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Updated: May 18, 2026
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Treating hearing loss in patients with infantile Bartter syndrome
Georgios Kontorinis1, Anja M Giesemann, Zoe Iliodromiti
1Department of Otolaryngology, Hanover Medical School, Hanover, Germany. gkontorinis@gmail.com
Insights
Infantile Bartter syndrome (BS) causes congenital, profound hearing loss. Cochlear implantation offers some benefits, but delayed treatment and comorbidities negatively impact speech development in children with BS.
Area of Science:
- Pediatric Nephrology
- Otolaryngology
- Genetics
Background:
- Infantile Bartter syndrome (BS) is primarily studied for renal dysfunction, with limited focus on associated hearing impairment.
- Hearing loss in BS is a significant but under-researched comorbidity.
Purpose of the Study:
- To evaluate hearing loss treatment in children with infantile BS.
- To analyze factors affecting outcomes of hearing impairment management, including cochlear implantation.
Main Methods:
- Retrospective chart review of children diagnosed with infantile BS over a 20-year period.
- Evaluation of demographic data, renal status, genetic information, hearing loss characteristics, and cochlear implant outcomes (Categories of Auditory Performance - CAP).
- Analysis of temporal bone imaging and correlation with clinical findings.
Main Results:
- Six children with infantile BS (4 female, 2 male) were identified, all with congenital, bilateral, profound sensorineural hearing loss.
- Five patients received cochlear implants, achieving moderate improvements in speech perception (CAP scores 4-6) attributed to delayed treatment and comorbidities.
- No inner ear malformations were detected; renal dysfunction was a significant comorbidity in all patients.
Conclusions:
- Hearing loss in infantile BS is congenital and profound, unrelated to inner ear malformations.
- Cochlear implantation provides some benefit, but overall health status and delayed intervention negatively impact speech development.
- Further research is needed to optimize management strategies for hearing loss in infantile BS.
Objectives/Hypothesis:
Most existing studies about infantile Bartter syndrome (BS) have focused on renal function, and deafness has not been closely studied. Our objective was to evaluate the treatment of hearing impairment in children with infantile BS and analyze relevant, unexplored issues.
Study Design:
Retrospective chart review.
Methods:
The present study was conducted in a tertiary referral center over a 20-year period involving children with infantile BS. Demographic factors, general health status, genetic information, features of hearing loss, and the outcome of cochlear implantation as determined mainly by the categories of auditory performance (CAP), as well as imaging of the temporal bones, were evaluated.
Results:
Six children with infantile BS were identified, four girls and two boys. One child had terminal renal insufficiency and one had undergone kidney transplantation; all children had several hospital admissions due to renal dysfunction. Sensorineural hearing loss was congenital, bilateral, and profound in all children. Five patients were treated with cochlear implants resulting in improved speech perception and development without any exceptional performance (CAP scores, 4-6), mainly because of the delayed treatment and the comorbidities. Anatomic ear anomalies were not observed in any case.
Conclusions:
Hearing loss in children with infantile BS is congenital and profound but not related to inner ear malformations. Although cochlear implantation results in certain benefits, general health status and delayed referral to cochlear implant centers have a negative impact on speech perception and development.
