February 17, 2004 Feature

Falling Through the Cracks: One Long Journey to Correct Identification

When our daughter Ruthie was born in December 1999, she failed the newborn hearing screening test administered at the hospital. In response to the technician's questions I explained that my husband, Aaron, and I have an older daughter, Hannah, then 2, with normal hearing, and that there was no known history of deafness in either of our families. Although my pregnancy had been normal, Ruthie was late in arriving and I was induced about a week past my due date. The technician informed me that Ruthie, like many post-term babies, probably had fluid in her ears. Once the fluid cleared, she would certainly pass the test.

After 24 hours in the hospital I was feeling fine and anxious to go home. Ruthie and I were discharged and sent on our way with a sheaf of papers, one of which informed me of the date and time for Ruthie's follow-up otoacoustic emissions test.

In all honesty, I forgot about it. My best friend, who had been fighting breast cancer for two years, died five days after Ruthie and I returned from the hospital. It certainly seems possible now that I was in a bit of a fog from lack of sleep and mourning, but the brief discussion I had with the newborn screening technician in the hospital raised no red flags, and the possibility that Ruthie really had a hearing problem seemed so remote that the necessity to retest her just did not register in my consciousness.

Ruthie was a robust, happy baby. She was easy, content to watch her sister, our dog, the fish in the fish tank. She startled when the dog barked and made some sounds on her own, mostly spitty blowing through her lips-"raspberries." We noticed that she did not babble as Hannah had, and by the time of her first birthday Ruthie certainly had less spoken language than Hannah, who began to speak at 9 months and has rarely paused since.

Ruthie said "Mama," "Hannah," and "Yad-yeah" (Dada). Once when her sister was sick, lying on the couch and watching television, Ruthie became so frustrated with her fruitless attempts to get Hannah up to play that she threw a sippy cup at her and screamed, "Hannah!" One evening, Aaron and I were sitting in our room soon after both girls had gone to bed when we heard a voice say "Hannah, Ha-nnah," very carefully over and over again. We assumed that it was Hannah herself, but when we went to investigate we found Hannah fast asleep and Ruthie lying on her back, speaking up at the ceiling.

Early Concerns

Even at the time, these incidents were noteworthy to us because Ruthie spoke so seldom. At her 1-year check-up we mentioned our concerns about Ruthie's slow speech, and her pediatrician, after counseling us not to worry because all children develop differently and in their own time, recommended that we have her hearing tested, just in case.

During the two weeks leading up to Ruthie's appointment with a local ENT for a hearing screening, Aaron and I began to watch her reactions to sound much more closely. Although her responses seemed inconsistent, we could both point to instances where she clearly seemed to not react to loud sounds. By the time of her appointment, we were both ready to accept-and expectant that we would learn-that her hearing was not perfect. And yet, when I mentioned our concerns to Ruthie's daycare providers, who cared for her three days a week while I was at work, and her grandparents, who saw her less frequently but were close observers of her, I was met with staunch disagreement. They each had their own favorite example of Ruthie responding to sound. How could she be deaf?

The ENT our pediatrician referred us to had a general practice treating both children and adults. Ruthie sat on Aaron's lap in the booth while two young audiologists conducted the test. Aaron, Ruthie, Hannah, and I waited afterward in an examining room for the ENT. He told us that Ruthie had tested "within normal limits" and asked what had prompted our concern. Aaron and I mentioned Ruthie's inconsistent response to sound and our speech concerns. The doctor chatted with Hannah for a bit and then told us that he had a similar situation in his own family: His oldest child, a daughter, spoke very early while his son was somewhat delayed in speech. He told us that with speech treatment his son was now doing quite well. Although protocol dictated that he suggest that Ruthie have a follow-up auditory brainstem response test (ABR), his recommendation was that we pursue speech treatment and forego the trauma of the ABR, for which Ruthie would need to be sedated.

Aaron and I left the ENT feeling strangely deflated. We were both increasingly concerned that something was wrong with Ruthie. But if it wasn't her hearing, what was it?

We discussed the ENT's recommendation with our pediatrician who reasoned that if Ruthie passed the hearing test, there was no real rationale for the ABR. He wrote us a referral for speech treatment at a local hospital.

We were unable to get an appointment to have Ruthie's speech and language evaluation for several months. Our pediatrician counseled patience, since many children's language delays resolve themselves. Perhaps once Ruthie's appointment rolled around she would be making more sounds and expanding her very limited vocabulary.

Unfortunately, the changes we noticed were all for the worse. Our formerly jolly, cheerful child was now regularly given to tantrums and would bang her head against the wall or floor in frustration. She had stopped trying to say "Dada" and said "Hannah" and "Mama" less frequently. As she entered her second year her inability to communicate became increasingly pronounced, and as she became more mobile her behavior was harder and harder to control. We waited anxiously for the speech and language evaluation.

As we expected, Ruthie was found to have below-average receptive and expressive language skills. At 17 months of age, the speech-language pathologist's report noted that on the Rosesetti Infant-Toddler Language Scale, Ruthie's language was 6 months (baseline) and scattered up to 15 months of age.

We decided to pursue speech services through our county's infant and toddler program. As part of the process to determine eligibility, Ruthie's speech and language skills were once again assessed. She enjoyed and cooperated with this evaluation much more than the previous one. Based on the Infant Mullen Scales of Early Learning, at 19 months old, Ruthie's receptive language was found to be at 15 months and her expressive language was at 10 months.

At home Ruthie's frustration continued. She had stopped saying "Hannah" and said "Mama" only occasionally. Her behavior seemed impulsive; she was becoming harder and harder to reach.

Treatment Begins

Ruthie began speech treatment at 21 months. We saw the clinician at home, at first every other week and then weekly. I enjoyed the sessions and the interaction with another adult who approached Ruthie's lack of communication with seriousness and compassion. Ruthie, however, was often resistant. She enjoyed playing, especially with baby dolls, but she fought to control the agenda and often refused to make eye contact with her clinician.

Although Ruthie's response was minimal, she did seem to be making some slow, halting progress. Her oral motor skills improved, she began to blow and kiss. She clicked her tongue against the roof of her mouth to mimic a horse's hooves. Playing with clay, she hissed like a snake. Very occasionally, and never with her clinician, she would whisper "open."

And still her frustration was unchecked. It seemed she could cry and rage for entire weekends. We introduced a few American Sign Language (ASL) signs to facilitate communication: open, more, help, mine. Ruthie readily used the signs.

Ruthie's behavior was a great and continuing concern. I sought advice from an early childhood behavioral specialist. While she was with us, Ruthie climbed onto my lap with a package of string cheese and whispered "open." The behavioral specialist suggested that Ruthie might have selective mutism-a possibility her SLP rejected, arguing that she would need to have a working vocabulary before she could willfully choose not to use it. The behavioral specialist also told me that sometimes mothers and children are simply not a good fit. I might be a fine mother for Hannah, but it seemed I was the wrong mother for Ruthie.
I remember thinking: I am ready to do anything, but that may be the one thing I can do nothing about.

When Ruthie turned 2, she enrolled in preschool three days a week at the school Hannah attended. She was very happy there, loved being part of the group, doing art projects, and playing outside. She had a wonderful team of teachers I knew well because they had taught Hannah as a 2-year-old.

Before the first month was out, Ruthie's teachers asked me if we had ever had her hearing tested. Ruthie could follow the routine at the table or at circle time, but they could never get her attention across the room. Aaron and I knew that after months of trying we were really no closer to unraveling Ruthie's trouble. We had carefully considered all suggestions from slight autism to "Einstein syndrome" (children who develop language late in deference to their precocious analytical skills). We needed to revisit the issue of her hearing.

Aaron took Ruthie to have her hearing tested again in mid-March 2002. Ruthie was 2 years, 3 months old. She was found to have a profound bilateral sensorineural hearing loss. A week later the ABR test confirmed the booth results; she showed no response at any frequency. Our best guess about Ruthie's hearing is that she was born with a loss that deteriorated over the course of her second year.

Ruthie at 3

Ruthie is now 3 years old. She is fluent in sign language and will soon mark her one-year cochlear implant anniversary. Currently enrolled five days a week in the school system's total communication preschool program, Ruthie loves school. Her awareness of sound, receptive language, and expressive abilities continue to improve. Hannah is in first grade and the designated sign language expert for her class.

Aaron and I faced a very steep learning curve, and continue to negotiate it. ASL was a godsend to our family, allowing us to open the lines of communication with Ruthie very rapidly once we understood her hearing loss. We are finding sign to be an invaluable tool and bridge as she pursues and gains skills in receptive and expressive English.

We have found wonderful support from our state school for the deaf, local school system, cochlear implant team, and maybe most of all from the parents of other children who are deaf.  

Anne Dooley, a freelance writer, and her husband, Aaron Ferster, live with their daughters Hannah and Ruthie in Rockville, MD.

cite as: Dooley, A. (2004, February 17). Falling Through the Cracks: One Long Journey to Correct Identification. The ASHA Leader.

Tightening the Safety Net

Ruthie was surrounded by well-meaning professionals, family, and friends, yet her proper diagnosis eluded us for more than a year. Here are some suggestions to follow up on the infant screening process so that children don't slip through the cracks:

  • Because deafness is a low-incidence disability, many otherwise well-trained child development specialists know very little about it. Basic information (like AGBell's "So Your Child Has Hearing Loss: Next Steps for Parents" pamphlet) should be made available to both health professionals and parents if a hearing loss is even remotely indicated.
  • For any hearing issues, pediatricians should direct parents to-and parents should insist on-audiologists and ENTs with special interest in pediatric patients.
  • If speech issues in young children are not significantly improved over a period of six months, parents should seriously consider having their child's hearing (re-)tested. Hearing can deteriorate rapidly.
  • In counseling parents, both ENTs and pediatricians should counter the possible negatives of the ABR test experience with the information that behavioral hearing testing is notoriously unreliable in young children.
  • When a hearing loss is suspected, parents will find the parents of other children who are deaf an invaluable resource of personal experience and perspective.


  

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