Adult normative data for the speech in babble (SiB) test.

Objective: The Speech in Babble (SiB) test assesses the perception of speech in noise in UK adults. Here, we define the normal range of SiB scores to enable the use of the test in clinic.Design: In each test, 25 monosyllabic words were played in background multi-talker babble. Listeners had to repeat the word they heard. An adaptive procedure was used to determine the signal-to-noise ratio needed to reach 50% correct responses (i.e. the Speech Reception Threshold). Eight distinct equivalent lists were available.Study sample: Sixty-nine normal-hearing adults (aged 20-57 years) with no reported listening difficulties participated in the study and completed the SiB test twice in both ears.Results: Normative SiB scores varied from -0.8 dB to 3.7 dB suggesting that patients outside these limits should be considered as having abnormal scores. No statistically significant difference between ears and no effect of age or sex was found. There was "fair" test-retest reliability.Conclusion: The SiB test is a short, valid and reliable test that can be used in UK clinics, e.g. as part of a standard APD battery or evaluating the performance of hearing impaired patients.

Preparing for Otoferlin gene therapy trials: A survey of NHS Paediatric Audiology and Cochlear Implant services on diagnosis and management of Auditory Neuropathy Spectrum Disorder.

OBJECTIVES: Gene therapy for monogenic hearing loss is on the horizon. The first trials in patients with Auditory Neuropathy Spectrum Disorder (ANSD) due to pathogenic variants in the Otoferlin (OTOF) gene will open this year. In the UK, the new NHS Genomic Medicine Service (GMS) offers genetic testing in each child diagnosed with congenital or early onset sensorineural hearing loss. This survey study aims to map preexisting clinical pathways for the diagnosis and management of children with ANSD and identify opportunities for improvement in early identification of OTOF- related ANSD. METHODS: A Google form with 24 questions in English covering the ANSD clinical pathway was developed with clinicians involved in the diagnosis and management ANSD. The survey was disseminated via email to all Lead clinicians of NHS Tertiary Paediatric Audiology and Cochlear Implant Services within the UK. RESULTS: Data was received from 27 (34 %) NHS Tertiary Paediatric Audiology Services and 8 (n = 57 %) Paediatric Cochlear Implant Services. Services follow existing national guidance and provide multidisciplinary care with structured patient pathways for referral, diagnosis, and management of children with ANSD and multidisciplinary input throughout. Clinicians are aware of the genetic causes of ANSD and new processes for genetic testing, but do not uniformly refer children with ANSD for testing for OTOF pathogenic variants. As such, they had difficulty estimating numbers of children with OTOF pathogenic variants under their care. CONCLUSION: Those results highlight the urgency of implementing hearing gene panel sequencing for all children with ANSD to provide opportunities for early diagnosis and candidacy for OTOF gene therapy trials.

Improving transition outcomes in adolescents with permanent hearing loss.

Young people with permanent hearing loss need to transition into adult services and engagement with the transition process is required to achieve this. Although there are clear national recommendations about the transition process and transfer to the adult services limited evidence has been published about the implementation and efficacy of transition programmes in young adults with permanent hearing loss. The primary aim of this quality improvement project was to significantly increase the number of teenagers with a clear transition protocol documented in their notes. The secondary aims were to ensure good medical and audiological outcomes based on all completing their medical investigations prior to transfer to the adult services and also based on a survey regarding attitudes to hearing aids use. A multiphase intervention programme was developed; (1) staff update with national transition protocols, (2) development of a clear transition protocol, (3) allocated key worker (4) access and (5) engagement. Through these interventions we increased the number of teenagers who had a clear transition protocol documented in their notes from 11% to 91%. 84% of our teenagers had completed their medical investigations, although documented discussion about the investigations had been done with 100% of the cohort. 9.2 % of adolescents felt pressurised to use hearing aids compared to 35% prior to the interventions. This project resulted in clear, measurable conclusions and we have shown that we better understand the needs of our patients and the improvement is sustainable.

What Can We Learn about Auditory Processing from Adult Hearing Questionnaires?

BACKGROUND: Questionnaires addressing auditory disability may identify and quantify specific symptoms in adult patients with listening difficulties. PURPOSE: (1) To assess validity of the Speech, Spatial, and Qualities of Hearing Scale (SSQ), the (Modified) Amsterdam Inventory for Auditory Disability (mAIAD), and the Hyperacusis Questionnaire (HYP) in adult patients experiencing listening difficulties in the presence of a normal audiogram. (2) To examine which individual questionnaire items give the worse scores in clinical participants with an auditory processing disorder (APD). RESEARCH DESIGN: A prospective correlational analysis study. STUDY SAMPLE: Clinical participants (N = 58) referred for assessment because of listening difficulties in the presence of normal audiometric thresholds to audiology/ear, nose, and throat or audiovestibular medicine clinics. Normal control participants (N = 30). DATA COLLECTION AND ANALYSIS: The mAIAD, HYP, and the SSQ were administered to a clinical population of nonneurological adults who were referred for auditory processing (AP) assessment because of hearing complaints, in the presence of normal audiogram and cochlear function, and to a sample of age-matched normal-hearing controls, before the AP testing. Clinical participants with abnormal results in at least one ear and in at least two tests of AP (and at least one of these tests to be nonspeech) were classified as clinical APD (N = 39), and the remaining (16 of whom had a single test abnormality) as clinical non-APD (N = 19). RESULTS: The SSQ correlated strongly with the mAIAD and the HYP, and correlation was similar within the clinical group and the normal controls. All questionnaire total scores and subscores (except sound distinction of mAIAD) were significantly worse in the clinical APD versus the normal group, while questionnaire total scores and most subscores indicated greater listening difficulties for the clinical non-APD versus the normal subgroups. Overall, the clinical non-APD group tended to give better scores than the APD in all questionnaires administered. Correlation was strong for the worse-ear gaps-in-noise threshold with the SSQ, mAIAD, and HYP; strong to moderate for the speech in babble and left-ear dichotic digit test scores (at p < 0.01); and weak to moderate for the remaining AP tests except the frequency pattern test that did not correlate. The worse-scored items in all three questionnaires concerned speech-in-noise questions. This is similar to worse-scored items by hearing-impaired participants as reported in the literature. Worse-scored items of the clinical group also included quality aspects of listening questions from the SSQ, which most likely pertain to cognitive aspects of listening, such as ability to ignore other sounds and listening effort. CONCLUSIONS: Hearing questionnaires may help assess symptoms of adults with APD. The listening difficulties and needs of adults with APD to some extent overlap with those of hearing-impaired listeners, but there are significant differences. The correlation of the gaps-in-noise and duration pattern (but not frequency pattern) tests with the questionnaire scores indicates that temporal processing deficits may play an important role in clinical presentation.

Patient-reported speech in noise difficulties and hyperacusis symptoms and correlation with test results.

OBJECTIVES/HYPOTHESIS: To compare self-reported symptoms of difficulty hearing speech in noise and hyperacusis in adults with auditory processing disorders (APDs) and normal controls; and to compare self-reported symptoms to objective test results (speech in babble test, transient evoked otoacoustic emission [TEOAE] suppression test using contralateral noise). STUDY DESIGN: A prospective case-control pilot study. METHODS: Twenty-two participants were recruited in the study: 10 patients with reported hearing difficulty, normal audiometry, and a clinical diagnosis of APD; and 12 normal age-matched controls with no reported hearing difficulty. All participants completed the validated Amsterdam Inventory for Auditory Disability questionnaire, a hyperacusis questionnaire, a speech in babble test, and a TEOAE suppression test using contralateral noise. RESULTS: Patients had significantly worse scores than controls in all domains of the Amsterdam Inventory questionnaire (with the exception of sound detection) and the hyperacusis questionnaire (P < .005). Patients also had worse TEOAE suppression test results in both ears than controls; however, this result was not significant after Bonferroni correction. Strong correlations were observed between self-reported symptoms of difficulty hearing speech in noise and speech in babble test results in the right ear (ρ = 0.624, P = .002), and between self-reported symptoms of hyperacusis and TEOAE suppression test results in the right ear (ρ = -0.597 P = .003). CONCLUSIONS: There was no significant correlation between the two tests. A strong correlation was observed between right ear speech in babble and patient-reported intelligibility of speech in noise, and right ear TEOAE suppression by contralateral noise and hyperacusis questionnaire.

The effect of repeated visual motion stimuli on visual dependence and postural control in normal subjects.

Patients with vestibular dysfunction, migraine and/or anxiety may experience visual vertigo (VV), whereby symptoms are provoked by disorienting visual environments (e.g. supermarkets). Patients with VV over rely on vision for balance (i.e. visually dependent). Visual vertigo significantly improves when vestibular rehabilitation incorporates exposure to optokinetic stimulation (OKS). However, whether OKS exposure induces a reduction in visual dependency is unknown. This study investigated this issue by measuring visual dependency before and after repeated OKS exposure. Twenty-six healthy subjects (10 males; mean age 29.8 years, range 20-42 years) were randomly allocated into an OKS group who underwent graded OKS exposure for five consecutive days, or a no intervention control group. Assessment included the 'Rod and Frame' and 'Rod and Disc' tests where subjects set the subjective visual vertical in darkness, facing a tilted luminous frame or luminous rotating disc, respectively. Postural sway measures were obtained with eyes open, closed and facing the rotating disc. Results showed significant reductions in subjective vertical tilt with the frame and rotating disc for the OKS group only (p≤0.01). Total sway path and mean deviation induced by the rotating stimulus decreased significantly only for the OKS group (p<0.01), as did the Kinetic Quotient (disc rotation/eyes open sway path ratio; p=0.04). The Romberg Quotient (eyes closed/eyes open ratio) showed no change. Findings suggest visual dependency, both at a perceptual and a postural level, can be reduced with short-term graded OKS exposure in healthy subjects. This has important implications for treatment of patients with VV and balance disorders.