Presbyacusis – aspekter avseende
epidemiologi och kognition
Ulf Rosenhall
kHz
0.25
0.5
1
0
2
4
8
Hur hör vi i Sverige?
20
30
40
20
Johan sson &
Arlinger, 2002
L,R
60
40
2 epidemiologiska us.
Kvinnor och män, 20 till
90 år gamla
70
70
60
75
80
80
Jönsson &
Rosenhall,
1998a,b, L
85
100
Men, 20 - 90 years
90
kHz
0.25
0.5
1
0
2
4
8
20
30
40
20
50
dB HL
dB HL
50
Johan sson &
Arlinger, 2002
L,R
60
40
70
70
60
75
80
80
85
100
Women, 20-90 years
90
Jönsson &
Rosenhall,
1998a,b, L
Åldershörselnedsättning
Presbyacusis
Age-related hearing loss
ARHL el. ARHI
Förekomst?
Orsaker?
Åtgärder?
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)
kHz
kHz
0.25
0.5
1
2
4
0.25
8
4
8
50
40
1
70
20
dB HL
dB HL
2
60
3
20
60
100
1
0
0
80
0.5
Women, 51.3 y
Women, 70 - 90 y
Left ear
Jönsson & Rosenhall 1998
Hederstierna et al, 2007
2
40
75
79
81
60
85
80
Men, 50 – 60 y
100
Men, 70 - 90 y
Left ear
Rudin et al, 1988
Pedersen et al, 1989
1
88
90
Men, L ear, 70-80/75 years
Men and women, 70 - 80 y
5 epidemiological studies
from Europe and USA
Unscreened populations
kHz
0.25
0.5
1
2
3 4
6 8
10
20
30
G ates et al. 199 0
Davi s 19 95
50
Jöns son & Ros enhall 199 8
60
Crui cks hanks et al. 19 98
70
Engdahl et al . 2005
80
Women, L ear, 70-80/75 years
90
kHz
0.25
0.5
1
2
3 4
6 8
10
20
Remarkable good
agreement between
different studies
from developed
countries
30
G ates et al . 1990
40
dB HL
dB HL
40
Davi s 1995
50
Jöns s on & Rosenhal l 1998
60
Crui cks hanks et al . 1998
70
80
90
Engdahl et al. 2005
Men, L/B ear, 80-90/85 years
k Hz
0.25
0.5
1
2
3 4
6 8
10
20
G ates et al. 1990
40
Parving et al. 1997
50
Jöns son & Ros enhall 1998
Crui cks hanks et al. 1998
60
Hietanen et al. 2004
70
Engdahl et a. 2005
80
Women, L/B ear, 80-90/85 years
90
k Hz
0.25
0.5
1
2
3 4
6 8
10
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
dB HL
dB HL
30
Men and women, 80 - 90 y
6 epidemiological studies
from Europe and USA
Unscreened populations
20
30
G ates et al . 1990
40
Parvi ng et al . 1997
50
Jöns son & Ros enhal l 1998
Crui ckshanks et al. 1998
60
70
80
90
Hietanen et al. 2004
Engdahl et al. 2005
Gender differences
70 - 80 y, 80 - 90 y
Ge n der Di ffe re n ce , 70-80 years
25
20
G ates et al. 1990
Pears on et al. 1995
10
Davis 1995
5
Jönss on & Ros enhall 1998
Cruickshanks et al. 1998
0
Ge n der Di ffe re n ce, 80-90 ye ars
-5
25
-10
0.25
0.5
1
2
3 4
6 8
20
k Hz
G ates et al . 199 0
15
Pears on et al . 199 5
10
Parvi ng et al. 199 7
dB
dB
15
Jönss on & Ros enhall 199 8
5
Cruickshanks et al . 199 8
0
Hietanen et al. 20 04
-5
-10
0.25
0.5
1
2
k Hz
3 4
6 8
Hörselskadade i Sverige
Självskattad hörselnedsättning
(HRF/SCB)
>1 milj.
Beräknat antal med HNS enligt tonaudiometri
20-70 år
Svår HNS – dövhet
Måttlig HNS (M4: 40-64 dB):
Lätt HNS (M4: 20-39 dB):
~130 000
>0,5 milj.
~1,4 milj.
Totalt:
>2 milj.
70+
1/3
2/3
2/3
1/3
Beräkning 2014
Antal svenskar med hörselnedsättning (tusental)
0 – 20 år
20 - 50 år
51 – 70 år
>70 år
Lätt
Måttlig – svår
M4 20-39 dB
M4 ≥40 dB
43t
229t
629t
474t
5t
45t
161t
430t
Sveriges befolkning 31/12 2013
Sveriges befolkning 31/12 2013
Sveriges befolkning 31/12 2013
Ålder, år
60
Beräknat behov,
Hörselrehab
~ 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
Sweden 2006
90y
80y
+70 y
More women than
men have hearing
loss
PTA
+40 dB
70y
60y
PTA
<20 dB
PTA
20-39 dB
PTA
20-39 dB
PTA
<20 dB
50y
Men 50+ 1.6 m
Women 50+ 1.8 m
Vad händer med hörseln på lång sikt?
Är hörseln konstant?
Försämras hörseln?
Förbättras hörseln?
Ökar eller minskar incidensen av hörselproblem?
Den pessimistiska synen:
Hörselproblemen ökar globalt
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
The pessimistic view
In the Alameda County Study prevalence rates of self-reported
trouble with hearing nearly doubled from 1965 to 1994
Wallhagen et al, 1997
Increases in prevalence of hearing loss in adolescents from
15% to 19.5% from 1988–1994 to 2005–2006
NHANES
Shargorodsky et al, 2010
Self-assessed hearing loss 1984 - 2005
Statistics Sweden, SCB
Marke Trak VIII Kochkin, 2005
11.7
16
14
11.3%
10.8%
10
14 %
8
6
4
Men
2
Total
20
05
20
04
-0
3
02
-0
1
00
-9
9
98
-9
7
96
-9
5
94
-9
3
92
-9
1
90
-8
9
88
-8
7
86
-8
5
0
Year
10.2%
9.9%
Women
10.5 %
84
Percent
12
The optimistic view
Prevention
NIHL – prevention
Vaccination programmes
ARHL – life-style factors
”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
The optimistic view
75-year olds over three decades: No audiometric changes
The Gerontological and Geriatric Population
Study in Gothenburg, Sweden
Rosenhall et al, 2013
dB HL 0
dB HL 0
Women 75 Right Ear
10
20
20
30
30
40
50
1RE
40
50
2RE
60
70
80
90
0,25
Men 75 Right Ear
10
1RE
2RE
60
4RE
4RE
70
6RE
80
0,5
1
2
4
6
8
kHz
90
0,25
6RE
0,5
1
2
4
6
8
kHz
Förändras hörseln från generation till generation?
18-åriga mönstrande svenska män
Prevalence any threshold >= 25 dB HL any ear 3,4 or 6 kHz
18
Prevalence, %
16
14
12
10
8
6
4
2
0
1971
1976
1981
1986
1991
1995
2000
2005
Year
Både bättre och sämre
(men mest bättre)
HNS-prevalens 3 - 6 kHz, något öra,
1971 – 2005
Muhr et al, 2016
18-åriga mönstrande svenska män
Lätt, måttlig och svår HNS i diskantområdet
1971 – 2004 (-2010), ett eller båda öronen
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) Sensory
3
2
4
1
2) Neural
uncommon, <1%
PARAN Gates et al, 2000
3) Strial (metabolic)
4) Cochlear conductive
5) Mixed
hypothetical
6) Indeterminate
Schuknecht & Gacek, 1993
Woman 75 y
Sensory type
Schuknecht’s classification
Man 74 y
Engström et al. 1987
Strial type
Schuknecht, 1994
A reduction in the EP is presumed
Presbyacusis Woman 83 y
Strial (metabolic) type
Schuknecht’s classification
Presbyacusis Woman 67 y
Gates & Mills, 2005
Presbyacusis Man 73 y
Mixed: sensory + strial types
Engström et al. 1987
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)
Noise – Unscreened
Occupational Noise - No Noise
Men, 70 y
Men, 75 y (70-80 y)
No Noise v s Occupation al No ise
kHz
0.25
0.5
1
2
3 4
The Göte borg Gerontol. Study
6 8
0
0
10
20
20
dB HL
dB HL
30
40
No Noise
40
Noise +1 5y
50
60
80
Highly scr eened
60
Noise scr eened
70
Unsc ree ned
80
n: 1 37 NN
n: 1 00 N+15y
0.25
0.5
1
2
4
8
kHz
Compilation of 8 studies
Highly screened for otological
disease and noise (green)
Screened for noise only (blue)
Unscreened studies, noise
exposed populations (red)
No noise (blue) vs
occupational noise (red)
H70
Interactions between Noise-Induced Hearing Loss
and ARHL
Additive effect
Noi se and Presbyacusi s
Gates e t al., 20 00
(adopted by ISO 1999)
1,75
1,5
Less-than-additive effect
Annual Decline, dB/y LE
1,25
1
No Noise
Mills et al, 1996; 1998
Noise
0,75
More-than-additive effect
0,5
0,25
Miller et al, 1998
0
0.25
0.5
1
2
3
4
6
8
kHz
Combined effect
Animal research indicates a
sensitising effect of noise
Kujawa & Liberman, 2006
Less ARHI in NIHL-frequencies
(4-6 kHz) Increased ARHI in
neighbouring frequencies, - 2 kHz
Gates et al., 2000
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
Speech in Noise Test
90
Age, Years
%
Speech Perception,
20
80
20
70
30
60
40
50
50
60
40
70
80
30
20
0
10
20
30
40
50
60
70
80
High Frequency Pure Tone Average, dB HL
Bar renäs, Wikstr öm 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
Auditory function in early Alzheimer’s disease
and Mild Cognitive Impairment
Idrizbegovic et al, 2011
Pure tone audiometry
Speech audiometry
PB words in quiet (SPQ)
In noise (SPN) 4 dB S/N
Dichotic digits test (DDT)
136 subjects, mean age:
64 years (range 50-78)
1) AD; 2) MCI;
3) SMC
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
