ABSTRACT
OBJECTIVE: Results of stapedectomy are reported in a Belgian 26-year-old woman and two Dutch brothers having the proximal symphalangism syndrome (McKusick 18580). STUDY DESIGN: Case reports are presented. A review of the results of ear surgery for congenital conductive hearing loss in this syndrome is given. SETTING: The Belgian patient was treated in a general hospital. The Dutch patients were treated in a university hospital, which was a tertiary referral center. PATIENTS: Patients were referred to have an evaluation of their hearing impairment. INTERVENTION: Based on the syndromal diagnosis and based on routine audiometric tests, a congenital ossicular fixation was considered to be the cause of the hearing loss. By exploratory tympanotomies, this was confirmed. Reconstructive procedures including stapedotomy were performed. MAIN OUTCOME MEASURES/RESULTS: Long-term audiometric data are presented to evaluate the outcome of the surgical interventions. CONCLUSIONS: Congenital stapes ankylosis eventually combined with a congenital fixation of the short process of the incus in the fossa incudis, causing the congenital conductive hearing loss. Surgical intervention is very successful in most reported cases, but negative side effects are incidentally found as well.
Subject(s)
Ankylosis/complications , Ankylosis/genetics , Finger Joint/abnormalities , Hearing Loss, Conductive/congenital , Hearing Loss, Conductive/etiology , Stapes/abnormalities , Adult , Animals , Audiometry, Pure-Tone , Blepharophimosis/complications , Female , Hearing Loss, Conductive/diagnosis , Humans , Postoperative Care , Preoperative Care , Stapes SurgeryABSTRACT
The presence of mast cells in the subepithelial connective tissue of the human endolymphatic sac has, to the best of our knowledge for the first time, been described. A hypothesis has been put forward in which these cells, which are known to contain histamin, heparin and serotonin, play an important role in the physiologic functions of the endolymphatic sac as well as in some pathologic states of the inner ear.
Subject(s)
Ear, Inner/cytology , Mast Cells , Animals , Connective Tissue Cells , Guinea Pigs , Heparin/analysis , Histamine/analysis , Humans , Mast Cells/analysis , Serotonin/analysisABSTRACT
The Schirmer test enables us to localise a lesion of the facial nerve as situated proximally or distally to the geniculate ganglion. Three cases of traumatic facial paralysis were surgically treated. One by transmastoidal decompression, two others by subtemporal suture or nerve graft. Partial to subtotal reinnervation took place in all three cases. The correct choice of transmastoidal or subtemporal approach was made according to the results of the Schirmer test, in the absence of X ray information.
