Unexpected ABR Results in Patients Suspected With Auditory Neuropathy Spectrum Disorder

Study Overview
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Brief Summary
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This study aimed to assess the clinical evaluations and potential underlying pathologies in patients diagnosed with Auditory Neuropathy Spectrum Disorder (ANSD) who exhibited unusual results in audiological tests. The research involved the retrospective analysis of audiological tests of 11 patients conducted by expert audiologists at Istanbul Medipol Mega Hospital between 2010 and 2020. These patients displayed abnormalities in their Auditory Brainstem Response (ABR) tests. The study sought to investigate patients with ANSD who presented atypical audiological findings, shedding light on the associated clinical aspects and potential causes of such anomalies.

Official Title
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Unexpected ABR Results in Patients Suspected With Auditory Neuropathy Spectrum Disorder

Conditions
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Auditory Neuropathy

Intervention / Treatment
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* Diagnostic Test: Hearing Thresholds Assessments


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Presence of wave V with cochlear microphonics Exclusion Criteria: Type B tympanogram

Ages Eligible for Study
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Minimum Age: 0 Years
Maximum Age: 46 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Diagnostic
Allocation: Randomized
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Intervention/Treatment |
| --- |
|Diagnostic Test: Hearing Thresholds Assessments|Auditory Neuropathy Spectrum Disorder (ANSD) is a type of hearing disorder that affects the transmission of sound signals from the inner ear to the brain. In individuals with ANSD, the outer hair cells in the cochlea, which are responsible for amplifying sound, typically function properly. However, there is a disruption in the transmission of auditory information from the cochlea to the auditory nerve and then to the brain. It is characterized by a mismatch between the results of objective hearing tests, such as Auditory Brainstem Response (ABR), and subjective hearing experiences. In ABR tests, the responses of the auditory nerve to sounds may be abnormal, while the individual perceives their hearing to be better than suggested by the test results. In this study we showed ANSD patients with abnormal ABR results by using ABR, Otoacoustic Emissions, Tympanometry and behavioral test batteries.|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Measuring Hearing Thresholds with Electrophysiological Tests | Participants' hearing thresholds and cochlear microphonics evaluated by using Auditory Brainstem Responses and Otoacoustic Emissions. | 6 years | 
| Evaluating Middle Ear Pathologies | Participants' middle ear pathologies evaluated by using Tympanometry | 6 years | 
| Evaluating Hearing Thresholds by Using Behavioral Tests | Participants' reactions to sounds were assessed using Free Field Audiometry or headphones. These evaluations were conducted initially at the time of diagnosis and subsequently after the patients had started using hearing aids. | 6 years |