Investigation of Third Window Symptoms in Patients With Enlarged Vestibular Aqueduct

Sunday, September 29, 2024

10:18 AM – 10:24 AM EDT

Location: MBCC, 231

Disclosure(s):

Kimberley S. Noij, MD, PhD:

faculty for this accredited education activity has no relationship(s) with ineligible companies to disclose.

Nadia L. Samaha, BS:

faculty for this accredited education activity has no relationship(s) with ineligible companies to disclose.

Bryan K. Ward, MD:

faculty for this accredited education activity has no relationship(s) with ineligible companies to disclose.

John P. Carey, MD:

faculty for this accredited education activity has no relationship(s) with ineligible companies to disclose.

Introduction: Enlarged vestibular aqueduct (EVA) is considered a third mobile window syndrome. Research on the prevalence of vestibular symptoms in this patient population is limited, and it is unknown whether this patient population experiences symptoms characteristic of other third-window syndromes.

Methods: In this cross-sectional study, adult patients and parents of pediatric patients previously seen at a tertiary care institution and diagnosed with EVA were contacted between July and December 2023. Patients were asked to complete a survey regarding their symptoms. The survey included questions on typical third-window symptoms such as autophony, hyperacusis, and sound and pressure-induced dizziness. Adults were also asked to fill out the autophony and hyperacusis indices, while parents of pediatric EVA patients were asked additional questions about gross motor milestones.

Results: Of 121 patients who met inclusion criteria, 35 patients and parents filled out the questionnaire (14 children and 21 adults). Most patients (71%) had bilateral EVA. All patients suffered from hearing loss, which was progressive or fluctuating (67%) and sensorineural (61%) or mixed (35%) in the majority of patients. Among adults, tinnitus (86%) and ear fullness (62%) were common symptoms, while hyperacusis was common among children (64%). The autophony index (average: 4.4; 95% CI: 1.8-7.2) and hyperacusis scores (average: 7.8; 95% CI 4.4-11.3) were low. About half of all patients reported experiencing vertigo (54%) or poor balance (49%). Sound-induced dizziness was uncommon in both children (7%) and adults (14%), while pressure-induced dizziness was more common in adults (43%) than in children (7%; p = 0.028). Most pediatric patients with EVA had delayed gross motor milestones (71%).

Conclusions: This is the first study investigating the prevalence of typical third window symptoms in patients with EVA and found that about half of adult EVA patients suffer from typical third mobile window symptoms such as autophony and pressure-induced dizziness, which were uncommon among children with EVA.

Presenting Author(s)

Kimberley S. Noij, MD, PhD

Resident
Johns Hopkins University
Baltimore, MD, United States

Disclosure(s):
faculty for this accredited education activity has no relationship(s) with ineligible companies to disclose.

Co-Author(s)

Emily Y. Huang, BS

Medical Student
Johns Hopkins School of Medicine
Baltimore, MD, United States

Disclosure information not submitted.
Nadia L. Samaha, BS

Research Fellow
Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University
Baltimore, MD, United States

Disclosure(s):
faculty for this accredited education activity has no relationship(s) with ineligible companies to disclose.
BW

Bryan K. Ward, MD

Associate Professor
Johns Hopkins University School of Medicine
Baltimore, Maryland, United States

Disclosure(s):
faculty for this accredited education activity has no relationship(s) with ineligible companies to disclose.
JC

John P. Carey, MD

Professor
Johns Hopkins
Baltimore, Maryland, United States

Disclosure(s):
faculty for this accredited education activity has no relationship(s) with ineligible companies to disclose.

Senior Author(s)

CJ

Carolyn M. Jenks, MD

Assistant Professor
Johns Hopkins University
Baltimore, MD, United States

Disclosure information not submitted.