Autoimmune hearing loss in chronic inflammatory demyelinating polyneuropathy

Abstract

Asymptomatic cranial nerve involvement, most commonly in oculomotor and facial nerves, can be seen in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). It is not always possible to delineate whether cranial nerve abnormalities are related to brainstem or extra axial involvement.1 However, disabling vestibular symptoms on one occasion2 and auditory symptoms on another occasion3 were described and attributed to peripheral nerve demyelination. We describe a patient with CIDP who developed sensorineural hearing loss suggestive of autoimmune inner ear disease (AIED). A 42-year-old previously healthy woman reported distal sensory symptoms in her feet and hands with distal weakness that began 1 year earlier. She also described difficulty hearing with the right ear from onset, which developed suddenly, without tinnitus. She had moderate intrinsic hand muscle, ankle, and toe dorsiflexion weakness. She was areflexic with only vibration deficits up to the ankles. The nerve conduction studies confirmed a demyelinating polyneuropathy with …