It’s a weird coincidence I’m writing this on ‘Rare Diseases Day’. But Clara has added another string to her already medically complex bow: Auditory Neuropathy Spectrum Disorder.
Auditory Neuropathy Spectrum Disorder is a hearing condition where the inner ear hears sounds normally but has trouble sending them to the brain for processing. It’s rare – Clara doesn’t tend to opt for common conditions clearly. Princess by name, Princess by nature!
At this stage we don’t know how much Clara can hear. Her hearing loss might be mild, or she could be completely deaf. The tests at this stage are unable to indicate any more, we need to wait until she’s older to find out. Waiting, again, is tough.
The hearing tests yesterday were carried out in the Audiology department in a special booth. Clara lay in her pushchair and they attached a sensor behind each ear and one on her forehead. They then played various frequencies through a headphone on one ear at a time, and the sensors would send a response to the computer. Unfortunately, the sensors didn’t send clear responses – that’s how part of the diagnosis was made.
Typical of ANSD, the inner ear works normally but the brain stem responses are abnormal or absent. The audiologist said it was very clear that Clara had Auditory Neuropathy Spectrum Disorder as this is exactly what the tests demonstrated.
Taking on this news was tough. I was devastated. Not for myself, but for Clara. Why should someone so little who’s already fighting so much, including a rare disease, have to fight something else simultaneously? It’s not fair on her.
Does this mean she’s never heard any of the thousand ‘I love you!’s? Does it mean every time I’ve sang to her it’s not been the song but the gentle rocking that’s nursed her to sleep? Why does she settle so well when I read to her, book after book after book? How do I make her happy now? How do I comfort her?
Does this mean Clara can’t hear Ewan? She loves him so much, talking and smiling at him. She must be able to interact with him somehow.
I’m convinced, and I’m hopeful, she can hear something. I’m confident she does react to sound on some occasions (she definitely doesn’t react at other times). But now it makes sense why she barely notices her music therapist in some sessions, and loves him in others. The ‘spectrum’ part of the disorder means some days she could hear better than others, even some hours she could hear better than others. Assuming she can hear, that is.
The problem is we won’t really know for a few more months. When she’s old enough to sit up and turn to face sounds they’ll test her again. In the meantime, we’ll be referred to family support workers for children with hearing loss and they’ll investigate what they think caused this to happen. Severe jaundice and prematurity are known risks – Clara ticks both those boxes, but it could be something else. There’s still white matter on her brain seen on an MRI that needs understanding.
Whether Clara can hear or not, it doesn’t matter. It doesn’t change how much we love her, it doesn’t change how successful she can be in life. She’ll just interpret life in a different way. It’s another reason Clara is special, and it’s another reason we need to give her every opportunity life has to offer.
Come on Clara, show us your strength again.