Vestibular dysfunction of patients with mutations of Connexin 26

Ingo Todt, Hans Christian Hennies, Dietmar Basta, Arne Ernst

Research output: Contribution to journalArticlepeer-review

32 Citations (Scopus)


The gap junctional network of the inner ear plays an important role in cochlear ionic homoeostasis. Mutations of connexin 26 can induce different types of hearing loss and even deafness. Therefore, it is hypothesized that gap junctions of the human vestibular organ are functionally impaired by mutations of connexin 26. In a prospective, nonrandomized study, the functional status of the semicircular canals and the otolith organs was assessed in one homozygous and six heterozygous carriers of connexin 26 mutations. Five out of seven patients (71.4%) had pathological vestibular evoked myogenic potentials, indicating a loss of saccular function. The utricular function (as tested by subjective haptic vertical) and the function of the semicircular canals (as tested by recording the vestibuloocular reflex) were largely normal. Thus, connexin 26 mutations can be associated with saccular defects of the vestibular receptors.

Original languageEnglish
Pages (from-to)1179-1181
Number of pages3
Issue number11
Publication statusPublished - 1 Aug 2005
Externally publishedYes


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