The Effects of Delayed Hearing Rehabilitation in a Patient with Myhre's Syndrome during COVID-19 Pandemic

Author(s): Carla Laria, Antonio Casarella, Angelica Rodio, Raffaele Nubi, Raffaele Izzo

Hearing loss (HL) is observed among pathologies in patients affected by Myhre syndrome and it is predominantly conductive and sometime sensorineural or mixed. Hearing loss if not treated properly can have impacts on cognitive behavioural and communicative language. We describe the benefits of hearing rehabilitation by using hearing aids in a patient with conductive HL, that carries a mutation (c.1498A>G; p.lle500Val) within the SMAD4 gene as detected by whole exome sequencing [1]. Despite the relevant delay of hearing aid and language rehabilitation in this baby girl because of the COVID-19 pandemic restrictions, we demonstrate the positive impact of the hearing rehabilitation on listening skills, on cognitive abilities and working memory in syndromic HL which is characterized by behavioural disorders and mild to moderate intellectual disability, thus improving quality of life.