NDLI: Brainstem auditory evoked potentials and cochlear microphonics in the HMSN family with auditory neuropathy

Content Provider	Springer Nature Link
Author	Butinar, Dušan Starr, Arld Vatovec, Jagoda
Copyright Year	2000
Abstract	The aim of this work was to assess the hearing impairment in patients with hereditary motor and sensory neuropathy (HMSN). Elevation of pure tone thresholds in the presence of preserved inner ear function as suggested by cochlear microphonics (CM), absent or markedly abnormal brainstem auditory evoked potentials (BAEP), and elevation of speech perception out of proportion to the pure tone loss were found in the patients. From 28 members of a Gypsy family, we examined two siblings aged 31 and 30 years and their nephew aged 20 years, all suffering from HMSN that was associated with auditory neuropathy. All three affected members with difficulty of understanding speech had following investigations: pure tone and speech audiograms, BAEP, cochlear microphonics, and nerve conduction studies (NCV). Results: the older two siblings had a flat 80 dB audiogram, whereas the younger one has flat 20 dB audiogram on the Lt. ear and 30 dB audiogram on the Rt. ear. All had no speech comprehension and no BAEP. Two patients had preserved cochlear microphonics on one ear. Peripheral nerves were electrically not elicitable, however, at the beginning of the disease nerve conduction was slow. Conclusion: in all three affected members with distinct clinical picture of HMSN their hearing impairment was proved to be due to severe auditory neuropathy in the presence of preserved inner ear function.
Starting Page	R204
Ending Page	R205
File Format	PDF
ISSN	00316768
Journal	Pflügers Archiv
Volume Number	439
Issue Number	7
e-ISSN	14322013
Language	English
Publisher	Springer Berlin Heidelberg
Publisher Date	2016-10-10
Publisher Place	Berlin, Heidelberg
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	auditory neuropathy brainstem auditory evoked potentials cochlear microphonics hereditary motor and sensory neuropathy Human Physiology Molecular Medicine Neurosciences Cell Biology Receptors
Content Type	Text
Resource Type	Article
Subject	Physiology Physiology (medical) Clinical Biochemistry

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