Sep 2026· The Laryngoscope· 0 citations· 2 references
Medicine
TL;DR
This case suggests that marked cranial nerve hypertrophy may be clinically unapparent in some hereditary polyneuropathies and supports future correlation of temporal bone pathology with imaging and genotype in confirmed cases.
Abstract
We report an archival temporal bone case from the Otopathology Archives at Johns Hopkins of a man with progressive distal-predominant weakness, sensory loss, areflexia, and bilateral pes cavus, with clinicopathologic features suggestive of a chronic demyelinating peripheral neuropathy. Bilateral temporal bone histopathology demonstrated diffuse enlargement of the facial nerve throughout its intratemporal course, with additional enlargement of the glossopharyngeal and vagus nerves. This case suggests that marked cranial nerve hypertrophy may be clinically unapparent in some hereditary polyneuropathies and supports future correlation of temporal bone pathology with imaging and genotype in confirmed cases.
Electrophysiological studies revealed a generalized, asymmetric, multifocal demyelinating neuropathy with conduction blocks at atypical, non-compressive sites, consistent with multifocal acquired demyelinating sensory and motor neuropathy (MADSAM).
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