Aural atresia, microtia, complex heart defect, and hearing loss syndrome: Additional case

Wave I latencies were used to predict the magnitude of conductive components in 80 infants and young children (122 ears) with normal hearing, conductive hearing loss due to otitis media or aural atresia, sensorineural hearing loss, and mixed hearing loss. Two prediction methods were used. The first method based predictions on a 0.03-ms wave I latency delay for each decibel of conductive hearing loss. The second method was based on a regression analysis of wave I latency delays and the magnitude of conductive component for the subjects in this study with normal cochlear status. On average, these prediction methods resulted in prediction errors of 15 dB or greater in over one-third of the ears with hearing loss. Therefore, the clinical use of wave I latencies to predict the presence or magnitude of conductive impairment is not recommended for infants and young children. Instead, bone-conduction ABR testing is recommended as a direct measure of cochlear status when behavioral evaluation is not possible.

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Aural Atresia: What Audiologists Need to Know About Bilateral Versus Unilateral Cases

Perspectives of the ASHA Special Interest Groups ◽

10.1044/persp1.sig9.49 ◽

2016 ◽

Vol 1 (9) ◽

pp. 49-59

Author(s):

Traci Flynn

Keyword(s):

Academic Achievement ◽

Hearing Loss ◽

Language Development ◽

Current Evidence ◽

Speech And Language ◽

Aural Atresia ◽

Speech And Language Development

Children with aural atresia (AA) present with a hearing loss due to differing degrees of malformations of the ear. The effects of this hearing loss on audition, speech/language development, and academic achievement are not well understood. The literature is quite sparse on this topic, including studies with only small numbers of children or retrospective chart reviews. The few studies that have been completed demonstrate that the hearing loss caused by AA affects auditory, as well as speech and language, development and, therefore, results in delays. Children with bilateral atresia are more adversely affected than children with unilateral atresia. However, the delays are not well understood with respect to whether or not amplification mitigates them. This paper presents the current evidence in the literature on the audiological management, the auditory and speech/language development, and the academic achievement of children with aural atresia.

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Hearing Loss in Infants with Craniofacial Anomalies

Otolaryngology ◽

10.1177/019459989411000104 ◽

1994 ◽

Vol 110 (1) ◽

pp. 39-45 ◽

Cited By ~ 6

Author(s):

Deborah Hayes

Keyword(s):

Hearing Loss ◽

Auditory Brainstem Response ◽

Early Identification ◽

Auditory Brainstem ◽

External Ear ◽

Craniofacial Anomalies ◽

Aural Atresia ◽

Bilateral Hearing Loss ◽

Brainstem Response ◽

Ear Anomalies

Results of auditory brainstem response (ABR) evaluation of 145 infants, ages 6 months and younger with craniofacial anomalies (CFA), were examined to determined predicted degree and nature of hearing loss. Approximately 50% of infants demonstrated at least mild bilateral hearing loss. Presence and degree of hearing loss varied by CFA. All infants with bilateral aural atresia exhibited at least a moderate bilateral hearing loss; less than 20% of infants with isolated external ear anomalies (ear tags, isolated microtia) exhibited any degree of hearing loss. In 92% of infants with hearing loss, results of ABR were consistent with primarily conductive dysfunction. Implications for early identification and audiologic management of infants with CFA and hearing loss are discussed.

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An Additional Case of the Recurrent 15q24.1 Microdeletion Syndrome and Review of the Literature

Twin Research and Human Genetics ◽

10.1375/twin.14.4.333 ◽

2011 ◽

Vol 14 (4) ◽

pp. 333-339 ◽

Cited By ~ 6

Author(s):

Ivy S. L. Ng ◽

Wai-Hoe Chin ◽

Eileen C. P. Lim ◽

Ene-Choo Tan

Keyword(s):

Hearing Loss ◽

Array Cgh ◽

Interstitial Deletion ◽

Additional Case ◽

Oligonucleotide Array ◽

Speech Delay ◽

Review Of The Literature ◽

Microdeletion Syndrome ◽

Mild Hearing Loss

We report a 9-year-old girl with 3 Mb interstitial deletion of chromosome 15q24 identified by oligonucleotide array comparative hybridization. She is of Chinese ancestry and shared some typical features of previously reported 15q24 deletion cases such as mild dysmorphism with developmental and speech delay. She also had mild hearing loss that was reported in one other case. We compared all 19 cases that are identified from array-CGH. The deletion occurred within an 8.3 Mb region from 15q23 to 15q24.3. The minimum overlapping deleted region is less than 0.5 Mb from 72.3 Mb to 72.7 Mb. The functions of the nine annotated genes within the region and how they might contribute to the microdeletion phenotype are discussed.

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Audiological results and subjective benefit of an active transcutaneous bone-conduction device in patients with congenital aural atresia

European Archives of Oto-Rhino-Laryngology ◽

10.1007/s00405-021-06938-8 ◽

2021 ◽

Author(s):

Veronika Volgger ◽

Inge Teresa Schießler ◽

Joachim Müller ◽

Florian Schrötzlmair ◽

Marlene Pollotzek ◽

...

Keyword(s):

Hearing Loss ◽

Signal To Noise Ratio ◽

Bone Conduction ◽

Monosyllabic Word ◽

Subjective Impression ◽

Aural Atresia ◽

Mixed Hearing Loss ◽

Functional Benefit ◽

Congenital Aural Atresia ◽

Bone Conduction Device

Abstract Purpose To review functional and subjective benefit after implantation of an active transcutaneous bone conduction device (BCD) in patients with congenital microtia with atresia or stenosis of the external auditory canal. Methods Retrospective chart analysis and questionnaire on the subjective impression of hearing ( Speech, Spatial and Qualities of Hearing Scale (SSQ-B) of patients treated between 2012 and 2015. ResultsResults 18 patients (24 ears) with conductive or mixed hearing loss in unilateral (n = 10) or bilateral (n = 8) atresia were implanted with a BCD. No major complications occurred after implantation. Preoperative unaided air conduction pure tone average at 0.5, 1, 2 and 4 kHz (PTA 4 ) was 69.2 ± 11.7 dB, while postoperative aided PTA 4 was 33.4 ± 6.3 dB, resulting in a mean functional hearing gain of 35.9 +/- 15.6 dB. Preoperatively, the mean monosyllabic word recognition score was 22.9 % ± 22.3 %, which increased to 87.1 % +/- 15.1 % in the aided condition. The Oldenburger Sentence Test at S0N0 revealed a decrease in signal-to-noise-ratio from − 0.58 ± 4.40 dB in the unaided to − 5.67 ± 3.21 dB in the postoperative aided condition for all patients investigated. 15 of 18 patients had a subjective benefit showing a positive SSQ-B score (mean 1.7). Conclusion The implantation of an active bone conduction device brings along subjective and functional benefit for patients with conductive or combined hearing loss.

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One-Year Follow-Up in Children with Conductive Hearing Loss Using ADHEAR

Audiology and Neurotology ◽

10.1159/000514087 ◽

2021 ◽

pp. 1-10

Author(s):

Mario E. Zernotti ◽

Elvira Alvarado ◽

Maximo Zernotti ◽

Natalia Claveria ◽

Maria F. Di Gregorio

Keyword(s):

Hearing Loss ◽

User Satisfaction ◽

Repeated Measures ◽

Conductive Hearing Loss ◽

Bone Conduction ◽

Free Field ◽

Aural Atresia ◽

Published Evidence ◽

Conductive Hearing ◽

Subjective Outcomes

Background: The ADHEAR™ system (MED-EL, Innsbruck, Austria) is a nonsurgical bone conduction device (BCD) to treat conductive hearing loss (CHL) and single-sided deafness. In contrast to the nonsurgical alternatives on headbands or spectacle frames, the audio processor of ADHEAR is placed retroauricularly on an adhesive adapter. The published evidence on the performance of this system is limited to studies with a trial period of 2–8 weeks. Objective: This study assesses audiological and subjective outcomes over a period of 12 months, on patients with congenital aural atresia (CAA) using the ADHEAR hearing system. Method: Fifteen children (mean age: 9.4 ± 4 years; range: 5–16 years) diagnosed with CAA (7 uni/8 bilateral) were included in this prospective, observational, repeated-measures study. Each subject used ADHEAR for 1 year, and the performance was evaluated after 1, 6, and 12 months. Free-field audiometry and speech discrimination tests were performed, and hearing-, general health- and device-specific questionnaires were used. Results: The unaided sound field threshold improved from an average PTA4 of 63.6 ± 3.4 dB HL to an aided average PTA4 of 29.3 ± 3.0 dB HL after 1 month of device use. The word recognition score (WRS) improved from an average of 27.9 ± 15.9% unaided to an aided average WRS of 91.3 ± 4.4% (p = 0.0003) after 1 month, 92.0 ± 4.1% (p = 0.0002) after 6 months, and 92.7 ± 5.3% (p < 0.0001) after 12 months using the ADHEAR system compared to the unaided condition for all 3 time points. The improvements in the speech in noise at 1, 6, and 12 months were as well consistent over time. The average improvement at the signal to noise ratio (SNR) of +5 dB was 58% and 53% at the SNR of +0 dB. No complications were reported, and all patients continued to use the ADHEAR after the study end. The questionnaire results revealed high user satisfaction and an average wearing time of 12 h per day. Conclusion: This 12-month trial of the nonsurgical adhesive BCD in CAA patients showed sufficient and reliable audiological and subjective outcomes, long wearing time, and high acceptance. The ADHEAR can be considered a suitable option to treat children with CAA for the given indication, without the drawbacks of nonsurgical devices that use pressure for retention of the audio processor or the costs and possible complications involved with a surgical alternative.

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