Clinical and genetic features of hearing loss in facioscapulohumeral muscular dystrophy.

ABSTRACT:

Objective

To describe the hearing loss in facioscapulohumeral muscular dystrophy (FSHD) and examine the relationship to genotype.

Methods

Medical records of all individuals with FSHD seen at the University of Iowa neuromuscular clinic between July 2006 and July 2012 (n = 59) were reviewed. Eleven had significant hearing loss and no non-FSHD cause. All available audiology records for these individuals were analyzed. The relationship between the FSHD mutation (EcoRI/BlnI fragment size) and hearing loss was evaluated using a logistic regression analysis.

Results

In patients with hearing loss, recalled age at onset of facial weakness ranged from birth to 5 years and shoulder weakness was 3 to 15 years. The age at diagnosis of hearing loss ranged from birth to 7 years. Onl

SUBMITTER: Lutz KL 

PROVIDER: S-EPMC3806909 | biostudies-literature | 2013 Oct

REPOSITORIES: biostudies-literature