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Hearing and dementia

Abstract

Hearing deficits associated with cognitive impairment have attracted much recent interest, motivated by emerging evidence that impaired hearing is a risk factor for cognitive decline. However, dementia and hearing impairment present immense challenges in their own right, and their intersection in the auditory brain remains poorly understood and difficult to assess. Here, we outline a clinically oriented, symptom-based approach to the assessment of hearing in dementias, informed by recent progress in the clinical auditory neuroscience of these diseases. We consider the significance and interpretation of hearing loss and symptoms that point to a disorder of auditory cognition in patients with dementia. We identify key auditory characteristics of some important dementias and conclude with a bedside approach to assessing and managing auditory dysfunction in dementia.

Introduction

Although hearing impairment is not generally regarded as a cardinal feature of dementia, hearing in patients with dementia is a focus of growing clinical interest. Recent evidence suggests that hearing loss may predict or accelerate cognitive deterioration [13], and alterations of hearing may manifest as complex cognitive and behavioural symptoms relevant to the differential diagnosis of dementias [410]. Interventions targeting auditory processes (most notably, music) have gained wide currency [4, 11]. However, the organisation of the human auditory brain is complex and incompletely understood. Moreover, neuropsychological frameworks for characterising hearing disorders produced by brain disease and practical tools for assessing auditory functions suitable for use in cognitively impaired patients are often lacking.

In this review, we outline a clinically oriented, symptom-based approach to hearing in dementia, informed by recent progress in the clinical auditory neuroscience of these diseases. We consider the problem of hearing loss (impaired detection of sound and how this interacts with cognitive function) and symptoms that point to a disorder of auditory cognition (impaired understanding or behavioural responses to sound). We identify key auditory characteristics of some important dementias. We conclude with a bedside approach to assessing and managing auditory dysfunction in dementia.

The auditory brain and dementia

Neuropsychology of hearing

Hearing (considered broadly as the function of the human auditory brain and its peripheral end organs) has been aligned with other complex neuropsychological processes based on studies of the normal brain and focal brain damage [12, 13]. Together, this evidence suggests a hierarchical organisation that differentiates categories and stages of auditory information processing (Table 1). Processing of sound begins in the ascending auditory pathways extending from cochlea to primary auditory cortex in Heschl’s gyrus: this is not a passive relay but involves considerable signal transformation [12]. While the terminology of hearing disorders is problematic, in consideration of disease associations, it is useful to attempt to distinguish between peripheral (predominantly cochlear or auditory nerve), subcortical (ascending auditory pathway), and cortical auditory dysfunction. Auditory cognition—processing beyond sound detection leading to auditory perception and understanding—is mediated by distributed networks involving auditory cortex and its cerebral connections; disorders affecting these networks produce characteristic symptoms and syndromes of auditory cognitive dysfunction (summarised in Table 1).

Table 1 An outline of core operations in auditory cognition and their clinical and neuroanatomical correlates

As a framework for analysing disorders of auditory cognition, it is useful to consider complex sounds (speech, voices, music, and environmental noises) as ‘auditory objects’ that must be disambiguated from the auditory background and organised into coherent perceptual representations [13]. The processing of such sound objects entails perceptual analysis (encoding of acoustic features, such as pitch, rhythm, and timbre) leading to semantic processing (extraction of associated meaning, leading to sound recognition) [13, 14]. In the world at large, sounds are embedded in auditory scenes that must be actively deconstructed to identify and track sounds of interest [10]: this, in turn, requires the representation of sound location and movement (auditory spatial analysis) and abstraction of identifying sound characteristics under varying listening conditions (auditory apperceptive processing). Many sounds also have emotional and behavioural relevance.

The burden of dementia

Dementia is arguably the most significant public health problem confronting ageing societies, with an estimated 800,000 sufferers currently in the United Kingdom alone [15]. However, ‘dementia’ designates a syndrome of acquired, progressive, socially and/or occupationally significant cognitive and/or behavioural decline: this definition embraces over a hundred highly diverse diseases, the most common of which is Alzheimer’s disease (AD) [4, 1618]. Here, we focus on major neurodegenerative causes of dementia in mid to later life, collectively characterised by pathogenic protein spread over large-scale cerebral networks and distinctive profiles of regional brain atrophy and clinical deficits (summarised in Table 2). Brain networks targeted by these diseases overlap the temporal, parietal, frontal, and subcortical circuitry that supports auditory cognition (Tables 1, 2): this is key to anticipating and understanding the disorders of hearing that accompany particular dementia syndromes.

Table 2 Summary of major neurodegenerative dementias, emphasising auditory characteristics

Hearing loss and dementia

Epidemiological evidence

Significant hearing loss (operationally, >20 dB elevation of threshold for pure tone detection) affects around 40 % of those aged over 65 [19] and has important links to cognitive impairment and dementia. Age-related hearing loss (presbycusis) commonly results from cochlear dysfunction, though age-related alterations in more central auditory pathways may also be relevant and have probably been under-recognised [20]. The balance of epidemiological evidence across populations suggests that hearing loss is associated with cognitive decline and constitutes a risk factor for development of dementia in older adults, though the strength of this association is somewhat variable [20, 21]. One meta-analysis concluded that cognitive and hearing impairment are correlated and that hearing loss impacts on multiple domains of cognition [21]; this is not simply attributable to hearing loss confounding speech-based cognitive tasks [20] and has been observed in those with and without dementia [22]. Hearing loss ~25 dB has an effect on cognitive deterioration equivalent to around 7 years of ageing [1] and risk of dementia increases with increasing severity of hearing impairment [2].

The role of peripheral hearing

While the association between hearing loss and cognitive decline appears robust, the mechanism remains unresolved. Hearing impairment might accelerate cognitive decline by compounding sensory and social isolation, increasing cognitive load, and thereby exhausting compensatory cognitive reallocation, or constitute a nonspecific marker of frailty [20]. However, the association between impaired hearing and cognition remains after controlling for other demographic and comorbidity factors [2, 23]. Peripheral hearing loss might hasten neurodegenerative processes more directly. Hearing loss in older adults correlates with tissue volume loss in auditory cortex [24], temporal lobe, and whole brain [3], and is associated with functional reorganisation of auditory cortical networks consistent with more effortful listening and reduced cognitive reserve [25]. Though limited histopathological evidence is available concerning the auditory system in common dementias, major auditory relay nuclei are involved pathologically in AD [26, 27], while animal models suggest that peripheral deafferentation disrupts hippocampal function [28, 29].

The role of ‘central’ auditory processing

Auditory deficits in AD may be disproportionate to any abnormality of sound detection or otological markers [20, 3034]: while the neuroanatomical correlates of ‘central’ hearing measures have not been fully defined, such deficits may reflect disordered cortical mechanisms of auditory scene analysis (Table 1). This is corroborated by other evidence that abnormalities of auditory cortical evoked potentials predate clinical symptoms in young carriers of pathogenic AD mutations [35]. Information for other dementias remains very limited. Relatively, a few studies of hearing in dementia have addressed cortical auditory processing specifically, perhaps partly accounting for the wide variation in reported frequency of hearing impairment in AD [4, 36]: an observation that seems otherwise difficult to reconcile with epidemiological data.

The effects of hearing impairment on cognitive decline might be most parsimoniously considered as an interaction of peripheral and more central factors. The auditory system has extensive efferent as well as afferent traffic [12] allowing for reciprocal interaction between cortical, brainstem, and peripheral mechanisms [37]. Moreover, in practice, these can be challenging to disambiguate in individual patients.

Syndromes of dementia and hearing loss

Syndromic associations of dementia with dysfunction of cochlea or ascending auditory pathways are uncommon and generally occur in the context of more complex neurological impairment, often in younger patients; examples are summarised in Table 3.

Table 3 Some syndromes with peripheral or subcortical hearing impairment and dementia

Symptoms of altered auditory cognition in dementia

Though auditory dysfunction is rarely the presenting feature, histopathological involvement of auditory cortices has been described in major neurodegenerative dementias [26, 3841], and deficits of auditory cognition (Table 1) are not uncommon early features of these diseases. Certain general observations suggest an auditory cognitive disorder: the patient typically experiences greater listening difficulties and derives less benefit with conventional binaural amplification than anticipated from the degree of audiometric loss and may also exhibit various abnormal behavioural responses to sounds. Matching of incoming sound information to stored neural ‘templates’ based on past experience of the auditory world may be a general operating principle of the auditory brain [14, 42]: disruption of this process with neurodegenerative pathologies may lead to deficient perception or to aberrant perception of sounds. Deficient sound perception or recognition not attributable to faulty peripheral encoding constitutes an auditory agnosia, which may be selective for particular kinds of sounds; aberrant ‘excessive’ processing may manifest as auditory hallucinations. These disorders of auditory cognition commonly coexist.

Here, we emphasise the differential diagnosis of auditory symptoms; characterisation of auditory deficits using neuropsychological tests is a complementary enterprise. Together, these approaches define auditory phenotypes, summarised for selected dementias in Table 2; neuroanatomical correlates are shown in Fig. 1.

Fig. 1
figure1

Neuroanatomical signatures of disordered auditory cognition in dementias. The cutaway brain schematic (centre) shows cerebral networks that mediate key components of auditory cognition, coded I to VI (below) and based on clinical and normal functional neuroanatomical evidence (see Tables 1, 2); ‘features’ here subsumes acoustic feature detection and analysis, ‘objects’ corresponds to auditory apperceptive processing and ‘recognition’ corresponds to auditory semantic processing. The left cerebral hemisphere is projected forward in the schematic; however, neuroanatomical correlates of auditory cognition are bi-hemispherically distributed, principally, including: a amygdala, ACC anterior cingulate cortex, ATL anterior temporal lobe, BG basal ganglia, h hippocampus, HG Heschl’s gyrus (containing primary auditory cortex), IFG inferior frontal gyrus/frontal operculum, ins insula, OFC orbitofrontal cortex, PFC prefrontal cortex, PMC posterior medial cortex (posterior cingulate, precuneus), STG superior temporal gyrus/superior temporal sulcus/planum temporale, TPJ temporo–parietal junction. Side panels show characteristic profiles of regional cerebral atrophy (coronal MRI sections) and auditory cognitive functions chiefly affected in selected dementias (see also Table 2): typical Alzheimer’s disease (AD), bilateral symmetrical mesial temporal and parietal lobe atrophy; behavioural variant frontotemporal dementia (bvFTD), asymmetric (predominantly right-sided) frontal and temporal lobe atrophy; logopenic aphasia (LPA) variant of Alzheimer’s disease, predominantly left-sided temporo-parietal atrophy; microtubule-associated protein tau (MAPT) gene mutations, bilateral (predominantly antero-mesial) temporal lobe atrophy; progressive nonfluent aphasia (PNFA), predominantly left-sided peri-Sylvian atrophy; and semantic dementia (SD), asymmetric (predominantly left-sided) anterior temporal lobe atrophy

Impaired perception of sound features

Patients with dementia may have reduced perception of sound disproportionate to any damage involving cochlea or ascending auditory pathways: this may manifest as cortical deafness (described rarely in prion disease: [43, 44]) or relatively selective ‘word deafness’ or auditory agnosia, more commonly described with progressive nonfluent aphasia and (for unknown reasons) in Japanese patients [4547]. A useful clinical clue to word deafness is substantially better comprehension of written than spoken language. Speech perception may be particularly vulnerable as it depends on precise temporal feature decoding but may signify a more generic impairment of auditory feature analysis in syndromes with peri-Sylvian degeneration [48, 49].

Impaired perception of auditory scenes and objects

Frank auditory disorientation is uncommon in dementia though does occur (usually accompanying visual disorientation) in patients with posterior cortical degenerations [50]. However, patients with both posterior cortical atrophy and clinically typical AD commonly report difficulty following conversations and other sounds against background noise, and this may contribute to their avoiding social situations and a general dislike of busy auditory environments [51]. Such symptoms may develop early in the course of the illness and without other evidence of hearing loss and (though often attributed to a nonspecific memory or attentional deficit) may signify AD-associated impairments of auditory scene and auditory spatial analysis, correlated in structural and functional neuroanatomical studies with disintegration of a core parieto-temporal network [6, 9, 10]. Auditory scene analysis depends on accurate parsing of the acoustic stream into constituent sound objects, mediated by sensory computational mechanisms under attentional and executive control; these mechanisms interact in temporo-parietal cortical ‘hub’ regions.

Other symptoms, experienced particularly by patients with AD and progressive nonfluent aphasia suggest auditory apperceptive dysfunction: disproportionate difficulty identifying or understanding sound objects under unusual or degraded listening conditions. The patient may not recognise a familiar voice over a noisy telephone line or may fail to understand a message delivered in an unfamiliar accent [8, 52]). Such symptoms may have a neuroanatomical substrate in posterior peri-Sylvian cortices, similar to that underpinning impaired auditory scene analysis.

Impaired recognition of sounds

Patients with semantic dementia develop deficits of nonverbal sound recognition (auditory associative agnosia) as part of a pan-modal erosion of semantic memory, linked to antero-mesial temporal lobe dysfunction [48, 53, 54]. Interestingly, in individual cases, there may be relatively preserved knowledge of melodies over environmental sounds [55, 56], perhaps because the abstract, nonreferential meaning systems of music have neural substrates that are separable from those mediating knowledge about the world at large (knowledge of musical instrument timbres may be affected comparably to other categories of objects). Impaired recognition of familiar voices (despite retained ability to distinguish between voices) may be a salient symptom of right temporal lobe degeneration [57, 58]: such ‘associative phonagnosia’ may be relatively selective for voices or accompany other deficits of person knowledge or more generalised auditory agnosia.

Auditory hallucinations

Patients with semantic dementia commonly report tinnitus (an elementary auditory hallucination), linked to structural alterations in a fronto–temporo–subcortical network [5]; while hallucinations of ‘muffled’ sounds or voices are often reported by patients with Lewy body dementia, frank verbal hallucinations are uncommon and generally occur as a component of more complex, multimodal hallucinations [59]. In contrast, persistent musical hallucinations (typically comprising familiar, banal tunes) are relatively commonly reported in patients with Lewy body disease and less frequently, other dementias [60]: only a minority has significant hearing loss, suggesting that aberrant cortical activity plays a key role though there may be a facilitatory effect from peripheral deafferentation [61].

Abnormal auditory behaviours

Altered emotional or ‘hedonic’ behavioural responses to sound are increasingly recognised in patients with dementia. Impaired processing of emotional prosody has been described in AD, Parkinson’s disease, behavioural variant frontotemporal dementia, and progressive aphasia syndromes [6265], and impaired recognition of musical and nonverbal vocal emotions in Parkinson’s disease and frontotemporal dementia syndromes [65, 66]. Many patients with frontotemporal dementia (and some with AD) exhibit sound aversion, while abnormal craving for music (musicophilia) is particularly associated with semantic dementia [51]; these patients may show increased sensitivity to sound (hyperacusis [5]), also described in prion disease [67]. Explicit behavioural responses may dissociate from autonomic responses to sound in dementia syndromes [68, 69]. Altered auditory hedonic behaviours in dementia have been linked to involvement of distributed cortico–subcortical circuitry that processes emotion and reward [51].

A practical approach to the patient with dementia and altered hearing

A clinical framework for assessing and managing the patient presenting with cognitive impairment and altered hearing is outlined in Table 4 and Fig. 2.

Table 4 Taking the auditory history in patients with cognitive impairment
Fig. 2
figure2

A clinical approach to the patient presenting with cognitive decline and altered hearing. Our approach is based on initial thorough bedside history taking and examination to identify key auditory symptoms (see also Table 4) supplemented by investigations to characterise the nature of the patient’s hearing and cognitive deficits. As clinical symptoms are rarely specific and disorders at different levels of the auditory processing hierarchy frequently coexist, we recommend a core hearing assessment battery in all cases, corroborated by general neuropsychological assessment and brain MRI. Together, these assessments often allow the patient’s hearing deficit to be localised predominantly to the cochlea or ascending auditory pathways (unfilled oblongs) or to cerebral circuitry (black filled oblongs) and direct further more specific assessment for the diagnoses listed in Tables 2, 3. Other patients will have auditory deficits that are more difficult to localise or may have mixed deficits (grey oblongs); speech-in-noise perception is a useful index of real world hearing impairment but needs care in interpretation as this can be affected by pathology at different levels of the auditory system. Management in all cases should involve consideration of environmental and behavioural modification strategies that optimise the patient’s residual hearing function (see text) and involvement of multidisciplinary services to assess their needs and plan appropriate care delivery; we have a low threshold for a trial of hearing aids or other assistive listening devices if there is the possibility of a contributing peripheral hearing loss and in patients with more complex or central auditory deficits, onward referral to a specialist cognitive or auditory clinic may be helpful. Asterisk particularly in younger patients or where there are associated neurological or systemic features; double asterisk more specialised tests of central hearing functions if available may be useful in defining the phenotype of an auditory cortical disorder, particularly where all standard tests of hearing are unremarkable; ABR auditory brainstem evoked responses, AHQ auditory handicap questionnaire, ALD assistive listening device, behav behavioural, env environmental, DLT dichotic listening test, GiN gap-in-noise perception, HA hearing aid, MBEA Montreal Battery for Evaluation of Amusia, MRI brain magnetic resonance imaging, NAB Newcastle Auditory Battery, neuropsych neuropsychology, OAE otoacoustic emissions, PTA pure tone audiometry, SiN speech-in-noise perception

Clinical assessment

Hearing function should be assessed in all patients receiving a diagnosis of dementia: to identify a factor that may be detracting from quality of life and impeding care [70], to gauge disability as fully as possible, and to address any reversible peripheral component. Assessment begins with a history to elicit key auditory symptoms (Table 4; Fig. 2) and systematic neurological and otological examination. Hearing impairment can easily go undetected in patients with dementia and may lead to misattribution or overestimation of cognitive compromise [71]; cognitive screening instruments that do not rely on hearing (such as the TYM test [72]) may be preferable to verbally administered tests such as the mini–mental state examination. The patient’s premorbid competence in particular domains (notably, music) should be documented, and an auditory handicap questionnaire may be useful in defining the functional consequences of hearing impairment [73].

In patients with known hearing impairment, any supervening cognitive decline should be thoroughly characterised. This is particularly challenging in those with pre-lingual profound hearing loss. British Sign Language (BSL) users form a close-knit minority group defined culturally as well as linguistically; they often have difficulty in accessing appropriate assessment services and, therefore, may have more advanced dementia when diagnosed. Ideally, assessment of such individuals should be undertaken in a clinic with special expertise in working with deaf people, engaging a neuropsychologist with relevant communication skills and expertise and BSL interpreters trained in the requirements of cognitive assessment. Until recently, there were no normative data on cognition in deaf subjects; however, a cognitive screening instrument based on British Sign Language has now been developed [74]. As with any cognitive assessment, it is essential to obtain a corroborating history from an advocate or caregiver.

Investigations

Pure tone audiometry and otoacoustic emissions (supplemented by brainstem auditory evoked potentials if available) are relatively simple and well-tolerated techniques to assess cochlear and ascending auditory pathway function in cognitively impaired patients. Measurement of speech-in-noise perception (Table 1) more closely reflects ‘real world’ hearing impairment than pure tone audiometry and (if impaired disproportionately to other indices of cochlear or ascending auditory pathway function) may provide an index of auditory cortical processing; this can be supplemented by tests such as gap-in-noise perception or dichotic listening that depend more sensitively on cortical processing of sound [75] (Table 1). If the patient or caregiver reports symptoms suggesting auditory cognitive dysfunction, a more comprehensive evaluation may be appropriate: age norms are available for the Newcastle Auditory Battery [76] and Montreal Battery for Evaluation of Amusia [77]. General neuropsychological assessment can identify concurrent executive or attentional deficits that may confound performance on auditory tests and may also define associated (visual and verbal) apperceptive or semantic impairments to corroborate any auditory cognitive deficit. If a complex or unusual disorder (such as auditory agnosia) is suspected, referral to a specialist clinic may be indicated. Investigation of any patient with suspected dementia should focus on identifying reversible processes and establishing the primary cause as accurately as possible (for general reviews, see [1618]); this should include brain MRI, which may further characterise the likely neuroanatomical basis of the auditory deficit (Table 2).

Management

While effects on specific aspects of cognitive function are difficult to predict, correction of reversible hearing deficits has been shown to benefit global functioning in daily life [20, 21, 23, 78, 79]. Simple interventions such as earwax removal can be highly effective [79]. In addition, prescription of hearing aids and other assistive listening devices where appropriate, environmental modification strategies may be useful in managing altered hearing in patients with dementia. Examples include the use of written communication aids and electronic devices, conducting conversations face-to-face and free of significant background noise, avoiding sounds known to provoke distress, and masking techniques for musical and other auditory hallucinations. Anecdotally, cholinesterase inhibitors may benefit some patients with musical hallucinations [80]; however, there is currently little role for specific pharmacotherapy in managing auditory dysfunction in dementia. Specific auditory training protocols based on speech and nonspeech sounds have been shown to improve speech intelligibility in hearing-impaired adults [81] but have yet to be adequately assessed in dementia. Though music is unquestionably a welcome source of solace for many patients and caregivers and often a useful displacement activity, evidence for a specific role of music therapy in the management of dementia awaits better-controlled trials [11]. Assistive listening devices incorporating a mobile microphone are a promising strategy to compensate for deficits of auditory scene processing [73], but their utility in patients with dementia remains to be established. Management of hearing loss in dementia is an inter-disciplinary enterprise, and close collaboration with audiology, speech and language therapy, and social services is invaluable.

Conclusions and future directions

Hearing has long been the poor relation of memory and vision in the cognitive clinic. The emerging epidemiological evidence may shortly transform this situation. The comprehensive assessment of hearing in dementia presents both challenges and opportunities. There is a need to develop practical and reliable tests that can disambiguate the effects of peripheral hearing and auditory cognitive dysfunction, to develop auditory interventions directed to cognitively impaired people and to assess these systematically and longitudinally in a range of dementias, referenced to healthy older people. In addition to capturing disability and improving quality of life, a more detailed picture of the spectrum of auditory dysfunction in dementia would have considerable neurobiological and clinical resonance. Sound is a dynamic and computationally demanding sensory signal that engages complex emotional and other behaviours: the processing of sounds taxes brain networks targeted by neurodegenerative pathologies and may yet yield novel cognitive ‘stress tests’ for diagnosis and treatment tracking in these diseases.

References

  1. 1.

    Lin FR, Ferrucci L, Metter EJ et al (2011) Hearing loss and cognition in the Baltimore Longitudinal Study of aging. Neuropsychology 25:763–770. doi:10.1037/a0024238

    PubMed  PubMed Central  Article  Google Scholar 

  2. 2.

    Lin FR, Metter EJ, O’Brien RJ et al (2011) Hearing loss and incident dementia. Arch Neurol 68:214–220. doi:10.1001/archneurol.2010.362.Hearing

    PubMed  PubMed Central  Google Scholar 

  3. 3.

    Lin F, Ferrucci L, An Y et al (2014) Association of hearing impairment with brain volume changes in older adults. Neuroimage 90:84–92. doi:10.1016/j.neuroimage.2013.12.059

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  4. 4.

    Johnson J, Chow M (2015) Hearing and music in dementia. Handb Clin Neurol. doi:10.14440/jbm.2015.54.A

    Google Scholar 

  5. 5.

    Mahoney CJ, Rohrer JD, Goll JC et al (2011) Structural neuroanatomy of tinnitus and hyperacusis in semantic dementia. J Neurol Neurosurg Psychiatry 82:1274–1278. doi:10.1136/jnnp.2010.235473

    PubMed  PubMed Central  Article  Google Scholar 

  6. 6.

    Goll JC, Kim LG, Ridgway GR et al (2012) Impairments of auditory scene analysis in Alzheimer’s disease. Brain 135:190–200. doi:10.1093/brain/awr260

    PubMed  Article  Google Scholar 

  7. 7.

    Goll JC, Kim LG, Hailstone JC et al (2011) Auditory object cognition in dementia. Neuropsychologia 49:2755–2765. doi:10.1016/j.neuropsychologia.2011.06.004

    PubMed  PubMed Central  Article  Google Scholar 

  8. 8.

    Hailstone JC, Ridgway GR, Bartlett JW et al (2012) Accent processing in dementia. Neuropsychologia 50:2233–2244. doi:10.1016/j.neuropsychologia.2012.05.027

    PubMed  PubMed Central  Article  Google Scholar 

  9. 9.

    Golden HL, Nicholas JM, Yong KXX et al (2015) Auditory spatial processing in Alzheimer’s disease. Brain 138:189–202. doi:10.1093/brain/awu337

    PubMed  Article  Google Scholar 

  10. 10.

    Golden HL, Agustus JL, Goll JC et al (2015) Functional neuroanatomy of auditory scene analysis in Alzheimer’s disease. Neuroimage Clin 7:699–708. doi:10.1016/j.nicl.2015.02.019

    PubMed  PubMed Central  Article  Google Scholar 

  11. 11.

    Vink AC, Birks JS, Bruinsma MS, Scholten RJS (2004) Music therapy for people with dementia. Cochrane database Syst Rev. doi:10.1002/14651858.CD003477.pub2

    PubMed  Google Scholar 

  12. 12.

    Cope TE, Baguley DM, Griffiths TD (2015) The functional anatomy of central auditory processing. Pract Neurol 15:302–308. doi:10.1136/practneurol-2014-001073

    PubMed  PubMed Central  Article  Google Scholar 

  13. 13.

    Goll JC, Crutch SJ, Warren JD (2010) Central auditory disorders: toward a neuropsychology of auditory objects. Curr Opin Neurol 23:617–627. doi:10.1097/WCO.0b013e32834027f6

    PubMed  PubMed Central  Article  Google Scholar 

  14. 14.

    Griffiths TD, Warren JD (2004) What is an auditory object? Nat Rev Neurosci 5:887–892. doi:10.1038/nrn1538

    CAS  PubMed  Article  Google Scholar 

  15. 15.

    Prince M, Knapp M, Guerchet M, McCrone P, Prina M, Comas-Herrera M et al (2014) Dementia UK: update. Alzheimer's Society, 136 p

  16. 16.

    Warren JD, Rohrer JD, Rossor MN (2013) Frontotemporal dementia. BMJ 4827:1–9. doi:10.1136/bmj.f4827

    Google Scholar 

  17. 17.

    Rossor MN, Fox NC, Mummery CJ et al (2010) The diagnosis of young-onset dementia. Lancet Neurol 9:793–806. doi:10.1016/S1474-4422(10)70159-9

    PubMed  PubMed Central  Article  Google Scholar 

  18. 18.

    Warren JD, Fletcher PD, Golden HL (2012) The paradox of syndromic diversity in Alzheimer disease. Nat Rev Neurol 8:451–464. doi:10.1038/nrneurol.2012.135

    CAS  PubMed  Google Scholar 

  19. 19.

    Gates GA, Mills JH (2005) Presbycusis. Lancet 366:1111–1120. doi:10.1016/S0140-6736(05)67423-5

    PubMed  Article  Google Scholar 

  20. 20.

    Panza F, Solfrizzi V, Seripa D et al (2015) Age-related hearing impairment and frailty in Alzheimer’s disease: interconnected associations and mechanisms. Front Aging Neurosci 7:113. doi:10.3389/fnagi.2015.00113

    PubMed  PubMed Central  Article  Google Scholar 

  21. 21.

    Schmulian Taljaard D, Olaithe M, Brennan-Jones CG et al (2015) The relationship between hearing impairment and cognitive function: a meta-analysis in adults. Clin Otolaryngol. doi:10.1111/coa.12607

    PubMed  Google Scholar 

  22. 22.

    Uhlmann R, Larson E, Rees T et al (1989) Relationship of hearing impairment to dementia and cognitive dysfunction in older adults. JAMA 261:1916–1919

    CAS  PubMed  Article  Google Scholar 

  23. 23.

    Dawes P, Emsley R, Cruickshanks KJ et al (2015) Hearing loss and cognition: the role of hearing aids, social isolation and depression. PLoS One 10:1–9. doi:10.1371/journal.pone.0119616

    Google Scholar 

  24. 24.

    Peelle JE, Troiani V, Grossman M, Wingfield A (2011) Hearing loss in older adults affects neural systems supporting speech comprehension. J Neurosci 31:12638–12643. doi:10.1523/JNEUROSCI.2559-11.2011

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  25. 25.

    Cardin V (2016) Effects of aging and adult-onset hearing loss on cortical auditory regions. Front Neurosci 10:00199. doi:10.3389/fnins.2016.00199

    Article  Google Scholar 

  26. 26.

    Sinha UK, Hollen KM, Rodriguez R, Miller CA (1993) Auditory system degeneration in Alzheimer’s disease. Neurology 43:779–785

    CAS  PubMed  Article  Google Scholar 

  27. 27.

    Parvizi J, Van Hoesen GW, Damasio A (2001) The selective vulnerability of brainstem nuclei to Alzheimer’s disease. Ann Neurol 49:53–66. doi:10.1002/1531-8249(200101)49:1<53:AID-ANA30>3.0.CO;2-Q

    CAS  PubMed  Article  Google Scholar 

  28. 28.

    Liu L, Shen P, He T et al (2016) Noise induced hearing loss impairs spatial learning/memory and hippocampal neurogenesis in mice. Sci Rep 6:20374. doi:10.1038/srep20374

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  29. 29.

    Park SY, Kim MJ, Sikandaner H et al (2016) A causal relationship between hearing loss and cognitive impairment. Acta Otolaryngol 136:480–483. doi:10.3109/00016489.2015.1130857

    PubMed  Article  Google Scholar 

  30. 30.

    Strouse AL, Hall JW, Burger MC (1995) Central auditory processing in Alzheimer’s disease. Ear Hear 16:230–238

    CAS  PubMed  Article  Google Scholar 

  31. 31.

    Gates GA, Cobb JL, Linn RT et al (1996) Central auditory dysfunction, cognitive dysfunction, and dementia in older people. Arch Otolaryngol Head Neck Surg 122:161–167

    CAS  PubMed  Article  Google Scholar 

  32. 32.

    Idrizbegovic E, Hederstierna C, Dahlquist M et al (2011) Central auditory function in early Alzheimer’s disease and in mild cognitive impairment. Age Ageing 40:249–254. doi:10.1093/ageing/afq168

    PubMed  Article  Google Scholar 

  33. 33.

    Quaranta N, Coppola F, Casulli M et al (2014) The prevalence of peripheral and central hearing impairment and its relation to cognition in older adults. Audiol Neurootol 19(Suppl 1):10–14. doi:10.1159/000371597

    PubMed  Google Scholar 

  34. 34.

    Gates GA, Anderson ML, McCurry SM et al (2011) Central auditory dysfunction as a harbinger of Alzheimer dementia. Arch Otolaryngol Head Neck Surg 137:390–395. doi:10.1001/archoto.2011.28

    PubMed  PubMed Central  Article  Google Scholar 

  35. 35.

    Golob EJ, Ringman JM, Irimajiri R et al (2009) Cortical event-related potentials in preclinical familial Alzheimer disease. Neurology 73:1649–1655. doi:10.1212/WNL.0b013e3181c1de77

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  36. 36.

    Gold M, Lightfoot LA, Hnath-Chisolm T et al (1996) Hearing loss in a memory disorders clinic. Arch Neurol 53:922. doi:10.1001/archneur.1996.00550090134019

    CAS  PubMed  Article  Google Scholar 

  37. 37.

    Vitale C, Marcelli V, Allocca R et al (2012) Hearing impairment in Parkinson’s disease: expanding the nonmotor phenotype. Mov Disord 27:1530–1535. doi:10.1002/mds.25149

    PubMed  Article  Google Scholar 

  38. 38.

    Baloyannis SJ, Manolides SL, Manolides LS (2011) Dendritic and spinal pathology in the acoustic cortex in Alzheimer’s disease: morphological estimation in Golgi technique and electron microscopy. Acta Otolaryngol 131:610–612. doi:10.3109/00016489.2010.539626

    PubMed  Article  Google Scholar 

  39. 39.

    Baloyannis SJ, Mauroudis I, Manolides SL, Manolides LS (2011) The acoustic cortex in frontotemporal dementia: a Golgi and electron microscope study. Acta Otolaryngol 131:359–361. doi:10.3109/00016489.2010.539267

    PubMed  Article  Google Scholar 

  40. 40.

    Baloyannis SJ, Manolidis SL, Manolidis LS (1995) Synaptic alterations in acoustic cortex in Creutzfeldt-Jacob disease. Acta Otolaryngol 115:202–205

    CAS  PubMed  Article  Google Scholar 

  41. 41.

    Baloyannis SJ (2005) The acoustic cortex in vascular dementia: a Golgi and electron microscope study. J Neurol Sci 229–230:51–55. doi:10.1016/j.jns.2004.11.025

    PubMed  Article  Google Scholar 

  42. 42.

    Clark CN, Warren JD (2016) Emotional caricatures in frontotemporal dementia. Cortex 76:134–136. doi:10.1016/j.cortex.2015.07.026

    PubMed  Article  Google Scholar 

  43. 43.

    Orimo S, Ozawa E, Uematsu M et al (2000) A case of Creutzfeldt-Jakob disease presenting with auditory agnosia as an initial manifestation. Eur Neurol 44:256–258. doi:10.1159/000008250

    CAS  PubMed  Article  Google Scholar 

  44. 44.

    Krishna P, Bauer C (2004) Hearing loss as the initial presentation of Creutzfeldt-Jakob disease. Ear Nose Throat J 83:535, 538, 540 passim

    PubMed  Google Scholar 

  45. 45.

    Otsuki M, Soma Y, Sato M et al (1998) Slowly progressive pure word deafness. Eur Neurol 951:135–140

    Article  Google Scholar 

  46. 46.

    Iizuka O, Suzuki K, Endo K (2007) Pure word deafness and pure anarthria in a patient with frontotemporal dementia. Eur J Neurol 14:473–475. doi:10.1111/j.1468-1331.2006.01671.x

    CAS  PubMed  Article  Google Scholar 

  47. 47.

    Kuramoto S, Hirano T, Uyama E et al (2002) A case of slowly progressive aphasia accompanied with auditory agnosia. Rinsho Shinkeigaku Clin Neurol 42:299–303

    Google Scholar 

  48. 48.

    Goll JC, Crutch SJ, Loo JHY et al (2010) Non-verbal sound processing in the primary progressive aphasias. Brain 133:272–285. doi:10.1093/brain/awp235

    PubMed  Article  Google Scholar 

  49. 49.

    Grube M, Bruffaerts R, Schaeverbeke J et al (2016) Core auditory processing deficits in primary progressive aphasia. Brain 139:1817–1829. doi:10.1093/brain/aww067

    PubMed  PubMed Central  Article  Google Scholar 

  50. 50.

    Uttner I, Mottaghy FM, Schreiber H et al (2006) Primary progressive aphasia accompanied by environmental sound agnosia: a neuropsychological, MRI and PET study. Psychiatry Res 146:191–197. doi:10.1016/j.pscychresns.2005.12.003

    PubMed  Article  Google Scholar 

  51. 51.

    Fletcher PD, Downey LE, Golden HL et al (2015) Auditory hedonic phenotypes in dementia: a behavioural and neuroanatomical analysis. Cortex 67:95–105. doi:10.1016/j.cortex.2015.03.021

    PubMed  PubMed Central  Article  Google Scholar 

  52. 52.

    Fletcher PD, Downey LE, Agustus JL et al (2013) Agnosia for accents in primary progressive aphasia. Neuropsychologia 51:1709–1715. doi:10.1016/j.neuropsychologia.2013.05.013

    PubMed  PubMed Central  Article  Google Scholar 

  53. 53.

    Bozeat S, Lambon Ralph MA, Patterson K et al (2000) Non-verbal semantic impairment in semantic dementia. Neuropsychologia 38:1207–1215. doi:10.1016/S0028-3932(00)00034-8

    CAS  PubMed  Article  Google Scholar 

  54. 54.

    Golden HL, Downey LE, Fletcher PD et al (2015) Identification of environmental sounds and melodies in syndromes of anterior temporal lobe degeneration. J Neurol Sci 352:94–98. doi:10.1016/j.jns.2015.03.007

    PubMed  PubMed Central  Article  Google Scholar 

  55. 55.

    Omar R, Hailstone JC, Warren JE et al (2010) The cognitive organization of music knowledge: a clinical analysis. Brain 133:1200–1213. doi:10.1093/brain/awp345

    PubMed  PubMed Central  Article  Google Scholar 

  56. 56.

    Hsieh S, Hornberger M, Piguet O, Hodges JR (2011) Neural basis of music knowledge: evidence from the dementias. Brain 134:2523–2534. doi:10.1093/brain/awr190

    PubMed  Article  Google Scholar 

  57. 57.

    Hailstone JC, Ridgway GR, Bartlett JW et al (2011) Voice processing in dementia: a neuropsychological and neuroanatomical analysis. Brain 134:2535–2547. doi:10.1093/brain/awr205

    PubMed  PubMed Central  Article  Google Scholar 

  58. 58.

    Hailstone JC, Crutch SJ, Vestergaard MD et al (2010) Progressive associative phonagnosia: a neuropsychological analysis. Neuropsychologia 48:1104–1114. doi:10.1016/j.neuropsychologia.2009.12.011

    PubMed  PubMed Central  Article  Google Scholar 

  59. 59.

    Holroyd S, Currie L, Wooten GF (2001) Prospective study of hallucinations and delusions in Parkinson’s disease. J Neurol Neurosurg Psychiatry 70:734–738

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  60. 60.

    Golden EC, Josephs KA (2015) Minds on replay: musical hallucinations and their relationship to neurological disease. Brain 138:3793–3802. doi:10.1093/brain/awv286

    PubMed  Article  Google Scholar 

  61. 61.

    Griffiths TD (2000) Musical hallucinosis in acquired deafness: phenomenology and brain substrate. Brain 123:2065–2076. doi:10.1093/brain/123.10.2065

    PubMed  Article  Google Scholar 

  62. 62.

    Horley K, Reid A, Burnham D (2010) Emotional prosody perception and production in dementia of the Alzheimer’s type. J Speech Lang Hear Res 53:1132–1146. doi:10.1044/1092-4388(2010/09-0030)

    PubMed  Article  Google Scholar 

  63. 63.

    Dara C, Kirsch-Darrow L, Ochfeld E et al (2013) Impaired emotion processing from vocal and facial cues in frontotemporal dementia compared to right hemisphere stroke. Neurocase 19:521–529. doi:10.1080/13554794.2012.701641

    PubMed  Article  Google Scholar 

  64. 64.

    Rohrer JD, Sauter D, Scott S et al (2012) Receptive prosody in nonfluent primary progressive aphasias. Cortex 48:308–316. doi:10.1016/j.cortex.2010.09.004

    PubMed  PubMed Central  Article  Google Scholar 

  65. 65.

    Lima CF, Garrett C, Castro SL (2013) Not all sounds sound the same: Parkinson’s disease affects differently emotion processing in music and in speech prosody. J Clin Exp Neuropsychol 35:373–392. doi:10.1080/13803395.2013.776518

    PubMed  Article  Google Scholar 

  66. 66.

    Omar R, Henley SMD, Bartlett JW et al (2011) The structural neuroanatomy of music emotion recognition: evidence from frontotemporal lobar degeneration. Neuroimage 56:1814–1821. doi:10.1016/j.neuroimage.2011.03.002

    PubMed  PubMed Central  Article  Google Scholar 

  67. 67.

    Merkler AE, Prasad M, Lavi E, Safdieh J (2014) Hyperacusis as the initial presentation of Creutzfeldt-Jakob disease. Neurol Neuroimmunol Neuroinflamm 1:e32. doi:10.1212/NXI.0000000000000032

    PubMed  PubMed Central  Article  Google Scholar 

  68. 68.

    Fletcher PD, Nicholas JM, Shakespeare TJ et al (2015) Physiological phenotyping of dementias using emotional sounds. Alzheimer’s Dement Diagn Assess Dis Monit 1:170–178. doi:10.1016/j.dadm.2015.02.003

    Google Scholar 

  69. 69.

    Fletcher PD, Nicholas JM, Shakespeare TJ et al (2015) Dementias show differential physiological responses to salient sounds. Front Behav Neurosci 9:73. doi:10.3389/fnbeh.2015.00073

    PubMed  PubMed Central  Article  Google Scholar 

  70. 70.

    Weinstein BE, Ventry IM (1982) Hearing impairment and social isolation in the elderly. J Speech Hear Res 25:593–599. doi:10.1044/jshr.2504.593

    CAS  PubMed  Article  Google Scholar 

  71. 71.

    Weinstein B (1986) Hearing loss and senile dementia in the institutionalized elderly. Clin Gerontol 4:3–15. doi:10.1300/J018v04n03_02

    Article  Google Scholar 

  72. 72.

    Brown J, Pengas G, Dawson K, Brown LA, Clatworthy P (2009) Self administered cognitive screening test (TYM) for detection of Alzheimer’s disease: cross sectional study. BMJ 338:b2030. doi:10.1136/bmj.b2030

    PubMed  PubMed Central  Article  Google Scholar 

  73. 73.

    Martin JS, Jerger JF (2005) Some effects of aging on central auditory processing. J Rehabil Res Dev 42:25–44. doi:10.1682/JRRD.2004.12.0164

    PubMed  Article  Google Scholar 

  74. 74.

    Atkinson J, Denmark T, Marshall J et al (2015) Detecting cognitive impairment and dementia in deaf people: the British Sign Language cognitive screening test. Arch Clin Neuropsychol 30:694–711. doi:10.1093/arclin/acv042

    PubMed  Article  Google Scholar 

  75. 75.

    Musiek FE, Chermak GD (2015) Psychophysical and behavioral peripheral and central auditory tests. Handb Clin Neurol 129:313–332. doi:10.1016/B978-0-444-62630-1.00018-4

    PubMed  Article  Google Scholar 

  76. 76.

    Griffiths TD, Dean JL, Woods W et al (2001) The Newcastle Auditory Battery (NAB). A temporal and spatial test battery for use on adult naive subjects. Hear Res 154:165–169

    CAS  PubMed  Article  Google Scholar 

  77. 77.

    Peretz I, Champod AS, Hyde K (2003) Varieties of musical disorders: the Montreal Battery of evaluation of amusia. Ann N Y Acad Sci 999:58–75. doi:10.1196/annals.1284.006

    PubMed  Article  Google Scholar 

  78. 78.

    Allen NH, Burns A, Newton V et al (2003) The effects of improving hearing in dementia. Age Ageing 32:189–193

    PubMed  Article  Google Scholar 

  79. 79.

    Sugiura S, Yasue M, Sakurai T et al (2014) Effect of cerumen impaction on hearing and cognitive functions in Japanese older adults with cognitive impairment. Geriatr Gerontol Int 14(Suppl 2):56–61. doi:10.1111/ggi.12251

    PubMed  Article  Google Scholar 

  80. 80.

    Blom JD, Adriaan J, Coebergh F et al (2015) Musical hallucinations treated with acetylcholinesterase inhibitors. Front Psychiatry. doi:10.3389/fpsyt.2015.00046

    PubMed  PubMed Central  Google Scholar 

  81. 81.

    Henshaw H, Ferguson MA (2013) Efficacy of individual computer-based auditory training for people with hearing loss: a systematic review of the evidence. PLoS One 8:e62836. doi:10.1371/journal.pone.0062836

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  82. 82.

    Clark CN, Golden HL, Warren JD (2015) Acquired amusia. Handb Clin Neurol 129:607–631. doi:10.1016/B978-0-444-62630-1.00034-2

    PubMed  Article  Google Scholar 

  83. 83.

    Zeigelboim BS, de Carvalho HAS, Teive HAG et al (2015) Central auditory processing in patients with spinocerebellar ataxia. Hear Res 327:235–244. doi:10.1016/j.heares.2015.07.006

    PubMed  Article  Google Scholar 

  84. 84.

    Kay J, Lesser R, Coltheart M (1992) Psycholinguistic assessments of language processing in aphasia. Lawrence Erlbaum Associates, Hove

    Google Scholar 

  85. 85.

    Johnson J, Chang C, Brambati S et al (2011) Music recognition in frontotemporal lobar degeneration and Alzheimer disease. Cogn Behav Neurol 24:74–84. doi:10.1097/WNN.0b013e31821de326.Music

    PubMed  PubMed Central  Article  Google Scholar 

  86. 86.

    Hsieh S, Hornberger M, Piguet O, Hodges JR (2012) Brain correlates of musical and facial emotion recognition: evidence from the dementias. Neuropsychologia 50:1814–1822. doi:10.1016/j.neuropsychologia.2012.04.006

    CAS  PubMed  Article  Google Scholar 

  87. 87.

    Brønnick KS, Nordby H, Larsen JP, Aarsland D (2010) Disturbance of automatic auditory change detection in dementia associated with Parkinson’s disease: a mismatch negativity study. Neurobiol Aging 31:104–113. doi:10.1016/j.neurobiolaging.2008.02.021

    PubMed  Article  Google Scholar 

  88. 88.

    Cheng C-H, Wang P-N, Hsu W-Y, Lin Y-Y (2012) Inadequate inhibition of redundant auditory inputs in Alzheimer’s disease: an MEG study. Biol Psychol 89:365–373. doi:10.1016/j.biopsycho.2011.11.010

    PubMed  Article  Google Scholar 

  89. 89.

    Grimes AM, Grady CL, Foster NL et al (1985) Central auditory function in Alzheimer’s disease. Neurology 35:352–358

    CAS  PubMed  Article  Google Scholar 

  90. 90.

    Grady CL, Grimes AM, Patronas N et al (1989) Divided attention, as measured by dichotic speech performance, in dementia of the Alzheimer type. Arch Neurol 46:317–320

    CAS  PubMed  Article  Google Scholar 

  91. 91.

    Eustache F, Lambert J, Cassier C et al (1995) Disorders of auditory identification in dementia of the Alzheimer type. Cortex 31:119–127. doi:10.1016/S0010-9452(13)80110-4

    CAS  PubMed  Article  Google Scholar 

  92. 92.

    Brandt J, Bakker A, Maroof DA (2010) Auditory confrontation naming in Alzheimer’s disease. Clin Neuropsychol 24:1326–1338. doi:10.1080/13854046.2010.518977

    PubMed  PubMed Central  Article  Google Scholar 

  93. 93.

    Rapcsak SZ, Kentros M, Rubens AB (1989) Impaired recognition of meaningful sounds in Alzheimer’s disease. Arch Neurol 46:1298–1300

    CAS  PubMed  Article  Google Scholar 

  94. 94.

    Bouma A, Gootjes L (2011) Effects of attention on dichotic listening in elderly and patients with dementia of the Alzheimer type. Brain Cogn 76:286–293. doi:10.1016/j.bandc.2011.02.008

    PubMed  Article  Google Scholar 

  95. 95.

    Grahn JA, Brett M (2009) Impairment of beat-based rhythm discrimination in Parkinson’s disease. Cortex 45:54–61. doi:10.1016/j.cortex.2008.01.005

    PubMed  Article  Google Scholar 

  96. 96.

    Hughes LE, Rowe JB (2013) The impact of neurodegeneration on network connectivity: a study of change detection in frontotemporal dementia. J Cogn Neurosci 25:802–813. doi:10.1162/jocn_a_00356

    PubMed  PubMed Central  Article  Google Scholar 

  97. 97.

    Agustus JL, Mahoney CJ, Downey LE et al (2015) Functional MRI of music emotion processing in frontotemporal dementia. Ann N Y Acad Sci 1337:232–240. doi:10.1111/nyas.12620

    PubMed  PubMed Central  Article  Google Scholar 

  98. 98.

    Goll JC, Ridgway GR, Crutch SJ et al (2012) Nonverbal sound processing in semantic dementia: a functional MRI study. Neuroimage 61:170–180. doi:10.1016/j.neuroimage.2012.02.045

    PubMed  PubMed Central  Article  Google Scholar 

  99. 99.

    Hardy CJD, Buckley AH, Downey LE et al (2015) The language profile of behavioral variant frontotemporal dementia. J Alzheimers Dis 50:359–371. doi:10.3233/JAD-150806

    PubMed  PubMed Central  Article  CAS  Google Scholar 

  100. 100.

    Kertesz A, Ang LC, Jesso S et al (2013) Psychosis and hallucinations in frontotemporal dementia with the C9ORF72 mutation: a detailed clinical cohort. Cogn Behav Neurol 26:146–154. doi:10.1097/WNN.0000000000000008

    PubMed  PubMed Central  Article  Google Scholar 

  101. 101.

    Rohrer JD, Crutch SJ, Warrington EK, Warren JD (2010) Progranulin-associated primary progressive aphasia: a distinct phenotype? Neuropsychologia 48:288–297. doi:10.1016/j.neuropsychologia.2009.09.017

    PubMed  PubMed Central  Article  Google Scholar 

  102. 102.

    Rohrer JD, Warren JD, Barnes J et al (2008) Mapping the progression of progranulin-associated frontotemporal lobar degeneration. Nat Clin Pract Neurol 4:455–460. doi:10.1038/ncpneuro0869

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  103. 103.

    Chow ML, Brambati SM, Gorno-Tempini ML et al (2010) Sound naming in neurodegenerative disease. Brain Cogn 72:423–429. doi:10.1016/j.bandc.2009.12.003

    PubMed  PubMed Central  Article  Google Scholar 

  104. 104.

    Ghosh BCP, Calder AJ, Peers PV et al (2012) Social cognitive deficits and their neural correlates in progressive supranuclear palsy. Brain 135:2089–2102. doi:10.1093/brain/aws128

    PubMed  PubMed Central  Article  Google Scholar 

  105. 105.

    Saft C, Schüttke A, Beste C et al (2008) fMRI reveals altered auditory processing in manifest and premanifest Huntington’s disease. Neuropsychologia 46:1279–1289. doi:10.1016/j.neuropsychologia.2007.12.002

    PubMed  Article  Google Scholar 

  106. 106.

    Cheng C-H, Soong B-W, Hsu W-Y, Lin Y-Y (2014) Reduced automatic frontal response to auditory deviance in Huntington’s disease as indexed by magnetic mismatch negativity. J Clin Neurosci 21:1773–1778. doi:10.1016/j.jocn.2014.01.019

    PubMed  Article  Google Scholar 

  107. 107.

    Cope TE, Grube M, Singh B et al (2014) The basal ganglia in perceptual timing: timing performance in Multiple System Atrophy and Huntington’s disease. Neuropsychologia 52:73–81. doi:10.1016/j.neuropsychologia.2013.09.039

    PubMed  PubMed Central  Article  Google Scholar 

  108. 108.

    Lampropoulos CE, Hughes GR (2004) The antiphospholipid (Hughes’) syndrome: changing the face of neurology. Eur J Intern Med 15:147–150. doi:10.1016/j.ejim.2004.01.017

    CAS  PubMed  Article  Google Scholar 

  109. 109.

    Furst M, Levine RA (2015) Hearing disorders in multiple sclerosis. Handb Clin Neurol 129:649–665. doi:10.1016/B978-0-444-62630-1.00036-6

    PubMed  Article  Google Scholar 

  110. 110.

    Gemignani G, Berrettini S, Bruschini P et al (1991) Hearing and vestibular disturbances in Behçet’s syndrome. Ann Otol Rhinol Laryngol 100:459–463

    CAS  PubMed  Article  Google Scholar 

  111. 111.

    Johnson PB, Melbourne-Chambers R, Saindane AM et al (2014) A Case of Neurosarcoidosis with Labyrinthine Involvement. Case Rep Radiol 2014:1–5. doi:10.1155/2014/530431

    Google Scholar 

  112. 112.

    Yurtsever B, Çabalar M, Kaya H et al (2015) A rare cause of hearing loss: Susac syndrome. J Int Adv Otol 11:167–169. doi:10.5152/iao.2015.1002

    PubMed  Article  Google Scholar 

  113. 113.

    Maslan MJ, Graham MD, Flood LM (1985) Cryptococcal meningitis: presentation as sudden deafness. Am J Otol 6:435–437

    CAS  PubMed  Google Scholar 

  114. 114.

    Shotland LI, Mastrioanni MA, Choo DL et al (2003) Audiologic manifestations of patients with post-treatment Lyme disease syndrome. Ear Hear 24:508–517. doi:10.1097/01.AUD.0000100205.25774.5F

    PubMed  Article  Google Scholar 

  115. 115.

    Cassilde AL, Barnaud G, Baccar S et al (2009) Neurosyphilis presenting with dementia, chronic chorioretinitis and adverse reactions to treatment: a case report. Eur Ann Otorhinolaryngol Head Neck Dis 131:389–391. doi:10.1016/j.anorl.2014.02.003

    Article  Google Scholar 

  116. 116.

    Phillips JS, King JA, Chandran S et al (2005) Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) presenting with sudden sensorineural hearing loss. J Laryngol Otol 119:148–151. doi:10.1258/0022215053419880

    PubMed  Google Scholar 

  117. 117.

    Chinnery PF, Elliott C, Green GR et al (2000) The spectrum of hearing loss due to mitochondrial DNA defects. Brain 123:82–92. doi:10.1093/brain/123.1.82

    PubMed  Article  Google Scholar 

  118. 118.

    Baets J, Duan X, Wu Y et al (2015) Defects of mutant DNMT1 are linked to a spectrum of neurological disorders. Brain 138:845–861. doi:10.1093/brain/awv010

    PubMed  PubMed Central  Article  Google Scholar 

  119. 119.

    Djamshidian A, Schaefer J, Haubenberger D et al (2009) A novel mutation in the VCP gene (G157R) in a German family with inclusion-body myopathy with Paget disease of bone and frontotemporal dementia. Muscle Nerve 39:389–391. doi:10.1002/mus.21225

    CAS  PubMed  Article  Google Scholar 

  120. 120.

    King KA, Gordon-Salant S, Yanjanin N et al (2013) Auditory phenotype of Niemann-Pick disease, type C1. Ear Hear 35:110–117. doi:10.1097/AUD.0b013e3182a362b8

    Article  Google Scholar 

  121. 121.

    Blevins G, Macaulay R, Harder S et al (2003) Oculoleptomeningeal amyloidosis in a large kindred with a new transthyretin variant Tyr69His. Neurology 60:1625–1630

    CAS  PubMed  Article  Google Scholar 

  122. 122.

    Bamiou D-E, Spraggs PRD, Gibberd FB et al (2003) Hearing loss in adult Refsum’s disease. Clin Otolaryngol Allied Sci 28:227–230

    PubMed  Article  Google Scholar 

  123. 123.

    Rance G, Fava R, Baldock H et al (2008) Speech perception ability in individuals with Friedreich ataxia. Brain 131:2002–2012. doi:10.1093/brain/awn104

    PubMed  Article  Google Scholar 

  124. 124.

    Middlebrooks JC, Nick HS, Subramony SH et al (2013) Mutation in the kv3.3 voltage-gated potassium channel causing spinocerebellar ataxia 13 disrupts sound-localization mechanisms. PLoS One 8:e76749. doi:10.1371/journal.pone.0076749

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  125. 125.

    Santarelli R (2010) Information from cochlear potentials and genetic mutations helps localize the lesion site in auditory neuropathy. Genome Med 2:91. doi:10.1186/gm212

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  126. 126.

    Salazar R, Cerghet M, Ramachandran V (2014) Bilateral hearing loss heralding sporadic Creutzfeldt-Jakob disease: a case report and literature review. Otol Neurotol 35:1327–1329. doi:10.1097/MAO.0000000000000485

    PubMed  Article  Google Scholar 

  127. 127.

    Fearnley JM, Stevens JM, Rudge P (1995) Superficial siderosis of the central nervous system. Brain 118:1051–1066. doi:10.1093/brain/118.4.1051

    PubMed  Article  Google Scholar 

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Correspondence to Jason D. Warren.

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Conflicts of interest

The Dementia Research Centre is supported by Alzheimer’s Research UK, the Brain Research Trust and the Wolfson Foundation. CJDH holds an MRC PhD Studentship. CRM is funded by a Clinical Research Fellowship from the Leonard Wolfson Experimental Neurology Centre. HLG was supported by an Alzheimer Research UK PhD Fellowship. CNC is supported by The National Brain Appeal—Frontotemporal Dementia Research Fund. JDW received salary support from the Wellcome Trust (Wellcome Trust Senior Clinical Fellowship (091673/Z/10/Z). The authors report no conflicts of interest.

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This work was funded by the Wellcome Trust, the UK Medical Research Council and the NIHR Queen Square Dementia Biomedical Research Unit.

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Hardy, C.J.D., Marshall, C.R., Golden, H.L. et al. Hearing and dementia. J Neurol 263, 2339–2354 (2016). https://doi.org/10.1007/s00415-016-8208-y

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Keywords

  • Hearing
  • Auditory
  • Dementia
  • Alzheimer’s disease
  • Frontotemporal dementia
  • Progressive aphasia
  • Lewy body disease