RESUMO
INTRODUCTION: Chiari type I malformations can present in different ways, but the most frequent symptom is an occipitocervical headache. Hearing loss as the main presenting symptom is rare. CASE: A young woman with progressive left-sided unilateral hearing loss was diagnosed with a Chiari type I malformation. She underwent a suboccipital craniectomy with C1 laminectomy and duraplasty. The hearing loss had resolved postoperatively with normalization of the audiometry. CONCLUSION: Chiari type I malformation can present solely with hearing loss. Improvement after surgical decompression is possible. This phenomenon is not emphasized well enough within the neurologic community. In this report, we present a summary of the pathophysiology and management in Chiari type I malformations.
Assuntos
Malformação de Arnold-Chiari , Perda Auditiva Unilateral , Feminino , Humanos , Perda Auditiva Unilateral/etiologia , Perda Auditiva Unilateral/cirurgia , Imageamento por Ressonância Magnética , Descompressão Cirúrgica , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/cirurgia , Laminectomia , Resultado do TratamentoRESUMO
A 39-year-old woman presented with sudden onset of double vision, right upper eyelid swelling and ptosis, and orbital pain. Imaging revealed an irregular mass of the upper right orbit with central non-enhancing areas. Upon inquiry, the patient recalled an intraorbital trauma with a crayon in her childhood, 35 years ago. Via translid anterior orbitotomy, remnants of a blue crayon embedded in an orbital fat mass were removed. Histopathology showed scavenger reaction of macrophages and sclerosis. Energy-dispersive X-ray spectroscopy revealed the presence of silicate particles. Repeated courses of corticosteroids were given. The patient deteriorated with reduced vision and frozen globe owing to severe orbital fibrosis of the entire orbit.
