Auditory system dysfunction due to infantile thiamine deficiency: Long-term auditory sequelae

J. Attias, E. Raveh, A. Aizer-Dannon, A. Bloch-Mimouni, A. Fattal-Valevski

Research output: Contribution to journalArticlepeer-review


Eleven infants who were fed a thiamine-deficient formula for a mean of 3 months were evaluated for immediate and long-term auditory abnormalities. At presentation, 8 infants had auditory neuropathy spectrum disorder (ANSD), which resolved with supplementary thiamine in 5 children, was permanent in 2 children, and deteriorated in 1 patient who died at the age of 7 years. An additional patient had an auditory pattern corresponding to that of auditory neuropathy of brain stem origin. The 2 remaining patients had unilateral cochlear hearing loss. Six to 8 years later, all patients with transient ANSD had normal audiograms, 2 patients had unilateral cochlear hearing loss, and the rest had neural hearing loss. All survivors had a language developmental delay and impaired speech intelligibility of varying degrees, especially in the presence of background noise. Thiamine is crucial for normal auditory development and function, and its deficiency may be considered an acquired metabolic cause of ANSD in infants.

Original languageEnglish
Pages (from-to)309-320
Number of pages12
JournalAudiology and Neurotology
Issue number5
StatePublished - Aug 2012


  • Auditory neuropathy of brain stem origin
  • Auditory neuropathy spectrum disorder
  • Auditory sequelae
  • B deficiency
  • Hearing loss
  • Infants
  • Language development

ASJC Scopus subject areas

  • Sensory Systems
  • Speech and Hearing
  • Physiology
  • Otorhinolaryngology


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