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Auditory function in adrenomyeloneuropathy.
[adrenomyeloneuropathy]
Auditory
brainstem
responses
(
ABR
)
,
ipsilateral
and
contralateral
acoustic
reflexes
and
the
masking
level
difference
for
speech
(
MLD
)
were
studied
in
29
patients
with
adrenomyeloneuropathy
(
AMN
)
.
Abnormalities
were
seen
for
all
ABR
components
with
Waves
V
and
III
affected
to
the
greatest
degree
.
For
male
patients
with
AMN
,
the
I
-
III
,
III
-V
and
I
-V
interpeak
latency
intervals
were
abnormal
for
a
majority
of
patients
.
For
female
patients
with
AMN
,
the
I
-V
and
III
-V
interpeak
latency
intervals
were
abnormal
for
a
majority
of
patients
with
the
I
-
III
interval
less
affected
.
Contralateral
acoustic
reflexes
were
elevated
or
absent
for
approximately
50
%
of
ears
.
Ipsilateral
acoustic
reflexes
were
abnormal
for
25
%
of
ears
.
MLDs
were
significantly
reduced
in
72
%
of
patients
.
When
considered
in
terms
of
the
earliest
ABR
wave
abnormality
,
the
earlier
components
of
the
ABR
(
i
.
e
.
,
Waves
III
and
I
)
were
the
initial
components
impaired
for
the
majority
of
ears
.
Word
recognition
in
quiet
was
relatively
unimpaired
for
all
subjects
.
Despite
the
presence
of
marked
ABR
abnormalities
,
patients
with
AMN
denied
the
presence
of
significant
difficulty
hearing
.
