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Auditory neuropathy: endocochlear lesion or temporal processing impairment? Implications for diagnosis and management
Petros V Vlastarakos1, Thomas P Nikolopoulos, Evangelia Tavoulari
1ENT Department, Hippokrateion General Hospital of Athens, 114 Vas. Sofias Avenue, Athens, 11527, Athens, Greece. pevlast@hotmail.com
Summary
Auditory neuropathy/dys-synchrony is a common cause of childhood hearing loss, affecting approximately 8% of cases. Early diagnosis and interventions like hearing aids, speech therapy, or cochlear implants are crucial for affected children.
Area of Science:
- Pediatric Audiology
- Neuroscience
- Genetics
Background:
- Auditory neuropathy/dys-synchrony (AN/AD) presents with absent auditory brainstem responses and normal otoacoustic emissions.
- It can significantly impact language acquisition in children, leading to ongoing debate.
- Understanding its pathophysiology and management is critical.
Purpose of the Study:
- To review current knowledge on Auditory neuropathy/dys-synchrony.
- To present therapeutic strategies for AN/AD management.
- To consider the underlying pathophysiology of AN/AD.
Main Methods:
- Comprehensive literature review of Medline and other databases.
- Inclusion of related books and various study types (clinical trials, cohort studies, case reports, reviews).
Main Results:
- AN/AD is more frequent than previously thought, accounting for ~8% of pediatric hearing loss.
- Risk factors include hyperbilirubinemia and hypoxia; genetic factors (e.g., otoferlin gene) and neuropathic disorders are implicated.
- Probable mechanisms include auditory nerve myelinopathy and neural discharge desynchrony.
Conclusions:
- AN/AD is a significant cause of hearing impairment in children.
- Accurate diagnosis using electrophysiological measures is vital.
- Habilitation involves hearing aids, speech/language therapy, sign language, and cochlear implantation.
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