Hearing acuity in nonagenarians aged 90 and 95 assessed in a home setting using standardized pure-tone audiometry.

OBJECTIVE: Knowledge regarding hearing acuity in the nonagenarian age group is sparse. In this study we aimed to advance our understanding of hearing loss in the 10th decade of life. DESIGN: A cross-sectional study in which standardised hearing measurements were performed during home visits, which included care home facilities and nursing homes to maximise participation. STUDY SAMPLE: Two unselected groups of individuals aged 90 (n = 42) and 95 (n = 49), sampled from the population-based Gothenburg H70 Birth Cohort Studies. RESULTS: 98% of the participants (95% CI [95, 100]) had some degree of hearing loss in their better ear, with 83% (95% CI [73, 89]) having a potentially disabling hearing loss of moderate degree or worse, according to WHO criteria. Furthermore, differences between the two age groups (five years apart) indicate an increasing hearing loss, primarily at frequencies ≥ 2 kHz. CONCLUSION: Hearing loss was present in almost all of the participants in the nonagenarian age group and among a majority of them potentially to a degree that would warrant rehabilitation. Carrying out standardised hearing measurements in a home setting was feasible in this age group and enhanced the representativeness of the study population.

Bilateral hearing loss affected almost all of the individuals in the nonagenarian age group with 8 in 10 having hearing loss of a degree severe enough to warrant intervention or hearing aid prescription.The findings provide valuable insight into hearing acuity among nonagenarians. Many earlier studies were limited to subjective hearing assessments, reviews of medical records and/or screening tests performed by non-audiologists.The final sample size was smaller than initially planned due to the COVID-19 pandemic. However, measures were taken to optimise the representativeness of the study sample.

Accuracy of automated pure-tone audiometry in population-based samples of older adults.

OBJECTIVE: Automated pure-tone audiometry is frequently used in teleaudiology and hearing screening. Given the high prevalence of age-related hearing loss, older adults are an important target population. This study aimed to investigate the accuracy of automated audiometry in older adults, and to examine the influence of test frequency, age, sex, hearing and cognitive status. DESIGN AND STUDY SAMPLE: In a population-based study, two age-homogeneous samples of 70-year-olds (n = 238) and 85-year-olds (n = 114) were tested with automated audiometry in an office using circum-aural headphones and, around 4 weeks later, with manual audiometry conducted to clinical standards. The differences were analysed for individual frequencies (range: 0.25-8 kHz) and pure-tone averages. RESULTS: The mean difference varied across test frequencies and age groups, the overall figure being -0.7 dB (SD = 8.8, p < 0.001), and 68% to 94% of automated thresholds corresponded within ±10 dB of manual thresholds. The poorest accuracy was found at 8 kHz. Age, sex, hearing and cognitive status were not associated with the accuracy (ordinal regression analysis). CONCLUSIONS: Automated audiometry seems to produce accurate assessments of hearing sensitivity in the majority of older adults, but with larger error margins than in younger populations, and is not affected by relevant patient factors associated with old age.

Pathophysiological and Clinical Aspects of Hearing Loss Among 85-Year-Olds.

PURPOSE: This study aimed to identify the prevalence of conductive/mixed and sensorineural hearing loss, with an attempt to differentiate between sensory and neural components in 85-year-olds. METHOD: A comprehensive auditory test protocol, including pure-tone audiometry, speech audiometry, auditory brainstem response (ABR), and distortion product otoacoustic emission (DPOAE), was used to identify different types of hearing loss in 85-year-olds. This study comprised a subsample (n = 125) selected from an unscreened cohort of 85-year-olds born in 1930, within the Gothenburg H70 Birth Cohort Studies in Sweden. RESULTS: Test results were reported descriptively. Sensorineural hearing loss was present in one or both ears in almost all participants (98%), and the majority had absent DPOAEs. Only approximately 6% had additional conductive hearing loss, that is, mixed hearing loss. Approximately 20% of the participants with a pure-tone average at 0.5-4 kHz < 60 dB HL had worse word recognition scores compared with predicted scores by the Speech Intelligibility Index (SII), whereas only two participants were classified with neural dysfunction with the use of ABR. CONCLUSIONS: Sensorineural hearing loss, likely related to outer hair cell loss, was present in the vast majority of 85-year-olds. Conductive/mixed hearing loss appears to be relatively rare in advanced age. Poor word recognition scores in relation to SII-predicted scores were relatively common (20%) in 85-year-olds, whereas auditory neuropathy was only rarely identified (1.6%) by the use of ABR latencies. To explain abnormal word recognition and to identify the neural component of hearing loss among the older-old population, future research should consider factors such as listening effort and cognition among the older-old population.

Hearing Loss and Cognitive Function in Early Old Age: Comparing Subjective and Objective Hearing Measures.

INTRODUCTION: Population-based research has consistently shown that people with hearing loss are at greater risk of cognitive impairment. We aimed to explore the cross-sectional association of both subjective and objective hearing measures with global and domain-specific cognitive function. We also examined the influence of hearing aid use on the relationship. METHODS: A population-based sample (n = 1,105, 52% women) of 70-year-olds that were representative of the inhabitants of the city of Gothenburg, Sweden completed a detailed cognitive examination, pure-tone audiometry, and a questionnaire regarding perceived hearing problems. A subsample (n = 247, 52% women) also completed a test of speech-recognition-in-noise (SPRIN). Multiple linear regression analyses were conducted to explore the association of hearing with cognitive function, adjusting for sex, education, cardiovascular factors, and tinnitus. RESULTS: Global cognitive function was independently associated with the better ear pure-tone average across 0.5-4 kHz (PTA4, ß = -0.13, 95% CI, -0.18, -0.07), the better ear SPRIN score (ß = 0.30, 95% CI, 0.19, 0.40), but not with the self-reported hearing measure (ß = -0.02, 95% CI, -0.07, 0.03). Both verbally loaded and nonverbally loaded tasks, testing a variety of cognitive domains, contributed to the association. Hearing aid users had better global cognitive function than nonusers with equivalent hearing ability. The difference was only significant in the mild hearing loss category. DISCUSSION: In a population-based sample of 70-year-old persons without dementia, poorer hearing was associated with poorer global and domain-specific cognitive function, but only when hearing function was measured objectively and not when self-reported. The speech-in-noise measure showed the strongest association. This highlights the importance of including standardized hearing tests and controlling for hearing status in epidemiological geriatric research. More research is needed on the role that hearing aid use plays in relation to age-related cognitive declines.

Benefit of Higher Maximum Force Output in Bone Anchored Hearing Systems: A Crossover Study.

OBJECTIVE: To investigate how higher maximum force output (MFO) in bone anchored hearing systems (BAHS) affects perceived benefit and the subjective experience of sound as well as hearing outcomes in subjects with mixed hearing loss. STUDY DESIGN: Prospective single-center, randomized crossover design (A-B-A) with within-subject control design. PATIENTS: The study included 19 experienced BAHS users with mixed hearing loss in the fitting range of a standard BAHS. INTERVENTIONS: The study evaluated two sound processors with differing MFOs and sizes-Device A: standard sound processor with a lower MFO and Device B: superpower sound processor with a higher MFO. OUTCOME MEASURES: Speech recognition in noise at different signal to noise ratios, aided thresholds, and questionnaires. RESULTS: Speech recognition test showed significant improvements using Device B compared with Device A at both 78 dB SPL (mean difference: 9%) and 75 dB SPL (mean difference: 12%) (p < 0.05). Moreover, speech, spatial and qualities of hearing scale (SSQ12-C) showed a significantly greater perceived benefit with Device B concerning spatial abilities (mean: 0.5-0.6) (p < 0.05). At the conclusion of the study, 58% of participants chose to keep Device A for further use. The main reasons for this were the size of the sound processor and a more comfortable sound experience. CONCLUSIONS: A BAHS sound processor with a higher MFO leads to improved speech-in-noise performance in loud/noisy listening situations and is perceived as significantly better to process spatial information in daily listening situations. However, the relation between cosmetics and performance is not straightforward, and several factors seem to affect the selection process of BAHS.

Prevalence of hearing loss and need for aural rehabilitation in 85-year-olds: a birth cohort comparison, almost three decades apart.

OBJECTIVE: Many individuals >80 years have difficulties with speech communication due to age-related hearing loss and would benefit from aural rehabilitation. As the proportion of older people increases, there is a need to investigate the prevalence of "disabling hearing loss" to calculate future rehabilitation need. The aims are to determine the prevalence of hearing loss in an unscreened birth cohort of 85-year olds, and to identify differences in audiometric results between two birth cohorts, born 28-29 years apart. DESIGN: This is a population-based, cross-sectional study that is part of the Gothenburg H70 Birth Cohort Studies. STUDY SAMPLE: Hearing thresholds were measured and compared between 85-year olds born in 1930 (n = 286) and 1901-1902 (n = 249). RESULTS: Based on the WHO criteria, the prevalence of "disabling hearing loss" was 45% for men and 43% for women in the latest birth cohort. Hearing thresholds (0.5-4 kHz) for men improved compared with the earlier birth cohort. No such difference was observed for women. CONCLUSION: The prevalence of age-related hearing loss over three decades has decreased among 85-year-old men, but has been retained in women. The improvement for men occurred predominantly in the low-mid frequencies. An increased need for aural rehabilitation is expected due to demographic changes.

Single-Sided Deafness-Outcomes of Three Interventions for Profound Unilateral Sensorineural Hearing Loss: A Randomized Clinical Trial.

OBJECTIVE: A comparison of three interventions for profound unilateral sensorineural hearing loss. STUDY DESIGN: Prospective, crossover randomized clinical trial. PARTICIPANTS: Fifteen participants with profound unilateral sensorineural hearing loss. INTERVENTIONS: Three potential technical interventions were compared: Bone Conduction Device on softband, Contralateral Routing of Signal (CROS), and Remote Microphone . Each intervention was randomly trialed for a period of 3 weeks, separated by a 1 week washout period. OUTCOME MEASURES: Speech in noise recognition test performed under four conditions (lateral noise poorer ear, lateral noise better ear, speech poorer ear, speech better ear). Standardized questionnaires (Abbreviated Profile of Hearing Aid Benefit, Bern Benefit in Single Sided Deafness Questionnaire, and Speech, Spatial, and Other Qualities 12) were used to evaluate amplification benefit at baseline and following each intervention. RESULTS: The use of remote microphone provided the best results in the speech recognition in noise test. A benefit in some signal-to-noise ratios was presented of the CROS over bone conduction device on softband in the Speech Poor Ear condition. On questionnaires of benefit, participants did not rate a particular intervention as significantly better than any other. Following the study, CROS was the intervention preferred by the 8 of 15 participants (53%). The majority of participants (80%) chose to continue with an intervention rather than no treatment. CONCLUSION: The use of all interventions resulted in increased performance in speech recognition in noise and rated higher on subjective benefits in comparison with baseline. People with SSD are a heterogeneous population when considering perceived difficulties. Future research should focus on segmenting the population of SSD depending on factors such as etiology, high frequency loss in the better ear, and age of acquired loss for the poorer ear. This stratification may possibly increase the benefit for the patient in terms of more individual-based clinical routines.

Auditory function and prevalence of specific ear and hearing related pathologies in the general population at age 70.

Objective: To describe the auditory function in early old age in detail based on both psychoacoustic and physiological measures, and to investigate the prevalence of specific audiological and otological pathologies.Design: An unscreened subsample from a population-based geriatric investigation was examined with otoscopy; tympanometry; pure-tone audiometry; word-recognition-in-noise test; distortion-product otoacoustic emissions; and auditory-evoked brainstem responses. Audiometric subtypes and diagnoses were established based on set criteria. The association between word scores and ABR was examined with linear regression analysis.Study Sample: 251 persons aged 70 (113 men, 138 women, born in 1944) that were representative of the inhabitants of the city of Gothenburg.Results: The prevalence of conductive pathology was 2% versus 49% for cochlear and 2% for auditory-neural pathology. Four percent had indeterminate type. Cochlear dysfunction was present in the majority of ears and around 20% performed worse-than-expected on speech testing. Poor performance on the speech in noise test was associated with prolonged interpeak latency interval of ABR waves I-V.Conclusion: Specific otological and audiological pathologies, other than cochlear hearing loss, are rare in the general population at age 70. Additionally, there is subtle evidence of age-related decline of the auditory nerve. Longitudinal follow-up would be of great interest.

Cross-sectional assessment of hearing acuity of an unscreened 85-year-old cohort - Including a 10-year longitudinal study of a sub-sample.

As the proportion of older people increases, it is important to investigate hearing acuity in older individuals and to calculate hearing decline for older ages, using standardised test protocols. The main aim of this study was to determine pure-tone hearing thresholds in an unscreened birth cohort of 85-year-olds born in 1930, living in an industrial Swedish city. A further aim was to describe hearing decline in men and women from 75 to 85 years of age with the aid of longitudinal data. The study was part of the Gothenburg H70 Birth Cohort Studies in Sweden. Hearing thresholds (0.25-8 kHz) were measured using automated pure-tone audiometry for 286 85-year-old participants. A subsample (n = 182) was hearing examined at 75 years of age and studied longitudinally from 75 to 85 years. At age 85 years, men had better hearing at low frequencies but poorer hearing at high frequencies than women. The longitudinal study showed a considerable decline between 75 and 85 years at mid-high frequencies (>1 kHz) and the amount of decline was similar between sexes. The results contribute to the estimation of the future need for hearing health services.

The Gothenburg H70 Birth cohort study 2014-16: design, methods and study population.

To improve health care for older persons, we need to learn more about ageing, e.g. identify protective factors and early markers for diseases. The Gothenburg H70 Birth Cohort Studies (the H70 studies) are multidisciplinary epidemiological studies examining representative birth cohorts of older populations in Gothenburg, Sweden. So far, six birth cohorts of 70-year-olds have been examined over time, and examinations have been virtually identical between studies. This paper describes the study procedures for the baseline examination of the Birth cohort 1944, conducted in 2014-16. In this study, all men and women born 1944 on specific dates, and registered as residents in Gothenburg, were eligible for participation (n = 1839). A total of 1203 (response rate 72.2%; 559 men and 644 women; mean age 70.5 years) agreed to participate in the study. The study comprised sampling of blood and cerebrospinal fluid, psychiatric, cognitive, and physical health examinations, examinations of genetics and family history, use of medications, social factors, functional ability and disability, physical fitness and activity, body composition, lung function, audiological and ophthalmological examinations, diet, brain imaging, as well as a close informant interview, and qualitative studies. As in previous examinations, data collection serves as a basis for future longitudinal follow-up examinations. The research gained from the H70 studies has clinical relevance in relation to prevention, early diagnosis, clinical course, experience of illness, understanding pathogenesis and prognosis. Results will increase our understanding of ageing and inform service development, which may lead to enhanced quality of care for older persons.

Improved hearing in Swedish 70-year olds-a cohort comparison over more than four decades (1971-2014).

Objective: the world population is ageing rapidly. In light of these demographic changes, it is of interest to generate current data regarding the prevalence and characteristics of age-related hearing loss. The purpose of this study was to investigate hearing acuity and the prevalence of hearing loss in a contemporary age-homogenous cohort of old adults, and to assess secular trends in hearing function during the last half-century (1971-2014). Methods: we performed a prospective population-based cohort comparison study of unscreened populations. As part of a geriatric population-based study (H70), a new cohort of 70-year olds (n = 1,135) born in 1944 was tested with computerised automated pure-tone audiometry. The hearing thresholds were compared to three earlier born cohorts of 70-year olds, born in 1901-02 (n = 376), 1906-07 (n = 297) and 1922 (n = 226), respectively. Results: significant improvements in median pure-tone thresholds were seen at several frequencies in both men (range: 5-20 dB, P < 0.01) and women (range: 5-10 dB, P < 0.01). When investigating the effect of birth cohort on hearing in a linear regression, significant trends were found. Men's hearing improved more than women's. The prevalence of hearing loss declined in the study period (1971-2014) from 53 to 28% for men and 37 to 23% for women (P < 0.01). Conclusions: these results indicate that the hearing acuity in Swedish 70-year olds has improved significantly over more than four decades. The largest improvements were seen at 4-6 kHz in men, possibly reflecting a decrease in occupational noise exposure. Further studies are required to pinpoint the reasons for improved hearing-health among older people.

A combination of two truncating mutations in USH2A causes more severe and progressive hearing impairment in Usher syndrome type IIa.

OBJECTIVES: Usher syndrome is an inherited disorder that is characterized by hearing impairment (HI), retinitis pigmentosa, and in some cases vestibular dysfunction. Usher syndrome type IIa is caused by mutations in USH2A. HI in these patients is highly heterogeneous and the present study evaluates the effects of different types of USH2A mutations on the audiometric phenotype. Data from two large centres of expertise on Usher Syndrome in the Netherlands and Sweden were combined in order to create a large combined sample of patients to identify possible genotype-phenotype correlations. DESIGN: A retrospective study on HI in 110 patients (65 Dutch and 45 Swedish) genetically diagnosed with Usher syndrome type IIa. We used methods especially designed for characterizing and testing differences in audiological phenotype between patient subgroups. These methods included Age Related Typical Audiograms (ARTA) and a method to evaluate the difference in the degree of HI developed throughout life between subgroups. RESULTS: Cross-sectional linear regression analysis of last-visit audiograms for the best hearing ear demonstrated a gradual decline of hearing over decades. The congenital level of HI was in the range of 16-33 dB at 0.25-0.5 kHz, and in the range of 51-60 dB at 1-8 kHz. The annual threshold deterioration was in the range of 0.4-0.5 dB/year at 0.25-2 kHz and in the range of 0.7-0.8 dB/year at 4-8 kHz. Patients with two truncating mutations, including homozygotes for the common c.2299delG mutation, developed significantly more severe HI throughout life than patients with one truncating mutation combined with one nontruncating mutation, and patients with two nontruncating mutations. CONCLUSIONS: The results have direct implications for patient counselling in terms of prognosis of hearing and may serve as baseline measures for future (genetic) therapeutic interventions.

Expressivity of hearing loss in cases with Usher syndrome type IIA.

OBJECTIVE: The purpose of this study was to compare the genotype/phenotype relationship between siblings with identical USH2A pathologic mutations and the consequent audiologic phenotypes, in particular degree of hearing loss (HL). Decade audiograms were also compared among two groups of affected subjects with different mutations of USH2A. DESIGN: DNA samples from patients with Usher syndrome type II were analysed. The audiological features of patients and affected siblings with USH2A mutations were also examined to identify genotype-phenotype correlations. STUDY SAMPLE: Genetic and audiometric examinations were performed in 18 subjects from nine families with Usher syndrome type IIA. RESULTS: Three different USH2A mutations were identified in the affected subjects. Both similarities and differences of the auditory phenotype were seen in families with several affected siblings. A variable degree of hearing loss, ranging from mild to profound, was observed among affected subjects. No significant differences in hearing thresholds were found the group of affected subjects with different pathological mutations. CONCLUSIONS: Our results indicate that mutations in the USH2A gene and the resulting phenotype are probably modulated by other variables, such as modifying genes, epigenetics or environmental factors which may be of importance for better understanding the etiology of Usher syndrome.

Hearing performance benefits of a programmable power baha® sound processor with a directional microphone for patients with a mixed hearing loss.

OBJECTIVES: New signal processing technologies have recently become available for Baha® sound processors. These technologies have led to an increase in power and to the implementation of directional microphones. For any new technology, it is important to evaluate the degree of benefit under different listening situations. METHODS: Twenty wearers of the Baha osseointegrated hearing system participated in the investigation. The control sound processor was the Baha Intenso and the test sound processor was the Cochlear™ Baha® BP110power. Performance was evaluated in terms of free-field audibility with narrow band noise stimuli. Speech recognition of monosyllabic phonetically balanced (PB) words in quiet was performed at three intensity settings (50, 65, and 80 dB sound pressure level [SPL]) with materials presented at 0 degrees azimuth. Speech recognition of sentences in noise using the Hearing in Noise Test (HINT) in an adaptive framework was performed with speech from 0 degrees and noise held constant at 65 dB SPL from 180 degrees. Testing was performed in both the omni and directional microphone settings. Loudness growth was assessed in randomly presented 10 dB steps between 30 and 90 dB SPL to narrow band noise stimuli at 500 Hz and 3,000 Hz. RESULTS: The test sound processor had significantly improved high frequency audibility (3,000-8,000 Hz). Speech recognition of PB words in quiet at three different intensity levels (50, 65, and 80 dB SPL) indicated a significant difference in terms of level (P<0.0001) but not for sound processor type (P>0.05). Speech recognition of sentences in noise demonstrated a 2.5 dB signal-to-noise ratio (SNR) improvement in performance for the test sound processor. The directional microphone provided an additional 2.3 dB SNR improvement in speech recognition (P<0.0001). Loudness growth functions demonstrated similar performance, indicating that both sound processors had sufficient headroom and amplification for the required hearing loss. CONCLUSION: The test sound processor demonstrated significant improvements in the most challenging listening situation (speech recognition in noise). The implementation of a directional microphone demonstrated a further potential improvement in hearing performance. Both the control and test sound processors demonstrated good performance in terms of audibility, word recognition in quiet and loudness growth.

Long-term ophthalmic health care in Usher syndrome type I from an ICF perspective.

PURPOSE: The aim was to explore ophthalmic health care in female patients with Usher Syndrome type I (USH I) over 20 years and to evaluate the relationship between the ophthalmic health care and the health state of the patients from a health perspective. METHODS: A retrospective study of records from ophthalmology departments (OD) and low vision clinics (LVC) from 1985 to 2004. Assessment of the reports was performed based on the International Classification of Functioning, Disability and Health (ICF). Findings were analysed by manifest content analysis with ICF as a framework and using four themes: health care system, procedure examinations, patient's functioning and disability and procedure actions. RESULTS: The records of nine female patients (aged 25-39 years, 1985) with USH I were selected from the national database of USH. A great number of notes were collected (OD 344 and LVC 566). Procedure examinations were exclusively oriented towards body structure and function. All patients showed aggravated visual impairment over and above the hearing and vestibular impairment. Procedure actions were oriented towards environmental factors. No correlation was found between procedures performed and patient's experience of disability. CONCLUSIONS: The high degree of resource allocation was not correlated to the patients' impairment. The study indicates that the ophthalmic health care was characterised by inefficiency. This conclusion is very serious because patients very likely face severe disability and emotional difficulties. ICF is ought to be incorporated in ophthalmic health care strategy to improve the health care.

Baha solutions for patients with severe mixed hearing loss.

Patients with a mixed hearing loss present special challenges. The amplification demands of mixed hearing loss can drive powerful digital hearing aids to their limits and introduce distortion through saturation. Conversely, the Baha System effectively bypasses the conductive component and focuses on compensating for the sensorineural component of the hearing loss. Ten patients with a mixed hearing loss participated in the present study. Results indicate that Baha provided significant benefits (p < 0.01) over conventional air conduction hearing instruments across the dimensions of audibility, speech understanding and sound quality. Given the increased output force of the latest Baha instruments, once the conductive component of a severe mixed hearing loss becomes greater than 30 dB, a Baha should be considered and evaluated on audiological grounds alone to provide optimal amplification.

Spectrum of USH2A mutations in Scandinavian patients with Usher syndrome type II.

Usher syndrome type II (USH2) is an autosomal recessive disorder, characterised by moderate to severe high-frequency hearing impairment, normal balance function and progressive visual impairment due to retinitis pigmentosa. Usher syndrome type IIa, the most common subtype, is defined by mutations in the USH2A gene encoding a short and a recently discovered long usherin isoform comprising 21 and 73 exons, respectively. More than 120 different disease-causing mutations have been reported, however, most of the previous reports concern mutations restricted to exons 1-21 of the USH2A gene. To explore the spectrum of USH2A disease-causing mutations among Scandinavian USH2 cases, patients from 118 unrelated families of which 27 previously had been found to carry mutations in exons 1-21 were subjected to extensive DNA sequence analysis of the full size USH2A gene. Altogether, 122 USH2A DNA sequence alterations were identified of which 57 were predicted to be disease-causing, 7 were considered to be of uncertain pathogenicity and 58 were predicted to be benign variants. Of 36 novel pathogenic USH2A mutations 31 were located in exons 22-73, specific to the long isoform. USH2A mutations were identified in 89/118 (75.4%) families. In 79/89 (88.8%) of these families two pathogenic mutations were identified whereas in 10/89 (11.2%) families the second mutation remained unidentified. In 5/118 (4.2%) families the USH phenotype could be explained by mutations in the USH3A gene. The results presented here provide a comprehensive picture of the genetic aetiology of Usher syndrome type IIA in Scandinavia as it is known to date.

Longterm visual prognosis in Usher syndrome types 1 and 2.

PURPOSE: To estimate the age at diagnosis of retinitis pigmentosa and to determine visual acuity deterioration, visual field impairment and the frequency of cataracts in Usher syndrome types 1 and 2. METHODS: We carried out a retrospective study of 328 affected subjects with Usher syndrome types 1 and 2. Study subjects were divided into seven different age groups by decade. Data were analysed using descriptive statistics, general linear model anova and survival analysis. RESULTS: Retinitis pigmentosa was diagnosed significantly earlier in subjects with Usher syndrome type 1 than in those with type 2. Visual acuity was significantly more impaired in affected subjects with Usher syndrome type 1 than in those with type 2 from 50 years of age onwards. Survival analysis revealed a significant difference in visual field loss (