Diagnosis and treatment of congenital stenosis of external auditory canal with external auditory canal cholesteatoma complicated with exudative effusion in the middle ear and mastoid process.

BACKGROUND: The optimal timing for surgery to promote postoperative recovery in children with congenital stenosis of the external auditory canal with external auditory canal cholesteatoma, who are susceptible to exudative inflammation of middle ear and mastoid process, is still uncertain. OBJECTIVES: To investigate the treatment of congenital stenosis of external auditory canal with external auditory canal cholesteatoma complicated with exudative inflammation. MATERIAL AND METHODS: A retrospective analysis of 45 patients with congenital stenosis of external auditory canal with external auditory canal cholesteatoma complicated with mastoiditis. Based on whether effusion had resolved at the time of surgery, the patients were divided into two groups. Pure-tone audiometry tests were performed before and after surgery for both groups. RESULTS: The average postoperative duration of dry ear was 36.38 days in Group 1 and 47.90 days in Group 2 (p < 0.05). Among patients who underwent hearing reconstruction, the average air conduction threshold decreased by 15-36 dBHL in Group 1 and by 7-22 dBHL in Group 2 (p < 0.05). CONCLUSIONS AND SIGNIFICANCE: The postoperative outcomes of dry ear and hearing improvement can be enhanced by performing operation after effusion resolution in cases of congenital external auditory canal stenosis with external auditory canal cholesteatoma exudative inflammation.

Association Between Maternal Exposure to SO2 and Congenital Ear Malformations in Offspring: A Population-Based Case-Control Study in Liaoning Province, China.

Objectives: To examine associations between maternal sulfur dioxide (SO2) exposure and congenital ear malformations risk in offspring. Methods: We surveyed 1676 cases with congenital ear malformations and 7950 controls from the Maternal and Child Health Certificate Registry of Liaoning Province between 2010 and 2015. SO2 concentrations were obtained from the Municipal Environment Protection Bureau of Liaoning Province. Multivariable logistic regression models and Restricted cubic splines (RCS) model were used to assess the aforementioned association. Results: There were significant associations between maternal SO2 exposure and congenital ear malformations risk during the 3 months before conception (OR Q4 vs. Q1 = 1.93, 95% CI = 1.43-2.59) and the 3 months after conception (OR Q4 vs. Q1 = 1.63, 95% CI = 1.22-2.18). Similar results were obtained in the analysis of single-month exposure windows, except for the third month before conception and the third month after conception. Moreover, these findings were broadly consistent across subgroups and robust in sensitivity analyses. There were non-linear dose-response associations between SO2 exposure and congenital ear malformations based on restricted cubic spline model analysis. Conclusion: Maternal SO2 exposure is associated with increased congenital ear malformations risk in offspring.

Bone-conduction hearing aid is effective in congenital oval window atresia.

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.

Down syndrome: an electrophysiological and radiological profile.

OBJECTIVES/HYPOTHESIS: To assess the hearing status of trisomy 21 patients by analyzing electrophysiological and radiological findings of any correlation between hearing impairment and major or minor inner ear malformations. STUDY DESIGN: Prospective radiological and electrophysiological study. METHODS: A group of 34 ears of Down syndrome subjects and 20 ears of a volunteer age- and sex-matched control group of 10 normal subjects were studied electrophysiologically by means of otoacoustic emissions and auditory brainstem response. Temporal bone computed tomography (CT) scans were carried out in both groups; radiological findings were compared. Inner ear structure measurements were applied attempting to disclose subtle bony labyrinthine anomalies. The findings from both groups were statistically analyzed employing the t test. RESULTS: The rate of sensorineural hearing loss (SNHL) in Down syndrome group was 41%. Temporal bone CT scans showed no ossicular malformation in all Down syndrome cases. Major inner ear abnormalities were disclosed in 5.8%; they corresponded to two cases of lateral semicircular canal dysplasia detected on CT images by visual inspection. The application of various inner ear structure measurements increased the overall detection rate of common inner ear malformations to 47%. A statistically significant correlation was found between hearing level and vestibule length (P = .009) and internal auditory canal length (P = .028). Vestibular aqueduct width was correlated to different otic abnormalities. CONCLUSIONS: SNHL is mainly secondary to the underestimated subtle inner ear malformations that are adequately demonstrated by adopting standardized inner ear structure measurements on petrous bone imaging. Vestibular height and internal auditory canal length were correlated to SNHL. LEVEL OF EVIDENCE: 3b.