# Modeling Neurofibromatosis Type 2 with Inner Ear Organoids

> **NIH NIH F30** · INDIANA UNIVERSITY INDIANAPOLIS · 2020 · $37,163

## Abstract

ABSTRACT
Neurofibromatosis Type 2 (NF2) is a genetic condition in which specific types of tumors form in various
locations throughout the body. Though the majority of these tumors grow slowly, the location of certain tumors
can cause significant dysfunction. One of these tumor types grows on the vestibular branch of cranial nerve
VIII, the vestibulocochlear nerve. The cells which gives rise to this tumor type is the Schwann cell, a glial cell
type. Thus, this tumor type is termed a vestibular Schwannoma (VS), a mass of Schwann cells on the
vestibular nerve. Due to the proximity of skull base cranial elements, a VS can cause dysfunction like hearing
loss, balance issues, or facial paralysis. The current treatment options are limited to invasive surgical
procedures which can create complications. Current knowledge of the mechanisms governing the development
and growth of VS is limited due to the fact that research has been restricted to mouse and simple human cell
culture systems. This proposed study will provide vertical impact by modeling NF2 in a new system, the inner
ear organoid. Inner ear organoid induction involves step-wise differentiation in a 3D culture system using
pluripotent stem cells. Inner ear organoids develop full sensory circuits of hair cells, sensory neurons, and
myelinating Schwann cells in vitro. We will create this model by introducing a genetic manipulation in our stem
cells which mimics the genetics of NF2 patients. In Aim 1, we will analyze biochemical pathways known to be
affected in NF2 patients in this new NF2 inner ear organoid. In Aim 2, we will evaluate druggable targets in
order to assess therapeutic effectiveness to counteract the introduced NF2 mutation. This study will provide
insight into the tumorigenic processes of VS.

## Key facts

- **NIH application ID:** 9991341
- **Project number:** 1F30DC018715-01
- **Recipient organization:** INDIANA UNIVERSITY INDIANAPOLIS
- **Principal Investigator:** Matthew Reed Steinhart
- **Activity code:** F30 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2020
- **Award amount:** $37,163
- **Award type:** 1
- **Project period:** 2020-01-31 → 2025-01-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/9991341

## Citation

> US National Institutes of Health, RePORTER application 9991341, Modeling Neurofibromatosis Type 2 with Inner Ear Organoids (1F30DC018715-01). Retrieved via AI Analytics 2026-05-22 from https://api.ai-analytics.org/grant/nih/9991341. Licensed CC0.

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