<abstract xml:lang="en" xmlns="http://www.w3.org/1999/xhtml"><p>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-year-old 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.</p></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-year-old 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.

Hypergonadotropic Hypogonadism, Progressive Early-Onset Spinocerebellar Ataxia, and Late-Onset Sensorineural Hearing Loss: Case Report and Literature Review

Centre for Reproductive Medicine, Zekai Tahir Burak Women's Health Research and Education Hospital, Ankara, Turkey

Department of Neurology, Zekai Tahir Burak Women's Health Research and Education Hospital, Ankara, Turkey

Balkan Journal of Medical Genetics

The association of ataxia, hypergonadotropic hypogonadism and hearing loss is extremely rare. Considerable heterogeneity exists in the literature of the...

Hypergonadotropic Hypogonadism, Progressive Early-Onset Spinocerebellar Ataxia, and Late-Onset Sensorineural Hearing Loss: Case Report and Literature Review

Pubblicato online: 08 dic 2011

Pagine: 77 - 88

DOI: https://doi.org/10.2478/v10034-011-0050-z

Parole chiave
Cerebellar ataxia, Hearing loss, Sensorineural, Premature ovarian failure

This content is open access.

Hypergonadotropic Hypogonadism, Progressive Early-Onset Spinocerebellar Ataxia, and Late-Onset Sensorineural Hearing Loss: Case Report and Literature Review

Pubblicato online: 08 dic 2011

Pagine: 77 - 88

DOI: https://doi.org/10.2478/v10034-011-0050-z

Parole chiaveCerebellar ataxia, Hearing loss, Sensorineural, Premature ovarian failure

This content is open access.

Parole chiave
Cerebellar ataxia, Hearing loss, Sensorineural, Premature ovarian failure