Hearing, talking, signing – Auditory Neuropathy at 14 months old

Your baby is always your baby, right? No matter how little or grown up they are.

Clara is 14 months old today and has noticeably grown so much in the past 4-6 weeks.

We sat in Pret in the children’s hospital this morning, before Clara’s latest audiology appointment, and she was contently reading her book whilst I had a coffee. Opposite us was a baby boy, no bigger than 4 months old. By comparison, Clara suddenly felt so grown up!

Clara’s hearing

If you’re new to our journey, it’s useful to know Clara has Auditory Neuropathy Spectrum Disorder (ANSD), a condition affecting the nerve between the ear and the brain.

Presently, we still don’t know to what extent Clara’s hearing and speech is impaired (if at all). On paper, when her Brainstem response is tested, she’s profoundly deaf – there’s no response at all. We’ve not been able to re test this successfully now she’s older as it requires her to sleep at a specific time in a test room at the hospital during her appointment – she’s too wise for that!

0.5% of the child population have ANSD, and of those the majority have significant hearing loss. Neonatal babies who spent time in intensive care are particularly high risk.However, Clara is proving she can hear. Another test she has involves playing actual sounds and rewarding her correct turn of the head with a puppet that lights up and moves. Unfortunately today she took a great dislike to these. We know she struggles to hear the quieter lower pitched noises as we rarely get a result then. But to the rest of the noise spectrum, she responds well.

She’s also confidently responding to verbal instruction:

If I say “breakfast, lunch, dinner, or eat” she smacks her lips together like a fish, replicating half of the sign language movement we taught her of bringing hand to mouth and chewing.

If I say No, she stops whatever she’s doing and looks at me. If she’s facing away from me, or has crawled away, she’ll turn around to look at me. Often, she’ll shake her head no in response.

If I say “say hello” or “say goodbye” she’ll wave, with no visual prompts.

If I say “aaaah”, she’ll tilt her head and look up bashfully, knowing she’s super cute!

If I sing, “Wind the bobbin up” she’ll do the hand movements, rolling her hands in motion and clapping at the right time.

If I say, “where’s my nose” or “where are your hands”, she’ll tap my nose or stretch out and slowly move her hands.

If I show her a picture or say “rabbit”, she’ll sign rabbit.

So we are just going to sit and wait, and let Clara do her thing.

Verbalisation and sign language

Clara has learnt she is able to communicate in a variety of ways. She’s found her voice – there’s a lot of shouting! She’ll shout “cat” excitedly when she sees our cat. “Dada” is said too when we see or talk about Dean. There’s babbling “mama” and “nana” too.

But the best bit so far is Clara’s ability to use sign language.

She’ll now confidently sign that she’s finished/all done. She’ll sign for more if she wants something (usually snacks!) to continue. She’ll sign “rabbit” and motion movements in songs at the right time.

In this video Clara signs “rabbit” after being verbally prompted. I then follow it up with the sign to confirm it was correct, supported by verbal praise.

In this video Clara indicates she’s finished by pushing the plate away and signs “all done” by a single repeated hand wave side to side.

We sign “nappy change” to her when we lay her down to change her because otherwise she protests. After the simple sign, she lay contently until it’s done.

Without hesitation, I recommend baby sign language classes (we went to sing & sign).

Clara might not be able to verbalise her thoughts, but she knows she’s able to communicate some of them which eases a common frustration at this age.