Journal of Otology

Journal of Otology

Volume 11, Issue 4, December 2016, Pages 185-191
Journal of Otology

Auditory neuropathy in a patient with hemochromatosis

https://doi.org/10.1016/j.joto.2016.10.002Get rights and content
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Abstract

Objective

To evaluate the auditory function of an individual with genetically confirmed hemochromatosis.

Methods

A 57 year old male with mildly impaired sound detection thresholds underwent a range of behavioural, electroacoustic and electrophysiologic assessments. These included the recording of otoacoustic emissions and auditory brainstem responses, measurement of monaural temporal resolution and evaluation of binaural speech processing. Findings for this patient were subsequently compared with those of 80 healthy controls with similar audiometric thresholds.

Results

The patient showed the three cardinal features of auditory neuropathy, presenting with evidence of normal cochlear outer hair cell function, disrupted neural activity in the auditory nerve/brainstem and impaired temporal processing. His functional hearing ability (speech perception) was significantly affected and suggested a reduced capacity to use localization cues to segregate signals in the presence of background noise.

Conclusion

We present the first case of an individual with hemochromatosis and auditory neuropathy. The findings for this patient highlight the need for careful evaluation of auditory function in individuals with the disorder.

Keywords

Hemochromatosis
Auditory neuropathy
Temporal processing
Speech perception

Cited by (0)

Peer review under responsibility of PLA General Hospital Department of Otolaryngology Head and Neck Surgery.