Klippel-Feil Syndrome and Associated Hearing Loss

Pterygium syndrome.

“…Deafness has been said to be the second most common anomaly associated with the syndrome (Palant and Carter, 1972;Stark and Borton, 1973;McLay and Maran, 1969). There is histological and x-ray evidence that the structure of the inner ear is abnormal (Palant and Carter, 1972).…”

Otological findings in cervico-oculo-auditory dysplasia

“…The defects are seen either unilaterally or bilaterally and are accompanied by deafness which may be perceptive, mixed or conductive in type. They are caused by the dysplasia of the labyrinth and/or of the internal auditory canal, or of the middle and outer ear (Terrahe, 1972;Stark and Borton, 1973). When the syndrome is combined with defects in the eyes, such as paralysis of the abductor nerve and retraction of the eyeball, it is then called the Wildervanck or Duane syndrome (Wildervanck etal, 1966).…”

“…In Stark and Borton's (1973) case, a patient with conductive hypakousia dectomy. Histological examination of the footplate showed no otosclerotic foci and this confirmed a congenital fixation of the footplate.…”

“…Franceschetti and Klein (1954) Everberge?«/. (1963) Wildervanck et al (1966) Singh et al (1969) McLay and Maran (1969) Stark and Borton (1973) Baumeister and Terrahe (1974) Jarvis and Sellars (1974) Daniilidise/o/. (1978) showed depletion of the left ossicle, dysplasia of the right incus, and depletion of the stapes footplate at exploratory tympanotomy.…”

Zervikal ausgelöste neurootologische Symptome des Klippel-Feil-Syndroms

“…Die Atiologie ist unklar, das weibliche und männliche Geschlecht scheinen g1eichermaen betroffen (43) Die Differentialdiagnose zu anderen Krankheiten gelingt aufgrund des typischen Rontgenbefundes der HWS und des vertebro-basilären Uberganges. Angiographisch lassen sich eventuell funktionelle GefãIanomalien der Arteriae vertebrales feststellen.…”