There are a lot of resources available to people who want to learn more about microtia, atresia, deafness and hearing disorders. The following are some of the links I find useful.
Please note that I am not "endorsing" any of these doctors; I'm just providing these links because the websites contain useful information.
GENERAL INFORMATION
Description of how hearing works
PHOTOS OF SOME LEADING TREATMENTS
Ears (Dr. John Reinisch, Beverly Hills, California)
- Atresia Microtia Conference which is held in various locations globally
- Earicles Foundation and Earicles Conferences with locations globally
Prevalence and Associations:
Luquetti, Daniela V., Heike, Carrie L., Hing, Anne V., Cunningham, Michael L., and Cox, Timothy C., “Microtia: Epidemiology and Genetics,” American Journal of Medical Genetics, Part A, 2012 Jan; 158A (1): 124-39
Abstract: The reported prevalence varies among regions, from 0.83 to 17.4 per 10,000 births, and the prevalence is considered to be higher in Hispanics, Asians, Native Americans, and Andeans. The etiology of microtia and the cause of this wide variability in prevalence are poorly understood. Strong evidence supports the role of environmental and genetic causes for microtia. Although some studies have identified candidate genetic variants for microtia, no causal genetic mutation has been confirmed. The application of novel strategies in developmental biology and genetics has facilitated elucidation of mechanisms controlling craniofacial development. In this paper we review current knowledge of the epidemiology and genetics of microtia, including potential candidate genes supported by evidence from human syndromes and animal models. We also discuss the possible etiopathogenesis in light of the hypotheses formulated to date: Neural crest cells disturbance, vascular disruption, and altitude.
Luquetti, Daniela V., Leoncini, Emanuele, and Mastroiacovo, Pierpaolo, “Microtia-anotia: A global review of prevalence rates,” Clinical and Molecular Teratology, Birth Defects Research Part A, Volume 91, Issue 9, September 2011, Pages 813-822. First published: 07 June 2011.
Abstract: Using data from birth defects surveillance programs around the world, authors conducted a systematic review on the frequency of microtia-anotia to further explore the differences in prevalence across countries. Ninety-two birth defects surveillance programs were evaluated with a total of 8917 cases of microtia-anotia. The prevalence per 10,000 births for each surveillance program for total cases of microtia-anotia (microtia types I to IV), microtia (types I to III), and anotia (type IV) were: microtia-anotia, 2.06 (confidence interval [CI], 2.02–2.10); microtia, 1.55 (CI, 1.50–1.60); and anotia 0.36 (CI, 0.34–0.38). Higher prevalences were observed for the Americas, Northern Europe and Asia, among Hispanics and Asians, and among active ascertainment and hospital-based surveillance programs. These results must be interpreted cautiously as this variability may be explained mainly by differences in surveillance methods. However, given the magnitude of some of the differences, other factors may also be involved.
Hearing and Socio-Psychological Issues:
Canete, Oscar; Purdy, Suzanne; Brown, Colin R. S., Neeff, Michel; Thorne, Peter R., “Behavioural performance and self-report measures in children with unilateral hearing loss due to congenital aural atresia,” Auris Nasus Larynx, 2021 Feb; 48 (1): 65-74.
Abstract: In an experiment of 12 UHL children and 12 controls, when speech was directed to the good ear, performance was poorer than for normal hearing controls. Sound localisation abilities were impaired in children with UHL. Children with UHL reported higher levels of difficulties in classroom settings compared to children with normal hearing, particularly for activities involving listening in noise and focused listening activities. Older children self-report and parents report difficulties for their children across all SSQ scales. Children with UHL showed a wide range of auditory difficulties. As expected, speech recognition in noise differed from controls. Sound localisation abilities were variable; greater variability was seen for right ear hearing losses suggesting that some of these children may have developed compensatory mechanisms. Younger children identified listening difficulties for school situations where focussed auditory attention was needed. Older children and parents reported greatest difficulty for activities requiring perception of the direction, distance, and movement of sound. Higher levels of effort and inability to ignore sounds were reported as major difficulties.
Du Jiamei, Chai Jiakei, Zhuang Hongxing, Guo Wanhou, Wang Yan, Liu Gaifen, “An investigation of psychological profiles and risk factors in congenital microtia patients,”
Journal of Plastic, Reconstructive & Aesthetic Surgery, Vol 61, Supplement 1, S37-S43, January 01, 2008, Published November 2, 2007
Abstract: This study sought to determine the prevalence of mood disorders among patients with microtia. The prevalence of mood disorders among microtia patients: ‘depression’ 20.2%, ‘interpersonal sensitivity/social difficulties’ 36.6% and ‘hostility/aggression’ 26.3%. Multivariate analyses suggested that age of patients, severity of microtia, low levels of maternal education, being teased by peers, family disharmony, psychological impact on parents and overprotection from parents are significantly associated with mood disorders of patients. Findings suggest that risk factors - such as being teased by peers, family disharmony, psychological impact on parents and overprotection from family - should be controlled.
Hamlet, Claire and Harcourt, Diana, “Exploring the Experiences of Adults with Microtia,” The Cleft Palate-Craniofacial Journal, 2020 Oct; 57(10): 1230–1237.
Abstract: Fifteen adults (12 females) aged between 20 and 62 years took part in semi-structured interviews. Three main themes were identified in the data: microtia as an invisible difference, surgery as a welcome opportunity, and living well with microtia. Participants had incorporated microtia into their self-concept and did not report a lasting negative impact on their lives. However, some psychosocial challenges were reported, including anxiety about showing their ears (even after reconstruction), disclosing their diagnosis to romantic partners, surgical decision-making, and feeling unsupported in the work environment. Individuals with microtia may benefit from psychosocial interventions to increase confidence, access to support for treatment decision-making, and guidance around disclosing microtia to employers.
Qiang Liu, Yibei Wang, Tengyu Yang, Yue Fan, Bo Hou, Yushan Chen, Jian Wang, Xiaowei Chen, “Poor speech recognition, sound localization and reorganization of brain activity in children with unilateral microtia-atresia,” Brain Imaging and Behaviour, 022 Feb;16(1):78-90.
Abstract: A questionnaire on speech recognition and spatial listening ability was administered to 40 subjects with UMA and 40 age- and sex-matched controls. UMA subjects showed poorer speech recognition in laboratory and poorer spatial listening ability. However, cognitive scores determined by the Montreal Cognitive Assessment (MoCA) and Wechsler Intelligence Scale for Children (WISC-IV) did not differ significantly in these two groups. The impact of hearing loss in UMA on brain functional organization was examined by comparing resting-state fMRIs (rs-fMRI) in 27 subjects with right-sided UMA and 27 matched controls. UMA subjects had increased nodal betweenness in visual networks and DMN but decreases in auditory and attention networks. These results indicate that UCHL in UMA causes significant abnormalities in brain organization. The impact of UCHL on cognition should be further examined with a battery of tests that are more challenging and better focused on the cognitive networks identified.
Surgical, Post-Surgical Complications and Related Topics:
Bhamare, Nilesh; Tardalkar, Kishor; Khadilkar, Archana; Parulekar, Pratima; Joshi, Meghnad, “Tissue engineering of human ear pinna,” Cell Tissue Bank, 2022 Feb 1.
Abstract: A decellularized goat ear cartilage was used as the scaffolding to create a human ear pinna that may be used for ear reconstruction in conditions such as microtia.
Khan, Nawal; Willette, Dominique; Melkonian, Jacklyn; Ziegler, Mary and Widgerow, Alan D., “Patient-Reported Satisfaction After Autologous Auricular Reconstruction in Patients with Microtia: A Systematic Review,” Facial Plastic Surgery & Aesthetic Medicine, Online Ahead of Print: January 25, 2022
Abstract: Findings: Nineteen studies utilizing autologous reconstruction technique, comprising 3694 patients, met inclusion criteria. No standardized patient satisfaction assessment was used throughout the studies, indicating criteria variability to measure outcomes. Auricular substructure analysis highlighted lower patient satisfaction with the tragus and antitragus compared with the upper units. In addition, satisfaction depended on patient perception, not on a low surgical complication rate. There is a clear need to incorporate a standardized validated surgery-specific questionnaire related to patient satisfaction in the auricular reconstruction protocol.
Ronde, E.M., Esposito, M., Lin, Y., van Etten-Jamaludin, F.S., Bulstrode, N.W., Bruegem, C.C., “Long-term complications of microtia reconstruction: A systematic review,” Journal of Plastic, Reconstructive and Aesthetic Surgery, 2021 Dec ;74 (12): 3235-3250
Abstract: Twenty-nine publications reported on complications during long-term follow-up. Overall long-term complication rates were not reported. The incidence of individual complications during long-term follow-up was less than 10% after ACC reconstruction and less than 15% in PPE reconstruction. Framework resorption and wire exposure were reported even after an extended follow-up of more than five years after ACC reconstruction, while reports on the extended long-term results of PPE reconstruction are limited. Data synthesis was limited due to heterogeneity and poor study quality. Future studies should report on long-term complications including framework exposure or extrusion, graft loss, framework resorption, wire exposure and scalp and auricular scar complications. We recommend a surgical follow-up of at least five years.
Tanzer, R.C., “Microtia--a long-term follow-up of 44 reconstructed auricles,” Plastic and Reconstructive Surgery, 01 Feb 1978, 61(2):161-166.
Abstract: Forty-three microtia patients have been reviewed 6 to 19 years after auricular reconstruction with autogenous rib cartilage. The adverse emotional reaction to microtia has been ameliorated in 88 percent of them. The need for subsequent revision of the reconstructed auricle has been almost eliminated.
