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Med Sci Monit ; 27: e933915, 2021 Sep 25.
Article En | MEDLINE | ID: mdl-34561413

BACKGROUND The aim of this study was to assess the effectiveness of bone conduction hearing aids in children under 2 years old who have congenital microtia and atresia. MATERIAL AND METHODS This prospective study involved 42 children under 2 years old with congenital microtia and atresia who were divided into 2 groups: 21 with unilateral defect and 21 with bilateral defect. All children were provided with bone conduction hearing aids on a softband. Air and bone auditory thresholds were assessed by auditory brainstem responses (ABRs). The LittlEARS questionnaire was used to evaluate auditory development at baseline and after 6 months of hearing aids use. Behavioral observation audiometry (BOA) was used to assess auditory thresholds and compare aided and unaided hearing. RESULTS After 6 months of hearing aid use, the total score of the LittlEARS questionnaire in children with unilateral defect was 24±5.60, while children with bilateral defect achieved a result of 26.29±6.17. Hearing thresholds in both groups with bone conduction hearing aids improved significantly and approached the normal level. CONCLUSIONS Our results confirm that bone conduction hearing aids provide an effective method of auditory rehabilitation for children with conductive and mixed hearing loss caused by microtia and atresia. Using bone conduction hearing aids in such children is crucial for proper hearing, speech, and language development.

Bone Conduction/physiology , Congenital Abnormalities/therapy , Congenital Microtia/therapy , Ear/abnormalities , Hearing Aids , Audiometry/methods , Congenital Abnormalities/physiopathology , Congenital Microtia/physiopathology , Ear/physiopathology , Female , Humans , Infant , Male , Prospective Studies , Surveys and Questionnaires , Treatment Outcome
Acta Otolaryngol ; 141(4): 321-327, 2021 Apr.
Article En | MEDLINE | ID: mdl-33439071

BACKGROUND: Implantable bone-conduction hearing aids (BCHA) are effective in patients with congenital ear malformations.However, there is no large sample study to verify the efficacy of Bonebridge in patients with congenital oval window atresia. OBJECTIVES: To investigate efficiency of implantable bone-conduction hearing aids in Mandarin-speaking patients with congenital oval window atresia. MATERIAL AND METHODS: We retrospectively analyzed 15 patients, who were confirmed with either unilateral or bilateral congenital oval window atresia by temporal bone CT. All patients were implanted with a bone-conduction hearing device between July 2016 and July 2019 at Beijing Tongren Hospital, Capital Medical University. Pure tone audiometry (PTA), air-bone gap (ABG), speech discrimination scores (SDSs), and hearing thresholds were performed. RESULTS: Postoperative complications including facial paralysis were particularly rare. Unaided mean sound field threshold was 62.2 ± 10.5 dBHL and that with implantable bone-conduction hearing aids was 39.1 ± 13.2 dBHL (p < 0.01). The mean speech discrimination scores improved greatly (p < 0.01), specifically with regard to sentence and disyllabic words. CONCLUSIONS: Patients with congenital oval window atresia often show moderate to severe conductive hearing loss. Implantable bone-conduction hearing aids are considerably safe and stable for hearing rehabilitation. It is a novel treatment modality for Mandarin-speaking patients with congenital oval window atresia.

Congenital Abnormalities , Hearing Aids , Hearing Loss, Conductive/rehabilitation , Oval Window, Ear/abnormalities , Adolescent , Adult , Auditory Threshold , Bone Conduction , Child , China , Ear/abnormalities , Female , Hearing Loss, Conductive/etiology , Humans , Male , Retrospective Studies , Young Adult
Ann Otol Rhinol Laryngol ; 130(9): 1004-1009, 2021 Sep.
Article En | MEDLINE | ID: mdl-33491463

OBJECTIVE: To record crossed acoustic reflex thresholds (xART's) postoperatively from patients after surgical repair of unilateral congenital aural atresia (CAA). To seek explanations for when xARTs can and cannot be recorded. We hope to understand the implications for this central auditory reflex despite early afferent deprivation. METHODS: Patients who underwent surgery to correct unilateral CAA at a tertiary academic medical were prospectively enrolled to evaluate for the presence of xART. Preoperative ARTs in the normal (non-atretic) ear, and postoperative ipsilateral ARTs (stimulus in the normal ear) and contralateral ARTs (stimulus in the newly reconstructed atretic ear; record in the normal ear) were measured at 500, 1000, and 2000 Hz. RESULTS: Four of 11 patients with normal ipsilateral reflex thresholds preoperatively demonstrated crossed acoustic reflexes postoperatively (stimulus in reconstructed ear; record from normal ear). Four other patients demonstrated normal ipsilateral thresholds preoperatively but did not have crossed reflexes postoperatively. No reflexes (pre- or postoperatively) could be recorded in 3 patients. Crossed reflex threshold is significantly correlated with the postoperative audiometric threshold. There was no correlation between ipsilateral and contralateral reflex thresholds. CONCLUSION: Crossed acoustic reflexes can be recorded from some but not all postoperative atresia patients, and the thresholds for those reflexes correlate with the postoperative pure tone threshold. The presence of acoustic reflexes implies an intact CN VIII-to-opposite CN VII central reflex arc despite early unilateral sound deprivation.

Auditory Pathways/physiopathology , Congenital Abnormalities/physiopathology , Ear/abnormalities , Efferent Pathways/physiopathology , Facial Nerve/physiopathology , Reflex, Acoustic/physiology , Vestibulocochlear Nerve/physiopathology , Audiometry, Pure-Tone , Auditory Threshold , Child , Congenital Abnormalities/surgery , Ear/physiopathology , Ear/surgery , Efferent Pathways/physiology , Facial Nerve/physiology , Female , Humans , Male , Middle Aged , Ossicular Replacement , Otologic Surgical Procedures , Prospective Studies , Vestibulocochlear Nerve/physiology
Auris Nasus Larynx ; 48(1): 65-74, 2021 Feb.
Article En | MEDLINE | ID: mdl-32736886

OBJECTIVE: To explore the behavioural and functional performance of a group of children with conductive unilateral hearing loss (UHL) due to congenital aural atresia. METHOD: Twelve children aged 7 to 16 years (Mage 10.0, SD 3.1 years) formed the UHL group and 15 age-matched children (Mage 9.5, SD 3.6 years) with normal hearing formed the control group. Auditory skills were assessed using tests of sound localisation, spatial speech perception in noise, and self-ratings of auditory abilities (Listening Inventory for Education; LIFE and Speech, Spatial and Qualities of Hearing scale; SSQ). RESULTS: 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. CONCLUSIONS: 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.

Congenital Abnormalities , Ear/abnormalities , Hearing Loss, Conductive , Hearing Loss, Unilateral , Sound Localization , Adolescent , Adult , Case-Control Studies , Child , Female , Hearing Loss, Conductive/physiopathology , Hearing Loss, Unilateral/physiopathology , Hearing Tests , Humans , Male , Parents , Perceptual Masking , Self Report , Speech Perception , Surveys and Questionnaires
Acta Otolaryngol ; 141(1): 34-38, 2021 Jan.
Article En | MEDLINE | ID: mdl-33043753

BACKGROUND: Detailed studies have not been conducted on sound localization and speech perception in noise in patients with unilateral congenital aural atresia (UCAA). AIMS/OBJECTIVES: To evaluate the benefits of the use of the Vibrant Soundbridge (VSB) for UCAA by performing audiometric and sound localization tests. MATERIALS AND METHODS: Four children with UCAA underwent VSB (VORP 503) implantation from 2018 to 2019. Speech perception tests in noise were conducted using the Japanese monosyllable test. The spatial configuration for speech testing consisted of speech presented from the front and noise presented into the normal ear (S0N90). The sound localization test was conducted using nine loudspeakers equally distributed in a semicircle. RESULTS: The children's speech perception in noise 6 months after VSB activation was significantly better than before activation (p < .05). Additionally, the children's sound localization ability after VSB activation was significantly better than before VSB activation. CONCLUSION AND SIGNIFICANCE: The benefits of VSB use in children with UCAA were revealed using audiometric and sound localization tests.

Congenital Abnormalities/surgery , Ear/abnormalities , Hearing Loss, Conductive/surgery , Ossicular Prosthesis , Speech Perception/physiology , Audiometry, Pure-Tone , Child , Child, Preschool , Congenital Abnormalities/physiopathology , Ear/physiopathology , Ear/surgery , Female , Hearing Loss, Conductive/physiopathology , Humans , Male , Prosthesis Design , Retrospective Studies
Acta Otolaryngol ; 141(2): 129-134, 2021 Feb.
Article En | MEDLINE | ID: mdl-33140681

BACKGROUND: Bonebridge is an suitable option for conductive hearing loss, however, the traditional approach cannot accomplished a satisfying implantation for patients with congenital malformation or radical mastoidectomy. OBJECTIVES: To evaluate the clinical application of retrosigmoid sinus approach in Bonebridge implantation and postoperative evaluation. MATERIALS AND METHODS: 11 patients who underwent retrosigmoid sinus approach Bonebridge implantation from March 2016 to September 2019 were retrospectively analyzed, including 6 males and 5 females, aged 12-54 years old (30.6 in average). Among them, 4 cases had undergone bilateral radical mastoidectomy, 6 cases had bilateral congenital aural atresia or stenosis, and 1 case had unilateral congenital aural atresia. RESULTS: All patients underwent Bonebridge implantation through retrosigmoid sinus approach according to the preoperative image reconstruction and plan. There was no surgical injury of sigmoid sinus or cerebrospinal fluid leakage during the operation. The aided threshold obtained an increase of 32.32 dB HL; the speech recognition rates of bisyllabic words, monosyllabic words and sentence were 79.6%, 67.8% and 75.0%, respectively. After 11-53 months of follow-up, the hearing effect was stable and no long-term complications occurred. CONCLUSION: The retrosigmoid sinus approach is an effective surgical approach for patients with congenital ear deformities or radical cavity after mastoidectomy.

Congenital Abnormalities , Ear/abnormalities , Hearing Aids , Hearing Loss, Conductive/surgery , Mastoidectomy/adverse effects , Otologic Surgical Procedures/methods , Adolescent , Adult , Audiometry, Pure-Tone , Bone Conduction , Child , Female , Hearing Loss, Conductive/etiology , Humans , Male , Middle Aged , Paranasal Sinuses , Retrospective Studies , Speech Reception Threshold Test , Temporal Bone/diagnostic imaging , Tomography, X-Ray Computed
J Appl Genet ; 62(1): 107-113, 2021 Feb.
Article En | MEDLINE | ID: mdl-33131036

Auriculocondylar syndrome (ACS) is an ultra-rare disorder that arises from developmental defects of the first and second pharyngeal arches. Three subtypes of ACS have been described so far, i.e., ACS1 (MIM: 602483), ACS2 (MIM: 600810), and ACS3 (MIM: 131240). The majority of patients, however, are affected by ACS2, which results from the mutations in the PLCB4 gene. Herein, we have described an 8-year-old male patient presenting with ACS2 and summarized the molecular and phenotypic spectrum of the syndrome. We have also compared the clinical features of our case to three other previously described cases (one sporadic and two familial) harboring the same heterozygous missense variant c.1862G>A, p.Arg621His in the PLCB4 gene. The mutation was detected using whole-exome sequencing (WES). Due to low coverage of WES and suspicion of somatic mosaicism, the variant was additionally reassessed by deep targeted next-generation sequencing panel of genes related to the craniofacial disorders, and next confirmed by Sanger sequencing. ACS2 presents high intra- and interfamilial phenotypic heterogeneity that impedes reaching an exact clinical and molecular diagnosis. Thus, describing additional cases, carrying even the known mutation, but resulting in variable phenotypes, is essential for better understanding of such orphan Mendelian diseases.

Ear Diseases/genetics , Ear/abnormalities , Child , Humans , Male , Mutation , Pedigree , Phenotype , Phospholipase C beta/genetics
Int J Pediatr Otorhinolaryngol ; 141: 110551, 2021 Feb.
Article En | MEDLINE | ID: mdl-33338703

OBJECTIVES: Patients with microtia and aural atresia have multiple options for treatment of conductive hearing loss (CHL) and auricle reconstruction; however, little is known about the factors influencing treatment selection. This study aims to review the socioeconomic and clinical data of microtia/atresia patients to evaluate congruency with national data and whether these factors affect treatment decisions. METHODS: Retrospective review of patients evaluated in the microtia and atresia multidisciplinary clinic (MDC) at a tertiary academic children's hospital between 2008 and 2018. Outcomes included demographic, socioeconomic and clinical factors associated with hearing surgery and framework surgery. RESULTS: 373 patients were seen in the Microtia MDC: 193 (51.7%) were male, 187 (50.1%) identified as Hispanic and 23 (6.2%) identified as Asian. 267 (75.6%) patients received a nonsurgical bone conduction hearing device (BCHD); fitting at a younger age was associated with better nonsurgical BCHD compliance. Multivariate analysis was performed on the patients that were eligible for surgery based on age and appropriate follow-up. 70 (18.8%) patients had placement of an osseointegrated BCHD; inconsistent compliance with nonsurgical BCHD decreased the odds of proceeding with osseointegrated BCHD placement. 60 (16.1%) patients underwent framework surgery for external reconstruction. Placement of osseointegrated BCHD was the only factor that was associated with proceeding with framework surgery. Other assessed demographic and socioeconomic factors were statistically not associated with selection of surgical intervention. CONCLUSION: Fitting a nonsurgical BCHD at a younger age is associated with higher likelihood of nonsurgical BCHD compliance, that is in turn associated with patients and families proceeding with osseointegrated BCHD and framework surgery.

Congenital Abnormalities , Congenital Microtia , Ear/abnormalities , Child , Hearing Aids , Hearing Loss, Conductive/surgery , Humans , Male , Retrospective Studies , Socioeconomic Factors
Laryngoscope ; 131(4): E1349-E1356, 2021 04.
Article En | MEDLINE | ID: mdl-32886384

OBJECTIVES: To characterize tracheal cartilage morphology in mouse models of fibroblast growth factor receptor (Fgfr2)-related craniosynostosis syndromes. To establish relationships between specific Fgfr2 mutations and tracheal cartilaginous sleeve (TCS) phenotypes in these mouse models. METHODS: Postnatal day 0 knock-in mouse lines with disease-specific genetic variations in the Fgfr2 gene (Fgfr2C342Y/C342Y , Fgfr2C342Y/+ , Fgfr2+/Y394C , Fgfr2+/S252W , and Fgfr2+/P253R ) as well as line-specific controls were utilized. Tracheal cartilage morphology as measured by gross analyses, microcomputed tomography (µCT), and histopathology were compared using Chi-squared and single-factor analysis of variance statistical tests. RESULTS: A greater proportion of rings per trachea were abnormal in Fgfr2C342Y/+ tracheas (63%) than Fgfr2+/S252W (17%), Fgfr2+/P253R (17%), Fgfr2+/Y394C (12%), and controls (10%) (P < .001 for each vs. Fgfr2C342Y/+ ). TCS segments were found only in Fgfr2C342Y/C342Y (100%) and Fgfr2C342Y/+ (72%) tracheas. Cricoid and first-tracheal ring fusion was noted in all Fgfr2C342Y/C342Y and 94% of Fgfr2C342Y/+ samples. The Fgfr2C342Y/C342Y and Fgfr2C342Y/+ groups were found to have greater areas and volumes of cartilage than other lines on gross analysis and µCT. Histologic analyses confirmed TCS among the Fgfr2C342Y/C342Y and Fgfr2C342Y/+ groups, without appreciable differences in cartilage morphology, cell size, or density; no histologic differences were observed among other Fgfr2 lines compared to controls. CONCLUSION: This study found TCS phenotypes only in the Fgfr2C342Y mouse lines. These lines also had increased tracheal cartilage compared to other mutant lines and controls. These data support further study of the Fgfr2 mouse lines and the investigation of other Fgfr2 variants to better understand their role in tracheal development and TCS formation. LEVEL OF EVIDENCE: NA Laryngoscope, 131:E1349-E1356, 2021.

Genetic Association Studies/methods , Receptor, Fibroblast Growth Factor, Type 2/genetics , Trachea/abnormalities , Tracheal Diseases/genetics , Acanthosis Nigricans/genetics , Acrocephalosyndactylia/genetics , Animals , Cartilage/pathology , Craniofacial Dysostosis/genetics , Craniosynostoses/genetics , Disease Models, Animal , Ear/abnormalities , Humans , Mice , Mutation , Phenotype , Scalp Dermatoses/genetics , Skin Abnormalities/genetics , Trachea/embryology , Trachea/pathology , Tracheal Diseases/diagnosis , Tracheal Diseases/pathology , X-Ray Microtomography/methods
Medicine (Baltimore) ; 99(30): e21313, 2020 Jul 24.
Article En | MEDLINE | ID: mdl-32791720

BACKGROUND: Congenital auricular deformities (CAD) are prevalent worldwide. The objective of this study is to investigate the effectiveness and safety of ear molding for children with CAD at their early days. METHODS: One hundred and nighty children (under 3 days) with CAD will be included in the study. Participants will be randomly allocated to treatment or waiting list group (n = 95). The treatment group will receive ear molding within 3 days after birth for 2 weeks. The control group will receive usual care and receive the same ear molding at 6th week if spontaneously recover is not occur. Physician and parent assessment of improvement, parent's anxiety, depression, and quality of life and adverse events will be measured at baseline, 3rd and 6th week of initial treatment. The primary outcome recovery rate will be compared between groups using Chi square test. Secondary continuous outcomes will be compared using analysis of variance. DISCUSSION: This study is the first randomized controlled trial to examine the effectiveness, safety and cost-effectiveness of ear molding for CAD comparing with waiting list, to inform clinical decision of CAD treatments and relevant guideline development.

Congenital Microtia/epidemiology , Cost-Benefit Analysis/methods , Ear/abnormalities , Hearing Aids/adverse effects , Case-Control Studies , Clinical Decision-Making , Ear/pathology , Hearing Aids/statistics & numerical data , Humans , Infant, Newborn , Parents/psychology , Quality of Life , Safety , Treatment Outcome , Visual Analog Scale , Waiting Lists
J Laryngol Otol ; 134(7): 610-622, 2020 Jul.
Article En | MEDLINE | ID: mdl-32686623

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.

Congenital Abnormalities/diagnostic imaging , Ear/abnormalities , Adolescent , Adult , Child , Child, Preschool , Congenital Abnormalities/diagnosis , Congenital Abnormalities/pathology , Congenital Microtia/diagnosis , Congenital Microtia/diagnostic imaging , Cross-Sectional Studies , Ear/diagnostic imaging , Ear/pathology , Ear Canal/diagnostic imaging , Ear Canal/pathology , Facial Nerve/diagnostic imaging , Facial Nerve/pathology , Female , Humans , Infant , Infant, Newborn , Male , Temporal Bone/diagnostic imaging , Temporal Bone/pathology , Tomography, X-Ray Computed/methods , Young Adult
J Comput Assist Tomogr ; 44(4): 559-561, 2020.
Article En | MEDLINE | ID: mdl-32697526

In this study, the percent of wormian bones in congenital aural atresia group was higher than healthy group, when tested by the Fisher exact test, there appeared to be no significant statistical difference. However, in congenital aural atresia surgery, surgical manipulation can dislodge these bones causing injury to the surrounding structures. Therefore, the radiologists should report the presence of wormian bones in the peritemporal area.

Congenital Abnormalities/diagnostic imaging , Cranial Sutures/diagnostic imaging , Ear/abnormalities , Adolescent , Adult , Case-Control Studies , Child , Child, Preschool , Ear/diagnostic imaging , Female , Humans , Infant , Male , Middle Aged , Radiographic Image Interpretation, Computer-Assisted , Retrospective Studies , Tomography, X-Ray Computed , Young Adult
J Craniofac Surg ; 31(6): 1780-1781, 2020 Sep.
Article En | MEDLINE | ID: mdl-32604297

Beare-Stevenson syndrome (BSS) is an extremely rare genetic disorder characterized by a broad range of congenital malformations including craniosynostosis, cutis gyrata, facial deformities, and abnormal genitalia. The authors report a case of a 7 month old female who developed a mechanical ptosis secondary to dermatochalasis as a complication of fronto-orbital advancement and remodeling (FOAR) surgery which subsequently required multiple lid surgeries to reverse ptosis. This is the first report of blepharoptosis correction in a child with BSS as a complication of FOAR.

Acanthosis Nigricans/surgery , Blepharoptosis/surgery , Craniosynostoses/surgery , Ear/abnormalities , Scalp Dermatoses/surgery , Skin Abnormalities/surgery , Acanthosis Nigricans/complications , Blepharoptosis/complications , Craniosynostoses/complications , Ear/surgery , Female , Humans , Infant , Orbit , Scalp Dermatoses/complications , Skin Abnormalities/complications
Am J Med Genet A ; 182(9): 2102-2109, 2020 09.
Article En | MEDLINE | ID: mdl-32706523

To describe special facial features of children with Williams syndrome in China by using method of three-dimensional craniofacial anthropometry. Using three-dimensional stereo photogrammetric device, 14 craniofacial anthropometric measurements were performed and five indices were calculated in 52 children with Williams syndrome and 208 age and sex matched controls of Han Chinese ethnicity. Except intercanthal width, mouth breadth, morphological face height, nasal height-breadth index, nasal breadth-depth index, morphological ear index, the Williams syndrome group under 3 years old were smaller than the control group in the other 12 variables. Compared with the control group, the Williams syndrome group aged 3-5 years old had smaller biocular breadth, nasal length, nasorostral angle, bitragal breadth, ear width, morphological ear index and face depth. The Williams syndrome group aged above 6 years old had smaller biocular breadth, nasal breadth, bitragal breadth, ear width, ear length and face depth than the control group. The craniofacial variability index of the Williams syndrome group was greater than the control group. Greater variation was found among children with Williams syndrome than normal in China, specifically at eye, nose, ear and face shape, which demonstrate the usefulness of three-dimensional stereo photogrammetric analysis in supporting accurate diagnose of the patient with Williams syndrome.

Anthropometry , Face/abnormalities , Skull/abnormalities , Williams Syndrome/genetics , /genetics , Cephalometry , Child , Child, Preschool , China/epidemiology , Ear/abnormalities , Ear/pathology , Face/pathology , Female , Humans , Male , Nose/abnormalities , Nose/pathology , Skull/pathology , Williams Syndrome/pathology
Ann Otol Rhinol Laryngol ; 129(12): 1221-1228, 2020 Dec.
Article En | MEDLINE | ID: mdl-32500728

OBJECTIVE: 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). METHODS: This single-center retrospective study included 16 Mandarin-speaking patients who had unilateral microtia accompanied by CAA. All patients were divided into two groups: the short-term follow-up group (n = 9) and the long-term follow-up group (n = 7). The floating mass transducer of the Vibrant Soundbridge (VSB) was positioned in the stapes head by the Clip coupler. The safety of the VSB was investigated by comparing preoperative and postoperative bone-conduction (BC) thresholds as well as by complications. The effectiveness was evaluated by functional gain (FG), word recognition score (WRS), speech reception threshold (SRT) and signal-to-noise ratio (SNR). RESULTS: Pre- and post-operative BC thresholds were no different in all patients. And no complications developed. VSB-aided thresholds in the free-field had improved significantly in both short- and long-term follow-up groups. The improvements of WRS were observed in two groups. The monosyllabic VSB-aided WRS in the long-term follow-up group was significantly higher than that in the short-term follow-up group. When speech was from the impaired ear and noise presented to the side of normal ear (SVSBNCL), lower SNRs were found in two groups after VSB implantation. However, there was no statistical difference in aided SNR between the two groups at SVSBNCL status. CONCLUSIONS: Our results show that the FMT connected to the stapes head is a secure and useful device for patients with unilateral CHL/MHL, not only in terms of improved hearing thresholds, but also improved speech intelligibility in quiet and noisy environments.

Bone Conduction , Congenital Abnormalities/surgery , Ear/abnormalities , Hearing Loss, Conductive/surgery , Hearing Loss, Mixed Conductive-Sensorineural/surgery , Ossicular Replacement/methods , Stapes Surgery/methods , Transducers , Adolescent , Adult , Audiometry, Pure-Tone , Auditory Threshold , Child , Congenital Abnormalities/physiopathology , Congenital Microtia/complications , Ear/physiopathology , Ear/surgery , Female , Hearing Loss, Conductive/etiology , Hearing Loss, Conductive/physiopathology , Hearing Loss, Mixed Conductive-Sensorineural/etiology , Hearing Loss, Mixed Conductive-Sensorineural/physiopathology , Humans , Male , Retrospective Studies , Signal-To-Noise Ratio , Speech Reception Threshold Test , Treatment Outcome , Young Adult
Int J Pediatr Otorhinolaryngol ; 134: 110040, 2020 Jul.
Article En | MEDLINE | ID: mdl-32361150

OBJECTIVE: To characterize the use of bone conduction hearing devices (BCHD) for hearing management in children with unilateral congenital aural atresia (CAA) at a tertiary pediatric center's microtia clinic while assessing challenges in acquisition. BCHD generally provides better audiologic outcomes than atresiaplasty in pediatric CAA. BCHD is formally recommended for only bilateral CAA, although literature has begun to show benefit in unilateral CAA. METHODS: A retrospective case series of patients born between 2014 and 2018 with unilateral microtia at an urban tertiary care children's hospital collected information on demographics, CAA laterality, hearing loss (HL) severity, management, and acquisition. Statistical analysis aided characterization of BCHD use. RESULTS: 131 patients (65% males) were included with a mean age of 3.5 years. 108 (82%) patients with unilateral microtia were used for further analysis, and right sided microtia was most common (67.6%). Of patients with auditory brain response (ABR) testing available, 70% demonstrated conductive HL, 16% mixed HL, 1% sensorineural HL, 6% no HL secondary to grade 1 microtia, and 7% were pending evaluation. Overall, 46 (42.6%) patients with unilateral microtia obtained a BCHD. 68.7% of those offered a BCHD after ABR opted for acquisition. The average length of time from offer to fit was about 6 months. CONCLUSION: Even with thorough consultation and follow up, less than half of the patients with unilateral CAA received a BCHD. Missed initial ABR appointments that lead to BCHD recommendation seem to be the greatest limiting factor, while demographics did not play a confounding role. Family education and future research on obstacles preventing BCHD use in unilateral CAA will help standardize management and improve hearing within this critical auditory period.

Bone Conduction , Congenital Abnormalities/rehabilitation , Congenital Microtia/rehabilitation , Ear/abnormalities , Hearing Aids , Hearing Loss, Conductive/rehabilitation , Child, Preschool , Congenital Microtia/complications , Evoked Potentials, Auditory, Brain Stem , Female , Hearing Loss, Conductive/etiology , Hearing Loss, Sensorineural/complications , Hearing Loss, Sensorineural/rehabilitation , Hearing Tests , Humans , Infant , Male , Retrospective Studies
Acta Otolaryngol ; 140(8): 675-681, 2020 Aug.
Article En | MEDLINE | ID: mdl-32432498

Background: Bonebridge (BB) and bone conduction hearing aid (BCHA) are effective in patients with bilateral congenital microtia-atresia (CMA).Objectives: To investigate and compare the outcomes of these devices in a large sample size.Materials and methods: This single center prospective study involved 100 patients with bilateral CMA who were implanted with BBs and used BCHAs before implantation. Sound field threshold (SFT), speech reception thresholds (SRTs) and word recognition scores (WRSs) were compared between unaided, BCHA used and implanted patients. The Abbreviated Profile of Hearing Aid Benefit (APHAB) was used to evaluate subjective satisfaction.Results: Compared to unaided condition, the SFT, WRS and SRT of BCHA and BB were significantly improved. With BCHA or BB, the three subscale scores of the APHAB (ease of communication, background noise and reverberation) significantly reduced. However, the aversiveness subscale scored significantly higher than unaided condition. All outcomes were better in BB condition than BCHA.Conclusions: BB or BCHA use can be considered as effective methods to improve audiological outcomes and subjective satisfaction. Although not as good as BB, BCHA use is critical for improving hearing in the early period of language and auditory pathway development before the skull is suitable for BB implantation.

Congenital Microtia/surgery , Hearing Aids , Hearing Loss, Conductive/surgery , Patient Satisfaction , Audiometry, Pure-Tone , Audiometry, Speech , Bone Conduction , Congenital Abnormalities/surgery , Ear/abnormalities , Ear/surgery , Equipment Design , Hearing Loss, Conductive/rehabilitation , Humans , Prospective Studies , Surveys and Questionnaires
J Deaf Stud Deaf Educ ; 25(4): 411-420, 2020 09 10.
Article En | MEDLINE | ID: mdl-32432679

This study compared the language, reading, classroom, and quality of life outcomes of primary school-aged children with aural atresia (AA) to matched controls. Participants included 10 children with AA (eight unilateral) and 10 children with typical hearing matched by chronological and mental age. All children with AA had been fitted with an amplification device. Outcome measures included standardized tests of language, reading, and functional communication questionnaires of children's classroom performance and hearing quality of life. The children with AA recorded significantly reduced hearing quality of life. The two groups did not differ on any other measures. The present preliminary findings suggest that children with AA who receive early amplification have similar language, communication, reading, and classroom outcomes as their typically hearing peers. Despite these promising outcomes, however, the children's quality of life is significantly reduced. Further research is needed to further elucidate these findings.

Congenital Abnormalities/psychology , Ear/abnormalities , Education of Hearing Disabled , Educational Measurement , Case-Control Studies , Child , Education of Hearing Disabled/methods , Educational Measurement/methods , Female , Humans , Language Tests , Male , Quality of Life/psychology , Reading , Surveys and Questionnaires