Spontaneous TAPS Donor Twins and Deafness

One of the most interesting findings that came out of the long-term effects of Twin Anemia Polycythemia Sequence research is that 1 in 8 spontaneous TAPS donors was profoundly deaf. This finding shocked many researchers, as it helped change the thinking that TAPS was a mild disease and not anything to worry about. We now know differently, and this article will talk about this complication and how you can advocate for the proper tests. Here’s what you need to know about deafness in TAPS twins.

deaf taps twins twin anemia polycythemia sequence donor auditory neuropathy spectrum disorder ansd

Long Term Effects

In the paper, “High risk of long-term neurodevelopmental impairment in donor twins with spontaneous twin anemia–polycythemia sequence.” by Tollenaar et al.,  published August 20th, 2019, this unique complication was described.  The deafness is caused by Auditory Neuropathy Spectrum Disorder (ANSD) and is unique to spontaneous TAPS donors. 15% of donors in the study (5/34) have been recorded with this type of deafness, and since the publication of this article, more cases have come to light. 

There are also cases where TAPS spontaneously resolved in utero where the donor twin is born with hearing problems.

deaf taps twins twin anemia polycythemia sequence donor auditory neuropathy spectrum disorder ansd

The incidence of ANSD in TAPS donors is higher than in prematurity (15% vs. 1-3%). We also know that post-laser TAPS donors seem to be unaffected by this complication. This hasn’t been recorded in either donor twins affected by TTTS or in babies affected by fetal anemia.

This is why it’s so important that we screen for TAPS and raise awareness of the long-term effects after birth. It’s not a mild disease.

What is ANSD?

ANSD is a specific type of hearing loss where there is a problem communicating sound from the inner ear to the brain.

deaf taps twins twin anemia polycythemia sequence donor auditory neuropathy spectrum disorder ansd

Sound enters the ears normally, but the sound isn’t correctly transmitted to the brain because of damage to the auditory nerve and/or cochlea. It’s like mixed or jumbled signals, meaning the brain can’t correctly interpret the sounds. ANSD is a spectrum, where sometimes it is mild hearing loss and other times quite severe.

Even if your child passes regular routine hearing tests, they must be tested for ANSD, as it can be missed in regular newborn screening.

Read more about ANSD here.

 Hearing Tests

There are 2 types of hearing tests that can be done at birth. 

EOAE – Evoked Otoacoustic Emissions.

 This is a test that uses a tiny plug in the baby’s ear while they sleep.  It measures the ear’s reaction to sounds and takes only a few minutes.  This is the most common test.

ABR – Auditory Brainstem Response. 

deaf taps twins twin anemia polycythemia sequence donor auditory neuropathy spectrum disorder ansd

This uses electrodes attached to the baby’s scalp. When the baby is sleeping, clicking sounds are made through tiny earphones on their ears.  It measures the brain’s responses to sounds.

Remember how we said above that ANSD is a complication where the brain doesn’t or can’t interpret sounds correctly? Your TAPS donor twin must be tested for deafness with ABR hearing testing at birth. Routine EOAE tests are less reliable in detecting ANSD.

Meet Piper

Meet the amazing dynamic duo of Piper and Jemma.  These amazing little girls were born at 32 weeks, with Piper in fetal distress.  At birth, her hemoglobin levels were so low, it was a miracle she survived. Piper and Jemma were diagnosed with spontaneous Twin Anemia Polycythemia Sequence after birth.

Piper is just one of many spontaneous TAPS donors who are born deaf from ANSD.  She was fitted at age 15 months with her first cochlear implant, and at age 2 with her second. This made a massive impact on her life.   Watch the amazing moment her first implant was activated in the video below.

Thank you to the Thornton family for sharing this video and Piper’s story with us.

Her family has shared this video to, show the importance of hearing testing and the experience they had when Piper heard for the first time. The Thornton family are amazing advocates for ABR hearing testing for spontaneous TAPS twins, because of the impact the diagnosis has had on their lives, and most importantly, the quality of Piper’s life

Testing Older TAPS Donor Twins

Not all TAPS twins are tested with ABR hearing testing at birth, and so we know that some deafness goes missed.

If your child has speech delays or is late to talk, or even is unresponsive to sounds, it’s a good idea to get a referral to an audiologist. Talk with them about the risks associated with spontaneous TAPS.

You can download the long-term effects of TAPS paper by clicking the button below, to help you make sure you get the right tests. Ask for a referral to an audiologist, and you can always contact us here to help you get everything you need. We’re here to support you through all stages of your TAPS diagnosis.

deaf taps twins twin anemia polycythemia sequence donor auditory neuropathy spectrum disorder ansd

Need More Information?

Deafness in TAPS donors is something that can be scary, but we’re here to connect you to the right information and research. Get in touch with us, or join our Facebook group to connect with other families affected by ANSD and TAPS.

References

Hille, E.T., Van Straaten, H.(., Verkerk, P.H. and (2007), Prevalence and independent risk factors for hearing loss in NICU infants. Acta Pædiatrica, 96: 1155-1158. https://doi.org/10.1111/j.1651-2227.2007.00398.x

Tollenaar, L.S.A., Lopriore, E., Slaghekke, F., Oepkes, D., Middeldorp, J.M., Haak, M.C., Klumper, F.J.C.M., Tan, R.N.G.B., Rijken, M. and Van Klink, J.M.M. (2020), High risk of long‐term neurodevelopmental impairment in donor twins with spontaneous twin anemia–polycythemia sequence. Ultrasound Obstet Gynecol, 55: 39-46. https://doi.org/10.1002/uog.20846

Arnold Starr, Terence W. Picton, Yvonnc Sininger, Linda J. Hood, Charles I. Berlin, Auditory neuropathy, Brain, Volume 119, Issue 3, June 1996, Pages 741–753, https://doi.org/10.1093/brain/119.3.741

Lindenburg, I.T.M., van Klink, J.M., Smits‐Wintjens, V.E.H.J., van Kamp, I.L., Oepkes, D. and Lopriore, E. (2013), Long‐term neurodevelopmental and cardiovascular outcome after intrauterine transfusions for fetal anaemia: a review. Prenat Diagn, 33: 815-822. https://doi.org/10.1002/pd.4152

van Klink JMM, Slaghekke F, Balestriero MA, et al. Neurodevelopmental outcome at 2 years in twin-twin transfusion syndrome survivors randomized for the Solomon trial. Am J Obstet Gynecol 2016;214:113.e1-7. https://doi.org/10.1016/j.ajog.2015.08.033

Tollenaar, Lisanne S.A.1,∗; Lopriore, Enrico2; Oepkes, Dick1; Haak, Monique C.1; Klumper, Frans J.C.M.1; Middeldorp, Johanna M.1; Klink, Jeanine M.M. Van2; Slaghekke, Femke1 Twin Anemia Polycythemia Sequence: Knowledge and Insights After 15 Years of Research, Maternal-Fetal Medicine: January 2021 – Volume 3 – Issue 1 – p 33-41 doi: 10.1097/FM9.0000000000000065