Auditory Acuity Research Articles

Auditory steady-state responses to multiple simultaneous stimuli in children with functional or sensorineural hearing loss

The goal of our study was to identify the role of auditory steady-state responses for hearing assessment in patients with functional hearing loss. The study design was to compare auditory steady-state response thresholds and standard pure-tone audiometry thresholds between patients with functional or sensorineural hearing loss. Subjects comprised 16 patients (24 ears) with functional hearing loss and 17 patients (24 ears) with sensorineural hearing loss. Differences and correlations between auditory steady-state response thresholds and standard pure-tone audiometry thresholds at 500, 1,000, 2,000 and 4,000 Hz were evaluated. In children with functional hearing loss, pure-tone audiometry thresholds and auditory steady-state response thresholds were significantly different at all frequencies and were not significantly correlated. In patients with sensorineural hearing loss, pure-tone audiometry thresholds and auditory steady-state response thresholds did not differ significantly at any frequencies and were significantly correlated. Auditory steady-state responses may have principal role in the assessment of auditory brainstem acuity, particularly at low frequencies in patients with functional hearing loss.

Perfil audiométrico de trabalhadores do distrito industrial de Maracanaú - CE

OBJETIVO: Estabelecer o perfil audiométrico de trabalhadores expostos ao ruído, acima do nível de ação (85 dB), em empresas do Distrito Industrial de Maracanaú, Ceará. MÉTODOS: Foi realizado um estudo descritivo de prevalência a partir da avaliação de dados de exames audiométricos, coletados de arquivos das empresas pesquisadas realizados no período de abril a outubro de 2006. Foram avaliados 5372 trabalhadores das 47 (52,80%) empresas que autorizaram a pesquisa e as variáveis analisadas foram: faixa etária, tempo total de exposição ao ruído, estado da acuidade auditiva, tipo de perda auditiva, lateralidade da perda auditiva e configuração audiométrica sugestiva de perda auditiva induzida por ruído nos trabalhadores e setor da atividade econômica e porte da empresa. RESULTADOS: Constatou-se que 19,00% da população estudada apresentaram alteração, predominando a perda auditiva sensório neural em 90,67% dos casos. Em 12,71% da população estudada observou-se quadro sugestivo de perda auditiva induzida por ruído (PAIR) e, em 71,77% da população portadora de perda auditiva sensório neural, há indícios de PAIR. Em relação à lateralidade da perda, 39,09% é unilateral, sendo 24,03% unilateral esquerda. CONCLUSÕES: Apesar dos resultados não representarem a prevalência total de perdas auditivas do Distrito, eles apontam para a necessidade não apenas de realização de audiometrias ocupacionais pontuais, mas também de monitoramento da audição dos trabalhadores de forma longitudinal, como parte de um programa de conservação auditiva.

Miglustat for treatment of Niemann-Pick C disease: a randomised controlled study

Niemann-Pick type C disease (NPC) is an inherited neurodegenerative disorder characterised by an intracellular lipid-trafficking defect with secondary accumulation of glycosphingolipids. Miglustat, a small iminosugar, reversibly inhibits glucosylceramide synthase, which catalyses the first committed step of glycosphingolipid synthesis. Miglustat is able to cross the blood-brain barrier, and is thus a potential therapy for neurological diseases. We aimed to establish the effect of miglustat on several markers of NPC severity. Patients aged 12 years or older who had NPC (n=29) were randomly assigned to receive either miglustat 200 mg three times a day (n=20) or standard care (n=9) for 12 months. 12 children younger than 12 years of age were included in an additional cohort; all received miglustat at a dose adjusted for body surface area. All participants were then treated with miglustat for an additional year in an extension study. The primary endpoint was horizontal saccadic eye movement (HSEM) velocity, based on its correlation with disease progression. This study is registered as an International Standard Randomised Controlled Trial, number ISRCTN26761144. At 12 months, HSEM velocity had improved in patients treated with miglustat versus those receiving standard care; results were significant when patients taking benzodiazepines were excluded (p=0.028). Children showed an improvement in HSEM velocity of similar size at 12 months. Improvement in swallowing capacity, stable auditory acuity, and a slower deterioration in ambulatory index were also seen in treated patients older than 12 years. The safety and tolerability of miglustat 200 mg three times a day in study participants was consistent with previous trials in type I Gaucher disease, where half this dose was used. Miglustat improves or stabilises several clinically relevant markers of NPC. This is the first agent studied in NPC for which there is both animal and clinical data supporting a disease modifying benefit.

Auditory Spatial Acuity Approximates the Resolving Power of Space-Specific Neurons

The relationship between neuronal acuity and behavioral performance was assessed in the barn owl (Tyto alba), a nocturnal raptor renowned for its ability to localize sounds and for the topographic representation of auditory space found in the midbrain. We measured discrimination of sound-source separation using a newly developed procedure involving the habituation and recovery of the pupillary dilation response. The smallest discriminable change of source location was found to be about two times finer in azimuth than in elevation. Recordings from neurons in its midbrain space map revealed that their spatial tuning, like the spatial discrimination behavior, was also better in azimuth than in elevation by a factor of about two. Because the PDR behavioral assay is mediated by the same circuitry whether discrimination is assessed in azimuth or in elevation, this difference in vertical and horizontal acuity is likely to reflect a true difference in sensory resolution, without additional confounding effects of differences in motor performance in the two dimensions. Our results, therefore, are consistent with the hypothesis that the acuity of the midbrain space map determines auditory spatial discrimination.

Falls in nonagenarians after 1-year of follow-up: The NonaSantfeliu study

An increase in the rate of falls may be an indicator of frailty. This study included a 12-month follow-up investigation into the rate of falls, in people over 89 years, living in an urban community and analyzed the differences between inhabitants with falls and those without falls. The study was conducted within the framework of the NonaSantfeliu study and 140 nonagenarians participated. Sociodemograhic data, Barthel index (BI), activities of daily living (ADL), Spanish version (MEC) of the mini mental state examination (MMSE), the mini nutritional assessment (MNA) questionnaire, near visual acuity by Snellen test and auditory acuity with the Whisper test were evaluated. The fall rate was 47.1%. The 1-year incidence of falls was 26.4%. The incidence of recurrent falls (two or more falls per year) was 10% ( n = 14). The prevalence of previous falls within the year preceding the study was 45.7%, 64 of 140 nonagenarians fell and 17 (26.5%) of them fell again during the follow-up. Seventy-six out of 140 (54.3%) nonagenarians had not fallen during the year prior to the study and during the year of follow-up, 20 (26.3%) of them had a new fall. In conclusion, the rate of falls among nonagenarians is high. These results emphasize the need to increase the awareness, to provide recommendations and to incorporate strategies to prevent falls.

Music Therapy and Hearing Loss: A 30-Year Retrospective

ABSTRACT: This article focuses on past, present, and future trends regarding music therapy and hearing losses. The article first reflects back on the policies, clinical practices, and research findings of the last three decades and then notes societal and scientific factors that have lead to current-day knowledge and clinical approaches. Finally, future directions for research and clinical practice will be discussed. In 1976, the United States was awash in red, white, and blue as Americans celebrated our country's Bicentennial, the 200th anniversary of the Declaration of Independence, which espoused equality and unalienable rights for all. How appropriate that such a year should also mark initial implementation of PL94-142, the Education for All Handicapped Children Act of 1975. This legislation called for free and appropriate education for children with disabilities in the least restrictive environment. PL94-142 has had an enormous impact on the education, rehabilitation, and the social integration of children with disabilities, including those who have significant hearing losses. Prior to 1976, many children with significant hearing losses were educated in residential schools for the deaf. These institutions were homes away from home for many children, in which they were acculturated into the communicative (e.g., oral or manual communication), social, and educational norms of the school. In some schools, the primary mode of communication was manual-American Sign Language; in others, oralism (the use of spoken communication) and optimal use of residual hearing were promoted. Although one might assume that the choice of manual or oral communication would emanate primarily from the hearing profile of the child (e.g., onset and severity of deafness), the decision of sign language or speech has often been based upon socio-cultural or political factors. For example, deaf parents may promote manual communication, while hearing parents may support spoken communication; deaf educators and audiologists have often endorsed the philosophy they learned in their own professional training. The voices of those with vested interests, along with political or even religious movements, have shaped the policies of state schools for the deaf regarding manual or oral communication (Baynton, 1996). The history of deaf education in the United States has been characterized by on-going and vigorous debates regarding manual vs. oral communication (Baynton, 1996; Darrow, 2005). Because music is an auditory form of communication, the place of music in the lives of persons with hearing losses has been a part of this debate. Historical records note the presence of music programs in some schools of the deaf as early as 1840 (Darrow & Heller, 1985). By 1976, clinical resources had been published describing music curricula and instructional methods for children who were deaf. Some articles suggested that music instruction might also enhance spoken communication. Music action songs and rhythmic exercises were recommended for improving body image, body vocabulary, socialization, speech production, and auditory acuity (e.g., Epley, 1972; Fahey & Birkenshaw, 1972; Galloway & Bean, 1974; Gilmore, 1966; Hummel, 1971; McDermott, 1971; Riordan, 1971; Stern, 1975; Vettese, 1974). Prior to 1976, these resources tended to be primarily curriculum-oriented or anecdotal accounts of personal experience (Darrow, 1987b). A handful of articles from that era included empirical studies on music perception which could inform music educators in terms of suitable instructional objectives (e.g., Korduba, 1975; Madsen & Mears, 1965; Raleigh & Odom, 1972). 1976-1986: The First Decade Following PL94-142 In the decade following the passage of PL94-142, there was a gradual shift in instructional placement with increasingly fewer children being educated in schools for the deaf. As more children enrolled in regular classes within their home school districts, music educators faced the challenge of making an auditory art form meaningful to children with significant hearing losses. …

CLASSIFICATION AND REGRESSION TREE ANALYSIS OF A NEUROLOGICALLY IMPAIRED AND NORMAL SAMPLE USING SENSORY-MOTOR TASKS

The ability to differentiate between neurologically impaired and normal individuals is an important component in a valid neuropsychological battery. However, limited research exists regarding the ability of sensory-motor batteries to differentiate between the two groups. This study used Classification and Regression Tree Analysis (CART) to identify which measures of sensory-motor functioning from the Dean-Woodcock Sensory Motor Battery (DWSMB) would best differentiate between neurologically impaired and normal individuals, as well as identify which subtests would provide the best pathognomic power. The results revealed that a number of clinically useful nodes emerged that enabled the differentiation between groups with a small number of tasks. The primary separation variable was the Gait and Station subtest, a measure of subcortical motor functioning. Auditory Acuity and Clock Construction also provide important pathognomic information. A cross validation was conducted to determine the integrity of the generated decision tree, and results revealed that the generated model correctly predicted 84.5% of the normal group and 71.4% of the neurologically impaired sample. The results from the present analysis provides further evidence for the construct validity of the DWSMB.

Chimp Facial Action Coding: An Important Forward Brachiation in Method

(1872) The Expression of the Emotions in Man and Animals. This work not only helped establish the conceptual basis of nonverbal behavior and communication but also sketched out the comparative method. In turn, comparing species on structure and function has played a key role in the development of the fields of physiology, neuroanatomy, ethology, and others, especially including nonverbal communication. One of the key benefits of cross-species comparisons is of course in providing insight into the functional value of particular communicative behavior (Marler & Vandenbergh, 1979). Animals living dense forests or dark waters or who are nocturnal are more likely to find their mates and alert their conspecifics with calls and other auditory cues than they are to rely on visual communication. There is thus also a significant selected increase in auditory acuity. Dogs and wolves, highly territorial and with their noses near the ground, use urine markers. Homo sapiens, with generally excellent visual acuity, detect their mates and potential aggressors mostly with visual cues rather than through sniffing. Such gross comparisons, tremendously important over the past century (Ekman, 1973), have become increasingly refined as particular nonverbal movements are better studied. Progress and refinements in the study of nonverbal behavior have often depended on methodological advances. Use of film and video, slow motion, the point-light technique, sound recordings and spectrograms, as well as statistical advances in coding and judgments have led to huge swings forward in understanding. Notably, in the area of facial behavior, the Facial Action Coding System (FACS), developed by Paul Ekman and colleagues, provided a standardized, neuro-anatomically based technique for the reliable coding and analysis of facial movement and expression (Ekman, Friesen, & Hager, 2002; Ekman & Rosenberg, 2005). The paper by Vick, Waller, Parr, Smith Pasqualini, and Bard (2007) in this issue describes a modification of the FACS for describing chimpanzee facial movement.

Reduced auditory acuity in rat pups from excess and deficient omega-3 fatty acid consumption by the mother

Consumption of the nutrients omega-3 fatty acids (ω-3 FA) during pregnancy and lactation is considered beneficial to fetal and infant development. It may also reduce the incidence and severity of preterm births by prolonging gestational length. However several recent human and animal studies have reported that over-supplementation with ω-3 FA, especially in the form of fish oil, can have adverse effects on fetal and infant development and the auditory brainstem response (ABR). Our goal was to assess further the effects of ω-3 FA excess and deficiency during pregnancy and lactation on the offspring's auditory acuity as evidenced by their ABR thresholds. Female Wistar rats were given diets that were either deficient, adequate (control) or excess in ω-3 FA from day 1 of pregnancy through lactation. The offspring were ABR-tested at the postnatal age of 24 days. The rat pups in the Excess treatment condition had significantly elevated (worse) ABR thresholds, postnatal growth restriction, and a trend for increased postnatal mortality in comparison to the Control group. The Deficient group was intermediate. In conclusion, excess or deficient amounts of ω-3 FA during pregnancy and lactation in the laboratory rat adversely affected the offspring's auditory acuity. Postnatal thriving was also adversely affected. Consuming or administering large or inadequate amounts of ω-3 FA during pregnancy and lactation seems inadvisable because of the potential for adverse effects on infant development.

Relación entre la pérdida sensorial y la capacidad funcional en personas mayores de 89 años. Estudio NonaSantfeliu

Objective alterations in the sense organs are frequent in the elderly. Our aim was to analyze the status of visual function and hearing in nonagenarians in order to evaluate the relationship between sensory impairment and the ability to perform basic or instrumental activities of daily life. Material and methods data were obtained from the NonaSantfeliu population-based study: sociodemograhic variables were collected. Functional status was determined by the Lawton-Brody Index (LI) and Barthel Index (BI) and cognition by the Spanish version of the Mental State Examination (Miniexamen cognitivo de Lobo [MEC]). Comorbidity was evaluated using the Charlson Index. Near visual acuity was evaluated by the Snellen test, and auditory acuity was assessed with the whisper test. Results the sample was composed of 186 nonagerians, with a mean age of 93.06 (3.1) years; 76.5% were women. Seventy-four percent lived at home and the remainder lived in nursing homes. Visual impairment was found in 38%, and hearing impairment in 40%. Combined visual and hearing impairment was found in 18% and devices to correct both impairments were required in 5.3%. Nonagenarians with visual or combined impairment had poor LI, BI and MEC values. In multivariate analyses a lower LI was associated with visual impairment (p < 0.001, odds ratio [OR] = 1.859, 95% confidence interval [CI] 1.441-2.398) and combined impairment (p < 0.001, OR = 1.995, 95% CI 1.320-3.016). Conclusions impairment of vision and hearing ability are common in nonagenarians. The ability to perform instrumental activities of daily life is decreased due to visual and combined sensory impairment in this population.

Evaluación de las caídas en las personas mayores de 89 años institucionalizadas. Estudio NonaSantfeliu

We studied the prevalence of falls in nonagenarians living in a nursing home and compared the characteristics of fallers with those of non-fallers. Forty-nine 49 institutionalized nonagenarians (89.7% women) were studied. Sociodemograhic data, the ability to perform basic and instrumental activities of daily living, cognitive status, and comorbidity were evaluated. Visual and auditory acuity were also assessed. Twenty-six patients (53.1%) had fallen at least once in the previous year. No significant differences were found between fallers and non-fallers. Because of the high percentage of falls in institutionalised nonagerians, preventive measures should be intensified in this group.

Hoc alterum auditus organi ossiculum est: Ear Ossicles in Physical Anthropology

In spite of the importance of auditory ossicles in anthropological studies, very little attention has been paid to these bones, which are only rarely recovered in archaeological excavations and in osteological collections. In this paper, we attempt to review some studies that started in the 1960 from when we first collected and prepared ossicles from the early Egyptian osteological collection of Giovanni Marro in Turin (Italy). We attempt to delineate the potential of ear bones in the study of man. In a few cases, archaeological ossicles were discussed in connection with some forms of pathology. Other studies examined the phylogeny of primates based on auditory ossicles. The function of the ossicles is to transmit sound waves to the cochlear endolymph. The energy transmitted through the ossicles is conserved by the action of two levers, which convert the relatively wide and low-energy movements of the hammer into smaller high-energy movements of the stirrup. It is a matter of argument whether the different proportions of the ossicles may imply variations in auditory perception in primates, including man. While the hammer of modern humans and that of the great apes show similar absolute sizes, nevertheless, the ape manubrium is appreciably greater than that of man. This difference, combined with stirrup proportions, causes a strong “low-gear” effect in apes and, as a consequence, probably a different auditory acuity. Although only a few fossil Neanderthal auditory ossicles are known, it may be, nevertheless, of interest studying the ossicles from the viewpoint of vibration transfer impedance function. The methodology may also be appropriate to study the few Australopithecine ossicles that are known. It is a matter of question whether the ossicles may have a meaning in distinguishing human populations; nevertheless, at least a case of clear distinction between human population has been achieved as in the case of Antinoe Roman–Egyptians.

Being on Guard: Intelligent Virtual Agents Reporting Information from Collaborative Virtual Environments

An important property of intelligent virtual agents (IVAs) and multiintelligent virtual agents systems (mIVAS) is their capability to acquire/perceive information from their environment. In this paper we present an auditory perceptual model for IVAs and mIVAS, which extends and reinterprets the key concepts of one of the awareness models developed for computer supported cooperative work (CSCW) applications, the spatial model of interaction (SMI). This perceptual model also introduces some factors typical of human perception—such as auditory acuity—as well as others associated with the sound source and its propagation with the aim of making perception in this kind of system more humanlike.

Estimating loudness in animals

Although auditory acuity experiments in animals are valuable, this is not how animals typically encounter real world sounds. It is also important to know how animals respond to stimuli at more ‘‘realistic’’ intensities. The perception of above-threshold level sounds has been well documented in humans, but is much less studied in animals, where acoustic communication is important for survival. In humans, equal loudness contours are used to show that loudness does not increase with intensity equally at all frequencies. Loudness is a subjective experience, however, and much harder to measure in animals. Reaction time is one untrained response that can be used to gauge how different or similar two stimuli are: high intensities yield short and low intensities yield long reaction times. Comparing reaction times across frequencies (equal latency contours) in birds and mammals show results that are similar to equal loudness contours obtained in humans. At low SPLs equal-latency contours closely parallel threshold curves, while at high SPLs the contours flatten and all frequencies are perceived as being about the same loudness. These results in animals should be considered when thinking about aspects of acoustic communication such as sound transmission, vocal signals designs, and sound-attenuating properties of the environment.

Change in Sensory Functioning Predicts Change in Cognitive Functioning: Results from a 6‐Year Follow‐Up in the Maastricht Aging Study

To examine the longitudinal relationship between sensory functioning and a broad range of cognitive functions after 6 years follow-up and whether cataract surgery or first-time hearing aid use affected cognition. Hierarchical regression procedures were employed to determine whether sensory functioning was predictive of cognitive performance. Maastricht University and the University Hospital Maastricht, the Netherlands. Older Dutch adults (>/=55) enrolled in the Maastricht Aging Study (N=418). Visual and auditory acuity, the Visual Verbal Learning Test (VVLT), the Stroop Color Word Test (SCWT), the Concept Shifting Task (CST), the Verbal Fluency Test, and the Letter-Digit Substitution Test (LDST). A change in visual acuity was associated with change in most cognitive measures, including the total and recall scores of the VVLT, the mean score of the first two SCWT cards, the mean score of the first two CST cards and the LDST. In addition, a change in auditory acuity predicted change in memory performance (VVLT total and recall scores), and auditory acuity measured at baseline predicted change in the mean score of the first two SCWT cards and the LDST. The findings support the notion of a strong connection between sensory acuity in auditory and visual domains and cognitive performance measures, both from a cross-sectional and a longitudinal perspective. They also suggest that it is essential to screen older individuals in a clinical context for sensory functioning so that changes in visual or auditory acuity are not interpreted as changes in cognitive performance.

Human temporal auditory acuity as assessed by envelope following responses

Temporal auditory acuity, the ability to discriminate rapid changes in the envelope of a sound, is essential for speech comprehension. Human envelope following responses (EFRs) recorded from scalp electrodes were evaluated as an objective measurement of temporal processing in the auditory nervous system. The temporal auditory acuity of older and younger participants was measured behaviorally using both gap and modulation detection tasks. These findings were then related to EFRs evoked by white noise that was amplitude modulated (25% modulation depth) with a sweep of modulation frequencies from 20 to 600 Hz. The frequency at which the EFR was no longer detectable was significantly correlated with behavioral measurements of gap detection (r = -0.43), and with the maximum perceptible modulation frequency (r = 0.72). The EFR techniques investigated here might be developed into a clinically useful objective estimate of temporal auditory acuity for subjects who cannot provide reliable behavioral responses.

Temporal Acuity and the Identification of Temporal Order: Related, but Distinct, Auditory Abilities

Nearly a half century ago, Ira Hirsh published the first of many articles establishing the difference between (A) auditory temporal acuity (the ability to distinguish two sounds by their temporal properties) and (B) the limits of the perception of the temporal order of sounds. The thresholds for both A and B are described in terms of stimulus onset asynchrony (SOA). For many sounds, A is in the order of microseconds if the two sounds are delivered dichotically, one to each ear, and reaches two to three milliseconds when both are presented to one ear. The limits of B are much greater, generally 20 milliseconds or more for pairs of sounds, and roughly the same limits are found for pairs of stimuli from more than one sensory modality: light-sound, sound-touch, light-touch. Although still not well understood, several experiments on these topics suggest that A is limited by more peripheral mechanisms of sensory processing, whereas B involves higher levels of the nervous system, where perceived temporal relations may be subject to control via mechanisms of selective attention, as in prior entry phenomena. Some evidence suggests a very broad range of SOA over which temporally correlated stimuli may be brought into registration, perhaps to enhance their mutual effects on the extraction of common information.

Impaired gap detection in juvenile microgyric rats

Previous research with adult animal models links the presence of cortical neuromigrational anomalies (i.e., microgyria similar to that found in brains of dyslexics) with rapid auditory processing (RAP) impairments. RAP impairments are in turn found in children with specific language impairment (SLI) and also in individuals with dyslexia. Gap detection, a simple measure of auditory temporal acuity, appears to be impaired in children with SLI but not in dyslexic adults, even though both groups exhibit impaired processing on more complex, rapid auditory tasks. In the current study, juvenile rats with bilateral microgyria, but not their adult counterparts, exhibited impaired detection of short duration silent gaps in white noise when compared to age-matched sham littermates. Results lend further support to: (1) an association between neuromigrational anomalies and RAP impairments; and (2) the validity of an animal model of RAP impairments associated with language disturbances in humans. Current results also support the view that auditory processing disturbances associated with cortical malformations may be evident early in development at a relatively “low” level (e.g., simple gap detection), but may require “higher-order” auditory discrimination tasks (e.g., tone sequences, phonemic discriminations) to be elicited later in life.

Theory-of-mind development in oral deaf children with cochlear implants or conventional hearing aids.

In the context of the established finding that theory-of-mind (ToM) growth is seriously delayed in late-signing deaf children, and some evidence of equivalent delays in those learning speech with conventional hearing aids, this study's novel contribution was to explore ToM development in deaf children with cochlear implants. Implants can substantially boost auditory acuity and rates of language growth. Despite the implant, there are often problems socialising with hearing peers and some language difficulties, lending special theoretical interest to the present comparative design. A total of 52 children aged 4 to 12 years took a battery of false belief tests of ToM. There were 26 oral deaf children, half with implants and half with hearing aids, evenly divided between oral-only versus sign-plus-oral schools. Comparison groups of age-matched high-functioning children with autism and younger hearing children were also included. No significant ToM differences emerged between deaf children with implants and those with hearing aids, nor between those in oral-only versus sign-plus-oral schools. Nor did the deaf children perform any better on the ToM tasks than their age peers with autism. Hearing preschoolers scored significantly higher than all other groups. For the deaf and the autistic children, as well as the preschoolers, rate of language development and verbal maturity significantly predicted variability in ToM, over and above chronological age. The finding that deaf children with cochlear implants are as delayed in ToM development as children with autism and their deaf peers with hearing aids or late sign language highlights the likely significance of peer interaction and early fluent communication with peers and family, whether in sign or in speech, in order to optimally facilitate the growth of social cognition and language.

Typical and atypical Cogan's syndrome: 32 cases and review of the literature.

To report our experience on a multicentre series of 32 patients with either typical or atypical Cogan's syndrome, to combine our results with a detailed review of the literature, and to compare the clinical manifestations of typical and atypical Cogan's syndrome. Patients were identified from a survey conducted with physicians affiliated to the French National Society for Internal Medicine, and were classified into typical or atypical Cogan's syndrome according to the Haynes criteria. Clinical data were collected in a standardized manner. A comprehensive literature review using the Medline database and the reference lists of identified articles was performed. Seventeen patients had typical Cogan's syndrome and 15 had atypical Cogan's syndrome. Apart from non-syphilitic interstitial keratitis, the ocular manifestations of patients with atypical Cogan's syndrome were mainly uveitis and episcleritis. All but one patient presented with Ménière-like syndrome, and at the end of follow-up 11 were deaf and 19 additional patients had developed a significant decrease in auditory acuity. Twenty-five patients (78%) developed systemic manifestations, including aortitis in four. Comparison of typical and atypical Cogan's syndrome showed that some systemic manifestations were more common in atypical Cogan's syndrome, but these differences may be explained by reporting bias in the literature. Differences regarding the associated systemic manifestations of typical and atypical Cogan's syndrome may reflect reporting bias in the literature. However, the diversity of the ocular and audiovestibular manifestations and the acceptable lengthy delay between the two types of involvement in atypical Cogan's syndrome should make one cautious before accepting this diagnosis as the diagnosis may mimic various other systemic diseases.