The auditory sense serves as an alerting system for
visual attention and general awareness.  We turn to
look for the source of a new sound.  
Far less often do
we listen for sound from something new that enters
our visual field; listening for thunder following a
lightning flash, or sounds from a supersonic jet
streaking across the sky are examples of visual
anticipation of sound onset, but they are
clearly less
frequent than
instinctive orienting to auditory stimuli.  
The auditory system may be central to general
awareness.

Deaf people are not lacking in general awareness, but
they depend upon alternatives to sound signals that
most people rely on instinctively.  Not hearing the
honk of a horn makes activities like driving a car or
riding a bicycle more hazardous for those with loss of
auditory acuity.  Deafness is a disabling condition,
whether present from early childhood or part of the
process of aging.  Deafness in childhood requires
special education for learning language.  Deafness in
old age often leads to withdrawal from social
interactions, and inability to follow a conversation
becomes an obstacle to full awareness of one's
environment [1].

Those who maintain that deafness should not be
considered a disability may be right, but need to go
beyond euphemistic assertions to be fully convincing.  
Deafness is certainly not a sign of mental disability.  
Deafness is usually associated with impairment of
structures of the ear or the cochlear nucleus.  Loss of
signal processing integrity higher up in the auditory
pathway may interfere more with alerting functions
and environmental awareness.  Dysfunction in higher
centers of the auditory pathway will also impact non-
auditory structures to and from which they send and
receive neural information.

Deafness that comes with aging may involve higher
auditory nuclei more than those close to the ear [1,
2].  The ability to focus on one of many sound
sources, such as conversation, in a noisy environment
becomes more difficult with advancing age.  Some
degree of auditory acuity begins to decline even in
childhood; the ability to learn a foreign language by
ear and without accent, the way young children do, is
not easy past the first decade of life.  Complex
features of sound that makeup conversational
streams of speech are more likely analyzed at higher
levels of the auditory system.  High metabolic activity
in the inferior colliculi and other structures in the
auditory pathway may lead to degrees of early decline
beginning in late childhood.

The auditory impairments of the young child with
autism may possibly be of the same degree as those
of an adolescent in a first year French class.  
Recognizing syllable and word boundaries of the
teacher's spoken French is
already more difficult for a
14-year-old student than it is for a preschool child
learning French as a second language in a French
speaking community.  I would further raise the
possibility that auditory impairments of the child with
severe autism may be of the same degree as those of
old age, when speech sounds can no longer easily be
distinguished from background noise, and noise of
any kind such as loud music can be distressing and
provoke an angry reaction.

At least twelve reports have been published (through
December 2005) of adults who became deaf or lost
understanding of spoken language following injury to
the inferior colliculi [3-10].  Injury in all but one of
these cases was detected through use of magnetic
resonance imaging (MRI).  A psychological cause for
loss of hearing or responsiveness to speech had
been thought responsible in several of these cases
until the lesion in the inferior colliculi was found on
MRI scans.  Impaired subcortical processing of
acoustic signals clearly can lead to problems
that can
be
even more serious than deficits in loudness
perception.
  1. Caspary DM et al. (1995)
    Central auditory aging: GABA
    changes in the inferior
    colliculus.
  2. Gonzalez-Lima F et al. (1997)
    Quantitative cytochemistry of
    cytochrome oxidase and
    cellular morphometry of the
    human inferior colliculus in
    control and Alzheimer's
    patients.
  3. Meyer B et al (1996) Pure
    word deafness after resection
    of a tectal plate glioma with
    preservation of wave V of
    brain stem auditory evoked
    potentials.
  4. Johkura K et al (1998)
    Defective auditory recognition
    after small hemorrhage in the
    inferior colliculi.
  5. Masuda S et al (2000) Word
    deafness after resection of a
    pineal body tumor in the
    presence of normal wave
    latencies of the auditory brain
    stem response.
  6. Vitte E et al (2002) Midbrain
    deafness with normal
    brainstem auditory evoked
    potentials.
  7. Hoistad DL et al (2003)
    Central hearing loss with a
    bilateral inferior colliculus
    lesion.
  8. Kimiskidis VK et al (2004)
    Sensorineural hearing loss
    and word deafness caused by
    a mesencephalic lesion:
    clinicoelectrophysiologic
    correlations.
  9. Pan CL et al (2004) Auditory
    agnosia caused by a tectal
    germinoma.
  10. Musiek FE et al (2004) Central
    deafness associated with a
    midbrain lesion.
3 – Hearing, attention, and degrees of deafness
References
Full References
top
  1. Caspary DM, Milbrandt JC, Helfert RH (1995) Central auditory aging: GABA changes in
    the inferior colliculus.  Experimental Gerontology 30:349-360.
  2. Gonzalez-Lima F, Valla J, Matos-Collazo S (1997) Quantitative cytochemistry of
    cytochrome oxidase and cellular morphometry of the human inferior colliculus in
    control and Alzheimer's patients. Brain Research 752:117-126.
  3. Meyer B, Kral T, Zentner J. (1996) Pure word deafness after resection of a tectal plate
    glioma with preservation of wave V of brain stem auditory evoked potentials. Journal of
    Neurology, Neurosurgery and Psychiatry. 61:423-4.
  4. Johkura K, Matsumoto S, Hasegawa O, Kuroiwa Y. (1998) Defective auditory
    recognition after small hemorrhage in the inferior colliculi. Journal of the Neurological
    Sciences. 161:91-6.
  5. Masuda S, Takeuchi K, Tsuruoka H, Ukai K, Sakakura Y. (2000) Word deafness after
    resection of a pineal body tumor in the presence of normal wave latencies of the
    auditory brain stem response. The Annals of otology, rhinology, and laryngology. 2000
    Dec;109(12 Pt 1):1107-12.
  6. Vitte E, Tankéré F, Bernat I, Zouaoui A, Lamas G, Soudant J. Midbrain deafness with
    normal brainstem auditory evoked potentials. Neurology 2002;58:970–973.
  7. Hoistad DL, Hain TC (2003) Central hearing loss with a bilateral inferior colliculus
    lesion. Audiol Neurootol 2003 Mar-Apr; 8(2):111-223
  8. Kimiskidis VK, Lalaki P, Papagiannopoulos S, Tsitouridis I, Tolika T, Serasli E, Kazis D,
    Tsara V, Tsalighopoulos MG, Kazis A. Sensorineural hearing loss and word deafness
    caused by a mesencephalic lesion: clinicoelectrophysiologic correlations. Otol
    Neurotol. 2004 Mar;25(2):178-82.
  9. Pan CL, Kuo MF, Hsieh ST. Auditory agnosia caused by a tectal germinoma. Neurology.
    2004 Dec 28;63(12):2387-9.
  10. Musiek FE et al (2004) Central deafness associated with a midbrain lesion. J Am Acad
    Audiol 2004 feb; 15(2):133-151.
References