The cochlear microphonic is a receptor potential believed to be generated primarily by outer hair cells. Its detection in surface recordings has been considered a distinctive sign of outer hair cell integrity in patients with auditory neuropathy. This report focuses on the results of an analysis performed on cochlear microphonic recorded by transtympanic electrocochleography in response to clic...

A pontine hemorrhage can evoke several neurological symptoms because the pons contains various nuclei and nerve fibers. Hearing loss can develop as a result of a pontine hemorrhage because there is an auditory conduction pathway in the cochlear nucleus of the pons. However, very few cases of hearing loss caused by pontine lesions have been reported, and there have been no reports of auditory ne...

Auditory neuropathy is a rare form of deafness characterized by an absent or abnormal auditory brainstem response with preservation of outer hair cell function. We have identified Diaphanous homolog 3 (DIAPH3) as the gene responsible for autosomal dominant nonsyndromic auditory neuropathy (AUNA1), which we previously mapped to chromosome 13q21-q24. Genotyping of additional family members narrow...

Auditory neuropathy spectrum disorder is a clinical disorder where the outer hair cell functioning is intact but the functioning of the auditory nerve is affected. Since, the 8(th) nerve is constituted by both the auditory and vestibular branch of nerve fibers, there are chances that the vestibular nerve might also be affected. Hence, the current study was carried out in order to determine the ...

Function of the olivocochlear reflex, measured by suppression of transient evoked otoacoustic emissions, is assessed in nine patients with bilateral auditory neuropathy/dys-synchrony and compared to matched control subjects with normal auditory function. TEOAEs were acquired using 65 dB peak sound pressure linear clicks with and without the presence of broad-band noise presented binaurally, ips...

OBJECTIVE To investigate auditory neural involvement in patients with Leber's hereditary optic neuropathy (LHON). METHODS Auditory assessment was undertaken in two patients with LHON. One was a 45 year old woman with Harding disease (multiple-sclerosis-like illness and positive 11778mtDNA mutation) and mild auditory symptoms, whose auditory function was monitored over five years. The other wa...

The terms auditory neuropathy/dys-synchrony (AN) and auditory dys-synchrony (AD) have been used to describe a form of hearing impairment in which cochlear amplification (outer hair cell) function is normal but afferent neural conduction in the auditory pathway is disordered (Starr et al., 1996; Berlin et al., 2001). This paper provides an overview of the clinical features associated with this c...