An Unusual Association: Arnold Chiari Deformity and Meniere's Disease

Abstract

Rare and fairly unknown, Arnold Chiari deformity is defined by the abnormally low position of the cerebellar tonsils that engage through the foramen magnum. Its association with the triad of tinnitus-hypoacousia-vertigo causes an authentic Meniere’s disease worth discussing. We report an unusual association of Arnold Chiari deformity with Meniere’s disease. A 46-years-old patient was diagnosed with left Meniere’s disease in 1994 on the classical diagnostic triad and the mode of progression: rotatory vertigo evolving by iterative crises; Intermittent buzzing tinnitus; left perception deafness with notion of wadded left ear. For a decade (1994 to 2015), he has been put under hygieno-dietary measures, vestibular re-education and medical treatment. The evolution was marked by the worsening of vertigo becoming progressively incapacitating, as well as an aggravation of the left deafness and persistence of tinnitus. Audiometry highlighted a severe endocochlear left perception deafness. The videonystagmography revealed a well-compensated left vestibular deficit. Cerebral magnetic resonance imaging (MRI) revealed a cerebellar tonsils’ ptosis through the foramen magnum. The diagnosis of Arnold-Chiari deformity associated with Meniere’s disease was then retained. The patient received a surgical left labyrinthectomy in 2015. The clinical course was uneventful, marked by the disappearance of vertigo twenty four months later. Association of Meniere’s disease and Arnold Chiari deformity is rare and must be diagnosed. These two pathologies being manifested by peripheral vertigo, a meticulous clinical and Para clinical examination is necessary to guide the diagnosis. Cerebrospinal fluid flow and pressure anomaly due to Arnold Chiari malformation can truly impact labyrinthine physiology, which explains the correlation between these two entities.