Co-Targeting the Dysregulated Ocular and Cochlear Blood Flow via Ophthalmic Nerve Stimulation for the Treatment of Type 2 Usher Syndrome: Prospective Case Series
- 1Musallam Eye Center of Jerusalem, Beit Hanina, Baldi Mall, B Building, Jerusalem 9160201, Israel
Abstract
Background/Objectives: Dysregulated ocular and cochlear blood flow are proposed pathways in the pathogenesis of type 2 Usher syndrome (USH2). This study aimed to evaluate the safety and preliminary efficacy of ophthalmic nerve stimulation (ONS) combined with intravenous ascorbic acid (AA) in patients with USH2. Methods: Nine participants with USH2 were enrolled in a prospective, open-label, interventional case series. All participants received daily ONS combined with intravenous AA over a two-week period. The primary endpoint was the 6-month change in self-reported scotopic function, assessed using the 10-item Low luminance questionnaire (LLQ-10; 0–100 scale). Responders were defined as participants demonstrating a ≥25-point increase in LLQ-10 score. Secondary outcomes included changes in best-corrected visual acuity (BCVA), contrast sensitivity (CS), and subjective hearing function. Results: ONS-based therapy resulted in a significant improvement in LLQ-10 scotopic scores at 6 months (mean change: +42.1 ± 11.3 points, p = 0.0001), with seven of nine participants (77.8%) classified as responders. Clinically meaningful improvements in BCVA (≥0.2 logMAR) and CS (≥0.3 log units) were observed in 22.2% of tested eyes. Additionally, subjective hearing improvement was reported by one-third (33.3%) of participants. No severe adverse events were reported. Conclusions: ONS combined with AA demonstrated safety and yielded significant subjective improvements in night vision quality among patients with USH2, alongside minor improvements in visual acuity, contrast sensitivity, and hearing in a subset of participants. Controlled trials incorporating objective scotopic and audiometric testing are warranted.
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BibTeX
@article{musallam2026cotargeting,
title = {{Co-Targeting the Dysregulated Ocular and Cochlear Blood Flow via Ophthalmic Nerve Stimulation for the Treatment of Type 2 Usher Syndrome: Prospective Case Series}},
author = {Ismail M. Musallam},
journal = {Journal of Clinical Medicine},
year = {2026},
month = oct,
doi = {10.3390/jcm15218302},
url = {https://www.mdpi.com/2077-0383/15/21/8302},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Co-Targeting the Dysregulated Ocular and Cochlear Blood Flow via Ophthalmic Nerve Stimulation for the Treatment of Type 2 Usher Syndrome: Prospective Case Series
AU - Ismail M. Musallam
PY - 2026
DA - 2026/10/25
JO - Journal of Clinical Medicine
DO - 10.3390/jcm15218302
UR - https://www.mdpi.com/2077-0383/15/21/8302
AB - Background/Objectives: Dysregulated ocular and cochlear blood flow are proposed pathways in the pathogenesis of type 2 Usher syndrome (USH2). This study aimed to evaluate the safety and preliminary efficacy of ophthalmic nerve stimulation (ONS) combined with intravenous ascorbic acid (AA) in patients with USH2. Methods: Nine participants with USH2 were enrolled in a prospective, open-label, interventional case series. All participants received daily ONS combined with intravenous AA over a two-week period. The primary endpoint was the 6-month change in self-reported scotopic function, assessed using the 10-item Low luminance questionnaire (LLQ-10; 0–100 scale). Responders were defined as participants demonstrating a ≥25-point increase in LLQ-10 score. Secondary outcomes included changes in best-corrected visual acuity (BCVA), contrast sensitivity (CS), and subjective hearing function. Results: ONS-based therapy resulted in a significant improvement in LLQ-10 scotopic scores at 6 months (mean change: +42.1 ± 11.3 points, p = 0.0001), with seven of nine participants (77.8%) classified as responders. Clinically meaningful improvements in BCVA (≥0.2 logMAR) and CS (≥0.3 log units) were observed in 22.2% of tested eyes. Additionally, subjective hearing improvement was reported by one-third (33.3%) of participants. No severe adverse events were reported. Conclusions: ONS combined with AA demonstrated safety and yielded significant subjective improvements in night vision quality among patients with USH2, alongside minor improvements in visual acuity, contrast sensitivity, and hearing in a subset of participants. Controlled trials incorporating objective scotopic and audiometric testing are warranted.
SN - 2077-0383
ER -