Congenital Fixation of the Head of the Stapes in Three Family Members

Abstract

We conducted a retrospective chart review at a university hospital on a family that exhibited a rare cause of conductive hearing loss. Four male patients of this family had congenital conductive hearing loss. Th ree of these family members underwent bilateral ossiculoplasty, a father and two of his three sons. All three were found to have mild or moderate conductive hearing loss due to fi xation of the head of the stapes by a bony bar that extended from the head of the stapes to the posterior ear canal wall. Surgical removal of the bony bar improved hearing in all cases. Th is family exhibits autosomal-dominant inheritance of this abnormality. Fixation of the head of the stapes by a bony bar that extends to the posterior ear canal wall is a rare cause of congenital, autosomal-dominant, mild or moderate conductive hearing loss that can be surgically corrected.

Congenital stapes suprastructure fixation presenting with fluctuating auditory symptoms: A case report

“…A previous study reviewed 22 cases of congenital middle ear minor malformation caused by stapes suprastructure fixation and subdivided the cases into the elongation of pyramidal eminence group, stapes-facial canal fixation group, and stapes-promontory fixation group[ 2 ]. Elongation of the pyramidal eminence or ossification of the stapedial tendon could occur due to an acquired reason, such as infection or inflammation, but could also be caused by congenital, autosomal-dominant or autosomal-recessive inheritance[ 10 - 12 ]. However, most previous reports have shown conductive hearing loss without past trauma or otitis media history, and the patients did not complain of any symptoms other than hearing discomfort.…”

A meta‐analysis of surgical success rates in Congenital stapes fixation and juvenile otosclerosis

“…Thus, six single‐case reports were included in this report along with larger sample reports . In addition to single‐case reports, there were also eight studies with the total number of cases less than 10 …”

“…1,[16][17][18][19][20] In addition to single-case reports, there were also eight studies with the total number of cases less than 10. 3,7,[21][22][23][24][25][26] Looking at the meta-analysis graph, we can see that small squares for each study are shown in different sizes depending on the sample size. Studies that reported more successful surgical results appear in the graph to be leaning to the right, whereas unsuccessful ones appear to be leaning to the left.…”

Stapes-Pyramidal Fixation by a Bony Bar

“…5 In contrast, Wetmore and Gross reported an autosomal dominant hereditary conductive hearing loss in 3 members of a family that resulted from fixation of the head of the stapes by a bony bar that extended to the posterior ear canal. 6 Thin-section CT has been postulated to be valuable in facilitating the preoperative diagnosis of the ossification of stapedius tendon, characterized by a linear area of soft-tissue attenuation extending from the pyramidal eminence to the stapes or a bony bar adjacent to it. 2 Surgical treatment can be performed by the simple division of the ossified stapedius tendon, or by stapedotomy/stapedectomy in patients with associated fixation of the stapes footplate.…”