ing loss, dirceted rehabilitation pro Sychological counselling, can help nd and acknowledge their problem. of the first and, most crucial, steps jal rehabili Inclusion of, and ner should also be pro: programme.*” ye individuals with a more severe jred headphones for use with Jer fr rollable telephones and g system such m. Lip-reading , alternative alerti rating pager syste valuable ent mes more severe, a hearing aid eneficial role. There is solid evi- sft to the patient in being fitted An 8 There is also good _ of follow-up and rehabilitative sre maximum benefit and hear- weed as part of the overall care wsiological methods (such as utilize a combination of cog- and sound therapy (including noise generators) and report egion of 60-70% in terms of ion of tinnitus and its sever- ; well ro similar treatment sires psychological treatment fany sufferers are unable to situs is a frequent symptom -nt strategies do seem helpful sther auditory symptoms. 167: NOISE-INDUGED HEARIN' IONS 707 | IG LOSS AND RELATED CONDITI Pursuing a claim . ; tn the clinical ating, once a diagnosis of NIHL js mace the patient may cretask advice about pursving.a claim for damages. There are two options. \ Ii the individual has worked for 10 years or more in one of the preseribed occupations, they may be entitled t0 or olny 1bSs compensation, available since 1974.7 The Mevtimn Toss ix calculated on the basis of the average hear- ing levels at 1kHz, 2kIz and SKI. “tetera wna which equates to a 20% are relatively modest. The entry point is SOdB HL, ability. The disability payments illustration, in 2016 a 20% disability attracted a payment of £33.60 per week and complete deafness (100% disabil- ity) £168. There are many who think this entry point Is too high; it equates to an approximate 38% disability in the ‘Black Book’. A similar scheme has operated for the military pension scheme since 1987. The second and more common option is to pursue a civil claim, where the burden of proof is on the claimant. For such a case to succeed, the claimant must demonstrate ‘on the balance of probabilities’ (i.e. more likely than not) that: + there has been exposure to excessive noise levels the hearing loss has been a consequence of that exposure there was a foreseeable risk of injury from the exposure «the case was brought in time (3 years from the time of awareness). The claimant must retain a solicitor to coordinate the case. Such a solicitor can normally be found through the employee's union or work federation, or through the Citizens Advice Bureau. 1g loss associated with noise for many years, remains ological HL, intratympanic administration of corticosteroids, and the use of antioxidant medication." > Expianation and understanding of these phenomena may also be helped by further research based on our knowledge that certain individuals naturally have ‘tough’ ears when it comes to noise exposure. .nces in our understanding of hair cell regeneration mayoucito HARI LOB AAD REL veo conpmons 769 ye wt he se i pencecal 8 m icidual resents W8h #7 sad an wm al) ‘roan the wba iw eas Ince fairy Trib fapese Slaw n that papaltoryeatron ot eatealy Mynganoed teen seihage tor ypiesd see the NOL eaes nthe eff fenedl and Unber ng ners Remora area hearing Wows Teaves 3 ‘an faverage’ valle fOr OBE ‘assumed NIT The choice of apprort rea to have ation with « control grenu here 18 0bNE acbyent wi a ighly heyy bexter Bearine vores Totessm@king an! Kata vost ing S NIH in b peres inl ea gor as dial TE a reaver exert of TTS. He, gexeened control groups and saesociarion with olds, Tay omc Be agficare Wearing Tass 1 (wih poorer 1 etened control BFOUP Solos, and hearing f ‘while a fess hil peat nearsxoronal for an wi ‘hreshoids) may SURE vidal of that age lf at ged i rece hCATT To che! Kr has almost completely SJreappeared from the UK Ae demign ave vnprovedl ae Tyas basal Yed to 2 mat lee mk ol es ase so inzoxicated individuals may vd? The combination of Indust ed machinery perlormanse A similar situatio has b Sa ee been far fewer ol solvents, and I, noted i also focus on t ‘and hyperacusis Mh processes have arise ard to industria 18 Henderson et 3 ‘often now lai Symptoms of Tvs sgl oartabilty Tuan scepribility al inate situation is COMPS: Hin rthermore, new indasth i A veh animal and human is such as call centres and covery Moreeillance. These have Mieet of other factors an it rough novel and unique Probiens wand patterns Of EXPO aroesculminatingina new Contino acoustic shock rec ycahere have been the problems. Mtladed to eatery ein a younger pOpUlASons expost! ve heat of recreational noise re toveasing, recognition OF ea0Y degenerat casi ther advencivios Hear O™ ee seThis sasied toa renewal of the debate regarding Hye most appropriare control BP for comparison.” THE Screening of tie contyal STouP fectvely removes all NEE rhatagy, seh as Gar SSE Tyead angatiess posit y history. of heating 10S alternative ROE “The net resuNe 183 BFOUD swith better so-called “typical population’ and an ing processes + available to make Jatter point Furthermore, there are OO differ “ch a clinician may be required to make 5 diagnosis: eat setting and ma medic® Althou gree of accuracy required ww often the course runately, there is NO specific tes nosis of NIHL context fe different, it 8 surprising es first) caw influ ugh the former (which often com he course in the Taerer. eis therefore most imporrane rearing thresholds than & possible is pursued BFONP ere far Less SereeMinE MAS raken place. This dis- Breton is exemplified DY Matabases A and B for the OF agnostic process 3S 3g in order to avoid late? inconsistet asaccurate a di igs, At the ¥ery fe clinical settin: ‘and reversals in the medicolegal proces an acknowledgement of the areas of uncertainty 1 For practical red. from a diagnostic assumption thats YFsome help, though, 1" the latter getting isthe requir” of NIHL, the individual will have & hearing, loss © to make palance of probabili- posed of three parts: 3 aqge-relaned componenis? nei Fnduced component nnd, finally, 3 wits adiopa Jegenerative COMPONENT, “phe clinician's task 15 10 che relative conteiburton (itany) which the el HL. There !s the enor J individual suscepnbilty = or simply ere are additional p? grapple when diagnosing val_variability 2” rare and calculate the three Sources:; Jed the age-related seyesociated with hearing loss aa ropathological duced from a Me based on eee ‘pes based 2421 have 21), other authors’ si ite with pathological findings lead: hat this represents useful distinetions trate of age-related hearing loss. a he different types and their The new the most Todk known to date hology was believed to be » longitudinal study" has indicated xed sensory-metabolic phenotypes SE nile ear Middle ear Stitfgning, thy vascularity Arthritic changes and ossification in ossicles and ossicular joints . Year Degeneration of middle ear muscles jtental colapse of ear 9 es oo Calcification of cartilaginots Betergement of pinna sOpporto Stee man Tl os ‘ — FRecuces en Biscreased hair growth brane etal i eeu uae Defining features Loss of hair cells and sustentacular cells® at the basal end of the organ of Corti (Figures 56.3 and 56.4) Degeneration of cochlear nerve neurons Cochlear ganglion cell loss Atrophy of the cochlear stria vascularis ‘L088 of strial tissue in the cochlea Primarity in the apical and middle tums of the o ‘ochlea®* Stitlened cochlear basilar membrane An ncreasein the number of fibrillar layers of the silar membrane® 25.0! the cochlear duct®® existence of a number 0 for age-related hearing } has been the main focus ogy of ARHL, the audit hecome interested in a »0 of cochlear synapses old elevation, Whilst t this in animal models th sibility of novel potential Central nervous s\ Age-related degenerativ system was suggested . related hearing loss by. auditory system is relati 2 significant consequen part the required for the elderly hearing rehabilitation by Central auditory pro and decrements in tests ¢ recognition and discrin Over recent years, ther demonstrated between a tive impairment includi Although a causal relat suggested that behaviou isolation resulting from reduced activation of th ing to decreased cogniti Animal models of ag inbred mice, have shed so logical changes with age mechanisms of age-relate research into the possible tionsand reactiveoxygen: will lead to thedevelopme humans although this prc accounts for GENETICS The genetics of congenita forms of deafness have t Of the responsible genes | Beneties of age-related de poorly defined and no a identified. The available «n detect duplications suse of AD SNHL was ) spanning the T/P gene nlogies now offer sensi: : variants such as copy jolecularbasisof SNUL JR-96, a gene encoding in complementary sit transcriptional repres ¢ other miRNAs may loss. See Table $8.1 for in sequencing tech: becoming more and oduced by NGS is are not well covered, assembled or poor! nilarities in sequences ay be difficult to ana- of sequencing the ively fast, the down- f time and expertise . analysts, molecular echnigues will gradu- 0 produce an accurate he main challenge in of the variants, from 5), insertions/deletion wence of one individ n 70000 and 90000 SNVs are detected in 1d future challenge is sease-causing variant, vel genes with unclear Jd with whole exome 30%; however, this is ield of other genomic ) and array compara- 15-20%). Currently, shenotypes have been of the approximately dicted to exist in the years, a huge number rome will be produced ata will lead 10 better ons and ident OSOMAL DOMINANT NOT N-SYNDROMIG FUNCTIONS OF GE! IN NON-SYNDROMIC SNHL D SNHIL genes encode a variety of pro ‘-ear function, Broadly, the non-sy"~ h known function 6 Non-syndromic A teins essential for inne! Uramic AD deafness-coding genes wit aroey divided into four categories (adapted from Hilger‘ Oe bundle morphogenesis (genes important for the development, structure and function of stereocilia #m the hair cells) \ peoteins of the yrtomkeles ee : 0 Se transport of tons within the “filled cochlea, often fey) > 182, G)ps. GJB6 > : KC] 4, SL_LC2, WEST. val acto genes w&dding proteins which a ing expression of other re important for controll genes, part uring developmeny S GRHL2, cous) (s}esceactol ric proneins {epeetating structural AS inoleculesi: TTA COOH om CONCLUSIONS Variants in 38 genes are currently known to cause none syndromic AD SNHL; 21 loci are still awaiting gene Rjentification together with numerous novel genes yet £0 he uliscovered, New sequencing technologies capable of sequencing the whole genome in a cost-effective manner are now, albeit still imperfect, in full flow and will lead to molecular diagnosis in many non-syndromic AD SNHL Mffected families. Due to high genetic heterogeneity, non- yndromic AD SNHL causing variants are individually rare or private, but collectively they play an important role in the pathogenesis of hearing loss. Molecular genetic diagnosis is of critica management of deafness, since it enables early and ac site clinical diagnosis, better prediction of the course of disease, early intervention essential for language and cognt tive development, genetic counselling, as well as potential development of molecular treatments, As the whole genome Sequence analysis is gradually making its way from research towards the diagnostic domain, clinicians must be aware of . = renetic tests 1 importance for