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Darwin's tubercle (or auricular tubercle) is a congenital ear condition which often presents as a thickening on the helix at the junction of the upper and middle thirds.
Many patients will report a history of vertigo as a result of fast head movements. Many patients are also capable of describing the exact head movements that provoke their vertigo. Purely horizontal nystagmus and symptoms of vertigo lasting more than one minute can also indicate BPPV occurring in the horizontal semicircular canal.
Patients do not experience other neurological deficits such as numbness or weakness, and if these symptoms are present, a more serious etiology, such as posterior circulation stroke or ischemia, must be considered.
The spinning sensation experienced from BPPV is usually triggered by movement of the head, will have a sudden onset, and can last anywhere from a few seconds to several minutes. The most common movements patients report triggering a spinning sensation are tilting their heads upwards in order to look at something, and rolling over in bed.
Vertigo is a medically recognized term for the symptom of vestibular system disturbance. It may include a feeling of rotation or illusory sensations of motion or both. The general term dizziness is used by nonmedical people for those symptoms but often refers to a feeling of light-headedness, giddiness, drowsiness, or faintness, all of which must be differentiated from true vertigo, since the latter symptoms might have other causes.
Motion sickness occurs more frequently in migraine patients (30–50% more than in controls). Benign paroxysmal vertigo of childhood is an example of migraine-associated vertigo in which headache does not often occur. Basilar artery migraine (BAM) consists of two or more symptoms (vertigo, tinnitus, decreased hearing, ataxia, dysarthria, visual symptoms in both hemifields or both eyes, diplopia, bilateral paresthesias, paresis, decreased consciousness and/or loss of consciousness) followed by throbbing headache. Auditory symptoms are rare. However, a study showed a fluctuating low-tone sensorineural hearing loss in more than 50% of patients with BAM with a noticeable change in hearing just before the onset of a migraine headache. The attacks of vertigo are usually concurrent with the headache and the family history is usually positive. The diagnostician must rule out: TIAs, and paroxysmal vestibular disorder accompanied by headache.
There is also a familial vestibulopathy, familial benign recurrent vertigo (fBRV), where episodes of vertigo occur with or without migraine headache. Testing may show profound vestibular loss. The syndrome responds to acetazolamide. Familial hemiplegic migraine (FHM) has been linked to mutations in the calcium channel gene. (Ophoff et al. 1966 cf. Lempert et al.)
There are two forms (also referred to as "classifications") of FCMS; bilateral and unilateral. The bilateral form is most common (also referred to as the "classical form") and is caused by the formation of lesions on both sides of the anterior or posterior region of the operculum. In contrast, the unilateral form is rare and is caused by the formation of lesions on one side of the anterior or posterior region of the operculum. Lesions located in the anterior regions of the operculum are associated with motor deficits and anarthria, a total absence of the ability to form speech or language. Lesions located in the posterior regions of the operculum are associated with parietal opercular functions. The two classifications of FCMS were established based on the location of the lesion, stroke, and trauma affecting the brain. Classifying FCMS based solely upon lesions yields five specific subtypes of FCMS currently known to fall into the bilateral and unilateral categories:
- Bilateral anterior opercular syndrome (lesion in both the anterior or in the frontal operculum)
- Opercular-subopercular syndrome (lesions in the opercular cortex on one side and the subopercular lesion in the contralateral side).
- Subopercular syndrome (lesions in the subcortical corticobulbar projections only).
- Unilateral anterior syndrome involving the frontal operculum.
- Posterior syndrome involving the junction between the frontal and the parietal lobe of the operculum.
Symptoms typically include imbalance and visual problems. Dark or unsure situations generally increase this imbalance.The imbalance is worse in the dark or in situations where footing is uncertain. Spinning vertigo is unusual. Oscillopsia, visual symptoms of Bilateral Vestibulopathy only occur when the head is moving. For instance, when driving, a person with Bilateral Vestibulopathy may see very blurry objects. Oscillopsia is often common during walking. Transient visual blurring occurs with quick movements of the head.
The bilateral form of FCMS ("also known as facio-labio-pharyngo-glosso-laryngo-brachial paralysis)" is consistent with the classic presentation of bilateral corticobulbar involvement. It is characterized by well-preserved automatic and reflex movements. It is caused by lesions in the cortical or subcortical region of the anterior opercular area surrounding the insula forming the gyri of the frontal, temporal, and parietal lobes.
Benign paroxysmal positional vertigo - Migraine is commonly associated with BPPV, the most common vestibular disorder in patients presenting with dizziness. The two may be linked by genetic factors or by vascular damage to the labyrinth.
Ménière's disease - There is an increased prevalence of migraine in patients with Ménière's disease and migraine leads to a greater susceptibility of developing Ménière’s disease. But they can be distinguished. Ménière's disease may go on for days or even years, while migraines typically do not last longer than 24 hours.
Motion sickness is more prevalent in patients with migraine.
Psychiatric syndromes Dizziness and spinning vertigo are the second most common symptom of panic attacks, and they can also present as a symptom of major depression. Migraine is a risk factor for developing major depression and panic disorder and vice versa.
This atavistic feature is so called because its description was first published by Charles Darwin in the opening pages of "The Descent of Man, and Selection in Relation to Sex", as evidence of a vestigial feature indicating common ancestry among primates which have pointy ears. However, Darwin himself named it the Woolnerian tip, after Thomas Woolner, a British sculptor who had depicted it in one of his sculptures and had first theorised that it was an atavistic feature.
Your physician will make on your history, physical examination or vestibular tests in a rotary chair. There are several different causes of Bilateral Vestibulopathy, including Gentamicin toxicity, but the rotary chair test will determine the effects on both ears. Tests for syphilis, an antibody test for autoimmune inner ear disease or audiograms may be important in determining if you have bilateral vestibulopathy.
Scrotal ultrasonography and transrectal ultrasonography (TRUS) are useful in detecting uni- or bilateral CBAVD, which may be associated with visible abnormalities or agenesis of the epididymis, seminal vesicles or kidneys.
Vertigo is a sensation of spinning while stationary. It is commonly associated with nausea or vomiting, unsteadiness (postural instability), falls, changes to a person's thoughts, and difficulties in walking. Recurrent episodes in those with vertigo are common and frequently impair the quality of life. Blurred vision, difficulty in speaking, a lowered level of consciousness, and hearing loss may also occur. The signs and symptoms of vertigo can present as a persistent (insidious) onset or an episodic (sudden) onset.
Persistent onset vertigo is characterized by symptoms lasting for longer than one day and is caused by degenerative changes that affect balance as people age. Naturally, the nerve conduction slows with aging and a decreased vibratory sensation is common.
Additionally, there is a degeneration of the ampulla and otolith organs with an increase in age. Persistent onset is commonly paired with central vertigo signs and symptoms.
The characteristics of an episodic onset vertigo is indicated by symptoms lasting for a smaller, more memorable amount of time, typically lasting for only seconds to minutes. Typically, episodic vertigo is correlated with peripheral symptoms and can be the result of but not limited to diabetic neuropathy or autoimmune disease.
Peripheral Territory Lesions
1. Contralateral homonymous hemianopsia
2. cortical blindness with bilateral involvement of the occipital lobe branches
3. visual agnosia
4. prosopagnosia
5. dyslexia, Anomic aphasia, color naming and discrimination problems
6. memory defect
7. topographic disorientation
Central Territory Lesions
1. central post-stroke (thalamic) pain: spontaneous pain, dysesthesias and sensory impairments
2. involuntary movements: chorea, intention tremor, hemiballismus
3. contralateral hemiplegia
4. Weber’s syndrome: occulomotor nerve palsy
5. Bálint's syndrome: loss of voluntary eye movements optic ataxia, asimultagnosia (inability to understand visual objects)
Vertigo that arises from injury to the balance centers of the central nervous system (CNS), often from a lesion in the brainstem or cerebellum, is called "central" vertigo and is generally associated with less prominent movement illusion and nausea than vertigo of peripheral origin. Central vertigo may have accompanying neurologic deficits (such as slurred speech and double vision), and pathologic nystagmus (which is pure vertical/torsional). Central pathology can cause disequilibrium which is the sensation of being off balance. The balance disorder associated with central lesions causing vertigo is often so severe that many patients are unable to stand or walk.
A number of conditions that involve the central nervous system may lead to vertigo including: lesions caused by infarctions or hemorrhage, tumors present in the cerebellopontine angle such as a vestibular schwannoma or cerebellar tumors, epilepsy, cervical spine disorders such as cervical spondylosis, degenerative ataxia disorders, migraine headaches, lateral medullary syndrome, Chiari malformation, multiple sclerosis, parkinsonism, as well as cerebral dysfunction. Central vertigo may not improve or may do so more slowly than vertigo caused by disturbance to peripheral structures.
Benign paroxysmal positional vertigo (BPPV) is a disorder arising from a problem in the inner ear. Symptoms are repeated, brief periods of vertigo with movement, that is, of a spinning sensation upon changes in the position of the head. This can occur with turning in bed or changing position. Each episode of vertigo typically lasts less than one minute. Nausea is commonly associated. BPPV is one of the most common causes of vertigo.
BPPV can result from a head injury or simply occur among those who are older. A specific cause is often not found. The underlying mechanism involves a small calcified otolith moving around loose in the inner ear. It is a type of balance disorder along with labyrinthitis and Ménière's disease. Diagnosis is typically made when the Dix–Hallpike test results in nystagmus (a specific movement pattern of the eyes) and other possible causes have been ruled out. In typical cases medical imaging is not needed.
BPPV is often treated with a number of simple movements such as the Epley maneuver or Brandt–Daroff exercises. Medications may be used to help with nausea. There is tentative evidence that betahistine may help with the vertigo but its use is not generally needed. BPPV is not a serious condition. Typically it resolves in one to two weeks. It however may recur in some people.
The first medical description of the condition occurred in 1921 by Robert Barany. About 2.4% of people are affected at some point in time. Among those who live until their 80s, 10% have been affected. BPPV affects females twice as often as males. Onset is typically in the person's 50s to 70s.
The diagnosis of PMG is merely descriptive and is not a disease in itself, nor does it describe the underlying cause of the brain malformation.
Polymicrogyria may be just one piece of a syndrome of developmental abnormalities, because children born with it may suffer from a wide spectrum of other problems, including global developmental disabilities, mild to severe mental retardation, motor dysfunctions including speech and swallowing problems, respiratory problems, and seizures. Though it is difficult to make a predictable prognosis for children with the diagnosis of PMG, there are some generalized clinical findings according to the areas of the brain that are affected.
- Bilateral frontal polymicrogyria (BFP) – Cognitive and motor delay, spastic quadriparesis, epilepsy
- Bilateral frontoparietal polymicrogyria (BFPP) – Severe cognitive and motor delay, seizures, dysconjugate gaze, cerebellar dysfunction
- Bilateral perisylvian polymicrogyria (BPP) – Pseudobulbar signs, cognitive impairment, epilepsy, some with arthrogryposis or lower motor neuron disease
- Bilateral parasagittal parieto-occipital polymicrogyria (BPPP) – Partial seizures, some with mental retardation
- Bilateral generalized polymicrogyria (BGP) – Cognitive and motor delay of variable severity, seizures
The vas deferens connect the sperm-producing testicles to the penis. Therefore, those who are missing both vas deferens are typically able to create sperm but are unable to transport them appropriately. Their semen does not contain sperm, a condition known as azoospermia.
The region in which unilateral polymicrogyria occurs has been generalized into different cortical areas. Features associated with this form of polymicrogyria are similar to the other forms and include spastic hemiparesis, mental retardation in variable degrees, and seizures. The features depend on the exact area and extent to which polymicrogyria has affected the cortex. Patients who have unilateral polymicrogyria have been reported to also have electrical status epilepticus during sleep (EPES), and all suffered from seizures.
Individuals with exploding head syndrome hear or experience loud imagined noises as they are falling asleep or waking up, have a strong, often frightened emotional reaction to the sound, and do not report significant pain; around 10% of people also experience visual disturbances like perceiving visual static, lightning, or flashes of light. Some people may also experience heat, strange feelings in their torso, or a feeling of electrical tinglings that ascends to the head before the auditory hallucinations occur. With the heightened arousal, people experience distress, confusion, myoclonic jerks, tachycardia, sweating, and the sensation that felt as if they had stopped breathing and had to make a deliberate effort to breathe again.
The pattern of the auditory hallucinations is variable. Some people report having a total of two or four attacks followed by a prolonged or total remission, having attacks over the course of a few weeks or months before the attacks spontaneously disappear, or the attacks may even recur irregularly every few days, weeks, or months for much of a lifetime.
Some individuals believe that EHS episodes are not natural events, but are the effects of directed energy weapons which create an auditory effect. Thus, EHS has been worked into conspiracy theories, but there is no scientific evidence that EHS has non-natural origins.
Michel aplasia, also known as complete labyrinthine aplasia (CLA), is a congenital abnormality of the inner ear. It is characterized by the bilateral absence of differentiated inner ear structures and results in complete deafness (anacusis).
Michel aplasia should not be confused with michel dysplasia. It may affect one or both ears.
"Aplasia" is the medical term for body parts that are absent or do not develop properly. In Michel aplasia, the undeveloped (anaplastic) body part is the bony labyrinth of the inner ear. Other nearby structures may be underdeveloped as well.
Exploding head syndrome is classified as a parasomnia and a sleep-related dissociative disorder by the 2005 International Classification of Sleep Disorders and is an unusual type of auditory hallucination in that it occurs in people who are not fully awake.
Benign lymphoepithelial lesion is a type of benign enlargement of the parotid and/or lacrimal glands. This pathologic state is sometimes, but not always, associated with Sjögren's syndrome.
Cortical deafness is a rare form of sensorineural hearing loss caused by damage to the primary auditory cortex. Cortical deafness is an auditory disorder where the patient is unable to hear sounds but has no apparent damage to the anatomy of the ear (see auditory system), which can be thought of as the combination of auditory verbal agnosia and auditory agnosia. Patients with cortical deafness cannot hear any sounds, that is, they are not aware of sounds including non-speech, voices, and speech sounds. Although patients appear and feel completely deaf, they can still exhibit some reflex responses such as turning their head towards a loud sound.
Cortical deafness is caused by bilateral cortical lesions in the primary auditory cortex located in the temporal lobes of the brain. The ascending auditory pathways are damaged, causing a loss of perception of sound. Inner ear functions, however, remains intact. Cortical deafness is most often cause by stroke, but can also result from brain injury or birth defects. More specifically, a common cause is bilateral embolic stroke to the area of Heschl's gyri. Cortical deafness is extremely rare, with only twelve reported cases. Each case has a distinct context and different rates of recovery.
It is thought that cortical deafness could be a part of a spectrum of an overall cortical hearing disorder. In some cases, patients with cortical deafness have had recovery of some hearing function, resulting in partial auditory deficits such as auditory verbal agnosia. This syndrome might be difficult to distinguish from a bilateral temporal lesion such as described above.
Problems with balance can occur when there is a disruption in any of the vestibular, visual, or proprioceptive systems. Abnormalities in balance function may indicate a wide range of pathologies from causes like inner ear disorders, low blood pressure, brain tumors, and brain injury including stroke.
Many different terms are often used for dizziness, including lightheaded, floating, woozy, giddy, confused, helpless, or fuzzy. Vertigo, Disequilibrium and pre-syncope are the terms in use by most physicians and have more precise definitions.
Vertigo
Vertigo is the sensation of spinning or having the room spin about you. Most people find vertigo very disturbing and report associated nausea and vomiting.
Disequilibrium
Disequilibrium is the sensation of being off balance, and is most often characterized by frequent falls in a specific direction. This condition is not often associated with nausea or vomiting.
Presyncope
Pre-syncope is a feeling of lightheadedness or simply feeling faint. Syncope, by contrast, is actually fainting. A circulatory system deficiency, such as low blood pressure, can contribute to a feeling of dizziness when one suddenly stands up.
Problems in the skeletal or visual systems, such as arthritis or eye muscle imbalance, may also cause balance problems.
Benign lymphoepithelial lesion is most likely to occur in adults around 50 years of age. There is a predilection for gender with 60–80% being female. The gland affected has a diffuse swelling. The swelling can be asymptomatic, but mild pain can also be associated. There is a preponderance of this disease in those who suffer from HIV infection.
Most cases of benign lymphoepithelial lesions appear in conjunction with Sjögren's syndrome. When Sjögren's syndrome is present, the swelling is usually bilateral. Otherwise, the affected glands are usually only on one side of the body.
In many cases, a biopsy is needed to distinguish benign lymphoepithelial lesions from sialadenosis (sialosis).
Signs and symptoms of CBPS typically appear in infancy or at birth, but can appear later in childhood. These include facial diplegia (paralysis on both sides), facial muscle spasms, pseudobulbar palsy, dysarthria (difficulty speaking), difficulty chewing, dysphagia (difficulty swallowing), epilepsy, and intellectual disability. Epileptic seizures in individuals with CBPS are different between individuals and can vary between episodes.