For fans of Dr. Who, you will know that "The Impossible Girl" refers to one of his companions, Clara Oswald, who seems to be rather typical but turns out to have quite a surprising life story. That, in a nutshell, describes Anandi.
Last week, we took Anandi to Spokane for a sedated ABR, which tests the function of the auditory nerve. She'd recentlly failed an electronic test of her cochlea in both ears in the frequency range that would correspond with a quieter speaking voice and below, which explained why she had started saying, "What you say?" and "I can't hear you!" at times. She had never been able to reliably complete a standard hearing screening that involved raising a hand in response to a tone administered through headphones, as her responses were too random. But over the years, speech therapists and others had reassured us that she seemed to have normal hearing, as she was able to imitate all the phonemes correctly, even though she needed a lot of coaching to pronounce things correctly on a consistent basis. She also had had a normal tympanogram (functional test of the middle ear/eardrum.)
Nevertheless, David and I too often experienced her as functionally deaf, and our gut instincts said we needed to follow up with further testing. We were also growing increasingly concerned by her expressive language delay, as her speech gains were no longer tracking her chronological gains.
The results of the ABR were unequivocal, and the doctor gave us a diagnosis of Auditory Neuropathy Spectrum Disorder (ANSD), formerly known as Auditory Neuropathy/Auditory Dyssynchrony (AN/AD). Functionally, this means that while Anandi can hear (although even that is impaired in the lower frequency ranges), her auditory nerve doesn't properly synchronize the incoming sounds so that they're intelligible to her. The analogy we were given is listening to static between two radio stations. You can hear words being spoken, but you have to struggle extremely hard to decode the words being transmitted. It tends to worsen throughout the day as the auditory nerve becomes fatigued, which correlates with our observation that after school and in the evenings she would become increasingly withdrawn, irritable, irrational, etc. It also makes it extremely difficult to impossible to distinguish speech when background noise is present. Hence, she has used her cleverness to follow cues and imitate what others are doing when she hasn't been able to understand in classroom settings, etc., and thus has managed to fly under the radar for having a severe hearing disorder diagnosis.
The amazing thing about this diagnosis is that it's normally given to much younger children, as their lack of language development would lead to this testing much earlier on. Only recently has she begun to verbalize not being able to hear/understand at times, but this isn't a progressive condition; she's most likely had it since birth. It's a very rare condition to begin with, but fewer than 1 in 14 diagnosed with it will ever develop normal speech and language. The fact that Anandi has coped and adapted to the distorted sounds she has been hearing and managed to develop the level of understandable expressive language she has (currently about a 4-year old level at age 7-1/2) without any interventions whatsoever is bordering on the...well, impossible. She has had to struggle with a very uncomfortable sensation for years without her parents and teachers being aware so that accommodations and interventions could be made. She has, in fact, been quite a trooper! I feel both humbled by her miraculous accomplishments despite our missing this staggering piece of information, and ashamed in retrospect for how impatient I've been with her at times for her "not listening" to me.
We will be following up with an audiologist to try both FM transmitters and hearing aids; the former is useful in classroom situations to boost the signal when there is background noise, and hearing aids to boost it in general. But sometimes the hearing aids merely amplify the static. Cochlear implants have sometimes had dramatic effects, seeming to synchronize the auditory nerve transmissions and clear up the static, but other times they have had no discernible effect, and we are by no means ready to consider such a drastic intervention. So we will be moving forward with prayer and hope that we will be able to find a way to improve her hearing, and that our "impossible girl" will have an easier time of things moving forward.
