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Hearing Problems in People with Spinocerebellar Ataxia

Hearing Problems in People with Spinocerebellar Ataxia. Gary Rance (PhD). SCA Australia Meeting, Nov 8, 2009. Gary Rance. Associate Professor The University of Melbourne Co-ordinator Master of Clinical Audiology program

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Hearing Problems in People with Spinocerebellar Ataxia

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  1. Hearing Problems in People with Spinocerebellar Ataxia Gary Rance (PhD) SCA Australia Meeting, Nov 8, 2009

  2. Gary Rance • Associate Professor The University of Melbourne • Co-ordinator Master of Clinical Audiology program • Wagstaff Research Fellow in Otolaryngology (Royal Victorian Eye & Ear Hospital)

  3. Project: Hearing Problems in People with SCA • Part of a broader study of auditory function in listeners with neurodegenerative disease • Friedreich ataxia (FRDA) • Charcot-Marie-Tooth syndrome (CMT) • Combined visual/auditory neuropathies • Lebers Hereditary Optic Neuropathy (LHON) • Autosomal Dominant Optic Atrophy (ADOA)

  4. Auditory Processing • Looking for problems in the way sound information is transmitted from the ear to the brain • Friedreich ataxia findings (2007 onwards) • Normal sound detection • Abnormal neural transmission • Disrupted perception of speech (particularly in background noise) • 30/30 FRDA subjects showed impaired speech understanding in “everyday” listening conditions

  5. Why Look for Hearing Problems in SCA patients? • Many studies (Abele et al, 1997; Kumagi et al, 2000; Perretti et al. 1996) have shown abnormal electrical responses in the auditory pathways of SCA patients (50-80% affected) • None of these studies looked at auditory perception • This project: • sound detection (standard hearing test) • electrical responses (auditory brainstem response) • auditory processing (timing cues) • perception (speech understanding) • habilitation

  6. Assessments • Ethics approval from the RVEEH ethics committee • Non-invasive • Based on standard clinical tests • Run from a laptop computer so the testing can be carried out at home if convenient

  7. Assessments: Auditory Brainstem Response (ABR) • Electrical responses to sound from the auditory pathway (brainstem) • Measured via three sensors placed on the scalp • Subject response: nil • Duration:  20 minutes

  8. AuditoryBrainstem Response (ABR) V III V I III Subject N8 (rarefaction) I III I V 0.5 V/Div * * * 0 2 4 6 8 10 12 14 ms

  9. AuditoryBrainstem Response (ABR) V III V I III Subject N8 (rarefaction) I III I V I III FAABR (rarefaction) V (N=14) 0.5 V/Div CM FAAN (rarefaction) * (N=14) * FAAN (compression) CM * (N=14) 0 2 4 6 8 10 12 14 ms

  10. Assessments: Auditory Processing • Perception of temporal (timing) cues • Aim: to find the smallest change in a rapidly fluctuating sound that a listener can perceive • Subject response: indicate when able to hear a change in a constant sound • Duration:  20 mins

  11. Speech Perception in Quiet & Background Noise

  12. Assessments: Speech Perception • CNC Word Test • recorded speech in quiet and different levels of background noise • Subject response: imitation • Duration:  30 minutes

  13. Rance et al., (2008) FAAN +20 +10 +5 0 Quiet

  14. Project Details • Testing carried out by Master of Clinical Audiology Students • Data collection: March-August 2010 • Typically requires a single 1.5 hour session to test both ears • Subjects: - all welcome - particularly interested in SCA groups 1, 2 & 6

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