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Delayed visual maturation associated with auditory neuropathy/dyssynchrony.

Mohammed Aldosari1, Ann Mabie, Aatif M Husain

  • 1Department of Pediatrics (Neurology), Duke University, Veterans Affairs Medical Center, Durham, North Carolina, USA.

Journal of Child Neurology
|June 26, 2003
PubMed
Summary
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Infants with delayed visual maturation may also have undiagnosed hearing loss. Early hearing evaluations and follow-up auditory testing are crucial for accurate diagnosis and intervention in these children.

Area of Science:

  • Pediatric Neurology
  • Neurodevelopmental Disorders
  • Auditory Neuroscience

Background:

  • Delayed visual maturation (DVM) describes infants with initial blindness and subsequent visual improvement, with unknown mechanisms.
  • Auditory neuropathy/dyssynchrony (AN/AD) involves hearing impairment with abnormal auditory evoked potentials but normal otoacoustic emissions.
  • Congenital hearing impairment requires careful audiological assessment in infants.

Observation:

  • A 9-month-old infant presented with no visual fixation for the first 3 months and congenital hearing impairment.
  • Initial auditory brainstem evoked potential (ABEP) testing at 2.5 months showed no response to 90 dB nHL click stimuli, despite normal otoacoustic emissions.
  • The infant later showed significant improvement in both vision and hearing.

Findings:

Related Experiment Videos

  • Follow-up ABEP testing at 9 months revealed reproducible waveforms, indicating improved auditory pathway function.
  • This case demonstrates a potential co-occurrence of DVM and auditory neuropathy/dyssynchrony.
  • The auditory dysfunction resolved or significantly improved over time.

Implications:

  • Highlights the importance of comprehensive hearing evaluations for infants diagnosed with delayed visual maturation.
  • Suggests the need for serial auditory brainstem evoked potential testing in infants with DVM before initiating audiological interventions.
  • Underscores the dynamic nature of auditory function in certain neurodevelopmental conditions.