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Auditory system dysfunction due to infantile thiamine deficiency: long-term auditory sequelae.
J Attias1, E Raveh, A Aizer-Dannon
1Department of Communication Sciences and Disorders, University of Haifa, Haifa, Israel. attiasj@netvision.net.il
Audiology & Neuro-Otology
|June 29, 2012
Summary
Thiamine deficiency in infants can cause auditory neuropathy spectrum disorder (ANSD) and hearing loss. Supplementation can resolve ANSD, but long-term developmental delays may persist.
Area of Science:
- Pediatrics
- Neuroscience
- Otolaryngology
Background:
- Thiamine (Vitamin B1) is essential for neural development and function.
- Infant nutrition plays a critical role in auditory system development.
- Auditory neuropathy spectrum disorder (ANSD) presents diagnostic challenges in infants.
Purpose of the Study:
- To evaluate immediate and long-term auditory abnormalities in infants with thiamine deficiency.
- To investigate the impact of thiamine supplementation on auditory outcomes.
- To establish thiamine deficiency as a potential acquired metabolic cause of ANSD.
Main Methods:
- Retrospective evaluation of eleven infants fed a thiamine-deficient formula.
- Auditory assessments including audiograms and brainstem auditory evoked potentials.
- Long-term follow-up (6-8 years) assessing auditory and language development.
Main Results:
- Eight of eleven infants presented with auditory neuropathy spectrum disorder (ANSD).
- Five infants with ANSD showed resolution with thiamine supplementation; two had permanent hearing loss.
- Survivors experienced language delays and impaired speech intelligibility, particularly with background noise.
Conclusions:
- Thiamine deficiency is a significant acquired metabolic cause of ANSD in infants.
- Early thiamine supplementation can reverse ANSD in some cases.
- Long-term auditory and speech deficits necessitate ongoing developmental monitoring.
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