Very rare. X-linked transmission of a mutation of the POU3F-4 gene (Xq21.1.) 

Mixed or perceptive hearing loss present at birth or appearing in the first years of life.

Presence of a cochlear perilymphatic fistula (scan of the petrous portion of the temporal bone) with vestibular hypo- or areflexia.

Anesthetic implications: 

anesthesia of a hearing-impaired or deaf (cochlear implant) child.

Updated: April 2019