Hearing loss in the cervico-oculo-acoustic (Wildervanck) syndrome

A 22-year-old woman with mixed hearing loss, Klippel-Feil anomalad (fused cervical vertebrae), and bilateral abducens palsy with retracted bulb (Duane's syndrome) is classified as having the cervico-oculo-acoustic syndrome or Wildervanck syndrome. In this syndrome, the unilateral or bilateral congenital hearing loss is usually severe and of neurosensory origin. To our knowledge, this is the third case of this syndrome with the classical triad and a mixed hearing loss. It is the second case in which an exploratory tympanotomy was performed. An ankylosis of the stapes was found. Stapedectomy and Teflon interposition were successfully carried out. Since the triad of the cervico-oculo-acoustic syndrome can be incomplete, a review of the association of Klippel-Feil anomalad and hearing loss is also presented, with special attention to the anomalies of the ossicular chain.