Agenesia de la supraestructura del estribo

Domínguez, Felipe Ordoño; Merlos, N. Mínguez; Paule, Pilar Navarro; Devis, I. Albaladejo; Mármol, M. Pintado; Rodríguez, Luis

doi:10.1016/s0001-6519(05)78654-4

Cited by 5 publications

(3 citation statements)

References 8 publications

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“…A flat pure-tone audiogram in conversational frequencies, with preserved bone conduction, is also a typical finding. The presence of Carhart's notch in patients with CAS has been described but is not pathognomonic of this condition nor can it always be found, as in our cases [10]. In those patients suspected to suffer from congenital middle ear malformations, high resolution 1 mm section thickness temporal bone CT scan is indicated [2].…”

Section: Discussionmentioning

confidence: 77%

“…It has been suggested that the malformation or agenesis of the stapes and oval window is related to the abnormal development of the facial nerve [4, 7, 8]. Regarding the embryologic origin, the malleus head and the incus body differentiate from the fist branchial arch while the malleus handle, long process of the incus, and the stapes develop from the second branchial arch or Reichert's cartilage [1–4, 6, 9, 10]. By the 5th to 6th week of gestation the mass of the stapes becomes recognizable and begins its growth towards the otic capsule [6].…”

Section: Discussionmentioning

confidence: 99%

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Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings

Undabeitia

Cianci

Padilla

et al. 2014

Case Reports in Otolaryngology

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Middle ear ossicle malformations are an uncommon event. Among them, the congenital absence of the stapes is a very rare condition that is seldom described in the literature. We report the cases of two women, aged 19 and 22 , who presented with a long history of conductive deafness. An exploratory tympanotomy was performed and the absence of the stapes superstructure and an abnormal position of the facial nerve could be observed. A bone anchored hearing aid (BAHA) was implanted in both patients with good results. It is believed that stapes agenesis is related to an abnormal development of the facial nerve, which by the 5th to 6th week of gestation would interpose between the otic capsule and the stapes blastema, preventing these structures from contacting. A long history of nonprogressive hearing loss from birth or early childhood is the key to reach a diagnosis. Several treatment options have been described. The authors opted for a hearing aid due to the high risk of facial nerve lesion, with good functional results.

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Section: Discussionmentioning

confidence: 77%

Section: Discussionmentioning

confidence: 99%

Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings

Undabeitia

Cianci

Padilla

et al. 2014

Case Reports in Otolaryngology

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“…Isolated defects of the stapes suprastructure (the combination of the capitulum, neck, and crura) in an otherwise intact ossicular chain is a rare condition resulting in conductive hearing loss. This can occur because of congenital dysplasia, traumatic fractures of the stapes crura, or localized destruction due to chronic middle ear infection (5,6). For cases of isolated malfunction or absence of the stapes suprastructure, there is currently no standard therapy.…”

Section: Introductionmentioning

confidence: 99%

The TORP-PORP: A Tympanoplasty Technique for Isolated Defects of the Stapes Suprastructure

Esser,

Klussmann,

Hüttenbrink

et al. 2024

Otol Neurotol

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Objective Investigating the outcomes of a surgical approach to treat isolated defects of the stapes suprastructure, using a modified total ossicular replacement prosthesis (TORP) prosthesis as a PORP between the footplate and the incus, effectively creating a TORP-PORP configuration. Patients Eleven patients (mean age, 37.2 years; 36% male and 64% female) between the years 2007 and 2022. Intervention(s) Therapeutic (ossiculoplasty). Main Outcome Measure(s) Hearing gain (in dB) in air conduction thresholds at 0.5, 1, 2, 3, and 4 kHz, stability of bone conduction, revision rate. Results Significant improvement in air conduction between the preoperative and the postoperative cohorts (p = 0.002) with a mean postoperative hearing level of 30.00 ± 5.25 dB. The bone conduction remained stable. We encountered no perioperative complications, and there were no revisions surgery. Conclusions The described ossiculoplasty procedure is a safe and effective approach to treat isolated defects of the stapes suprastructure.

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Bilateral stapes agenesis: What can we find?

Bergeron

Côté

2017

Acta Oto-Laryngologica Case Reports

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We describe the youngest case of bilateral stapes agenesis and systematically reviewed the literature. Among those cases, 8/12 (66.7%) showed a bilateral agenesis. There is a male predominance 8/12 (66.7%). The most common associated anomaly reported being either a dehiscent or displaced facial nerve (7/12-58%). Furthermore, 5/12 patients (42%) had a narrow or an absent oval window. In our case, a 5-year-old male patient was referred for bilateral conductive hearing loss (air-bone gap 65 dB, bilateral). Operating findings on the left side included an inferiorly displaced facial nerve over the promontory, a narrow ossified oval window and an absent stapes. A stapedotomy with insertion of a modified stapes prosthesis allowed us to close the conductive gap. Even with possible good outcomes, surgical management should not be taken lightly, considering amplification is almost always a safe and effective option.

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Agenesia de la supraestructura del estribo

Cited by 5 publications

References 8 publications

Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings

Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings

The TORP-PORP: A Tympanoplasty Technique for Isolated Defects of the Stapes Suprastructure

Bilateral stapes agenesis: What can we find?

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