Child's Nervous System

, Volume 24, Issue 9, pp 1063–1066

Chiari I malformation presenting with hearing loss: surgical treatment and literature review


  • Gregory G. Heuer
    • Division of NeurosurgeryChildren’s Hospital of Philadelphia
  • Brandon Gabel
    • Division of NeurosurgeryChildren’s Hospital of Philadelphia
  • Paul S. Lemberg
    • Allen Ear, Nose, and Throat Association
    • Division of NeurosurgeryChildren’s Hospital of Philadelphia
Case Report

DOI: 10.1007/s00381-008-0652-2

Cite this article as:
Heuer, G.G., Gabel, B., Lemberg, P.S. et al. Childs Nerv Syst (2008) 24: 1063. doi:10.1007/s00381-008-0652-2



Chiari I malformations can present with a number of clinical signs and symptoms.


We present a case of an 11-year-old girl that presented with significant sensorineural hearing loss as her only Chiari-related symptom. The patient had four audiograms that all demonstrated progressive bilateral hearing loss between 10 and 30 dB. On magnetic resonance scan, the patient was found to have a Chiari I malformation. The patient had 9 mm of tonsillar herniation but no syrinx or hydrocephalus was present. On exam, the patient did not exhibit any other symptoms of her Chiari malformation or cranial nerve abnormalities other than sensorineural hearing loss. The patient underwent a suboccipital craniotomy, C1 laminectomy, and duraplasty. The patient noted a subjective improvement in hearing and an audiogram performed at 3 months postoperatively demonstrated normal hearing bilaterally.


Sensorineural hearing loss may be caused by Chiari I malformations. This symptom may improve following decompression.


Chiari malformationSensorineuralHearing lossSurgery

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© Springer-Verlag 2008