Benign paroxysmal positional vertigo with congenital nystagmus: A case report

Benign paroxysmal positional vertigo (BPPV) is a form of temporary vertigo induced by moving the head to a specific position. It is a self-limited, peripheral, vestibular disease and can be divided into primary and secondary forms. Congenital nystagmus (CN), an involuntary, rhythmic, binocular-symmetry, conjugated eye movement, is found at birth or within 3 mo of birth. According to the pathogenesis, CN can be divided into sensory-defect nystagmus and motor-defect nystagmus. The coexistence of BPPV and CN is rarely seen in the clinic.

A 62-year-old woman presented to our clinic complaining of a 15-d history of recurrent positional vertigo. The vertigo lasting less than 1 min occurred when she turned over, sometimes accompanied by nausea and vomiting. Both the patient and her father had CN. Her spontaneous nystagmus was horizontal to right; however, the gaze test revealed variable horizontal nystagmus with the same degree when the eyes moved. The patient's Dix-Hallpike test was normal, except for persistent nystagmus, and the roll test showed severe variable horizontal nystagmus, which lasted for about 20 s in the same direction as her head movement to the right and left, although the right-side nystagmus was stronger than the left-side. Since these symptoms were accompanied by nausea, she was diagnosed with BPPV with CN and treated by manual reduction.

Though rare, if BPPV with CN is correctly identified and diagnosed, reduction treatment is comparably effective to other vertigo types.