With the help of Adventist Hinsdale Hospital, Dr. Robert Battista has Photo of upside down child started the Adventist Pediatric Audiology and Cochlear Implant Program. The Adventist Pediatric Audiology and Cochlear Implant Program has been designed to evaluate and treat children of any age with a hearing disorder. The program is located in the Paulson Pediatric Rehabilitation Center, which provides a state-of-the-art facility in a child-friendly atmosphere. Members of the Audiology and Cochlear Implant Team consist of professionals of various disciplines associated with the evaluation and treatment of children with hearing loss. These professional disciplines include: pediatric audiology, otology/neurotology, speech and language pathology, pediatric psychology, genetics, and pediatric ophthalmology.

When a child has a hearing loss, the child’s entire support system is affected. For this reason, the Adventist Pediatric Audiology and Cochlear Implant Team is dedicated to working closely with the child’s family, physician, and school professionals in order to ensure the best outcome for the child.

The following is a summary of the services provided by the Adventist Pediatric Audiology and Cochlear Implant Program.

Newborn Hearing Screening
Hearing Evaluation for Infants to School Age Children
Pediatric Hearing Aid Fitting
Cochlear Implant Evaluation/Programming
Speech/Language Therapy for the hearing impaired

Location/contact information about the Adventist Pediatric Audiology and Cochlear Implant Program (located in the Paulson Pediatric Rehabilitation Center):

Adventist Paulson Pediatric Rehab
222 E. Ogden Ave.
Hinsdale, IL 60521

630-856-2600
fax 630-856-2634

Family history of hearing loss (genetic hearing loss)

Genetic sensorineural hearing loss is divided into two general categories: non-syndromic, meaning hearing loss and nothing else, and syndromic, meaning hearing loss with other clinical findings, such as heart, kidney or skin abnormalities. By far, the more common is non-syndromic sensorineural hearing loss, which includes 2/3 of all genetic hearing losses.

There are numerous genes that are associated with sensorineural hearing loss. One gene, known as GJB2 is responsible for approximately 1/3 of all the cases of genetic sensorineural hearing loss. GJB2 is a gene that is necessary to produce a protein known as Connexin-26 (abbreviated CX26). CX26 is a protein necessary for the inner ear to develop. So, when there are abnormalities of the GJB2 gene, then CX26 is not produced, and the inner ear does develop properly. Children with CX26 abnormalities most commonly have a bilateral, severe-to-profound sensorineural hearing loss. Abnormalities of the GJB2 gene can be identified with a special blood test.

Congenital infection

Congenital means that something occurred during pregnancy or at the time of birth. Sensorineural hearing loss may develop if a woman contracts certain types of infections during pregnancy or passes the infection to the child during birth. The types infections associated with pediatric sensorineural hearing loss include: toxoplasmosis, syphilis, rubella (German measles), cytomegalovirus (also known by the initials CMV) and herpes. The amount of hearing loss that can result varies widely and some babies show no hearing loss at all, even if they have one of these infections. Congenital CMV infection is the leading cause of acquired hearing loss in infants.

Bacterial meningitis

One illness that carries a high risk of causing hearing loss and/or balance problems is bacterial meningitis. Because meningitis is an infection of the lining of the brain and spinal cord, the sense organs of hearing and balance are especially sensitive to this infection. Pneumococcal meningitis is the most common cause of permanent, sensorineural hearing loss. Other causes of meningitis, such as E.Coli and group B streptococcal, are also causes of sensorineural hearing loss.

Ototoxic medications

Sometimes medications that are known to be ototoxic (damaging to hearing) are prescribed to babies, usually to treat serious infections or birth complications. The most common ototoxic medications used at this time include a family of antibiotics called aminoglycosides with names such as gentamicin, tobramycin, kanamycin, and streptomycin. The aminoglycosides present more of a risk to hearing when they are used multiple times or in combination with other medications, such as diuretics.

Hyperbilirubinemia

Hyperbilirubinemia (jaundice) that is severe enough to require a blood transfusion can also result in sensorineural hearing loss. This is related to the potential damage that high levels of bilirubin can cause to the nerves of hearing and portions of the brain used for hearing.

Auditory neuropathy/auditory dyssynchrony

Auditory neuropathy/dyssynchrony is a condition in which the inner ear (cochlea) is working properly, but the hearing (auditory) nerve that carries the hearing signal to the brain is not. The condition is called auditory neuropathy/auditory dyssynchrony because the auditory nerve is not working properly (neuropathy) and the signal sent to the brain is not in sync (hence the term, dyssynchrony) with the electrical signal generated by the normally functioning cochlea. The amount of dyssynchrony can vary from person to person and can fluctuate in an individual over time.

Frequently asked questions regarding auditory neuropathy/dyssynchrony:

(click on the question to show the answer; to close the answer, click on the question again)

+ How is auditory neuropathy/auditory dyssynchrony diagnosed?

The condition is diagnosed when a patient has normal otoacoustic emissions (OAEs) and abnormal auditory brainstem responses (ABR). OAEs measure the ability of the cochlea to collect sound. Thereby, OAEs do not measure how the auditory nerve or brain responds to sound. Therefore, typical newborn hearing screening programs that only use OAEs for hearing screening may not identify a child with auditory neuropathy/auditory dyssynchrony if the OAE is normal.

+ Are there risk factors or case history information that can help in identifying children who potentially have auditory neuropathy/auditory dyssynchrony (AN/AD)?

Yes, but many children who have AN/AD do not have any risk factors. The risk factors that have been associated with AN/AD are (1) hyperbilirubinemia ranging from just above normal to quite high, including infants that have gone through exchange transfusions, (2) premature (25 to 36 weeks gestational age), and (3) perinatal asphyxia (i.e. lack of oxygen at the time of birth). AN/AD can also run in families, following both recessive and dominant inheritance patterns.

AN/AD should be suspected when a child has some level of measurable hearing with routine hearing testing, has been fit with high-quality hearing aids and still does not progress in speech and language development.

+ Can cochlear implants benefit people with auditory neuropathy/auditory dyssynchrony?

Yes. Research has shown that electrical stimulation, such as that from a cochlear implant, can help to synchronize activity of the impaired portions of the brain and allow use of auditory information. Even for children who receive implants, it is important to allow them to gradually move from a visual language system to an auditory language. Abrupt removal of the communication system that a child has depended upon prior to receiving a cochlear implant is not recommended and could interrupt smooth continuation of progress.

Newborn hearing screening is mandatory in the state of Illinois. Even if a Newborn ear child passes newborn hearing screening, however, adults or caregivers should be alert for signs of hearing loss. This is because hearing loss may develop after a child is a newborn. In fact, almost 30% of children with a hearing loss are diagnosed some time later in life, even after passing their newborn screening.

The following is a checklist that will assist in determining whether a child has a hearing loss. Please read each item carefully, and make a note of those factors that pertain to your child:

Indicators for Hearing Loss

Newborn (birth to 28 days of age)

Weighed less than 3.5 pounds at birth
Has an unusual appearance of the face or ears
Was jaundiced (yellow skin) at birth and had an exchange blood transfusion
Was in neonatal intensive care unit (NICU) for more than five days
Received an antibiotic medication given through a needle in a vein
Had meningitis
Failed newborn hearing screening test

Infant (29 days to 2 years)

Received an antibiotic medication given through a needle in a vein
Had meningitis
Has a neurological disorder
Had a severe injury with a fracture of the ear with or without bleeding from the ear
Has recurring ear infections with fluid in ears for more than three months

Response to the Environment (speech and language development)

Birth to 6 months

Does not startle, move, cry or react in any way to unexpected loud noises
Does not awaken to loud noises
Does not freely imitate sound
Cannot be soothed by voice alone
Does not turn her/his head in the direction of a voice

6 through 12 months

Does not point to familiar persons or objects when asked
Does not babble or babbling has stopped
By 12 months does not understand simple phrases such as “wave by-by”, “clap hands” by listening alone

3 months through 2 years

Does not accurately turn in the direction of a soft voice on the first call
Is not alert to environmental sounds
Does not respond on first call
Does not respond to sounds or does not locate where sound is coming from
Does not begin to imitate and use simple words for familiar people and things around home
Does not sound like or use speech like other children of similar age
Does not listen to television at a normal volume
Does not show consistent growth in the understanding and the use of words

If one or more of these items pertains to your child or a child that you know, then the child may have a hearing loss. If so, then the child’s hearing should be fully evaluated. When appropriate, his or her speech should also be evaluated by a speech and language pathologist.