Title- Outcome of cochlear implantation in two children with a genetic diagnosis of Aymé-Gripp Syndrome.
Objectives- Aymé-Gripp  syndrome is a clinically homogeneous phenotype characterized by congenital cataracts, sensorineural hearing loss, intellectual disability, seizures, brachycephaly, a distinctive flat facial appearance, and reduced growth (Niceta et al, 2015). According to Amudhavalli & Gadea (2020), only 21 individuals representing 19 families have been reported in the literature. 2 patients with a diagnosis of Aymé-Gripp  Syndrome have received cochlear implants (CI) at NHIRC. This study reviewed the management process and outcome of these two children.
Methods:  Retrospective review of medical charts, and programming files of two children with Aymé-Gripp  Syndrome with CI.
Results:
Child 1 is a female who received a right-sided CI in 2008 at age 23 months. She had presented with bilateral profound sensorineural hearing loss, congenital cataracts, ventricular dilatation with white matter changes on MRI and developmental delay. She had an initial diagnosis of congenital CMV. She received the diagnosis of Autism at age 6 and the diagnosis of Ayme Gripp syndrome at age 11. Initially, she appeared to tolerate her CI (no datalogging available) and reportedly showed inconsistent responses to sounds such as to musical toys. Unfortunately, she needed revision surgery at age 3 due to device failure. Three years after revision surgery, she still had no consistent response to environmental sounds. Her device usage started decreasing over time. The child eventually became a non-user.
Child 2 is a male who received bilateral CI in 2018 at age 22 months. He had presented with bilateral profound sensorineural hearing loss along with an initial diagnosis of Pierre Robin sequence, micrognathia, cleft palate, cataracts and respiratory failure. He received the diagnosis of Ayme Gripp syndrome at age 20 months. From the beginning, tolerance of the external devices was challenging. Removing the right CI improved daily usage of the left CI but usage is still under 6 hours. Although parents report enjoyment of music with CI, benefit is difficult to gauge clinically. The child is on regular review.
Conclusion:
The two children in our study with a diagnosis of Aymé-Gripp  syndrome have had limited or no benefit from CI.

**Title**- Outcome of cochlear implantation in two children with a genetic diagnosis of Aymé-Gripp Syndrome.
**Objectives-** Aymé-Gripp  syndrome is a clinically homogeneous phenotype characterized by congenital cataracts, sensorineural hearing loss, intellectual disability, seizures, brachycephaly, a distinctive flat facial appearance, and reduced growth (Niceta et al, 2015). According to Amudhavalli & Gadea (2020), only 21 individuals representing 19 families have been reported in the literature. 2 patients with a diagnosis of Aymé-Gripp  Syndrome have received cochlear implants (CI) at NHIRC. This study reviewed the management process and outcome of these two children.
**Methods:**  Retrospective review of medical charts, and programming files of two children with Aymé-Gripp  Syndrome with CI.
**Results:**
Child 1 is a female who received a right-sided CI in 2008 at age 23 months. She had presented with bilateral profound sensorineural hearing loss, congenital cataracts, ventricular dilatation with white matter changes on MRI and developmental delay. She had an initial diagnosis of congenital CMV. She received the diagnosis of Autism at age 6 and the diagnosis of Ayme Gripp syndrome at age 11. Initially, she appeared to tolerate her CI (no datalogging available) and reportedly showed inconsistent responses to sounds such as to musical toys. Unfortunately, she needed revision surgery at age 3 due to device failure. Three years after revision surgery, she still had no consistent response to environmental sounds. Her device usage started decreasing over time. The child eventually became a non-user.
Child 2 is a male who received bilateral CI in 2018 at age 22 months. He had presented with bilateral profound sensorineural hearing loss along with an initial diagnosis of Pierre Robin sequence, micrognathia, cleft palate, cataracts and respiratory failure. He received the diagnosis of Ayme Gripp syndrome at age 20 months. From the beginning, tolerance of the external devices was challenging. Removing the right CI improved daily usage of the left CI but usage is still under 6 hours. Although parents report enjoyment of music with CI, benefit is difficult to gauge clinically. The child is on regular review.
**Conclusion:**
The two children in our study with a diagnosis of Aymé-Gripp  syndrome have had limited or no benefit from CI.

Outcome of cochlear implantation in two children with a genetic diagnosis of Ayme-Gripp Syndrome.

It is with great pleasure and enthusiasm, that on behalf of the Cochlear Implant centers of the Radboud UMC in Nijmegen and Erasmus MC Sophia Children’s Hospital in Rotterdam to invite you to attend the 16th European Symposium on Pediatric Cochlear Implantation to Rotterdam, the Netherlands in 2023.

Yours sincerely, on behalf of the CI Centers at Radboud UMC and Erasmus MC,