A Case Study Unilateral cochlear dysplasia in a 9-month child diagnosis and intervention Zhang Hua, M.D. Beijing Tongren Hospital Capital Medical University Beijing Institute of Otolaryngology 8 10 2017 Case Report

Basic Information Infant, female, date of birth: August 30, 2016 come to the hospital on May 22, 2017 (nearly nine months old) Neonatal hearing screening in left ear did not pass the sound response in general: sometimes chasing sound. Local hospital diagnosed sensorineural hearing loss in the left ear Birth: full term birth, jaundice after two days, followed by medical treatment (medicine). Denied suffocation, hypoxia. Maternal pregnancy history: hyperglycemia (high blood sugar); mid-pregnancy "cold" without medication; child with umbilical cord around the neck a week. Deaf regular gene screening: normal. Family history: (-) Language situation: can pronouce "baba" and other meaningless syllable now. 0至6岁听力残疾儿童15.8万人，重度极重度听障者占20%至30%。 Case Report 2

Local hospital inspection results AI: Tympanogram: Right: As with single peak; Left: A with single peak ABR (by air) R 30 dB nHL; L 90 dB nHL 40 Hz AERP: R 30 dB nHL; L 70 dB nHL ASSR: 500 1000 2000 4000 Hz without masking R 14 19 17 31 L 50 59 87 71 (dB eHL) DPOAE: right ear pass, left ear did not pass 2013版人工耳蜗植入指南对人工耳蜗植入术后效果评估方法和内容进行了分类 Case Report 3

ABR by another local Hospital without masking 国 内 Case Repoet 4

Re-Examination Otologic: intact tympanic membrane, Bone drums complete, clear anatomic landmarks;     nose (-);  pharynx(-). Inner ear MRI: left cochlear nerve slim, dysplasia. padiatric behavioral audiometry (naked ears, plug-in headphones, VRA condition was established well) 250 500 1000 2000 4000 Hz R 25 25 20 25 25 L 60 60 65 70 70 (dB HL) Case Report 5

Cross section on the right side Cross section on the left side MRI Image Left side of the cochlear nerve Right side of the cochlear nerve Cross section on the right side 国 内 Cross section Cross section on the left side Case Report 6

Left side of the sagittal plane Right side of the sagittal plane MRI Image 国 内 Left side of the sagittal plane Right side of the sagittal plane Case Report 7

Visual Reinforcement Audiometry ,VRA 国 内 8 Case Report

Pediatric behavioral Result Studies have shown that when the test tone is a pure tone, the minimum interaural attenuation of the plug-in headphones can be up to 50 dB. When the test tone is broadband click, interaural attenuation is 55 dB *. The right ear is normal, and the actual threshold may be less than the VRA result. Both thresholds exceed 55 dB. Due to the small age of child, subjective test can not be masked; Due to the left side of the child with cochlear dysplasia, consider that the objective examinations were used to determine the threshold of the left side *Munro KJ, Contractor A. Inter-aural attenuation with insert earphones. Int J Audiol. 2010, 49(10): 799-801. 国 内 Case Report 9

本院客观电生理检查结果 国 内 Case Report 10

ABR Air conduction waveforms 国 内 Air conduction： R 20 dB nHL; L 100 dB nHL no reaction Case Report 11

ABR Bone conduction waveform 国 内 Bone conduction： R 30 dB nHL; L 45 dB nHL no reaction 12 Case Report

CM Waveforms 国 内 On the left side, the waves with the opposite polarity are obtained by using the rarefaction wave (upper) and the dense wave (bottom) respectively. Case Report 13

Diagnosis 内 Left ear ABR was not induced, there are repeatable CM; Left cochlear nerve dysplasia; ABR results do not match with ASSR; Can be diagnosed as auditory neuropathy spectrum disorder with cochlear nerve dysplasia of the left ear. * Zhang Jiao, Zhang Qiujing, Qi Yue, et al. Auditory analysis of patients with neurological disorders in cochlear nerve dysplasia. Chinese Journal of Otology, 2015,13 (2): 242 ~ 245. 国 内 Case Report 14

Intervention trying to wear Oticon Nera2 BTE 85 with earmold in the left Use the NAL-NL2 formula no frightening reaction with drum beating and ringing bell; Tuning fork (1024 Hz) test with hearing aid: obvious sound localization between left and right (horizontal). Parents finally accepted the hearing aid fitting in the left. Follow-up planned. 国 内 Case Report 15

Discussion For the unilateral hearing loss: the possibility of masking should be carefully considered, in particular for uncooperative young children. The objective audiometry with masking for the determination of unilateral hearing loss is important to avoid misdiagnosis. Because it is hard to know the etiology of ANSD (not this case), and the clinical manifestations have large differences, the choice of intervention is still a question. Current studies have shown that cochlear implant for ANSD has 50% positive effect. 国 内 Case Report 16

Discussion In this case, the child with unilateral hearing loss, the right ear hearing is normal. cochlear implant is not recommended (destroy the inner ear construction and the result is not predicted). The left side of the optional hearing aid helps to perceive the outside world, enhance the sense of sound localization and stimulate the cerebral cortex. This may help to enhance its sensitivity to the sound. Further follow-up are necessary. 国 内 Case Report 17

Thanks ! 2011 undergraduate students thesis defense 18