PMID- 10653192
OWN - NLM
STAT- MEDLINE
DCOM- 20000215
LR  - 20130520
IS  - 0031-6768 (Print)
IS  - 0031-6768 (Linking)
VI  - 439
IP  - 3 Suppl
DP  - 2000
TI  - Brainstem auditory evoked potentials and cochlear microphonics in the HMSN family 
      with auditory neuropathy.
PG  - R204-5
AB  - 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.
FAU - Butinar, D
AU  - Butinar D
AD  - Institute of Clinical Neurophysiology, University Medical Centre, Ljubljana, 
      Slovenia.
FAU - Starr, A
AU  - Starr A
FAU - Vatovec, J
AU  - Vatovec J
LA  - eng
GR  - DC02618-O1A2/DC/NIDCD NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - Germany
TA  - Pflugers Arch
JT  - Pflugers Archiv : European journal of physiology
JID - 0154720
SB  - IM
MH  - Adult
MH  - Audiometry, Pure-Tone
MH  - Child, Preschool
MH  - *Cochlear Microphonic Potentials
MH  - *Cochlear Nerve/physiopathology
MH  - Cranial Nerve Diseases/*etiology/*physiopathology
MH  - *Evoked Potentials, Auditory, Brain Stem
MH  - Hereditary Sensory and Motor Neuropathy/*complications
MH  - Humans
MH  - Male
EDAT- 2000/02/01 09:00
MHDA- 2000/02/19 09:00
CRDT- 2000/02/01 09:00
PHST- 2000/02/01 09:00 [pubmed]
PHST- 2000/02/19 09:00 [medline]
PHST- 2000/02/01 09:00 [entrez]
PST - ppublish
SO  - Pflugers Arch. 2000;439(3 Suppl):R204-5.