Related Experiment Video
Updated: Jul 28, 2025
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Co-Occurrence of Sensorineural Hearing Loss and Congenital Heart Disease: Etiologies and Management
Tengyu Yang1, Xinmiao Fan1, Yue Fan1
1Department of Otolaryngology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
Insights
Sensorineural hearing loss (SNHL) and congenital heart disease (CHD) co-occur rarely. Cochlear implants (CI) in children with both conditions showed varied outcomes, highlighting the need for timely hearing intervention and cardiac care.
Area of Science:
- Pediatrics
- Genetics
- Otolaryngology
Background:
- Sensorineural hearing loss (SNHL) and congenital heart disease (CHD) often co-occur, presenting complex diagnostic and therapeutic challenges.
- Understanding the underlying etiologies and clinical profiles is crucial for effective management.
Purpose of the Study:
- To investigate the etiologies, clinical characteristics, and outcomes of cochlear implantation (CI) in children with co-occurring SNHL and CHD.
- To assess the impact of early hearing intervention and cardiac management on patient outcomes.
Main Methods:
- A retrospective analysis of children with SNHL and CHD who received CI at a tertiary hospital (2016-2021).
- A comprehensive literature review was conducted to identify and analyze additional cases.
- Data included genetic and non-genetic etiologies, SNHL severity, CI timing, and developmental outcomes.
Main Results:
- Out of 382 CI recipients, 8 children (2.1%) had co-occurring SNHL and CHD. The literature review identified 1525 additional patients, totaling 1533.
- Common genetic causes included CHARGE syndrome (36.3%) and Turner syndrome (8.4%). Non-genetic causes included congenital rubella syndrome (22.9%).
- Most patients had congenital, bilateral, severe-to-profound SNHL requiring early CI. Half of the 126 children who received CI showed delayed speech development.
Conclusions:
- Co-occurring SNHL and CHD is a rare condition with diverse etiologies, necessitating a multidisciplinary approach.
- Timely hearing intervention, long-term follow-up, and appropriate cardiac surgery timing are vital for optimizing outcomes in affected children.
Objectives/Hypothesis:
The co-occurrence of sensorineural hearing loss (SNHL) and congenital heart disease (CHD) is a rare condition with complex etiologies. The purpose of this study is to assess the etiologies, clinical features, and outcomes of cochlear implant (CI) in this patient population.
Study Design:
Case series and literature review.
Methods:
Clinical data of children who were diagnosed with SNHL and CHD and received CIs at a tertiary hospital from 2016 to 2021 were retrospectively analyzed. A literature review was performed to identify patients with SNHL and CHD.
Findings:
Of the 382 children who underwent cochlear implantation at our center, eight (2.1%) were diagnosed with SNHL and CHD. A literature review identified 1525 patients from 254 studies; the database therefore consisted of 1533 patients. The most common genetic etiologies of co-occurring SNHL and CHD were CHARGE syndrome (36.3%), Turner syndrome (8.4%), 22q11.2 deletion (3.0%), Noonan syndrome (2.9%), and Down syndrome (2.5%), whereas the most common non-genetic etiologies were congenital rubella syndrome (22.9%) and SNHL after early cardiac surgery (5.5%). Most of the patients presented with congenital, bilateral, severe-profound SNHL requiring early rehabilitation. Of the 126 children who received CIs at a median age of 2.5 years, half showed delayed speech development at last follow-up.
Conclusions:
Co-occurring SNHL and CHD is a rare condition with complex etiologies. Timely hearing intervention with long-term follow-up and proper timing of heart surgery is essential for these children.
Level Of Evidence:
4, case series Laryngoscope, 134:400-409, 2024.
Related Concept Videos
01:26Mitral Stenosis III: Medical Management
01:25Mitral Regurgitation III: Medical Management
01:28Mitral Regurgitation IV: Nursing Management
01:27Mitral Stenosis IV: Nursing Management
01:20Rheumatic Heart Disease IV: Nursing Management
01:21Rheumatic Heart Disease III: Medical Management