Audiometric and Otologic Findings in Children and Young Adults with Neurofibromatosis Type 1 and Plexiform Neurofibromas.

ABSTRACT:

Objectives

To characterize otologic and audiologic manifestations in our NF1 cohort and explore the relationship between otologic and audiologic findings in a subset of patients with ear-related plexiform neurofibromas (PNs).

Methods

Audiologic and otologic clinical evaluations were conducted on 102 patients with NF1 in a natural history study (5-45 years; M = 14.4 years; Mdn = 14). Testing included pure tone and speech audiometry, middle ear function, neurodiagnostic auditory brainstem response (ABR), auditory processing, and MRIs of the head and neck region. Patients referred to this study had an overall higher incidence and burden of PNs than the overall NF1 population.

Results

The majority of subjects in this cohort had normal hearing sensitivity (81%) and normal

SUBMITTER: Idowu V 

PROVIDER: S-EPMC10354688 | biostudies-literature | 2023 Oct

REPOSITORIES: biostudies-literature