293 Functional Neuroanatomy

Text and Atlas

One of the bones of the middle ear. So named by Vesalius in 1543, but probably seen much
earlier.
Meatus (Latin meo, “passage”).
The external auditory meatus is a path or a way for sound waves.
Meyer, Adolph (1866–1950).
Swiss neuropsychiatrist who immigrated to the United States and was on the faculty of Cornell
and Johns Hopkins. Described the part of the optic radiation that loops around the tip of the
temporal horn (Meyer's loop).
Mitral (Latin mitra, “a kind of a hat with two cusps, a tur-ban, or head band”).
Modiolus (Latin, “the hub of a wheel”).
The central pillar of the cochlea. Described and so named by Eustachio in 1563. Its structure
suggests the hub of the wheel with radiating spokes (lamina spiralis) attached to it.
Nyctalopia (Greek nyx, “night”; alaos, “blind”; opia, “eye”). Night blindness.
Olfactory (Latin olfacere, “to smell”).
Pertaining to the sense of smell. The olfactory nerve, the first cranial nerve in today's
classification, was proposed by Soemmerring (1755–1830), the German anatomist, although it
was not included as a cranial nerve by Galen. The olfactory nerves were first noted by
Theophilus Protospatharius, physician to the Emperor Heraclius in the seventh century A.D. The
function of the olfactory nerve was correctly stated by Achillini, and its relationship to the
neuroepithelium of the nasal mucosa was demonstrated by Max Schultze in 1856.
Organ of Corti.
Auditory end organ in the inner ear. Named after Marchese Alfonso Corti (1822–1888), the
Italian histologist who is known for his investigations of the mammalian cochlea in 1851.
Pinna (Latin, “a feather”).
The part of the external ear that projects from the side of the head. So named by Rufus of
Ephesus.
Protanopia (Greek protos, “first”; an, “negative”; opia, “vi-sion”).
Absence of red sensitive pigment in cones. Patients are insensitive to red light and confuse red
and green light.
Pythagoras.
Greek mathematician. Proposed that sound is an air vibration. He also was the first to advance
the concept that the power of reasoning is in the brain.
Reissner's membrane.
The membrane that separates the scalae vestibuli and media. Described in 1851 by Ernst
Reissner (1824–1878), a German anatomist.
Scala (Latin, “stairway or ladder”).
The scala tympani and scala vestibuli are so named because of their circular staircase
appearance.
Sclera (Greek skleros, “hard”).
The outermost, tough, fibrous coat of the eyeball. The term was first used to refer to the whole
white outer layer by Salomon Albertus, professor of anatomy in Wittenburg in 1585.
Stapes (Latin, “a stirrup”).
The stapes of the middle ear resembles a stirrup. So named by Ingrassias in 1546.
Tectorial membrane (Latin tego, “a covering”).
Tritanopia (Greek tritos, “third”; an, “negative”; opia, “vision”).
Absence of blue sensitive pigment in retinal cones. Patients are insensitive to blue light but do
not confuse red and green.
Vesalius, Andreas (1514–1564).
Belgian anatomist. Considered one of the greatest anatomists of all time.
SUGGESTED READINGS
Barbur JL et al: Human visual responses in the absence of the geniculo-calcarine projection. Brain 1980; 103:905–928.
Brown KT: Physiology of the retina. In Mountcastle VB (ed): Medical Physiology, 14th ed, vol 1. St. Louis, Mosby, 1980:504.
Goldstein MH: The auditory periphery. In Mountcastle VB (ed): Medical Physiology, 14th ed, vol 1. St. Louis, Mosby, 1980:428.
Hubel DH, Wiesel TN: Functional architecture of macaque monkey visual cortex. Proc R Soc Lond [B] 1977; 198:1–59.
Hubel DH, Wiesel TN: Brain mechanisms of vision. Sci Am 1979; 241(3):150–162.
Hudspeth AJ: The hair cells of the inner ear. Sci Am 1983; 248:54–64.
Kaneko A: Physiology of the retina. Annu Rev Neurosci 1979; 2:169–191.
Lim DJ: Functional structure of the organ of Corti: A review. Hear Res 1986; 22:117–146.
Mafee MF et al: Large vestibular aqueduct and congenital sensorineural hearing loss. AJNR 1992; 13:805–819.
Merigan WH, Maunsell JHR: How parallel are the primate visual pathways: Annu Rev Neurosci 1993; 16:369–402.
Nelson GM: Biology of taste buds and the clinical problem of taste loss. The Anat Rec (New Anat) 1998; 253:70–78.
Shepherd GM: Synaptic organization of the mammalian olfactory bulb.Physiol Rev 1972; 52:864–917.
Uesaka Y et al: The pathway of gustatory fibers of the human ascends ipsilaterally in the pons. Neurology 1998; 50:827–828.
Zeki S: The representation of colours in the cerebral cortex. Nature 1980; 284:412–418.
Zihl J, von Cramon D: The contribution of the “second” visual system to directed visual attention in man. Brain 1979;
102:835–856.
----------------------------------------------------------------------------------------------------------------------------

24  
    
      
Disorders of Olfaction
Abnormalities in Taste
Disorders of Vision
 294

Disorders of Hearing
Vestibular Disorders
KEY CONCEPTS
1) The olfactory system may be involved in disease processes at the olfactory receptors
(common cold), olfactory nerve (fractures of the cribriform plate of the ethmoid), olfactory
bulb and tract (inflammation, tumors), and olfactory cortex (tumor, epilepsy).
2) Taste may be affected in lesions of the facial, glossopharyngeal, and vagus cranial nerves
and in lesions of or near the primary gustatory cortex. Taste loss is also associated with a
variety of medical conditions, drugs, and radiation.
3) Lesions of the optic nerve are associated with monocular blindness. Lesions of the optic
chiasma are associated with bitemporal hemianopia. Lesions of the optic tract are
associated with contralateral homonymous hemianopia.
4) Lesions of the optic radiation in the temporal and parietal lobes are associated with
contralateral quadrantanopia.
5) Lesions of the upper or the lower bank of the calcarine sulcus are associated with
contralateral quadranta-nopia.
6) Lesions of the primary visual cortex (both banks of the calcarine sulcus) are associated with
contralateral homony-mous hemianopia.
7) In vascular lesions of the primary visual cortex (occlusion of posterior cerebral artery),
macular (central) vision is preserved (macular sparing).
8) Sensorineural deafness results from disorders that interfere with function of the auditory
end organ, cochlear nerve, cochlear nuclei, or central auditory pathways.
9) Vestibular disorders (peripheral and central) are associated with vertigo, nystagmus, and
truncal ataxia.
DISORDERS OF OLFACTION

T
he olfactory system can be affected in several sites with resulting derangements in the
sense of smell. Olfactory receptors are decreased in number with age and are affected in
common colds, resulting in bilateral diminution or loss of smell (anosmia). Olfactory nerve
fibers may be affected in their course through the cribriform plate of the ethmoid bone in
fractures of the plate.
The olfactory bulb and tracts may be involved in inflammatory processes of the meninges
(meningitis) or tumors (meningioma) in the frontal lobe or the anterior cranial fossa. Unilateral
loss of smell may be the earliest clinical manifestation in such processes. Loss of dopamine in
the olfactory bulb of Parkinson's patients is responsible for the decrease of the sense of smell in
such patients.
Pathologic processes in the region of the primary olfactory cortex (the uncus of the temporal
lobe) usually give rise to hallucinations of smell (uncinate fits). The odor experienced in such
cases is often described as unpleasant. Such hallucinations may herald an epileptic seizure or
be part of it. They also may be a manifestation of a tumor in that region.
ABNORMALITIES IN TASTE
Abnormal taste sensations (usually unpleasant sensations) occur preceding a temporal lobe
seizure or as part of the seizure, especially if the epileptic focus is close to the uncus of the
temporal lobe (uncinate seizures) or to the primary gustatory cortex in the inferior part of the
somesthetic cortex.
Taste loss (ageusia), decrease in taste (hypogeusia), and abnormal taste (dysgeusia) are
common disorders.
They occur as a natural phenomenon of aging or in association with pregnancy, menopause,
and a variety of illnesses.
Taste sensations are impaired ipsilateral to lesions in the facial (CN VII), glossopharyngeal (CN
IX), and vagus (CN X) nerves. These nerves convey taste sensations from the anterior two-thirds
of the tongue (CN VII), posterior third of the tongue (CN IX), and the epiglottis (CN X).
Patients with xerostomia (dry mouth), Sjögren syndrome (salivary glands inflammation)
diabetes, and zinc deficiency may experience loss of taste.
Chemotherapeutic agents (methotrexate) used in the treatment of cancer, as well as numerous
drugs (dexamethasone, antihypertensive agents, H2 receptor agonists, antimicrobial agents),
can also induce loss of taste.
Taste loss usually follows radiation therapy to the oral cavity.
DISORDERS OF VISION
The visual system can be affected in several sites. Alterations in length of the eyeball result in
refraction errors. Normally, distant objects are brought to focus on the retina. In persons with
elongated eyeballs, distant objects are brought to focus in front of the retina (myopic eyes). In
such persons, only near objects can be brought to focus on the retina (nearsightedness). In
persons with flattened eyeballs, distant objects are brought to focus behind the retina
(hyperopic eyes). Both conditions can be corrected by use of appropriate lenses.
Night blindness (nyctalopia) is encountered in individuals with vitamin A deficiency.
Photoreceptor pigment is formed of vitamin A aldehyde and a protein. Thus, in vitamin A
deficiency states, the total amount of visual pigment is reduced, decreasing the sensitivity to
light of both rods and cones. This reduction in visual pigment, while not affecting bright-light
(daylight) vision, does significantly interfere with dim-light (night) vision. This condition is
treatable by vitamin A administration.
Color blindness is associated with deficiency or lack of a particular color cone. Most color blind
persons are red-green blind; a minority are blue blind. Color blindness for red and green is
 295 Functional Neuroanatomy
Text and Atlas
inherited by X-linked recessive gene; hence it is more prevalent in males. Color blindness for
blue is inherited by autosomal recessive gene.
Lesions of the optic nerve (tumor, demyelination) (see Fig. 23-6) result in monocular blindness
(blindness in one eye). Lesions of the optic chiasma (see Fig. 23-6), where partial crossing of
optic nerve fibers occurs, result in bitemporal hemianopia (blindness in both temporal visual
fields) due to involvement of the crossing fibers. Such a visual defect is seen in association with
lesions in the pituitary gland (pituitary adenoma) or tumors in the hypothalamus. Lesions in the
optic tract result (see Fig. 23-6) in homonymous hemianopia contralateral to the lesion in the
optic tract due to involvement of crossed fibers from the contralateral retina and uncrossed
fibers from the ipsilateral retina.
Lesions of the direct path of the optic radiation in the parietal lobe or of the indirect path of the
optic radiation (Meyer's loop) in the temporal lobe (see Fig. 23-6) result in quadrantic
hemianopia. The inferior quadrants of the visual field will be affected in parietal lobe lesions
and the superior quadrants in temporal lobe lesions.
Similarly, lesions of the upper or lower banks of the calcarine sulcus will result in a quadrantic
visual field defect, inferior in upper bank lesions and superior in lower bank lesions.
Lesions of the primary visual cortex (upper and lower banks) (see Fig. 23-6) result in
contralateral homonymous hemianopia.
If the lesion is vascular (occlusion of posterior cerebral or calcarine arteries), there will be
macular sparing due to collateral supply of the macular area from the middle cerebral artery.
DISORDERS OF HEARING
Disorders of hearing are generally of two types: conductive and sensorineural. Conductive
hearing loss is associated with processes that interfere with conduction of sound waves in the
external and middle ears. Such processes include wax (cerumen) accumulations in the external
auditory meatus, chronic otitis media, and ossicle sclerosis (otosclerosis).
Sensorineural hearing loss is associated with lesions of the hair cells in the organ of Corti, the
cochlear nerve (tumors of the nerve, such as in cerebellopontine angle tumors, labyrinthine
artery occlusion), cochlear nuclei in the pons, or the central auditory pathways. Hearing loss is
ipsilateral to the lesion in disorders of the hair cells, cochlear nerve, and cochlear nuclei.
Lesions of the central auditory pathways (lateral lemniscus, medial geniculate body, auditory
cortex) result in a bilateral decrease in hearing more marked contralateral to the lesion.
Ringing, buzzing, hissing, or paper crushing noises (tinnitus) in the ear are early signs of
diseases of the cochlea.
The two types of hearing disorders (conductive and sensorineural) are differentiated by placing
a vibrating tuning fork on the vertex in the midline of the skull (Weber test) or alternately on the
mastoid process and next to the auricle (Rinne test). Using the Weber test, a person with normal
hearing will hear the sound of the vibrating tuning fork equally well in both ears. A person with
conductive deafness in one ear will hear the sound louder in the deaf ear because the masking
effect of environmental noises is absent on the affected side. A person with sensorineural
deafness will hear the sound louder in the normal ear. With the Rinne test, a person with normal
hearing will continue to hear the sound of the vibrating tuning fork placed next to the ear (air
conduction) after he or she stops hearing the sound of the tuning fork placed on the mastoid
process (bone conduction). A person with conductive deafness will not hear the vibrations of
the tuning fork in air after bone conduction is over. A person with sensorineural deafness will
continue to hear vibrations in air after bone conduction is over.
Cochlear implants are used today to treat patients with sensorineural loss resulting from
cochlear disease provided the auditory nerve and central auditory pathways are intact.
VESTIBULAR DISORDERS
The vestibular system can be affected in several sites, including the peripheral end organ in the
inner ear, vestibular nerve, vestibular nuclei, and central vestibular pathway, and by a variety
of disease processes, including infection, demyelination, vascular disorders, and tumor.
Disorders of the vestibular system are manifested by an illusory sensation of motion (vertigo),
oscillatory involuntary eye movements (nystagmus), and postural disequilibrium (truncal
ataxia).
Lesions of the semicircular canals induce rotatory vertigo, whereas disease of the utricle or
saccule produces sensations of tilt or levitation. An example of end-organ vertigo is sea
sickness, which is caused by irregular continuous movement of endolymph in susceptible
individuals. Vertigo also may occur with disease of vestibular structures in the brain stem. This
is usually associated with other signs of brain stem damage such as hemiparesis, hemisensory
loss, and cranial nerve signs.
Both central and peripheral vestibular lesions induce nystagmus, an involuntary back-and-forth
movement of the eyes in horizontal, vertical, or rotatory pattern. Peripheral and central
nystagmus are differentiated from each other by the following: (1) fixation of the eyes
suppresses peripheral but not central nystagmus, and (2) pure vertical or torsional nystagmus is
usually central.
Truncal (vestibular) ataxia occurs in association with peripheral and central vestibular disease.
A dramatic feature of such patients is the inability to stand upright without support and a
staggering gait with a tendency to fall toward the side of the lesion.
TERMINOLOGY
Anosmia (Greek a, “negative”; osme, “smell”).
Loss of sense of smell.
Calcarine (Latin calcarinus, “spur-shaped”).
Pertaining to the calcar, a structure resembling a spur.
 296

Cribriform plate (Latin cribrum, “a sieve”; forma, “form”).

The cribriform plate of the ethmoid bone has many holes (like a sieve) through which olfactory
nerve fibers pass.
Gustatory (Latin gustatorius, “pertaining to the sense of taste”).
Relating to the sense of taste.
Hemianopia (Greek hemi, “half”; an, “negative”; opia, “eye”).
Loss of vision in one-half the visual field of each eye.
Homonymous (Greek homo, “same”; onoma, “name”).
Per-taining to the corresponding halves of the visual field.
Hyperopia (Greek hyper, “above”; opia, “eye”).
An error of refraction (farsightedness) in which the entering light rays are focused behind the
retina as a result of a short eyeball from front to back.
Meyer's loop.
Also known as Flechsig loop. The part of the optic radiation that loops around the tip of the
temporal horn before reaching the primary visual cortex in the occipital lobe. Named after
Adolph Meyer (1866–1950), a Swiss-American neurologist and psychiatrist who described this
loop.
Myopia (Greek myein, “to shut”; opia, “eye”).
An error of refraction (shortsightedness) in which light rays fall in front of the retina as a result of
a too long eyeball from front to back.
Nyctalopia (Greek nyx, “night”; alaos, “blind”; opia, “eye”).
Impairment of night vision. The original Greek usage referred to the ability to see by night only.
Galen changed the meaning to impairment of night vision.
Nystagmus (Greek nystagmos, “drowsiness,” from nystazein, “to nod”).
Involuntary, rapid rhythmic oscillations of the eyes.
Otosclerosis (Greek otos, “ear”; sklerosis, “hardening”).
Conductive hearing loss due to sclerosis of the ossicles in the middle ear.
Rinne test.
Hearing test to compare air and bone conduction of sound by placing a vibrating tuning fork on
the mastoid process and in front of the ear. Named after H. A. Rinne (1819–1868), a German
otolaryngologist who described the test.
Tinnitus (Latin “ringing”).
Hallucinatory sound associated with cochlear disorders.
Uncinate (Latin, “hooked”).
Pertaining to the uncus. Uncinate fits are complex partial seizures in which olfactory or
gustatory hallucinations occur. The term was used by Hughlings-Jackson, a British neurologist in
1899.
Vertigo (Latin vertigo, “turning or whirling around”).
Hallucination of movement, a sign of peripheral or central vestibular system disorders.
Weber test.
A hearing test to differentiate conductive and sensorineural deafness by placing a vibrating
tuning fork on the vertex of the skull. Named after Ernest Heinrich Weber, a German anatomist
who described the test in 1834.
SUGGESTED READINGS
Borruat FX et al: Congruous quadrantanopia and optic radiation lesion. Neurology 1993; 43:1430–1432.
Brandt T: Man in motion: Historical and clinical aspects of vestibular function. Brain 1991; 114:2159–2174.
Brandt T, Daroff RB: The multisensory physiological and pathological vertigo syndromes. Ann Neurol 1980; 7:195–203.
Collard M, Chevalier Y: Vertigo. Curr Opin Neurol 1994; 7:88–92.
D'Amico DJ: Disease of the retina. N Engl J Med 1994; 331:95–106.
Luxon LM: Disorders of hearing. In Asbury AK et al (eds): Diseases of the Nervous System: Clinical Neurobiology. Philadelphia,
Saunders, 1992:434.
Masdeu JC: The localization of lesions in the oculomotor system. In Brazis PW et al (eds): Localization in Clinical Neurology.
Boston, Little, Brown, 1985:118.
Nelson GM: Biology of taste buds and the clinical problem of taste loss. The Anat Rec (New Anat) 1998; 253:70–78.
Newman NJ: Neuro-ophthalmology: The afferent visual system. Curr Opin Neurol 1993; 6:738–746.
Sharpe JA, Johnston JL: Vertigo and nystagmus. Curr Opin Neurol Neurosurg 1990; 3:789–795.
Troost BT: Nystagmus: A clinical review. Rev Neurol 1989; 145:417–428.
Zeki S: The visual image in mind and brain. Sci Am 1992; 267(September):69–76.
----------------------------------------------------------------------------------------------------------------------------

25      $ 
   $ 
 
Development
Embryogenesis
Histogenesis
Regional Development
Myelination
Prenatal Brain Performance
Postnatal Development and Growth
Functional Maturation
Cerebral Oxygen Consumption
Cerebral Blood Flow
Cerebral Metabolic Rate for Glucose