Objective: The aim is to recommend a minimum standard set of clinician-reported outcome measures (CROMs) and patient-reported outcome measures (PROMs) on hearing for people with osteogenesis imperfecta (OI). This project is part of the larger “Key4OI” project initiated by the “Care4BrittleBones foundation” of which the goal is to improve quality of life of people with OI. Key4OI provides a standard set of outcome measures and covers a large set of domains affecting the well-being of people with OI. Methods: An international team of experts in OI, comprising specialists in audiological science, medical specialists, and an expert patient representative, used a modified Delphi consensus process to select CROMs and PROMs to evaluate hearing problems in people with OI. In addition, focus groups of people with OI identified key consequences of their hearing loss. These criteria were matched to categories of preselected questionnaires to select a PROM that matched their specific hearing-related concerns best. Results: Consensus on PROMs for adults and CROMs for adults and children was reached. The focus of the CROMs was on specific audiological outcome measures and standardized follow-up. Conclusions: This project resulted in a clear consensus statement for standardization of hearing-related PROMs and CROMs and follow-up management of patients with OI. This standardization of outcome measurements will facilitate comparability of research and easier international cooperation in OI and hearing loss. Furthermore, it can improve standard of care in people with OI and hearing loss by incorporating the recommendations into care pathways.

Studies about the effects of SARS-CoV-2 on pregnant women and children born to positive women are controversial with regard to possible inner ear-related damage but most of them do not detect the involvement of this virus in auditory function. However, only a few studies on long-term effects on language development are currently available because of the recent onset of the pandemic. The aim of this study was to investigate the impact of SARS-CoV-2 infection on perceptual and expressive abilities and the emerging development of communication in young children. To this purpose, the MacArthur–Bates Communicative Development Inventory—Words and Gestures form (CDI-WG), was administered to parents. In total, 115 children whose mother was infected by SARS-CoV-2 during pregnancy were enrolled in the study and evaluated at the Audiology Service of the Modena University Hospital. All children underwent Otoacoustic Emissions (OAE) at birth: 114/115 had a “pass” result bilaterally, while 1 case had a unilateral “refer” result. Overall, 110/115 newborns (95.65%) underwent audiological evaluation between 10–18 months of age. In 5/110 patients (3.6%), the Pure Tone Average (PTA) result was equal to 35 dB; one case had a hearing threshold of around 50 dB due to a bilateral effusive otitis media. A notable finding was the percentage of children with tubal dysfunction in both evaluations, within 2 months of age and around 12 months of age. Most children revealed normal hearing. The CDI-WG was completed by 56/115 families. The rate of children below the fifth percentile was 8.9% for sentences understood, 12.5% for words understood, and 5.4% for words produced. Concerning CDI-Gestures, only 2 children (3.6%) were below the fifth percentile. A structured audiological follow-up in association with the evaluation of communication skills of children appears fundamental, particularly in the years of maximum neuroplasticity. Long-term studies are still necessary to evaluate the possible consequences of the pandemic.