Stapes Gusher And Klippel‐Feil Syndrome

Abstract

The stapes gusher which is the most dramatic complication of stapedectomy arises from an abnormal communication between the subarchnoid and perilymphatic spaces. This congenital defect may be associated with other anomalies such as the Klippel‐Feil syndrome. Two cases of stapes gusher in patients with congenital fixation of the footplate are described. One of them was combined with Klippel‐Feil syndrome. The object of this paper is to emphasize the necessity of a complete preoperative examination of all cases of congenital footplate fixation.

Apert syndrome and hearing loss with ear anomalies: a case report and literature review

“…Congenital inner ear anomalies can occur with syndromic associations, such as Pfeiffer, Klippel-Feil, osteogenesis imperfecta, Crouzon, Treacher-Collins, branchio-otorenal and X-linked recessive progressive mixed deafness with perilymph gusher during stapes surgery [25]. Interestingly, stapes gusher in a retrospectively-diagnosed case of Klippel-Feil syndrome has been reported [26].…”

Audiological abnormalities in the Klippel-Feil syndrome

“…Although some have investigated cases further with radiological imaging,26 most cases have not been extensively investigated. Some cases with profound hearing impairment need further assessment to ascertain the feasibility of cochlear implantation or whether other modes of auditory rehabilitation such as surgical intervention27 28 can be undertaken.…”

Recurrent meningitis in children due to inner ear abnormalities

“…Recurrent meningitis has also been reported associated with brucellosis, sarcoidosis, Behc,et's disease and in Mollaret's meningitis; these conditions and others are reviewed by Hermans et al, (1972). When examining patients to exclude a CSF fistula associated with inner ear abnormality, a general examination is needed to exclude syndromes associated with severe inner ear deformity such as Klippel-Fiel, Pendred, Trisomy 21, De George and Alports (Richards and Gibbin, 1977;Danilidis et al, 1978;Schuknecht, 1980). Otoscopy may not prove to be useful; only one of our seven cases had an abnormal ear drum (Case 3), tympanometry confirmed fluid in the middle ear in this case and was useful in a further case (Case 4).…”