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Updated: Nov 2, 2025
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Intrathecal Application of a Fluorescent Dye for the Identification of Cerebrospinal Fluid Leaks in Cochlear Malformation
Published on: February 29, 2020
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Chiari Type I Malformation Presenting with Unilateral Hearing Loss
Abdulhamid Ciçek1,2, Jeroen Cortier1, Sarah Hendrickx1
1Department of Neurosurgery, AZ Delta, Roeselare, Belgium.
Summary
Chiari type I malformation, a condition often causing headaches, can rarely present as hearing loss. Surgical decompression led to hearing recovery in a patient, highlighting this unusual presentation.
Area of Science:
- Neurology
- Otolaryngology
- Neurosurgery
Background:
- Chiari type I malformations are structural defects in the cerebellum.
- Occipitocervical headache is the most common symptom.
- Hearing loss as a primary symptom is rare.
Purpose of the Study:
- To report a case of Chiari type I malformation presenting solely with hearing loss.
- To discuss the pathophysiology and management of Chiari type I malformations.
- To emphasize the potential for hearing loss as a presenting symptom.
Main Methods:
- A case study of a young woman with unilateral hearing loss.
- Diagnosis of Chiari type I malformation.
- Surgical intervention: suboccipital craniectomy with C1 laminectomy and duraplasty.
Main Results:
- Postoperative resolution of unilateral hearing loss.
- Normalization of audiometry following surgical decompression.
- Successful treatment of hearing loss attributed to Chiari type I malformation.
Conclusions:
- Chiari type I malformation can manifest primarily as hearing loss.
- Surgical decompression can effectively restore hearing.
- This presentation warrants greater awareness in the neurological and otolaryngological communities.
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