Transitional Intervention for Hyperacusis in Autism

The Hyperacusis Questionnaire is one of the earliest developed and most widely reported subjective tools for assessing supra-threshold auditory hypersensitivity in adults. It has been used both as a screening tool for hyperacusis and as an outcome measure of treatment-related change in hyperacusis severity. The HQ contains 14 items, each rated on a 4-point Likert scale with scores ranging from 0 (no) to 3 (yes, a lot). The total scoring range spans from 0 to 42 with attentional, social, and emotional subscales. Aazh and Moore (2017) proposed an HQ global score ≥ 22 as a cutoff indicator of a LH condition, with greater scores indicative of a greater problem.

The GÜF is a tool developed and validated in adult tinnitus patients. In this study, we will use the translation of the questionnaire from the original German to English. The GÜF was designed to evaluate subjective distress and annoyance associated with hypersensitivity to sound using 16 items, each rated on a 4-point Likert scale with scores ranging from 0 (never correct) to 3 (always correct). The GÜF total score range spans from 0 to 45, with four levels of distress (mild to severe) and three subscales (cognitive reactions to hyperacusis; actional/somatic behavior; and emotional reaction to external noises).

The Tampa Scale of Hyperacusis is an English version of the Tampa Scale for Kinesiophobia modified for Hyperacusis. The questionnaire was originally developed as a 17-item index of excessive, irrational, and debilitating fear of movement, physical activity, and fear avoidance related to (re)injury in chronic pain patients with musculoskeletal disorders. It was adapted for use as a subjective tool to assess pain of suffering due to sound exposure in adults. We have translated their Swedish version for use with English-speaking hyperacusis patients. It has 17-items and uses a 4-point response scale with scores ranging from 1 (strongly disagree) to 4 (strongly agree). Higher scores indicate hyperacusis of greater severity; the cutoff for a clinically meaningful condition is ≥ 37.

The Tinnitus Questionnaire is a 52-item questionnaire designed to establish tinnitus severity, a condition that is highly co-morbid with Loudness Hyperacusis. It consists of 52 items scored on a three-point Likert scale (0, 1, 2). The questions are grouped into five subscales: psychological distress, intrusiveness, hearing difficulties, sleep disturbances, and somatic complaints. This was the primary outcome measure in our successful Phase III NIH-sponsored clinical trial that investigated the efficacy of Tinnitus Retraining Therapy. The TQ was chosen for its excellent validity and generalizability properties. Higher TQ scores indicate greater tinnitus severity.

The Noise Avoidance Questionnaire assesses the avoidance of sound in daily life and those events and places avoided. The first 15 of the 25 items address avoidance behaviors using the introductory phrase "In order to expose myself to no or less noise…."; the remaining 10 items, 16 to 25, address the events and places avoided using the introductory phrase "In order to expose myself to no or less noise I avoid…". Each item is ranked on a 5-point Likert scale with scores ranging from 0 (never) to 4 (very often/always). Scores range from 0 to 100, with a higher score indicating a greater sound- or noise-related avoidance problem.

The Weinstein Noise Sensitivity Scale (WNSS-21) is designed to measure one's sensitivity to noise based on self-report. It consists of 21 items that probe reactions and attitudes related to environmental sounds encountered in everyday life. Items are scored on a 6-point Likert scale ranging from 0 (strong disagreement) to 5 (strong agreement). Total score ranges from 0 to 105 with a higher score indicating greater sensitivity to noise.

The Duke-Vanderbilt Misophonia Screening Questionnaire (DVMSQ) is a self-report measure of symptoms associated with misophonia (i.e., strong dislike, hatred, or annoyance of certain sounds). It consists of 19 items that reflect diagnostic criteria and takes into consideration definitions of misophonia. The first screening question evokes a "yes or "no" response. The second item is open-ended and allows the respondent to list sounds that are extremely bothersome, even when they are soft. Responses to the next 18 items are on a five-point Likert scale ranging from 0 (never) to 4 (very often). Scoring options include a total score (0 to 68, used in this trial), a symptom score (0 to 40), and an impairment score (0 to 28).

The DSM-5 Phonophobia Severity Scale is based on the DSM-5 Specific Phobia Severity Scale that has been modified for phonophobia. Specifically, the phrase prior to each question has been modified to read "Over the past seven days, how often have you experienced the following regarding situations when you are exposed to loud or unpleasant sounds? During the past 7 days I have:" This preamble is followed by 10 questions scored on a 5-point Likert scale ranging from 0 (never) to 4 (all of the time).

The PROMIS Global-10 quality of life questionnaire (PROMIS 10) is a 10-item tool commonly used in autism research with well-established psychometric properties and a simple software scoring system . Developed under the NIH PROMIS Initiative, the PROMIS-10 probes overall quality of life with sub-domains focused on physical and mental health, physical functioning, satisfaction and ability to carry out social activities, emotional distress, pain, and fatigue. The instrument has been tested and validated with adults and in several clinical populations including ASD. Scoring supports separate analyses of individual items as well as global physical health and global mental health domain scores. Quality of life is interpreted in terms of T-scores parsed into five categories (1) Poor (T-score <33.5), (2) Fair (T-score between 33.5 and 43.3), (3) Good (T-score between 43.4 and 53.3), (Very good (T-score between 53.4 and 64.5), and (5) Excellent (T-score >64.5).

The Social Responsiveness Scale-2 (SRS-2) was designed to measure the severity of autism symptoms and consists of 65 items parsed into five content areas: social awareness, social cognition, social communication, social motivation, and restricted interests and repetitive behavior. The instrument has been validated for use with adults. The SRS-2 domain scores include four categories: Within normal limits (≤ 49), Mild (60 to 65), Moderate (66 to 75), and Severe (≥ 76).

The Short Sensory Profile 2 (SSP-2) is a widely used instrument designed to classify the degree of sensory abnormality. It consists of 38 items broken into seven sensitivity subscales: tactile, taste/smell, movement, under-responsive/seeks sensation, auditory filtering, low energy/weak, and visual/auditory sensitivity.

The End of Intervention Survey is begins with an overview of the three components followed by 25 questions evaluating perceptions of potential strengths and weaknesses of the study components along with questions that would support computation of a net promoter score.

The EFR (Envelope Following Response) Growth Function is an index computed from an electroencephalography (EEG) recording designed as an objective measure to assess the change in neural response as sound level increases from low-to-high in a manner that is analogous to behavioral loudness growth tasks such as the Contour Test. By slowly increasing the intensity of the stimulus and adjusting the sound levels according to the participant's unique comfort levels, the protocol is designed to avoid causing discomfort while maximizing the amount of data acquired across the auditory dynamic range.