Temperature-dependent auditory neuropathy: is it an acoustic Uhthoff-like phenomenon? A case report.
OBJECTIVE
We describe the case of a young girl in whom transient deafness occurred when her core body temperature rose.


METHODS
The patient was referred for a series of audiological and neurologic evaluations performed over time in both afebrile and febrile states, as well as after a stress test (with a treadmill) in which the body temperature rise simulated the febrile state.


RESULTS
The patient was found to have a temporary bilateral hearing loss, but had normal distortion product otoacoustic emissions. Moreover, auditory brain stem responses revealed the absence of neural synchrony when her core body temperature increased.


CONCLUSIONS
These results are consistent with a temperature-dependent auditory neuropathy, a rare condition in which patients show normal outer hair cell function and abnormal neural function of the eighth cranial nerve. The symptom is reminiscent of Uhthoff's phenomenon, which is described as transient visual loss and is usually observed in multiple sclerosis. This case of temperature-dependent auditory neuropathy is noteworthy because it sheds light on a disorder of which there have been few reports in the literature. We discuss its similarity to Uhthoff's phenomenon.
Cianfrone
Giancarlo
G
Department of Otorhinolaryngology, Audiology and Phoniatrics "Giorgio Ferreri", University of Rome La Sapienza, v.le del Policlinico 155, 00185 Rome, Italy.
Turchetta
Rosaria
R
Mazzei
Filippo
F
Bartolo
Michelangelo
M
Parisi
Leoluca
L
D001301:Audiometry, Pure-Tone; D001306:Auditory Pathways / Q000503:physiopathology; D002648:Child; D005072:Evoked Potentials, Auditory; D005260:Female; D005334:Fever / Q000150:complications* / Q000503:physiopathology; D006319:Hearing Loss, Sensorineural / Q000209:etiology / Q000503:physiopathology*; D006801:Humans; D017084:Otoacoustic Emissions, Spontaneous / Q000502:physiology*; D000159:Vestibulocochlear Nerve / Q000503:physiopathology*