Temperature-sensitive Recurrent, Reversible Auditory Neuropathy in Two Iranian Siblings

Two sibling cases with a temperature-sensitive form of auditory neuropathy from a consanguineous marriage of Iranian descent have been described. They complained about the temporary loss of hearing and compromised speech comprehension after a slight fever or elevating the body temperature by vigorous exercises. A series of tests including brain MRI, pure-tone audiometry, speech audiometry in quiet and noise, tympanometry, reflexometry, TEOAEs, cochlear microphonic, and ABR were performed in a 24-hours interval at both febrile and afebrile states, and results are reported here. This report is the first example of temperature-sensitive auditory neuropathy in this geographical region.