CORTICAL DEAFNESS

Oleh: dr. Laila Fajri

Pembimbing: dr. Novina Rahmawati, M.Si, Med, Sp.THT-KL, FICS
Where is auditory cortex?
It is the core that preserves tonotopy
Cortical deafness is The ascending
caused by bilateral auditory pathways
cortical lesions in are damaged,
the primary causing a loss of
auditory cortex perception of
located in the sound, inner ear
temporal lobes of functions remains
the brain. intact.

In some cases,
Cortical deafness is patients with
most often cause cortical deafness
by stroke, but can have had recovery
also result from of some hearing
brain injury or birth function, resulting
defects. in partial auditory
deficits.
Cases

A 58-year-old male, with past history of diabetes mellitus

and ischemic heart disease for 12 years' duration was
admitted with a new onset seizure episode. After
recovery from postictal state, bystanders noticed that he
had difficulty in hearing. They felt he was speaking
abnormally, repeating his own words and also without
any stimulus. There was no history of fever, headache,
vomiting, cranial nerve dysfunction, weakness of limbs,
or sensory abnormalities. On examination he was
conscious but gave inappropriate response to
commands. His vision was normal. He followed written
commands, repeating written words or sentences. He
could recognize his relatives. Cranial nerves and
sensorimotor system were within normal limits.There
were no signs of meningeal irritation.
Blood routine: normal hemogram, random blood sugar was 326
mg/dl with an HbA1C of 11.1 g/dl, serum creatinine was 1.3 mg/dl.

Electrocardiogram: normal sinus rhythm with ischemic changes in

the anterior leads.

ENT: normal auricles and tympanic membranes.

Pure tone audiometry: bilateral profound sensorineural hearing

loss.

CT scan: bilateral acute infarcts in both temporal lobes with

haemorrhagic transformation on the left side.

MRI brain confirmed bilateral acute infarcts in Heschl gyrus of both

temporal lobes with haemorrhagic transformation on left.
BERA: normal response confirming that brainstem was intact.

Middle latency response test (MLR): decreased amplitude in Pa

wave with increased latency.

Late latency response test (LLR): amplitude was reduced in the

P1-N1-P2 complex with increased latency.

OAE test was done to test the function of outer hair cells which
was also normal.

Echocardiography: mild LV dysfunction with no evidence of any

intracardiac thrombus.

On follow-up, his hearing deficit persists but he is able to

communicate with others with the aid of speech therapy
Diagnosis

Bilateral lesions near the primary auditory cortex in the temporal lobe
are important criteria.

Cortical deafness requires demonstration that brainstem auditory

responses are normal, but cortical evoked potentials are impaired.

Another important aspect of cortical deafness that is often overlooked

is that patients feel deaf. They are aware of their inability to hear
environmental sounds, non-speech and speech sounds.

Cortical deafness relies on the anatomic and functional disconnection

of the auditory cortex from acoustic impulses.
Treatment

Lipreading,
learning American
Sign Language,
Electrical as well as speech
stimulation of the and occupational
peripheral therapy.
Cochlear or auditory system.
auditory
brainstem
implantation.
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