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Updated: Aug 17, 2026
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Pediatric fluctuating sensorineural hearing loss: problems in medical management
E A Dinces1, S Yang, A O Balogun
1Department of Otolaryngology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York 10467, USA.
Insights
Most pediatric fluctuating sensorineural hearing loss (FSNHL) is idiopathic. Autoimmune inner ear disease (AIED) was suggested in some cases, but treatment was often delayed or not pursued.
Area of Science:
- Pediatric Otolaryngology
- Audiology
- Immunology
Background:
- Fluctuating sensorineural hearing loss (FSNHL) in children presents diagnostic and management challenges.
- Autoimmune inner ear disease (AIED) is a potential, though less common, cause of pediatric hearing loss.
Purpose of the Study:
- To review the diagnosis and management of pediatric FSNHL.
- To specifically investigate cases potentially related to AIED.
Main Methods:
- Retrospective chart review of 40 children with progressive hearing loss.
- Identified 22 children meeting criteria for FSNHL (threshold variations ≥15 dB).
- Analyzed audiology, medical history, and laboratory data.
Main Results:
- 15 of 22 FSNHL cases were idiopathic.
- 3 cases showed positive lymphocyte transformation tests (LTT) suggestive of AIED.
- 4 cases had middle ear fistula; average fluctuation was 29.1 dB over 4.95 years.
Conclusions:
- Idiopathic causes dominate pediatric FSNHL.
- Limited treatment was initiated for suspected AIED despite positive LTTs, often due to parental/physician reluctance or loss to follow-up.
- Significant audiometric fluctuations were observed across all identified groups.
Objectives:
To discuss the diagnosis and management of children with fluctuating sensorineural hearing loss, especially focusing on those problems dealing with autoimmune inner ear disease.
Study Design:
A retrospective chart review of a large pediatric otolaryngology practice. A series of 40 children with progressive hearing losses was identified. Of that group, 22 children, aged 1.5 to 12.2 years at first audiogram, were considered to have fluctuating sensorineural hearing loss (FSNHL). Criteria for inclusion in the FSNHL group were threshold variations of 15 dB or more in at least one ear at two or more of the standard audiometric frequencies on at least 2 testing days.
Methods:
Charts were reviewed for age, sex, otologic history, laboratory evaluations, medical or surgical treatments, significant medical history, and family medical history.
Results:
Twenty-two children met the criteria for fluctuating sensorineural hearing loss. Of those with fluctuating hearing loss, 15 were idiopathic, 3 had positive lymphocyte transformation tests (LTT) suggestive of autoimmune inner ear disease (AIED), and 4 had fistula on middle ear exploration. Average fluctuation for all groups was 29.1 dB. Average duration of fluctuations was 4.95 years.
Conclusions:
The majority of pediatric FSNHL cases (15 of 22) were idiopathic in nature. Of those FSNHL children with positive LTTs, only one was treated with steroid therapy. In the other patients with positive LTTs, parents or other physicians were often reluctant to treat, or the patient was lost to follow-up. Mean fluctuations varied substantially across all standard audiometric frequencies for all groups.
