Related Experiment Video

Updated: May 21, 2026

Semi-Automated Analysis of Peak Amplitude and Latency for Auditory Brainstem Response Waveforms Using R
06:01

Semi-Automated Analysis of Peak Amplitude and Latency for Auditory Brainstem Response Waveforms Using R

Published on: December 9, 2022

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
PubMed

Insights

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.

More Related Videos

Infant Auditory Processing and Event-related Brain Oscillations06:34

Infant Auditory Processing and Event-related Brain Oscillations

Published on: July 1, 2015

Related Concept Videos

Auditory Pathway01:15

Auditory Pathway

Auditory pathways constitute the complex neural circuits responsible for transmitting and interpreting auditory information from the peripheral auditory system to the brain. Sound waves are initially captured by the outer ear, funneled through the ear canal, and reach the tympanic membrane (eardrum). These vibrations are transmitted via the middle ear's ossicles to the inner ear's cochlea.
When viewed cross-sectionally, the cochlea reveals the scala vestibuli and scala tympani flanking the...
Vitamins01:30

Vitamins

Vitamins, derived from the Latin word for life, are essential organic substances required in small quantities for optimal growth and overall well-being. Unlike other organic nutrients, vitamins don't act as sources of energy or building materials but rather facilitate these nutrients' utilization by the body. Vitamins are predominantly coenzymes, assisting enzymes in specific chemical actions, like the oxidation of glucose for energy involving B vitamins. Most vitamins are not produced in our...
Unrenewable Cells00:50

Unrenewable Cells

In humans, the photoreceptor cells of the eye and sensory hair cells of the ear lack stem cells. These cells are thus unrenewable and cannot be replaced when they are damaged or destroyed.
Photoreceptors
The retina is composed of several layers and contains specialized cells called photoreceptors. The photoreceptors (rods and cones) change their membrane potential when stimulated by light energy. There are two types of photoreceptors—rods and cones—which differ in the shape of their outer...
Hearing01:31

Hearing

When we hear a sound, our nervous system is detecting sound waves—pressure waves of mechanical energy traveling through a medium. The frequency of the wave is perceived as pitch, while the amplitude is perceived as loudness.
Traumatic Brain Injury l: Introduction01:28

Traumatic Brain Injury l: Introduction

DefinitionTraumatic brain injury, or TBI, is a disturbance of normal brain function induced by an external mechanical force, such as a direct blow to the head or a penetrating injury. It can affect both brain structure and function, producing a wide range of clinical outcomes. TBI is a heterogeneous condition, meaning its effects may differ based on the type, location, and severity of the injury.Basis of ClassificationTBI is classified based on severity, injury mechanism, or pathophysiology. In...
Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...