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Quiz Case
David A. Lehman, BA;
Anthony A. Mancuso, MD;
Patrick J. Antonelli, MD
Gainesville, Fla
Arch Otolaryngol Head Neck Surg. 2001;127:331-332.
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A 2-YEAR-OLD white girl with a history of neurofibromatosis type 1 (NF-1)
and rhabdomyosarcoma of the right temporal bone presented with a 4-week history
of right facial nerve paralysis and ataxic gait. Prior radiographic studies
revealed multiple neurofibromas, including involvement of the ipsilateral
fallopian canal in the proximal mastoid segment (Figure 1, arrow), but no involvement
of the internal auditory canal (Figure 2).
The rhabdomyosarcoma had been treated with chemotherapy according to the Intergroup
Rhabdomyosarcoma Study IV protocol, regimen 45 (vincristine, actinomycin,
and cyclophosphamide), for 1 year. Radiation therapy was not performed because
of the mother's concerns regarding adverse effects.
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Figure 1.
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Figure 2.
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Physical examination revealed right-sided facial paralysis, House-Brackmann
grade VI. No significant otologic abnormality or cervical adenopathy was identified.
Audiometric evaluation demonstrated sound-field thresholds of 35 dB from 500
to 4000 . . . [Full Text of this Article]
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