Autism and peripheral hearing loss: a systematic review

Int J Pediatr Otorhinolaryngol. 2014 Jan;78(1):96-101. doi: 10.1016/j.ijporl.2013.10.063. Epub 2013 Nov 15.


Objective: To systematically review the literature describing the relationship between autism spectrum disorder (ASD) and peripheral hearing loss including literature recommendations for audiological assessment and auditory habilitation in cases where peripheral hearing loss and ASD coexist.

Data sources: Published studies indexed in MEDLINE (1948-2011).

Review methods: The search strategy identified 595 potential studies. After a review of the titles, 115 abstracts were reviewed and 39 articles were retrieved and assessed independently by at least two authors for possible inclusion. 22 articles pertained to children with ASD and peripheral hearing loss, hearing assessment in children with ASD, audiological habilitation for children with ASD or hyper-responsiveness in children with ASD. 17 further studies were garnered from the reference section of the 22 papers.

Results: Controversy exists in the literature regarding prevalence of hearing impairment among individuals with ASD. In cases where ASD and hearing impairment co-exist, diagnosis of one condition often leads to a delay in diagnosing the other. Audiological assessment can be difficult in children with ASD and test-retest reliability of behavioural thresholds can be poor. In cases where hearing impairment exists and hearing aids or cochlear implantation are recommended, devices are often fit with special considerations for the child with ASD. Hyper-responsiveness to auditory stimuli may be displayed by individuals with ASD. Evidence or the suspicion of hyper-responsiveness may be taken into consideration when fitting amplification and planning behavioural intervention.

Conclusions: Prevalence rates of hearing impairment among individuals with ASD continue to be debated. At present there is no conclusive evidence that children with ASD are at increased risk of peripheral hearing loss. A complete audiological assessment is recommended in all cases where ASD is suspected so as not to delay the diagnosis of hearing impairment in the event that hearing loss and ASD co-exist. Objective assessment measures should be used to confirm behavioural testing in order to ensure reliability of audiological test results. Fitting of hearing aids or cochlear implantation are not contraindicated when hearing loss is present in children with ASD; however, success with these devices can be variable.

Keywords: Autism; Cochlear implant; Hearing aids; Hearing loss; Hyper-responsiveness; Hyperacusis.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Autistic Disorder / complications*
  • Cochlear Implantation
  • Hearing Aids
  • Hearing Loss / complications*
  • Hearing Loss / epidemiology
  • Hearing Loss / therapy
  • Hearing Tests
  • Humans
  • Prevalence