Familial clustering of migraine, episodic vertigo, and Ménière's disease

Abstract

Objective: To evaluate the association between migraine, episodic vertigo, and Ménière's disease in families.

Study design: Clinical report.

Setting: University Neurotology Clinic.

Patients: Index patients identified with Ménière's disease and migraine and their family members.

Intervention: Structured interview to assess a diagnosis of migraine, episodic vertigo, and Ménière's disease in 6 families. Genotyping was performed on 3 sets of twins to analyze monozygosity or dizygosity.

Main outcome measures: Clinical history of migraine, episodic vertigo, and Ménière's disease.

Results: Six index patients and 57 family members were interviewed either by a senior neurologist in person or over the phone by a trained study coordinator. An additional 6 family members completed questionnaires by mail. All 6 index patients had Ménière's disease and migraine. Twenty-six (41%) of the 63 relatives met International Classification of Headache Disorders II criteria for migraine headaches. Thirteen (50%) of these 26 experienced migraine with aura. Three others experienced typical aura without headache. Seventeen (27%) of 63 family members experienced recurrent spells of spontaneous episodic vertigo. There was one twin pair in each of 3 families; 2 pairs were monozygotic and one was dizygotic. In each twin pair, one twin had migraine and Ménière's disease, whereas the other experienced migraine and episodic vertigo without auditory symptoms.

Conclusion: The frequent association of episodic vertigo, migraine, and Ménière's disease in closely related individuals, including identical twins supports the heritability of a migraine-Ménière's syndrome, with variable expression of the individual features of hearing loss, episodic vertigo, and migraine headaches.

FIG. 1

Diagram showing pedigrees of families with migraine, episodic vertigo, and Ménière’s disease. Female family members are represented by circles; male family members by squares. Index patients are indicated with an arrowhead. Affected status is indicated by shading in of quadrants according to the following scheme: upper left, migraine; upper right, aura (visual or somatosensory); lower left, hearing loss; lower right, episodic vertigo. Numbers within the circles or squares indicate the number of unaffected individuals in the sibship. TLE indicates temporal lobe epilepsy.