Mediterr. J. Otol 2007; 3:(2)

Familial temperature-sensitive auditory neuropathy/auditory dyssynchrony

Nevma Madanoğlu, Ufuk Derinsu

Department of Audiology, Marmara University Medical School, İstanbul, Turkey. uderinsu@yahoo.com

Auditory neuropathy/auditory dyssynchrony is a hearing disorder characterized by impairment of neural synchrony in the presence of a normal cochlear outer hair cell function. Clinical findings are normal hearing or sensorineural hearing loss, absent/abnormal auditory brainstem response, normal otoacoustic emissions, normal cochlear microphonics, and poor speech perception abilities that are disproportionate to the hearing sensitivity.

We identified 5 patients, all related, with auditory neuropathy. Pure-tone audiometry, acoustic immittancemetry, evoked potentials, otoacoustic emission tests, and magnetic resonance imaging were performed on all of them. We followed the patients for 5 years.

We determined that their audiologic findings showed variations when they had influenza, due to high body temperature.