Family hopes operation helps boy hear again

From: Hibbing Daily Tribune, MN - 07 Jan 2003

By KELLY GRINSTEINNER
The Daily Tribune
Last Updated: Tuesday, January 07th, 2003 09:06:26 AM

HIBBING — For most children, the noisier a toy is, the better.

But for Alex Jensen, who will turn four years old in a matter of days, a clamorous toy means a bit more. Its gives him a better chance of hearing and recognizing sound.

Jensen suffers from auditory neuropathy, a condition in which a person has normal organ function but can not hear sounds. He was diagnosed with the condition in October.

“He can hear sounds but somewhere between his ear and his brain there is short circuit,” explained Lenora Jensen, Alex’s mother. “He only gets bits and pieces.”

Lenora compared Alex’s hearing to bad radio reception — sound enters a perfectly operational radio but is not able to be transmitted through the wire and into the speaker correctly. To help him hear, Alex wears a hearing aid in each ear.

“He has lost almost all of his hearing,” said Lenora in a slightly raised voice enabling her to be heard above Alex’s loud toy. She smiles at him, knowing the toy isn’t loud in Alex’s mind.

Alex will be one of the first children in Minnesota north of Duluth to receive a cochlear implant. He will undergo surgery at the Mayo Clinic this month.

“We (she and husband Craig) didn’t feel he was getting enough benefit from the hearing aids,” said Lenora. “They amplify sound but Alex needs specialized sounds. The implant will help him process sound.”

Alex was born at the University Medical Center-Mesabi at a time when newborns were not screened. The family is unsure whether Alex was born with any hearing loss. Between 18 months and two years of age, the family became suspicious.

“He wouldn’t respond when he was called,” said Lenora. “And he wasn’t developing speech. He could say ‘mama’ and ‘daddy’ but wasn’t able to form full sentences, which he should have been able to do at that age.”

The family also noticed Alex would have good hearing days and bad hearing days. By two and a half years of age, Alex stopped speaking completely. It was then the family decided to seek help.

Alex underwent a series of hearing tests in late October of 2001. From those tests, doctors were able to determine Alex was experiencing moderate to severe bilateral sensoriuneural — nerve damage in both of his ears causing hearing loss. He was quickly fitted with hearing aids.

The aids seemed to work for a while. Alex was speaking again and able to pick up a few more words. But less than a year after being fitted with the hearing devices, Alex stopped saying things clearly.

“His words were becoming mushy,” Lenora said, noting Alex makes more vowel than consonant sounds. “We could tell he was getting worse.”

The Jensens then sought the assistance of doctors at the Mayo Clinic in Rochester, Minn. Through more hearing tests, they were able to identify Alex’s condition. It was also at this time they considered a cochlear implant.

“We want to give him access to everything he wants to do in life,” said Lenora. “It is no different than a parent wanting a child who suffers from diabetes to have the new insulin pumps. We as parents do whatever we can for our children, and this is what is best for our child.”

Doctors believe Alex’s condition is hereditary, but Lenora said she and Craig have yet to find a relative in their lineage with such an affliction.

A cochlear implant is a highly technical, surgically implanted medical device that electrically stimulates the hearing nerve in the cochlea, or inner ear. The simulation is designed to allow individuals with severe to profound hearing loss perceive sound.

The implant was first developed in the late 1950s. After years of research and testing, it was approved by the Food and Drug Administration for adults in 1985 and for children in 1990. They have been used on auditory neuropathy patients since the mid- to late-1990s.

Over 30,000 people worldwide have cochlear implants, according to the Cochlear Implant Association, Inc. Over 8,000 of that population are children.

A cochlear implant consists of three parts: internal components, a speech processor and speech coding strategies.

Surgery is required for placement of the internal components. An electrode array, a thin coil which simulates sound by simulating nerve fibers, will be placed in Alex’s cochlea.

Once the internal components are programmed, he will wear an external headset and a fully digital, body-worn speech processor. The speech processor is a microcomputer that converts sound and speech into electrical information which is then sent to the implant and on to the auditory nerve where it is perceived as sound. The processor is attached to the headset with a thin cord that sends electrical sound information to the implant.

Speech coding strategy refers to the technique the speech processor uses to translate external sound information into the signal that is delivered to the implant.

Alex’s family and teachers will have to undergo auditory training to enable them to communicate with the youngster once the implant becomes his main means of communication.

He is currently in the Early Childhood Family Education program and is expected to attend preschool next year. He will likely need deaf/hard of hearing assistants.

Positive the implant is the right decision for Alex, Lenora at this point simply hopes the surgical procedure goes well.

“I highly expect the implant will be great for him in the long run,” Lenora said, adding the family will continue to learn sign language. “It will be a lot of work at first. Not more work, just different.”

© 2003 The Daily Tribune