Temperature-dependent auditory neuropathy: is it an acoustic Uhthoff-like phenomenon? A case report.

Ann Otol Rhinol Laryngol

Department of Otorhinolaryngology, Audiology and Phoniatrics "Giorgio Ferreri", University of Rome La Sapienza, v.le del Policlinico 155, 00185 Rome, Italy.

Published: July 2006

AI Article Synopsis

  • A young girl experienced temporary deafness related to increased body temperature, prompting a series of audiological and neurologic tests.
  • The audiological evaluations showed a temporary bilateral hearing loss with normal outer hair cell function but abnormal responses in the auditory brain stem when her temperature rose.
  • This case highlights a rare condition called temperature-dependent auditory neuropathy, akin to Uhthoff's phenomenon, and adds valuable insights to the limited literature on this disorder.

Article Abstract

Objectives: 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.

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http://dx.doi.org/10.1177/000348940611500706DOI Listing

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