Acquired Ear Canal Cholesteatoma in Congenital Aural Atresia/Stenosis

Casale, Garrett G.; Nicholas, Brian D.; Kesser, Bradley W.

doi:10.1097/mao.0000000000000495

“…Previous studies analysing the clinical features of CAS involved small sample sizes, and no studies focused on the stability of the hearing outcomes567. This study uses the largest sample of CAS, providing guidance in consultation and emphasizing important aspects for long-term follow-up.…”

mentioning

confidence: 99%

“…Patients with stenosis sizes of 2 mm or less are at high risk for developing cholesteatoma and should undergo surgery; however, the data in the present study challenged this viewpoint. To date, there are no large sample studies focusing on the clinical features and long-term outcomes of CAS, and no studies have described the important parameter of measuring the diameter of EAC 5 6 7 8 9 10 11 .…”

mentioning

confidence: 99%

“…The aim of the present study was to comprehensively evaluate the clinical features and long-term outcomes of CAS, and challenge the previous viewpoint. Previous studies analysing the clinical features of CAS involved small sample sizes, and no studies focused on the stability of the hearing outcomes 5 6 7 . This study uses the largest sample of CAS, providing guidance in consultation and emphasizing important aspects for long-term follow-up.…”

mentioning

confidence: 99%

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Congenital Aural Stenosis: Clinical Features and Long-term Outcomes

Li

¹

,

Ying

²

,

Chen

³

et al. 2016

Sci Rep

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The aim of the present study was to comprehensively evaluate the clinical features and long-term outcomes of congenital aural stenosis (CAS). This study presents a retrospective review of patients who underwent meatoplasty for CAS at a tertiary referral hospital from 2008 to 2015. A total of 246 meatoplasty procedures were performed on 232 patients in the present study. We performed multivariate regression analysis. Except in the age < 6 years group, no significant difference was observed among different age groups for cholesteatoma formation, p > 0.05. Except for the stenosis of the external auditory canal (EAC) (>4 mm) group, the other stenosis of EAC groups were not associated with cholesteatoma formation, p > 0.05. Postoperative air-bone gaps (ABG) less than 30 dB occurred in 77.3% (99/128) of the patients, and the Jahrsdoerfer score was associated with postoperative ABG, p < 0.001. The complication rate of CAS was 13.8% (20/144), and males showed a higher risk for postoperative complications (OR, 6.563; 95% CI, 1.268–33.966, p = 0.025). These results indicate that meatoplasty was an effective surgical intervention for CAS, showing a stable hearing outcome with prolonged follow-up. There was no significant difference between the cholesteatoma and no cholesteatoma groups for hearing outcomes, p > 0.05.

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“…A previous study by Cole and Jahrsdoerfer 17 reported external auditory canal cholesteatoma in 48 per cent of ears with congenital aural stenosis. A study by Casale et al 18 reported that 19 per cent of ears with congenital aural stenosis had acquired ear canal cholesteatoma. We found a significant association between external auditory canal cholesteatoma formation and Schuknecht type A (stenotic type) external auditory canals ( p = 0.03).…”

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confidence: 99%

High-resolution computed tomography evaluation of congenital aural atresia – how useful is this?

Gautam

¹

,

Kumar

²

,

Pradhan

³

et al. 2020

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Objective To depict various temporal bone abnormalities on high-resolution computed tomography in congenital aural atresia patients, and correlate these findings with auditory function test results and microtia subgroup. Methods Forty patients (56 ears) with congenital malformation of the auricle and/or external auditory canal were evaluated. Auricles were graded according to Marx's classification, divided into subgroups of minor (grades I and II) and major (III and IV) microtia. Other associated anomalies of the external auditory canal, tympanic cavity, ossicular status, oval and round windows, facial nerve, and inner ear were evaluated. Results Minor and major microtia were observed in 53.6 and 46.4 per cent of ears respectively. Mean hearing levels were 62.47 and 62.37 dB respectively (p = 0.98). The malleus was the most commonly dysplastic ossicle (73.3 vs 80.8 per cent of ears respectively, p = 0.53). Facial nerve (mastoid segment) abnormalities were associated (p = 0.04) with microtia subgroup (80 vs 100 per cent in minor vs major subgroups). Conclusion Microtia grade was not significantly associated with mean hearing levels or other ear malformations, except for external auditory canal and facial nerve (mastoid segment) anomalies. High-resolution computed tomography is essential in congenital aural atresia, before management strategy is decided.

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“…Patients with stenosis sizes of 2 mm or less are at high risk for developing cholesteatoma and should undergo surgery; however, the data in the present study challenged this viewpoint. To date, there are no large sample studies focusing on the clinical features and long-term outcomes of CAS, and no studies have described the important parameter of measuring the diameter of EAC [5][6][7][8][9][10][11] . The aim of the present study was to comprehensively evaluate the clinical features and long-term outcomes of CAS, and challenge the previous viewpoint.…”

mentioning

confidence: 99%

Congenital Aural Stenosis: Clinical Features and Long-term Outcomes

Li

¹

,

Chen

²

,

Chen

³

et al. 2016

J. Laryngol. Otol.

0

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The aim of the present study was to comprehensively evaluate the clinical features and long-term outcomes of congenital aural stenosis (CAS). This study presents a retrospective review of patients who underwent meatoplasty for CAS at a tertiary referral hospital from 2008 to 2015. A total of 246 meatoplasty procedures were performed on 232 patients in the present study. We performed multivariate regression analysis. Except in the age < 6 years group, no significant difference was observed among different age groups for cholesteatoma formation, p > 0.05. Except for the stenosis of the external auditory canal (EAC) (>4 mm) group, the other stenosis of EAC groups were not associated with cholesteatoma formation, p > 0.05. Postoperative air-bone gaps (ABG) less than 30 dB occurred in 77.3% (99/128) of the patients, and the Jahrsdoerfer score was associated with postoperative ABG, p < 0.001. The complication rate of CAS was 13.8% (20/144), and males showed a higher risk for postoperative complications (OR, 6.563; 95% CI, 1.268-33.966, p = 0.025). These results indicate that meatoplasty was an effective surgical intervention for CAS, showing a stable hearing outcome with prolonged follow-up. There was no significant difference between the cholesteatoma and no cholesteatoma groups for hearing outcomes, p > 0.05.Congenital aural stenosis (CAS) carries a much greater risk of cholesteatoma compared with congenital aural atresia (CAA). Cole and Jahrsdoerfer defined CAS as an external auditory canal (EAC) with a diameter of 4 mm or less that frequently occurs in conjunction with grade 1 and grade 2 microtia. This diameter was selected because none of the patients with canal openings larger than 4 mm developed cholesteatoma 1,2 . The diagnosis of CAS is based on clinical examination (anamnesis, physical and audiometric evaluation) and imaging, particularly high-resolution computed tomography (HRCT) of the temporal bones 3 . The treatment of EAC cholesteatoma depends on the extent of the disease. Small lesions can typically be controlled with regular debridement, combined with the administration of topical antibiotics, while large and destructive lesions require surgery 4 . The data on patients with CAS are limited, and the fundamental knowledge of CAS is still based on Cole and Jahrsdoerfer (1990). Two important parameters to consider when managing with CAS are patient age and stenosis size 1 . Patients with stenosis sizes of 2 mm or less are at high risk for developing cholesteatoma and should undergo surgery; however, the data in the present study challenged this viewpoint. To date, there are no large sample studies focusing on the clinical features and long-term outcomes of CAS, and no studies have described the important parameter of measuring the diameter of EAC [5][6][7][8][9][10][11] . The aim of the present study was to comprehensively evaluate the clinical features and long-term outcomes of CAS, and challenge the previous viewpoint. Previous studies analysing the clinical features of CAS involved small sample sizes,...

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Acquired Ear Canal Cholesteatoma in Congenital Aural Atresia/Stenosis

Abstract: Approximately 1 in 5 patients with congenital aural stenosis were found to have ear canal cholesteatoma. Female sex is a risk factor; basic audiometric parameters provide no diagnostic utility in distinguishing ears with cholesteatoma from those without cholesteatoma.

Cited by 36 publications

References 9 publications

Congenital Aural Stenosis: Clinical Features and Long-term Outcomes

Congenital Aural Stenosis: Clinical Features and Long-term Outcomes

High-resolution computed tomography evaluation of congenital aural atresia – how useful is this?

Congenital Aural Stenosis: Clinical Features and Long-term Outcomes

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