Development of inner ear and vestibular system

Dr T Balasubramanian

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Introduction: Inner ear is the first sense organ to complete its developement rather early in foetal life. Studies have shown that the entire ear completes its developement much before the first trimester of pregnancy gets over. In fact a child should be able to hear during the first trimester itself. The sense of balance is a conglomeration of outputs of three different sensations. They are vision, vestibular sensations and proprioception. Structurally speaking these three organs are fully developed by birth. Right from infancy, the balance function continues to mature with aquisition of milestones like head control, sitting, standing and walking. This process of learning and adaptation continues till adolescence. Vestibular system anatomy an overview: The vestibular system includes two otolith organs and three semicircular canals. The otolith organs are the saccule and utricle. They sense linear acceleration (gravity and transitional movements). The three semicircular canals are positioned at 90 degrees from each other (orthogonal) and are responsive to angular acceleration. The sensory hair cells are located in the maculae of utricle and saccule and ampullae of the semicircular canals. These hair cells are activated by endolymphatic fluid flow. Afferent impulses generated by the hair cells are transmitted to the bipolar cells of the vestibular nerve ganglion.

Figure showing the vestibular system Axons from these bipolar neurons pass through the internal auditory canal to reach the medulla along with the cochlear nerve. At the level of internal acoustic meatus the vestibular fibers are seggregated into two distinct bundles (superior and inferior vestibular nerves). The superior vestibular nerve supplies the superior and lateral semicircular canals as well as the utricle. The inferior vestibular nerve supplies the posterior canal and the saccule. The superior and inferior Drtbalu's otolaryngology online

vestibular nerves join to form a common bundle to enter the brainstem. The first order neurons terminate in the vestibular nuclei in the floor of the 4th ventricle. Important feature here is they dont cross the mid line. Vestibular nuclei lying in the floor of the 4th venricle are four in number. They are: 1. Superior vestibular nuclei of Bechterew 2. Lateral nucleus of Deiter 3. Medial vestibular nuclei 4. Inferior / descending vestibular nuclei. It is from these nuclei projections go to: Cerebellum Motor nuclei of extraocular muscles Antigravity muscles Opposite vestibular nuclei

Diagram showing vestibular nuclei and their connections Cortical representation of vestibular system is at the level of Parietal and insular regions of cortex. Stage I of inner ear development: This stage occurs fairly early in the embryonic developement. I.e. About second week of gestation. They begin as diffuse thickening of surface ectoderm on either side of embryonic hindbrain (Rhomboencephalon). Stage II or stage of invagination: This stage begins during the 3rd week of gestation. In this stage the thickened ectoderm begins to Drtbalu's otolaryngology online

invaginate forming areas of thickening known as Otic placodes. Stage III (Formation of otic pits): This stage occurs during the 4th week of gestation. In this stage the otic placodes get surrounded by proliferating mesoderm submerging it. This causes a small pit known as otic pits. These otic pits retain their communication with the surface ectoderm for sometime before being pinched off forming vesicle like structures known as otic vesicles.

Diagram showing development of inner ear

Diagram showing development of otic vesicle

Stage IV: (Further development of otic vesicles):

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The otic vesicles differentiate into upper and lower portions. The upper portion forms the vestibular apparatus and the lower one forms the membranous cochlea. The growth rates among the various components of the otic vesicles are rather different suggesting that each part of the inner ear follows a predetermined distinct trajecteries of development. The vestibular portion of the inner ear is located superiorly in relation to cochlea. It is known to develop earlier and grows faster than the cochlear component. Development of vestibular system proper: The superior portion of the elongating superior portion of otocyst differentiates to form a dorsal utricular and ventral saccular projections. The utricular portion gives rise to semicircular canals and utricle. Of the three semicircular canals the superior semicircular canal is the first to form, followed by posterior and then lateral canal. The saccular portion becomes the saccule and the cochlear duct. The communication between the saccule and membranous cochlea narrows to form the ductus reuniens. Formation of bony capsule around developing membranous labyrinth: This occurs rapidly from the embryonic mesoderm between 19-23 weeks. This lasts approximately for 5 weeks. Maturation of vestibular receptors: During 3rd week of gestation sensory epithelia airse from the ectoderm in the cristae forming the semicircular canals and in the maculae forming the otolith organs. By about 7 weeks small amounts of otoconia are present in the utricle. Within a weeks time the amount of otoconia in the utricle and saccule dramatically increases. By the end of 3rd month the calcium content of otoconia increases in both utricle and saccule. The utricular otoconia matures faster than saccular otoconia, their size and shape also appears to be more varied. Development of hair cells: Hair cells begin to make their appearance by 7 weeks of gestation. Eventhough they are not fully differentiated they synapse with vestibular hair cells at 7 weeks of gestation. Differentiation of these hair cells into type I and type II types begins during the 11th week of gestation. Maturation takes place from apex to base in the cristae and from center to periphery in the maculae. The ampullary cristae becomes active by the 8th week of foetal life. By 8th month of gestation the vestibular system is fully activated and moro's reflex can also be elicited. Development of vestibular neuronal pathways: The vestibular ganglion cells are of various shapes until 21st week of gestation. By 24th week they assume uniform shape by which time the development of inner ear is nearly complete. These ganglion cells continue to grow till 39th week and they reach maturity at the time of birth. Neuronal communications between labyrinth and oculomotor nuclei in the brain stem occur between 12 and 24 weeks of gestation. Myelination of the vestibular nerve begins around 20th week of gestation and infact it is the first cranial nerve to compete myelination. The vestibular nucleus complex is fully functional by 21 weeks of gestation. Developmental reflexes: Drtbalu's otolaryngology online

The study of certain primitive reflexes will help us to understand the process of maturation of the entire vestibular system. These primitive reflexes disappear as the child matures and it indicates the integrity of the brain stem and spinal cord. Persistance of these reflexes beyond their normal age of dissipation would indicate either delayed maturation or impaired nervous system function. Asymetry of these reflexes suggest either a central or peripheral nervous system disorder.

Moro reflex: This is elicited by holding the child supine and allowing the head to drop approximately 30 degrees in relation to the trunk. Immediatly there is extension and abduction of the arms with fanning out of the fingers, followed by adduction of the arms which takes place at the level of the shoulder. This response is considered to be normal and it disappears by the age of 6 months.

Figure showing the Moro reflex Moro's reflex could be absent during the following situations: 1. Complete absence of labyrinthine control 2. In severly asphixated hypotonic CNS affected child 3. Severe myopathic disorders Asymmetric Tonic neck reflex: This test is performed by turning the head of the child to one side while supine with shoulders fixed. The arm and leg on the opposite side will flex. This relfex too disappears when the child reaches the age of 6 months.

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Figure showing asymmetric tonic neck reflex

Symmetric tonic neck reflex: This reflex has two stages. Stage I: The baby is held in a horizontal position with its chest in the examiner's arm /lap. Dorsiflexion of the head will produce extension of upper extremities and flexion of lower ones. Stage II: In this stage abrupt ventroflexion of the head will produce flexion of upper extremities and extension of lower extremities.

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Figure showing symmetric tonic neck reflex Head righting reflex: This reflex develops by the age of 4 6 months. To test this reflex the child's trunk is held 30 degrees from vertical, and a normal infant will tilt the head so as to remain vertical. At about 5 months of age the child will move the lower limbs additionally away from the side of tilt. This indicates a functional integration of visual, vestibular and proprioceptive stimuli.

Figure showing righting reflex Parachute reaction: This reflex is elicited beyond the age of 5 months. Sudden downward movement of a vertically held child causes lower limbs to extend and abduct. This represents visual vestibular interaction in Drtbalu's otolaryngology online

which otoliths are predominantly involved. Doll's eye response: This reflex is normally found in full term babies within 2 weeks of birth. This can be performed by holding the baby in the hand and when it is rotated round then the eyes move in the opposite direction. This indicates normal vestibular activity. Initially since the saccadic system is immature the fast component is absent. Later as the development proceeds the fast component appears in the direction opposite to the rotation.

Development of vestibular reflexes: Balance and equilibrium are maintained by interactions between vestibular / visual / somatosensory / proprioceptive sensations. Inputs from all these components are processed at the level of vestibular nuclei and cerebellum. Cerebellum plays a vital role in calibration of these inputs. Vestibular nuclei in response to these sensori inputs and inputs from the cerebellum sends out impulses that activates the motor components of vestibular reflex. These motor components can be classified into: vestibulo ocular / vestibulo spinal / and vestibulo collic reflexes. These reflexes helps us to maintain the balance and equilibrium of our body. Through examination of these reflexes in the new born and infants play a vital role in the understanding of vestibular system development. Vestibulo ocular reflex: The main use of this reflex is to stabilize gaze and maintain clear vision even while the head and body is in motion. This helps in retaining objects of visual interest within the confines of fovea of the retina. This is made possible via inputs from the semicircular canals and otolith organs. This reflex can be modified by the following factors: 1. Attention of the subject 2. State of arousal 3. Unintended ocular fixation due to light leaks 4. Insufficient head stabilization during the test Techniques used to record Vestibulo ocular reflex in children include: Caloric stimulation Rotational stimulation (torsion swing) Passive whole body rotation techniques The parameters studied include: Gain: This is the ratio of peak eye velocity to peak head velocity Phase: Timing difference between head and eye velocities Symmetry: Comparison between rightward and leftward eye velocities Drtbalu's otolaryngology online

It has been shown that vestibulo ocular reflex is present at birth but its values are approximately one half of normal adult values in neonates. This value reaches adult values by the age of 2 months. This has been attributed to the fact that visual pathways are immature at birth. Absence of this reflex beyond the age of 10 months should be considered to be abnormal.

Vestibulo spinal reflex: Whether the body is stationary or in motion continuous afferent signals from visual and vestibular inputs detects the body's orientation and relationship to gravity. These inputs are a combination of inputs from touch receptors of skin, proprioceptors on the soles, hands, joints and torso. Sum of these inputs provides information needed to generate the vestibulospinal reflex. Vestibulo spinal reflex out puts travel along three major pathways which include lateral spinothalamic tract, medial spinothalamic tract and reticulo spinal tracts. On activation these tracts stimulate the anterior horn cells of spinal cord thereby generating myotactic deep tendon reflexes in the antigravity skeletal muscles of the limbs and trunk. This reflex continues to develop till the age of 15. Vestibulo collic reflex: Drtbalu's otolaryngology online

This plays an important role in stabilizing vision by compensating for head movements when the body is in motion. Patterned contraction of neck muscles prevent undue head bobbing during walking and running. During walking / running vestibular signals caused by linear translations stimulate receptors of the saccule. In response saccule transmits afferent signals along the inferior vestibular nerve to the vestibular nuclear complex in the brain stem. Efferents from vestibular nucleus travel via the medial vestibulospinal tract and spinal accessory nerve to the neck muscles which include the sternomastoid muscle. This reflex is the one which is assessed by recording VEMP signals (Vestibular evoked myogenic potentials). This test is rather popular because it provides information pertaining to saccule and inferior vestibular nerve. This test can objectively be performed even in small infants by placing surface electrodes over sternomastoid muscle area. VEMP recordings appear as biphasic electromyographic potentials with an initial positive deflection at 13 milliseconds after the stimulus (i.e. High intensity auditory clicks) and negative deflection at 23 milliseconds. Spontaneous nystagmus: This is very important in the examination of vestibular system in an infant. Spontaneous nystagmus should be differentiated from gaze evoked nystagmus. Presence of spontaneous nystagmus indicates asymmetry in vestibular function and gaze evoked nystagmus indicates CNS disorder. Spontaneous nystagmus can be easily brought out by removing fixation with Frenzel's glasses. Examination for sponataneous nystagmus should be performed in all cardinal positions of gaze, but not in extremes of gaze. Nystagmus seen in extremes of gaze is physiological due to extraocular muscle activity. Patients who are recovering from acute peripheral vestibular injury the progression of central compensatory mechanisms can be observed by looking for spontaneous nystagmus which progresses from third stage to first stage. When the compensation is complete then spontaneous nystagmus is not present at all. Vertical nystagmus if present indicates central cause. Up beating nystagmus indicate pontine lesion while down beating nystagmus lesions at cranial cervical junction (chiari malformations). Hyperventilation will unmask nystagmus due to demyelinating lesions of brain parenchyma / or due to compression involving 8th cranial nerve. Fistula sign: Tragal compression causes horizontal nystagmus to occur in patients with fistula involving the lateral canal. Vertical / rotatory nystagmus indicate superior canal dehiscence. Cervical vertigo: Neck rotation with the head held stable produces nystagmus which is known as cervical nystagmus. Tests to identify dynamic imbalance: Head thrust maneuver / Head impulse test: While performing this test the patient is asked to fix the eyes on a stationary object while the head is moved laterally and vertically. If vestibulo ocular reflex is defective then corrective saccades can be seen.

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