Ataxia secondary to labyrinthine fistula

Abstract In patients presenting with severe gait disturbance, the otolaryngologist is not likely to consider labyrinthine disease as a possible cause. During the past few years there have been several reports of sudden deafness secondary to mechanical rupture of the inner ear windows. The clinical picture has usually been one of sudden deafness with tinnitus and vertigo. Mild ataxia was noted in three out of 18 reported cases. A case is presented of a 59‐year‐old female with a two month history of severe ataxia. Seven years previously she had had head trauma without fracture. Examination revealed marked ataxia, positive Rhomberg, positional vertigo and nystagmus and a 45 db mixed hearing loss. Tympanotomy revealed subluxation of the stapes with a fistula at the antero‐inferior margin of the footplate. Stapedectomy and soft tissue closure of the oval window were carried out. Postoperatively, the patient had no further ataxia or vertigo, and her hearing remained unchanged for 18 months. Three of the 18 reported cases also had given a history of head trauma with or without skull fracture, and two patients had had positional vertigo. The explanation of the positional vertigo in a case of labyrinthine fistula is not evident; perhaps gravity in the affected ear down position facilitates a flow of perilymph through the fistula with resulting distortion and stimulation of the cristae. Conclusion. In the future, peripheral labyrinthine disease such as a fistula should be suspected in cases which present with ataxia and episodic vertigo of obscure etiology. A tympanotomy may be necessary to establish the diagnosis and to allow correction of the pathology.