Labyrinthine Problems in Infants

The labyrinth is the inner ear network responsible for auditory ability and balance. In specific, it is composed of small, protective bones surrounding delicate organs responsible for hearing, including semicircular canals, saccules, the cochlea and the utricle. Damage to any of these organs or bony framework in infants results in debilitating conditions that can render a baby deaf and/or unable to develop motor skills.

Congenital Viruses

• According to researcher Larry E. Davis, infants exposed prenatally to rubella may be born completely deaf or hearing impaired. Rubella virus contracted from the mother causes malformations in the temporal bones of the labyrinth including cochleo-saccular degeneration and vascular stria atrophy. The wall of the saccule and the Reissner's membrane often sag and collapse. Sometimes the tectorial membrane develops separately from the corti organ. According to otoneurologist Dr. Timothy Hain, infants can contract cytomegalovirus in utero or after birth via breast milk. Varying forms of hearing loss and motor development delay result from untreated congenital cytomegalovirus. Mild to severe hearing loss can manifest with a normal cochlea and malformations to the inner ear canals and vestibular aqueducts. Malformed cochleas are representative of moderate to profound deafness and appear as holes in the cochlea and incomplete inner ear partitions.

Affects of Congenital Bacterial Infection

• Similar to viral rubella exposure, infants who contract syphilis from their mothers do not experience apparent balance or vestibular deficiencies but may become deaf. Deafness often occurs in both ears of equal severity. Labyrinth biopsies performed on affected infants revealed syphilis organism infiltration and complete destruction of the inner ear. Subsequent aggressive symptoms that accompany deafness include stiff necks, inflamed optic nerves, swollen lymph nodes, skin rashes, headaches and cranial nerve palsies. While children over eight years of age may experience a reversal in hearing loss with the use of adequate antibacterial treatment, deafness is a permanent consequence of congenital syphilis.

Balance Dysfunction

• According to otolaryngology specialist, Dr. T. Balasubramanian, doctors perform several tests of infant reflexes to diagnose inner ear-related imbalance and dizziness problems. The absence of certain reflexes are representative of labyrinthine disorders. From 32 weeks to five months of age, healthy babies placed in certain positions demonstrate reflexes of the neck, which are reflective of normal distribution of endolymphatic fluids through inner ear canals. From ages 6 to 18 months, positioning tests are performed to elicit a normal response known as the righting reflex during which a baby will attempt to position its head to the right in an effort to maintain balance.

Genetic Syndromic Deafness

• Dr. Timothy Hain explains that there are two genetic abnormalities that result in syndromes that involve complications and malformations of labyrinth components. These include Klippel-Feil syndrome and Usher's syndrome. Labyrinth anomalies of Klippel-Feil syndrome involve a malformed inner ear, enlarged vestibular aqueducts, low hairlines, restricted neck movements and abnormally short necks. Babies with Usher's syndrome often initially experience hearing loss, vision loss and/or balance impairments. Dr. Hain further explains that some Usher's syndrome patients benefit from cochlea implants.