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Glucose transporter type 1 deficiency syndrome (GLUT1-DS) is an uncommon condition represented by an infantile-onset disorder, frequently arising from heterozygous mutations in the gene. Individuals with GLUT1-DS may present with early-onset seizures (typically manifesting before 4 years of age), developmental delay, and complex movement disorders. In fewer cases, stroke-like events or hemiplegic migraine-like symptoms are also reported, defined by unilateral paresis affecting 1 side of the body and/or one-half of the face, occasionally accompanied by speech impairment.

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Facial nerve schwannomas (FNSs) eroding through the external auditory canal (EAC) are unusual and present difficult management options.When facial nerve function is normal, observation is generally recommended.If the tumor completely obstructs the EAC creating a conductive hearing loss as in this case, mapping of the motor fibers of the facial nerve may be considered with partial resection to alleviate the conductive hearing loss.

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