Familial Isolated Stapes-Pyramidal Fixation by a Bony Bar With Normal Stapedius Tendon

Doi, Tadashi; Nakazawa, Hiroko; Adachi, Mari; Kaneko, Toshihiko; Munemoto, Yumi; Komeda, Mototane; Ikeda, Hiroki; Kitajiri, Masanori; Yamashita, Toshio

doi:10.1001/archotol.131.4.349

Cited by 9 publications

(4 citation statements)

References 5 publications

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“…Doi et al described another Japanese family spanning three generations that exhibited conductive hearing loss. 11 Th ree of their patients underwent bilateral ossiculoplasty with fi ndings identical to those of Hara's patients, with a bony bar extending from the pyramidal eminence to the head of the stapes with a monopod stapes in each case. In conclusion, we have described a father and two sons with congenital autosomal-dominant inheritance of an anomaly that presented with a mild to moderate, bilateral conductive hearing loss.…”

Section: Discussionmentioning

confidence: 95%

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

Wetmore

Gross

2011

Ear Nose Throat J

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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.

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Section: Discussionmentioning

confidence: 95%

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

Wetmore

Gross

2011

Ear Nose Throat J

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“…In both cases, the footplate of the stapes was mobile. Other rare stapes anomalies found in the literature included a case of elongated pyramidal eminence causing adhesion of the ossicle [ 21 ] and a familial stapes fixation to the medial wall [ 22 , 23 ].…”

Section: Discussionmentioning

confidence: 99%

Stapedotomy in Congenital Stapes Ankylosis with Mobile Footplate: A Case Report

Porowski

Skarżyński

2022

Am J Case Rep

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Patient: Female, 15-year-old Final Diagnosis: Fixation of the middle ear ossicles Symptoms: Conductive hearing loss of the right ear of up to 40 dB • tomography confirmed the presence of the middle ear ossicles Medication: — Clinical Procedure: — Specialty: Otolaryngology Objective: Congenital defects/diseases Background: This paper presents a case of a 15-year-old child with a rare congenital anomaly of the middle ear in which the stapes was fused to the medial wall of the tympanic cavity. Case Report: This defect coexisted with partial malleus fixation in the attic and caused conductive hearing loss at an average level of 35-40 dB. Two exploratory tympanotomies were performed, where excess bone between the stapes and promontory was removed and the head of the malleus was released in the attic. The good effect of these procedures was unstable, mainly due to re-attachment of the stapes to the medial wall of the tympanic cavity. At the next surgery it was decided to perform stapedotomy, despite the mobile stapes footplate. The operation was performed with a small-fenestra stapedotomy technique. Perforation of the footplate was done using a microdrill with a balanced speed. A KURZ prosthesis with a diameter of 0.5 mm was used. The postoperative period passed without any complications. Three and 6 months after the surgery, control pure tone audiometry was performed and showed significant improvement in hearing thresholds. During the follow-up period of more than 6 months, hearing improvement remained stable. Conclusions: We concluded that it was safe to perform stapedotomy in the presence of a mobile stapes footplate when congenital anomaly of the stapes superstructure caused its severe fixation in the middle ear. In our opinion, in a child with congenital ear anomaly, consideration should be given to the multifocal origin of the hearing loss.

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“…3 To date, several reports have described fixation of the stapes superstructure by a bony bar or ossified stapes tendon. 4,5 Accurate intraoperative identification of the pathology in cases of conductive hearing loss is critical because excision or ablation of a stapedial bar will restore hearing and obviate the need for stapedotomy. This study was exempted from Institutional Review Board approval.…”

Section: Discussionmentioning

confidence: 99%

Stapes bar

Hertzano¹,

Eisenman²

2010

Otolaryngol.--head neck surg.

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A 13-year-old male presented with a three-year history of left-sided hearing loss with no other otologic symptoms or family history of hearing loss. Binocular microotoscopic examination was normal. The audiogram demonstrated a left moderate, rising to mild conductive hearing loss, with a pure tone average of 10 dBHL on the right and 45 dBHL on the left for air conduction, and with air-bone gaps ranging from 15 to 40 decibels. Word recognition was 100 percent bilaterally, and tympanograms were type A. Computerized tomography scan of the temporal bones revealed no temporal bone anomalies. Left middle ear exploration was performed for a presumptive diagnosis of juvenile otosclerosis. Intraoperatively, an osseous bar was identified extending from the pyramidalis process to the stapes capitulum, running superior and parallel to an intact stapes tendon (Fig 1A). The bar was vaporized with an argon laser (Fig 1B), restoring stapes mobility. A six-week postoperative audiogram demonstrated closure of the airbone gap through 3000 Hz, with a left ear pure tone average of 13 dBHL.

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Familial Isolated Stapes-Pyramidal Fixation by a Bony Bar With Normal Stapedius Tendon

Cited by 9 publications

References 5 publications

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

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

Stapedotomy in Congenital Stapes Ankylosis with Mobile Footplate: A Case Report

Stapes bar

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