Chronic inflammatory demyelinating polyneuropathy (CIDP) has occasiona
lly been associated with clinical or laboratory evidence (magnetic res
onance imaging, [MRI], visual evoked response, and brainstem auditory
evoked response [BAER]) of cranial neuropathy. In most cases, the rela
tionship of cranial nerve involvement to CIDP remains unclear. A 45-ye
ar-old woman noted foot numbness, limb weakness, gait and postural ins
tability, and oscillopsia. An IgG kappa monoclonal gammopathy of undet
ermined significance was found. Bilateral vestibulopathy was documente
d by clinical examination, bithermal calorics, rotary chair testing, B
AERs, and dynamic posturography. MRI with gadolinium demonstrated enha
ncement of cranial nerve VIII bilaterally. Over the next 6 years, the
patient's relapsing and remitting course of CIDP and vestibulopathy wa
s assessed by quantitative muscle and vestibular function testing (cli
nically and neurophysiologically), and dynamic visual acuity. There wa
s a striking synchronization between her CIDP and vestibulopathy with
respect to clinical course including relapses and responses to immune
therapy. The response to therapy, and evidence derived from clinical a
nd laboratory investigations, suggest that the vestibular dysfunction
was immune mediated.