Kernicterus and Hearing Loss

The most consistent impairment resulting from kernicterus is hearing loss. Newborns with excessive jaundice who suffered bilirubin toxicity may have hearing disorders while not showing any of the other hallmark signs of kernicterus.

During the onset of kernicterus brain damage, excess bilirubin moves from the blood into certain regions of the newborn’s brain. Both the cochlear nuclei in the brainstem and the auditory nerve are very prone to the toxic effects of bilirubin, even at comparatively low levels. The bilirubin staining damages the cell bodies of the basal ganglia and the auditory nerve that carries sound signals to the brain. As these areas in the baby’s brain are attacked, the end results are varying degrees of deafness and other hearing impairments. 

Some babies may appear to have normal hearing at first, as auditory problems often do not become fully visible until the child is able to display delayed speech development, or otherwise shows signs of hearing trouble.

Kernicterus hearing loss generally manifests as

  • Sensorineural hearing loss
  • Auditory neuropathy

Sensorineural Hearing Loss From Kernicterus

The mildest result of kernicterus is sensorineural hearing loss (sen-sor-ee-NUR-al) which basically reduces a child’s ability to hear sounds properly. Although speech or sounds may be loud and clear, to the child with sensorineural hearing loss,

  • voices sound dim or muffled
  • certain sounds seem too loud
  • it is hard to follow conversations if more than one person is speaking
  • it is difficult to hear in noisy areas

Sensorineural hearing loss can range from mild to profound, and is rarely surgically or medically reversed.

Auditory Neuropathy From Kernicterus

Neuropathy means malfunction of the nerves. Auditory neuropathy (AN) is a form of sensorineural hearing loss where the auditory nerve is damaged and sound cannot process properly. The inner ear receives sounds normally, but the transmission of signals from the inner ear to the brain is impaired.

Audiograms of children with AN vary from hearing in the normal range, to difficulty hearing over background noise, to profound hearing loss. Most people with auditory neuropathy experience sounds as out of sync and fading in and out. They have problems distinguishing sounds and difficulty understanding speech.

Treatment for Kernicterus-related Hearing Disorders

Hearing professionals generally urge parents of children with kernicterus hearing loss to work with a team of hearing health experts, in order to engage in the best ways to teach these children how to communicate, as well as promote their overall education. Methods for teaching communication may include using sign language as a first language and developing special listening skills, often in combination with hearing aids or cochlear implants.

According to the National Institute on Deafness and Other Communication Disorders (NIDCD), researchers are actively pursuing effective treatments for children with AN. The benefits of cochlear implants have been positive for deaf children with kernicterus-induced auditory neuropathy; children appear to respond favorably, with many being able to hear and speak after implementation.

Living With Kernicterus Hearing Loss

Even limited loss of hearing can interfere with a young child’s ability to speak and understand language. Most children with kernicterus are fully cognitive, aware, and able to learn and interact. However, these children are often mistakenly perceived as being mentally impaired due to their hearing disorders and resultant flawed speech, gaze abnormalities, or the telltale movement disorders of cerebral palsy

Parents of children with kernicterus hearing loss are trying to understand their child’s condition, and learn where they can turn for help. There are excellent rehabilitative and financial resources that can help you and your child adapt to a fulfilling and productive life.

Read more about these special children who will develop through their teenage years to become adults living with kernicterus hearing loss or cerebral palsy.

The failure to identify the medical emergency of severe jaundice is wholly avoidable. Ratzan Law Group helps families face the challenges of kernicterus through our belief in pursuing and speaking the truth about this preventable birth injury.

Kernicterus Information Center