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Pediatric Meniere's disease
Chi Wang1, Chien-Hao Wu2, Po-Wen Cheng2
1Department of Otolaryngology, National Taiwan University Hospital, Taipei, Taiwan.
Insights
Pediatric Meniere
Area of Science:
- Otolaryngology
- Pediatric Neurology
- Vestibular System Disorders
Background:
- Meniere's disease (MD) is a rare inner ear disorder.
- Pediatric MD accounts for only 2.3% of all MD cases.
- Understanding the progression of inner ear deficits in children is crucial.
Purpose of the Study:
- To investigate the sequence of inner ear deficits in pediatric Meniere's disease.
- To utilize a comprehensive inner ear test battery including audiometry, oVEMP, cVEMP, and caloric tests.
- To compare the deficit sequence in children with that observed in adults.
Main Methods:
- A cohort of 24 children (<15 years) with MD was studied between 2005 and 2016.
- Included otoscopy, CT scan, blood tests, and an inner ear test battery.
- Assessed hearing, cochlear (cVEMP), saccular (oVEMP), and semicircular canal (caloric) function.
Main Results:
- Inner ear deficits progressed from cochlear (hearing loss) to saccular (cVEMP), then utricular (oVEMP), and finally semicircular canals (caloric tests).
- Abnormalities were observed in 25% for hearing, 20% for cVEMP, 13% for oVEMP, and 12% for caloric tests.
- A positive correlation was found between family history and laterality of the disease.
Conclusions:
- Pediatric Meniere's disease exhibits a distinct sequence of inner ear dysfunction, mirroring adult patterns.
- The observed sequence involves cochlear, saccular, utricular, and semicircular canal deficits.
- A positive family history in one-third of cases may contribute to bilateral and symmetrical Meniere's disease in children.
Objective:
This study adopted an inner ear test battery comprising audiometry, and ocular vestibular evoked myogenic potential (oVEMP), cervical VEMP (cVEMP), and caloric tests to investigate the sequence of inner ear deficits in pediatric Meniere's disease (MD).
Methods:
From 2005 to 2016, a total of 24 MD children aged <15 years old underwent otoscopy, CT scan, blood test, and an inner ear test battery. Nine subjects were males and 15 were females, with mean age of 12 years. Right ear was affected in 3 patients, left ear in 5 patients, and both ears in 16 patients (67%). Eight (33%) of the 24 MD children had positive family history.
Results:
Inner ear deficits in MD children ran from abnormal hearing (25%) to abnormal cVEMP (20%), oVEMP (13%), and caloric (12%) tests, exhibiting a significantly declining sequence from the cochlea, to the saccule, utricle and semicircular canals. Analysis between the laterality and family history revealed a significantly positive correlation.
Conclusion:
Pediatric MD is rare, accounting for 2.3% prevalence in MD patients. The inner ear deficits in pediatric MD showed a significantly declining sequence from the cochlea, to the saccule, utricle and semicircular canals, mimicking the sequence in adult MD. One-third MD children had positive family history, which may explain the high rate of bilateral affliction and symmetrical hearing levels on both ears.