HYPERGONADOTROPIC HYPOGONADISM,
PROGRESSIVE EARLY-ONSET SPINOCEREBELLAR ATAXIA,
AND LATE-ONSET SENSORINEURAL HEARING LOSS:
CASE REPORT AND LITERATURE REVIEW
Sarikaya E,1 Ensert CG,2 Gulerman HC1
*Corresponding Author: Esma Sarikaya, Centre for Reproductive Medicine, Zekai Tahir Burak Women’s
Health Research and Education Hospital, Talatpasa Bulvari Hamamonu 06230, Ankara, Turkey; Tel.: +90-312-
310-3100; Fax: +90-312-312-4931; E-mail: sudesarikaya@hotmail.com
page: 77
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Abstract
The association of ataxia, hypergonadotropic hypogonadism
and hearing loss is extremely rare. Considerable
heterogeneity exists in the literature of the neurological
manifestations, age of onset, clinical severity
and associated abnormalities. We describe a 24-yearold
woman with secondary hypergonadotropic amenorrhea,
early-onset progressive spinocerebellar ataxia
(SCA), late-onset sensorineural hearing loss and normal
intelligence and compare it with reported cases.
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