Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P231

severe-deep SNHL according to International Audiophonologie
classification. Genetic variants were described following the
guidelines of the American College of Medical Genetics. We
found 12 (21.81%) pathogenic variants in TECTA (4), KCNQ4
(2), GJB2 (2), ACTG1 (1), COL11A1 (1), COL2A1 (1), and
ABHD12 (1); there were 7 (12.7%) variants of unknown significance and 36 (65.5%) negatives.
Conclusions: Gene panels have a relatively high diagnostic
yield for SNHL in adults. Finding a genetic cause underlying
SNHL not only implicates its etiology but carries clinical
implications, such as genetic counseling for inheritance pattern
and screening of syndromic causes for which other symptoms
could develop, and it gives information about requirements
and evolution of SNHL.
Genome Editing as a Therapeutic Approach
to Progressive Hearing Loss
Aida Nourbakhsh, MD, PhD (Presenter); Xiaoya Chen;
Nicholas Gosstola; Zaohua Huang; Katherina Walz; Derek
Dykxhoorn; Zheng-Yi Chen; Xue-Zhong Liu, MD PhD
Objectives: Progressive hearing loss (PHL) is the most common cause of sensory impairment globally. We have identified
the p.V60L mutation in P2X2 purinoreceptor as the cause of
DFNA41 form of PHL, with associated vulnerability to noise.
Data have shown that the heterozygous mutation leads to
uncoupling of ATP binding and channel opening, which
impairs the cochlear ability to attenuate sound transduction in
response to loud noise. The goal of this study is to create a
gene therapeutic mechanism using the CRISPR-Cas9 technology in our P2RX2 mouse and induced pluripotent stem cell
(iPSC) model of genetic hearing loss.
Methods: CRISPR-Cas9 was used to correct P2RX2 mutation in patient-derived iPSCs. Briefly, Cas9 protein and guide
RNA complex were co-introduced into iPSCs, along with the
repair template, via electroporation. The correction of mutation in isolated clone was confirmed with sequencing. Also,
mice with heterozygous P2rx2 p.V60L mutation were analyzed by auditory brainstem response (ABR) and distortion
product otoacoustic emissions (DPOAEs) to assess the impact
of the mutant allele on PHL.
Results: First, we performed successful correction of the
P2RX2 p.V60L mutation using the CRISPR technique in
iPSCs. Second, we showed that heterozygous P2rx2 mice
have severe PHL. Older mice exhibited substantial elevation
in ABR and DPOAE thresholds.
Conclusions: In this study we created an in vitro model that
validates the role of CRISPR/Cas9 genome editing in the
reversal of dominant mutations in PHL. The heterozygous
knock in mice exhibit severe PHL and hence provide an excellent in vivo model to test the efficacy of CRISPR/Cas9 geneediting technology in rescue of auditory manifestation of the
P2RX2 p.V60L mutation.

P231
The Great Transformation of the Inner Ear:
From Schwann Cell to Neuron
Judith S. Kempfle, MD (Presenter); Ngoc-Nhi Luu, MD;
Marco Petrillo; Reef Al-Asad; Andrea Zhang;
Albert Edge, PhD
Objectives: Sensorineural hearing loss is irreversible and can
be caused by loss of auditory neurons. Regeneration of neurons
from Schwann cells may offer a future alternative to implantable hearing devices. Neurons and Schwann cells in the peripheral nervous system are closely related and originate from a
common progenitor. Prior work in our lab indicated that in the
postnatal mouse inner ear, proteolipid protein 1 (Plp1) expressing glial cells could turn into neurons in vitro.
Methods: To stimulate inner ear Schwann cells to turn into
neurons, we used a mouse model to transiently upregulate
Lin28, a key neural stem cell regulator, both in vitro and in
vivo after damage to auditory neurons.
Results: Gene expression analysis and immunohistochemistry revealed that Lin28 promoted proliferation and neural conversion of inner ear Schwann cells into neurons in vitro. In
vivo, we found that transient Lin28 overexpression in adult
Schwann cells after damage led to early expression of neural
stem cell markers and increased subsequent conversion of
inner ear Schwann into neurons.
Conclusions: Inner ear Schwann cells harbor the potential to
replace auditory neurons after damage and may be tapped for
future endogenous regeneration therapies.
Hearing Outcome Variability After Cochlear
Implantation for Single-Sided Deafness May
Be Related to Postoperative Test Strategies
Barry Kriegsman (Presenter); Marc Polanik;
Danielle R. Trakimas, MD; Julia Buirkle; Nicole Seymour;
Aaron K. Remenschneider, MD, MPH
Objectives: Cochlear implants (CIs) recently received Food
and Drug Administration approval for use in patients with
single-sided deafness (SSD). Postoperative speech perception-
testing protocols have not been standardized. Herein, we
examine a cohort of patients receiving CI for SSD using traditional contralateral masking tests and direct audio input (DAI)
of the implanted ear via remote microphone without masking.
Methods: Case review of individuals receiving CI for SSD at
an academic medical center from March 2017 to October 2019.
Pre-CI, patients were evaluated by speech audiometry, including
consonant-nucleus-consonant (CNC) whole word and AzBio
sentences. Post-CI, patients underwent identical tests with (1)
sound masking in the normal-hearing ear and (2) DAI via
MyLink, Roger Pen, or Mini Mic without contralateral masking.
Scores were assessed at the most recent audiology follow-up.
Results: Post-CI hearing was assessed at a mean of
11 months postactivation (range, 1-26 months). Eight of the

Otology/Neurotology

Poster Presentations	



Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts

Table of Contents for the Digital Edition of Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts

Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts
Contents
Accreditation Information
2020 President Duane J. Taylor, MD
2020 Annual Meeting Program Committee
2020 Star Reviewers
2020 Distinguished Service Award Recipients
2020 Honor Award Recipients
Honorary Guest Lecturers 2020
Panel Presentations
Oral Presentations
Poster Presentations
Author Index
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Intro
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Cover2
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - A1
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - A2
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - A3
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Contents
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - A5
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - A6
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Accreditation Information
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - 2020 President Duane J. Taylor, MD
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - 2020 Annual Meeting Program Committee
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P4
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - 2020 Star Reviewers
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - 2020 Honor Award Recipients
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P7
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Honorary Guest Lecturers 2020
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P9
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Panel Presentations
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Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Oral Presentations
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Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Poster Presentations
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Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P274
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P275
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P276
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P277
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P278
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P279
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P280
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P281
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P282
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P283
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P284
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P285
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P286
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P287
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P288
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P289
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P290
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P291
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P292
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P293
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P294
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P295
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P296
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P297
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P298
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P299
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P300
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P301
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P302
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P303
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P304
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P305
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P306
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P307
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P308
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P309
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P310
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P311
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P312
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Author Index
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P316
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P317
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P318
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P319
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P320
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P321
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P322
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P323
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P324
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P325
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P326
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P327
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P328
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P329
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P330
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P331
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - P332
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Cover3
Otolaryngology - Head & Neck Surgery Supplement - 2020 Official Program Abstracts - Cover4
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