[Hearing loss in patients with Fabry disease].
BACKGROUND
Fabry disease is an X-linked lysosomal storage disease involving deficient activity of alpha-galactosidase A, which leads initially to pain, and later to renal insufficiency, cardiomyopathy and stroke. Until now few details are available on hearing impairment in patients with Fabry disease, and especially few relating to female patients.


METHODS
We examined 43 female and 29 male patients. In this study we looked into the question of whether and to what extent patients of both genders are affected by hearing impairment.


RESULTS
Hearing loss is characteristic being more severe at high frequencies frequencies. Overall, 22 female and 15 male patients were found to have suffered a hearing loss. Patients with severe symptoms of Fabry disease usually demonstrate more prominent hearing losses.


CONCLUSIONS
Both men and women with Fabry disease are affected by hearing impairment. It seems that the hearing loss is less marked in female than in male patients. Children with Fabry disease complain of tinnitus more frequently than other children and quite early in the course of the disease.
Limberger
A
A
Klinik für HNO und Kommunikationsstörungen, Universitätsklinik Mainz, Mainz. limberger@kommunikation.klinik.uni-mainz.de
Beck
M
M
Delgado-Sanchez
S
S
Keilmann
A
A
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D000795:Fabry Disease / Q000175:diagnosis*; D005260:Female; D034381:Hearing Loss / Q000175:diagnosis*; D006320:Hearing Tests; D006801:Humans; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D012720:Severity of Illness Index; D012737:Sex Factors