Chiari I malformation presenting with hearing loss: surgical treatment and literature review.
OBJECTIVE
Chiari I malformations can present with a number of clinical signs and symptoms.


METHODS
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.


CONCLUSIONS
Sensorineural hearing loss may be caused by Chiari I malformations. This symptom may improve following decompression.
Division of Neurosurgery, Children's Hospital of Philadelphia, Wood Center, 6th Floor, 34th and Civic Center Blvd, Philadelphia, PA 19104, USA.
Gregory G
GG
Heuer
Brandon
B
Gabel
Paul S
PS
Lemberg
Leslie N
LN
Sutton
D000293:Adolescent; D001139:Arnold-Chiari Malformation / Q000150:complications* / Q000601:surgery*; D002648:Child; D003399:Craniotomy; D019299:Decompression, Surgical; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008582:Meningitis, Aseptic / Q000209:etiology; D011183:Postoperative Complications