Congenital defects of the middle ear - uncommon cause of pediatric hearing loss

Esteves, Sara Sena; Silva, Ana Pereira da; Coutinho, Maria Francisca; Abrunhosa, José; Sousa, Cecília Almeida e

doi:10.1016/j.bjorl.2013.10.002

Cited by 16 publications

(19 citation statements)

References 19 publications

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“…However, accurately diagnosing unilateral isolated CHL cases requires effort and is more time consuming, and diagnosis and treatment are often delayed, leading to impaired language development and learning. 2,11 In our study, the mean age of the patients was 19 years, and the time from discovery to final diagnosis and surgery was 8 years, similar to the value of 6.4 years reported in another study. 12 These data indicate that preoperative recognition of the congenital nature of CHL can be challenging.…”

Section: Discussionsupporting

confidence: 89%

“…Furthermore, the facial nerve is a second branchial arch derivative, and can be malformed or be found in an aberrant course. 11,22 As stated previously, by comparing preoperative HRCT with surgical findings, we were able to determine the following characteristics of our cases: (1) Stapes anomaly with or without incus anomaly were the most frequent anomalies, and comprised 79.3% of the cases. (2) Circumstances that are difficult to identify in preoperative HRCT (negative findings) include the fixation of the ossicular chain.…”

Section: Discussionmentioning

confidence: 91%

“…Thus, the long process of the incus and the stapes’ superstructure have the same embryological origin, so that the two groups of malformations may exist independently of each other. 3,11,21 Understanding the embryological processes is essential for understanding the pattern of CMEM. Malformation of the long process of the incus and the superstructure of the stapes often occur together.…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, the facial nerve is a second branchial arch derivative, and can be malformed or be found in an aberrant course. 11,22…”

Section: Discussionmentioning

confidence: 99%

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Isolated Congenital Middle Ear Malformations: Comparison of Preoperative High-Resolution CT and Surgical Findings

Zhang

et al. 2019

Ann Otol Rhinol Laryngol

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Objectives: To compare preoperative temporal bone high-resolution computed tomography (HRCT) readings to intraoperative findings during exploratory tympanotomy for suspected cases of isolated congenital middle ear malformations (CMEMs) and summarize the malformations that can and cannot be diagnosed with HRCT. Methods: A retrospective study was conducted. All cases were confirmed as isolated CMEMs during surgery. Detailed clinical records were reviewed, with a focus on imaging and surgical findings. Results: One hundred and thirty-two patients and 145 ears were reviewed. Ninety cases (62.1%) could be identified as isolated CMEMs and at least one as middle ear anomaly using preoperative HRCT. Fifty-five cases (37.9%) were reported to be completely normal and the patients underwent exploratory tympanotomy to determine the final diagnosis. Stapes fixation, either alone or associated with other ossicular chain anomalies, contributed to 53.1% of the cases. Most cases of aplasia or dysplasia of the ossicular chain, for example, aplasia/dysplasia of the long process of the incus, aplasia of the stapes’ superstructure, and atresia of the oval window were easily identified in preoperative HRCT. However, fixation of the ossicular chain can be elusive in HRCT, and exploratory tympanotomy is needed for a definitive diagnosis. Conclusions: HRCT provides helpful preoperative clinical information in CMEM and may obviate the need for middle ear exploration in some cases. The negative findings (anomalies that are difficult to identify through preoperative HRCT) and the positive findings (anomalies that are relatively easy to identify through preoperative HRCT) were summarized.

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Section: Discussionsupporting

confidence: 89%

Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 99%

“…Furthermore, the facial nerve is a second branchial arch derivative, and can be malformed or be found in an aberrant course. 11,22…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Isolated Congenital Middle Ear Malformations: Comparison of Preoperative High-Resolution CT and Surgical Findings

Zhang

et al. 2019

Ann Otol Rhinol Laryngol

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“…However, for class IV anomalies, surgical treatment such as vestibulotomy with piston insertion, presents a high risk of facial nerve injury and inner ear damage. Several authors have reported the clinical features and surgical outcomes of patients with minor ear anomalies as determined using the Teunissen and Cremers classification 10–13 ; however, the number of patients was limited in these studies and the breakdown of classification and hearing outcomes differed among the studies.…”

Section: Introductionmentioning

confidence: 99%

Multicenter Study of Congenital Middle Ear Anomalies. Report on 246 Ears

et al. 2021

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Objectives/Hypothesis Congenital middle ear anomalies represent a relatively rare condition. This study aimed to describe the characteristics and the surgical outcomes for patients with middle ear anomalies. Methods A multicenter study was conducted of consecutive patients with congenital middle ear anomalies who underwent primary surgical treatment between January 2008 and December 2017. Demographics, surgical procedures, and audiometric data were registered into the institutional database. Hearing changes and postoperative air‐bone gap (ABG) were evaluated 1 year after surgery. Results A total of 246 patients (246 ears) (median age: 14 years, range: 4–75 years old) were included in this study. Anomalies were subdivided using the Teunissen and Cremers classification: 53 ears (22%) were categorized as class I, comprising only stapes ankylosis; 35 ears (14%) as class II, having ossicular chain anomalies with stapes ankylosis; 139 ears (57%) as class III, having ossicular chain anomalies with a mobile stapes‐footplate; and 19 ears (8%) as class IV, with aplasia of the oval window. Evaluation of hearing outcomes for 198 ears with more than 1 year of follow‐up revealed that good postoperative ABG (≤20 dB) was achieved in 82% of class I, 68% of class II, 74% of class III, and 23% of class IV anomalies. The postoperative ABG in class IV was significantly worse than in class I (P < .001) or class III (P < .01). Conclusions This study demonstrated that class III anomalies comprised the majority of middle ear anomalies and surgical outcomes for class IV anomalies are unfavorable. Level of Evidence 4 Laryngoscope, 131:E2323–E2328, 2021

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