Congenital Aural Atresia

A hereditary autosomal dominant ear abnormality where defects in structures of the AUDITORY CANAL or MIDDLE EAR result in CONDUCTIVE HEARING LOSS. Decreased odor sensitivity and discrimination may also occur in some patients due to hypoplasia of the OLFACTORY BULB. Mutations in the TSHZ1 gene have been identified. OMIM: 607842

Also Known As:

Networked: 61 relevant articles (5 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pathologic Constriction (Stenosis)
2.	Congenital Microtia
3.	Hearing Loss (Hearing Impairment)
4.	Otosclerosis
5.	Atrioventricular Block

Experts

1.	Yamasoba, Tatsuya: 3 articles (11/2015 - 12/2011)
2.	Zhao, S Q: 2 articles (04/2021 - 10/2016)
3.	Chen, Peiwei: 2 articles (02/2021 - 12/2020)
4.	Gao, Mengdie: 2 articles (02/2021 - 12/2020)
5.	Liu, Yujie: 2 articles (02/2021 - 12/2020)
6.	Yang, Jinsong: 2 articles (02/2021 - 12/2020)
7.	Zhao, Shouqin: 2 articles (02/2021 - 12/2020)
8.	Iwasaki, Satoshi: 2 articles (01/2021 - 09/2011)
9.	Kobayashi, Toshimitsu: 2 articles (06/2020 - 09/2011)
10.	Kesser, Bradley W: 2 articles (02/2018 - 02/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Congenital Aural Atresia:

1.	TitaniumIBA 04/01/2015 - "Titanium mesh tubing is safe and effective for reconstruction of the external ear canal during surgery for congenital aural atresia. " 04/01/2015 - "Reconstruction of the external ear canal with a titanium mesh tube was performed in 16 patients (16 ears) with congenital aural atresia at the First People's Hospital of Foshan. " 04/01/2015 - "Applications of titanium mesh tubing in external ear canal reconstruction in congenital aural atresia." 04/01/2015 - "The aim of this study was to observe the effect of a titanium tube on external auditory canal reconstruction in congenital aural atresia and to assess the tube's effectiveness in preventing external canal stenosis or atresia after reconstruction. "
2.	ArgonIBA 09/01/2004 - "The use of argon laser, thinner split-thickness skin graft, Silastic sheets in the external auditory canal, and Merocel wicks, as a group, has helped to improve hearing results and decrease the incidence of complications in congenital aural atresia surgery. " 09/01/2004 - "To compare modifications in the surgical technique of congenital aural atresia (use of argon laser, thinner split-thickness skin graft, Silastic sheets in the external auditory canal, and Merocel wicks) by examining hearing results and complications before and after initiation of these changes. "
3.	polyvinyl alcohol formaldehyde foamIBA 09/01/2004 - "The use of argon laser, thinner split-thickness skin graft, Silastic sheets in the external auditory canal, and Merocel wicks, as a group, has helped to improve hearing results and decrease the incidence of complications in congenital aural atresia surgery. " 09/01/2004 - "To compare modifications in the surgical technique of congenital aural atresia (use of argon laser, thinner split-thickness skin graft, Silastic sheets in the external auditory canal, and Merocel wicks) by examining hearing results and complications before and after initiation of these changes. "
4.	baysilon (polydimethylsiloxane)IBA 09/01/2004 - "The use of argon laser, thinner split-thickness skin graft, Silastic sheets in the external auditory canal, and Merocel wicks, as a group, has helped to improve hearing results and decrease the incidence of complications in congenital aural atresia surgery. " 09/01/2004 - "To compare modifications in the surgical technique of congenital aural atresia (use of argon laser, thinner split-thickness skin graft, Silastic sheets in the external auditory canal, and Merocel wicks) by examining hearing results and complications before and after initiation of these changes. "
5.	trans-crotonin (CTN)IBA 12/01/2022 - "This study aimed to describe the course of the CTN observed during atresiaplasty for congenital aural atresia (CAA) and explore the feasibility of CTN preservation. "
6.	MedporIBA 12/01/2015 - "This study describes a reliable flap technique for combining atresiaplasty with total auricular reconstruction by performing the total auricular reconstruction with the MEDPOR framework at the same time as the atresiaplasty for patients with microtia and congenital aural atresia. "
7.	Growth Hormone (Somatotropin)IBA 07/01/2009 - "The 18q deletion syndrome (18q-) is a multiple-anomaly disorder associated with mental retardation, white matter anomalies in the brain, growth hormone deficiency, congenital aural atresia, orofacial cleft (OFC), and palate abnormalities. "
8.	SolutionsIBA 01/01/2015 - "We describe the novel solution adopted in positioning middle ear implant in a child with bilateral congenital aural atresia and craniofacial dysmorphism that have posed a significant challenge for the safe and correct management of deafness. "
9.	PlasticsIBA 02/01/2018 - "Atresiaplasty in Congenital Aural Atresia: What the Facial Plastic Surgeon Needs to Know."

Therapies and Procedures

1.	Ossicular Prosthesis (TORP) 06/01/2011 - "Facing anatomic variations in congenital aural atresia, active middle ear implants can provide substantial hearing improvement in safe conditions and open new strategies for hearing rehabilitation." 08/01/2023 - "To assess hearing rehabilitation in patients with congenital aural atresia using an active middle-ear implant (Vibrant Soundbridge). " 08/01/2023 - "Congenital aural atresia: hearing rehabilitation using active middle-ear implants." 04/01/2022 - "To evaluate the safety, speech performance in noise and subjective satisfaction of patients with congenital aural atresia (CAA) implanted with the active middle ear implant. " 04/01/2022 - "Speech performance and subjective satisfaction of middle ear implant in congenital aural atresia."
2.	Hearing Aids (Hearing Aid) 10/05/2016 - "Objective:To evaluate the efficacy of Bone anchored hearing aid(Baha) application in patients with bilateral congenital aural atresia.Method" 10/05/2016 - "[The efficacy of bone anchored hearing aid application in patients with bilateral congenital aural atresia]." 01/01/2014 - "The objective of this study was to compare outcomes in hearing, complications, and quality of life of surgical reconstruction of the external auditory canal reconstruction (EACR) and bone-anchored hearing aid (BAHA) in a pediatric population with congenital aural atresia. " 01/01/2014 - "Bone-Anchored Hearing Aid vs. Reconstruction of the External Auditory Canal in Children and Adolescents with Congenital Aural Atresia: A Comparison Study of Outcomes." 01/01/2020 - "The effects of FM system fitted into the normal hearing ear (NHE) or a cartilage conduction hearing aid (CCHA) fitted into the affected ear (AE) on the speech recognition ability in noise were examined in children with unilateral congenital aural atresia (UCAA). "
3.	Lasers (Laser) 01/01/2005 - "Simulated 3D models of two cases of congenital aural atresia were prototyped using a selective laser sintering method. " 09/01/2004 - "The use of argon laser, thinner split-thickness skin graft, Silastic sheets in the external auditory canal, and Merocel wicks, as a group, has helped to improve hearing results and decrease the incidence of complications in congenital aural atresia surgery. " 09/01/2004 - "To compare modifications in the surgical technique of congenital aural atresia (use of argon laser, thinner split-thickness skin graft, Silastic sheets in the external auditory canal, and Merocel wicks) by examining hearing results and complications before and after initiation of these changes. "
4.	Surgical Instruments (Clip) 12/01/2020 - "To evaluate the long-term safety and efficacy of the Clip coupler attached to the stapes head in patients with unilateral congenital aural atresia (CAA). " 12/01/2020 - "Long-term Outcomes of Clip Coupler Implantation in Patients with Unilateral Congenital Aural Atresia."
5.	Tympanoplasty 11/01/2015 - "The present study aimed to determine favorable prognostic factors for long-term postoperative hearing outcome after canal-tympanoplasty for congenital aural atresia (CAA). " 03/08/2023 - "Patients with congenital aural atresia (CAA) can present with concomitant temporomandibular joint (TMJ) retroposition, implying that even with a high Jahrsdoerfer score, canaloplasty and tympanoplasty cannot be performed. " 11/01/2015 - "Prognostic factors for long-term hearing preservation after canal-tympanoplasty for congenital aural atresia." 05/01/1994 - "There was little difference in postoperative hearing among the various types of congenital aural atresia and the type of tympanoplasty that had been performed." 04/01/1984 - "The four surgical methods currently in use to improve hearing in patients with congenital aural atresia are (i) fenestration of the lateral semicircular canal, (ii) type III tympanoplasty, (iii) canal plasty and (iv) canal plasty with homograft tympanoplasty. "