Wells Jankovic syndrome

Overview

Wells Jankovic syndrome: A syndrome that is characterised with spastic paraparesis and deafness

Symptoms

Spastic paraparesis Rigidity Deafness Visual difficulties Abnormal gait

Causes

Infection Trauma Drugs Perilymph fistula Neoplasm Ménière disease

Diagnosis

Audiometry and caloric testing or electronystagmography should be done in almost all cases in which the ear examination is normal. It is probably wise to consult an otolaryngologist at this point. Tympanography will be helpful in diagnosing subtle cases of serous otitis media. X-rays of the mastoids, petrous bones, and internal auditory canal should be done for chronic otitis media, cholesteatoma, and acoustic neuroma. If an acoustic neuroma is suspected, however, an MRI of the brain and auditory canals must be done. If basilar artery insufficiency is suspected, four-vessel cerebral angiography should be done. Magnetic resonance angiography is an excellent noninvasive alternative for diagnosing vertebral-basilar artery disease. If multiple sclerosis is suspected, MRI of the brain, BSEP and VEP studies, and a spinal tap for spinal fluid analysis may be done. Rather than perform these tests, the most cost-effective approach would be to refer the patient to a neurologist if other focal neurologic findings are evident