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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

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Late-onset unilateral auditory neuropathy/dysynchrony: a case study.

Andrew Stuart¹, Kristal N Mills

¹Department of Communication Sciences and Disorders, East Carolina University, Greenville, NC 27858-4353, USA. stuarta@ecu.edu

Journal of the American Academy of Audiology

|November 26, 2009

Summary

This study reports a rare case of late-onset unilateral auditory neuropathy/dyssynchrony (AN/AD) in a 64-year-old woman. Findings include asymmetric hearing loss and abnormal auditory brainstem responses, challenging typical AN/AD presentation.

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Area of Science:

Audiology
Neuroscience
Otolaryngology

Background:

Auditory neuropathy/dyssynchrony (AN/AD) is a hearing disorder typically presenting in early childhood and affecting both ears.
The condition is characterized by abnormal neural synchrony in the auditory pathway, despite intact cochlear function.

Observation:

A 64-year-old female presented with a two-to-three-year history of progressive, unilateral hearing loss.
Diagnostic evaluations included a comprehensive behavioral and electrophysiological test battery.

Findings:

Audiometric results revealed a unilateral notched hearing loss centered at 1000 Hz.
Key electrophysiological findings included present bilateral otoacoustic emissions and cochlear microphonics, absent ipsilateral acoustic reflexes, abnormal auditory brainstem responses on the affected side, and reduced speech recognition disproportionate to the audiogram.
Middle- and long-latency evoked responses showed reduced amplitude on the affected side.

Implications:

This case highlights the possibility of late-onset, unilateral AN/AD, expanding the known clinical spectrum of the disorder.
The findings underscore the importance of a thorough audiological and electrophysiological assessment for diagnosing atypical AN/AD presentations.
Understanding such cases can improve diagnostic accuracy and inform potential management strategies for patients with unusual auditory pathway dysfunction.