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Definition

A form of hearing loss characterized by absent or severely abnormal auditory brainstem response, abnormal middle ear reflexes, abnormal speech discrimination, loss of outer hair cell function, and cochlear nerve hypoplasia. DFNX5 patients manifest auditory neuropathy with childhood onset, associated with distal sensory impairment affecting the peripheral nervous system.

Acronym

DFNX5

Synonyms

Auditory neuropathy, X-linked, 1, with peripheral sensory neuropathy
AUNX1

Keywords

Deafness
Neuropathy