Ulf Rosenhall

Presbyacusis – aspekter avseende

epidemiologi och kognition

Hur hör vi i Sverige?

2 epidemiologiska us.

Kvinnor och män, 20 till

90 år gamla

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HL

0.25 0.5 1 2 4 8

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85

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Men, 20 - 90 years

Johan sson &Arlinger, 2002L,R

Jönsson &Rosenhall,1998a,b, L

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Johan sson &Arlinger, 2002L,R

Jönsson &Rosenhall,1998a,b, L

Women, 20-90 years

Åldershörselnedsättning

Presbyacusis

Age-related hearing loss

ARHL el. ARHI

Förekomst?

Orsaker?

Åtgärder?

Jönsson & Rosenhall 1998

Hederstierna et al, 2007

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Women, 70 - 90 yLeft ear

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HL

0.25 0.5 1 2 4 8

kHz

90

88

85

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75

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Men, 70 - 90 yLeft ear

Rudin et al, 1988

Pedersen et al, 1989

50

60

Women, 51.3 y Men, 50 – 60 y

Three Swedish Epidemiological Studies

1)The Gerontological and Geriatric Population Study in Gothenburg: H70 (1971-2005)

2) The Study of Men born in 1913 and 1923 (1973)

3) Health and Psychosocial Work Conditions in Middle Aged Women (2005)

1

1

2

3

Men and women, 70 - 80 y

5 epidemiological studies

from Europe and USA

Unscreened populations

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20

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50

60

70

80

90

dB

HL

0.25 0.5 1 2 3 4 6 8

kHz

Engdahl et al. 2005

Cruickshanks et al . 1998

Jönsson & Rosenhal l 1998

Davi s 1995

Gates et al . 1990

Women, L ear, 70-80/75 years

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20

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50

60

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80

90

dB

HL

0.25 0.5 1 2 3 4 6 8

kHz

Engdahl et al . 2005

Cruickshanks et al. 1998

Jönsson & Rosenhall 1998

Davi s 1995

Gates et al. 1990

Men, L ear, 70-80/75 years

Remarkable good

agreement between

different studies

from developed

countries

Men and women, 80 - 90 y

6 epidemiological studies

from Europe and USA

Unscreened populations

10

20

30

40

50

60

70

80

90

dB

HL

0 .25 0.5 1 2 3 4 6 8

kHz

Engdahl et a. 2005

Hietanen et al. 2004

Cruickshanks et al. 1998

Jönsson & Rosenhall 1998

Parving et al. 1997

Gates et al. 1990

Men, L/B ear, 80-90/85 years

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20

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50

60

70

80

90

dB

HL

0.25 0.5 1 2 3 4 6 8

kHz

Engdahl et al. 2005

Hietanen et al. 2004

Cruickshanks et al. 1998

Jönsson & Rosenhal l 1998

Parving et al . 1997

Gates et al . 1990

Women, L/B ear, 80-90/85 years

The prevalence of child and adult

hearing impairment is substantially

higher in middle- and low-income

countries than in high-income

countries, demonstrating

the global need for attention to

hearing impairment

Stevens et al, 2013

-10

-5

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5

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25

dB

0.25 0.5 1 2 3 4 6 8

kHz

Ge nder Di ffe re nce , 70-80 years

Cruickshanks et al. 1998

Jönsson & Rosenhall 1998

Davis 1995

Pearson et al. 1995

Gates et al. 1990

-10

-5

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5

10

15

20

25

dB

0.25 0.5 1 2 3 4 6 8

kHz

Ge nder Di ffe re nce, 80-90 ye ars

Hietanen et al. 2004

Cruickshanks et al . 1998

Jönsson & Rosenhall 1998

Parving et al. 1997

Pearson et al . 1995

Gates et al . 1990

Gender differences

70 - 80 y, 80 - 90 y

Hörselskadade i Sverige

Självskattad hörselnedsättning >1 milj.

(HRF/SCB)

Beräknat antal med HNS enligt tonaudiometri

Svår HNS – dövhet ~130 000

Måttlig HNS (M4: 40-64 dB): >0,5 milj.

Lätt HNS (M4: 20-39 dB): ~1,4 milj.

Totalt: >2 milj.

20-70 år 70+

1/3 2/3

2/3 1/3

Beräkning 2014

Antal svenskar med hörselnedsättning (tusental)

Lätt Måttlig – svår

M4 20-39 dB M4 ≥40 dB

0 – 20 år 43t 5t

20 - 50 år 229t 45t

51 – 70 år 629t 161t

>70 år 474t 430t

Sveriges befolkning 31/12 2013

Sveriges befolkning 31/12 2013

Sveriges befolkning 31/12 2013

Ålder, år Beräknat behov,

Hörselrehab

60 ~ 5%

70 10 – 20%

80 40 – 45%

90 60 – 70%

Andel av befolkningen i Sverige 2009 2015 2060

65+ 15% 17% 25%

80+ 4% 9%

65+ 2,7 milj. 2060

50y

60y

70y

80y

90y

PTA

<20 dB

PTA

<20 dB

PTA

20-39 dB

PTA

20-39 dB

PTA

+40 dB

Men 50+ 1.6 m Women 50+ 1.8 m

Sweden 2006

+70 y

More women than

men have hearing

loss

Vad händer med hörseln på lång sikt?

Är hörseln konstant?

Försämras hörseln?

Förbättras hörseln?

WHO 2002 - 2030

DALYs (Disability-Adjusted Life Years)

Adult onset hearing loss: 2002 ranking # 13 globally

Year 2030 estimated ranking # 9 (2.5 DALYs)

High-income countries # 7 (4,1 DALYs)

Middle-income countries # 9 (2,9 DALYs)

Low-income countries < #10

Mathers & Loncar, 2006

Ökar eller minskar incidensen av hörselproblem?

Den pessimistiska synen:

Hörselproblemen ökar globalt

The pessimistic view

11.7

11.3%

10.8%

10.2%

9.9%

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16

Per

cen

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84-8

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86-8

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88-8

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90-9

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92-9

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94-9

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96-9

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98-9

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00-0

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02-0

320

0420

05

Year

Self-assessed hearing loss 1984 - 2005 Statistics Sweden, SCB

Total

Men

Women10.5 %

14 %

Marke Trak VIII Kochkin, 2005

Increases in prevalence of hearing loss in adolescents from

15% to 19.5% from 1988–1994 to 2005–2006

NHANES Shargorodsky et al, 2010

In the Alameda County Study prevalence rates of self-reported

trouble with hearing nearly doubled from 1965 to 1994

Wallhagen et al, 1997

Prevention

NIHL – prevention

Vaccination programmes

ARHL – life-style factors

The optimistic view

”Americans hear as well or better

today compared with 40 years ago”

NHANES, NHES Hoffman et al, 2010

Persons from later birth cohorts had lower

prevalences of hearing impairment than those

from earlier birth cohorts

Beaver Dam Study, EHLS Zhan et al, 2010

Men 75 Right Ear

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1RE

2RE

4RE

6RE

Women 75 Right Ear

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90

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1RE

2RE

4RE

6RE

75-year olds over three decades: No audiometric changes

The Gerontological and Geriatric Population

Study in Gothenburg, Sweden Rosenhall et al, 2013

The optimistic view

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18

1971 1976 1981 1986 1991 1995 2000 2005

Pre

va

len

ce

, %

Year

Prevalence any threshold >= 25 dB HL any ear 3,4 or 6 kHz

HNS-prevalens 3 - 6 kHz, något öra,

1971 – 2005

18-åriga mönstrande svenska män

Förändras hörseln från generation till generation?

Både bättre och sämre

(men mest bättre)

Muhr et al, 2016

Lätt, måttlig och svår HNS i diskantområdet

1971 – 2004 (-2010), ett eller båda öronen

18-åriga mönstrande svenska män

Histopathology of ARHL

Cochlear degeneration

• OHC degeneration in basal and apical coil

• IHC degeneration in basal coil

• Strial degeneration

• Alterations and derangement of hair bundles

• Intracellular inclusions

Types of ARHL

1

1) Sensory

2

2) Neural 3

3) Strial (metabolic)

4 4) Cochlear conductive

5) Mixed

6) Indeterminate

Schuknecht & Gacek, 1993

hypothetical

uncommon, <1%

PARAN Gates et al, 2000

Engström et al. 1987

Woman 75 y

Man 74 y

Sensory type

Schuknecht’s classification

Strial type

Schuknecht, 1994

A reduction in the EP is presumed

Presbyacusis Woman 67 y

Presbyacusis Woman 83 y

Strial (metabolic) type

Schuknecht’s classification

Gates & Mills, 2005

Engström et al. 1987

Presbyacusis Man 73 y

Mixed: sensory + strial types

Age, Cohort, and Period Effects

Causes of ARHL

Intrinsic causes – Age, Cohort Effects

Biological ageing

Biological ageing is probably only a minor

contributor to ARHL of ”younger elderly people”,

but increases in importance in advanced age, 80+

Telomeres?

Genetic factors

Important contributors to ARHL

Mutation in mtDNA

The 4,977-bp deletion

Bai et al, 1997; Ueda et al, 1998

Genetic influences of ARHL in man

• ARHL has a multifactorial aetiology

• Familial aggregations occur for sensory and strial ARHL

phenotypes

• Heritability: 35-55% (sensory type)

• The heritability estimate was greater for the strial than the sensory

phenotypes

• Women: Genetic effect on ARHL

• Men: Mixed, genetically/aquired ARHL

Gates et al, 1999

• Candidate genes in man:

10q26, 11q13.5, 11q25 (DFNB20), 11p, 14q, 18q

KCNQ4, DFNA18

DeStefano et al, 2003; Fransen et al, 2003;

Garringer et al, 2006; van Eyken et al, 2006; a.o.

Genetics and ARHL – Twin Studies

Audiometric Study of Male Twins

Variation in high frequency hearing is related

to genetic and extrinsic factor. Heritability: 47%, 64+ years, men

The environmental effect becomes more important with age

Karlsson et al, 1997

Danish Twin Registry

Heritability for self-reported hearing loss 40%, 75+ years

Christensen et al, 2001

NAS-NRC Twin Panel

Heritability: 60% (self-reported). HI susceptibility locus on chromosome

3, DFNA18 locus Reed et al, 2000; Garringer et al, 2006

Finnish Twin Study on Aging

Heritability: >60%, measured hearing, 63-76 years, women

Self-reported hearing related to environmental factors

Viljanen et al, 2007

Causes of ARHL

Environmental, acquired

Period Effects

Vocational noise exposure

Ototoxic drugs

Solvents, other chemicals

e.g. carbon monoxide

Traumatic hearing loss

Infections

Middle ear disease

Heavy metals (Hg)

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40

60

80

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HL

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Unsc ree ned

Noise scr eened

Highly scr eened

Noise – Unscreened

Men, 75 y (70-80 y)

Compilation of 8 studies

Highly screened for otological

disease and noise (green)

Screened for noise only (blue)

Unscreened studies, noise

exposed populations (red)

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10

20

30

40

50

60

70

80

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HL

0.25 0.5 1 2 4 8

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No Noise v s Occupational Noise

The Göteborg Gerontol. Study

Noise +15y

No Noise

n: 137 NN

n: 100 N+15y

Occupational Noise - No Noise

Men, 70 y

No noise (blue) vs

occupational noise (red)

H70

Interactions between Noise-Induced Hearing Loss

and ARHL

Additive effect

(adopted by ISO 1999)

Less-than-additive effect

Mills et al, 1996; 1998

More-than-additive effect

Miller et al, 1998

Combined effect

Less ARHI in NIHL-frequencies

(4-6 kHz) Increased ARHI in

neighbouring frequencies, - 2 kHz

Gates et al., 2000

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0,25

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0,75

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1,5

1,75

Ann

ual D

eclin

e, d

B/y

LE

0.25 0.5 1 2 3 4 6 8

kHz

Noise and Presbyacusis

Gates e t al., 2000

Noise

No Noise

Animal research indicates a

sensitising effect of noise

Kujawa & Liberman, 2006

Annual decline dB/year

Three longitudinal studies

H70 Noise vs No Noise

Hederstierna, Rosenhall, 2015

BLSA Noise vs No Noise

Pearson et al, 1995

MUSC No Noise

Lee et al, 2005

Causes of ARHL

Environmental, extrinsic

Life style factors

Every day noise exposure

Leisure time noise exposure, e.g.

music, shooting

Smoking

Alcohol (severe abuse)

Diet

Physical fitness

Interfererande sjukdomar

Kognitiva störningar – Demens

Metabolt syndrom – DM2

AD:

3 – 10% - 65 y

25 – 50% >85 y

Hörsel och kognition hos äldre

Presbyacusis – interaktion med kognitiva störningar

Lin et al, 2013; Lin & Albert, 2014

Positiv effekt av hörselrehabilitering vid

presbyacusis och demens

Amieva et al, 2016

20

30

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80

90

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High Frequency Pure Tone Average, dB HL

Speech in Noise Test

80

70

60

50

40

30

20

Age, YearsS

pe

ech

Pe

rcep

tion

, %

Barrenäs, Wikström 2000

Magnusson, 1996

Hörsel och kognition hos äldre

Centrala auditiva störningar

Central Auditory Processing Disorder (CAPD)

CAPD har påvisats vid demens och vid impressiv afasi

Gates et al, 1996, 2002, 2008; Idrizbegovic et al, 2011; 2013

Ramsing et al, 1996

Pure tone audiometry

Speech audiometry

PB words in quiet (SPQ)

In noise (SPN) 4 dB S/N

Dichotic digits test (DDT)

Central Auditory Processing (CAP) dysfunction

is highly evident in early AD, and even in MCI

Evaluation of CAP might provide an auditory

diagnostic complement in monitoring the

progression of AD and MCI

Auditory function in early Alzheimer’s disease

and Mild Cognitive Impairment

Idrizbegovic et al, 2011

136 subjects, mean age:

64 years (range 50-78)

1) AD; 2) MCI;

3) SMC