Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency

Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency Publisher

Talebi H¹ ; Yaghini O²

Show Affiliations

1. Department of Audiology, Faculty of Rehabilitation, Communication Disorders Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
2. Department of Pediatrics, School of Medicine, Growth and Development Research Center, Isfahan University of Medical Sciences, Isfahan, Iran

Source: Journal of Audiology and Otology Published:2016

Abstract

Biotinidase deficiency is a disorder inherited autosomal recessively showing evidence of hearing loss and optic atrophy in addition to seizures, hypotonia, and ataxia. In the present study, a 2-year-old boy with Biotinidase deficiency is presented in which clinical symptoms have been reported with auditory neuropathy/auditory dyssynchrony (AN/AD). In this case, transient-evoked otoacoustic emissions showed bilaterally normal responses representing normal function of outer hair cells. In contrast, acoustic reflex test showed absent reflexes bilaterally, and visual reinforcement audiometry and auditory brainstem responses indicated severe to profound hearing loss in both ears. These results suggest AN/AD in patients with Biotinidase deficiency. © 2016 The Korean Audiological Society.

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Mohammad Amin Tabatabaiefar (4)

Hossein Talebi (2)

Style	Citing Format
MLA	Talebi H, Yaghini O. "Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency." Journal of Audiology and Otology, vol. 20, no. 1, 2016, pp. 53-54.
APA	Talebi H, Yaghini O (2016). Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency. Journal of Audiology and Otology, 20(1), 53-54.
Chicago	Talebi H, Yaghini O. "Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency." Journal of Audiology and Otology 20, no. 1 (2016): 53-54.
Harvard	Talebi H, Yaghini O (2016) 'Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency', Journal of Audiology and Otology, 20(1), pp. 53-54.
Vancouver	Talebi H, Yaghini O. Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency. Journal of Audiology and Otology. 2016;20(1):53-54.
BibTex	@article{ author = {Talebi H and Yaghini O}, title = {Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency}, journal = {Journal of Audiology and Otology}, volume = {20}, number = {1}, pages = {53-54}, year = {2016} }
RIS	TY - JOUR AU - Talebi H AU - Yaghini O TI - Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency JO - Journal of Audiology and Otology VL - 20 IS - 1 SP - 53 EP - 54 PY - 2016 ER -

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