The International Standard Set of Outcome Measures for the Assessment of Hearing in People with Osteogenesis Imperfecta.

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.

Assessment of speech recognition abilities in quiet and in noise: a comparison between self-administered home testing and testing in the clinic for adult cochlear implant users.

Self speech recognition tests in quiet and noise at home are compared to the standard tests performed in the clinic. Potential effects of stimuli presentation modes (loudspeaker or audio cable) and assessment (clinician or self-assessment at home) on test results were investigated. Speech recognition in quiet was assessed using the standard Dutch test with monosyllabic words. Speech recognition in noise was assessed with the digits-in-noise test. Sixteen experienced CI users (aged between 44 and 83 years) participated. No significant difference was observed in speech recognition in quiet between and presentation modes. Speech recognition in noise was significantly better with the audio cable than with the loudspeaker. There was no significant difference in speech recognition in quiet at 65 dB and in speech recognition in noise between self-assessment at home and testing in the clinic. At 55 dB, speech recognition assessed at home was slightly but significantly better than that assessed in the clinic. The results demonstrate that it is feasible for experienced CI users to perform self-administered speech recognition tests at home. Self-assessment by CI users of speech recognition in quiet and noise within the home environment could serve as an alternative to the tests performed in the clinic.

Improving the Efficiency of Speech-In-Noise Hearing Screening Tests.

OBJECTIVE: Speech-in-noise hearing screening tests have become increasingly popular. These tests follow an adaptive procedure with a fixed number of presentations to estimate the speech reception threshold. The speech reception threshold is compared with an established cutoff signal to noise ratio (SNR) for a pass result or refer result. A fixed SNR procedure was developed to improve the efficiency of speech-in-noise hearing screening tests. DESIGN: The cutoff SNR is used for all presentations in the fixed-SNR procedure. After each response a reliable test result is given (pass/refer) or an extra stimulus is presented. The efficiency and pass/refer rates between the adaptive procedure and the fixed-SNR procedure were compared. RESULTS: An average reduction of 67% in the number of presentations can be achieved (from 25 to an average of 8.3 presentations per test). CONCLUSIONS: The fixed-SNR procedure is superior in efficiency to the adaptive procedure while having nearly equal refer and pass rates.

Assessment of hearing in very young children receiving carboplatin for retinoblastoma.

Children with retinoblastoma have increasingly been treated with carboplatin in the past decade. Ototoxicity is a known, possible, side-effect of carboplatin. Since retinoblastoma patients are very young and frequently have impaired vision, the evaluation of hearing loss is very important. The hearing status of 25 children with retinoblastoma treated with carboplatin (median cumulative dose 2,240 mg/m(2)) was evaluated in detail. Median age at first carboplatin administration was 7 months. The evaluation of hearing loss was performed by an age-appropriate measurement protocol consisting of tympanometry, otoacoustic emission measurements, auditory brainstem responses and (high-frequency) visual reinforcement audiometry (VRA) or play-audiometry. The median follow-up time after last carboplatin dose was 25 months (range 1-94 months). In none of the children was hearing loss detected after carboplatin administration. A measurement protocol that includes tympanometry, distortion product otoacoustic emission measurements and high-frequency VRA is recommended for young children receiving carboplatin or other ototoxic drugs.