Congenital ossicular chain malformations with mobile stapes in children: Results in 17 cases

Vincent, Robert; Wegner, Inge; Derks, Laura S. M.; Grolman, Wilko

doi:10.1002/lary.25351

Cited by 23 publications

(26 citation statements)

References 22 publications

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“…The outcomes of surgery in patients with class I, class II, and class III congenital malformations were described previously . A postoperative ABG closure to 10 dB or less was achieved in 73% of class I cases, 50% of class II cases, and in 63% of class III cases.…”

Section: Discussionmentioning

confidence: 91%

“…The remaining 52 cases underwent surgery for class I, class II, and class III congenital malformations. The outcomes of surgery in these patients were described elsewhere …”

Section: Methodsmentioning

confidence: 99%

“…The outcomes of surgery in these patients were described elsewhere. 9,10 Study Population This is a nonrandomized, nonblinded, study of prospectively collected data of 14 children, under 18 years of age, who underwent 17 consecutive operations for class IV congenital middle ear malformations from 1993 to 2014. An international board review was not required for this study.…”

Section: Methodsmentioning

confidence: 99%

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Congenital oval or round window malformations in children: Surgical findings and results in 17 cases

et al. 2016

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Section: Discussionmentioning

confidence: 91%

“…The remaining 52 cases underwent surgery for class I, class II, and class III congenital malformations. The outcomes of surgery in these patients were described elsewhere …”

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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Congenital oval or round window malformations in children: Surgical findings and results in 17 cases

et al. 2016

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“…Further comparison was made between these cases and those of ossicular malformation without CCs. Since Teunissen and Cremers [20] had published a large case series of 144 congenital ossicular anomalies in the early 1990s, a few subsequent studies provided dozens of additional cases to allow for a better understanding of this congenital defect [2,[21][22][23][24]. Based on the literature, the most common ossicular anomalies of discontinuity were absent or underdeveloped long process of the incus and the superstructure of the stapes.…”

Section: Discussionmentioning

confidence: 99%

Concurrent Occurrence of Congenital Ossicular Anomaly and Localized Cholesteatoma: Series of 10 Cases

Zhao

Chen

Zhang

et al. 2020

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Objective: The objective of this study is to describe the clinical features, managements and outcomes of a rare coexistence of congenital ossicular anomaly and localized cholesteatoma. A literature review on these cases and each congenital disorder is also presented. Methods: A retrospective chart review was performed on patients diagnosed with congenital ossicular anomaly with concurrent localized cholesteatoma from 2008 to 2017. Clinical data of these patients were collected. Results: A total of 10 patients were identified. All patients presented with unilateral hearing loss. Pure-tone audiometry showed conductive hearing loss in all affected ears with an average air conduction (AC) threshold of 59 dB. High-resolution computed tomography scans of the temporal bone diagnosed ossicular anomaly for 90% (9/10); however, only 50% (5/10) had a diagnosis of localized cholesteatoma. A transcanal exploratory tympanotomy under the microscope was performed to discover whether the localized tinysized cholesteatoma around the ossicular chain did not have direct contact with the ossicular chain, which could be diagnosed as congenital cholesteatoma. We removed the localized cholesteatoma and reconstructed the ossicular chain in each patient. All localized cholesteatomas were found in the posterior-superior quadrant of the middle ear. Ossicular chain anom-alies were associated with the incus and/or the stapes in all cases. Hearing improvement was achieved in each of the 6 patients who were followed up postoperatively, with an average AC threshold of 35 dB. The clinical features of congenital ossicular anomaly with concurrent congenital cholesteatoma were compared with those of each congenital disorder. The pathogenesis of each condition was also discussed. Conclusions: Congenital ossicular anomaly with concurrent congenital cholesteatoma is rare. It shares similar clinical features with congenital ossicular anomaly occurring alone, therefore awareness should be raised for a possible concurrent congenital cholesteatoma which was easy to miss in the diagnosis (50%) by the radiologist. A patient's hearing level can be improved by removal of the cholesteatoma and reconstruction of the ossicular chain. Localized cholesteatoma does not usually show residuals or recurrence.

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“…Vincent et al published a case series and literature review analyzing audiometric results following surgical treatment of Teunissen and Cremers Class III patients (19). They reported an postoperative air-bone gap closure tot 10 dB HL or less in 63%, and an postoperative air-bone gap closure to 20 dB or less in 75% (19). In our patient, although her hearing improved mainly in the low frequencies, the air-bone gap postoperatively was 25 dB HL, averaged over frequencies 0.5-4 kHz.…”

Section: Discussionmentioning

confidence: 99%

Case Report: Challenging Otologic Surgery in Patients With 22q11.2 Deletion Syndrome

et al. 2020

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Patients with 22q11.2 deletion syndrome frequently have conductive hearing loss and/or chronic otitis media. Otologic surgery is often opted for. We present two patients undergoing otologic surgery. This case report outlines the typical otologic surgical challenges in patients with 22q11.2 deletion syndrome. Case one is a 52 year old male patient with chronic otitis media who underwent a mastoidectomy. The pre-operative CT scan showed a fused lateral semicircular canal and vestibule. Peroperatively, the lateral semicircular canal could not be used as a landmark to identify the facial nerve. Case two is a 10 year old female patient with conductive hearing loss. A middle ear inspection was performed where a bony epitympanic fixation of the malleus was encountered. In addition, the manubrium of the malleus was atrophic and also fixated. The bony fixation was removed, as was the manubrium of the malleus. Otologists should be aware of these typical anatomical variations in patients with 22q11.2 deletion syndrome. We recommend to use CT scanning of the middle and inner ear when preparing for otologic surgery in 22q11.2 deletion syndrome.

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Congenital ossicular chain malformations with mobile stapes in children: Results in 17 cases

Abstract: 4. Laryngoscope, 126:682-688, 2016.

Cited by 23 publications

References 22 publications

Congenital oval or round window malformations in children: Surgical findings and results in 17 cases

Congenital oval or round window malformations in children: Surgical findings and results in 17 cases

Concurrent Occurrence of Congenital Ossicular Anomaly and Localized Cholesteatoma: Series of 10 Cases

Case Report: Challenging Otologic Surgery in Patients With 22q11.2 Deletion Syndrome

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