MRC Institute of Hearing Research

The Speech, Spatial and Qualities of Hearing Scale (SSQ) is designed to measure a range of hearing disabilities across several domains. Particular attention is given to hearing speech in a variety of competing contexts, and to the directional, distance and movement components of spatial hearing. In addition, the abilities both to segregate sounds and to attend to simultaneous speech streams are assessed, reflecting the reality of hearing in the everyday world. Qualities of hearing experience include ease of listening, and the naturalness, clarity and identifiability of different speakers, different musical pieces and instruments, and different everyday sounds. Application of the SSQ to 153 new clinic clients prior to hearing aid fitting showed that the greatest difficulty was experienced with simultaneous speech streams, ease of listening, listening in groups and in noise, and judging distance and movement. SSQ ratings were compared with an independent measure of handicap. After differences in hearing level were controlled for, it was found that identification, attention and effort problems, as well as spatial hearing problems, feature prominently in the disability handicap relationship,. along with certain features of speech hearing. The results implicate aspects of temporal and spatial dynamics of hearing disability in the experience of handicap. The SSQ shows promise as an instrument for evaluating interventions of various kinds, particularly (but not exclusively) those that implicate binaural function.

The use of functional magnetic resonance imaging (fMRI) to explore central auditory function may be compromised by the intense bursts of stray acoustic noise produced by the scanner whenever the magnetic resonance signal is read out. We present results evaluating the use of one method to reduce the effect of the scanner noise: "sparse" temporal sampling. Using this technique, single volumes of brain images are acquired at the end of stimulus and baseline conditions. To optimize detection of the activation, images are taken near to the maxima and minima of the hemodynamic response during the experimental cycle. Thus, the effective auditory stimulus for the activation is not masked by the scanner noise. In experiment 1, the course of the hemodynamic response to auditory stimulation was mapped during continuous task performance. The mean peak of the response was at 10.5 sec after stimulus onset, with little further change until stimulus offset. In experiment 2, sparse imaging was used to acquire activation images. Despite the fewer samples with sparse imaging, this method successfully delimited broadly the same regions of activation as conventional continuous imaging. However, the mean percentage MR signal change within the region of interest was greater using sparse imaging. Auditory experiments that use continuous imaging methods may measure activation that is a result of an interaction between the stimulus and task factors (e.g., attentive effort) induced by the intense background noise. We suggest that sparse imaging is advantageous in auditory experiments as it ensures that the obtained activation depends on the stimulus alone.

The Glasgow Benefit Inventory (GBI) is a measure of patient benefit developed especially for otorhinolaryngological (ORL) interventions. Patient benefit is the change in health status resulting from health care intervention. The GBI was developed to be patient-oriented, to be maximally sensitive to ORL interventions, and to provide a common metric to compare benefit across different interventions. The GBI is an 18-item, postintervention questionnaire intended to be given to patients to fill in at home or in the outpatient clinic. In the first part of the paper, five different ORL interventions were retrospectively studied: middle ear surgery to improve hearing, provision of a cochlear implant, middle ear surgery to eradicate ear activity, rhinoplasty, and tonsillectomy. A criterion that was specific to the intervention was selected for each study, so that the patient outcome could be classified as above and below criterion. In all five interventions, the GBI was found to discriminate between above- and below-criterion outcomes. The second part of the paper reports on the results and implications of a factor analysis of patient responses. The factor structure was robust across the study, and so led to the construction of subscales. These subscales yield a profile score that provides information on the different types of patient benefit resulting from ORL interventions. The GBI is sensitive to the different ORL interventions, yet is sufficiently general to enable comparison between each pair of interventions. It provides a profile score, which enables further breakdown of results. As it provides a patient-oriented common metric, it is anticipated that the GBI will assist audit, research, and health policy planning.

This paper summarizes twenty studies, published since 1989, that have measured experimentally the relationship between speech recognition in noise and some aspect of cognition, using statistical techniques such as correlation or factor analysis. The results demonstrate that there is a link, but it is secondary to the predictive effects of hearing loss, and it is somewhat mixed across study. No one cognitive test always gave a significant result, but measures of working memory (especially reading span) were mostly effective, whereas measures of general ability, such as IQ, were mostly ineffective. Some of the studies included aided listening, and two reported the benefits from aided listening: again mixed results were found, and in some circumstances cognition was a useful predictor of hearing-aid benefit.

Davis A C (MRC Institute of Hearing Research, University of Nottingham, University Park, Nottingham NG7 2RD, UK). The prevalence of hearing impairment and reported hearing disability in adults in Great Britain. International Journal of Epidemiology 1989, 18: 911–917. Estimates for the prevalence of self-reported hearing disability and measured hearing impairment as a function of age in the adult population of Great Britain (GB) are reported from two 2-stage surveys. The main study was conducted in Cardiff, Glasgow, Nottingham and Southampton, with rigorous audiological assessment at the second stage. A supplementary study used a sample representative of GB with simplified domiciliary audiological assessments. In the main study, neither stage showed any gross bias arising from the particular cities chosen; the estimates from the first stage are free of bias arising from non-response. The estimates from the second stage are relatively free of bias arising from non-attendance. For the present purposes, defining a ‘significant’ level of hearing impairment as at least 25 dBHL averaged over the frequencies 0.5, 1, 2, 4 kHz, 16% of the adult population (17–80 years) have a bilateral, and about one in four a unilateral or bilateral, hearing impairment. About 10% of the adult population (aged 17+) report bilateral hearing difficulty in a quiet environment.

Hearing loss with increasing age adversely affects the ability to understand speech, an effect that results partly from reduced audibility. The aims of this study were to establish whether aging reduces speech intelligibility for listeners with normal audiograms, and, if so, to assess the relative contributions of auditory temporal and cognitive processing. Twenty-one older normal-hearing (ONH; 60-79 years) participants with bilateral audiometric thresholds ≤ 20 dB HL at 0.125-6 kHz were matched to nine young (YNH; 18-27 years) participants in terms of mean audiograms, years of education, and performance IQ. Measures included: (1) identification of consonants in quiet and in noise that was unmodulated or modulated at 5 or 80 Hz; (2) identification of sentences in quiet and in co-located or spatially separated two-talker babble; (3) detection of modulation of the temporal envelope (TE) at frequencies 5-180 Hz; (4) monaural and binaural sensitivity to temporal fine structure (TFS); (5) various cognitive tests. Speech identification was worse for ONH than YNH participants in all types of background. This deficit was not reflected in self-ratings of hearing ability. Modulation masking release (the improvement in speech identification obtained by amplitude modulating a noise background) and spatial masking release (the benefit obtained from spatially separating masker and target speech) were not affected by age. Sensitivity to TE and TFS was lower for ONH than YNH participants, and was correlated positively with speech-in-noise (SiN) identification. Many cognitive abilities were lower for ONH than YNH participants, and generally were correlated positively with SiN identification scores. The best predictors of the intelligibility of SiN were composite measures of cognition and TFS sensitivity. These results suggest that declines in speech perception in older persons are partly caused by cognitive and perceptual changes separate from age-related changes in audiometric sensitivity.

OBJECTIVE: There is growing interest in the concepts of listening effort and fatigue associated with hearing loss. However, the theoretical underpinnings and clinical meaning of these concepts are unclear. This lack of clarity reflects both the relative immaturity of the field and the fact that research studies investigating listening effort and fatigue have used a variety of methodologies including self-report, behavioural, and physiological measures. DESIGN: This discussion paper provides working definitions for listening effort and listening-related fatigue. Using these definitions as a framework, methodologies to assess these constructs are reviewed. RESULTS: Although each technique attempts to characterize the same construct (i.e. the clinical presentation of listening effort and fatigue), different assumptions are often made about the nature of these phenomena and their behavioural and physiological manifestations. CONCLUSION: We suggest that researchers consider these assumptions when interpreting their data and, where possible, make predictions based on current theoretical knowledge to add to our understanding of the underlying mechanisms of listening effort and listening-related fatigue. FOREWORD: Following recent interest in the cognitive involvement in hearing, the British Society of Audiology (BSA) established a Special Interest Group on Cognition in Hearing in May 2013. In an exploratory group meeting, the ambiguity surrounding listening effort and fatigue was discussed. To address this problem, the group decided to develop a 'white paper' on listening effort and fatigue. This is a discussion document followed by an international set of commentaries from leading researchers in the field. An approach was made to the editor of the International Journal of Audiology who agreed to this suggestion. This paper, and the associated commentaries that follow, are the result.

Hearing loss is associated with poor cognitive performance and incident dementia and may contribute to cognitive decline. Treating hearing loss with hearing aids may ameliorate cognitive decline. The purpose of this study was to test whether use of hearing aids was associated with better cognitive performance, and if this relationship was mediated via social isolation and/or depression. Structural equation modelling of associations between hearing loss, cognitive performance, social isolation, depression and hearing aid use was carried out with a subsample of the UK Biobank data set (n = 164,770) of UK adults aged 40 to 69 years who completed a hearing test. Age, sex, general health and socioeconomic status were controlled for as potential confounders. Hearing aid use was associated with better cognition, independently of social isolation and depression. This finding was consistent with the hypothesis that hearing aids may improve cognitive performance, although if hearing aids do have a positive effect on cognition it is not likely to be via reduction of the adverse effects of hearing loss on social isolation or depression. We suggest that any positive effects of hearing aid use on cognition may be via improvement in audibility or associated increases in self-efficacy. Alternatively, positive associations between hearing aid use and cognition may be accounted for by more cognitively able people seeking and using hearing aids. Further research is required to determine the direction of association, if there is any direct causal relationship between hearing aid use and better cognition, and whether hearing aid use results in reduction in rates of cognitive decline measured longitudinally.

Abstract Objective: To estimate the prevalence of confirmed permanent childhood hearing impairment and its profile across age and degree of impairment in the United Kingdom. Design: Retrospective total ascertainment through sources in the health and education sectors by postal questionnaire. Setting: Hospital based otology and audiology departments, community health clinics, education services for hearing impaired children. Participants: Children born from 1980 to 1995, resident in United Kingdom in 1998, with permanent childhood hearing impairment (hearing level in the better ear >40 dB averaged over 0.5, 1, 2, and 4 kHz). Main outcome measures: Numbers of cases with date of birth and severity of impairment converted to prevalences for each annual birth cohort (cases/1000 live births) and adjusted for underascertainment. Results: 26 000 notifications ascertained 17 160 individual children. Prevalence rose from 0.91 (95% confidence interval 0.85 to 0.98) for 3 year olds to 1.65 (1.62 to 1.68) for children aged 9-16 years. Adjustment for underascertainment increased estimates to 1.07 (1.03 to 1.12) and 2.05 (2.02 to 2.08). Comparison with previous studies showed that prevalence increases with age, rather than declining with year of birth. Conclusions: Prevalence of confirmed permanent childhood hearing impairment increases until the age of 9 years to a level higher than previously estimated. Relative to current yields of universal neonatal hearing screening in the United Kingdom, which are close to 1/1000 live births, 50-90% more children are diagnosed with permanent childhood hearing impairment by the age of 9 years. Paediatric audiology services must have the capacity to achieve early identification and confirmation of these additional cases. What is already known on this topic The prevalence of confirmed permanent childhood hearing impairment (>40 dB HL) in the United Kingdom has been estimated to rise with age to 1.33/1000 live births among children aged 5 years and older It has been predicted that only an additional 16% of children will remain to be detected in the postnatal years, given current yields from universal neonatal hearing screening What this study adds The prevalence of confirmed permanent childhood hearing impairment (>40 dB HL) in the United Kingdom has risen with age to at least 1.65/1000 live births (and may be as high as 2.05/1000 live births) among children 9 years of age and older If the current yield from screening is sustained, then an additional 50-90% of children will remain to be detected in the postnatal years

The intelligibility of sentences presented in noise improves when the listener can view the talker's face. Our aims were to quantify this benefit, and to relate it to individual differences among subjects in lipreading ability and among sentences in lipreading difficulty. Auditory and audiovisual speech-reception thresholds (SRTs) were measured in 20 listeners with normal hearing. Sixty sentences, selected to range in the difficulty with which they could be lipread (with vision alone) from easy to hard, were presented for identification in white noise. Using the ascending method of limits, the SRT was defined as the lowest signal-to-noise ratio at which all three 'key words' in each sentence could be identified correctly. Measured as the difference in dB between auditory-alone and audiovisual SRTs, 'audiovisual benefit' averaged 11 dB, ranging from 6 to 15 dB among subjects, and from 3 to 22 dB among sentences. As predicted, audiovisual benefit is a measure of lipreading ability. It was highly correlated with visual-alone performance (n = 20, r = 0.86, P less than 0.01). Likewise, those sentences which were easiest to lipread gave a higher measure of benefit from vision in audiovisual conditions than did sentences that were hard to lipread (n = 60, r = 0.92, P less than 0.01). The results establish the basis of an efficient test of speech-reception disability in which measures are freed from the floor and ceiling effects encountered when percentage correct is used as the dependent variable.

BACKGROUND: Otitis media with effusion (OME; 'glue ear') is common in childhood and surgical treatment with grommets (ventilation tubes) is widespread but controversial. OBJECTIVES: To assess the effectiveness of grommet insertion compared with myringotomy or non-surgical treatment in children with OME. SEARCH STRATEGY: We searched the Cochrane ENT Disorders Group Trials Register, other electronic databases and additional sources for published and unpublished trials (most recent search: 22 March 2010). SELECTION CRITERIA: Randomised controlled trials evaluating the effect of grommets. Outcomes studied included hearing level, duration of middle ear effusion, language and speech development, cognitive development, behaviour and adverse effects. DATA COLLECTION AND ANALYSIS: Data from studies were extracted by two authors and checked by the other authors. MAIN RESULTS: We included 10 trials (1728 participants). Some trials randomised children (grommets versus no grommets), others ears (grommet one ear only). The severity of OME in children varied between trials. Only one 'by child' study (MRC: TARGET) had particularly stringent audiometric entry criteria. No trial was identified that used long-term grommets.Grommets were mainly beneficial in the first six months by which time natural resolution lead to improved hearing in the non-surgically treated children also. Only one high quality trial that randomised children (N = 211) reported results at three months; the mean hearing level was 12 dB better (95% CI 10 to 14 dB) in those treated with grommets as compared to the controls. Meta-analyses of three high quality trials (N = 523) showed a benefit of 4 dB (95% CI 2 to 6 dB) at six to nine months. At 12 and 18 months follow up no differences in mean hearing levels were found.Data from three trials that randomised ears (N = 230 ears) showed similar effects to the trials that randomised children. At four to six months mean hearing level was 10 dB better in the grommet ear (95% CI 5 to 16 dB), and at 7 to 12 months and 18 to 24 months was 6 dB (95% CI 2 to 10 dB) and 5 dB (95% CI 3 to 8 dB) dB better.No effect was found on language or speech development or for behaviour, cognitive or quality of life outcomes.Tympanosclerosis was seen in about a third of ears that received grommets. Otorrhoea was common in infants, but in older children (three to seven years) occurred in < 2% of grommet ears over two years of follow up. AUTHORS' CONCLUSIONS: In children with OME the effect of grommets on hearing, as measured by standard tests, appears small and diminishes after six to nine months by which time natural resolution also leads to improved hearing in the non-surgically treated children. No effect was found on other child outcomes but data on these were sparse. No study has been performed in children with established speech, language, learning or developmental problems so no conclusions can be made regarding treatment of such children.

Tinnitus is a common experience with up to one third of the adult population experiencing it at some time in their life. Less than 1% of the adult population have tinnitus of sufficient severity to affect their quality of life seriously (although up to 8% may seek medical advice about it). Much of the severity of tinnitus relates to the individuals' psychological response to the abnormal tinnitus signal. The prevalence of tinnitus increases in association with high frequency hearing loss. There is, unfortunately, no diagnostic test that either confirms the presence of tinnitus or its severity. Currently there is no satisfactory severity grading system. A five-point severity grading scheme is therefore proposed and the entry criteria detailed. The five severity points are: slight, mild, moderate, severe and catastrophic. Categorization as 'severe' or 'catastrophic' should be, by epidemiological definition, very rare. General guidance, theory and evidential support are contained within.

The stria vascularis of the mammalian cochlea is composed primarily of three types of cells. Marginal cells line the lumen of the cochlear duct and are of epithelial origin. Basal cells also form a continuous layer and they may be mesodermal or derived from the neural crest. Intermediate cells are melanocyte-like cells, presumably derived from the neural crest, and are scattered between the marginal and basal cell layers. The marginal cells form extensive interdigitations with the basal and intermediate cells in the normal adult stria. The stria also contains a rich supply of blood vessels. We investigated the role of melanocytes in the stria vascularis by studying its development in a mouse mutant, viable dominant spotting, which is known to have a primary neural crest defect leading to an absence of recognisable melanocytes in the skin. Melanocytes were not found in the stria of most of the mutants examined, and from about 6 days of age onwards a reduced amount of interdigitation amongst the cells of the stria was observed. These ultrastructural anomalies were associated with strial dysfunction. In the normal adult mammal, the stria produces an endocochlear potential (EP), a resting dc potential in the endolymph in the cochlear duct, which in mice is normally about +100 mV. In our control mice, EP rose to adult levels between 6 and 16 days after birth. In most of the mutants we studied, EP was close to zero at all ages from 6 to 20 days. Melanocyte-like cells appear to be vital for normal stria vascularis development and function. They may be necessary to facilitate the normal process of interdigitation between marginal and basal cell processes at a particular stage during development, and the lack of adequate interdigitation in the mutants may be the cause of their strial dysfunction. Alternatively, melanocytes may have some direct, essential role in the production of an EP by the stria. Melanocytes may be important both for normal strial development and for the production of the EP. We believe this is the clearest demonstration yet of a role for migratory melanocytes other than their role in pigmentation.

BACKGROUND: This review was commissioned because of the increasing doubt about the ability of existing screening programmes (mainly the health visitor distraction test (HVDT) at 7-8 months) to identify children with congenital hearing impairment, and technological advances which have made neonatal hearing screening an alternative option. OBJECTIVES: To review the available literature on the screening of permanent childhood hearing impairment. To provide commissioners and providers of health care with information about how to deliver a more uniform service, better outcomes, and more cost-effective screening. To identify areas for further research and service development. HOW THE RESEARCH WAS CONDUCTED: The research involved a review of the available published and unpublished literature, and a comprehensive survey of current pre-school hearing screening provision in the UK coupled with a health economics study of hearing screening costs. The research also included a number of focus groups and visits to key centres in the UK and North America. RESEARCH FINDINGS: EPIDEMIOLOGY OF PERMANENT CHILDHOOD HEARING IMPAIRMENT: There are approximately 840 children a year born in the UK with significant permanent hearing impairment likely to affect their own and their family's quality of life. Present services will miss about 400 of these children by 1 1/2 years of age, and about 200 of these children by 3 1/2 years of age. Such late identification of hearing impairment greatly reduces the responsiveness of the services for individual children. EVIDENCE FOR IMPROVED OUTCOMES WITH EARLIER IDENTIFICATION: Hearing-impaired children identified late are at risk of substantial delay in their acquisition of language and communication skills, with consequent longer-term risk to education achievement, mental health and quality of life. Theoretical arguments on neural development support the limited evidence here for the increased benefit for child and family associated with very early identification. In general, parents and professionals want very early identification, which, if implemented properly, does not cause undue anxiety. CURRENT UK PRACTICE: The survey of current practice indicated a major problem with poor information systems. This problem was further highlighted as a major concern by the multi-disciplinary focus groups. Practice varies. There are two District-wide programmes in which all newborn babies are neonatally screened, a large number of ad hoc programmes for neonatal screening of 'at-risk' babies, a variety of early surveillance programmes, and widespread use of the HVDT. Intervention and habilitation for the majority of those screened neonatally is routinely undertaken within 6 months of birth. For those screened only by the health visitor, identification was on average at about 26 months of age with intervention at about 32 months on average. (ABSTRACT TRUNCATED)

We have used a probe derived from TRP-2/DT to detect migratory melanoblasts shortly after they emerge from the neural crest, as early as 10 days post coitum (dpc). TRP-2/DT expression is otherwise restricted to the presumptive pigmented retinal epithelium, the developing telencephalon and the endolymphatic duct. The pattern of steel and c-kit hybridisation in the developing brain differed from that of TRP-2. TRP-1 and tyrosinase probes also detected melanoblasts but were both expressed later in development than TRP-2. We used the TRP-2/DT probe to investigate the way that the Steel-dickie (Sld) mutation interferes with melanocyte development, and found that the membrane-bound steel growth factor which is missing in Sld/Sld mutants is necessary for the survival of melanoblasts but not for their early migration and initial differentiation.

If two vowels with different fundamental frequencies (fo's) are presented simultaneously and monaurally, listeners often hear two talkers producing different vowels on different pitches. This paper describes the evaluation of four computational models of the auditory and perceptual processes which may underlie this ability. Each model involves four stages: (i) frequency analysis using an "auditory" filter bank, (ii) determination of the pitches present in the stimulus, (iii) segregation of the competing speech sources by grouping energy associated with each pitch to create two derived spectral patterns, and (iv) classification of the derived spectral patterns to predict the probabilities of listeners' vowel-identification responses. The "place" models carry out the operations of pitch determination and spectral segregation by analyzing the distribution of rms levels across the channels of the filter bank. The "place-time" models carry out these operations by analyzing the periodicities in the waveforms in each channel. In their "linear" versions, the place and place-time models operate directly on the waveforms emerging from the filters. In their "nonlinear" versions, analogous operations are applied to the output of an additional stage which applied a compressive nonlinearity to the filtered waveforms. Compared to the other three models, the nonlinear place-time model provides the most accurate estimates of the fo's of paris of concurrent synthetic vowels and comes closest to predicting the identification responses of listeners to such stimuli. Although the model has several limitations, the results are compatible with the idea that a place-time analysis is used to segregate competing sound sources.

Detectability of a 400-ms, 1000-Hz pure-tone signal was examined in bandlimited noise where different spectral regions were given similar waveform envelope characteristics. As expected, in random noise the threshold increased as the noise bandwidth was increased up to a critical bandwidth, but remained constant for further increases in bandwidth. In the noise with envelope coherence however, threshold decreased when the noise bandwidth was made wider than the critical bandwidth. The improvement in detectability was attributed to a process by which energy outside the critical band is used to help differentiate signal from masking noise, provided that the waveform envelope characteristics of the noise inside and outside the critical band are similar. With flanking coherent noise bands either lower or higher in frequency than a noise band centered on the signal, it was next determined that the frequency relation and remoteness of the coherent noise did not particularly influence the magnitude of the unmasking effect. An interpretation in terms of nonsimultaneous masking was reconciled with some aspects of the data, and with an interpretation in terms of across-frequency temporal pattern analysis. This paradigm, in which detection is based upon across-frequency temporal envelope coherence, was termed "comodulation masking release." Comodulation offers a controlled way to investigate some of the mechanisms which permit signals to be detected at adverse signal-to-noise ratios.

OBJECTIVE: To develop and evaluate a 12-item version of the Speech, Spatial and Qualities of Hearing scale for use in clinical research and rehabilitation settings, and provide a formula for converting scores between the full (SSQ49) and abbreviated (SSQ12) versions. DESIGN: Items were selected independently at the three centres (Eriksholm Research Centre, MRC Institute of Hearing Research, University of New England) to be representative of the complete scale. A consensus was achieved after discussion. STUDY SAMPLE: The data set (n = 1220) used for a factor analysis (Akeroyd et al, submitted) was re-analysed to compare original SSQ scores (SSQ49) with scores on the short version (SSQ12). RESULTS: A scatter-plot of SSQ12 scores against SSQ49 scores showed that SSQ12 score was about 0.6 of a scale point lower than the SSQ49 (0-10 scale) in the re-analysis of the Akeroyd et al data. SSQ12 scores lay on a slightly steeper slope than scores on the SSQ49. CONCLUSIONS: The SSQ12 provides similar results to SSQ49 in a large clinical research sample. The slightly lower average SSQ12 score and the slightly steeper slope reflect the composition of this short form relative to the SSQ49.

This paper reviews progress in understanding the psychology of lipreading and audio-visual speech perception. It considers four questions. What distinguishes better from poorer lipreaders? What are the effects of introducing a delay between the acoustical and optical speech signals? What have attempts to produce computer animations of talking faces contributed to our understanding of the visual cues that distinguish consonants and vowels? Finally, how should the process of audio-visual integration in speech perception be described; that is, how are the sights and sounds of talking faces represented at their conflux?

The mouse shaker-1 locus, Myo7a, encodes myosin VIIA and mutations in the orthologous gene in humans cause Usher syndrome type 1B or non-syndromic deafness. Myo7a is expressed very early in sensory hair cell development in the inner ear. We describe the effects of three mutations on cochlear hair cell development and function. In the Myo7a816SB and Myo7a6J mutants, stereocilia grow and form rows of graded heights as normal, but the bundles become progressively more disorganised. Most of these mutants show no gross electrophysiological responses, but some did show evidence of hair cell depolarisation despite the disorganisation of their bundles. In contrast, the original shaker-1 mutants, Myo7ash1, had normal early development of stereocilia bundles, but still showed abnormal cochlear responses. These findings suggest that myosin VIIA is required for normal stereocilia bundle organisation and has a role in the function of cochlear hair cells.