Integrated microtia and aural atresia management

Truong, Mai Thy; Liu, Yi‐Chun Carol; Kohn, Jocelyn; Chinnadurai, Sivakumar; Zopf, David; Tribble, Melissa S; Tanner, Paul; Sie, Kathleen C.Y.; Chang, Kay W.

doi:10.3389/fsurg.2022.944223

Cited by 5 publications

(4 citation statements)

References 61 publications

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“…Any ear canal surgery is ideally done after microtia reconstruction. 29,30 The 16p13.11 microdeletion has been reported with wide clinical expressivity ranging from apparently normal individuals to syndromic cases with severe neurological impairments as congenital severe microcephaly and congenital heart defects. [21][22][23] Clinical presentation may express with facial dysmorphism, developmental speech and language delay, cognitive impairment, schizophrenia, autism spectrum disorder, psychiatric and behavioral problems, and diverse spectrum of sporadic epilepsy syndromes.…”

Section: Discussionmentioning

confidence: 99%

“… 26 With regard to the treatment of this disorder is a challenge for specialists in this field and there is need to create a collaborative and multidisciplinary net by all of them. As suggested by Truong et al, 29 the list of consultants should include otorhinolaryngology, pediatric audiology, genetics, craniofacial clinic, plastic surgery, oral-maxillofacial surgery, microtia specialist, and speech pathology. 29 For ear malformations as those presented by the proband affected by microtia-associated aural atresia, auricular reconstruction for the microtia must precede the aural atresia surgery.…”

Section: Discussionmentioning

confidence: 99%

“…As suggested by Truong et al, 29 the list of consultants should include otorhinolaryngology, pediatric audiology, genetics, craniofacial clinic, plastic surgery, oral-maxillofacial surgery, microtia specialist, and speech pathology. 29 For ear malformations as those presented by the proband affected by microtia-associated aural atresia, auricular reconstruction for the microtia must precede the aural atresia surgery. 1 Surgical and nonsurgical options for hearing management and auricular reconstruction, and the treatment timeline for each option need to be carefully considered.…”

Section: Discussionmentioning

confidence: 99%

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Severe Unilateral Microtia with Aural Atresia, Hair White Patch, Stereotypes in a Young Boy with De novo 16p13.11 Deletion: Reasons for a New Genotype–Phenotype Correlation

Pavone,

Pappalardo,

Parano

et al. 2023

Glob Med Genet

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Background Microtia is an uncommon congenital malformation ranging from mild anatomic structural abnormalities to partial or complete absence of the ear leading to hearing impairment. Congenital microtia may present as a single malformation (isolated microtia) or sometimes associated with other congenital anomalies involving various organs. Microtia has been classified in three degrees according to the complexity of the auricular malformation and to anotia referred to the total absence of the ear. Genetic role in causing auricular malformation has been widely demonstrated, and genotype–phenotype correlation has been reported in cases of syndromic microtia. Case Presentation We report here a young patient with a third degree of scale classification and aural atresia. The patient showed unspecific facial dysmorphism, speech delay, precocious teething, hair white patch, and stereotypic anomalous movements. Genetic analysis displayed a de novo 16p13.11 deletion. Conclusion Microtia with aural atresia is an uncommon and severe birth defect, which affects functional and esthetic aspects, often associated with other malformations. As traumatic this disorder may be for the parents, the microtia and aural atresia are treatable, thanks to the improving and evolving surgical techniques. Based on the genetic analysis and the clinical features observed in the present case, a genotype–phenotype correlation has been proposed.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Severe Unilateral Microtia with Aural Atresia, Hair White Patch, Stereotypes in a Young Boy with De novo 16p13.11 Deletion: Reasons for a New Genotype–Phenotype Correlation

Pavone,

Pappalardo,

Parano

et al. 2023

Glob Med Genet

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“…Patients with bilateral closed ears usually require hearing aids [ 21 , 22 , 23 ]. The percentage of previous hearing aid users in the bilateral closed-ear group was highest in the six groups.…”

Section: Discussionmentioning

confidence: 99%

Comparative Analysis of Cartilage Conduction Hearing Aid Users and Non-Users: An Investigative Study

Sugimoto,

Yoshida,

Fukunaga

et al. 2023

Audiology Research

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Clinical findings on cartilage conduction hearing aids (CCHAs) have gradually become clear; however, few reports include a large number of cases. This study included 91 ears from 69 patients who underwent CCHA fitting in our hospital. Their ears were divided into six groups (i.e., bilateral aural atresia or severe canal stenosis, unilateral aural atresia or severe canal stenosis, chronic otitis media or chronic otitis externa with otorrhea, sensorineural hearing loss, mixed hearing loss, and conductive hearing loss) according to their clinical diagnosis and type of hearing loss. Most clinical diagnoses were aural atresia or meatal stenosis (bilateral, 21.8%; unilateral, 39.6%). The purchase rate of CCHAs was higher in the closed-ear group (bilateral, 77.3%; unilateral, 62.5%). In the bilateral closed-ear group, air conduction thresholds at 1000, 2000, and 4000 Hz and aided thresholds with CCHAs at 4000 Hz were significantly lower in the purchase group than the non-purchase group. No significant difference was observed between the purchase and non-purchase groups in the unilateral closed-ear group. In the bilateral closed-ear group, air conduction thresholds and aided thresholds were associated with the purchase rate of CCHAs. In the unilateral closed-ear group, factors other than hearing might have affected the purchase rate of CCHAs.

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Innovated surgery incision for patients of congenital malformation of the middle and outer ear with infection (CMMOEI)

Zou,

Sun,

Liu

et al. 2023

Acta Oto-Laryngologica

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Integrated microtia and aural atresia management

Cited by 5 publications

References 61 publications

Severe Unilateral Microtia with Aural Atresia, Hair White Patch, Stereotypes in a Young Boy with De novo 16p13.11 Deletion: Reasons for a New Genotype–Phenotype Correlation

Severe Unilateral Microtia with Aural Atresia, Hair White Patch, Stereotypes in a Young Boy with De novo 16p13.11 Deletion: Reasons for a New Genotype–Phenotype Correlation

Comparative Analysis of Cartilage Conduction Hearing Aid Users and Non-Users: An Investigative Study

Innovated surgery incision for patients of congenital malformation of the middle and outer ear with infection (CMMOEI)

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