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Application of otoacoustic emissions in the diagnosis of hearing loss Bradley McPherson PhD Centre for Communication Disorders University of Hong Kong Otoacoustic emissions • Otoacoustic emissions (OAE) are low intensity audiofrequency sounds • Produced by the cochlea as part of the normal hearing process • Kemp (1978) English biophysicist discovered otoacoustic emission and published the first scientific description of transient-evoked otoacoustic emissions (TEOAEs) • In 1979 first work with distortion product otoacoustic emissions (DPOAEs) • TEOAEs are elicited by clicks or brief tonal stimuli, and DPOAEs are elicited by two simultaneously presented tones of slightly different frequency Uses of OAEs Screening using OAEs: Neonatal hearing screening Community-based hearing screening School-based hearing screening NIHL screening Uses of OAEs Overall response = 20.4 dB SPL, 81% reproducibility Response at 1.5 kHz = 12 dB SPL, 94% reproducibility Response at 2.2 kHz = 11 dB SPL, 94% reproducibility Response at 3 kHz = 7 dB SPL, 84% reproducibility TEOAE result 3 day old neonate Uses of OAEs Diagnostic uses of OAEs: NIHL Functional hearing loss Retrocochlear hearing loss Ménière’s disease Sudden hearing loss 3. Gross anatomy of ear Case history 1 • Screening program for auditory neuropathy – January 2002 – Children attending school for deaf screened using otoscopy, tympanometry and TEOAE measures – KK tested positive in both ears – Bilateral TEOAEs present • Follow-up – Detailed case history from KK’s father and teacher – Diagnostic assessment in February 2002 Initial screening results KK’s history • 9;6 years, female • Initially diagnosed at 3 years with severe bilateral sensorineural hearing loss • Fitted with hearing aid for left ear at 3;4 years • At first full assessment also diagnosed with low muscle tone and balance disorder • Normal CT and MRI findings KK’s history • Family history: mother and younger brother have hearing loss of unknown aetiology • Brother has mild loss but good speech and language development KK’s history • Speech production: some unintelligible vowel-like utterances • Speech reception: very poor • Communication by sign at home and with classmates • Depends greatly on visual cues • Oticon BTE hearing aid in left ear of no significant benefit, even with FM system KK’s audiogram OAE results • Confirmed TEOAE in right ear • Noted high frequency TEOAE in left ear • DPOAE results consistent with TEOAE results • DPOAE amplitude growth results also consistent with other findings Right ear OAEs Left ear OAEs DPOAE I/O function Other test results • Tympanometry: bilateral Type A • Acoustic reflexes: absent contralateral and ipsilateral • Speech recognition: 0% unaided in monaural and binaural conditions, 0% aided left Other test results • ABR: no synchronous responses at 95 dB nHL in right or left ear • Cochlear microphonic present bilaterally • Middle latency response: absent right and left • Late evoked response: N1-P2 present bilaterally with ipsi- or contralateral stimulation ABR in right ear Cochlear microphonic MLR in right ear Late responses in right ear P2 N1 Case history 2 • 26-year-old Chinese woman seen for an audiological evaluation in May 2003 • Main complaint was persistent hearing difficulties in situations with background noise, particularly in speech perception • She reported only occasional difficulty with speech understanding in quiet Case history 2 • Recent pure-tone audiometric assessment had indicated normal hearing • Her reported communication disorder occurred after an episode of transient ischemic attack in November 2002 • Previously, she had been diagnosed with moyamoya disease following hospitalization for left temporoparietal intracerebral hematoma Moyamoya disease • A rare condition - the progressive narrowing of the distal internal carotid arteries and proximal portions of the anterior and middle cerebral arteries • Most patients with moyamoya disease are children or adults in the third or fourth decades of life • Nearly two-thirds are female • In the later stages of MD ischemic episodes are common, with clinical symptoms such as impaired consciousness, focal motor symptoms, speech dysfunction, seizure and sensory disorders Moyamoya disease • Moyamoya first identified in Japan in 1959 • ‘Moyamoya’ is Japanese for a ‘puff of smoke’ • Refers to the wispy cloud of fragile blood vessels seen on brain angiograms • These develop where normal vessels are blocked Case history 2 • Clear, unoccluded ear canals; tympanic membranes were of normal appearance • Consistent responses to standard pure-tone audiometry • Normal hearing thresholds in the left ear and a very slight low frequency sensorineural hearing loss noted in the right ear • Tympanometry results were consistent with normal middle function in both ears. Acoustic reflexes with ipsilateral and contralateral stimuli were present in both the left and right ears Case history 2 -10 Decibels Hearing Level (dB HL) 0 10 20 RE (AC) 30 40 LE (AC) 50 60 LE (BC) 70 80 RE (BCM) 90 100 110 120 250 500 1000 2000 Frequency (Hz) 4000 8000 Case history 2 • Transient evoked OAEs showed clear responses - right and left ears • Over full range of frequencies measured (1000 Hz to 5000 Hz) • Normal OAEs with slightly reduced right ear thresholds indicated a need for further testing • Possible retrocochlear disorder Case 2 TEOAEs Right ear Left ear Case history 2 CAPD evaluation was also performed • • • Hearing in Noise Test - Cantonese version: Binaural 50% correct threshold for speech in quiet = 26 dBA. Binaural speech in noise composite threshold was -3.0 dBA. Both values outside the 99th percentile for normal listeners Pitch Pattern Sequence: Results also abnormal 58% correct in the left ear and 70% in the right ear (norms = 99% correct score) Random Gap Detection: All thresholds were markedly abnormal compared to clinical normative values (50 msec compared to 6.4 msec) Case history 2 • CAPD test results were consistent with a diagnosis of central auditory processing disorder • Results were explained to the patient, who was relieved to learn that her auditory problems were ‘real’ despite earlier, pure-tone only evaluations having stated her hearing was normal • We were able to give the patient a clear understanding of the reason for her difficulties in situations of environmental noise In conclusion • OAE information can be of great value in diagnostic audiology • Can help determine site of disorder • Quick, easy to perform and noninvasive • Should always be included in a full diagnostic assessment Acknowledgements • • • • These case studies were only possible with the assistance of: Dr Man-tak Leung, Teaching Fellow, HKU Ms Lena Wong, Assistant Professor, HKU Mr Kevin Yuen, Research student, CUHK Ms Juvy Lee & Ms Tempo Tang, Research students, HKU