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Neuroaudiologic abnormalities in patients with type 1 neurofibromatosis
M L Pensak1, R W Keith, P S Dignan
1University of Cincinnati Medical Center, Department of Otolaryngology, OH 45267.
The Laryngoscope
|July 1, 1989
Summary
Neurofibromatosis in children often presents with early neurotologic issues. Auditory Brainstem Response (ABR) and acoustic reflex testing revealed significant abnormalities in 32% of pediatric neurofibromatosis patients.
Area of Science:
- Pediatric Neurology
- Neuro-otology
- Genetics
Background:
- Neurofibromatosis (NF) is a genetic disorder with diverse symptoms.
- Early neurotologic abnormalities in pediatric NF are poorly understood.
- Limited research exists on auditory evoked potentials in young NF patients.
Purpose of the Study:
- To investigate the prevalence of neurotologic abnormalities in children with neurofibromatosis.
- To assess the utility of Auditory Brainstem Response (ABR) and acoustic reflex testing in this population.
Main Methods:
- A standardized neuroaudiologic diagnostic battery was administered.
- 44 children diagnosed with neurofibromatosis were included.
- Auditory Brainstem Response (ABR) and acoustic reflex dynamic tests were performed.
Main Results:
- 32% of the pediatric neurofibromatosis patients exhibited significant abnormalities.
- This prevalence is higher than previously reported.
- Abnormalities were detected using ABR and acoustic reflex testing.
Conclusions:
- Early neurotologic screening is crucial for pediatric neurofibromatosis.
- ABR and acoustic reflex testing are valuable diagnostic tools.
- Findings may inform updated evaluation and management protocols for pediatric NF.