Pediatric fluctuating sensorineural hearing loss: problems in medical management.
OBJECTIVE
To discuss the diagnosis and management of children with fluctuating sensorineural hearing loss, especially focusing on those problems dealing with autoimmune inner ear disease.


METHODS
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.
Dinces
E A
EA
Department of Otolaryngology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York 10467, USA.
Yang
S
S
Balogun
A O
AO
D000293:Adolescent; D001299:Audiometry; D001309:Auditory Threshold / Q000502:physiology; D001327:Autoimmune Diseases / Q000150:complications; D015153:Blotting, Western; D001844:Bone Conduction / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004427:Ear Diseases / Q000150:complications; D004432:Ear, Middle / Q000473:pathology; D005402:Fistula / Q000150:complications; D005500:Follow-Up Studies; D018840:HSP70 Heat-Shock Proteins / Q000032:analysis; D006319:Hearing Loss, Sensorineural / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology / Q000628:therapy; D006801:Humans; D007223:Infant; D007759:Labyrinth Diseases / Q000150:complications; D008213:Lymphocyte Activation / Q000276:immunology; D010498:Perilymph; D012189:Retrospective Studies; D013067:Speech Perception / Q000502:physiology; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome
D018840:HSP70 Heat-Shock Proteins; D013256:Steroids