Accepted Manuscript Central auditory processing in patients with spinocerebellar ataxia Bianca Simone Zeigelboim, Hugo Amilton Santos de Carvalho, Hélio Afonso Ghizoni Teive, Paulo Breno Noronha Liberalesso, Ari Leon Jurkiewicz, Edna Márcia da Silva Abdulmassih, Jair Mendes Marques, Mara L. Cordeiro PII:

S0378-5955(15)00146-X

DOI:

10.1016/j.heares.2015.07.006

Reference:

HEARES 6976

To appear in:

Hearing Research

Received Date: 16 April 2015 Revised Date:

2 July 2015

Accepted Date: 8 July 2015

Please cite this article as: Zeigelboim, B.S., de Carvalho, H.A.S., Teive, H.A.G., Liberalesso, P.B.N., Jurkiewicz, A.L., da Silva Abdulmassih, E.M., Marques, J.M., Cordeiro, M.L., Central auditory processing in patients with spinocerebellar ataxia, Hearing Research (2015), doi: 10.1016/j.heares.2015.07.006. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

ACCEPTED MANUSCRIPT Central auditory processing in patients with spinocerebellar ataxia Bianca Simone Zeigelboim 1, Hugo Amilton Santos de Carvalho* 1, Hélio Afonso Ghizoni Teive 2, Paulo Breno Noronha Liberalesso 3, Ari Leon Jurkiewicz 1, Edna Márcia

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da Silva Abdulmassih 2, Jair Mendes Marques 1, Mara L. Cordeiro 4,5,6

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University Tuiuti of Paraná, Otoneurology Research Center, Curitiba, Brazil

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Neurology Service, Department of Clinical Medical, Clinical Hospital, Federal

University of Paraná, Curitiba/PR, Brazil

Department of Neuropediatrics, Little Prince Children’s Hospital, Curitiba, Brazil

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Neurosciences Research Group, Pelé Little Prince Research Institute, Curitiba, Brazil

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Faculdades Little Prince, Curitiba, Brazil

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Department of Psychiatry and Biobehavioral Sciences of the David Geffen School of

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Medicine, Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles, USA

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*Corresponding author: Hugo Amilton Santos de Carvalho.

Address: Rua Visconde de Nácar, 72 – Apt 31 – Centro – Curitiba/PR, Brazil

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E-mail: [email protected]

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ACCEPTED MANUSCRIPT Corresponding Author: Hugo Amilton Santos de Carvalho Address: Rua Visconde de Nácar, 72 – Apt 31 – Centro – Curitiba/PR, Brazil E-mail: [email protected]

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1. Introduction

Autosomal dominant spinocerebellar ataxias (SCAs) are a group of rare and heterogeneous neurodegenerative diseases characterized by the presence of

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progressive cerebellar ataxia. SCA cases can be classified according to genetic inheritance as autosomal recessive, autosomal dominant, X-linked, or mitochondrial

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(Teive et al., 2010).

SCA has a worldwide prevalence of 3 to 4.2 cases per 100.000 people (Magana et al., 2012). Subtype prevalence rates vary among geographic regions. For example, the highest prevalence rates for SCA2 are found in Cuba, India, England, France, and

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the USA, whereas SCA3 is most prevalent in Portugal, Brazil, Germany, Japan, and China. Meanwhile, SCA6 is particularly prevalent in Japan, Australia, and Germany, SCA7 is most prevalent in Sweden, Finland, the USA, and China, and SCA10 cases

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are concentrated in Mexico and Brazil (Teive, 2009; Teive et al., 2010). More than 30 genetic subtypes are known, with SCA3 (Machado-Joseph

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disease) and SCA2 being the most and second-most prevalent forms in Brazil, respectively. Most SCA subtypes involve an expansion of trinucleotide CAG repeats (Arruda et al., 1991; Kim et al., 2001; Rub et al., 2008; Teive, 2009; Magana et al., 2012; Rub et al., 2013). When the transmission is paternal, there is a greater tendency for the number of repetitions to increase in the next generation, resulting in earlier and more intense manifestations (Kim et al., 2001; Rub et al., 2013). Patients with SCA are classified according to the disease’s impact on sensory, frontal, vestibular, and cerebellar functions (Teive, 2009; Teive et al., 2010). Commonly studied clinical manifestations of SCA are abnormal gait and appendicular ataxia

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dysdiadochokinesia,

intention

tremor),

dysarthria,

nystagmus,

ophthalmoplegia, dysphagia, hearing loss, pyramidal signs, cognitive dysfunction, epilepsy, visual disturbances (retinopathy pigmentosa), peripheral neuropathy, dementia, and movement disorders (including parkinsonism, dystonia, myoclonia, and

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chorea) (Arruda et al., 1991; Klockgether, 2000; Teive, 2009; Teive et al., 2010; Magana et al., 2012).

There is a correlation between ethnic background and the prevalence of a particular mutation that causes the SCA, thus, the allele frequency of the expansions is

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also characteristic for each population (Klockgether, 2000). Hereditary ataxias have great genotypic and phenotypic heterogeneity. Phenotypic heterogeneity implies that

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the same genotype can determine several different phenotypes, and the genotypic heterogeneity requires the same phenotype may be due to various genotypes (Teive, 2009). With advances in molecular genetic techniques through the use of PCR (Polymerase chain reaction diagnostics), several genetic loci and genes have been

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discovered in different chromosomes, thus enabling a more rational use of genetic clinical classification (Klockgether, 2000). The polyglutamine neurodegenerative diseases are characterized by the expansion of a polyglutamine tract in the mutant

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protein causing the disease. This mutant protein leads to a progressive loss of neuronal function and subsequent neurodegeneration of a specific group of neurons

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causing the phenotypic forms of each disease, in addition to the patient’s ethnic origin, is of paramount importance. The appreciation of different neurological signs in SCAs and genetic study have provided a great aid in genotype-phenotype (Teive, 2009). In order to measure the severity of cerebellar ataxia in an easier and more

practical way, Schmitz-Hübsch et al. (2006) proposed a scale for the assessment and rating of ataxia (SARA) which was translated and validated for Brazilian Portuguese by Braga - Neto el al. (2010). SARA has eight items that yield a total score of 0 (in ataxia) to 40 (most severe ataxia); 1: gait (score 0 to 8), 2: stance (score 0 to 6), 3: sitting (score 0 to 4), 4: speech disorder (score 0 to 6), 5: finger-chase test (score 0 to 4 ), 6:

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nose-finger test (score 0 to 4), 7: fast alternating hand movements (score 0 to 4), 8: heel-shin slide (score 0 to 4). Limb kinetic functions (items 5 to 8) are rated independently for both sides, and the arithmetic mean of both sides is included in the SARA total score (Schmitz-Hübsch et al., 2006).

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This scale proved to be a reliable and valid measurement for patients with autosomal dominant spinocerebellar ataxia.

The evaluation of the peripheral auditory system is performed by behavioral, electrophysiological and electroacoustic tests. Among the electrophysiological

tool

in

research

for

the

evaluation

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techniques, the Brainstem Auditory Evoked Potential (BAEP) has been an important central

auditory

function

involving

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neurodegenerative diseases and has shown that the most evident changes occur in the inferior colliculus region, lateral lemniscus and cochlear nucleus (Nachmanoff et al., 1997; Biacabe et al., 2001).

Central auditory function is distinct from peripheral hearing function in that the

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latter involves the amplification and conduction of sound waves, which produce mechanical activity that the cochlea transforms into nerve impulses in the auditory nerves, whereas the former involves neural processing in the brain subsequent to

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auditory nerve signals. The neural signaling underlying central auditory function in the brain is referred to more specifically as central auditory processing (CAP).CAP is

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critical for various behavioral phenomena, including language comprehension Friederici, (2002) which requires that acoustic signals be analyzed and interpreted such that they can be transformed into meaningful communication (Sauer et al., 2006). Moreover, CAP contributes to sound discrimination, localization, and recognition as well as to attention, and memory (Musiek and Baran, 1997; Pereira and Schochat, 1997). Deficits in CAP can occur as a result of congenital anomalies, structural lesions, or degenerative processes (Musiek and Baran, 1997; Palfery and Duff, 2007). The CAP assessment provides information to identify, quantify and qualify the dysfunctions in specific brain areas showing what skills will form the baseline for the rehabilitation

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program to be more effective (Pereira and Schochat, 1997; Bamiou et al., 2001; Palfery and Duff, 2007). The battery of behavioral tests used to detect CAPD includes tasks that are monotic (sounds presented to each ear separately), diotic (identical sound stimuli

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presented to both ears simultaneously), dichotic (different stimuli presented to the two ears simultaneously), and temporal processing tasks (Demanez and Demanez, 2003; Liberalesso et al., 2012). The first auditory processing tests emerged due to the need to evaluate the central auditory pathway for the purpose of identifying cerebral injuries

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(Broadbent, 1954). In recent decades, an increasing number of studies involving auditory function and neurodegenerative diseases has been observed. With this,

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several tests that make up the CAP assessment battery have been applied. Among them, we emphasize the most used as: the Filtered Speech Test and Binaural Fusion Test – both used in identifying brainstem injury; Frequency or Duration Pattern tests, Gap In Noise (GIN) and the Random Gap Detection Test (RGDT) - assess temporal

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resolution ability; Synthetic Sentence Identification test with Ipsilateral Competing Message - used in the identification and location of the brainstem lesion; Threshold Change test using masking - used in locating lesions of the lower portion of the brain

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stem; Dichotic test - used on suspicion of cortical and/ or hemispheric inter involvement. This test is sensitive to brainstem, auditory cortex, and inter-hemispheric

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connection lesions; and Staggered Spondaic Word (SSW) Test - used in adults with neurological damage (Schochat, 2012). To verify the integrity and the functioning of central auditory pathways and

describe the hearing abilities in adults with hearing dysfunctions in the population studied, we opted for: SSW tests to evaluate binaural integration and separation and, in so doing, the integrity of central hearing function (involvement of the brain stem, of the right and left hemispheres, and inter- and intra-hemispheric connections) Katz and Smith, (1991) and RGDT to evaluate temporal resolution ability (the ability to differentiate one stimulus from two stimuli separated by an interval of silence called the

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auditory fusion threshold) (Keith, 2000). The diagnosis for a CAP disorder is characterized by a loss of hearing ability, and should be considered a hearing disorder, which also may be due to neurological changes as mentioned above (Musiek and Baran, 1997)

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The aim of the present study was to assess central auditory function in patients with SCA. We compared SSW and RGDT performance, as well audiological, acoustic immittance and BAEP results, between patients with different types of SCA to gain

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insight into the general clinical phenotypes of the subtypes.

2.1 Participants and study design

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2. Materials and Methods

The research protocol was approved by the Ethics Committee on Research Involving Human Subjects (registration number CEP 058/2008) at University Tuiuti of Paraná in Curitiba, Brazil. All examinations were performed after formal written

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informed consent was obtained from all participants.

The study had a retrospective cross-sectional design. We evaluated 43 patients (17 females and 26 males) with a conclusive diagnosis of SCA. Polymerase chain

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reaction (PCR) diagnostics indicated that the group included 12 patients with SCA3, 8 with SCA2, 1 with SCA4, 1 with SCA6, 1 with SCA7, and 6 with SCA10 (Schols et al.,

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2004; Pearson et al., 2005; Duenas et al., 2006). It was not possible to determine the subtype of SCA in the remaining 14 patients, who were grouped together as an undetermined type subgroup. The age of the patients ranged from 18 to 70 years (mean, 41.6 ± 13 years). The duration of the disease ranged from 1 to 15 years (mean, 7.9 ± 3.9 years) (Tables 1 and 2). Molecular genetic study was a major advance to the use of PCR, which is a method that allows the production of large quantities of a particular DNA segment from only one DNA molecule, without the need to insert this DNA molecule into bacteria. This is an extremely ingenious procedure revolutionized

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DNA analysis (Pulst, 2003). The PCR reaction is based on the fact that the oligonucleotides (primers) hybridize specifically to a DNA mold, enabling the production of numerous copies of specific sequential DNA (Pulst, 2003). The SCAs are characterized by great genetic heterogeneity (phenotypic and genotypic) and

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neurological clinical assessment of affected patients is always a difficult job. Different clinical and neuropathological studies have shown that each SCA specific genetic entity has a constellation of signs and symptoms that are related to disease duration, and with the size of trinucleotide repeat expansion (CAG). Thus, the combination of

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molecular genetic data and clinical signs provide useful information that serve as the basis for the classification of SCAs (Schelhaas et al., 2000; Stevanin et al., 2000).

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However, we did exclude patients with severe auditory loss, patients who presented any apparent difficulties with language comprehension, and patients who demonstrated difficulties with completing any of the tests. We point out that 2 patients with SCA3 (numbers 7 and 10) and 1 patient with SCA10 (number 28) did not perform

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the SSW and RGDT tests because they had hearing thresholds that prevented their completion. The research excluded 12 patients (4 died, 5 declined to participate, and 3 had severe auditory loss).

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It is known that auditory neuropathy (AN) consists of an impairment of the auditory nerve that generates a loss of synchronous nerve conduction, probably related

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to a change in the myelination of these fibers. The precise location of the alteration is not defined and may differ in several cases, but should occur in the inner hair cells, the synapses between the inner hair cells and the VIII cranial nerve, or in several of these strutures (Starr et al., 1996). Lately AN, until recently unknown, has been more and more studied and diagnosed. It is known that the diagnosis of AN is through an important absence or change in BAEP from wave I, along with the presence of normal otoacoustic emissions (OAE). The acoustic reflex is altered and invariably the difficulty of vocal discrimination is one of the major complaints (Starr et al., 1996). In addition, patients with AN have an alteration in the suppressor effect of OAE caused by efferent

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auditory pathways. Patients with AN may have a long cochlear microphonic that, during the course of BAEP, can be confused with a possible response of the auditory nerve. Reversing the polarity of the stimulus, for example, changing the application of a condensate stimulus, reverses the polarity of the waves if these are a product of

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cochlear microphonics, but the polarity is unchanged if response is from the auditory nerve. This method, easy to perform, should always be used as a way of fine-tuning the test for the more reliable detection of responses. In the present study, we did not identify such an extension of the cochlear microphonic. AN is described in the literature

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in many cases accompanying cases of hereditary ataxia and peripheral neuropathies, for example, Refsum syndrome, Charcot-Marie-Tooth syndrome, Friedreich's Ataxia

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and Guillain-Barre syndrome. In all of these diseases, concomitant optical involvement can occur (van Bogaert and Martin, 1974). Patients were given the BAEP test to rule out AN, and make the results of this study more reliable.

All patients underwent a case history review, an otorhinolaryngological

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examination, and a peripheral audiological evaluation, which included pure tone audiometry, speech recognition threshold (SRT), acoustic immittance testing and the BAEP exam to exclude AN. The audiological evaluations were performed in a single

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session which took, on average, 45 min. Subsequently, the participants were subjected to the SSW and RGDT.

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The simplest psychoacoustic methods used to evaluate temporal resolution (TR) are based on the detection of interstimulus intervals, so-called gaps, with the purpose of establishing the lowest perceived gap interval between two sounds (gap detection threshold). We currently have available for clinical use two TR tests based on gap detection: the RGDT and the GIN. Parameters in the two tests differ in the length of each gap, presentation of the stimulus, and requested task. In clinical practice, due to a lack of consensus as to which TR assessment method to assess is more effective or practical for incorporation into the battery, the choice is left up to the professional. In addition, the RGDT is a quick and easy to apply test, especially when compared to the

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GIN, which demands greater runtime. The RGDT requires a more complex response cognitively than GIN, since the patient is instructed to say or demonstrate hand movement if detecting one or two stimuli. The GIN test is more extensive but requires a simpler response, considered nonverbal, in which the patient only needs to signal when

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the stimulus is interrupted. Fatigue may negatively influence the patient's performance. The execution time of each procedure needs to be considered, and has been a determining factor in the choice made by the professional applying the battery of tests. The RGDT is a binaural test and the GIN is a monaural test, moreover, Chemark and

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Lee, (2005) refer to the fact that RGDT parameters prompt the patient to perform a complex auditory task involving not only TR but also auditory fusion, however the GIN

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test could be considered a test exclusively for TR. For the reasons set out above, this study opted for the application of RGDT.

The SSW test is a dichotic test and enables the evaluation of auditory analysissynthesis ability and memory, and involves identifying four different disyllables

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presented simultaneously in both ears (binaural). It is an easy test to apply and testing may be performed on patients with neurological alterations.

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2.2 Audiology equipment

Peripheral audiological and CAP evaluations were performed in a soundproof

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booth with an Interacoustics AC-40 model audiometer connected to TDH 39P headphones (calibrated to ANSI-69). Hearing loss was characterized in accordance with previously established criteria (Davis and Silverman, 1970; Silman and Silverman, 1997). The audiometer was connected to a Sony Discman (model D88). We used compact discs (CDs) for the SSW (CD from a CAP assessment kit volume 2, track 6 (Pereira and Schochat, 1997) and RGDT (CD developed by Keith, (2000), tracks 2–8 in accordance with the Auditec manual). Acoustic immittance was measured with a clinical impedance audiometer (model AZ-26) attached to TDH 39P headphones in

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accordance with published criteria in research on contralateral reflex and both ears (.50 – 4kHz) (Jerger, 1970).

2.3 Brainstem Auditory Evoked Potential

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Two channels were used with stimulus clicks at an intensity of 90 dBnHL, alternating polarity with a presentation frequency of 21.1 c/ s, a 15ms window, a filter of 30 to 3 kHz, and at least 2.000 stimuli and two reproduction records. Kendall Medtrace 2000 brand electrodes were positioned on the right and left mastoid in Fz (10-20

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system), and the ground electrode was placed on the forehead. Clicks were presented with 3A insert earphones. The latencies of waves I, III and V and interpeak intervals I-

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III, III-V and IV were analyzed. The equipment used was Bio-logic's Evoked Potential System and the analysis criteria were proposed by the author (Hall, 1992).

2.4 Staggered Spondaic Word Test

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The SSW test described by Katz and Smith, (1991) was adapted to Brazilian Portuguese by Borges, (1986). Patients were instructed to listen carefully to a group of four words in sequence and then to repeat them in the order heard; a total of 40 groups

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of four words were played for each subject. Words were presented in pairs, one in each ear, with a partial overlap between the two ears; that is, the second syllable of the first

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word played while the first syllable of the second word played (Borges, 1986; Pereira and Schochat, 1997; Sauer et al., 2006). Subjects were tested in a right ear competing (RC) condition, in which the words supplied to the right ear were presented without any interfering noise while the second word in the pair was presented to the left ear with simultaneous competing noise, and vice versa in the left ear competing (LC) condition. The stimuli were delivered at an intensity of 50 dB sound pressure level (tritonal average). Before starting the test, each patient received guidance as to how to respond to the test questions. Quantitative analysis was conducted wherein errors were analyzed for each of the conditions of the ears separately and computed as a

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percentage of correct responses to identify the severity of deviation. A correct response rate of ≥90% was considered normal. The results were analyzed according to the same criteria established for the original test adapted by Borges, (1986).

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2.5 Random Gap Detection Test The RGDT was applied as described in detail elsewhere (Keith, 2000; Liberalesso et al., 2012). We employed the Auditec version in which pairs of pure tones were delivered at frequencies of (500, 1000, 2000 e 4000Hz) with the interval between

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two tones ranging from 0 to 40 milliseconds (ms), and the inter-stimulus interval set to 4.5 s to allow ample time for the subjects to respond. The test shows a training range

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that was performed before the start of the test. The result is measured by the smallest interval at which the patient starts to identify the presence of two stimuli. The result is calculated for each frequency and a calculation is also performed among the results for

2.6 Statistical analysis

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the four frequencies.

We applied the Difference of Proportions test in order to compare results from the audiological, acoustic immittance, BAEP, SSW and RGDT tests (dependent

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variable: proportion normal vs. abnormal) in relation to SCA type. In all cases, p < .05

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was considered statistically significant.

3. Results

3.1 Patient characteristics The age, sex, and genetic findings for each of the 43 participants are reported

in Table 1. The prevalence rates at which symptoms were experienced by the patients are listed in Table 3. The most common clinical complaints among our study participants (in order from more to less common) were staggering when walking, difficulty speaking, dizziness, dysphagia, dysphonia, and hearing loss.

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3.2 Audiometric thresholds and Acoustic immittance The audiological and acoustic immittance results for individual patients are reported in Table 4. Overall, abnormal audiological findings were obtained for 14/43

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patients (32.5%). Some of these patients (N = 11; 25.6% of total group) had abnormal findings bilaterally. One patient had abnormal findings only in the right ear. Two patients had abnormal findings only in the left ear. The subjects’ SRT results were consistent with the pure-tone thresholds recorded for them (data not shown). The most

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common alterations observed were a sloping audiometric configuration from 4 kHz bilaterally and the absence of the acoustic reflex at 3 kHz and 4 kHz bilaterally.

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Of the patients with the most common disease types, SCA3 and SCA2, 4/12 and 1/8, respectively, had abnormal results. Half of the patients with SCA10 had abnormal findings. All of the patients with SCA4, SCA6, and SCA7 had abnormal findings. Among the 14 patients with undetermined SCA type, only 3 had abnormal audiological

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findings. Difference of Proportions Tests indicated that the proportions of normal versus abnormal cases differed significantly as a function of SCA type for bilateral (p = .0001), right ear (p < .0001), and left ear (p < .0001) impairment.

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Altered acoustic immittance was detected in 19/43 participants, with all 19 showing alterations bilaterally. Parsing the results in relation to SCA type, we found that 6/12

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patients with SCA3, 4/8 with SCA2, 1/1 with SCA4, 1/1 with SCA6, 1/1 with SCA7, 2/6 with SCA10, and 4/14 patients with undetermined SCA had impaired immittance. The acoustic immittance results for individual patients’ are reported in Table 4. A Difference of Proportions Test yielded a p = .2843, indicating that the frequency of immittance abnormalities did not differ significantly as a function of SCA type.

3.3 BAEP performance In the BAEP assessment, 20 of 43 patients (46.5%) had an abnormal examination (11.6% in the left ear and 34.9% bilaterally), with 7 of 12 (58.3%) having

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SCA3, 5 of 8 (62.5%) SCA2, 100% of the patients with SCA6 and SCA7, 4 of 6 (66.7%) SCA10 and 2 of 14 (14.2%) with undetermined type SCA (see Table 5 for individual patients' results). Difference of Proportions Tests indicated that the proportions of normal versus abnormal cases differed significantly as a function of SCA type for left

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ear (p.9999

SSW, N (%)

SCA 2

SCA 3

SCA 10

Normal Altered TOTAL P RGDT, N (%)

5 (62.5%) 3 (37.5%) 8 (100%) .3343 SCA 2

4 (40%) 6 (60%) 10 (100%) .3829 SCA 3

1 (20%) 4 (80%) 5 (100%) .0943 SCA 10

Normal Altered TOTAL P Acoustic immittance, N (%)

2 (25%) 6 (75%) 8 (100%) .0653 SCA 2

2 (20%) 8 (80%) 10 (100%) .0152* SCA 3

Normal Altered TOTAL P BAEP, N (%) Normal Altered TOTAL P

4 (50%) 4 (50%) 8 (100%) >.9999 SCA 2 3 (37.5%) 5 (62.5%) 8 (100%) .3343

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6 (50%) 6 (50%) 12 (100%) >.9999 SCA 3 5 (41.7%) 7 (58.3%) 12 (100%) .4248

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*Significant comparison; p values for Difference of Proportions Tests shown.

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1 (20%) 4 (80%) 5 (100%) .0943 SCA 10

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Normal Altered TOTAL P

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SCA2

4 (66.7%) 2 (33.3%) 6 (100%) .2742 SCA 10 2 (33.3%) 4 (66.7%) 6 (100%) .2742

ACCEPTED MANUSCRIPT Highlights

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• We detected impaired CAuP in patients with SCA using the SSW and, especially, the RGDT • Our results point to difficulties with integration skills and binaural temporal resolution • CAuP evaluations, such as SSW and RGDT, are important for detecting CAPD in therapeutic examinations of patients with SCA

Central auditory processing in patients with spinocerebellar ataxia.

Autosomal dominant spinocerebellar ataxias (SCAs) are a group of rare and heterogeneous neurodegenerative diseases characterized by the presence of pr...
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