Neurophysiology of hearing in patients with mucopolysaccharidosis type IV.

ABSTRACT:

Background

Hearing impairment is a common problem in patients with mucopolysaccharidosis IV (MPS IV) throughout their life. Many of the adult patients with MPS IV exhibit permanent or severe hearing loss. However, there has been no systematic review of detailed audiological test results in MPS IV.

Materials and methods

Fourteen individuals with MPS IV (13 MPS IVA and 1 MPS IVB; aged between 12 and 38 years old) participated in the current study. We obtained auditory neurophysiological responses (auditory brainstem responses and otoacoustic emissions test) in addition to pure-tone audiometry and middle ear function tests (tympanometry and acoustic reflexes).

Results

The results indicated various levels and types of hearing loss with abnormal neurophysiological response

SUBMITTER: Nagao K 

PROVIDER: S-EPMC5891367 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature