main Results:
- Patients with AN/AD can present with normal OAEs but absent or abnormal ABRs, inconsistent with audiograms.
- This condition may account for up to 10% of diagnosed deafness.
Conclusions:
- Accurate AN/AD diagnosis relies on specific ABR testing to differentiate CM from neural activity.
- Management requires visual language exposure (e.g., ASL, Cued Speech) and ongoing patient monitoring due to potential auditory function changes.