Auditory neuropathy in Friedreich ataxia: A report of two cases

International Journal of Pediatric Otorhinolaryngology

Volumn 67, Issue 6, 2003, Pages 641-648

  López Díaz De León, Elizabeth ^a   Silva Rojas, Andrés ^a   Ysunza, Antonio ^b   Amavisca, Raúl ^a   Rivera, Refugio ^a  

^a HOSPITAL GENERAL DE MÉXICO   (Mexico)

^b HOSPITAL GENERAL DR MANUEL GEA GONZÁLEZ   (Mexico)

Author keywords

Auditory neuropathy; Brainstem auditory evoked potentials; Friedreich ataxia; Otoacoustic emissions

Indexed keywords

ADOLESCENT; ADULT; ANAMNESIS; ARTICLE; BABINSKI REFLEX; CALORIC VESTIBULAR TEST; CASE REPORT; CEREBELLUM DISEASE; CLINICAL EXAMINATION; COCHLEAR NERVE; ELECTROCARDIOGRAM; ELECTRONYSTAGMOGRAPHY; EVOKED BRAIN STEM RESPONSE; EVOKED SOMATOSENSORY RESPONSE; FEMALE; FRIEDREICH ATAXIA; HUMAN; MOTOR NERVE CONDUCTION; NEUROPATHY; NUCLEAR MAGNETIC RESONANCE IMAGING; OTOACOUSTIC EMISSION; PERCEPTION DEAFNESS; PHYSICAL EXAMINATION; PRIORITY JOURNAL; PURE TONE AUDIOMETRY; STEREOSCOPIC VISION; VIBRATION;

EID: 0038329099     PISSN: 01655876     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0165-5876(03)00036-3     Document Type: Article

References (14)

1. Dürr A., Cossee M., Agid Y., Campuzano V., Mignard C., Penet C., et al. Clinical and genetic abnormalities in patients with Friedreich ataxia. N. Engl. J. Med. 335:1996;1169-1175.
2. Pandolfo M. Molecular pathogenesis of Friedreich ataxia. Arch. Neurol. 56:1999;1201-1208.
3. Campuzano V., Montermini L., Moltó M. Friedreich ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science. 271:1996;1423-1427.
4. Harding A. Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clusterin of clinical features. Brain. 104:1981;589-620.
5. Shanon E., Himelfarb M., Gold S. Auditory function in Friedreich's ataxia. Arch. Otolaryngol. 107:1981;254-256.
6. Jabbari B., Schwartz D., MacNeil D., Coker S. Early abnormalities of brainstem auditory evoked potentials in Friedreich's ataxia: evidence of primary brainstem dysfunction. Neurology. 33:1983;1071-1074.
7. Ell J., Prasher D., Rudge P. Neuro-otological abnormalities in Friedreich's ataxia. J. Neurol. Neurosurg. Psychiatry. 47:1984;26-32.
8. Satya-Murti S., Cacace A., Hanson P. Auditory dysfunction in Friedreich ataxia: result of spiral ganglion degeneration. Neurology. 30:1980;1047-1053.
9. Starr A., Picton T., Sininger Y., Hood L., Berlin C. Auditory neuropathy. Brain. 119:1996;741-753.
10. Berlin C., Hood L., Hurley A., Wen H. Contralateral supression of otoacoustic emissions: an index of the function of the medial olivocochlear system. Arch. Otolaryngol. Head Neck Surg. 110:1994;3-21.
11. Baloh R., Konrad H., Honrubia V. Vestibulo ocular function in patients with cerebellar atrophy. Neurology. 25:1975;160-168.
12. Fujikawa S., Starr A. Vestibular neuropathy accompanyng auditory and peripheral neuropathies. Arch. Otolaryngol. Head Neck Surg. 126:2000;1453-1456.
13. Spöndlin H. Optic and cochleovestibular degenerations in hereditary ataxias. Brain. 97:1974;41-48.
14. Collet L., Veuillet E., Bene J., Morgon A. Effects of contralateral white noise on click-evoked emissions in normal and sensorineural ears: towards an exploration of the medial olivocochlear system. Audiology. 31:1992;1-7.