Related Experiment Video
Updated: May 13, 2026
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Mixed hearing loss in children: Etiology, management, and audiological outcomes
Mirko Aldè1, Chiara Rasom2, Ludovica Sara Zanon2
1Department of Clinical Sciences and Community Health, Department of Excellence 2023-2027, University of Milan, Milan, Italy; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Department of Specialist Surgical Sciences, Audiology Unit, Milan, Italy.
Insights
This study highlights that pediatric mixed hearing loss (MHL) is diverse and often linked to genetic syndromes. Personalized management and long-term follow-up are crucial for improving hearing outcomes in children with MHL.
Area of Science:
- Pediatric Audiology
- Genetics
- Otolaryngology
Background:
- Mixed hearing loss (MHL) in children, combining conductive and sensorineural components, requires further characterization.
- Understanding the etiology and outcomes of pediatric MHL is critical for effective intervention.
Purpose of the Study:
- To investigate the causes, audiological features, treatments, and 1-year hearing results in children diagnosed with MHL.
- To characterize the heterogeneity of pediatric MHL and its association with genetic factors.
Main Methods:
- Retrospective review of medical charts for children aged 5-12 years with MHL.
- Analysis of demographic, clinical, audiological, radiological, and genetic data from a tertiary audiology center.
Main Results:
- Pathogenic genetic variants were found in 38.8% of pediatric MHL cases, often associated with syndromes like Branchio-Oto-Renal and Down syndrome.
- Syndromic MHL was linked to higher rates of otologic malformations and recurrent otitis media.
- Hearing improved significantly after interventions including hearing devices and middle ear surgery, with a mean pure-tone average improvement of 5.7 dB HL.
Conclusions:
- Pediatric MHL is a heterogeneous condition frequently associated with genetic syndromes.
- Effective management necessitates long-term audiological monitoring and individualized treatment plans.
Objectives:
Mixed hearing loss (MHL), defined as the coexistence of conductive and sensorineural components, remains insufficiently characterized in the pediatric population. This study aimed to investigate the etiology, audiological characteristics, management strategies, and 1-year hearing outcomes in children with MHL.
Methods:
The medical charts of children aged 5-12 years with HL referred to a tertiary-level audiologic center between January 1, 2017, and August 31, 2025, were reviewed. The study specifically included children with MHL, and demographic, clinical, audiological (baseline and 1-year follow-up), radiological, and genetic data were analyzed.
Results:
Of 5618 first visits for pediatric HL, 129 children (mean age: 88.3 ± 26.0 months; 53.5% male) met the inclusion criteria. Pathogenic genetic variants were identified in 50 children (38.8%), with most cases (88.0%) associated with syndromic conditions, primarily Branchio-Oto-Renal, CHARGE, Down, and Pendred syndromes. Children with syndromic MHL showed a significantly higher prevalence of otologic malformations (72.7% vs. 40.0%, P < 0.001) and chronic or recurrent otitis media (84.1% vs. 23.5%, P < 0.001). Treatment of MHL included hearing devices in 57.4% of cases and middle ear surgery in 34.9%. The mean pure-tone average (PTA-4) improved significantly from 60.3 ± 19.3 dB HL at baseline to 54.6 ± 25.8 dB HL at the 1-year follow-up (P < 0.001).
Conclusion:
This study is the first to focus exclusively on pediatric MHL and demonstrates that MHL is a highly heterogeneous condition frequently associated with genetic syndromes. Long-term audiological follow-up and a personalized therapeutic approach are essential for the optimal management of children with MHL.
Related Concept Videos
01:31Hearing
01:11The Auditory Ossicles
The aptly named stapes look very much like a stirrup. The three ossicles are unique to mammals, and each plays a role in...